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Progressive Widespread Telangiectasias
The Diagnosis: Cutaneous Collagenous Vasculopathy
Histopathologic examination revealed ectatic blood vessels lined with unremarkable endothelial cells and thickened, hyalinized vessel walls scattered within the papillary dermis (Figure 1). The epidermis was unremarkable. There was minimal associated inflammation and no extravasation of erythrocytes. The hyalinized material was weakly positive on periodic acid-Schiff staining (Figure 2) and negative on Congo red staining, which supported of a diagnosis of cutaneous collagenous vasculopathy (CCV).
The patient previously had been given a suspected diagnosis of generalized essential telangiectasia by an outside dermatologist several years prior to the current presentation, as CCV had yet to be recognized as its own entity and therefore few cases had been described in the literature. She had a known history of obesity, hypertension, hyperlipidemia, and type 2 diabetes mellitus, which are associated with the condition. Multiple specialists concluded that the disease was too extensive for laser treatment. A review of PubMed articles indexed for MEDLINE yielded no established treatment options.
Cutaneous collagenous vasculopathy is a rare acquired microangiopathy involving the small vessels of the skin. Its clinical presentation is indistinguishable from that of generalized essential telangiectasia (GET). Patients generally present with asymptomatic, widespread, blanching, symmetric telangiectasias that classically begin on the legs and steadily progress upward with classic sparing of the face (Figure 3). Whereas GET has been reported to involve the oral and conjunctival mucosa, mucosal involvement is not typically observed in CCV and is considered to be a distinguishing factor between the 2 conditions.1,2 However, our patient reported oral symptoms, and oral erosions were seen on multiple physical examinations; therefore, ours is a rare case of mucosal involvement in conjunction with CCV. Given this finding, it is possible that more cases of CCV with mucosal involvement may exist but have been clinically misdiagnosed as GET.
First described by Salama and Rosenthal3 in 2000, CCV remains a rarely reported entity, with approximately 33 reported cases in the worldwide literature.2,4-7 The condition typically arises in adults with an equal predilection for males and females.2 The true incidence of CCV is unknown and likely is underreported given its close similarities to GET, which often is diagnosed clinically. The unique histopathologic finding of superficial ectatic vascular spaces with eosinophilic hyalinized vessel walls in CCV is key to distinguishing these similar entities, and even this finding can be subtle and is easily overlooked. Inflammation is sparse to absent. Deposited material is positive on periodic acid-Schiff and cytokeratin IV staining (representing reduplicated basement membrane-type collagen) and is diastase resistant. Smooth muscle actin staining is diminished or absent. Ultrastructural examination reveals reduplicated, laminated basement membrane; Luse bodies (abnormally long, widely spaced collagen fibers); and a decrease in or loss of pericytes. Of note, Luse bodies are nonspecific and their absence does not exclude a diagnosis of CCV.1
The etiology of CCV is unclear, and multiple pathogenetic mechanisms have been proposed. Ultimately, this entity is thought to arise from repeated endothelial cell damage, although the trigger for the endothelial cell injury is not completely understood. Diabetes mellitus sometimes is associated with microangiopathy and may be a confounding but not causative factor in some cases.1 Some investigators believe CCV is caused by a genetic defect that alters collagen production in the small vessels of the skin.5 Others have hypothesized that it is a secondary manifestation of an underlying disease or is associated with a medication; however, no disease or drug has been convincingly implicated in CCV.8
Cutaneous collagenous vasculopathy is limited to the skin, with no known reports of systemic involvement in the literature.7 There are no recommended laboratory studies to aid in diagnosis.1 It is critical to exclude hereditary hemorrhagic telangiectasia (HHT), as these patients can have life-threatening systemic involvement. Patients with CCV generally have no history of a bleeding diathesis, patients with HHT classically report recurrent epistaxis and gastrointestinal bleeding.7 A family history of HHT also is helpful for diagnosis, as the condition is autosomal dominant.1 Neither HHT or telangiectasia macularis eruptiva perstans, which also can be included in the differential diagnosis, demonstrate vessel wall hyalinization.
Treatment options for CCV are limited. Basso et al6 reported notable improvement in a patient with CCV treated with a combined 595-nm pulsed dye laser and 1064-nm Nd:YAG laser and optimized pulsed light. In one patient, treatment with a 585-nm pulsed dye laser produced a blanching response, suggesting that this may be a potential treatment option.7 Treatment with sclerotherapy has been ineffective.2
It is critical for both dermatologists and dermatopathologists to recognize and report this newly described entity, as the unique finding of vessel wall hyalinization in CCV may be indicative of a certain pathogenetic mechanism and effective treatment avenue that has yet to be established due to the relatively few number of reports that currently exist in the literature.
- Burdick LM, Lohser S, Somach SC, et al. Cutaneous collagenous vasculopathy: a rare cutaneous microangiopathy. J Cutan Pathol. 2012;39:741-746.
- Brady BG, Ortleb M, Boyd AS, et al. Cutaneous collagenous vasculopathy. J Clin Aesthet Dermatol. 2015;8:49-52.
- Salama S, Rosenthal D. Cutaneous collagenous vasculopathy with generalized telangiectasia: an immunohistochemical and structural study. J Cutan Pathol. 2000;27:40-48.
- Toda-Brito H, Resende C, Catorze G, et al. Cutaneous collagenous vasculopathy: a rare cause of generalised cutaneous telangiectasia. BMJ Case Rep. 2015. doi: 10.1136/bcr-2015-210635.
- Ma DL, Vano-Galvan S. Images in clinical medicine: cutaneous collagenous vasculopathy. N Engl J Med. 2015;373:E14.
- Basso D, Ribero S, Blazek C, et al. Cutaneous collagenous vasculopathy: a rare form of microangiopathy successfully treated with a combination of multiplex laser and optimized pulsed light with a review of the literature. Dermatology. 2016;232:107-111.
- Moteqi SI, Yasuda M, Yamanaka M, et al. Cutaneous collagenous vasculopathy: report of first Japanese case and review of the literature. Australas J Dermatol. 2017;58:145-149.
- González Fernández D, Gómez Bernal S, Vivanco Allende B, et al. Cutaneous collagenous vasculopathy: description of two new cases in elderly women and review of the literature. Dermatology. 2012;225:1-8.
The Diagnosis: Cutaneous Collagenous Vasculopathy
Histopathologic examination revealed ectatic blood vessels lined with unremarkable endothelial cells and thickened, hyalinized vessel walls scattered within the papillary dermis (Figure 1). The epidermis was unremarkable. There was minimal associated inflammation and no extravasation of erythrocytes. The hyalinized material was weakly positive on periodic acid-Schiff staining (Figure 2) and negative on Congo red staining, which supported of a diagnosis of cutaneous collagenous vasculopathy (CCV).
The patient previously had been given a suspected diagnosis of generalized essential telangiectasia by an outside dermatologist several years prior to the current presentation, as CCV had yet to be recognized as its own entity and therefore few cases had been described in the literature. She had a known history of obesity, hypertension, hyperlipidemia, and type 2 diabetes mellitus, which are associated with the condition. Multiple specialists concluded that the disease was too extensive for laser treatment. A review of PubMed articles indexed for MEDLINE yielded no established treatment options.
Cutaneous collagenous vasculopathy is a rare acquired microangiopathy involving the small vessels of the skin. Its clinical presentation is indistinguishable from that of generalized essential telangiectasia (GET). Patients generally present with asymptomatic, widespread, blanching, symmetric telangiectasias that classically begin on the legs and steadily progress upward with classic sparing of the face (Figure 3). Whereas GET has been reported to involve the oral and conjunctival mucosa, mucosal involvement is not typically observed in CCV and is considered to be a distinguishing factor between the 2 conditions.1,2 However, our patient reported oral symptoms, and oral erosions were seen on multiple physical examinations; therefore, ours is a rare case of mucosal involvement in conjunction with CCV. Given this finding, it is possible that more cases of CCV with mucosal involvement may exist but have been clinically misdiagnosed as GET.
First described by Salama and Rosenthal3 in 2000, CCV remains a rarely reported entity, with approximately 33 reported cases in the worldwide literature.2,4-7 The condition typically arises in adults with an equal predilection for males and females.2 The true incidence of CCV is unknown and likely is underreported given its close similarities to GET, which often is diagnosed clinically. The unique histopathologic finding of superficial ectatic vascular spaces with eosinophilic hyalinized vessel walls in CCV is key to distinguishing these similar entities, and even this finding can be subtle and is easily overlooked. Inflammation is sparse to absent. Deposited material is positive on periodic acid-Schiff and cytokeratin IV staining (representing reduplicated basement membrane-type collagen) and is diastase resistant. Smooth muscle actin staining is diminished or absent. Ultrastructural examination reveals reduplicated, laminated basement membrane; Luse bodies (abnormally long, widely spaced collagen fibers); and a decrease in or loss of pericytes. Of note, Luse bodies are nonspecific and their absence does not exclude a diagnosis of CCV.1
The etiology of CCV is unclear, and multiple pathogenetic mechanisms have been proposed. Ultimately, this entity is thought to arise from repeated endothelial cell damage, although the trigger for the endothelial cell injury is not completely understood. Diabetes mellitus sometimes is associated with microangiopathy and may be a confounding but not causative factor in some cases.1 Some investigators believe CCV is caused by a genetic defect that alters collagen production in the small vessels of the skin.5 Others have hypothesized that it is a secondary manifestation of an underlying disease or is associated with a medication; however, no disease or drug has been convincingly implicated in CCV.8
Cutaneous collagenous vasculopathy is limited to the skin, with no known reports of systemic involvement in the literature.7 There are no recommended laboratory studies to aid in diagnosis.1 It is critical to exclude hereditary hemorrhagic telangiectasia (HHT), as these patients can have life-threatening systemic involvement. Patients with CCV generally have no history of a bleeding diathesis, patients with HHT classically report recurrent epistaxis and gastrointestinal bleeding.7 A family history of HHT also is helpful for diagnosis, as the condition is autosomal dominant.1 Neither HHT or telangiectasia macularis eruptiva perstans, which also can be included in the differential diagnosis, demonstrate vessel wall hyalinization.
Treatment options for CCV are limited. Basso et al6 reported notable improvement in a patient with CCV treated with a combined 595-nm pulsed dye laser and 1064-nm Nd:YAG laser and optimized pulsed light. In one patient, treatment with a 585-nm pulsed dye laser produced a blanching response, suggesting that this may be a potential treatment option.7 Treatment with sclerotherapy has been ineffective.2
It is critical for both dermatologists and dermatopathologists to recognize and report this newly described entity, as the unique finding of vessel wall hyalinization in CCV may be indicative of a certain pathogenetic mechanism and effective treatment avenue that has yet to be established due to the relatively few number of reports that currently exist in the literature.
The Diagnosis: Cutaneous Collagenous Vasculopathy
Histopathologic examination revealed ectatic blood vessels lined with unremarkable endothelial cells and thickened, hyalinized vessel walls scattered within the papillary dermis (Figure 1). The epidermis was unremarkable. There was minimal associated inflammation and no extravasation of erythrocytes. The hyalinized material was weakly positive on periodic acid-Schiff staining (Figure 2) and negative on Congo red staining, which supported of a diagnosis of cutaneous collagenous vasculopathy (CCV).
The patient previously had been given a suspected diagnosis of generalized essential telangiectasia by an outside dermatologist several years prior to the current presentation, as CCV had yet to be recognized as its own entity and therefore few cases had been described in the literature. She had a known history of obesity, hypertension, hyperlipidemia, and type 2 diabetes mellitus, which are associated with the condition. Multiple specialists concluded that the disease was too extensive for laser treatment. A review of PubMed articles indexed for MEDLINE yielded no established treatment options.
Cutaneous collagenous vasculopathy is a rare acquired microangiopathy involving the small vessels of the skin. Its clinical presentation is indistinguishable from that of generalized essential telangiectasia (GET). Patients generally present with asymptomatic, widespread, blanching, symmetric telangiectasias that classically begin on the legs and steadily progress upward with classic sparing of the face (Figure 3). Whereas GET has been reported to involve the oral and conjunctival mucosa, mucosal involvement is not typically observed in CCV and is considered to be a distinguishing factor between the 2 conditions.1,2 However, our patient reported oral symptoms, and oral erosions were seen on multiple physical examinations; therefore, ours is a rare case of mucosal involvement in conjunction with CCV. Given this finding, it is possible that more cases of CCV with mucosal involvement may exist but have been clinically misdiagnosed as GET.
First described by Salama and Rosenthal3 in 2000, CCV remains a rarely reported entity, with approximately 33 reported cases in the worldwide literature.2,4-7 The condition typically arises in adults with an equal predilection for males and females.2 The true incidence of CCV is unknown and likely is underreported given its close similarities to GET, which often is diagnosed clinically. The unique histopathologic finding of superficial ectatic vascular spaces with eosinophilic hyalinized vessel walls in CCV is key to distinguishing these similar entities, and even this finding can be subtle and is easily overlooked. Inflammation is sparse to absent. Deposited material is positive on periodic acid-Schiff and cytokeratin IV staining (representing reduplicated basement membrane-type collagen) and is diastase resistant. Smooth muscle actin staining is diminished or absent. Ultrastructural examination reveals reduplicated, laminated basement membrane; Luse bodies (abnormally long, widely spaced collagen fibers); and a decrease in or loss of pericytes. Of note, Luse bodies are nonspecific and their absence does not exclude a diagnosis of CCV.1
The etiology of CCV is unclear, and multiple pathogenetic mechanisms have been proposed. Ultimately, this entity is thought to arise from repeated endothelial cell damage, although the trigger for the endothelial cell injury is not completely understood. Diabetes mellitus sometimes is associated with microangiopathy and may be a confounding but not causative factor in some cases.1 Some investigators believe CCV is caused by a genetic defect that alters collagen production in the small vessels of the skin.5 Others have hypothesized that it is a secondary manifestation of an underlying disease or is associated with a medication; however, no disease or drug has been convincingly implicated in CCV.8
Cutaneous collagenous vasculopathy is limited to the skin, with no known reports of systemic involvement in the literature.7 There are no recommended laboratory studies to aid in diagnosis.1 It is critical to exclude hereditary hemorrhagic telangiectasia (HHT), as these patients can have life-threatening systemic involvement. Patients with CCV generally have no history of a bleeding diathesis, patients with HHT classically report recurrent epistaxis and gastrointestinal bleeding.7 A family history of HHT also is helpful for diagnosis, as the condition is autosomal dominant.1 Neither HHT or telangiectasia macularis eruptiva perstans, which also can be included in the differential diagnosis, demonstrate vessel wall hyalinization.
Treatment options for CCV are limited. Basso et al6 reported notable improvement in a patient with CCV treated with a combined 595-nm pulsed dye laser and 1064-nm Nd:YAG laser and optimized pulsed light. In one patient, treatment with a 585-nm pulsed dye laser produced a blanching response, suggesting that this may be a potential treatment option.7 Treatment with sclerotherapy has been ineffective.2
It is critical for both dermatologists and dermatopathologists to recognize and report this newly described entity, as the unique finding of vessel wall hyalinization in CCV may be indicative of a certain pathogenetic mechanism and effective treatment avenue that has yet to be established due to the relatively few number of reports that currently exist in the literature.
- Burdick LM, Lohser S, Somach SC, et al. Cutaneous collagenous vasculopathy: a rare cutaneous microangiopathy. J Cutan Pathol. 2012;39:741-746.
- Brady BG, Ortleb M, Boyd AS, et al. Cutaneous collagenous vasculopathy. J Clin Aesthet Dermatol. 2015;8:49-52.
- Salama S, Rosenthal D. Cutaneous collagenous vasculopathy with generalized telangiectasia: an immunohistochemical and structural study. J Cutan Pathol. 2000;27:40-48.
- Toda-Brito H, Resende C, Catorze G, et al. Cutaneous collagenous vasculopathy: a rare cause of generalised cutaneous telangiectasia. BMJ Case Rep. 2015. doi: 10.1136/bcr-2015-210635.
- Ma DL, Vano-Galvan S. Images in clinical medicine: cutaneous collagenous vasculopathy. N Engl J Med. 2015;373:E14.
- Basso D, Ribero S, Blazek C, et al. Cutaneous collagenous vasculopathy: a rare form of microangiopathy successfully treated with a combination of multiplex laser and optimized pulsed light with a review of the literature. Dermatology. 2016;232:107-111.
- Moteqi SI, Yasuda M, Yamanaka M, et al. Cutaneous collagenous vasculopathy: report of first Japanese case and review of the literature. Australas J Dermatol. 2017;58:145-149.
- González Fernández D, Gómez Bernal S, Vivanco Allende B, et al. Cutaneous collagenous vasculopathy: description of two new cases in elderly women and review of the literature. Dermatology. 2012;225:1-8.
- Burdick LM, Lohser S, Somach SC, et al. Cutaneous collagenous vasculopathy: a rare cutaneous microangiopathy. J Cutan Pathol. 2012;39:741-746.
- Brady BG, Ortleb M, Boyd AS, et al. Cutaneous collagenous vasculopathy. J Clin Aesthet Dermatol. 2015;8:49-52.
- Salama S, Rosenthal D. Cutaneous collagenous vasculopathy with generalized telangiectasia: an immunohistochemical and structural study. J Cutan Pathol. 2000;27:40-48.
- Toda-Brito H, Resende C, Catorze G, et al. Cutaneous collagenous vasculopathy: a rare cause of generalised cutaneous telangiectasia. BMJ Case Rep. 2015. doi: 10.1136/bcr-2015-210635.
- Ma DL, Vano-Galvan S. Images in clinical medicine: cutaneous collagenous vasculopathy. N Engl J Med. 2015;373:E14.
- Basso D, Ribero S, Blazek C, et al. Cutaneous collagenous vasculopathy: a rare form of microangiopathy successfully treated with a combination of multiplex laser and optimized pulsed light with a review of the literature. Dermatology. 2016;232:107-111.
- Moteqi SI, Yasuda M, Yamanaka M, et al. Cutaneous collagenous vasculopathy: report of first Japanese case and review of the literature. Australas J Dermatol. 2017;58:145-149.
- González Fernández D, Gómez Bernal S, Vivanco Allende B, et al. Cutaneous collagenous vasculopathy: description of two new cases in elderly women and review of the literature. Dermatology. 2012;225:1-8.
A 55-year-old woman presented for evaluation of widespread asymptomatic telangiectasias of several years' duration that first appeared on the legs and steadily progressed to involve the trunk and arms. A review of systems was remarkable for episodic glossitis and oral erosions that developed at the same time as the eruption. The patient had no history of bleeding diasthesis, and her family history was unremarkable. A laboratory workup (including autoimmune screening) and a malignancy workup were negative. Physical examination revealed confluent sheets of erythematous and purple blanching telangiectasias scattered symmetrically on the trunk, bilateral arms and legs, buttocks, and dorsal aspects of the feet with sparing of the palms, soles, and head and neck regions. A small, shallow erosion was present on the lateral aspect of the tongue. A 4-mm punch biopsy of a thigh lesion revealed ectatic blood vessels with hyalinized walls.
Keep pushing the envelope
By the time this column is published, we will have wrapped up Hospital Medicine 2018 in Orlando, it will be well into spring, and I will have completed my year as past president as well as my 6-year tenure on the Society of Hospital Medicine board of directors.
I can imagine that will feel like a relief and a milestone, and it also will feel like a loss to no longer be part of something that I have contributed my time, energy, passion, and emotion to for so long. I will retire at the ripe age of 48 – a pretty typical age for ending SHM board tenure, and it’s terribly important for SHM that I do so.
If you attended HM18, I hope you appreciated, as I do every year, the energy, enthusiasm, and youth – if not in years, then in spirit – of the event and of hospitalists. As a society and a profession, we take risks. We have set standards for excellence in hospital medicine programs. We have recognized a unique set of competencies and then not only attempted to expand them with education but also defined a specialty around them. We have welcomed practitioners and administrators as equals into our fold. These and many other accomplishments are the work of a board, committees, chapter leaders, and members who look for opportunity to expand our work into new and necessary domains, and not be limited by precedent.
On the SHM board and committees, we tackle issues of governance and strategy. For most of us, the SHM board is our first exposure to nonprofit oversight. And, to be sure, there is a steep learning curve as new members discover the issues and substance of the work of the society. I recall that I barely spoke the first year on the board, uncertain that I understood items fully, and I also was burned once or twice by making suggestions that reflected my lack of knowledge. While ignorance slowly gave way to experience, we also matured as a group as we found ways to debate and resolve tough, sometimes ambiguous, issues.
I came to appreciate that the strength of the board – and of SHM – is that we join the board naive to much of the past. After 6 years, while I may have come to understand issues with greater depth, I also see that the newer members bring fresher thinking, more creative energy, and even thoughts about how the group could function differently and perhaps better. Over the last few years, I realized that we veterans had developed a cadence and predictability to our work, and every year’s new members disrupt that rhythm. This disruption forces us to challenge each other and to be a better board – and hopefully – represent and advocate for you, our membership, better.
So, it’s time for me to move on. Even though I certainly feel like I still could contribute, it’s time to retire my own way of thinking from the leadership of SHM. The fact that we term-limit out at a (relatively) young age is, I believe, an extraordinary aspect of our organization, which is reflected in the work that our staff, our committees, and our members do.
SHM is an organization that, from the top down, embraces change in ways that few other organizations do. I believe we owe it to you to keep pushing the envelope of creativity – of what our goals are, of what a society can accomplish, of what an annual meeting can consist of. My ask of all of you is that you continue to challenge the leadership of SHM to be disruptive, to push the profession to better places, and to always strive to be more diverse, more inclusive, more communicative, more visible – and to stay young. In spirit and attitude if not in age. Thank you for giving me the opportunity to work on your behalf. It has been the greatest privilege of my career.
Dr. Harte is a past president of SHM and president of Cleveland Clinic Akron General and Southern Region.
By the time this column is published, we will have wrapped up Hospital Medicine 2018 in Orlando, it will be well into spring, and I will have completed my year as past president as well as my 6-year tenure on the Society of Hospital Medicine board of directors.
I can imagine that will feel like a relief and a milestone, and it also will feel like a loss to no longer be part of something that I have contributed my time, energy, passion, and emotion to for so long. I will retire at the ripe age of 48 – a pretty typical age for ending SHM board tenure, and it’s terribly important for SHM that I do so.
If you attended HM18, I hope you appreciated, as I do every year, the energy, enthusiasm, and youth – if not in years, then in spirit – of the event and of hospitalists. As a society and a profession, we take risks. We have set standards for excellence in hospital medicine programs. We have recognized a unique set of competencies and then not only attempted to expand them with education but also defined a specialty around them. We have welcomed practitioners and administrators as equals into our fold. These and many other accomplishments are the work of a board, committees, chapter leaders, and members who look for opportunity to expand our work into new and necessary domains, and not be limited by precedent.
On the SHM board and committees, we tackle issues of governance and strategy. For most of us, the SHM board is our first exposure to nonprofit oversight. And, to be sure, there is a steep learning curve as new members discover the issues and substance of the work of the society. I recall that I barely spoke the first year on the board, uncertain that I understood items fully, and I also was burned once or twice by making suggestions that reflected my lack of knowledge. While ignorance slowly gave way to experience, we also matured as a group as we found ways to debate and resolve tough, sometimes ambiguous, issues.
I came to appreciate that the strength of the board – and of SHM – is that we join the board naive to much of the past. After 6 years, while I may have come to understand issues with greater depth, I also see that the newer members bring fresher thinking, more creative energy, and even thoughts about how the group could function differently and perhaps better. Over the last few years, I realized that we veterans had developed a cadence and predictability to our work, and every year’s new members disrupt that rhythm. This disruption forces us to challenge each other and to be a better board – and hopefully – represent and advocate for you, our membership, better.
So, it’s time for me to move on. Even though I certainly feel like I still could contribute, it’s time to retire my own way of thinking from the leadership of SHM. The fact that we term-limit out at a (relatively) young age is, I believe, an extraordinary aspect of our organization, which is reflected in the work that our staff, our committees, and our members do.
SHM is an organization that, from the top down, embraces change in ways that few other organizations do. I believe we owe it to you to keep pushing the envelope of creativity – of what our goals are, of what a society can accomplish, of what an annual meeting can consist of. My ask of all of you is that you continue to challenge the leadership of SHM to be disruptive, to push the profession to better places, and to always strive to be more diverse, more inclusive, more communicative, more visible – and to stay young. In spirit and attitude if not in age. Thank you for giving me the opportunity to work on your behalf. It has been the greatest privilege of my career.
Dr. Harte is a past president of SHM and president of Cleveland Clinic Akron General and Southern Region.
By the time this column is published, we will have wrapped up Hospital Medicine 2018 in Orlando, it will be well into spring, and I will have completed my year as past president as well as my 6-year tenure on the Society of Hospital Medicine board of directors.
I can imagine that will feel like a relief and a milestone, and it also will feel like a loss to no longer be part of something that I have contributed my time, energy, passion, and emotion to for so long. I will retire at the ripe age of 48 – a pretty typical age for ending SHM board tenure, and it’s terribly important for SHM that I do so.
If you attended HM18, I hope you appreciated, as I do every year, the energy, enthusiasm, and youth – if not in years, then in spirit – of the event and of hospitalists. As a society and a profession, we take risks. We have set standards for excellence in hospital medicine programs. We have recognized a unique set of competencies and then not only attempted to expand them with education but also defined a specialty around them. We have welcomed practitioners and administrators as equals into our fold. These and many other accomplishments are the work of a board, committees, chapter leaders, and members who look for opportunity to expand our work into new and necessary domains, and not be limited by precedent.
On the SHM board and committees, we tackle issues of governance and strategy. For most of us, the SHM board is our first exposure to nonprofit oversight. And, to be sure, there is a steep learning curve as new members discover the issues and substance of the work of the society. I recall that I barely spoke the first year on the board, uncertain that I understood items fully, and I also was burned once or twice by making suggestions that reflected my lack of knowledge. While ignorance slowly gave way to experience, we also matured as a group as we found ways to debate and resolve tough, sometimes ambiguous, issues.
I came to appreciate that the strength of the board – and of SHM – is that we join the board naive to much of the past. After 6 years, while I may have come to understand issues with greater depth, I also see that the newer members bring fresher thinking, more creative energy, and even thoughts about how the group could function differently and perhaps better. Over the last few years, I realized that we veterans had developed a cadence and predictability to our work, and every year’s new members disrupt that rhythm. This disruption forces us to challenge each other and to be a better board – and hopefully – represent and advocate for you, our membership, better.
So, it’s time for me to move on. Even though I certainly feel like I still could contribute, it’s time to retire my own way of thinking from the leadership of SHM. The fact that we term-limit out at a (relatively) young age is, I believe, an extraordinary aspect of our organization, which is reflected in the work that our staff, our committees, and our members do.
SHM is an organization that, from the top down, embraces change in ways that few other organizations do. I believe we owe it to you to keep pushing the envelope of creativity – of what our goals are, of what a society can accomplish, of what an annual meeting can consist of. My ask of all of you is that you continue to challenge the leadership of SHM to be disruptive, to push the profession to better places, and to always strive to be more diverse, more inclusive, more communicative, more visible – and to stay young. In spirit and attitude if not in age. Thank you for giving me the opportunity to work on your behalf. It has been the greatest privilege of my career.
Dr. Harte is a past president of SHM and president of Cleveland Clinic Akron General and Southern Region.
Check SVS Website for New Research Opportunities
Looking for a research opportunity? Check our updated website for current programs in your area. If your institution has an opportunity to promote, let us know at communications@vascularsociety.org.
Looking for a research opportunity? Check our updated website for current programs in your area. If your institution has an opportunity to promote, let us know at communications@vascularsociety.org.
Looking for a research opportunity? Check our updated website for current programs in your area. If your institution has an opportunity to promote, let us know at communications@vascularsociety.org.
Submit a case to VAM's “Ask the Experts"
Help build the Vascular Annual Meeting educational program for the new “Ask the Experts” sessions, to be held daily, Wednesday through Saturday. Topics are coding, aortic care for occlusive disease, hemodialysis and PAD. Learn more about case submission here.
And if you haven’t registered for VAM yet, do so! The premier meeting for vascular surgeons is just five weeks (and one day) away. Learn more and register here. And obtain a hotel room here.
Help build the Vascular Annual Meeting educational program for the new “Ask the Experts” sessions, to be held daily, Wednesday through Saturday. Topics are coding, aortic care for occlusive disease, hemodialysis and PAD. Learn more about case submission here.
And if you haven’t registered for VAM yet, do so! The premier meeting for vascular surgeons is just five weeks (and one day) away. Learn more and register here. And obtain a hotel room here.
Help build the Vascular Annual Meeting educational program for the new “Ask the Experts” sessions, to be held daily, Wednesday through Saturday. Topics are coding, aortic care for occlusive disease, hemodialysis and PAD. Learn more about case submission here.
And if you haven’t registered for VAM yet, do so! The premier meeting for vascular surgeons is just five weeks (and one day) away. Learn more and register here. And obtain a hotel room here.
For Members Only: View “Negotiations” Webinar Materials
Did you miss the April 30 webinar on "Negotiating Physician Employment Contracts," presented by the SVS and the SVS Community Practice Committee? Materials -- available only to SVS members -- can be viewed here. Topics include benefits, call pay, termination rights, non-compete clauses, tenure opportunities and more.
Did you miss the April 30 webinar on "Negotiating Physician Employment Contracts," presented by the SVS and the SVS Community Practice Committee? Materials -- available only to SVS members -- can be viewed here. Topics include benefits, call pay, termination rights, non-compete clauses, tenure opportunities and more.
Did you miss the April 30 webinar on "Negotiating Physician Employment Contracts," presented by the SVS and the SVS Community Practice Committee? Materials -- available only to SVS members -- can be viewed here. Topics include benefits, call pay, termination rights, non-compete clauses, tenure opportunities and more.
Device-related thrombus associated with ischemic events
BOSTON – Device-related thrombus (DRT) does not occur often after left atrial appendage closure with the Watchman device. When it does, however, it is associated with a significantly higher rate of stroke and systemic embolism compared with that of patients with no DRT, according to a recent analysis presented at the annual scientific sessions of the Heart Rhythm Society.
Given the negative implications of DRT, a judicious surveillance strategy should be considered, especially when DRT risk factors are present, investigators said in a report on the study, which was published simultaneously in Circulation.
“Certainly, DRT is associated with an increased risk of stroke, and therapeutic anticoagulation should be resumed when discovered with rigorous transesophageal echocardiography follow-up to ensure resolution,” noted senior investigator Vivek Y. Reddy, MD.
Despite the higher rates of all strokes, ischemic strokes, and hemorrhagic strokes linked with DRT, the complication did not link with a higher rate of all-cause mortality compared with patients who never had a DRT. The results also suggested a causal link between DRT and subsequent stroke in about half the patients with DRT because their strokes occurred within a month following DRT diagnosis.
Despite these findings, a majority – 74% – of patients with an identified DRT did not have a stroke, and 87% of the strokes that occurred in the patients who received the Watchman device occurred in the absence of a DRT, reported Dr. Reddy, who presented the findings at the meeting.
The most immediate implication of the findings is the strong case they make for rethinking the timing of planned follow-up transesophageal echocardiography (TEE) examinations of patients after they receive a Watchman device. The current, standard protocol schedules a TEE at 45 days after Watchman placement, when routine anticoagulation usually stops, and then a second TEE 12 months after placement. A better schedule might be to perform the first TEE 3-4 months after Watchman placement to give a potential DRT time to form once oral anticoagulant therapy stops, suggested Dr. Reddy, professor and director of the cardiac arrhythmia service at Mount Sinai Hospital and Health System in New York.
“Surveillance is very important. I don’t think DRT usually occurs unless anticoagulation is suboptimal or stops.” Dr. Reddy noted that he and his associates are analyzing the best time for TEE surveillance in other large databases of patients treated with left atrial appendage (LAA) closure. Newer models of LAA closure devices structurally modified to reduce thrombus formation are nearing clinical use, he added.
The analysis, believed to be the largest to date of DRT following left atrial appendage closure, was based on prospective data from four clinical trials. That included two randomized controlled trials, PROTECT AF and PREVAIL, as well as the CAP and CAP2 prospective registries.
Among 1,739 patients in those studies receiving an implant, 65 (3.74%) had DRT, the investigators found.
Over 1 year of follow-up, 25% of patients with DRT had an ischemic stroke or systemic embolism, versus 6.8% of patients without DRTs (P less than .001), they reported. That worked out to an event rate of 6.28 and 1.65 events per 100 patient years, respectively.
The strongest predictors of DRT in multivariable analysis included vascular disease, history of stroke or transient ischemic attack, permanent atrial fibrillation, and left atrial appendage diameter, according to the report. Conversely, increasing left ventricular ejection fraction was protective against DRT.
Taken together, these data support reevaluating the transesophageal echocardiography strategy, according to Dr. Reddy and his coauthors. Those approaches might include targeting patients with DRT risk factors, routine additional surveillance at 6 months, or delaying the first transesophageal echocardiography to 4 months.
“Importantly, none of these strategies have been rigorously compared, so these suggestions are subject to future studies,” the researchers wrote.
“DRT remains a problem despite increased operator experience with LAA occlusion and improved occluding devices,” commented David B. De Lurgio, MD, a cardiac electrophysiologist at Emory Healthcare in Atlanta. “What is a little alarming is that the risk for DRT extends beyond the period of prescribed anticoagulation. Although the risk from DRT mitigates the benefit of LAA closure compared with warfarin, it does not mitigate the benefit from occlusion compared with no treatment,” said Dr. De Lurgio, designated discussant for the report. “Prevention and management of DRT may require that each patient receive a tailored regimen of anticoagulation and surveillance.”
The Watchman studies were funded by Boston Scientific, the company that markets the device. Dr. Reddy has been a consultant to and has received research funding from Boston Scientific and from Abbott and Biosense-Webster, and he reported having an equity interest in Javelin and Surecor. Coauthors reported disclosures related to Boston Scientific, Johnson & Johnson, Abbott, and other medical device companies. Dr. De Lurgio has been a consultant to Boston Scientific.
Updated, 5/17/18: This article has been updated with reporting from Mitchel L. Zoler at the meeting, and has been revised for clarity and to reflect that the results were presented by Dr. Reddy.
SOURCE: Dukkipati SR et al. Circulation. 2018 May 11. doi: 10.1161/CIRCULATIONAHA.118.035090.
BOSTON – Device-related thrombus (DRT) does not occur often after left atrial appendage closure with the Watchman device. When it does, however, it is associated with a significantly higher rate of stroke and systemic embolism compared with that of patients with no DRT, according to a recent analysis presented at the annual scientific sessions of the Heart Rhythm Society.
Given the negative implications of DRT, a judicious surveillance strategy should be considered, especially when DRT risk factors are present, investigators said in a report on the study, which was published simultaneously in Circulation.
“Certainly, DRT is associated with an increased risk of stroke, and therapeutic anticoagulation should be resumed when discovered with rigorous transesophageal echocardiography follow-up to ensure resolution,” noted senior investigator Vivek Y. Reddy, MD.
Despite the higher rates of all strokes, ischemic strokes, and hemorrhagic strokes linked with DRT, the complication did not link with a higher rate of all-cause mortality compared with patients who never had a DRT. The results also suggested a causal link between DRT and subsequent stroke in about half the patients with DRT because their strokes occurred within a month following DRT diagnosis.
Despite these findings, a majority – 74% – of patients with an identified DRT did not have a stroke, and 87% of the strokes that occurred in the patients who received the Watchman device occurred in the absence of a DRT, reported Dr. Reddy, who presented the findings at the meeting.
The most immediate implication of the findings is the strong case they make for rethinking the timing of planned follow-up transesophageal echocardiography (TEE) examinations of patients after they receive a Watchman device. The current, standard protocol schedules a TEE at 45 days after Watchman placement, when routine anticoagulation usually stops, and then a second TEE 12 months after placement. A better schedule might be to perform the first TEE 3-4 months after Watchman placement to give a potential DRT time to form once oral anticoagulant therapy stops, suggested Dr. Reddy, professor and director of the cardiac arrhythmia service at Mount Sinai Hospital and Health System in New York.
“Surveillance is very important. I don’t think DRT usually occurs unless anticoagulation is suboptimal or stops.” Dr. Reddy noted that he and his associates are analyzing the best time for TEE surveillance in other large databases of patients treated with left atrial appendage (LAA) closure. Newer models of LAA closure devices structurally modified to reduce thrombus formation are nearing clinical use, he added.
The analysis, believed to be the largest to date of DRT following left atrial appendage closure, was based on prospective data from four clinical trials. That included two randomized controlled trials, PROTECT AF and PREVAIL, as well as the CAP and CAP2 prospective registries.
Among 1,739 patients in those studies receiving an implant, 65 (3.74%) had DRT, the investigators found.
Over 1 year of follow-up, 25% of patients with DRT had an ischemic stroke or systemic embolism, versus 6.8% of patients without DRTs (P less than .001), they reported. That worked out to an event rate of 6.28 and 1.65 events per 100 patient years, respectively.
The strongest predictors of DRT in multivariable analysis included vascular disease, history of stroke or transient ischemic attack, permanent atrial fibrillation, and left atrial appendage diameter, according to the report. Conversely, increasing left ventricular ejection fraction was protective against DRT.
Taken together, these data support reevaluating the transesophageal echocardiography strategy, according to Dr. Reddy and his coauthors. Those approaches might include targeting patients with DRT risk factors, routine additional surveillance at 6 months, or delaying the first transesophageal echocardiography to 4 months.
“Importantly, none of these strategies have been rigorously compared, so these suggestions are subject to future studies,” the researchers wrote.
“DRT remains a problem despite increased operator experience with LAA occlusion and improved occluding devices,” commented David B. De Lurgio, MD, a cardiac electrophysiologist at Emory Healthcare in Atlanta. “What is a little alarming is that the risk for DRT extends beyond the period of prescribed anticoagulation. Although the risk from DRT mitigates the benefit of LAA closure compared with warfarin, it does not mitigate the benefit from occlusion compared with no treatment,” said Dr. De Lurgio, designated discussant for the report. “Prevention and management of DRT may require that each patient receive a tailored regimen of anticoagulation and surveillance.”
The Watchman studies were funded by Boston Scientific, the company that markets the device. Dr. Reddy has been a consultant to and has received research funding from Boston Scientific and from Abbott and Biosense-Webster, and he reported having an equity interest in Javelin and Surecor. Coauthors reported disclosures related to Boston Scientific, Johnson & Johnson, Abbott, and other medical device companies. Dr. De Lurgio has been a consultant to Boston Scientific.
Updated, 5/17/18: This article has been updated with reporting from Mitchel L. Zoler at the meeting, and has been revised for clarity and to reflect that the results were presented by Dr. Reddy.
SOURCE: Dukkipati SR et al. Circulation. 2018 May 11. doi: 10.1161/CIRCULATIONAHA.118.035090.
BOSTON – Device-related thrombus (DRT) does not occur often after left atrial appendage closure with the Watchman device. When it does, however, it is associated with a significantly higher rate of stroke and systemic embolism compared with that of patients with no DRT, according to a recent analysis presented at the annual scientific sessions of the Heart Rhythm Society.
Given the negative implications of DRT, a judicious surveillance strategy should be considered, especially when DRT risk factors are present, investigators said in a report on the study, which was published simultaneously in Circulation.
“Certainly, DRT is associated with an increased risk of stroke, and therapeutic anticoagulation should be resumed when discovered with rigorous transesophageal echocardiography follow-up to ensure resolution,” noted senior investigator Vivek Y. Reddy, MD.
Despite the higher rates of all strokes, ischemic strokes, and hemorrhagic strokes linked with DRT, the complication did not link with a higher rate of all-cause mortality compared with patients who never had a DRT. The results also suggested a causal link between DRT and subsequent stroke in about half the patients with DRT because their strokes occurred within a month following DRT diagnosis.
Despite these findings, a majority – 74% – of patients with an identified DRT did not have a stroke, and 87% of the strokes that occurred in the patients who received the Watchman device occurred in the absence of a DRT, reported Dr. Reddy, who presented the findings at the meeting.
The most immediate implication of the findings is the strong case they make for rethinking the timing of planned follow-up transesophageal echocardiography (TEE) examinations of patients after they receive a Watchman device. The current, standard protocol schedules a TEE at 45 days after Watchman placement, when routine anticoagulation usually stops, and then a second TEE 12 months after placement. A better schedule might be to perform the first TEE 3-4 months after Watchman placement to give a potential DRT time to form once oral anticoagulant therapy stops, suggested Dr. Reddy, professor and director of the cardiac arrhythmia service at Mount Sinai Hospital and Health System in New York.
“Surveillance is very important. I don’t think DRT usually occurs unless anticoagulation is suboptimal or stops.” Dr. Reddy noted that he and his associates are analyzing the best time for TEE surveillance in other large databases of patients treated with left atrial appendage (LAA) closure. Newer models of LAA closure devices structurally modified to reduce thrombus formation are nearing clinical use, he added.
The analysis, believed to be the largest to date of DRT following left atrial appendage closure, was based on prospective data from four clinical trials. That included two randomized controlled trials, PROTECT AF and PREVAIL, as well as the CAP and CAP2 prospective registries.
Among 1,739 patients in those studies receiving an implant, 65 (3.74%) had DRT, the investigators found.
Over 1 year of follow-up, 25% of patients with DRT had an ischemic stroke or systemic embolism, versus 6.8% of patients without DRTs (P less than .001), they reported. That worked out to an event rate of 6.28 and 1.65 events per 100 patient years, respectively.
The strongest predictors of DRT in multivariable analysis included vascular disease, history of stroke or transient ischemic attack, permanent atrial fibrillation, and left atrial appendage diameter, according to the report. Conversely, increasing left ventricular ejection fraction was protective against DRT.
Taken together, these data support reevaluating the transesophageal echocardiography strategy, according to Dr. Reddy and his coauthors. Those approaches might include targeting patients with DRT risk factors, routine additional surveillance at 6 months, or delaying the first transesophageal echocardiography to 4 months.
“Importantly, none of these strategies have been rigorously compared, so these suggestions are subject to future studies,” the researchers wrote.
“DRT remains a problem despite increased operator experience with LAA occlusion and improved occluding devices,” commented David B. De Lurgio, MD, a cardiac electrophysiologist at Emory Healthcare in Atlanta. “What is a little alarming is that the risk for DRT extends beyond the period of prescribed anticoagulation. Although the risk from DRT mitigates the benefit of LAA closure compared with warfarin, it does not mitigate the benefit from occlusion compared with no treatment,” said Dr. De Lurgio, designated discussant for the report. “Prevention and management of DRT may require that each patient receive a tailored regimen of anticoagulation and surveillance.”
The Watchman studies were funded by Boston Scientific, the company that markets the device. Dr. Reddy has been a consultant to and has received research funding from Boston Scientific and from Abbott and Biosense-Webster, and he reported having an equity interest in Javelin and Surecor. Coauthors reported disclosures related to Boston Scientific, Johnson & Johnson, Abbott, and other medical device companies. Dr. De Lurgio has been a consultant to Boston Scientific.
Updated, 5/17/18: This article has been updated with reporting from Mitchel L. Zoler at the meeting, and has been revised for clarity and to reflect that the results were presented by Dr. Reddy.
SOURCE: Dukkipati SR et al. Circulation. 2018 May 11. doi: 10.1161/CIRCULATIONAHA.118.035090.
REPORTING FROM HEART RHYTHM 2018
Key clinical point: Device-related thrombus (DRT) following left atrial appendage closure occurs in less than 4% of patients but is associated with a higher rate of stroke and systemic embolism vs. no DRT.
Major finding: The rate of ischemic stroke and systemic embolism was 6.28 and 1.65 per 100 patient years for patients with DRT and with no DRT, respectively (P less than .001).
Study details: Analysis of data from the device arms of four prospective clinical trials.
Disclosures: The Watchman studies were funded by Boston Scientific, the company that markets the device. Dr. Reddy has been a consultant to and has received research funding from Boston Scientific and from Abbott and Biosense-Webster, and he reported having an equity interest in Javelin and Surecor. Coauthors reported disclosures related to Boston Scientific, Johnson & Johnson, Abbott, and other medical device companies. Dr. De Lurgio has been a consultant to Boston Scientific.
Source: Dukkipati SR et al. Circulation. 2018 May 11. doi: 10.1161/CIRCULATIONAHA.118.035090.
Air pollution linked to childhood hypertension
Third-trimester exposure to airborne particulate matter (PM) smaller than 2.5 mcm in size (PM2.5) has been linked to higher levels of systolic blood pressure during childhood.
Mingyu Zhang of Johns Hopkins Bloomberg School of Public Health, Baltimore, and his associates found that the highest tertile exposure was tied to an increased likelihood of childhood elevated BP, defined as systolic blood pressure higher than the 90th percentile, compared with those in the lowest tertile (relative risk, 1.60; 95% confidence interval, 1.12-2.27).
Previous studies have shown a relationship between exposure to PM2.5and elevated BP in children and adults. Mouse models suggest that PM2.5 may interfere with in utero development of the cardiovascular system. One previous study found evidence that third-trimester exposure to PM2.5was linked to heightened BP in newborns, while a retrospective analysis showed no association with BP in adolescents.
They identified PM2.5 exposure by matching the mother’s residential address to the U.S. Environmental Protection Agency’s local air quality monitors, from which they extracted daily PM2.5 values.
For every 5 mcg/m3 increment in PM2.5 exposure, the relative risk for heightened BP in a child at a single visit was 1.46 (95% CI, 1.17-1.83). The relative risk increased after independent additions to the confounder model of birth weight (RR, 3.39; 95% CI, 0.63-6.15), gestational age (RR, 3.08; 95% CI, 0.33-5.82), and child body mass index (BMI) z score (RR, 2.75; 95% CI, 0.01-5.50).
A multivariable-adjusted cubic spline model revealed a significant increase in risk of elevated BP at a cutoff threshold of 13 mcg/m3 (RR, 1.80; 95% CI, 1.33-2.44).
Further analysis suggested that 35% of the association between exposure and elevated BP risk was mediated by birth weight and BMI z score during childhood. When these factors were added to the models, the association between PM exposure and BP risk was no longer significant.
Although the findings are intriguing, they cannot prove causation, according to the researchers.
The study was funded by the National Institutes of Health and the Maternal and Child Health Bureau. Dr. Gold and Dr. Zanobetti have received funding from NIH.
SOURCE: Zhang et al. 2018 Jul. doi: 10.1161/hypertensionaha.117.10944.
A paper recently published by the same group outlines a possible mechanism by which PM2.5 might cause harm – specifically, an increase in placental intrauterine inflammation. The study adds evidence to the case that air pollution is linked to hypertension as it includes more economically disadvantaged subjects, children of color, preterm births, and small-for-gestational-age births. Overall, the study imaginatively made use of pediatric data in a disadvantaged urban setting to better understand how air pollution affects an important physiological outcome.
Diane Gold, MD, and Antonella Zanobetti, PhD, are at the Harvard T.H. Chan School of Public Health, Boston.
A paper recently published by the same group outlines a possible mechanism by which PM2.5 might cause harm – specifically, an increase in placental intrauterine inflammation. The study adds evidence to the case that air pollution is linked to hypertension as it includes more economically disadvantaged subjects, children of color, preterm births, and small-for-gestational-age births. Overall, the study imaginatively made use of pediatric data in a disadvantaged urban setting to better understand how air pollution affects an important physiological outcome.
Diane Gold, MD, and Antonella Zanobetti, PhD, are at the Harvard T.H. Chan School of Public Health, Boston.
A paper recently published by the same group outlines a possible mechanism by which PM2.5 might cause harm – specifically, an increase in placental intrauterine inflammation. The study adds evidence to the case that air pollution is linked to hypertension as it includes more economically disadvantaged subjects, children of color, preterm births, and small-for-gestational-age births. Overall, the study imaginatively made use of pediatric data in a disadvantaged urban setting to better understand how air pollution affects an important physiological outcome.
Diane Gold, MD, and Antonella Zanobetti, PhD, are at the Harvard T.H. Chan School of Public Health, Boston.
Third-trimester exposure to airborne particulate matter (PM) smaller than 2.5 mcm in size (PM2.5) has been linked to higher levels of systolic blood pressure during childhood.
Mingyu Zhang of Johns Hopkins Bloomberg School of Public Health, Baltimore, and his associates found that the highest tertile exposure was tied to an increased likelihood of childhood elevated BP, defined as systolic blood pressure higher than the 90th percentile, compared with those in the lowest tertile (relative risk, 1.60; 95% confidence interval, 1.12-2.27).
Previous studies have shown a relationship between exposure to PM2.5and elevated BP in children and adults. Mouse models suggest that PM2.5 may interfere with in utero development of the cardiovascular system. One previous study found evidence that third-trimester exposure to PM2.5was linked to heightened BP in newborns, while a retrospective analysis showed no association with BP in adolescents.
They identified PM2.5 exposure by matching the mother’s residential address to the U.S. Environmental Protection Agency’s local air quality monitors, from which they extracted daily PM2.5 values.
For every 5 mcg/m3 increment in PM2.5 exposure, the relative risk for heightened BP in a child at a single visit was 1.46 (95% CI, 1.17-1.83). The relative risk increased after independent additions to the confounder model of birth weight (RR, 3.39; 95% CI, 0.63-6.15), gestational age (RR, 3.08; 95% CI, 0.33-5.82), and child body mass index (BMI) z score (RR, 2.75; 95% CI, 0.01-5.50).
A multivariable-adjusted cubic spline model revealed a significant increase in risk of elevated BP at a cutoff threshold of 13 mcg/m3 (RR, 1.80; 95% CI, 1.33-2.44).
Further analysis suggested that 35% of the association between exposure and elevated BP risk was mediated by birth weight and BMI z score during childhood. When these factors were added to the models, the association between PM exposure and BP risk was no longer significant.
Although the findings are intriguing, they cannot prove causation, according to the researchers.
The study was funded by the National Institutes of Health and the Maternal and Child Health Bureau. Dr. Gold and Dr. Zanobetti have received funding from NIH.
SOURCE: Zhang et al. 2018 Jul. doi: 10.1161/hypertensionaha.117.10944.
Third-trimester exposure to airborne particulate matter (PM) smaller than 2.5 mcm in size (PM2.5) has been linked to higher levels of systolic blood pressure during childhood.
Mingyu Zhang of Johns Hopkins Bloomberg School of Public Health, Baltimore, and his associates found that the highest tertile exposure was tied to an increased likelihood of childhood elevated BP, defined as systolic blood pressure higher than the 90th percentile, compared with those in the lowest tertile (relative risk, 1.60; 95% confidence interval, 1.12-2.27).
Previous studies have shown a relationship between exposure to PM2.5and elevated BP in children and adults. Mouse models suggest that PM2.5 may interfere with in utero development of the cardiovascular system. One previous study found evidence that third-trimester exposure to PM2.5was linked to heightened BP in newborns, while a retrospective analysis showed no association with BP in adolescents.
They identified PM2.5 exposure by matching the mother’s residential address to the U.S. Environmental Protection Agency’s local air quality monitors, from which they extracted daily PM2.5 values.
For every 5 mcg/m3 increment in PM2.5 exposure, the relative risk for heightened BP in a child at a single visit was 1.46 (95% CI, 1.17-1.83). The relative risk increased after independent additions to the confounder model of birth weight (RR, 3.39; 95% CI, 0.63-6.15), gestational age (RR, 3.08; 95% CI, 0.33-5.82), and child body mass index (BMI) z score (RR, 2.75; 95% CI, 0.01-5.50).
A multivariable-adjusted cubic spline model revealed a significant increase in risk of elevated BP at a cutoff threshold of 13 mcg/m3 (RR, 1.80; 95% CI, 1.33-2.44).
Further analysis suggested that 35% of the association between exposure and elevated BP risk was mediated by birth weight and BMI z score during childhood. When these factors were added to the models, the association between PM exposure and BP risk was no longer significant.
Although the findings are intriguing, they cannot prove causation, according to the researchers.
The study was funded by the National Institutes of Health and the Maternal and Child Health Bureau. Dr. Gold and Dr. Zanobetti have received funding from NIH.
SOURCE: Zhang et al. 2018 Jul. doi: 10.1161/hypertensionaha.117.10944.
FROM HYPERTENSION
Key clinical point: Maternal air pollution exposure could be useful in screening and prevention of childhood hypertension.
Major finding: Each 5 µg/m3 exposure increment was associated with 46% increased odds of elevated BP.
Study details: Prospective study of 1,293 mothers.
Disclosures: The study was funded by the NIH and the Maternal and Child Health Bureau. Dr. Gold and Dr. Zanobetti have received funding from NIH.
Source: Zhang et al. 2018 Jul. doi: 10.1161/hypertensionaha.117.10944.
Rapid Deterioration and Death Caused by Bilateral Phlegmasia Cerulea Dolens
Phlegmasia cerulea dolens (PCD), a life-threatening complication of deep venous thrombosis (DVT), is characterized by massive iliofemoral thrombus that extends to the collateral veins, leading to fluid sequestration and elevated compartment pressures that ultimately compromise arterial flow. Phlegmasia cerulea dolens can rapidly progress to compartment syndrome and gangrene.1,2 The affected limbs of patients with PCD can be hypoxic and appear purple in color due to substantial lack of blood flow, with diminished or absent pulses. Risk factors for PCD include malignancy, hypercoagulable states, venous stasis, contraceptive agents, inferior vena cava (IVC) filter, aneurysm, history of DVT, trauma, heparin-induced thrombocytopenia, femoral vein catheterization, antiphospholipid syndrome, or pregnancy.3-6 Failure to treat PCD early and aggressively carries an amputation rate of up to 50% and a mortality rate of up to 40%.4
We present the case of a patient with PCD, whose condition rapidly deteriorated despite prompt diagnosis and treatment.
Case
A 58-year-old woman presented to the ED with a 1-day history of back and leg pain and difficulty walking. When asked about the severity of her pain, she rated her leg pain at 10 on a scale of 0 to 10. The patient’s history was significant for DVT and pulmonary embolism (PE), for which a Greenfield IVC had been placed and for which she was on prophylactic warfarin therapy. The patient stated that she had been taken off warfarin several weeks prior to presentation in preparation for an elective colonoscopy and dental procedure, but had restarted the warfarin therapy 2 days prior to presentation. She had no history of diabetes mellitus or renal disease.
Initial vital signs at presentation were: blood pressure, 120/91 mm Hg; heart rate, 110 beats/min; respiratory rate, 24 breaths/min; and temperature, 96.6°F. Oxygen saturation was 100% on a nonrebreather mask.
On examination, the patient was alert and oriented to person, time, and place, but appeared dyspneic. An electrocardiogram revealed sinus tachycardia. On physical examination, lung sounds were clear to auscultation bilaterally with good air movement, and the abdomen was soft and nontender with normal bowel sounds. The dorsalis pedis and posterior tibial pulses were absent bilaterally, lower extremity capillary refill was 3 seconds, and the legs appeared mildly erythematous and cool to touch. No speech or neurological deficits were present.
Laboratory evaluation was remarkable for metabolic acidosis, venous pH, 7.11; bicarbonate, 11.7; partial pressure of carbon dioxide, 37.6; lactic acid, 8.8 mEq/L leukocytosis, 24,900 u/L; glucose, 296 mg/dL; creatinine, 2.41 mg/dL; and international normalized ratio, 1.36.
Before additional laboratory studies and imaging could be obtained, the patient developed altered mental status, hypotension, and paralysis of the lower extremities. She was orally intubated for airway protection and was given a total of 4 L of normal saline intravenously (IV) for hypotension and acidosis; sodium bicarbonate for metabolic acidosis; norepinephrine for hypotension; fentanyl for pain; and ondansetron for nausea. A central line and arterial line were placed for administering medication and hemodynamic monitoring.
Computed tomography (CT) angiography of the chest, abdomen, and pelvis demonstrated multiple subsegmental bilateral PE with no arterial pathology (Figure 1). Beside ultrasound revealed extensive bilateral DVTs involving the superficial and common femoral veins (Figure 2). The patient’s bilateral DVTs, arterial compromise, and leg cyanosis led to the diagnosis of PCD.
Critical care and vascular surgery services were consulted, and the patient was admitted to the intensive care unit. Since the patient was too unstable to undergo thrombectomy, she was given IV tissue plasminogen activator. Despite aggressive pharmacological treatment, the patient’s condition continued to deteriorate. On hospital day 2, the patient’s family changed the patient’s code status to do-not-resuscitate/comfort-care only; she died shortly thereafter.
Discussion
This case illustrates the severity and complications of PCD and the rapidity with which this condition can deteriorate. At the time of ED presentation, the patient had already developed bilateral PCD, metabolic acidosis, and bilateral PE. Unfortunately, due to decreased venous return, decreased cardiac output, and severe shock, she quickly became unstable and progressed rapidly to multisystem organ failure leading to death.
Risk Factors
A prior patient history DVT and an IVC filter are both significant risk factors for the progression of DVT to PCD;3,6 however, in this case, IVC filter failed to prevent emboli from reaching the lungs. Extensive thrombi led to severely decreased venous return and cardiac output, causing life-threatening shock, ischemia, and metabolic acidosis. A lactic acid level taken on hospital day 2 was elevated at 19 mEq/L, demonstrating the severity, morbidity, and progression of PCD.
Signs and Symptoms
The three cardinal signs that lead to a clinical diagnosis of PCD are edema, pain, and violaceous discoloration or skin mottling.3 Although most commonly found in the lower extremity, PCD can occur in any limb due to occlusion of venous outflow.7 Unfortunately, a clinical diagnosis of PCD is not often made until the venous occlusion becomes severe enough to impair arterial flow and cause venous gangrene, tissue ischemia, shock, and death.8
Although IVC filters are designed to prevent life-threatening PE, there are risk factors associated with their use. Whether placed recently or decades prior, urgent investigation, such as immediate CT scan, should be undertaken in patients presenting with DVT-like symptoms who have a history of an IVC filter, to ensure the filter has not shifted from its original placement and is not occluding the IVC.
Conclusion
Phlegmasia cerulea dolens is an uncommon vascular emergency, but one that has a high-morbidity and high-mortality rate. This case demonstrates the importance of early diagnosis, aggressive treatment, and the severe complications that can develop in PCD.
There are cases in the literature where patients diagnosed with PCD had a successful outcome with pharmacological or surgical intervention such as thrombectomy. Treatment for PCD is most effective when instituted early in onset. As seen in our patient, the tendency for rapid deterioration in PCD can limit potentially lifesaving therapeutic options, decreasing the chances of a successful outcome. Emergency physicians, therefore, must be aware of the high-mortality rate associated with this disorder and the possibility of rapid progression from stable to critical condition.
1. Kesieme E, Kesieme C, Jebbin N, Irekpita E, Dongo A. Deep vein thrombosis: a clinical review. J Blood Med. 2011;2:59-69. doi:10.2147/JBM.S19009.
2. Bhatt S, Wehbe C, Dogra VS. Phlegmasia cerulea dolens. J Clin Ultrasound. 2007;35(7):401-404. doi:10.1002/jcu.20317.
3. Maiti A, Das A, Smith DT. Phlegmasia cerulean dolens. Postgrad Med J. 2016;pii: postgradmedj-2016-134185. doi:10.1136/postgradmedj-2016-134185.
4. Abdul W, Hickey B, Wilson C. Lower extremity compartment syndrome in the setting of iliofemoral deep vein thrombosis, phlegmasia cerulea dolens and factor VII deficiency. BMJ Case Rep. 2016;2016:pii:bcr2016215078. doi:10.1136/bcr-2016-215078.
5. Onuoha CU. Phlegmasia cerulea dolens: A rare clinical presentation. Am J Med. 2015;128(9):e27-e28. doi:10.1016/j.amjmed.2015.04.009.
6. Chinsakchai K, Ten Duis K, Moll FL, de Borst GJ. Trends in management of phlegmasia cerulea dolens. Vasc Endovascular Surg. 2011;45(1):5-14. doi:10.1177/1538574410388309.
7. Bagenal JD, Nasralla D. Bilateral phlegmasia cerulea dolens in an occluded inferior vena cava filter. BMJ Case Rep. 2013;pii: bcr2013009302. doi:10.1136/bcr-2013-009302.
8. Kiefer CS, Colletti JE. Phlegmasia cerulea dolens in a patient with an inferior vena cava filter. J Emerg Med. 2013;44(1):e95-e97. doi:10.1016/j.jemermed.2012.01.018.
Phlegmasia cerulea dolens (PCD), a life-threatening complication of deep venous thrombosis (DVT), is characterized by massive iliofemoral thrombus that extends to the collateral veins, leading to fluid sequestration and elevated compartment pressures that ultimately compromise arterial flow. Phlegmasia cerulea dolens can rapidly progress to compartment syndrome and gangrene.1,2 The affected limbs of patients with PCD can be hypoxic and appear purple in color due to substantial lack of blood flow, with diminished or absent pulses. Risk factors for PCD include malignancy, hypercoagulable states, venous stasis, contraceptive agents, inferior vena cava (IVC) filter, aneurysm, history of DVT, trauma, heparin-induced thrombocytopenia, femoral vein catheterization, antiphospholipid syndrome, or pregnancy.3-6 Failure to treat PCD early and aggressively carries an amputation rate of up to 50% and a mortality rate of up to 40%.4
We present the case of a patient with PCD, whose condition rapidly deteriorated despite prompt diagnosis and treatment.
Case
A 58-year-old woman presented to the ED with a 1-day history of back and leg pain and difficulty walking. When asked about the severity of her pain, she rated her leg pain at 10 on a scale of 0 to 10. The patient’s history was significant for DVT and pulmonary embolism (PE), for which a Greenfield IVC had been placed and for which she was on prophylactic warfarin therapy. The patient stated that she had been taken off warfarin several weeks prior to presentation in preparation for an elective colonoscopy and dental procedure, but had restarted the warfarin therapy 2 days prior to presentation. She had no history of diabetes mellitus or renal disease.
Initial vital signs at presentation were: blood pressure, 120/91 mm Hg; heart rate, 110 beats/min; respiratory rate, 24 breaths/min; and temperature, 96.6°F. Oxygen saturation was 100% on a nonrebreather mask.
On examination, the patient was alert and oriented to person, time, and place, but appeared dyspneic. An electrocardiogram revealed sinus tachycardia. On physical examination, lung sounds were clear to auscultation bilaterally with good air movement, and the abdomen was soft and nontender with normal bowel sounds. The dorsalis pedis and posterior tibial pulses were absent bilaterally, lower extremity capillary refill was 3 seconds, and the legs appeared mildly erythematous and cool to touch. No speech or neurological deficits were present.
Laboratory evaluation was remarkable for metabolic acidosis, venous pH, 7.11; bicarbonate, 11.7; partial pressure of carbon dioxide, 37.6; lactic acid, 8.8 mEq/L leukocytosis, 24,900 u/L; glucose, 296 mg/dL; creatinine, 2.41 mg/dL; and international normalized ratio, 1.36.
Before additional laboratory studies and imaging could be obtained, the patient developed altered mental status, hypotension, and paralysis of the lower extremities. She was orally intubated for airway protection and was given a total of 4 L of normal saline intravenously (IV) for hypotension and acidosis; sodium bicarbonate for metabolic acidosis; norepinephrine for hypotension; fentanyl for pain; and ondansetron for nausea. A central line and arterial line were placed for administering medication and hemodynamic monitoring.
Computed tomography (CT) angiography of the chest, abdomen, and pelvis demonstrated multiple subsegmental bilateral PE with no arterial pathology (Figure 1). Beside ultrasound revealed extensive bilateral DVTs involving the superficial and common femoral veins (Figure 2). The patient’s bilateral DVTs, arterial compromise, and leg cyanosis led to the diagnosis of PCD.
Critical care and vascular surgery services were consulted, and the patient was admitted to the intensive care unit. Since the patient was too unstable to undergo thrombectomy, she was given IV tissue plasminogen activator. Despite aggressive pharmacological treatment, the patient’s condition continued to deteriorate. On hospital day 2, the patient’s family changed the patient’s code status to do-not-resuscitate/comfort-care only; she died shortly thereafter.
Discussion
This case illustrates the severity and complications of PCD and the rapidity with which this condition can deteriorate. At the time of ED presentation, the patient had already developed bilateral PCD, metabolic acidosis, and bilateral PE. Unfortunately, due to decreased venous return, decreased cardiac output, and severe shock, she quickly became unstable and progressed rapidly to multisystem organ failure leading to death.
Risk Factors
A prior patient history DVT and an IVC filter are both significant risk factors for the progression of DVT to PCD;3,6 however, in this case, IVC filter failed to prevent emboli from reaching the lungs. Extensive thrombi led to severely decreased venous return and cardiac output, causing life-threatening shock, ischemia, and metabolic acidosis. A lactic acid level taken on hospital day 2 was elevated at 19 mEq/L, demonstrating the severity, morbidity, and progression of PCD.
Signs and Symptoms
The three cardinal signs that lead to a clinical diagnosis of PCD are edema, pain, and violaceous discoloration or skin mottling.3 Although most commonly found in the lower extremity, PCD can occur in any limb due to occlusion of venous outflow.7 Unfortunately, a clinical diagnosis of PCD is not often made until the venous occlusion becomes severe enough to impair arterial flow and cause venous gangrene, tissue ischemia, shock, and death.8
Although IVC filters are designed to prevent life-threatening PE, there are risk factors associated with their use. Whether placed recently or decades prior, urgent investigation, such as immediate CT scan, should be undertaken in patients presenting with DVT-like symptoms who have a history of an IVC filter, to ensure the filter has not shifted from its original placement and is not occluding the IVC.
Conclusion
Phlegmasia cerulea dolens is an uncommon vascular emergency, but one that has a high-morbidity and high-mortality rate. This case demonstrates the importance of early diagnosis, aggressive treatment, and the severe complications that can develop in PCD.
There are cases in the literature where patients diagnosed with PCD had a successful outcome with pharmacological or surgical intervention such as thrombectomy. Treatment for PCD is most effective when instituted early in onset. As seen in our patient, the tendency for rapid deterioration in PCD can limit potentially lifesaving therapeutic options, decreasing the chances of a successful outcome. Emergency physicians, therefore, must be aware of the high-mortality rate associated with this disorder and the possibility of rapid progression from stable to critical condition.
Phlegmasia cerulea dolens (PCD), a life-threatening complication of deep venous thrombosis (DVT), is characterized by massive iliofemoral thrombus that extends to the collateral veins, leading to fluid sequestration and elevated compartment pressures that ultimately compromise arterial flow. Phlegmasia cerulea dolens can rapidly progress to compartment syndrome and gangrene.1,2 The affected limbs of patients with PCD can be hypoxic and appear purple in color due to substantial lack of blood flow, with diminished or absent pulses. Risk factors for PCD include malignancy, hypercoagulable states, venous stasis, contraceptive agents, inferior vena cava (IVC) filter, aneurysm, history of DVT, trauma, heparin-induced thrombocytopenia, femoral vein catheterization, antiphospholipid syndrome, or pregnancy.3-6 Failure to treat PCD early and aggressively carries an amputation rate of up to 50% and a mortality rate of up to 40%.4
We present the case of a patient with PCD, whose condition rapidly deteriorated despite prompt diagnosis and treatment.
Case
A 58-year-old woman presented to the ED with a 1-day history of back and leg pain and difficulty walking. When asked about the severity of her pain, she rated her leg pain at 10 on a scale of 0 to 10. The patient’s history was significant for DVT and pulmonary embolism (PE), for which a Greenfield IVC had been placed and for which she was on prophylactic warfarin therapy. The patient stated that she had been taken off warfarin several weeks prior to presentation in preparation for an elective colonoscopy and dental procedure, but had restarted the warfarin therapy 2 days prior to presentation. She had no history of diabetes mellitus or renal disease.
Initial vital signs at presentation were: blood pressure, 120/91 mm Hg; heart rate, 110 beats/min; respiratory rate, 24 breaths/min; and temperature, 96.6°F. Oxygen saturation was 100% on a nonrebreather mask.
On examination, the patient was alert and oriented to person, time, and place, but appeared dyspneic. An electrocardiogram revealed sinus tachycardia. On physical examination, lung sounds were clear to auscultation bilaterally with good air movement, and the abdomen was soft and nontender with normal bowel sounds. The dorsalis pedis and posterior tibial pulses were absent bilaterally, lower extremity capillary refill was 3 seconds, and the legs appeared mildly erythematous and cool to touch. No speech or neurological deficits were present.
Laboratory evaluation was remarkable for metabolic acidosis, venous pH, 7.11; bicarbonate, 11.7; partial pressure of carbon dioxide, 37.6; lactic acid, 8.8 mEq/L leukocytosis, 24,900 u/L; glucose, 296 mg/dL; creatinine, 2.41 mg/dL; and international normalized ratio, 1.36.
Before additional laboratory studies and imaging could be obtained, the patient developed altered mental status, hypotension, and paralysis of the lower extremities. She was orally intubated for airway protection and was given a total of 4 L of normal saline intravenously (IV) for hypotension and acidosis; sodium bicarbonate for metabolic acidosis; norepinephrine for hypotension; fentanyl for pain; and ondansetron for nausea. A central line and arterial line were placed for administering medication and hemodynamic monitoring.
Computed tomography (CT) angiography of the chest, abdomen, and pelvis demonstrated multiple subsegmental bilateral PE with no arterial pathology (Figure 1). Beside ultrasound revealed extensive bilateral DVTs involving the superficial and common femoral veins (Figure 2). The patient’s bilateral DVTs, arterial compromise, and leg cyanosis led to the diagnosis of PCD.
Critical care and vascular surgery services were consulted, and the patient was admitted to the intensive care unit. Since the patient was too unstable to undergo thrombectomy, she was given IV tissue plasminogen activator. Despite aggressive pharmacological treatment, the patient’s condition continued to deteriorate. On hospital day 2, the patient’s family changed the patient’s code status to do-not-resuscitate/comfort-care only; she died shortly thereafter.
Discussion
This case illustrates the severity and complications of PCD and the rapidity with which this condition can deteriorate. At the time of ED presentation, the patient had already developed bilateral PCD, metabolic acidosis, and bilateral PE. Unfortunately, due to decreased venous return, decreased cardiac output, and severe shock, she quickly became unstable and progressed rapidly to multisystem organ failure leading to death.
Risk Factors
A prior patient history DVT and an IVC filter are both significant risk factors for the progression of DVT to PCD;3,6 however, in this case, IVC filter failed to prevent emboli from reaching the lungs. Extensive thrombi led to severely decreased venous return and cardiac output, causing life-threatening shock, ischemia, and metabolic acidosis. A lactic acid level taken on hospital day 2 was elevated at 19 mEq/L, demonstrating the severity, morbidity, and progression of PCD.
Signs and Symptoms
The three cardinal signs that lead to a clinical diagnosis of PCD are edema, pain, and violaceous discoloration or skin mottling.3 Although most commonly found in the lower extremity, PCD can occur in any limb due to occlusion of venous outflow.7 Unfortunately, a clinical diagnosis of PCD is not often made until the venous occlusion becomes severe enough to impair arterial flow and cause venous gangrene, tissue ischemia, shock, and death.8
Although IVC filters are designed to prevent life-threatening PE, there are risk factors associated with their use. Whether placed recently or decades prior, urgent investigation, such as immediate CT scan, should be undertaken in patients presenting with DVT-like symptoms who have a history of an IVC filter, to ensure the filter has not shifted from its original placement and is not occluding the IVC.
Conclusion
Phlegmasia cerulea dolens is an uncommon vascular emergency, but one that has a high-morbidity and high-mortality rate. This case demonstrates the importance of early diagnosis, aggressive treatment, and the severe complications that can develop in PCD.
There are cases in the literature where patients diagnosed with PCD had a successful outcome with pharmacological or surgical intervention such as thrombectomy. Treatment for PCD is most effective when instituted early in onset. As seen in our patient, the tendency for rapid deterioration in PCD can limit potentially lifesaving therapeutic options, decreasing the chances of a successful outcome. Emergency physicians, therefore, must be aware of the high-mortality rate associated with this disorder and the possibility of rapid progression from stable to critical condition.
1. Kesieme E, Kesieme C, Jebbin N, Irekpita E, Dongo A. Deep vein thrombosis: a clinical review. J Blood Med. 2011;2:59-69. doi:10.2147/JBM.S19009.
2. Bhatt S, Wehbe C, Dogra VS. Phlegmasia cerulea dolens. J Clin Ultrasound. 2007;35(7):401-404. doi:10.1002/jcu.20317.
3. Maiti A, Das A, Smith DT. Phlegmasia cerulean dolens. Postgrad Med J. 2016;pii: postgradmedj-2016-134185. doi:10.1136/postgradmedj-2016-134185.
4. Abdul W, Hickey B, Wilson C. Lower extremity compartment syndrome in the setting of iliofemoral deep vein thrombosis, phlegmasia cerulea dolens and factor VII deficiency. BMJ Case Rep. 2016;2016:pii:bcr2016215078. doi:10.1136/bcr-2016-215078.
5. Onuoha CU. Phlegmasia cerulea dolens: A rare clinical presentation. Am J Med. 2015;128(9):e27-e28. doi:10.1016/j.amjmed.2015.04.009.
6. Chinsakchai K, Ten Duis K, Moll FL, de Borst GJ. Trends in management of phlegmasia cerulea dolens. Vasc Endovascular Surg. 2011;45(1):5-14. doi:10.1177/1538574410388309.
7. Bagenal JD, Nasralla D. Bilateral phlegmasia cerulea dolens in an occluded inferior vena cava filter. BMJ Case Rep. 2013;pii: bcr2013009302. doi:10.1136/bcr-2013-009302.
8. Kiefer CS, Colletti JE. Phlegmasia cerulea dolens in a patient with an inferior vena cava filter. J Emerg Med. 2013;44(1):e95-e97. doi:10.1016/j.jemermed.2012.01.018.
1. Kesieme E, Kesieme C, Jebbin N, Irekpita E, Dongo A. Deep vein thrombosis: a clinical review. J Blood Med. 2011;2:59-69. doi:10.2147/JBM.S19009.
2. Bhatt S, Wehbe C, Dogra VS. Phlegmasia cerulea dolens. J Clin Ultrasound. 2007;35(7):401-404. doi:10.1002/jcu.20317.
3. Maiti A, Das A, Smith DT. Phlegmasia cerulean dolens. Postgrad Med J. 2016;pii: postgradmedj-2016-134185. doi:10.1136/postgradmedj-2016-134185.
4. Abdul W, Hickey B, Wilson C. Lower extremity compartment syndrome in the setting of iliofemoral deep vein thrombosis, phlegmasia cerulea dolens and factor VII deficiency. BMJ Case Rep. 2016;2016:pii:bcr2016215078. doi:10.1136/bcr-2016-215078.
5. Onuoha CU. Phlegmasia cerulea dolens: A rare clinical presentation. Am J Med. 2015;128(9):e27-e28. doi:10.1016/j.amjmed.2015.04.009.
6. Chinsakchai K, Ten Duis K, Moll FL, de Borst GJ. Trends in management of phlegmasia cerulea dolens. Vasc Endovascular Surg. 2011;45(1):5-14. doi:10.1177/1538574410388309.
7. Bagenal JD, Nasralla D. Bilateral phlegmasia cerulea dolens in an occluded inferior vena cava filter. BMJ Case Rep. 2013;pii: bcr2013009302. doi:10.1136/bcr-2013-009302.
8. Kiefer CS, Colletti JE. Phlegmasia cerulea dolens in a patient with an inferior vena cava filter. J Emerg Med. 2013;44(1):e95-e97. doi:10.1016/j.jemermed.2012.01.018.
Bell Palsy Mimics
Facial paralysis is a common medical complaint—one that has fascinated ancient and contemporary physicians alike.1 An idiopathic facial nerve paresis involving the lower motor neuron was described in 1821 by Sir Charles Bell. This entity became known as a Bell’s palsy, the hallmark of which was weakness or complete paralysis of the muscles of one side of the face, with no sparing of the muscles of the forehead. However, not all facial paralysis is due to Bell’s palsy.
We present a case of a patient with a Bell’s palsy mimic to facilitate and guide the differential diagnosis and distinguish conditions from the classical presentation that Bell first described to the more concerning symptoms that may not be immediately obvious. Our case further underscores the importance of performing a thorough assessment to determine the presence of other neurological findings.
Case
A 61-year-old woman presented to the ED for evaluation of right facial droop and sensation of “room spinning.” The patient stated both symptoms began approximately 36 hours prior to presentation, upon awakening.
The patient denied any headache, neck or chest pain, extremity numbness, or weakness, but stated that she felt like she was going to fall toward her right side whenever she attempted to walk. The patient’s medical history was significant for hypertension, for which she was taking losartan. Her surgical history was notable for a left oophorectomy secondary to an ovarian cyst. Regarding the social history, the patient admitted to smoking 90 packs of cigarettes per year, but denied alcohol or illicit drug use.
Upon arrival at the ED, the patient’s vital signs were: blood pressure, 164/86 mm Hg: pulse, 89 beats/min; respiratory rate, 18 breaths/min; and temperature, 98.6°F. Oxygen saturation was 98% on room air.
Physical examination revealed the patient had a right facial droop consistent with right facial palsy. She was unable to wrinkle her right forehead or fully close her right eye. There were no field cuts on confrontation. The patient’s speech was noticeable for a mild dysarthria. The motor examination revealed mild weakness of the left upper extremity and impaired right facial sensation. There were no rashes noted on the face, head, or ears. The patient had slightly impaired hearing in the right ear, which was new in onset. The remainder of the physical examination was unremarkable.
Although the patient exhibited the classic signs of Bell’s palsy, including complete paralysis of the muscles of one side of the face, inability to wrinkle the muscle of the right forehead, and inability to fully close the right eye, she also had concerning symptoms of vertigo, dysarthria, and contralateral upper extremity weakness.
A computed tomography (CT) scan of the head was ordered, which revealed a large mass lesion centered in the right petrous apex, with an associated large component extending medially into the right cerebellopontine angle (CPA) that caused a mass effect on the adjacent brainstem (Figures 1a and 1b).
Upon these findings, the patient was transferred to another facility for neurosurgical evaluation. Magnetic resonance imaging (MRI) studies performed at the receiving hospital demonstrated a large expansile heterogeneous mass lesion centered in the right petrous apex with an associated large, probable hemorrhagic soft-tissue component extending medially into the right CPA, causing a mass effect on the adjacent brainstem and mild obstructive hydrocephalus (Figures 2a and 2b).
The patient was given dexamethasone 10 mg intravenously and taken to the operating room for a right suboccipital craniotomy with subtotal tumor removal. Intraoperative high-voltage stimulation of the fifth to eighth cranial nerves showed no response, indicating significant impairment.
While there were no intraoperative complications, the patient had significant postoperative dysphagia and resultant aspiration. A tracheostomy and percutaneous endoscopic gastrostomy tube were subsequently placed. Results of a biopsy taken during surgery identified an atypical meningioma. The patient remained in the hospital for 4 weeks, after which she was discharged to a long-term care (LTC) and rehabilitation facility.
A repeat CT scan taken 2 months after surgery demonstrated absence of the previously identified large mass (Figure 1b). Three months after discharge from the LTC-rehabilitation facility, MRI of the brain showed continued interval improvement of the previously noted mass centered in the right petrous apex (Figures 3a and 3b).
Discussion
Accounts of facial paralysis and facial nerve disorders have been noted throughout history and include accounts of the condition by Hippocrates.1 Bell’s palsy was named after surgeon Sir Charles Bell, who described a peripheral-nerve paralysis of the facial nerve in 1821. Bell’s work helped to elucidate the anatomy and functional role of the facial nerve.1,2
Signs and Symptoms
The classic presentation of Bell’s palsy is weakness or complete paralysis of the muscles of one side of the face, with no sparing of the muscles of the forehead. The eyelid on the affected side generally does not close, which can result in ocular irritation due to ineffective lubrication.
A scoring system has been developed by House and Brackmann which grades the degree impairment based on such characteristics as facial muscle function and eye closure.3,4 Approximately 96% of patients with a Bell’s palsy will improve to a House-Brackmann score of 2 or better within 1 year from diagnosis,5 and 85% of patients with Bell’s palsy will show at least some improvement within 3 weeks of onset (Table).2 Although the classic description of Bell’s palsy notes the condition as idiopathic, there is an increasing body of evidence in the literature showing a link to herpes simplex virus 1.5-7
Ramsey-Hunt Syndrome
The relationship between Bell’s palsy and Ramsey-Hunt syndrome is complex and controversial. Ramsey-Hunt syndrome is a constellation of possible complications from varicella-virus infection. Symptoms of Ramsey-Hunt syndrome include facial paralysis, tinnitus, hearing loss, vertigo, hyperacusis (increased sensitivity to certain frequencies and volume ranges of sound), and decreased ocular tearing.8 Due to the nature of symptoms associated with Ramsey-Hunt syndrome, it is apparent that the condition involves more than the seventh cranial nerve. In fact, studies have shown that Ramsey-Hunt syndrome can affect the fifth, sixth, eighth, and ninth cranial nerves.8
Ramsey-Hunt syndrome, which can present in the absence of cutaneous rash (referred to as zoster sine herpete), is estimated to occur in 8% to 20% of unilateral facial nerve palsies in adult patients.8,9 Regardless of the etiology of Bell’s palsy, a review of the literature makes it clear that facial nerve paralysis is not synonymous with Bell’s palsy.10 In one example, Yetter et al10 describe the case of a patient who, though initially diagnosed with Bell’s palsy, ultimately was found to have a facial palsy due to a parotid gland malignancy.
Likewise, Stomeo11 describes a case of a patient with facial paralysis and profound ipsilateral hearing loss who ultimately was found to have a mucoepithelial carcinoma of the parotid gland. In their report, the authors note that approximately 80% of facial nerve paralysis is due to Bell’s palsy, while 5% is due to malignancy.
In another report, Clemis12 describes a case in which a patient who initially was diagnosed with Bell’s palsy eventually was found to have an adenoid cystic carcinoma of the parotid. Thus, the authors appropriately emphasize in their report that “all that palsies is not Bell’s.”
Differential Diagnosis
Historical factors, including timing and duration of symptom onset, help to distinguish a Bell’s palsy from other disorders that can mimic this condition. In their study, Brach VanSwewaringen13 highlight the fact that “not all facial paralysis is Bell’s palsy.” In their review, the authors describe clues to help distinguish conditions that mimic Bell’s palsy. For example, maximal weakness from Bell’s Palsy typically occurs within 3 to 7 days from symptom onset, and that a more gradual onset of symptoms, with slow or negligible improvement over 6 to 12 months, is more indicative of a space-occupying lesion than Bell’s palsy.13It is, however, important to note that although the patient in our case had a central lesion, she experienced an acute onset of symptoms.
The presence of additional symptoms may also suggest an alternative diagnosis. Brach and VanSwearingen13 further noted that symptoms associated with the eighth nerve, such as vertigo, tinnitus, and hearing loss may be found in patients with a CPA tumor. In patients with larger tumors, ninth and 10th nerve symptoms, including the impaired hearing noted in our patient, may be present. Some patients with ninth and 10th nerve symptoms may perceive a sense of facial numbness, but actual sensory changes in the facial nerve distribution are unlikely in Bell’s palsy. Gustatory changes, however, are consistent with Bell’s palsy.
Ear pain is consistent with Bell’s palsy and is a signal to be vigilant for the possible emergence of an ear rash, which would suggest the diagnosis of herpes zoster oticus along the trajectory of Ramsey-Hunt syndrome. Facial pain in the area of the facial nerve is inconsistent with Bell’s palsy, while hyperacusis is consistent with Bell’s palsy. Hearing loss is an eighth nerve symptom that is inconsistent with Bell’s palsy.
Similarly, there are physical examination findings that can help distinguish a true Bell’s palsy from a mimic. Changes in tear production are consistent with Bell’s palsy, but imbalance and disequilibrium are not.14
As previously noted, the patient in this case had difficulty walking and felt as if she was falling toward her right side.
One way to organize the causes of facial paralysis has been proposed by Adour et al.15 In this system, etiologies are listed as either acute paralysis or chronic, progressive paralysis. Acute paralysis (ie, the sudden onset of symptoms with maximal severity within 2 weeks), of which Bell’s palsy is the most common, can be seen in cases of polyneuritis.
A new case of Bell’s palsy has been estimated to occur in the United States every 10 minutes.8 Guillain-Barré syndrome and Lyme disease are also in this category, as is Ramsey-Hunt syndrome. Patients with Lyme disease may have a history of a tick bite or rash.14
Trauma can also cause acute facial nerve paralysis (eg, blunt trauma-associated facial fracture, penetrating trauma, birth trauma). Unilateral central facial weakness can have a neurological cause, such as a lesion to the contralateral cortex, subcortical white matter, or internal capsule.2,15 Otitis media can sometimes cause facial paralysis.16 A cholesteatoma can cause acute facial paralysis.2 Malignancies cause 5% of all cases of facial paralysis. Primary parotid tumors of various types are in this category. Metastatic disease from breast, lung, skin, colon, and kidney may cause facial paralysis. As our case illustrates, CPA tumors can cause facial paralysis.15 It is important to also note that a patient can have both a Bell’s palsy and a concurrent disease. There are a number of case reports in the literature that describe acute onset of facial paralysis as a presenting symptom of malignancy.17 In addition, there are cases wherein a neurological finding on imaging, such as an acoustic neuroma, was presumed to be the cause of facial paralysis, yet the patient’s symptoms resolved in a manner consistent with Bell’s palsy.18
For example, Lagman et al19 described a patient in which a CPA lipoma was presumed to be the cause of the facial paralysis, but the eventual outcome showed the lipoma to have been an incidentaloma.
Conclusion
This case demonstrates a presenting symptom of facial palsy and the presence of a CPA tumor. The presence of vertigo along with other historical and physical examination findings inconsistent with Bell’s palsy prompted the CT scan of the head. A review of the literature suggests a number of important findings in patients with facial palsy to assist the clinician in distinguishing true Bell’s palsy from other diseases that can mimic this condition. This case serves as a reminder of the need to perform a thorough and diligent workup to determine the presence or absence of other neurologic findings prior to closing on the diagnosis of Bell’s palsy.
1. Glicenstein J. Ann Chir Plast Esthet. 2015;60(5):347-362. doi:10.1016/j.anplas.2015.05.007.
2. Tiemstra JD, Khatkhate N. Bell’s palsy: diagnosis and management. Am Fam Physician. 2007;76(7):997-1002.
3. House JW, Brackmann DE. Facial nerve grading system. Otolaryngol Head Neck Surg. 1985;93(2):146-147. doi:10.1177/019459988509300202.
4. Reitzen SD, Babb JS, Lalwani AK. Significance and reliability of the House-Brackmann grading system for regional facial nerve function. Otolaryngol Head Neck Surg. 2009;140(2):154-158. doi:10.1016/j.otohns.2008.11.021.
5. Yeo SW, Lee DH, Jun BC, Chang KH, Park YS. Analysis of prognostic factors in Bell’s palsy and Ramsay Hunt syndrome. Auris Nasus Larynx. 2007;34(2):159-164. doi:10.1016/j.anl.2006.09.005.
6. Ahmed A. When is facial paralysis Bell palsy? Current diagnosis and treatment. Cleve Clin J Med. 2005;72(5):398-401, 405.
7. Gilden DH. Clinical practice. Bell’s palsy. N Engl J Med. 2004;351(13):1323-1331. doi:10.1056/NEJMcp041120.
8. Adour KK. Otological complications of herpes zoster. Ann Neurol. 1994;35:Suppl:S62-S64.
9. Furuta Y, Ohtani F, Mesuda Y, Fukuda S, Inuyama Y. Early diagnosis of zoster sine herpete and antiviral therapy for the treatment of facial palsy. Neurology. 2000;55(5):708-710.
10. Yetter MF, Ogren FP, Moore GF, Yonkers AJ. Bell’s palsy: a facial nerve paralysis diagnosis of exclusion. Nebr Med J. 1990;75(5):109-116.
11. Stomeo F. Possibilities of diagnostic errors in paralysis of the 7th cranial nerve. Acta Otorhinolaryngol Ital. 1989;9(6):629-633.
12. Clemis JD. All that palsies is not Bell’s: Bell’s palsy due to adenoid cystic carcinoma of the parotid. Am J Otol. 1991;12(5):397.
13. Brach JS, VanSwearingen JM. Not all facial paralysis is Bell’s palsy: a case report. Arch Phys Med Rehabil. 1999;80(7):857-859.
14. Albers JR, Tamang S. Common questions about Bell palsy. Am Fam Physician. 2014;89(3):209-212.
15. Adour KK, Hilsinger RL Jr, Callan EJ. Facial paralysis and Bell’s palsy: a protocol for differential diagnosis. Am J Otol. 1985;Suppl:68-73.
16. Morrow MJ. Bell’s palsy and herpes zoster. Curr Treat Options Neurol. 2000;2(5):407-416.
17. Quesnel AM, Lindsay RW, Hadlock TA. When the bell tolls on Bell’s palsy: finding occult malignancy in acute-onset facial paralysis. Am J Otolaryngol. 2010;31(5):339-342. doi:10.1016/j.amjoto.2009.04.003.
18. Kaushal A, Curran WJ Jr. For whom the Bell’s palsy tolls? Am J Clin Oncol. 2009;32(4):450-451. doi:10.1097/01.coc.0000239141.22916.22.
19. Lagman C, Choy W, Lee SJ, et al. A Case of Bell’s palsy with an incidental finding of a cerebellopontine angle lipoma. Cureus. 2016;8(8):e747. doi:10.7759/cureus.747.
Facial paralysis is a common medical complaint—one that has fascinated ancient and contemporary physicians alike.1 An idiopathic facial nerve paresis involving the lower motor neuron was described in 1821 by Sir Charles Bell. This entity became known as a Bell’s palsy, the hallmark of which was weakness or complete paralysis of the muscles of one side of the face, with no sparing of the muscles of the forehead. However, not all facial paralysis is due to Bell’s palsy.
We present a case of a patient with a Bell’s palsy mimic to facilitate and guide the differential diagnosis and distinguish conditions from the classical presentation that Bell first described to the more concerning symptoms that may not be immediately obvious. Our case further underscores the importance of performing a thorough assessment to determine the presence of other neurological findings.
Case
A 61-year-old woman presented to the ED for evaluation of right facial droop and sensation of “room spinning.” The patient stated both symptoms began approximately 36 hours prior to presentation, upon awakening.
The patient denied any headache, neck or chest pain, extremity numbness, or weakness, but stated that she felt like she was going to fall toward her right side whenever she attempted to walk. The patient’s medical history was significant for hypertension, for which she was taking losartan. Her surgical history was notable for a left oophorectomy secondary to an ovarian cyst. Regarding the social history, the patient admitted to smoking 90 packs of cigarettes per year, but denied alcohol or illicit drug use.
Upon arrival at the ED, the patient’s vital signs were: blood pressure, 164/86 mm Hg: pulse, 89 beats/min; respiratory rate, 18 breaths/min; and temperature, 98.6°F. Oxygen saturation was 98% on room air.
Physical examination revealed the patient had a right facial droop consistent with right facial palsy. She was unable to wrinkle her right forehead or fully close her right eye. There were no field cuts on confrontation. The patient’s speech was noticeable for a mild dysarthria. The motor examination revealed mild weakness of the left upper extremity and impaired right facial sensation. There were no rashes noted on the face, head, or ears. The patient had slightly impaired hearing in the right ear, which was new in onset. The remainder of the physical examination was unremarkable.
Although the patient exhibited the classic signs of Bell’s palsy, including complete paralysis of the muscles of one side of the face, inability to wrinkle the muscle of the right forehead, and inability to fully close the right eye, she also had concerning symptoms of vertigo, dysarthria, and contralateral upper extremity weakness.
A computed tomography (CT) scan of the head was ordered, which revealed a large mass lesion centered in the right petrous apex, with an associated large component extending medially into the right cerebellopontine angle (CPA) that caused a mass effect on the adjacent brainstem (Figures 1a and 1b).
Upon these findings, the patient was transferred to another facility for neurosurgical evaluation. Magnetic resonance imaging (MRI) studies performed at the receiving hospital demonstrated a large expansile heterogeneous mass lesion centered in the right petrous apex with an associated large, probable hemorrhagic soft-tissue component extending medially into the right CPA, causing a mass effect on the adjacent brainstem and mild obstructive hydrocephalus (Figures 2a and 2b).
The patient was given dexamethasone 10 mg intravenously and taken to the operating room for a right suboccipital craniotomy with subtotal tumor removal. Intraoperative high-voltage stimulation of the fifth to eighth cranial nerves showed no response, indicating significant impairment.
While there were no intraoperative complications, the patient had significant postoperative dysphagia and resultant aspiration. A tracheostomy and percutaneous endoscopic gastrostomy tube were subsequently placed. Results of a biopsy taken during surgery identified an atypical meningioma. The patient remained in the hospital for 4 weeks, after which she was discharged to a long-term care (LTC) and rehabilitation facility.
A repeat CT scan taken 2 months after surgery demonstrated absence of the previously identified large mass (Figure 1b). Three months after discharge from the LTC-rehabilitation facility, MRI of the brain showed continued interval improvement of the previously noted mass centered in the right petrous apex (Figures 3a and 3b).
Discussion
Accounts of facial paralysis and facial nerve disorders have been noted throughout history and include accounts of the condition by Hippocrates.1 Bell’s palsy was named after surgeon Sir Charles Bell, who described a peripheral-nerve paralysis of the facial nerve in 1821. Bell’s work helped to elucidate the anatomy and functional role of the facial nerve.1,2
Signs and Symptoms
The classic presentation of Bell’s palsy is weakness or complete paralysis of the muscles of one side of the face, with no sparing of the muscles of the forehead. The eyelid on the affected side generally does not close, which can result in ocular irritation due to ineffective lubrication.
A scoring system has been developed by House and Brackmann which grades the degree impairment based on such characteristics as facial muscle function and eye closure.3,4 Approximately 96% of patients with a Bell’s palsy will improve to a House-Brackmann score of 2 or better within 1 year from diagnosis,5 and 85% of patients with Bell’s palsy will show at least some improvement within 3 weeks of onset (Table).2 Although the classic description of Bell’s palsy notes the condition as idiopathic, there is an increasing body of evidence in the literature showing a link to herpes simplex virus 1.5-7
Ramsey-Hunt Syndrome
The relationship between Bell’s palsy and Ramsey-Hunt syndrome is complex and controversial. Ramsey-Hunt syndrome is a constellation of possible complications from varicella-virus infection. Symptoms of Ramsey-Hunt syndrome include facial paralysis, tinnitus, hearing loss, vertigo, hyperacusis (increased sensitivity to certain frequencies and volume ranges of sound), and decreased ocular tearing.8 Due to the nature of symptoms associated with Ramsey-Hunt syndrome, it is apparent that the condition involves more than the seventh cranial nerve. In fact, studies have shown that Ramsey-Hunt syndrome can affect the fifth, sixth, eighth, and ninth cranial nerves.8
Ramsey-Hunt syndrome, which can present in the absence of cutaneous rash (referred to as zoster sine herpete), is estimated to occur in 8% to 20% of unilateral facial nerve palsies in adult patients.8,9 Regardless of the etiology of Bell’s palsy, a review of the literature makes it clear that facial nerve paralysis is not synonymous with Bell’s palsy.10 In one example, Yetter et al10 describe the case of a patient who, though initially diagnosed with Bell’s palsy, ultimately was found to have a facial palsy due to a parotid gland malignancy.
Likewise, Stomeo11 describes a case of a patient with facial paralysis and profound ipsilateral hearing loss who ultimately was found to have a mucoepithelial carcinoma of the parotid gland. In their report, the authors note that approximately 80% of facial nerve paralysis is due to Bell’s palsy, while 5% is due to malignancy.
In another report, Clemis12 describes a case in which a patient who initially was diagnosed with Bell’s palsy eventually was found to have an adenoid cystic carcinoma of the parotid. Thus, the authors appropriately emphasize in their report that “all that palsies is not Bell’s.”
Differential Diagnosis
Historical factors, including timing and duration of symptom onset, help to distinguish a Bell’s palsy from other disorders that can mimic this condition. In their study, Brach VanSwewaringen13 highlight the fact that “not all facial paralysis is Bell’s palsy.” In their review, the authors describe clues to help distinguish conditions that mimic Bell’s palsy. For example, maximal weakness from Bell’s Palsy typically occurs within 3 to 7 days from symptom onset, and that a more gradual onset of symptoms, with slow or negligible improvement over 6 to 12 months, is more indicative of a space-occupying lesion than Bell’s palsy.13It is, however, important to note that although the patient in our case had a central lesion, she experienced an acute onset of symptoms.
The presence of additional symptoms may also suggest an alternative diagnosis. Brach and VanSwearingen13 further noted that symptoms associated with the eighth nerve, such as vertigo, tinnitus, and hearing loss may be found in patients with a CPA tumor. In patients with larger tumors, ninth and 10th nerve symptoms, including the impaired hearing noted in our patient, may be present. Some patients with ninth and 10th nerve symptoms may perceive a sense of facial numbness, but actual sensory changes in the facial nerve distribution are unlikely in Bell’s palsy. Gustatory changes, however, are consistent with Bell’s palsy.
Ear pain is consistent with Bell’s palsy and is a signal to be vigilant for the possible emergence of an ear rash, which would suggest the diagnosis of herpes zoster oticus along the trajectory of Ramsey-Hunt syndrome. Facial pain in the area of the facial nerve is inconsistent with Bell’s palsy, while hyperacusis is consistent with Bell’s palsy. Hearing loss is an eighth nerve symptom that is inconsistent with Bell’s palsy.
Similarly, there are physical examination findings that can help distinguish a true Bell’s palsy from a mimic. Changes in tear production are consistent with Bell’s palsy, but imbalance and disequilibrium are not.14
As previously noted, the patient in this case had difficulty walking and felt as if she was falling toward her right side.
One way to organize the causes of facial paralysis has been proposed by Adour et al.15 In this system, etiologies are listed as either acute paralysis or chronic, progressive paralysis. Acute paralysis (ie, the sudden onset of symptoms with maximal severity within 2 weeks), of which Bell’s palsy is the most common, can be seen in cases of polyneuritis.
A new case of Bell’s palsy has been estimated to occur in the United States every 10 minutes.8 Guillain-Barré syndrome and Lyme disease are also in this category, as is Ramsey-Hunt syndrome. Patients with Lyme disease may have a history of a tick bite or rash.14
Trauma can also cause acute facial nerve paralysis (eg, blunt trauma-associated facial fracture, penetrating trauma, birth trauma). Unilateral central facial weakness can have a neurological cause, such as a lesion to the contralateral cortex, subcortical white matter, or internal capsule.2,15 Otitis media can sometimes cause facial paralysis.16 A cholesteatoma can cause acute facial paralysis.2 Malignancies cause 5% of all cases of facial paralysis. Primary parotid tumors of various types are in this category. Metastatic disease from breast, lung, skin, colon, and kidney may cause facial paralysis. As our case illustrates, CPA tumors can cause facial paralysis.15 It is important to also note that a patient can have both a Bell’s palsy and a concurrent disease. There are a number of case reports in the literature that describe acute onset of facial paralysis as a presenting symptom of malignancy.17 In addition, there are cases wherein a neurological finding on imaging, such as an acoustic neuroma, was presumed to be the cause of facial paralysis, yet the patient’s symptoms resolved in a manner consistent with Bell’s palsy.18
For example, Lagman et al19 described a patient in which a CPA lipoma was presumed to be the cause of the facial paralysis, but the eventual outcome showed the lipoma to have been an incidentaloma.
Conclusion
This case demonstrates a presenting symptom of facial palsy and the presence of a CPA tumor. The presence of vertigo along with other historical and physical examination findings inconsistent with Bell’s palsy prompted the CT scan of the head. A review of the literature suggests a number of important findings in patients with facial palsy to assist the clinician in distinguishing true Bell’s palsy from other diseases that can mimic this condition. This case serves as a reminder of the need to perform a thorough and diligent workup to determine the presence or absence of other neurologic findings prior to closing on the diagnosis of Bell’s palsy.
Facial paralysis is a common medical complaint—one that has fascinated ancient and contemporary physicians alike.1 An idiopathic facial nerve paresis involving the lower motor neuron was described in 1821 by Sir Charles Bell. This entity became known as a Bell’s palsy, the hallmark of which was weakness or complete paralysis of the muscles of one side of the face, with no sparing of the muscles of the forehead. However, not all facial paralysis is due to Bell’s palsy.
We present a case of a patient with a Bell’s palsy mimic to facilitate and guide the differential diagnosis and distinguish conditions from the classical presentation that Bell first described to the more concerning symptoms that may not be immediately obvious. Our case further underscores the importance of performing a thorough assessment to determine the presence of other neurological findings.
Case
A 61-year-old woman presented to the ED for evaluation of right facial droop and sensation of “room spinning.” The patient stated both symptoms began approximately 36 hours prior to presentation, upon awakening.
The patient denied any headache, neck or chest pain, extremity numbness, or weakness, but stated that she felt like she was going to fall toward her right side whenever she attempted to walk. The patient’s medical history was significant for hypertension, for which she was taking losartan. Her surgical history was notable for a left oophorectomy secondary to an ovarian cyst. Regarding the social history, the patient admitted to smoking 90 packs of cigarettes per year, but denied alcohol or illicit drug use.
Upon arrival at the ED, the patient’s vital signs were: blood pressure, 164/86 mm Hg: pulse, 89 beats/min; respiratory rate, 18 breaths/min; and temperature, 98.6°F. Oxygen saturation was 98% on room air.
Physical examination revealed the patient had a right facial droop consistent with right facial palsy. She was unable to wrinkle her right forehead or fully close her right eye. There were no field cuts on confrontation. The patient’s speech was noticeable for a mild dysarthria. The motor examination revealed mild weakness of the left upper extremity and impaired right facial sensation. There were no rashes noted on the face, head, or ears. The patient had slightly impaired hearing in the right ear, which was new in onset. The remainder of the physical examination was unremarkable.
Although the patient exhibited the classic signs of Bell’s palsy, including complete paralysis of the muscles of one side of the face, inability to wrinkle the muscle of the right forehead, and inability to fully close the right eye, she also had concerning symptoms of vertigo, dysarthria, and contralateral upper extremity weakness.
A computed tomography (CT) scan of the head was ordered, which revealed a large mass lesion centered in the right petrous apex, with an associated large component extending medially into the right cerebellopontine angle (CPA) that caused a mass effect on the adjacent brainstem (Figures 1a and 1b).
Upon these findings, the patient was transferred to another facility for neurosurgical evaluation. Magnetic resonance imaging (MRI) studies performed at the receiving hospital demonstrated a large expansile heterogeneous mass lesion centered in the right petrous apex with an associated large, probable hemorrhagic soft-tissue component extending medially into the right CPA, causing a mass effect on the adjacent brainstem and mild obstructive hydrocephalus (Figures 2a and 2b).
The patient was given dexamethasone 10 mg intravenously and taken to the operating room for a right suboccipital craniotomy with subtotal tumor removal. Intraoperative high-voltage stimulation of the fifth to eighth cranial nerves showed no response, indicating significant impairment.
While there were no intraoperative complications, the patient had significant postoperative dysphagia and resultant aspiration. A tracheostomy and percutaneous endoscopic gastrostomy tube were subsequently placed. Results of a biopsy taken during surgery identified an atypical meningioma. The patient remained in the hospital for 4 weeks, after which she was discharged to a long-term care (LTC) and rehabilitation facility.
A repeat CT scan taken 2 months after surgery demonstrated absence of the previously identified large mass (Figure 1b). Three months after discharge from the LTC-rehabilitation facility, MRI of the brain showed continued interval improvement of the previously noted mass centered in the right petrous apex (Figures 3a and 3b).
Discussion
Accounts of facial paralysis and facial nerve disorders have been noted throughout history and include accounts of the condition by Hippocrates.1 Bell’s palsy was named after surgeon Sir Charles Bell, who described a peripheral-nerve paralysis of the facial nerve in 1821. Bell’s work helped to elucidate the anatomy and functional role of the facial nerve.1,2
Signs and Symptoms
The classic presentation of Bell’s palsy is weakness or complete paralysis of the muscles of one side of the face, with no sparing of the muscles of the forehead. The eyelid on the affected side generally does not close, which can result in ocular irritation due to ineffective lubrication.
A scoring system has been developed by House and Brackmann which grades the degree impairment based on such characteristics as facial muscle function and eye closure.3,4 Approximately 96% of patients with a Bell’s palsy will improve to a House-Brackmann score of 2 or better within 1 year from diagnosis,5 and 85% of patients with Bell’s palsy will show at least some improvement within 3 weeks of onset (Table).2 Although the classic description of Bell’s palsy notes the condition as idiopathic, there is an increasing body of evidence in the literature showing a link to herpes simplex virus 1.5-7
Ramsey-Hunt Syndrome
The relationship between Bell’s palsy and Ramsey-Hunt syndrome is complex and controversial. Ramsey-Hunt syndrome is a constellation of possible complications from varicella-virus infection. Symptoms of Ramsey-Hunt syndrome include facial paralysis, tinnitus, hearing loss, vertigo, hyperacusis (increased sensitivity to certain frequencies and volume ranges of sound), and decreased ocular tearing.8 Due to the nature of symptoms associated with Ramsey-Hunt syndrome, it is apparent that the condition involves more than the seventh cranial nerve. In fact, studies have shown that Ramsey-Hunt syndrome can affect the fifth, sixth, eighth, and ninth cranial nerves.8
Ramsey-Hunt syndrome, which can present in the absence of cutaneous rash (referred to as zoster sine herpete), is estimated to occur in 8% to 20% of unilateral facial nerve palsies in adult patients.8,9 Regardless of the etiology of Bell’s palsy, a review of the literature makes it clear that facial nerve paralysis is not synonymous with Bell’s palsy.10 In one example, Yetter et al10 describe the case of a patient who, though initially diagnosed with Bell’s palsy, ultimately was found to have a facial palsy due to a parotid gland malignancy.
Likewise, Stomeo11 describes a case of a patient with facial paralysis and profound ipsilateral hearing loss who ultimately was found to have a mucoepithelial carcinoma of the parotid gland. In their report, the authors note that approximately 80% of facial nerve paralysis is due to Bell’s palsy, while 5% is due to malignancy.
In another report, Clemis12 describes a case in which a patient who initially was diagnosed with Bell’s palsy eventually was found to have an adenoid cystic carcinoma of the parotid. Thus, the authors appropriately emphasize in their report that “all that palsies is not Bell’s.”
Differential Diagnosis
Historical factors, including timing and duration of symptom onset, help to distinguish a Bell’s palsy from other disorders that can mimic this condition. In their study, Brach VanSwewaringen13 highlight the fact that “not all facial paralysis is Bell’s palsy.” In their review, the authors describe clues to help distinguish conditions that mimic Bell’s palsy. For example, maximal weakness from Bell’s Palsy typically occurs within 3 to 7 days from symptom onset, and that a more gradual onset of symptoms, with slow or negligible improvement over 6 to 12 months, is more indicative of a space-occupying lesion than Bell’s palsy.13It is, however, important to note that although the patient in our case had a central lesion, she experienced an acute onset of symptoms.
The presence of additional symptoms may also suggest an alternative diagnosis. Brach and VanSwearingen13 further noted that symptoms associated with the eighth nerve, such as vertigo, tinnitus, and hearing loss may be found in patients with a CPA tumor. In patients with larger tumors, ninth and 10th nerve symptoms, including the impaired hearing noted in our patient, may be present. Some patients with ninth and 10th nerve symptoms may perceive a sense of facial numbness, but actual sensory changes in the facial nerve distribution are unlikely in Bell’s palsy. Gustatory changes, however, are consistent with Bell’s palsy.
Ear pain is consistent with Bell’s palsy and is a signal to be vigilant for the possible emergence of an ear rash, which would suggest the diagnosis of herpes zoster oticus along the trajectory of Ramsey-Hunt syndrome. Facial pain in the area of the facial nerve is inconsistent with Bell’s palsy, while hyperacusis is consistent with Bell’s palsy. Hearing loss is an eighth nerve symptom that is inconsistent with Bell’s palsy.
Similarly, there are physical examination findings that can help distinguish a true Bell’s palsy from a mimic. Changes in tear production are consistent with Bell’s palsy, but imbalance and disequilibrium are not.14
As previously noted, the patient in this case had difficulty walking and felt as if she was falling toward her right side.
One way to organize the causes of facial paralysis has been proposed by Adour et al.15 In this system, etiologies are listed as either acute paralysis or chronic, progressive paralysis. Acute paralysis (ie, the sudden onset of symptoms with maximal severity within 2 weeks), of which Bell’s palsy is the most common, can be seen in cases of polyneuritis.
A new case of Bell’s palsy has been estimated to occur in the United States every 10 minutes.8 Guillain-Barré syndrome and Lyme disease are also in this category, as is Ramsey-Hunt syndrome. Patients with Lyme disease may have a history of a tick bite or rash.14
Trauma can also cause acute facial nerve paralysis (eg, blunt trauma-associated facial fracture, penetrating trauma, birth trauma). Unilateral central facial weakness can have a neurological cause, such as a lesion to the contralateral cortex, subcortical white matter, or internal capsule.2,15 Otitis media can sometimes cause facial paralysis.16 A cholesteatoma can cause acute facial paralysis.2 Malignancies cause 5% of all cases of facial paralysis. Primary parotid tumors of various types are in this category. Metastatic disease from breast, lung, skin, colon, and kidney may cause facial paralysis. As our case illustrates, CPA tumors can cause facial paralysis.15 It is important to also note that a patient can have both a Bell’s palsy and a concurrent disease. There are a number of case reports in the literature that describe acute onset of facial paralysis as a presenting symptom of malignancy.17 In addition, there are cases wherein a neurological finding on imaging, such as an acoustic neuroma, was presumed to be the cause of facial paralysis, yet the patient’s symptoms resolved in a manner consistent with Bell’s palsy.18
For example, Lagman et al19 described a patient in which a CPA lipoma was presumed to be the cause of the facial paralysis, but the eventual outcome showed the lipoma to have been an incidentaloma.
Conclusion
This case demonstrates a presenting symptom of facial palsy and the presence of a CPA tumor. The presence of vertigo along with other historical and physical examination findings inconsistent with Bell’s palsy prompted the CT scan of the head. A review of the literature suggests a number of important findings in patients with facial palsy to assist the clinician in distinguishing true Bell’s palsy from other diseases that can mimic this condition. This case serves as a reminder of the need to perform a thorough and diligent workup to determine the presence or absence of other neurologic findings prior to closing on the diagnosis of Bell’s palsy.
1. Glicenstein J. Ann Chir Plast Esthet. 2015;60(5):347-362. doi:10.1016/j.anplas.2015.05.007.
2. Tiemstra JD, Khatkhate N. Bell’s palsy: diagnosis and management. Am Fam Physician. 2007;76(7):997-1002.
3. House JW, Brackmann DE. Facial nerve grading system. Otolaryngol Head Neck Surg. 1985;93(2):146-147. doi:10.1177/019459988509300202.
4. Reitzen SD, Babb JS, Lalwani AK. Significance and reliability of the House-Brackmann grading system for regional facial nerve function. Otolaryngol Head Neck Surg. 2009;140(2):154-158. doi:10.1016/j.otohns.2008.11.021.
5. Yeo SW, Lee DH, Jun BC, Chang KH, Park YS. Analysis of prognostic factors in Bell’s palsy and Ramsay Hunt syndrome. Auris Nasus Larynx. 2007;34(2):159-164. doi:10.1016/j.anl.2006.09.005.
6. Ahmed A. When is facial paralysis Bell palsy? Current diagnosis and treatment. Cleve Clin J Med. 2005;72(5):398-401, 405.
7. Gilden DH. Clinical practice. Bell’s palsy. N Engl J Med. 2004;351(13):1323-1331. doi:10.1056/NEJMcp041120.
8. Adour KK. Otological complications of herpes zoster. Ann Neurol. 1994;35:Suppl:S62-S64.
9. Furuta Y, Ohtani F, Mesuda Y, Fukuda S, Inuyama Y. Early diagnosis of zoster sine herpete and antiviral therapy for the treatment of facial palsy. Neurology. 2000;55(5):708-710.
10. Yetter MF, Ogren FP, Moore GF, Yonkers AJ. Bell’s palsy: a facial nerve paralysis diagnosis of exclusion. Nebr Med J. 1990;75(5):109-116.
11. Stomeo F. Possibilities of diagnostic errors in paralysis of the 7th cranial nerve. Acta Otorhinolaryngol Ital. 1989;9(6):629-633.
12. Clemis JD. All that palsies is not Bell’s: Bell’s palsy due to adenoid cystic carcinoma of the parotid. Am J Otol. 1991;12(5):397.
13. Brach JS, VanSwearingen JM. Not all facial paralysis is Bell’s palsy: a case report. Arch Phys Med Rehabil. 1999;80(7):857-859.
14. Albers JR, Tamang S. Common questions about Bell palsy. Am Fam Physician. 2014;89(3):209-212.
15. Adour KK, Hilsinger RL Jr, Callan EJ. Facial paralysis and Bell’s palsy: a protocol for differential diagnosis. Am J Otol. 1985;Suppl:68-73.
16. Morrow MJ. Bell’s palsy and herpes zoster. Curr Treat Options Neurol. 2000;2(5):407-416.
17. Quesnel AM, Lindsay RW, Hadlock TA. When the bell tolls on Bell’s palsy: finding occult malignancy in acute-onset facial paralysis. Am J Otolaryngol. 2010;31(5):339-342. doi:10.1016/j.amjoto.2009.04.003.
18. Kaushal A, Curran WJ Jr. For whom the Bell’s palsy tolls? Am J Clin Oncol. 2009;32(4):450-451. doi:10.1097/01.coc.0000239141.22916.22.
19. Lagman C, Choy W, Lee SJ, et al. A Case of Bell’s palsy with an incidental finding of a cerebellopontine angle lipoma. Cureus. 2016;8(8):e747. doi:10.7759/cureus.747.
1. Glicenstein J. Ann Chir Plast Esthet. 2015;60(5):347-362. doi:10.1016/j.anplas.2015.05.007.
2. Tiemstra JD, Khatkhate N. Bell’s palsy: diagnosis and management. Am Fam Physician. 2007;76(7):997-1002.
3. House JW, Brackmann DE. Facial nerve grading system. Otolaryngol Head Neck Surg. 1985;93(2):146-147. doi:10.1177/019459988509300202.
4. Reitzen SD, Babb JS, Lalwani AK. Significance and reliability of the House-Brackmann grading system for regional facial nerve function. Otolaryngol Head Neck Surg. 2009;140(2):154-158. doi:10.1016/j.otohns.2008.11.021.
5. Yeo SW, Lee DH, Jun BC, Chang KH, Park YS. Analysis of prognostic factors in Bell’s palsy and Ramsay Hunt syndrome. Auris Nasus Larynx. 2007;34(2):159-164. doi:10.1016/j.anl.2006.09.005.
6. Ahmed A. When is facial paralysis Bell palsy? Current diagnosis and treatment. Cleve Clin J Med. 2005;72(5):398-401, 405.
7. Gilden DH. Clinical practice. Bell’s palsy. N Engl J Med. 2004;351(13):1323-1331. doi:10.1056/NEJMcp041120.
8. Adour KK. Otological complications of herpes zoster. Ann Neurol. 1994;35:Suppl:S62-S64.
9. Furuta Y, Ohtani F, Mesuda Y, Fukuda S, Inuyama Y. Early diagnosis of zoster sine herpete and antiviral therapy for the treatment of facial palsy. Neurology. 2000;55(5):708-710.
10. Yetter MF, Ogren FP, Moore GF, Yonkers AJ. Bell’s palsy: a facial nerve paralysis diagnosis of exclusion. Nebr Med J. 1990;75(5):109-116.
11. Stomeo F. Possibilities of diagnostic errors in paralysis of the 7th cranial nerve. Acta Otorhinolaryngol Ital. 1989;9(6):629-633.
12. Clemis JD. All that palsies is not Bell’s: Bell’s palsy due to adenoid cystic carcinoma of the parotid. Am J Otol. 1991;12(5):397.
13. Brach JS, VanSwearingen JM. Not all facial paralysis is Bell’s palsy: a case report. Arch Phys Med Rehabil. 1999;80(7):857-859.
14. Albers JR, Tamang S. Common questions about Bell palsy. Am Fam Physician. 2014;89(3):209-212.
15. Adour KK, Hilsinger RL Jr, Callan EJ. Facial paralysis and Bell’s palsy: a protocol for differential diagnosis. Am J Otol. 1985;Suppl:68-73.
16. Morrow MJ. Bell’s palsy and herpes zoster. Curr Treat Options Neurol. 2000;2(5):407-416.
17. Quesnel AM, Lindsay RW, Hadlock TA. When the bell tolls on Bell’s palsy: finding occult malignancy in acute-onset facial paralysis. Am J Otolaryngol. 2010;31(5):339-342. doi:10.1016/j.amjoto.2009.04.003.
18. Kaushal A, Curran WJ Jr. For whom the Bell’s palsy tolls? Am J Clin Oncol. 2009;32(4):450-451. doi:10.1097/01.coc.0000239141.22916.22.
19. Lagman C, Choy W, Lee SJ, et al. A Case of Bell’s palsy with an incidental finding of a cerebellopontine angle lipoma. Cureus. 2016;8(8):e747. doi:10.7759/cureus.747.
