Case Reports

Glomus tumors are neoplasms that originate from normal glomus bodies in the skin and are most commonly found in the subungual areas of the digits.¹ Glomus bodies are neuromyoarterial structures in the reticular dermis that serve as specialized arteriovenous anastomoses. These bodies contain afferent arterioles and efferent veins with multiple connections, and glomus cells have contractile properties because of their similarity to smooth muscle cells.^1,2 Glomus bodies help regulate blood flow and temperature of the skin and are found in their largest concentration in the fingertips, palms of the hands, and soles of the feet.^3,4

Glomus tumors represent hyperplastic glomus bodies and make up 1% to 4.5% of upper extremity neoplasms, with approximately 75% in the hand and 50% in the subungual area.^1,5,6 These tumors can also present in multiple locations at once and can occur in atypical and ectopic locations.³ Although generally benign, glomus tumors can also exhibit malignant and metastatic potential in rare cases.^7,8 They can also be locally aggressive with bony destruction of the distal phalynx.^2,9,10 Tumors typically present as painful solitary soft-tissue lesions that are exquisitely tender to palpation, dark red-purple or bluish, and hypersensitive to cold.^5,10 Van Geertruyden and colleagues¹⁰ reported that the diagnosis of glomus tumor can be made clinically in 90% of cases. However, glomus tumors can easily be mistaken for other lesions, such as hemangiomas, angiomas, neuromas, neurofibromas, lipomas, and ganglion cysts. An inaccurate or incomplete workup can result in persistent pain and symptoms along with intraoperative complications.³ Magnetic resonance imaging (MRI), the most sensitive imaging modality for detecting glomus tumors of the hand, can assist in the workup.^3,11,12

Extradigital glomus tumors are difficult to diagnose because of their rarity and unspecific symptoms and presentation.¹³ Misdiagnosis and delayed diagnosis can result in significant chronic pain, disuse syndromes, and disability.^1,10 Correct diagnosis and surgical resection are generally curative with complete resolution of symptoms.

In this article, we report a case of a large atypical glomus tumor that occurred on the wrist and involved the radial artery. This tumor was successfully treated with surgical excision. The patient provided written informed consent for print and electronic publication of this case report.

Case Report

A 63-year-old man presented to clinic with an extremely tender soft-tissue mass on his nondominant, left wrist. The mass had been increasing in size for a year. It was painless at rest but very painful to light palpation, with referred pain proximally up to the shoulder.

The patient did not recall any traumatic or inciting event, had not undergone any prior workup or treatment for symptoms, and had no history of masses elsewhere on the body. Past medical history was significant for type 2 diabetes and colon and prostate cancer, which had been treated with chemotherapy and was now in remission.

Physical examination revealed a 2×2.5-cm well-circumscribed soft-tissue mass on the volar-radial aspect of the left wrist proximal to the thenar eminence and radial to the flexor carpi radialis tendon (Figure 1). The mass was soft, mobile, and nonfluctuant and did not transilluminate. The overlying skin was normal in color and appearance—no discoloration, erythema, wounds, or drainage. The radial artery was palpable, and the mass did not pulsate or have a bruit. The patient had normal wrist range of motion limited by pain on compression of the mass with motor and sensation intact throughout the hand. Plain radiographs of the wrist showed no bony pathology or involvement from the mass. A soft-tissue shadow was visible around the wrist without calcifications. A wrist MRI was performed to better evaluate the mass, and the T2-weighted images showed a heterogeneous subcutaneous mass adjacent to the radial artery with increased signal intensity from surrounding feeding vessels (Figure 2).

Given the clinical and imaging findings, there was concern for a possible vascular tumor. Therefore, excisional biopsy was recommended over needle biopsy because of the bleeding risk. With the patient under general anesthesia, and a tourniquet used without exsanguination, a Brunner-type zigzag incision was made centered over the mass with elevated skin flaps. The 2.7×2.6×1.1-cm mass was superficial and involved the radial artery (Figure 3). After the radial artery was dissected proximally and distally, 2 perforating vessels were found entering the mass. These vessels were ligated, which allowed the mass to be peeled completely off the artery. Histology with hematoxylin-eosin staining showed solid sheets of uniform round cells with interspersed capillaries and centrally placed nuclei without evidence of malignancy (Figure 4).

The tourniquet was released before skin closure, and adequate hemostasis was obtained. The wound was closed, and the patient was placed in a volar wrist splint for immobilization. Pain relief after excision of the mass was immediate, and the postoperative course uneventful. After surgery, immunohistochemistry of the mass showed minimal mitotic activity, with a positive immunoperoxidase stain for smooth muscle actin confirming a diagnosis of glomus tumor (Figure 5). At 3-year follow-up, the patient had no pain, symptoms, or tumor recurrence.

Discussion

Glomus tumors are an established cause of pain in the subungual areas of the hand; numerous cases have been reported.^1,5,10,14 However, extradigital glomus tumors, particularly those involving the wrist, are rare, and only a few have been described. Given the lack of consistent findings and presentations, diagnosis is difficult. Case series have documented an overall 2:1 female-to-male predominance of glomus tumors,⁶ but extradigital tumors are more common in men (4.6:1 male-to-female ratio).³ Extradigital glomus tumors are commonly diagnosed between ages 40 and 80 years. Classic symptoms of subungual tumors include pain, localized tenderness, and cold hypersensitivity,^1,10 but symptoms are much more variable with extradigital locations. Previous trauma or injury to the lesion area is reported in 20% to 30% of cases before symptom onset.^3,15 Intravascular locations of glomus tumors are extremely rare; only 4 cases of tumors involving venous structures have been reported.^16-19 In the present case, the patient’s main complaints were pain and localized tenderness associated with a progressively increasing mass without any history of trauma. The large size of his mass (~2.5 cm in diameter) on examination was unique, as was involvement of the radial artery.

Misdiagnosis and delayed diagnosis of extradigital glomus tumors are common, and symptoms such as chronic pain typically persist for 7 to 11 years before the correct diagnosis is made.^1,10 On average, 2.5 physician consultants (including psychiatrists) evaluate the patient before glomus tumor is identified.¹⁰ There are other reports of atypical or ectopic glomus tumors taking 5 to 25 years to be diagnosed.^20-22 The differential diagnosis for glomus tumors includes hemangiomas, cellular or cavernous hemangiomas, vascular tumors, neuromas, neurofibromas, lipomas, paragangliomas, ganglion cysts, pigmented nevi, Pacinian corpuscle hyperplasia, and foreign bodies. A key element of clinical diagnosis is the disproportionate amount of pain and localized tenderness caused by the lesion relative to its size. The hypersensitivity of this tumor is thought to result from enlargement of the tumor and impingement on nearby Pacinian corpuscles, nerve endings in the skin that are responsible for sensitivity to vibration and pressure.^2,9

Plain radiographs can be useful in detecting glomus tumors of the hand but are less helpful with extradigital tumors, with identification rates of 24% in certain series.³ MRI is the most sensitive imaging modality for diagnosing glomus tumors of the hand; a detection rate of 80% to 100% has been reported in various case series.^3,11,12 Specificity of MRI for glomus tumors has been reported at 50%.^11,23 Placement of a radiographic marker directly over the area of most pain can assist in tumor localization.³ Glomus tumors typically have decreased signal intensity on T1-weighted images and increased intensity on T2-weighted images, but signal patterns are variable and particularly difficult to differentiate with small tumors. MRI is useful in the setting of recurrent glomus tumors, where incomplete excision is possible. In 24 cases of continued pain after glomus tumor excision, Theumann and colleagues²⁴ used MRI to identify a nodule consistent with recurrent glomus tumor in all patients. Three-dimensional contrast-enhanced magnetic resonance angiography (MRA) can also help diagnose glomus tumors while providing valuable information regarding size and location for surgical planning.^25,26 With MRA, it is crucial to evaluate the arterial or arteriovenous phase of imaging, as the glomus tumor is richly vascularized and shows contrast enhancement after intravenous injection of gadolinium.²⁷ Angiography, ultrasonography, thermography, and scintigraphy have all been used to diagnose glomus tumors but have shown limited utility and accuracy.¹¹

Treatment of glomus tumors is complete surgical excision because of their relatively small size and subcutaneous location. Resection success rates are consistently higher than 95%, with resolution of all symptoms.^1,10,14 Local recurrence of tumors after excision occurs in 1% to 33% of cases, depending on series, and may be immediate or delayed, with immediate recurrence commonly caused by inadequate excision.^1,10,15,28 Delayed recurrence is less common and presents several years after excision, typically with a new growth near the previous excision.¹⁰ Recurrence years after surgery may also represent multiple tumors unrecognized during initial workup and can be treated with repeat excision or radiotherapy.

Robert and colleagues²⁹ recently reported the case of a glomus tumor, on the dorsal aspect of the wrist, discovered incidentally in a 71-year-old patient and treated with surgical excision. Several years earlier, Chim and colleagues³⁰ described a similar case, of a large wrist glomus tumor worked up with MRI. In a retrospective review of all extradigital glomus tumors seen over a 20-year period, Schiefer and colleagues³ reported 4 glomus tumors of the wrist out of 56 tumors total. The most common sites were forearm (11 cases) and knee (10 cases), and the majority of patients presented with pain and localized tenderness. Mean tumor size was 0.66 cm (range, 0.1-0.3 cm), with 77% of tumors less than 1 cm. Our patient’s 2.7×2.6×1.1-cm tumor was large for a glomus tumor. Its involvement with the radial artery feeding vessels likely contributed to its large and progressively increasing size. It is worth noting that, in the series by Schiefer and colleagues,³ the only patient with symptoms persisting after excision had a large (3 cm in diameter) deep tumor of the foot; the entire tumor was removed, and there was no recurrence by 10-year follow-up. Folpe and colleagues⁷ suggested that deep tumors larger than 2 cm should be at higher suspicion for malignancy. Joseph and Posner²¹ reported 3 cases of glomus tumors, on the ulnar side of the wrist, diagnosed with help of a provocative test using ethyl chloride spray.

Conclusion

Overall, glomus tumors are rare and challenging to diagnosis and should be in the differential in any symptomatic patient with a painful soft-tissue mass of the wrist. Advanced imaging studies, such as MRI, can assist in localization, diagnosis, and preoperative planning. Histology and immunohistochemistry are essential to differentiate glomus tumor from other vascular tumors, and complete excision is necessary to prevent local recurrence.

Glomus tumors are neoplasms that originate from normal glomus bodies in the skin and are most commonly found in the subungual areas of the digits.¹ Glomus bodies are neuromyoarterial structures in the reticular dermis that serve as specialized arteriovenous anastomoses. These bodies contain afferent arterioles and efferent veins with multiple connections, and glomus cells have contractile properties because of their similarity to smooth muscle cells.^1,2 Glomus bodies help regulate blood flow and temperature of the skin and are found in their largest concentration in the fingertips, palms of the hands, and soles of the feet.^3,4

Glomus tumors represent hyperplastic glomus bodies and make up 1% to 4.5% of upper extremity neoplasms, with approximately 75% in the hand and 50% in the subungual area.^1,5,6 These tumors can also present in multiple locations at once and can occur in atypical and ectopic locations.³ Although generally benign, glomus tumors can also exhibit malignant and metastatic potential in rare cases.^7,8 They can also be locally aggressive with bony destruction of the distal phalynx.^2,9,10 Tumors typically present as painful solitary soft-tissue lesions that are exquisitely tender to palpation, dark red-purple or bluish, and hypersensitive to cold.^5,10 Van Geertruyden and colleagues¹⁰ reported that the diagnosis of glomus tumor can be made clinically in 90% of cases. However, glomus tumors can easily be mistaken for other lesions, such as hemangiomas, angiomas, neuromas, neurofibromas, lipomas, and ganglion cysts. An inaccurate or incomplete workup can result in persistent pain and symptoms along with intraoperative complications.³ Magnetic resonance imaging (MRI), the most sensitive imaging modality for detecting glomus tumors of the hand, can assist in the workup.^3,11,12

Extradigital glomus tumors are difficult to diagnose because of their rarity and unspecific symptoms and presentation.¹³ Misdiagnosis and delayed diagnosis can result in significant chronic pain, disuse syndromes, and disability.^1,10 Correct diagnosis and surgical resection are generally curative with complete resolution of symptoms.

In this article, we report a case of a large atypical glomus tumor that occurred on the wrist and involved the radial artery. This tumor was successfully treated with surgical excision. The patient provided written informed consent for print and electronic publication of this case report.

Case Report

A 63-year-old man presented to clinic with an extremely tender soft-tissue mass on his nondominant, left wrist. The mass had been increasing in size for a year. It was painless at rest but very painful to light palpation, with referred pain proximally up to the shoulder.

The patient did not recall any traumatic or inciting event, had not undergone any prior workup or treatment for symptoms, and had no history of masses elsewhere on the body. Past medical history was significant for type 2 diabetes and colon and prostate cancer, which had been treated with chemotherapy and was now in remission.

Physical examination revealed a 2×2.5-cm well-circumscribed soft-tissue mass on the volar-radial aspect of the left wrist proximal to the thenar eminence and radial to the flexor carpi radialis tendon (Figure 1). The mass was soft, mobile, and nonfluctuant and did not transilluminate. The overlying skin was normal in color and appearance—no discoloration, erythema, wounds, or drainage. The radial artery was palpable, and the mass did not pulsate or have a bruit. The patient had normal wrist range of motion limited by pain on compression of the mass with motor and sensation intact throughout the hand. Plain radiographs of the wrist showed no bony pathology or involvement from the mass. A soft-tissue shadow was visible around the wrist without calcifications. A wrist MRI was performed to better evaluate the mass, and the T2-weighted images showed a heterogeneous subcutaneous mass adjacent to the radial artery with increased signal intensity from surrounding feeding vessels (Figure 2).

Given the clinical and imaging findings, there was concern for a possible vascular tumor. Therefore, excisional biopsy was recommended over needle biopsy because of the bleeding risk. With the patient under general anesthesia, and a tourniquet used without exsanguination, a Brunner-type zigzag incision was made centered over the mass with elevated skin flaps. The 2.7×2.6×1.1-cm mass was superficial and involved the radial artery (Figure 3). After the radial artery was dissected proximally and distally, 2 perforating vessels were found entering the mass. These vessels were ligated, which allowed the mass to be peeled completely off the artery. Histology with hematoxylin-eosin staining showed solid sheets of uniform round cells with interspersed capillaries and centrally placed nuclei without evidence of malignancy (Figure 4).

The tourniquet was released before skin closure, and adequate hemostasis was obtained. The wound was closed, and the patient was placed in a volar wrist splint for immobilization. Pain relief after excision of the mass was immediate, and the postoperative course uneventful. After surgery, immunohistochemistry of the mass showed minimal mitotic activity, with a positive immunoperoxidase stain for smooth muscle actin confirming a diagnosis of glomus tumor (Figure 5). At 3-year follow-up, the patient had no pain, symptoms, or tumor recurrence.

Discussion

Glomus tumors are an established cause of pain in the subungual areas of the hand; numerous cases have been reported.^1,5,10,14 However, extradigital glomus tumors, particularly those involving the wrist, are rare, and only a few have been described. Given the lack of consistent findings and presentations, diagnosis is difficult. Case series have documented an overall 2:1 female-to-male predominance of glomus tumors,⁶ but extradigital tumors are more common in men (4.6:1 male-to-female ratio).³ Extradigital glomus tumors are commonly diagnosed between ages 40 and 80 years. Classic symptoms of subungual tumors include pain, localized tenderness, and cold hypersensitivity,^1,10 but symptoms are much more variable with extradigital locations. Previous trauma or injury to the lesion area is reported in 20% to 30% of cases before symptom onset.^3,15 Intravascular locations of glomus tumors are extremely rare; only 4 cases of tumors involving venous structures have been reported.^16-19 In the present case, the patient’s main complaints were pain and localized tenderness associated with a progressively increasing mass without any history of trauma. The large size of his mass (~2.5 cm in diameter) on examination was unique, as was involvement of the radial artery.

Misdiagnosis and delayed diagnosis of extradigital glomus tumors are common, and symptoms such as chronic pain typically persist for 7 to 11 years before the correct diagnosis is made.^1,10 On average, 2.5 physician consultants (including psychiatrists) evaluate the patient before glomus tumor is identified.¹⁰ There are other reports of atypical or ectopic glomus tumors taking 5 to 25 years to be diagnosed.^20-22 The differential diagnosis for glomus tumors includes hemangiomas, cellular or cavernous hemangiomas, vascular tumors, neuromas, neurofibromas, lipomas, paragangliomas, ganglion cysts, pigmented nevi, Pacinian corpuscle hyperplasia, and foreign bodies. A key element of clinical diagnosis is the disproportionate amount of pain and localized tenderness caused by the lesion relative to its size. The hypersensitivity of this tumor is thought to result from enlargement of the tumor and impingement on nearby Pacinian corpuscles, nerve endings in the skin that are responsible for sensitivity to vibration and pressure.^2,9

Plain radiographs can be useful in detecting glomus tumors of the hand but are less helpful with extradigital tumors, with identification rates of 24% in certain series.³ MRI is the most sensitive imaging modality for diagnosing glomus tumors of the hand; a detection rate of 80% to 100% has been reported in various case series.^3,11,12 Specificity of MRI for glomus tumors has been reported at 50%.^11,23 Placement of a radiographic marker directly over the area of most pain can assist in tumor localization.³ Glomus tumors typically have decreased signal intensity on T1-weighted images and increased intensity on T2-weighted images, but signal patterns are variable and particularly difficult to differentiate with small tumors. MRI is useful in the setting of recurrent glomus tumors, where incomplete excision is possible. In 24 cases of continued pain after glomus tumor excision, Theumann and colleagues²⁴ used MRI to identify a nodule consistent with recurrent glomus tumor in all patients. Three-dimensional contrast-enhanced magnetic resonance angiography (MRA) can also help diagnose glomus tumors while providing valuable information regarding size and location for surgical planning.^25,26 With MRA, it is crucial to evaluate the arterial or arteriovenous phase of imaging, as the glomus tumor is richly vascularized and shows contrast enhancement after intravenous injection of gadolinium.²⁷ Angiography, ultrasonography, thermography, and scintigraphy have all been used to diagnose glomus tumors but have shown limited utility and accuracy.¹¹

Treatment of glomus tumors is complete surgical excision because of their relatively small size and subcutaneous location. Resection success rates are consistently higher than 95%, with resolution of all symptoms.^1,10,14 Local recurrence of tumors after excision occurs in 1% to 33% of cases, depending on series, and may be immediate or delayed, with immediate recurrence commonly caused by inadequate excision.^1,10,15,28 Delayed recurrence is less common and presents several years after excision, typically with a new growth near the previous excision.¹⁰ Recurrence years after surgery may also represent multiple tumors unrecognized during initial workup and can be treated with repeat excision or radiotherapy.

Robert and colleagues²⁹ recently reported the case of a glomus tumor, on the dorsal aspect of the wrist, discovered incidentally in a 71-year-old patient and treated with surgical excision. Several years earlier, Chim and colleagues³⁰ described a similar case, of a large wrist glomus tumor worked up with MRI. In a retrospective review of all extradigital glomus tumors seen over a 20-year period, Schiefer and colleagues³ reported 4 glomus tumors of the wrist out of 56 tumors total. The most common sites were forearm (11 cases) and knee (10 cases), and the majority of patients presented with pain and localized tenderness. Mean tumor size was 0.66 cm (range, 0.1-0.3 cm), with 77% of tumors less than 1 cm. Our patient’s 2.7×2.6×1.1-cm tumor was large for a glomus tumor. Its involvement with the radial artery feeding vessels likely contributed to its large and progressively increasing size. It is worth noting that, in the series by Schiefer and colleagues,³ the only patient with symptoms persisting after excision had a large (3 cm in diameter) deep tumor of the foot; the entire tumor was removed, and there was no recurrence by 10-year follow-up. Folpe and colleagues⁷ suggested that deep tumors larger than 2 cm should be at higher suspicion for malignancy. Joseph and Posner²¹ reported 3 cases of glomus tumors, on the ulnar side of the wrist, diagnosed with help of a provocative test using ethyl chloride spray.

Conclusion

Overall, glomus tumors are rare and challenging to diagnosis and should be in the differential in any symptomatic patient with a painful soft-tissue mass of the wrist. Advanced imaging studies, such as MRI, can assist in localization, diagnosis, and preoperative planning. Histology and immunohistochemistry are essential to differentiate glomus tumor from other vascular tumors, and complete excision is necessary to prevent local recurrence.

Glomus tumors are neoplasms that originate from normal glomus bodies in the skin and are most commonly found in the subungual areas of the digits.¹ Glomus bodies are neuromyoarterial structures in the reticular dermis that serve as specialized arteriovenous anastomoses. These bodies contain afferent arterioles and efferent veins with multiple connections, and glomus cells have contractile properties because of their similarity to smooth muscle cells.^1,2 Glomus bodies help regulate blood flow and temperature of the skin and are found in their largest concentration in the fingertips, palms of the hands, and soles of the feet.^3,4

Glomus tumors represent hyperplastic glomus bodies and make up 1% to 4.5% of upper extremity neoplasms, with approximately 75% in the hand and 50% in the subungual area.^1,5,6 These tumors can also present in multiple locations at once and can occur in atypical and ectopic locations.³ Although generally benign, glomus tumors can also exhibit malignant and metastatic potential in rare cases.^7,8 They can also be locally aggressive with bony destruction of the distal phalynx.^2,9,10 Tumors typically present as painful solitary soft-tissue lesions that are exquisitely tender to palpation, dark red-purple or bluish, and hypersensitive to cold.^5,10 Van Geertruyden and colleagues¹⁰ reported that the diagnosis of glomus tumor can be made clinically in 90% of cases. However, glomus tumors can easily be mistaken for other lesions, such as hemangiomas, angiomas, neuromas, neurofibromas, lipomas, and ganglion cysts. An inaccurate or incomplete workup can result in persistent pain and symptoms along with intraoperative complications.³ Magnetic resonance imaging (MRI), the most sensitive imaging modality for detecting glomus tumors of the hand, can assist in the workup.^3,11,12

Extradigital glomus tumors are difficult to diagnose because of their rarity and unspecific symptoms and presentation.¹³ Misdiagnosis and delayed diagnosis can result in significant chronic pain, disuse syndromes, and disability.^1,10 Correct diagnosis and surgical resection are generally curative with complete resolution of symptoms.

In this article, we report a case of a large atypical glomus tumor that occurred on the wrist and involved the radial artery. This tumor was successfully treated with surgical excision. The patient provided written informed consent for print and electronic publication of this case report.

Case Report

A 63-year-old man presented to clinic with an extremely tender soft-tissue mass on his nondominant, left wrist. The mass had been increasing in size for a year. It was painless at rest but very painful to light palpation, with referred pain proximally up to the shoulder.

The patient did not recall any traumatic or inciting event, had not undergone any prior workup or treatment for symptoms, and had no history of masses elsewhere on the body. Past medical history was significant for type 2 diabetes and colon and prostate cancer, which had been treated with chemotherapy and was now in remission.

Physical examination revealed a 2×2.5-cm well-circumscribed soft-tissue mass on the volar-radial aspect of the left wrist proximal to the thenar eminence and radial to the flexor carpi radialis tendon (Figure 1). The mass was soft, mobile, and nonfluctuant and did not transilluminate. The overlying skin was normal in color and appearance—no discoloration, erythema, wounds, or drainage. The radial artery was palpable, and the mass did not pulsate or have a bruit. The patient had normal wrist range of motion limited by pain on compression of the mass with motor and sensation intact throughout the hand. Plain radiographs of the wrist showed no bony pathology or involvement from the mass. A soft-tissue shadow was visible around the wrist without calcifications. A wrist MRI was performed to better evaluate the mass, and the T2-weighted images showed a heterogeneous subcutaneous mass adjacent to the radial artery with increased signal intensity from surrounding feeding vessels (Figure 2).

Given the clinical and imaging findings, there was concern for a possible vascular tumor. Therefore, excisional biopsy was recommended over needle biopsy because of the bleeding risk. With the patient under general anesthesia, and a tourniquet used without exsanguination, a Brunner-type zigzag incision was made centered over the mass with elevated skin flaps. The 2.7×2.6×1.1-cm mass was superficial and involved the radial artery (Figure 3). After the radial artery was dissected proximally and distally, 2 perforating vessels were found entering the mass. These vessels were ligated, which allowed the mass to be peeled completely off the artery. Histology with hematoxylin-eosin staining showed solid sheets of uniform round cells with interspersed capillaries and centrally placed nuclei without evidence of malignancy (Figure 4).

The tourniquet was released before skin closure, and adequate hemostasis was obtained. The wound was closed, and the patient was placed in a volar wrist splint for immobilization. Pain relief after excision of the mass was immediate, and the postoperative course uneventful. After surgery, immunohistochemistry of the mass showed minimal mitotic activity, with a positive immunoperoxidase stain for smooth muscle actin confirming a diagnosis of glomus tumor (Figure 5). At 3-year follow-up, the patient had no pain, symptoms, or tumor recurrence.

Discussion

Glomus tumors are an established cause of pain in the subungual areas of the hand; numerous cases have been reported.^1,5,10,14 However, extradigital glomus tumors, particularly those involving the wrist, are rare, and only a few have been described. Given the lack of consistent findings and presentations, diagnosis is difficult. Case series have documented an overall 2:1 female-to-male predominance of glomus tumors,⁶ but extradigital tumors are more common in men (4.6:1 male-to-female ratio).³ Extradigital glomus tumors are commonly diagnosed between ages 40 and 80 years. Classic symptoms of subungual tumors include pain, localized tenderness, and cold hypersensitivity,^1,10 but symptoms are much more variable with extradigital locations. Previous trauma or injury to the lesion area is reported in 20% to 30% of cases before symptom onset.^3,15 Intravascular locations of glomus tumors are extremely rare; only 4 cases of tumors involving venous structures have been reported.^16-19 In the present case, the patient’s main complaints were pain and localized tenderness associated with a progressively increasing mass without any history of trauma. The large size of his mass (~2.5 cm in diameter) on examination was unique, as was involvement of the radial artery.

Misdiagnosis and delayed diagnosis of extradigital glomus tumors are common, and symptoms such as chronic pain typically persist for 7 to 11 years before the correct diagnosis is made.^1,10 On average, 2.5 physician consultants (including psychiatrists) evaluate the patient before glomus tumor is identified.¹⁰ There are other reports of atypical or ectopic glomus tumors taking 5 to 25 years to be diagnosed.^20-22 The differential diagnosis for glomus tumors includes hemangiomas, cellular or cavernous hemangiomas, vascular tumors, neuromas, neurofibromas, lipomas, paragangliomas, ganglion cysts, pigmented nevi, Pacinian corpuscle hyperplasia, and foreign bodies. A key element of clinical diagnosis is the disproportionate amount of pain and localized tenderness caused by the lesion relative to its size. The hypersensitivity of this tumor is thought to result from enlargement of the tumor and impingement on nearby Pacinian corpuscles, nerve endings in the skin that are responsible for sensitivity to vibration and pressure.^2,9

Plain radiographs can be useful in detecting glomus tumors of the hand but are less helpful with extradigital tumors, with identification rates of 24% in certain series.³ MRI is the most sensitive imaging modality for diagnosing glomus tumors of the hand; a detection rate of 80% to 100% has been reported in various case series.^3,11,12 Specificity of MRI for glomus tumors has been reported at 50%.^11,23 Placement of a radiographic marker directly over the area of most pain can assist in tumor localization.³ Glomus tumors typically have decreased signal intensity on T1-weighted images and increased intensity on T2-weighted images, but signal patterns are variable and particularly difficult to differentiate with small tumors. MRI is useful in the setting of recurrent glomus tumors, where incomplete excision is possible. In 24 cases of continued pain after glomus tumor excision, Theumann and colleagues²⁴ used MRI to identify a nodule consistent with recurrent glomus tumor in all patients. Three-dimensional contrast-enhanced magnetic resonance angiography (MRA) can also help diagnose glomus tumors while providing valuable information regarding size and location for surgical planning.^25,26 With MRA, it is crucial to evaluate the arterial or arteriovenous phase of imaging, as the glomus tumor is richly vascularized and shows contrast enhancement after intravenous injection of gadolinium.²⁷ Angiography, ultrasonography, thermography, and scintigraphy have all been used to diagnose glomus tumors but have shown limited utility and accuracy.¹¹

Treatment of glomus tumors is complete surgical excision because of their relatively small size and subcutaneous location. Resection success rates are consistently higher than 95%, with resolution of all symptoms.^1,10,14 Local recurrence of tumors after excision occurs in 1% to 33% of cases, depending on series, and may be immediate or delayed, with immediate recurrence commonly caused by inadequate excision.^1,10,15,28 Delayed recurrence is less common and presents several years after excision, typically with a new growth near the previous excision.¹⁰ Recurrence years after surgery may also represent multiple tumors unrecognized during initial workup and can be treated with repeat excision or radiotherapy.

Robert and colleagues²⁹ recently reported the case of a glomus tumor, on the dorsal aspect of the wrist, discovered incidentally in a 71-year-old patient and treated with surgical excision. Several years earlier, Chim and colleagues³⁰ described a similar case, of a large wrist glomus tumor worked up with MRI. In a retrospective review of all extradigital glomus tumors seen over a 20-year period, Schiefer and colleagues³ reported 4 glomus tumors of the wrist out of 56 tumors total. The most common sites were forearm (11 cases) and knee (10 cases), and the majority of patients presented with pain and localized tenderness. Mean tumor size was 0.66 cm (range, 0.1-0.3 cm), with 77% of tumors less than 1 cm. Our patient’s 2.7×2.6×1.1-cm tumor was large for a glomus tumor. Its involvement with the radial artery feeding vessels likely contributed to its large and progressively increasing size. It is worth noting that, in the series by Schiefer and colleagues,³ the only patient with symptoms persisting after excision had a large (3 cm in diameter) deep tumor of the foot; the entire tumor was removed, and there was no recurrence by 10-year follow-up. Folpe and colleagues⁷ suggested that deep tumors larger than 2 cm should be at higher suspicion for malignancy. Joseph and Posner²¹ reported 3 cases of glomus tumors, on the ulnar side of the wrist, diagnosed with help of a provocative test using ethyl chloride spray.

Conclusion

Overall, glomus tumors are rare and challenging to diagnosis and should be in the differential in any symptomatic patient with a painful soft-tissue mass of the wrist. Advanced imaging studies, such as MRI, can assist in localization, diagnosis, and preoperative planning. Histology and immunohistochemistry are essential to differentiate glomus tumor from other vascular tumors, and complete excision is necessary to prevent local recurrence.

The contemporary management of differentiated thyroid cancer includes posttreatment monitoring for recurrence or metastasis.¹ This monitoring includes clinical, biochemical, and imaging evaluation. Follow-up treatment can then be tailored based on the results of this monitoring.

Our patient was a 61-year-old man with a history of papillary thyroid carcinoma, including lymph node involvement and an extension of the primary focus into skeletal muscle (pT3N1bMX, stage IVa). The patient’s status was posttotal thyroidectomy and radioiodine ablation therapy (196.2 mCi iodine-131) in April 2009. The patient underwent follow-up thyrotropin alpha stimulated whole-body radioiodine surveillance scanning in May 2010.

Images demonstrated residual thyroid tissue/carcinoma regional to the thyroid bed, corresponding to prior posttherapy images. Whole body scintiphotos also demonstrated abnormal iodine localization that raised the possibility of distant bony metastasis in the region of the right hip (see Figures 1A and 1B). Current treatment standards for isolated bony metastases recommend repeated radioactive iodine therapy and potential external beam radiation. Imaging is required for accurate verification.¹ This abnormal osseous finding was questionable on initial review, as it was present on the posterior, not anterior, view. The patient was instructed to continue hydration and return for additional delayed scintiphotos for further evaluation.

The patient returned 4 days later for delayed scintiphotos, which again demonstrated abnormal iodine localization near the right hip. However, iodine distribution was different, including now being visible on both the anterior and posterior views (see Figures 2A and 2B on the next page).

• What is your diagnosis?
• How would you treat this patient? 

[Click through to the next page to see the answer.]

Our Treatment

The patient had no pain in the area and, upon further questioning, reported that he returned wearing the same athletic shorts. Given that radioiodine is excreted in the urine, this atypical distribution was thought to reflect urinary contamination. When images were taken again with the shorts removed, no abnormal radioiodine activity was present (see Figures 2C and 2D). Additional findings with thyrotropin alfa stimulation included increased quantitative thyroglobulin values of 20.2 ng/mL with antithyroglobulin antibody < 20.0 U/mL. Radioiodine ablation therapy using thyrotropin alfa was repeated. Iodine localization also was not present in the hip on posttherapy imaging (not shown).

Despite advances in imaging techniques, radioiodine scanning remains an imperfect science. Artifacts and pitfalls have been identified; in part, these are related to the accumulation of iodide in organs other than the thyroid, such as the nasopharynx and stomach, as well as the apparent accumulation due to excretion in the gut and bladder.^2-4 These variations can be divided into ectopic normal thyroid tissue, physiologic accumulation in nonthyroidal tissue, and contamination by physiologic secretions. Recent case reports have confirmed this classification. Abnormal radioiodine uptake has been described in vertebral hemangioma,⁵ liver abscess⁶ and hydatid cyst,⁷ bronchiectasis,⁸ bronchogenic cyst and mucinous cystadenoma (2 fluid-filled cavities),⁹ chronic submandibular sialadenitis,¹⁰ esophageal diverticulum,¹¹ hiatal hernia,¹² appendix,¹³ indwelling Hickman catheter,¹⁴ renal cyst,¹⁵ and, similar to this case, contamination of the hair.¹⁶

Contaminated clothing is not uncommon; however, a persistent abnormality from contaminated clothing on repeat follow-up is unusual and could easily be misinterpreted.² It would be valuable for all providers to be aware of the pitfalls of imaging before embarking on an unnecessary and potentially hazardous—not to mention costly—treatment course.

Acknowledgments
The authors acknowledge the assistance of Richard Cacciato, MLIS, Medical Librarian, who assisted in the literature review.

Author disclosures
The authors report no actual or potential conflicts of interest with regard to this article.

Disclaimer
The opinions expressed herein are those of the authors and do not necessarily reflect those of Federal Practitioner, Frontline Medical Communications Inc., the U.S. Government, or any of its agencies. This article may discuss unlabeled or investigational use of certain drugs. Please review the complete prescribing information for specific drugs or drug combinations—including indications, contraindications, warnings, and adverse effects—before administering pharmacologic therapy to patients.

The contemporary management of differentiated thyroid cancer includes posttreatment monitoring for recurrence or metastasis.¹ This monitoring includes clinical, biochemical, and imaging evaluation. Follow-up treatment can then be tailored based on the results of this monitoring.

Our patient was a 61-year-old man with a history of papillary thyroid carcinoma, including lymph node involvement and an extension of the primary focus into skeletal muscle (pT3N1bMX, stage IVa). The patient’s status was posttotal thyroidectomy and radioiodine ablation therapy (196.2 mCi iodine-131) in April 2009. The patient underwent follow-up thyrotropin alpha stimulated whole-body radioiodine surveillance scanning in May 2010.

Images demonstrated residual thyroid tissue/carcinoma regional to the thyroid bed, corresponding to prior posttherapy images. Whole body scintiphotos also demonstrated abnormal iodine localization that raised the possibility of distant bony metastasis in the region of the right hip (see Figures 1A and 1B). Current treatment standards for isolated bony metastases recommend repeated radioactive iodine therapy and potential external beam radiation. Imaging is required for accurate verification.¹ This abnormal osseous finding was questionable on initial review, as it was present on the posterior, not anterior, view. The patient was instructed to continue hydration and return for additional delayed scintiphotos for further evaluation.

The patient returned 4 days later for delayed scintiphotos, which again demonstrated abnormal iodine localization near the right hip. However, iodine distribution was different, including now being visible on both the anterior and posterior views (see Figures 2A and 2B on the next page).

• What is your diagnosis?
• How would you treat this patient? 

[Click through to the next page to see the answer.]

Our Treatment

The patient had no pain in the area and, upon further questioning, reported that he returned wearing the same athletic shorts. Given that radioiodine is excreted in the urine, this atypical distribution was thought to reflect urinary contamination. When images were taken again with the shorts removed, no abnormal radioiodine activity was present (see Figures 2C and 2D). Additional findings with thyrotropin alfa stimulation included increased quantitative thyroglobulin values of 20.2 ng/mL with antithyroglobulin antibody < 20.0 U/mL. Radioiodine ablation therapy using thyrotropin alfa was repeated. Iodine localization also was not present in the hip on posttherapy imaging (not shown).

Despite advances in imaging techniques, radioiodine scanning remains an imperfect science. Artifacts and pitfalls have been identified; in part, these are related to the accumulation of iodide in organs other than the thyroid, such as the nasopharynx and stomach, as well as the apparent accumulation due to excretion in the gut and bladder.^2-4 These variations can be divided into ectopic normal thyroid tissue, physiologic accumulation in nonthyroidal tissue, and contamination by physiologic secretions. Recent case reports have confirmed this classification. Abnormal radioiodine uptake has been described in vertebral hemangioma,⁵ liver abscess⁶ and hydatid cyst,⁷ bronchiectasis,⁸ bronchogenic cyst and mucinous cystadenoma (2 fluid-filled cavities),⁹ chronic submandibular sialadenitis,¹⁰ esophageal diverticulum,¹¹ hiatal hernia,¹² appendix,¹³ indwelling Hickman catheter,¹⁴ renal cyst,¹⁵ and, similar to this case, contamination of the hair.¹⁶

Contaminated clothing is not uncommon; however, a persistent abnormality from contaminated clothing on repeat follow-up is unusual and could easily be misinterpreted.² It would be valuable for all providers to be aware of the pitfalls of imaging before embarking on an unnecessary and potentially hazardous—not to mention costly—treatment course.

Acknowledgments
The authors acknowledge the assistance of Richard Cacciato, MLIS, Medical Librarian, who assisted in the literature review.

Author disclosures
The authors report no actual or potential conflicts of interest with regard to this article.

Disclaimer
The opinions expressed herein are those of the authors and do not necessarily reflect those of Federal Practitioner, Frontline Medical Communications Inc., the U.S. Government, or any of its agencies. This article may discuss unlabeled or investigational use of certain drugs. Please review the complete prescribing information for specific drugs or drug combinations—including indications, contraindications, warnings, and adverse effects—before administering pharmacologic therapy to patients.

The contemporary management of differentiated thyroid cancer includes posttreatment monitoring for recurrence or metastasis.¹ This monitoring includes clinical, biochemical, and imaging evaluation. Follow-up treatment can then be tailored based on the results of this monitoring.

Our patient was a 61-year-old man with a history of papillary thyroid carcinoma, including lymph node involvement and an extension of the primary focus into skeletal muscle (pT3N1bMX, stage IVa). The patient’s status was posttotal thyroidectomy and radioiodine ablation therapy (196.2 mCi iodine-131) in April 2009. The patient underwent follow-up thyrotropin alpha stimulated whole-body radioiodine surveillance scanning in May 2010.

Images demonstrated residual thyroid tissue/carcinoma regional to the thyroid bed, corresponding to prior posttherapy images. Whole body scintiphotos also demonstrated abnormal iodine localization that raised the possibility of distant bony metastasis in the region of the right hip (see Figures 1A and 1B). Current treatment standards for isolated bony metastases recommend repeated radioactive iodine therapy and potential external beam radiation. Imaging is required for accurate verification.¹ This abnormal osseous finding was questionable on initial review, as it was present on the posterior, not anterior, view. The patient was instructed to continue hydration and return for additional delayed scintiphotos for further evaluation.

The patient returned 4 days later for delayed scintiphotos, which again demonstrated abnormal iodine localization near the right hip. However, iodine distribution was different, including now being visible on both the anterior and posterior views (see Figures 2A and 2B on the next page).

• What is your diagnosis?
• How would you treat this patient? 

[Click through to the next page to see the answer.]

Our Treatment

The patient had no pain in the area and, upon further questioning, reported that he returned wearing the same athletic shorts. Given that radioiodine is excreted in the urine, this atypical distribution was thought to reflect urinary contamination. When images were taken again with the shorts removed, no abnormal radioiodine activity was present (see Figures 2C and 2D). Additional findings with thyrotropin alfa stimulation included increased quantitative thyroglobulin values of 20.2 ng/mL with antithyroglobulin antibody < 20.0 U/mL. Radioiodine ablation therapy using thyrotropin alfa was repeated. Iodine localization also was not present in the hip on posttherapy imaging (not shown).

Despite advances in imaging techniques, radioiodine scanning remains an imperfect science. Artifacts and pitfalls have been identified; in part, these are related to the accumulation of iodide in organs other than the thyroid, such as the nasopharynx and stomach, as well as the apparent accumulation due to excretion in the gut and bladder.^2-4 These variations can be divided into ectopic normal thyroid tissue, physiologic accumulation in nonthyroidal tissue, and contamination by physiologic secretions. Recent case reports have confirmed this classification. Abnormal radioiodine uptake has been described in vertebral hemangioma,⁵ liver abscess⁶ and hydatid cyst,⁷ bronchiectasis,⁸ bronchogenic cyst and mucinous cystadenoma (2 fluid-filled cavities),⁹ chronic submandibular sialadenitis,¹⁰ esophageal diverticulum,¹¹ hiatal hernia,¹² appendix,¹³ indwelling Hickman catheter,¹⁴ renal cyst,¹⁵ and, similar to this case, contamination of the hair.¹⁶

Contaminated clothing is not uncommon; however, a persistent abnormality from contaminated clothing on repeat follow-up is unusual and could easily be misinterpreted.² It would be valuable for all providers to be aware of the pitfalls of imaging before embarking on an unnecessary and potentially hazardous—not to mention costly—treatment course.

Acknowledgments
The authors acknowledge the assistance of Richard Cacciato, MLIS, Medical Librarian, who assisted in the literature review.

Author disclosures
The authors report no actual or potential conflicts of interest with regard to this article.

Disclaimer
The opinions expressed herein are those of the authors and do not necessarily reflect those of Federal Practitioner, Frontline Medical Communications Inc., the U.S. Government, or any of its agencies. This article may discuss unlabeled or investigational use of certain drugs. Please review the complete prescribing information for specific drugs or drug combinations—including indications, contraindications, warnings, and adverse effects—before administering pharmacologic therapy to patients.

THE CASES

CASE 1 › A 32-year-old G2P1 with an uncomplicated prenatal course presented for induction at 41 weeks and 2 days of gestation. Fetal heart tracing showed no abnormalities. A compound presentation and a prolonged second stage of labor made vacuum assistance necessary. The infant had both a true umbilical cord knot (TUCK) (FIGURE 1A) and double nuchal cord.

CASE 2 › A 46-year-old G3P0 at 38 weeks of gestation by in vitro fertilization underwent an uncomplicated primary low transverse cesarean (C-section) delivery of dichorionic/diamniotic twins. The C-section had been necessary because baby A had been in the breech position. Fetal heart tracing showed no abnormalities. Baby A had a velamentous cord insertion, and baby B had a succenturiate lobe and a TUCK.

CASE 3 › A 23-year-old G2P1 with an uncomplicated prenatal course chose to have a repeat C-section and delivered at 41 weeks in active labor. Fetal heart monitoring showed no abnormalities. Umbilical artery pH and venous pH were normal. A TUCK was noted at time of delivery.

CASE 4 › A 30-year-old G1P0 with an uncomplicated prenatal course presented in active labor at 40 weeks and 4 days of gestation. At 7 cm cervical dilation, monitoring showed repeated deep variable fetal heart rate decelerations. The patient underwent an uncomplicated primary C-section. Umbilical artery pH and venous pH were normal. A TUCK (FIGURE 1B) and double nuchal cord were found at time of delivery.

DISCUSSION

TUCKs are thought to occur when a fetus passes through a loop in the umbilical cord. They occur in <2% of term deliveries.^1,2 TUCKs differ from false knots. False knots are exaggerated loops of cord vasculature.

Risk factors that have been independently associated with TUCK include advanced maternal age (AMA; >35 years), multiparity, diabetes mellitus, gestational diabetes, polyhydramnios, and previous spontaneous abortion.^1-3 In one study, 72% of women with a TUCK were multiparous.³ Hershkovitz et al² suggested that laxity of uterine and abdominal musculature in multiparous patients may contribute to increased room for TUCK formation.

The adjusted odds ratio of having a TUCK is 2.53 in women with diabetes mellitus.³ Hyperglycemia can contribute to increased fetal movements, thereby increasing the risk of TUCK development.² Polyhydramnios is often found in patients with diabetes mellitus and gestational diabetes.³ The incidence is higher in monoamniotic twins.⁴

Being a male and having a longer umbilical cord may also increase the risk of TUCK. On average, male infants have longer cords than females, which may predispose them to TUCKs.³ Räisänen et al³ found that the mean cord length in TUCK infants was 16.9 cm longer than in infants without a TUCK.

Of our 4 patients, one was of AMA, 2 were multiparous, and 3 of the 4 infants who developed TUCK were male.

TUCK is usually
 diagnosed at delivery


Most cases of TUCK are found incidentally at the time of delivery. Antenatal diagnosis is difficult, because loops of cord lying together are easily mistaken for knots on ultrasound.⁵ Sepulveda et al⁶ evaluated the use of 3D power Doppler in 8 cases of suspected TUCK; only 63% were confirmed at delivery. Some researchers have found improved detection of TUCK with color Doppler and 4D ultrasound, which have demonstrated a “hanging noose sign” (a transverse section of umbilical cord surrounded by a loop of cord) as well as views of the cord under pressure.^7-10

Outcomes associated with TUCK vary greatly. Neonates affected by TUCK have a 4% to 10% increased risk of stillbirth, usually attributed to knot tightening.^2,4,11,12

In addition, there is an increased incidence of fetal heart rate abnormalities during labor.^1,3,12,13

Infants with true umbilical cord knots have an increased incidence of heart rate abnormalities during labor. There is no increase in the incidence of assisted vaginal or C-section delivery.¹² And as for whether TUCK affects an infant’s size or weight, one study found TUCK infants had a 3.2-fold higher risk of measuring small for gestational age, potentially due to chronic umbilical cord compromise; however, mean birth weight between study and control groups did not differ significantly.³

Outcomes for our patients and their infants. All 4 cases had good outcomes (TABLE). The umbilical cord knot produced no detectable fetal compromise in cases 1 through 3. In Case 4, electronic fetal monitoring showed repeated variable fetal heart rate decelerations, presumably associated with cord compression.

THE TAKEAWAY

Pregnant women who may be at risk for experiencing a TUCK include those who are older than age 35, multiparous, carrying a boy, or have diabetes mellitus, gestational diabetes, or polyhydramnios. While it is good to be aware of these risk factors, there are no recommended changes in management based on risk or ultrasound findings unless there is additional concern for fetal compromise.

Antenatal diagnosis of TUCK is challenging, but Doppler ultrasound may be able to identify the condition. Most cases of TUCK are noted on delivery, and outcomes are generally positive, although infants in whom the TUCK tightens may have an increased risk of heart rate abnormalities or stillbirth. 

THE CASES

CASE 1 › A 32-year-old G2P1 with an uncomplicated prenatal course presented for induction at 41 weeks and 2 days of gestation. Fetal heart tracing showed no abnormalities. A compound presentation and a prolonged second stage of labor made vacuum assistance necessary. The infant had both a true umbilical cord knot (TUCK) (FIGURE 1A) and double nuchal cord.

CASE 2 › A 46-year-old G3P0 at 38 weeks of gestation by in vitro fertilization underwent an uncomplicated primary low transverse cesarean (C-section) delivery of dichorionic/diamniotic twins. The C-section had been necessary because baby A had been in the breech position. Fetal heart tracing showed no abnormalities. Baby A had a velamentous cord insertion, and baby B had a succenturiate lobe and a TUCK.

CASE 3 › A 23-year-old G2P1 with an uncomplicated prenatal course chose to have a repeat C-section and delivered at 41 weeks in active labor. Fetal heart monitoring showed no abnormalities. Umbilical artery pH and venous pH were normal. A TUCK was noted at time of delivery.

CASE 4 › A 30-year-old G1P0 with an uncomplicated prenatal course presented in active labor at 40 weeks and 4 days of gestation. At 7 cm cervical dilation, monitoring showed repeated deep variable fetal heart rate decelerations. The patient underwent an uncomplicated primary C-section. Umbilical artery pH and venous pH were normal. A TUCK (FIGURE 1B) and double nuchal cord were found at time of delivery.

DISCUSSION

TUCKs are thought to occur when a fetus passes through a loop in the umbilical cord. They occur in <2% of term deliveries.^1,2 TUCKs differ from false knots. False knots are exaggerated loops of cord vasculature.

Risk factors that have been independently associated with TUCK include advanced maternal age (AMA; >35 years), multiparity, diabetes mellitus, gestational diabetes, polyhydramnios, and previous spontaneous abortion.^1-3 In one study, 72% of women with a TUCK were multiparous.³ Hershkovitz et al² suggested that laxity of uterine and abdominal musculature in multiparous patients may contribute to increased room for TUCK formation.

The adjusted odds ratio of having a TUCK is 2.53 in women with diabetes mellitus.³ Hyperglycemia can contribute to increased fetal movements, thereby increasing the risk of TUCK development.² Polyhydramnios is often found in patients with diabetes mellitus and gestational diabetes.³ The incidence is higher in monoamniotic twins.⁴

Being a male and having a longer umbilical cord may also increase the risk of TUCK. On average, male infants have longer cords than females, which may predispose them to TUCKs.³ Räisänen et al³ found that the mean cord length in TUCK infants was 16.9 cm longer than in infants without a TUCK.

Of our 4 patients, one was of AMA, 2 were multiparous, and 3 of the 4 infants who developed TUCK were male.

TUCK is usually
 diagnosed at delivery


Most cases of TUCK are found incidentally at the time of delivery. Antenatal diagnosis is difficult, because loops of cord lying together are easily mistaken for knots on ultrasound.⁵ Sepulveda et al⁶ evaluated the use of 3D power Doppler in 8 cases of suspected TUCK; only 63% were confirmed at delivery. Some researchers have found improved detection of TUCK with color Doppler and 4D ultrasound, which have demonstrated a “hanging noose sign” (a transverse section of umbilical cord surrounded by a loop of cord) as well as views of the cord under pressure.^7-10

Outcomes associated with TUCK vary greatly. Neonates affected by TUCK have a 4% to 10% increased risk of stillbirth, usually attributed to knot tightening.^2,4,11,12

In addition, there is an increased incidence of fetal heart rate abnormalities during labor.^1,3,12,13

Infants with true umbilical cord knots have an increased incidence of heart rate abnormalities during labor. There is no increase in the incidence of assisted vaginal or C-section delivery.¹² And as for whether TUCK affects an infant’s size or weight, one study found TUCK infants had a 3.2-fold higher risk of measuring small for gestational age, potentially due to chronic umbilical cord compromise; however, mean birth weight between study and control groups did not differ significantly.³

Outcomes for our patients and their infants. All 4 cases had good outcomes (TABLE). The umbilical cord knot produced no detectable fetal compromise in cases 1 through 3. In Case 4, electronic fetal monitoring showed repeated variable fetal heart rate decelerations, presumably associated with cord compression.

THE TAKEAWAY

Pregnant women who may be at risk for experiencing a TUCK include those who are older than age 35, multiparous, carrying a boy, or have diabetes mellitus, gestational diabetes, or polyhydramnios. While it is good to be aware of these risk factors, there are no recommended changes in management based on risk or ultrasound findings unless there is additional concern for fetal compromise.

Antenatal diagnosis of TUCK is challenging, but Doppler ultrasound may be able to identify the condition. Most cases of TUCK are noted on delivery, and outcomes are generally positive, although infants in whom the TUCK tightens may have an increased risk of heart rate abnormalities or stillbirth. 

THE CASES

CASE 1 › A 32-year-old G2P1 with an uncomplicated prenatal course presented for induction at 41 weeks and 2 days of gestation. Fetal heart tracing showed no abnormalities. A compound presentation and a prolonged second stage of labor made vacuum assistance necessary. The infant had both a true umbilical cord knot (TUCK) (FIGURE 1A) and double nuchal cord.

CASE 2 › A 46-year-old G3P0 at 38 weeks of gestation by in vitro fertilization underwent an uncomplicated primary low transverse cesarean (C-section) delivery of dichorionic/diamniotic twins. The C-section had been necessary because baby A had been in the breech position. Fetal heart tracing showed no abnormalities. Baby A had a velamentous cord insertion, and baby B had a succenturiate lobe and a TUCK.

CASE 3 › A 23-year-old G2P1 with an uncomplicated prenatal course chose to have a repeat C-section and delivered at 41 weeks in active labor. Fetal heart monitoring showed no abnormalities. Umbilical artery pH and venous pH were normal. A TUCK was noted at time of delivery.

CASE 4 › A 30-year-old G1P0 with an uncomplicated prenatal course presented in active labor at 40 weeks and 4 days of gestation. At 7 cm cervical dilation, monitoring showed repeated deep variable fetal heart rate decelerations. The patient underwent an uncomplicated primary C-section. Umbilical artery pH and venous pH were normal. A TUCK (FIGURE 1B) and double nuchal cord were found at time of delivery.

DISCUSSION

TUCKs are thought to occur when a fetus passes through a loop in the umbilical cord. They occur in <2% of term deliveries.^1,2 TUCKs differ from false knots. False knots are exaggerated loops of cord vasculature.

Risk factors that have been independently associated with TUCK include advanced maternal age (AMA; >35 years), multiparity, diabetes mellitus, gestational diabetes, polyhydramnios, and previous spontaneous abortion.^1-3 In one study, 72% of women with a TUCK were multiparous.³ Hershkovitz et al² suggested that laxity of uterine and abdominal musculature in multiparous patients may contribute to increased room for TUCK formation.

The adjusted odds ratio of having a TUCK is 2.53 in women with diabetes mellitus.³ Hyperglycemia can contribute to increased fetal movements, thereby increasing the risk of TUCK development.² Polyhydramnios is often found in patients with diabetes mellitus and gestational diabetes.³ The incidence is higher in monoamniotic twins.⁴

Being a male and having a longer umbilical cord may also increase the risk of TUCK. On average, male infants have longer cords than females, which may predispose them to TUCKs.³ Räisänen et al³ found that the mean cord length in TUCK infants was 16.9 cm longer than in infants without a TUCK.

Of our 4 patients, one was of AMA, 2 were multiparous, and 3 of the 4 infants who developed TUCK were male.

TUCK is usually
 diagnosed at delivery


Most cases of TUCK are found incidentally at the time of delivery. Antenatal diagnosis is difficult, because loops of cord lying together are easily mistaken for knots on ultrasound.⁵ Sepulveda et al⁶ evaluated the use of 3D power Doppler in 8 cases of suspected TUCK; only 63% were confirmed at delivery. Some researchers have found improved detection of TUCK with color Doppler and 4D ultrasound, which have demonstrated a “hanging noose sign” (a transverse section of umbilical cord surrounded by a loop of cord) as well as views of the cord under pressure.^7-10

Outcomes associated with TUCK vary greatly. Neonates affected by TUCK have a 4% to 10% increased risk of stillbirth, usually attributed to knot tightening.^2,4,11,12

In addition, there is an increased incidence of fetal heart rate abnormalities during labor.^1,3,12,13

Infants with true umbilical cord knots have an increased incidence of heart rate abnormalities during labor. There is no increase in the incidence of assisted vaginal or C-section delivery.¹² And as for whether TUCK affects an infant’s size or weight, one study found TUCK infants had a 3.2-fold higher risk of measuring small for gestational age, potentially due to chronic umbilical cord compromise; however, mean birth weight between study and control groups did not differ significantly.³

Outcomes for our patients and their infants. All 4 cases had good outcomes (TABLE). The umbilical cord knot produced no detectable fetal compromise in cases 1 through 3. In Case 4, electronic fetal monitoring showed repeated variable fetal heart rate decelerations, presumably associated with cord compression.

THE TAKEAWAY

Pregnant women who may be at risk for experiencing a TUCK include those who are older than age 35, multiparous, carrying a boy, or have diabetes mellitus, gestational diabetes, or polyhydramnios. While it is good to be aware of these risk factors, there are no recommended changes in management based on risk or ultrasound findings unless there is additional concern for fetal compromise.

Antenatal diagnosis of TUCK is challenging, but Doppler ultrasound may be able to identify the condition. Most cases of TUCK are noted on delivery, and outcomes are generally positive, although infants in whom the TUCK tightens may have an increased risk of heart rate abnormalities or stillbirth. 

THE CASE

A 23-year-old immunocompetent woman was referred to our spinal clinic with a 6-month history of low back pain that radiated to her right flank, buttock, and groin. She’d had intermittent urinary problems, including mild dysuria and frequency, and had been treated with antibiotics for a presumed urinary tract infection on 3 previous occasions, but her pain gradually increased and eventually became constant.

The patient had no history of fever, malaise, or weight loss. She denied consuming unpasteurized milk or undercooked poultry, and hadn’t recently experienced diarrhea or vomiting.

Eight years earlier, she had undergone anterior fusion of her spine for idiopathic scoliosis. At that time, she was at Risser grade 1, and her Cobb angle was 50°; metallic instrumentation was implanted at T10 to L3 to prevent progression of the scoliosis. Her recovery had been uneventful.

During examination, her temperature, pulse, respiratory rate, blood pressure, and nervous system were all normal. Her hips appeared normal, as well, and a straight leg raise was negative bilaterally. The patient had mild midline lumbar tenderness. Spinal range of movement revealed decreased flexion and mild pain.

X-rays (FIGURE 1) showed no changes in the previous metalwork in her spine. There was decreased disk height at the L3/4 level, but no significant bony erosion or soft-tissue shadows. Laboratory testing revealed a C-reactive protein (CRP) level of 240 mg/dL (normal, <1 mg/dL) and her erythrocyte sedimentation rate (ESR) was 102 mm/h—more than 5 times higher than it should have been.¹ The patient had a normal peripheral white cell count (WCC). Midstream urine cultures were negative.


The patient was admitted to the hospital for further work-up. Magnetic resonance imaging (MRI) of the lumbar spine showed gross abnormality at the L3-L4 disk level with erosion of the end plates, fluid in the disk space, marked enhancing edema, and mild surrounding soft-tissue edematous changes, but no evidence of any epidural abscess (FIGURE 2). The patient had a fluoroscopy-guided needle biopsy of the disk on the same day and received intravenous (IV) ceftriaxone 2 g twice a day. Blood and urine cultures were negative.

THE DIAGNOSIS

We suspected our patient had spondylodiscitis, an infection of the spine that includes spondylitis (inflammation of the vertebrae) and discitis (inflammation of the vertebral disk space). After 48 hours, the biopsy sample grew Salmonella typhimurium and confirmed the diagnosis. The organism was sensitive to ceftriaxone and ciprofloxacin; parenteral ceftriaxone was continued and the patient wore a thoracolumbar brace for immobilization. For 3 days, her inflammatory marker levels were checked daily, then every other day for the rest of that first week, and then 2 more times in the following week.

DISCUSSION

Thoracic and lumbar vertebrae are the most common sites of spondylodiscitis.² Spondylodiscitis accounts for 3% to 5% of pyogenic osteomyelitis in patients in developed countries.³ The incidence of pyogenic spondylodiscitis may be rising due to an increase in the number of elderly and immunocompromised patients, as well as a rise in invasive medical procedures.^4-6

If left untreated, spondylodiscitis can spread longitudinally (involving the adjacent levels), posteriorly (causing bacterial meningitis, abscess formation, and cord compromise), or anteriorly (causing paravertebral abscess). Untreated spondylodiscitis can also send distant infective emboli and cause endocarditis^7-9 or mycotic abdominal aneurysm.¹⁰

Historically, mortality in patients with vertebral osteomyelitis has been as high as 25%.¹¹ The combination of earlier diagnosis, antibiotics, and surgical debridement and stabilization has decreased mortality to less than 15%.^12-14

Risk factors for spondylodiscitis include male sex, IV drug abuse, diabetes, morbid obesity, having had a genitourinary or spinal procedure, and being immunocompromised (eg, from alcohol abuse, malignancy, organ transplantation, chemotherapy, or corticosteroid use).^12,15,16

Gram-positive organisms cause most spine infections in both adults and children, with 40% to 90% caused by Staphylococcus aureus.¹⁷ Gram-negative organisms (Escherichia coli, Pseudomonas, and Proteus), which can also cause spondylodiscitis, typically occur after genitourinary infections or procedures. IV drug abusers are prone to Pseudomonas infections.¹⁸ Anaerobic infections may be seen in patients with diabetes or after penetrating trauma.¹⁵ Salmonella species can cause spondylodiscitis, especially in patients with sickle cell disease from an intestinal source.¹⁹

Mycobacterium tuberculosis is the most common nonpyogenic infecting agent that also can cause spondylodiscitis. Infection caused by tuberculosis (TB) has had a recent resurgence with resistant strains, especially in patients with human immunodeficiency virus.¹⁵ Although less than 10% of patients with TB have skeletal involvement, 50% of the skeletal involvement occurs in the spine.¹⁵

The clinical presentation of spondylodiscitis depends on the location of the infection, the virulence of the organism, and the immune status of the patient. Discitis can present as pain in the back, hip, abdomen (especially in children²⁰) and, occasionally, with meningeal involvement.¹¹ Patients with discitis often have a normal temperature.^15,21 In patients with discitis, the patient’s WCC will be normal, but the ESR is almost always elevated.^15,22 Suspect spondylodiscitis in patients who present with persistent or increasing pain 3 to 4 weeks after back surgery. For such patients, measure inflammatory markers and order imaging of the spine.

Risk factors for spondylodiscitis include IV drug abuse, diabetes, morbid obesity, and having had a genitourinary or spinal procedure. X-ray findings for patients with spondylodiscitis will include osteolysis and end plate erosions (early) and narrowing and collapse of the disk space (late). (In TB, relative preservation of the disk spaces is seen, with significant vertebral destruction.)

MRI is the modality of choice for diagnosis and assessment of suspected spondylodiscitis because it can provide imaging of the soft tissue, neural elements, and bony changes with a high sensitivity and specificity.²³ Once infection is suspected, the diagnosis should be confirmed by fluoroscopic- or computed tomography-guided biopsy before starting antibiotic treatment.

Long-term antibiotics
 are required to prevent recurrence


IV antibiotics are the mainstay of treatment for spondylodiscitis;²⁴ the specific drug used will depend upon the organism identified. Patients typically receive 2 to 6 weeks of IV therapy. Then, once the patient improves and inflammatory markers return to normal levels, the patient receives a course of oral antibiotics for 2 to 6 more weeks. Grados et al¹⁹ found recurrence rates of 10% to 15% for patients who were treated 4 to 8 weeks compared to 3.9% in those treated for 12 weeks or longer; therefore, a total duration of 12 weeks is commonly chosen.^25-28

To minimize the risk of spondylolisthesis, kyphosis, and fractures of the infected bone, patients are advised to rest and the spine is often immobilized with a spinal brace. Surgery may be needed if antibiotics are not effective, or for patients who develop complications such as fluid collection, neurologic deficits, or deformity.

Our patient’s pain improved after 2 weeks and she became more comfortable wearing the thoracolumbar brace. Her CRP and ESR also improved and there was no radiologic evidence of fluid collection. The patient was discharged with a peripherally inserted central catheter in place and received IV ceftriaxone for 6 more weeks at home. This was followed by 4 weeks of oral ciprofloxacin 750 mg twice daily, thereby completing a 12-week course of antibiotics.

Our patient’s response to treatment was monitored clinically and the inflammatory markers were checked weekly after discharge until the end of treatment and at 6 and 12 months after start of treatment. At 12 months, our patient’s CRP was <1 mg/dL and ESR was 22 mm/h. One year later, our patient remained asymptomatic with normal inflammatory marker levels and no evidence of recurrence.

THE TAKEAWAY

Spondylodiscitis is an important differential diagnosis of lower back, flank, groin, and buttock pain. It’s important to be aware of this diagnosis, especially in patients who have risk factors such as IV drug abuse, diabetes, and morbid obesity. Although previous spinal surgery is a risk factor, spondylodiscitis should be considered in patients with persistent back pain even if they haven’t had spinal surgery. It can be present even when there is no tenderness over the spinous process or any fever.

Checking inflammatory markers is a reasonable next step if a patient’s pain does not resolve after at least 4 weeks. If levels of inflammatory markers such as CRP and ESR are elevated and symptoms continue, MRI can confirm or rule out the presence of spondylodiscitis. Treatments include orthotic support, antibiotics, and surgical intervention when complications arise.

THE CASE

A 23-year-old immunocompetent woman was referred to our spinal clinic with a 6-month history of low back pain that radiated to her right flank, buttock, and groin. She’d had intermittent urinary problems, including mild dysuria and frequency, and had been treated with antibiotics for a presumed urinary tract infection on 3 previous occasions, but her pain gradually increased and eventually became constant.

The patient had no history of fever, malaise, or weight loss. She denied consuming unpasteurized milk or undercooked poultry, and hadn’t recently experienced diarrhea or vomiting.

Eight years earlier, she had undergone anterior fusion of her spine for idiopathic scoliosis. At that time, she was at Risser grade 1, and her Cobb angle was 50°; metallic instrumentation was implanted at T10 to L3 to prevent progression of the scoliosis. Her recovery had been uneventful.

During examination, her temperature, pulse, respiratory rate, blood pressure, and nervous system were all normal. Her hips appeared normal, as well, and a straight leg raise was negative bilaterally. The patient had mild midline lumbar tenderness. Spinal range of movement revealed decreased flexion and mild pain.

X-rays (FIGURE 1) showed no changes in the previous metalwork in her spine. There was decreased disk height at the L3/4 level, but no significant bony erosion or soft-tissue shadows. Laboratory testing revealed a C-reactive protein (CRP) level of 240 mg/dL (normal, <1 mg/dL) and her erythrocyte sedimentation rate (ESR) was 102 mm/h—more than 5 times higher than it should have been.¹ The patient had a normal peripheral white cell count (WCC). Midstream urine cultures were negative.


The patient was admitted to the hospital for further work-up. Magnetic resonance imaging (MRI) of the lumbar spine showed gross abnormality at the L3-L4 disk level with erosion of the end plates, fluid in the disk space, marked enhancing edema, and mild surrounding soft-tissue edematous changes, but no evidence of any epidural abscess (FIGURE 2). The patient had a fluoroscopy-guided needle biopsy of the disk on the same day and received intravenous (IV) ceftriaxone 2 g twice a day. Blood and urine cultures were negative.

THE DIAGNOSIS

We suspected our patient had spondylodiscitis, an infection of the spine that includes spondylitis (inflammation of the vertebrae) and discitis (inflammation of the vertebral disk space). After 48 hours, the biopsy sample grew Salmonella typhimurium and confirmed the diagnosis. The organism was sensitive to ceftriaxone and ciprofloxacin; parenteral ceftriaxone was continued and the patient wore a thoracolumbar brace for immobilization. For 3 days, her inflammatory marker levels were checked daily, then every other day for the rest of that first week, and then 2 more times in the following week.

DISCUSSION

Thoracic and lumbar vertebrae are the most common sites of spondylodiscitis.² Spondylodiscitis accounts for 3% to 5% of pyogenic osteomyelitis in patients in developed countries.³ The incidence of pyogenic spondylodiscitis may be rising due to an increase in the number of elderly and immunocompromised patients, as well as a rise in invasive medical procedures.^4-6

If left untreated, spondylodiscitis can spread longitudinally (involving the adjacent levels), posteriorly (causing bacterial meningitis, abscess formation, and cord compromise), or anteriorly (causing paravertebral abscess). Untreated spondylodiscitis can also send distant infective emboli and cause endocarditis^7-9 or mycotic abdominal aneurysm.¹⁰

Historically, mortality in patients with vertebral osteomyelitis has been as high as 25%.¹¹ The combination of earlier diagnosis, antibiotics, and surgical debridement and stabilization has decreased mortality to less than 15%.^12-14

Risk factors for spondylodiscitis include male sex, IV drug abuse, diabetes, morbid obesity, having had a genitourinary or spinal procedure, and being immunocompromised (eg, from alcohol abuse, malignancy, organ transplantation, chemotherapy, or corticosteroid use).^12,15,16

Gram-positive organisms cause most spine infections in both adults and children, with 40% to 90% caused by Staphylococcus aureus.¹⁷ Gram-negative organisms (Escherichia coli, Pseudomonas, and Proteus), which can also cause spondylodiscitis, typically occur after genitourinary infections or procedures. IV drug abusers are prone to Pseudomonas infections.¹⁸ Anaerobic infections may be seen in patients with diabetes or after penetrating trauma.¹⁵ Salmonella species can cause spondylodiscitis, especially in patients with sickle cell disease from an intestinal source.¹⁹

Mycobacterium tuberculosis is the most common nonpyogenic infecting agent that also can cause spondylodiscitis. Infection caused by tuberculosis (TB) has had a recent resurgence with resistant strains, especially in patients with human immunodeficiency virus.¹⁵ Although less than 10% of patients with TB have skeletal involvement, 50% of the skeletal involvement occurs in the spine.¹⁵

The clinical presentation of spondylodiscitis depends on the location of the infection, the virulence of the organism, and the immune status of the patient. Discitis can present as pain in the back, hip, abdomen (especially in children²⁰) and, occasionally, with meningeal involvement.¹¹ Patients with discitis often have a normal temperature.^15,21 In patients with discitis, the patient’s WCC will be normal, but the ESR is almost always elevated.^15,22 Suspect spondylodiscitis in patients who present with persistent or increasing pain 3 to 4 weeks after back surgery. For such patients, measure inflammatory markers and order imaging of the spine.

Risk factors for spondylodiscitis include IV drug abuse, diabetes, morbid obesity, and having had a genitourinary or spinal procedure. X-ray findings for patients with spondylodiscitis will include osteolysis and end plate erosions (early) and narrowing and collapse of the disk space (late). (In TB, relative preservation of the disk spaces is seen, with significant vertebral destruction.)

MRI is the modality of choice for diagnosis and assessment of suspected spondylodiscitis because it can provide imaging of the soft tissue, neural elements, and bony changes with a high sensitivity and specificity.²³ Once infection is suspected, the diagnosis should be confirmed by fluoroscopic- or computed tomography-guided biopsy before starting antibiotic treatment.

Long-term antibiotics
 are required to prevent recurrence


IV antibiotics are the mainstay of treatment for spondylodiscitis;²⁴ the specific drug used will depend upon the organism identified. Patients typically receive 2 to 6 weeks of IV therapy. Then, once the patient improves and inflammatory markers return to normal levels, the patient receives a course of oral antibiotics for 2 to 6 more weeks. Grados et al¹⁹ found recurrence rates of 10% to 15% for patients who were treated 4 to 8 weeks compared to 3.9% in those treated for 12 weeks or longer; therefore, a total duration of 12 weeks is commonly chosen.^25-28

To minimize the risk of spondylolisthesis, kyphosis, and fractures of the infected bone, patients are advised to rest and the spine is often immobilized with a spinal brace. Surgery may be needed if antibiotics are not effective, or for patients who develop complications such as fluid collection, neurologic deficits, or deformity.

Our patient’s pain improved after 2 weeks and she became more comfortable wearing the thoracolumbar brace. Her CRP and ESR also improved and there was no radiologic evidence of fluid collection. The patient was discharged with a peripherally inserted central catheter in place and received IV ceftriaxone for 6 more weeks at home. This was followed by 4 weeks of oral ciprofloxacin 750 mg twice daily, thereby completing a 12-week course of antibiotics.

Our patient’s response to treatment was monitored clinically and the inflammatory markers were checked weekly after discharge until the end of treatment and at 6 and 12 months after start of treatment. At 12 months, our patient’s CRP was <1 mg/dL and ESR was 22 mm/h. One year later, our patient remained asymptomatic with normal inflammatory marker levels and no evidence of recurrence.

THE TAKEAWAY

Spondylodiscitis is an important differential diagnosis of lower back, flank, groin, and buttock pain. It’s important to be aware of this diagnosis, especially in patients who have risk factors such as IV drug abuse, diabetes, and morbid obesity. Although previous spinal surgery is a risk factor, spondylodiscitis should be considered in patients with persistent back pain even if they haven’t had spinal surgery. It can be present even when there is no tenderness over the spinous process or any fever.

Checking inflammatory markers is a reasonable next step if a patient’s pain does not resolve after at least 4 weeks. If levels of inflammatory markers such as CRP and ESR are elevated and symptoms continue, MRI can confirm or rule out the presence of spondylodiscitis. Treatments include orthotic support, antibiotics, and surgical intervention when complications arise.

THE CASE

A 23-year-old immunocompetent woman was referred to our spinal clinic with a 6-month history of low back pain that radiated to her right flank, buttock, and groin. She’d had intermittent urinary problems, including mild dysuria and frequency, and had been treated with antibiotics for a presumed urinary tract infection on 3 previous occasions, but her pain gradually increased and eventually became constant.

The patient had no history of fever, malaise, or weight loss. She denied consuming unpasteurized milk or undercooked poultry, and hadn’t recently experienced diarrhea or vomiting.

Eight years earlier, she had undergone anterior fusion of her spine for idiopathic scoliosis. At that time, she was at Risser grade 1, and her Cobb angle was 50°; metallic instrumentation was implanted at T10 to L3 to prevent progression of the scoliosis. Her recovery had been uneventful.

During examination, her temperature, pulse, respiratory rate, blood pressure, and nervous system were all normal. Her hips appeared normal, as well, and a straight leg raise was negative bilaterally. The patient had mild midline lumbar tenderness. Spinal range of movement revealed decreased flexion and mild pain.

X-rays (FIGURE 1) showed no changes in the previous metalwork in her spine. There was decreased disk height at the L3/4 level, but no significant bony erosion or soft-tissue shadows. Laboratory testing revealed a C-reactive protein (CRP) level of 240 mg/dL (normal, <1 mg/dL) and her erythrocyte sedimentation rate (ESR) was 102 mm/h—more than 5 times higher than it should have been.¹ The patient had a normal peripheral white cell count (WCC). Midstream urine cultures were negative.


The patient was admitted to the hospital for further work-up. Magnetic resonance imaging (MRI) of the lumbar spine showed gross abnormality at the L3-L4 disk level with erosion of the end plates, fluid in the disk space, marked enhancing edema, and mild surrounding soft-tissue edematous changes, but no evidence of any epidural abscess (FIGURE 2). The patient had a fluoroscopy-guided needle biopsy of the disk on the same day and received intravenous (IV) ceftriaxone 2 g twice a day. Blood and urine cultures were negative.

THE DIAGNOSIS

We suspected our patient had spondylodiscitis, an infection of the spine that includes spondylitis (inflammation of the vertebrae) and discitis (inflammation of the vertebral disk space). After 48 hours, the biopsy sample grew Salmonella typhimurium and confirmed the diagnosis. The organism was sensitive to ceftriaxone and ciprofloxacin; parenteral ceftriaxone was continued and the patient wore a thoracolumbar brace for immobilization. For 3 days, her inflammatory marker levels were checked daily, then every other day for the rest of that first week, and then 2 more times in the following week.

DISCUSSION

Thoracic and lumbar vertebrae are the most common sites of spondylodiscitis.² Spondylodiscitis accounts for 3% to 5% of pyogenic osteomyelitis in patients in developed countries.³ The incidence of pyogenic spondylodiscitis may be rising due to an increase in the number of elderly and immunocompromised patients, as well as a rise in invasive medical procedures.^4-6

If left untreated, spondylodiscitis can spread longitudinally (involving the adjacent levels), posteriorly (causing bacterial meningitis, abscess formation, and cord compromise), or anteriorly (causing paravertebral abscess). Untreated spondylodiscitis can also send distant infective emboli and cause endocarditis^7-9 or mycotic abdominal aneurysm.¹⁰

Historically, mortality in patients with vertebral osteomyelitis has been as high as 25%.¹¹ The combination of earlier diagnosis, antibiotics, and surgical debridement and stabilization has decreased mortality to less than 15%.^12-14

Risk factors for spondylodiscitis include male sex, IV drug abuse, diabetes, morbid obesity, having had a genitourinary or spinal procedure, and being immunocompromised (eg, from alcohol abuse, malignancy, organ transplantation, chemotherapy, or corticosteroid use).^12,15,16

Gram-positive organisms cause most spine infections in both adults and children, with 40% to 90% caused by Staphylococcus aureus.¹⁷ Gram-negative organisms (Escherichia coli, Pseudomonas, and Proteus), which can also cause spondylodiscitis, typically occur after genitourinary infections or procedures. IV drug abusers are prone to Pseudomonas infections.¹⁸ Anaerobic infections may be seen in patients with diabetes or after penetrating trauma.¹⁵ Salmonella species can cause spondylodiscitis, especially in patients with sickle cell disease from an intestinal source.¹⁹

Mycobacterium tuberculosis is the most common nonpyogenic infecting agent that also can cause spondylodiscitis. Infection caused by tuberculosis (TB) has had a recent resurgence with resistant strains, especially in patients with human immunodeficiency virus.¹⁵ Although less than 10% of patients with TB have skeletal involvement, 50% of the skeletal involvement occurs in the spine.¹⁵

The clinical presentation of spondylodiscitis depends on the location of the infection, the virulence of the organism, and the immune status of the patient. Discitis can present as pain in the back, hip, abdomen (especially in children²⁰) and, occasionally, with meningeal involvement.¹¹ Patients with discitis often have a normal temperature.^15,21 In patients with discitis, the patient’s WCC will be normal, but the ESR is almost always elevated.^15,22 Suspect spondylodiscitis in patients who present with persistent or increasing pain 3 to 4 weeks after back surgery. For such patients, measure inflammatory markers and order imaging of the spine.

Risk factors for spondylodiscitis include IV drug abuse, diabetes, morbid obesity, and having had a genitourinary or spinal procedure. X-ray findings for patients with spondylodiscitis will include osteolysis and end plate erosions (early) and narrowing and collapse of the disk space (late). (In TB, relative preservation of the disk spaces is seen, with significant vertebral destruction.)

MRI is the modality of choice for diagnosis and assessment of suspected spondylodiscitis because it can provide imaging of the soft tissue, neural elements, and bony changes with a high sensitivity and specificity.²³ Once infection is suspected, the diagnosis should be confirmed by fluoroscopic- or computed tomography-guided biopsy before starting antibiotic treatment.

Long-term antibiotics
 are required to prevent recurrence


IV antibiotics are the mainstay of treatment for spondylodiscitis;²⁴ the specific drug used will depend upon the organism identified. Patients typically receive 2 to 6 weeks of IV therapy. Then, once the patient improves and inflammatory markers return to normal levels, the patient receives a course of oral antibiotics for 2 to 6 more weeks. Grados et al¹⁹ found recurrence rates of 10% to 15% for patients who were treated 4 to 8 weeks compared to 3.9% in those treated for 12 weeks or longer; therefore, a total duration of 12 weeks is commonly chosen.^25-28

To minimize the risk of spondylolisthesis, kyphosis, and fractures of the infected bone, patients are advised to rest and the spine is often immobilized with a spinal brace. Surgery may be needed if antibiotics are not effective, or for patients who develop complications such as fluid collection, neurologic deficits, or deformity.

Our patient’s pain improved after 2 weeks and she became more comfortable wearing the thoracolumbar brace. Her CRP and ESR also improved and there was no radiologic evidence of fluid collection. The patient was discharged with a peripherally inserted central catheter in place and received IV ceftriaxone for 6 more weeks at home. This was followed by 4 weeks of oral ciprofloxacin 750 mg twice daily, thereby completing a 12-week course of antibiotics.

Our patient’s response to treatment was monitored clinically and the inflammatory markers were checked weekly after discharge until the end of treatment and at 6 and 12 months after start of treatment. At 12 months, our patient’s CRP was <1 mg/dL and ESR was 22 mm/h. One year later, our patient remained asymptomatic with normal inflammatory marker levels and no evidence of recurrence.

THE TAKEAWAY

Spondylodiscitis is an important differential diagnosis of lower back, flank, groin, and buttock pain. It’s important to be aware of this diagnosis, especially in patients who have risk factors such as IV drug abuse, diabetes, and morbid obesity. Although previous spinal surgery is a risk factor, spondylodiscitis should be considered in patients with persistent back pain even if they haven’t had spinal surgery. It can be present even when there is no tenderness over the spinous process or any fever.

Checking inflammatory markers is a reasonable next step if a patient’s pain does not resolve after at least 4 weeks. If levels of inflammatory markers such as CRP and ESR are elevated and symptoms continue, MRI can confirm or rule out the presence of spondylodiscitis. Treatments include orthotic support, antibiotics, and surgical intervention when complications arise.

Physeal separation of the distal humerus in a newborn is a rare and severe injury that requires immediate treatment. This fracture was reported as an extremely rare complication of cesarean section.¹ The correct diagnosis can be established by clinical and radiologic findings. However, this injury can be easily overlooked and misdiagnosed. Presentation often involves swelling, tenderness, and agitation with movement of the elbow.

We report a case in which neonatal physeal separation of the distal humerus occurred during cesarean section. The diagnosis was based on clinical and radiologic/arthrographic findings and treated with closed reduction and percutaneous fixation. The patient’s guardian provided written informed consent for print and electronic publication of this case report.

Case Report

A full-term (40-week gestation) male neonate weighing 3690 g was born through cesarean section at the mother’s request. Apgar score was 9 at 1 minute and 10 at 5 minutes. The vertex position of the fetus was confirmed with preoperative ultrasonography. This was the mother’s first pregnancy and an in vitro fertilization. On his second day of life, the patient was referred to the orthopedic department for evaluation of local swelling and diminished spontaneous motion of the right elbow.

Examination revealed local tenderness and swelling in the anterior and lateral aspects of the elbow. Passive elbow range of motion (ROM) caused agitation, and elbow instability was present. A complete neurovascular examination was performed, and neurovascular injury and compartment syndrome were ruled out. Hematologic workup showed no signs of septic arthritis. Radiographs showed posteromedial displacement of the humeroulnar joint. The patient was placed in a long-arm splint, and no reduction was attempted initially.

The patient was taken to the operating room the same day. With the patient under general anesthesia, an arthrogram of the right elbow was obtained. It showed posteromedial displacement of the distal humeral epiphysis (Figure 1A). Closed reduction was performed, and the quality of the reduction was confirmed by intraoperative imaging. Percutaneously, a single 2-mm Kirschner wire (K-wire) was placed in an oblique fashion from the inferolateral aspect of the distal fragment to the contralateral metaphysis of the humerus (Figure 1B). The patient was put in a long-arm splint with the elbow flexed at 90° and the forearm in midpronation.

Follow-up visits were scheduled for 1 week, 3 weeks, and 5 weeks after surgery. Three weeks after surgery, callus formation was confirmed, and the K-wire was removed. Five weeks after surgery, the long-arm splint was removed.

At 6-month follow-up, the patient was pain-free and had full elbow ROM, and radiographs (Figures 2A, 2B) confirmed anatomical restoration of the fracture.

Discussion

Madsen² reported the incidence of birth-related long-bone fractures, including fractures of the humerus, the femur, and the tibia (< 0.1%). According to that review, only 1 of 105,119 patients sustained traumatic physeal separation of the distal humerus.

Different mechanisms have been described for this rare fracture. As the physeal region is the weakest part of the distal humerus, it is prone to injury by rotational shear forces,^3,4 hyperextension of the elbow, or a backward thrust on the forearm with the elbow flexed.⁵ Excessive traction applied during cesarean delivery might cause physeal separation, which was the possible cause in the present case. Most patients have a complicated birth history.

This injury should be suspected in an irritable newborn with swelling, tenderness, and reduced mobility of the upper extremity. Osteomyelitis and septic arthritis should be considered in the differential diagnosis. Brachial plexus injury and dislocation of the elbow joint should also be kept in mind. Child abuse and metabolic bone diseases (eg, osteogenesis imperfecta) should also be considered.

Anteroposterior and lateral plain radiographs of the elbow usually establish the diagnosis. Alteration of humeroulnar alignment and displacement of the proximal forearm are the key points leading to the diagnosis.

The cartilaginous part of the distal humerus and humeroulnar alignment can be demonstrated by ultrasonography.⁶ Magnetic resonance imaging (MRI) can be helpful in diagnosis but is seldom required,⁷ and the sedation or general anesthesia used is a disadvantage. Arthrography is useful not only in diagnosis but in determining the quality of the reduction.⁸ An arthrogram may show that open reduction is unnecessary.

Treatment differs widely. In neonates, who have a tremendous healing capability, this fracture almost always heals uneventfully. An effective treatment method is closed reduction and cast immobilization. However, valgus malalignment and limited elbow ROM were noted in 5% of the patients treated with this method.⁴

Jacobsen and colleagues⁴ reported on 6 neonates who sustained traumatic separation of the distal epiphysis of the humerus at birth and who were treated with casting with or without closed reduction. The authors described good results. One patient had varus malalignment, which was attributed to fragment internal rotation caused by rotational instability.

As our patient’s instability was noted during surgery, we performed percutaneous pinning after arthrography-assisted closed reduction. We considered using 2 lateral pins for fixation, but, after the first pin was placed, fluoroscopic stress testing with the patient under anesthesia demonstrated adequate stability. A second, smaller pin could have been used to control rotation, if needed. Medial pin placement that avoids the ulnar nerve is difficult in the newborn elbow; medial pins should probably be avoided in the newborn, if possible.

Early diagnosis and treatment are essential. Late diagnosis was reported to lead to complications such as varus deformity and restriction of joint ROM.⁴

Our patient healed without any complications and achieved full ROM. Long-term follow-up is needed to diagnose any physeal bar that might lead to secondary deformities.

Conclusion

Cesarean section is reported to reduce birth complications, but it might cause fractures of the femur and humerus.¹ Avoiding application of excessive traction to the forearm can prevent physeal separation of the distal humerus. This entity should be kept in mind as a potential complication of cesarean section. Arthrography is helpful in treatment and may help avoid unnecessary open reduction.

Physeal separation of the distal humerus in a newborn is a rare and severe injury that requires immediate treatment. This fracture was reported as an extremely rare complication of cesarean section.¹ The correct diagnosis can be established by clinical and radiologic findings. However, this injury can be easily overlooked and misdiagnosed. Presentation often involves swelling, tenderness, and agitation with movement of the elbow.

We report a case in which neonatal physeal separation of the distal humerus occurred during cesarean section. The diagnosis was based on clinical and radiologic/arthrographic findings and treated with closed reduction and percutaneous fixation. The patient’s guardian provided written informed consent for print and electronic publication of this case report.

Case Report

A full-term (40-week gestation) male neonate weighing 3690 g was born through cesarean section at the mother’s request. Apgar score was 9 at 1 minute and 10 at 5 minutes. The vertex position of the fetus was confirmed with preoperative ultrasonography. This was the mother’s first pregnancy and an in vitro fertilization. On his second day of life, the patient was referred to the orthopedic department for evaluation of local swelling and diminished spontaneous motion of the right elbow.

Examination revealed local tenderness and swelling in the anterior and lateral aspects of the elbow. Passive elbow range of motion (ROM) caused agitation, and elbow instability was present. A complete neurovascular examination was performed, and neurovascular injury and compartment syndrome were ruled out. Hematologic workup showed no signs of septic arthritis. Radiographs showed posteromedial displacement of the humeroulnar joint. The patient was placed in a long-arm splint, and no reduction was attempted initially.

The patient was taken to the operating room the same day. With the patient under general anesthesia, an arthrogram of the right elbow was obtained. It showed posteromedial displacement of the distal humeral epiphysis (Figure 1A). Closed reduction was performed, and the quality of the reduction was confirmed by intraoperative imaging. Percutaneously, a single 2-mm Kirschner wire (K-wire) was placed in an oblique fashion from the inferolateral aspect of the distal fragment to the contralateral metaphysis of the humerus (Figure 1B). The patient was put in a long-arm splint with the elbow flexed at 90° and the forearm in midpronation.

Follow-up visits were scheduled for 1 week, 3 weeks, and 5 weeks after surgery. Three weeks after surgery, callus formation was confirmed, and the K-wire was removed. Five weeks after surgery, the long-arm splint was removed.

At 6-month follow-up, the patient was pain-free and had full elbow ROM, and radiographs (Figures 2A, 2B) confirmed anatomical restoration of the fracture.

Discussion

Madsen² reported the incidence of birth-related long-bone fractures, including fractures of the humerus, the femur, and the tibia (< 0.1%). According to that review, only 1 of 105,119 patients sustained traumatic physeal separation of the distal humerus.

Different mechanisms have been described for this rare fracture. As the physeal region is the weakest part of the distal humerus, it is prone to injury by rotational shear forces,^3,4 hyperextension of the elbow, or a backward thrust on the forearm with the elbow flexed.⁵ Excessive traction applied during cesarean delivery might cause physeal separation, which was the possible cause in the present case. Most patients have a complicated birth history.

This injury should be suspected in an irritable newborn with swelling, tenderness, and reduced mobility of the upper extremity. Osteomyelitis and septic arthritis should be considered in the differential diagnosis. Brachial plexus injury and dislocation of the elbow joint should also be kept in mind. Child abuse and metabolic bone diseases (eg, osteogenesis imperfecta) should also be considered.

Anteroposterior and lateral plain radiographs of the elbow usually establish the diagnosis. Alteration of humeroulnar alignment and displacement of the proximal forearm are the key points leading to the diagnosis.

The cartilaginous part of the distal humerus and humeroulnar alignment can be demonstrated by ultrasonography.⁶ Magnetic resonance imaging (MRI) can be helpful in diagnosis but is seldom required,⁷ and the sedation or general anesthesia used is a disadvantage. Arthrography is useful not only in diagnosis but in determining the quality of the reduction.⁸ An arthrogram may show that open reduction is unnecessary.

Treatment differs widely. In neonates, who have a tremendous healing capability, this fracture almost always heals uneventfully. An effective treatment method is closed reduction and cast immobilization. However, valgus malalignment and limited elbow ROM were noted in 5% of the patients treated with this method.⁴

Jacobsen and colleagues⁴ reported on 6 neonates who sustained traumatic separation of the distal epiphysis of the humerus at birth and who were treated with casting with or without closed reduction. The authors described good results. One patient had varus malalignment, which was attributed to fragment internal rotation caused by rotational instability.

As our patient’s instability was noted during surgery, we performed percutaneous pinning after arthrography-assisted closed reduction. We considered using 2 lateral pins for fixation, but, after the first pin was placed, fluoroscopic stress testing with the patient under anesthesia demonstrated adequate stability. A second, smaller pin could have been used to control rotation, if needed. Medial pin placement that avoids the ulnar nerve is difficult in the newborn elbow; medial pins should probably be avoided in the newborn, if possible.

Early diagnosis and treatment are essential. Late diagnosis was reported to lead to complications such as varus deformity and restriction of joint ROM.⁴

Our patient healed without any complications and achieved full ROM. Long-term follow-up is needed to diagnose any physeal bar that might lead to secondary deformities.

Conclusion

Cesarean section is reported to reduce birth complications, but it might cause fractures of the femur and humerus.¹ Avoiding application of excessive traction to the forearm can prevent physeal separation of the distal humerus. This entity should be kept in mind as a potential complication of cesarean section. Arthrography is helpful in treatment and may help avoid unnecessary open reduction.

Physeal separation of the distal humerus in a newborn is a rare and severe injury that requires immediate treatment. This fracture was reported as an extremely rare complication of cesarean section.¹ The correct diagnosis can be established by clinical and radiologic findings. However, this injury can be easily overlooked and misdiagnosed. Presentation often involves swelling, tenderness, and agitation with movement of the elbow.

We report a case in which neonatal physeal separation of the distal humerus occurred during cesarean section. The diagnosis was based on clinical and radiologic/arthrographic findings and treated with closed reduction and percutaneous fixation. The patient’s guardian provided written informed consent for print and electronic publication of this case report.

Case Report

A full-term (40-week gestation) male neonate weighing 3690 g was born through cesarean section at the mother’s request. Apgar score was 9 at 1 minute and 10 at 5 minutes. The vertex position of the fetus was confirmed with preoperative ultrasonography. This was the mother’s first pregnancy and an in vitro fertilization. On his second day of life, the patient was referred to the orthopedic department for evaluation of local swelling and diminished spontaneous motion of the right elbow.

Examination revealed local tenderness and swelling in the anterior and lateral aspects of the elbow. Passive elbow range of motion (ROM) caused agitation, and elbow instability was present. A complete neurovascular examination was performed, and neurovascular injury and compartment syndrome were ruled out. Hematologic workup showed no signs of septic arthritis. Radiographs showed posteromedial displacement of the humeroulnar joint. The patient was placed in a long-arm splint, and no reduction was attempted initially.

The patient was taken to the operating room the same day. With the patient under general anesthesia, an arthrogram of the right elbow was obtained. It showed posteromedial displacement of the distal humeral epiphysis (Figure 1A). Closed reduction was performed, and the quality of the reduction was confirmed by intraoperative imaging. Percutaneously, a single 2-mm Kirschner wire (K-wire) was placed in an oblique fashion from the inferolateral aspect of the distal fragment to the contralateral metaphysis of the humerus (Figure 1B). The patient was put in a long-arm splint with the elbow flexed at 90° and the forearm in midpronation.

Follow-up visits were scheduled for 1 week, 3 weeks, and 5 weeks after surgery. Three weeks after surgery, callus formation was confirmed, and the K-wire was removed. Five weeks after surgery, the long-arm splint was removed.

At 6-month follow-up, the patient was pain-free and had full elbow ROM, and radiographs (Figures 2A, 2B) confirmed anatomical restoration of the fracture.

Discussion

Madsen² reported the incidence of birth-related long-bone fractures, including fractures of the humerus, the femur, and the tibia (< 0.1%). According to that review, only 1 of 105,119 patients sustained traumatic physeal separation of the distal humerus.

Different mechanisms have been described for this rare fracture. As the physeal region is the weakest part of the distal humerus, it is prone to injury by rotational shear forces,^3,4 hyperextension of the elbow, or a backward thrust on the forearm with the elbow flexed.⁵ Excessive traction applied during cesarean delivery might cause physeal separation, which was the possible cause in the present case. Most patients have a complicated birth history.

This injury should be suspected in an irritable newborn with swelling, tenderness, and reduced mobility of the upper extremity. Osteomyelitis and septic arthritis should be considered in the differential diagnosis. Brachial plexus injury and dislocation of the elbow joint should also be kept in mind. Child abuse and metabolic bone diseases (eg, osteogenesis imperfecta) should also be considered.

Anteroposterior and lateral plain radiographs of the elbow usually establish the diagnosis. Alteration of humeroulnar alignment and displacement of the proximal forearm are the key points leading to the diagnosis.

The cartilaginous part of the distal humerus and humeroulnar alignment can be demonstrated by ultrasonography.⁶ Magnetic resonance imaging (MRI) can be helpful in diagnosis but is seldom required,⁷ and the sedation or general anesthesia used is a disadvantage. Arthrography is useful not only in diagnosis but in determining the quality of the reduction.⁸ An arthrogram may show that open reduction is unnecessary.

Treatment differs widely. In neonates, who have a tremendous healing capability, this fracture almost always heals uneventfully. An effective treatment method is closed reduction and cast immobilization. However, valgus malalignment and limited elbow ROM were noted in 5% of the patients treated with this method.⁴

Jacobsen and colleagues⁴ reported on 6 neonates who sustained traumatic separation of the distal epiphysis of the humerus at birth and who were treated with casting with or without closed reduction. The authors described good results. One patient had varus malalignment, which was attributed to fragment internal rotation caused by rotational instability.

As our patient’s instability was noted during surgery, we performed percutaneous pinning after arthrography-assisted closed reduction. We considered using 2 lateral pins for fixation, but, after the first pin was placed, fluoroscopic stress testing with the patient under anesthesia demonstrated adequate stability. A second, smaller pin could have been used to control rotation, if needed. Medial pin placement that avoids the ulnar nerve is difficult in the newborn elbow; medial pins should probably be avoided in the newborn, if possible.

Early diagnosis and treatment are essential. Late diagnosis was reported to lead to complications such as varus deformity and restriction of joint ROM.⁴

Our patient healed without any complications and achieved full ROM. Long-term follow-up is needed to diagnose any physeal bar that might lead to secondary deformities.

Conclusion

Cesarean section is reported to reduce birth complications, but it might cause fractures of the femur and humerus.¹ Avoiding application of excessive traction to the forearm can prevent physeal separation of the distal humerus. This entity should be kept in mind as a potential complication of cesarean section. Arthrography is helpful in treatment and may help avoid unnecessary open reduction.