Pediatrics

Patients with childhood-onset systemic lupus erythematosus (SLE) who transitioned to adult care without a formal transitioning process had long periods without care despite having moderate disease activity and also frequently reported anxiety and depression in a retrospective study of 50 patients during a 3-year period at Brigham and Women’s Hospital Lupus Center in Boston.

Dr. Mary Beth Son of Boston Children’s Hospital and her colleagues found that the patients went a mean of nearly 9 months from their last pediatric rheumatology visit to their first adult rheumatology visit, and 72% had at least one gap in care, defined as no appointments in a recommended time frame. A total of 32% had more than one missed appointment, although there was an average of only 9% of appointments missed per patient. The investigators determined that missed appointments were significantly associated with white race, education below the high school level, and medication nonadherence (Lupus. 2016 Mar 23. doi: 10.1177/0961203316640913).

©Stuart Jenner/Thinkstock

“The finding of lower educational level leading to a suboptimal transition outcome may help to delineate an at-risk population that requires further support during the process of transition. ... Although missed appointments weren’t prominent, the majority of our patients experienced gaps in care whereby they didn’t schedule appointments within the recommended time frame per the treating physician. The lack of appropriate scheduling with its concomitant potentially serious consequences demonstrates the need to educate transition patients regarding the importance of scheduling their own appointments,” the investigators wrote.

Scores of the SLE Disease Activity Index remained stable from a mean of 5.7 at baseline to 4.7 at year 3, but Systemic Lupus International Collaborating Clinics/ACR Damage Index for Systemic Lupus Erythematosus scores rose significantly from 0.46 to 0.78. Depression and anxiety diagnoses or symptoms increased significantly from 10% to 26%, and the investigators noted that “more than one-quarter of them required support from social work for a variety of serious circumstances including homelessness, substance abuse, and incarceration.”

The patients were diagnosed at a mean age of 14.5 years, and most were white (42%), African American (22%), Hispanic (22%), or Asian (10%). They had a mean age of 19.5 years at their first Lupus Center visit and 21.9 years at their last.

The investigators noted that the patients’ relatively high socioeconomic status (zip code–based annual household income of $50,000-$100,000 in 60% and $100,000 or more in 32%) may limit the generalizability of the study.

The authors reported having no relevant conflicts of interest. Dr. Son was supported by a Boston Children’s Hospital Career Development Fellowship and another author’s grant from the National Institutes of Health helped to support the study.

jevans@frontlinemedcom.com

Patients with childhood-onset systemic lupus erythematosus (SLE) who transitioned to adult care without a formal transitioning process had long periods without care despite having moderate disease activity and also frequently reported anxiety and depression in a retrospective study of 50 patients during a 3-year period at Brigham and Women’s Hospital Lupus Center in Boston.

Dr. Mary Beth Son of Boston Children’s Hospital and her colleagues found that the patients went a mean of nearly 9 months from their last pediatric rheumatology visit to their first adult rheumatology visit, and 72% had at least one gap in care, defined as no appointments in a recommended time frame. A total of 32% had more than one missed appointment, although there was an average of only 9% of appointments missed per patient. The investigators determined that missed appointments were significantly associated with white race, education below the high school level, and medication nonadherence (Lupus. 2016 Mar 23. doi: 10.1177/0961203316640913).

©Stuart Jenner/Thinkstock

“The finding of lower educational level leading to a suboptimal transition outcome may help to delineate an at-risk population that requires further support during the process of transition. ... Although missed appointments weren’t prominent, the majority of our patients experienced gaps in care whereby they didn’t schedule appointments within the recommended time frame per the treating physician. The lack of appropriate scheduling with its concomitant potentially serious consequences demonstrates the need to educate transition patients regarding the importance of scheduling their own appointments,” the investigators wrote.

Scores of the SLE Disease Activity Index remained stable from a mean of 5.7 at baseline to 4.7 at year 3, but Systemic Lupus International Collaborating Clinics/ACR Damage Index for Systemic Lupus Erythematosus scores rose significantly from 0.46 to 0.78. Depression and anxiety diagnoses or symptoms increased significantly from 10% to 26%, and the investigators noted that “more than one-quarter of them required support from social work for a variety of serious circumstances including homelessness, substance abuse, and incarceration.”

The patients were diagnosed at a mean age of 14.5 years, and most were white (42%), African American (22%), Hispanic (22%), or Asian (10%). They had a mean age of 19.5 years at their first Lupus Center visit and 21.9 years at their last.

The investigators noted that the patients’ relatively high socioeconomic status (zip code–based annual household income of $50,000-$100,000 in 60% and $100,000 or more in 32%) may limit the generalizability of the study.

The authors reported having no relevant conflicts of interest. Dr. Son was supported by a Boston Children’s Hospital Career Development Fellowship and another author’s grant from the National Institutes of Health helped to support the study.

jevans@frontlinemedcom.com

Patients with childhood-onset systemic lupus erythematosus (SLE) who transitioned to adult care without a formal transitioning process had long periods without care despite having moderate disease activity and also frequently reported anxiety and depression in a retrospective study of 50 patients during a 3-year period at Brigham and Women’s Hospital Lupus Center in Boston.

Dr. Mary Beth Son of Boston Children’s Hospital and her colleagues found that the patients went a mean of nearly 9 months from their last pediatric rheumatology visit to their first adult rheumatology visit, and 72% had at least one gap in care, defined as no appointments in a recommended time frame. A total of 32% had more than one missed appointment, although there was an average of only 9% of appointments missed per patient. The investigators determined that missed appointments were significantly associated with white race, education below the high school level, and medication nonadherence (Lupus. 2016 Mar 23. doi: 10.1177/0961203316640913).

©Stuart Jenner/Thinkstock

“The finding of lower educational level leading to a suboptimal transition outcome may help to delineate an at-risk population that requires further support during the process of transition. ... Although missed appointments weren’t prominent, the majority of our patients experienced gaps in care whereby they didn’t schedule appointments within the recommended time frame per the treating physician. The lack of appropriate scheduling with its concomitant potentially serious consequences demonstrates the need to educate transition patients regarding the importance of scheduling their own appointments,” the investigators wrote.

Scores of the SLE Disease Activity Index remained stable from a mean of 5.7 at baseline to 4.7 at year 3, but Systemic Lupus International Collaborating Clinics/ACR Damage Index for Systemic Lupus Erythematosus scores rose significantly from 0.46 to 0.78. Depression and anxiety diagnoses or symptoms increased significantly from 10% to 26%, and the investigators noted that “more than one-quarter of them required support from social work for a variety of serious circumstances including homelessness, substance abuse, and incarceration.”

The patients were diagnosed at a mean age of 14.5 years, and most were white (42%), African American (22%), Hispanic (22%), or Asian (10%). They had a mean age of 19.5 years at their first Lupus Center visit and 21.9 years at their last.

The investigators noted that the patients’ relatively high socioeconomic status (zip code–based annual household income of $50,000-$100,000 in 60% and $100,000 or more in 32%) may limit the generalizability of the study.

The authors reported having no relevant conflicts of interest. Dr. Son was supported by a Boston Children’s Hospital Career Development Fellowship and another author’s grant from the National Institutes of Health helped to support the study.

jevans@frontlinemedcom.com

Pregnant woman

Photo by Nina Matthews

Results of a large study suggest that female survivors of childhood cancer may have more luck than their male peers when it comes to conceiving a child.

Both male and female childhood cancer survivors (CCSs) reported fewer pregnancies and live births than their healthy siblings.

However, chemotherapeutic agents appeared to have a much greater impact on the fertility of male CCSs than female CCSs.

Eric Chow, MD, of the Fred Hutchinson Cancer Research Center in Seattle, Washington, and his colleagues reported these findings in The Lancet Oncology.

Previous research has shown that fertility can be compromised by several types of chemotherapy, mainly alkylating drugs. However, little is known about the dose effects on pregnancy from newer drugs, such as ifosfamide and cisplatin, in CCSs.

With this in mind, Dr Chow and his colleagues analyzed data from the Childhood Cancer Survivor Study, which tracks subjects who were diagnosed with the most common types of childhood cancer before the age of 21 and treated at 27 institutions across the US and Canada between 1970 and 1999.

Patients had been diagnosed with leukemias, lymphomas, and neuroblastoma, as well as kidney, brain, soft tissue, and bone tumors. All had survived at least 5 years after diagnosis.

The researchers examined the impact of various doses of 14 commonly used chemotherapy drugs on pregnancy and live birth in 10,938 male and female CCSs, compared with 3949 siblings.

The team specifically focused on CCSs who were treated with chemotherapy and did not receive any radiotherapy to the pelvis or the brain.

The drugs the researchers evaluated were busulfan, carboplatin, carmustine, chlorambucil, chlormethine, cisplatin, cyclophosphamide, dacarbazine, ifosfamide, lomustine, melphalan, procarbazine, temozolomide, and thiotepa.

Outcomes

Multivariable analysis showed that CCSs were significantly less likely than their siblings to have or sire a pregnancy. For male CCSs, the hazard ratio (HR) was 0.63 (P<0.0001). For female CCSs, the HR was 0.87 (P<0.0001).

CCSs were also significantly less likely to have a live birth. For male CCSs, the HR was 0.63 (P<0.0001). For female CCSs, the HR was 0.82 (P<0.0001).

The researchers noted that, overall, female CCSs were less likely to conceive and have a child when compared to their siblings, but the effect was much smaller than that observed among the men.

In addition, the difference between CCSs and siblings was more pronounced for women who delayed pregnancy until they were 30 or older, possibly because chemotherapy exposure might accelerate the natural depletion of eggs and hasten menopause.

Impact of specific drugs

In male CCSs, the reduced likelihood of siring a pregnancy was associated with upper tertile doses of cyclophosphamide (HR=0.60, P<0.0001), ifosfamide (HR=0.42, P=0.0069), procarbazine (HR=0.30, P<0.0001), and cisplatin (HR=0.56, P=0.0023).

Cyclophosphamide-equivalent dose in male CCSs was significantly associated with a decreased likelihood of siring a pregnancy per 5000 mg/m² increments (HR=0.82, P<0.0001).

In female CCSs, the reduced likelihood of becoming pregnant was associated with busulfan—both at doses less than 450 mg/m² (HR=0.22, P=0.020) and at doses of 450 mg/m² or higher (HR=0.14, P=0.0051)—and with doses of lomustine at 411 mg/m² or greater (HR=0.41, P=0.046).

Cyclophosphamide-equivalent dose in female CCSs was associated with risk only at the highest doses in analyses categorized by quartile (upper quartile vs no exposure, HR=0.85, P=0.023).

Limitations and implications

The researchers noted that a limitation of this study is that it relied on self-reported pregnancy and live birth, and some pregnancies may go unrecognized.

And although the findings are consistent with others in the field, this study did not account for other factors such as marital or cohabitation status, the intention to conceive, or length of time attempting to conceive.

The researchers also noted that, although the total number of CCSs in this study is large, the number of patients who were exposed to individual drugs varied significantly. So while the overall conclusions of the study are consistent with previous studies, more research is needed to estimate the exact risk of some less commonly used drugs.

“We think these results will be encouraging for most women who were treated with chemotherapy in childhood,” Dr Chow said. “However, I think we, as pediatric oncologists, still need to do a better job discussing fertility and fertility preservation options with patients and families upfront before starting cancer treatment.”

“In particular, all boys diagnosed post-puberty should be encouraged to bank their sperm to maximize their reproductive options in the future. The current options for post-pubertal girls remain more complicated but include oocyte and embryo cryopreservation.”

Pregnant woman

Photo by Nina Matthews

Results of a large study suggest that female survivors of childhood cancer may have more luck than their male peers when it comes to conceiving a child.

Both male and female childhood cancer survivors (CCSs) reported fewer pregnancies and live births than their healthy siblings.

However, chemotherapeutic agents appeared to have a much greater impact on the fertility of male CCSs than female CCSs.

Eric Chow, MD, of the Fred Hutchinson Cancer Research Center in Seattle, Washington, and his colleagues reported these findings in The Lancet Oncology.

Previous research has shown that fertility can be compromised by several types of chemotherapy, mainly alkylating drugs. However, little is known about the dose effects on pregnancy from newer drugs, such as ifosfamide and cisplatin, in CCSs.

With this in mind, Dr Chow and his colleagues analyzed data from the Childhood Cancer Survivor Study, which tracks subjects who were diagnosed with the most common types of childhood cancer before the age of 21 and treated at 27 institutions across the US and Canada between 1970 and 1999.

Patients had been diagnosed with leukemias, lymphomas, and neuroblastoma, as well as kidney, brain, soft tissue, and bone tumors. All had survived at least 5 years after diagnosis.

The researchers examined the impact of various doses of 14 commonly used chemotherapy drugs on pregnancy and live birth in 10,938 male and female CCSs, compared with 3949 siblings.

The team specifically focused on CCSs who were treated with chemotherapy and did not receive any radiotherapy to the pelvis or the brain.

The drugs the researchers evaluated were busulfan, carboplatin, carmustine, chlorambucil, chlormethine, cisplatin, cyclophosphamide, dacarbazine, ifosfamide, lomustine, melphalan, procarbazine, temozolomide, and thiotepa.

Outcomes

Multivariable analysis showed that CCSs were significantly less likely than their siblings to have or sire a pregnancy. For male CCSs, the hazard ratio (HR) was 0.63 (P<0.0001). For female CCSs, the HR was 0.87 (P<0.0001).

CCSs were also significantly less likely to have a live birth. For male CCSs, the HR was 0.63 (P<0.0001). For female CCSs, the HR was 0.82 (P<0.0001).

The researchers noted that, overall, female CCSs were less likely to conceive and have a child when compared to their siblings, but the effect was much smaller than that observed among the men.

In addition, the difference between CCSs and siblings was more pronounced for women who delayed pregnancy until they were 30 or older, possibly because chemotherapy exposure might accelerate the natural depletion of eggs and hasten menopause.

Impact of specific drugs

In male CCSs, the reduced likelihood of siring a pregnancy was associated with upper tertile doses of cyclophosphamide (HR=0.60, P<0.0001), ifosfamide (HR=0.42, P=0.0069), procarbazine (HR=0.30, P<0.0001), and cisplatin (HR=0.56, P=0.0023).

Cyclophosphamide-equivalent dose in male CCSs was significantly associated with a decreased likelihood of siring a pregnancy per 5000 mg/m² increments (HR=0.82, P<0.0001).

In female CCSs, the reduced likelihood of becoming pregnant was associated with busulfan—both at doses less than 450 mg/m² (HR=0.22, P=0.020) and at doses of 450 mg/m² or higher (HR=0.14, P=0.0051)—and with doses of lomustine at 411 mg/m² or greater (HR=0.41, P=0.046).

Cyclophosphamide-equivalent dose in female CCSs was associated with risk only at the highest doses in analyses categorized by quartile (upper quartile vs no exposure, HR=0.85, P=0.023).

Limitations and implications

The researchers noted that a limitation of this study is that it relied on self-reported pregnancy and live birth, and some pregnancies may go unrecognized.

And although the findings are consistent with others in the field, this study did not account for other factors such as marital or cohabitation status, the intention to conceive, or length of time attempting to conceive.

The researchers also noted that, although the total number of CCSs in this study is large, the number of patients who were exposed to individual drugs varied significantly. So while the overall conclusions of the study are consistent with previous studies, more research is needed to estimate the exact risk of some less commonly used drugs.

“We think these results will be encouraging for most women who were treated with chemotherapy in childhood,” Dr Chow said. “However, I think we, as pediatric oncologists, still need to do a better job discussing fertility and fertility preservation options with patients and families upfront before starting cancer treatment.”

“In particular, all boys diagnosed post-puberty should be encouraged to bank their sperm to maximize their reproductive options in the future. The current options for post-pubertal girls remain more complicated but include oocyte and embryo cryopreservation.”

Pregnant woman

Photo by Nina Matthews

Results of a large study suggest that female survivors of childhood cancer may have more luck than their male peers when it comes to conceiving a child.

Both male and female childhood cancer survivors (CCSs) reported fewer pregnancies and live births than their healthy siblings.

However, chemotherapeutic agents appeared to have a much greater impact on the fertility of male CCSs than female CCSs.

Eric Chow, MD, of the Fred Hutchinson Cancer Research Center in Seattle, Washington, and his colleagues reported these findings in The Lancet Oncology.

Previous research has shown that fertility can be compromised by several types of chemotherapy, mainly alkylating drugs. However, little is known about the dose effects on pregnancy from newer drugs, such as ifosfamide and cisplatin, in CCSs.

With this in mind, Dr Chow and his colleagues analyzed data from the Childhood Cancer Survivor Study, which tracks subjects who were diagnosed with the most common types of childhood cancer before the age of 21 and treated at 27 institutions across the US and Canada between 1970 and 1999.

Patients had been diagnosed with leukemias, lymphomas, and neuroblastoma, as well as kidney, brain, soft tissue, and bone tumors. All had survived at least 5 years after diagnosis.

The researchers examined the impact of various doses of 14 commonly used chemotherapy drugs on pregnancy and live birth in 10,938 male and female CCSs, compared with 3949 siblings.

The team specifically focused on CCSs who were treated with chemotherapy and did not receive any radiotherapy to the pelvis or the brain.

The drugs the researchers evaluated were busulfan, carboplatin, carmustine, chlorambucil, chlormethine, cisplatin, cyclophosphamide, dacarbazine, ifosfamide, lomustine, melphalan, procarbazine, temozolomide, and thiotepa.

Outcomes

Multivariable analysis showed that CCSs were significantly less likely than their siblings to have or sire a pregnancy. For male CCSs, the hazard ratio (HR) was 0.63 (P<0.0001). For female CCSs, the HR was 0.87 (P<0.0001).

CCSs were also significantly less likely to have a live birth. For male CCSs, the HR was 0.63 (P<0.0001). For female CCSs, the HR was 0.82 (P<0.0001).

The researchers noted that, overall, female CCSs were less likely to conceive and have a child when compared to their siblings, but the effect was much smaller than that observed among the men.

In addition, the difference between CCSs and siblings was more pronounced for women who delayed pregnancy until they were 30 or older, possibly because chemotherapy exposure might accelerate the natural depletion of eggs and hasten menopause.

Impact of specific drugs

In male CCSs, the reduced likelihood of siring a pregnancy was associated with upper tertile doses of cyclophosphamide (HR=0.60, P<0.0001), ifosfamide (HR=0.42, P=0.0069), procarbazine (HR=0.30, P<0.0001), and cisplatin (HR=0.56, P=0.0023).

Cyclophosphamide-equivalent dose in male CCSs was significantly associated with a decreased likelihood of siring a pregnancy per 5000 mg/m² increments (HR=0.82, P<0.0001).

In female CCSs, the reduced likelihood of becoming pregnant was associated with busulfan—both at doses less than 450 mg/m² (HR=0.22, P=0.020) and at doses of 450 mg/m² or higher (HR=0.14, P=0.0051)—and with doses of lomustine at 411 mg/m² or greater (HR=0.41, P=0.046).

Cyclophosphamide-equivalent dose in female CCSs was associated with risk only at the highest doses in analyses categorized by quartile (upper quartile vs no exposure, HR=0.85, P=0.023).

Limitations and implications

The researchers noted that a limitation of this study is that it relied on self-reported pregnancy and live birth, and some pregnancies may go unrecognized.

And although the findings are consistent with others in the field, this study did not account for other factors such as marital or cohabitation status, the intention to conceive, or length of time attempting to conceive.

The researchers also noted that, although the total number of CCSs in this study is large, the number of patients who were exposed to individual drugs varied significantly. So while the overall conclusions of the study are consistent with previous studies, more research is needed to estimate the exact risk of some less commonly used drugs.

“We think these results will be encouraging for most women who were treated with chemotherapy in childhood,” Dr Chow said. “However, I think we, as pediatric oncologists, still need to do a better job discussing fertility and fertility preservation options with patients and families upfront before starting cancer treatment.”

“In particular, all boys diagnosed post-puberty should be encouraged to bank their sperm to maximize their reproductive options in the future. The current options for post-pubertal girls remain more complicated but include oocyte and embryo cryopreservation.”

IN THIS ARTICLE

• Diagnosis
• Management
• Newborn hip evaluation algorithm

Developmental dysplasia of the hip (DDH), previously known as congenital dislocation of the hip, follows a spectrum of irregular anatomic hip development spanning from acetabular dysplasia to irreducible dislocation at birth. Early detection is critical to improve the overall prognosis. Prompt diagnosis requires understanding of potential risk factors, proficiency in physical examination techniques, and implementation of appropriate screening tools when indicated. Although current guidelines direct timing for physical exam screenings, imaging, and treatment, it is ultimately up to the provider to determine the best course of action on a case-by-case basis. This article provides a review of these topics and more.

CURRENT GUIDELINES

In 2000, the American Academy of Pediatrics (AAP) developed guidelines for detection of hip dysplasia, including recommendation of relevant physical exam screenings for all newborns.¹ In 2007, the Pediatric Orthopaedic Society of North America (POSNA) encouraged providers to follow the AAP guidelines with a continued recommendation to perform newborn screening for hip instability and routine follow-up evaluations until the child achieves walking.² The American Academy of Orthopaedic Surgeons (AAOS) also established clinical guidelines in 2014 that are endorsed by both AAP and POSNA.³ These guidelines support routine clinical screening; research evaluated infants up to 6 months old, however, limiting the recommendations to that age-group.

Failure to treat DDH early has been associated with serious negative sequelae that include chronic pain, degenerative arthritis, postural scoliosis, and early gait disturbances.⁴ Primary care providers are expected to perform thorough newborn hip exams with associated specialized tests (ie, Ortolani and Barlow, which are discussed in “Physical exam”) at each routine follow-up. Heightened clinical suspicion and risk factor awareness are key for primary care providers to promptly identify patients requiring orthopedic referral. With early diagnosis, a removable soft abduction brace can be applied as the initial treatment. When treatment is delayed, however, closed reduction under anesthesia or complex surgical intervention may be required.

EPIDEMIOLOGY

The etiology for DDH remains unknown. Hip dysplasia typically presents unilaterally but can also occur bilaterally. DDH is more likely to affect the left hip than the right.⁵

Reported incidence varies, ranging from 0.06 to 76.1 per 1,000 live births, and is largely affected by race and geographic location.⁵ Incidence is higher in countries where routine screening is required, by either physical examination or ultrasound (1.6 to 28.5 and 34.0 to 60.3 per 1,000, respectively), compared with countries not requiring routine screening (1.3 per 1,000). This may suggest that the majority of hip dysplasia cases are transient and resolve spontaneously without treatment.^6,7

RISK FACTORS AND PATIENT HISTORY

Known risk factors for DDH include breech presentation (see Figure 1), positive family history, and female gender.^5,8-10 Female infants are eight times more likely than males to develop DDH.¹⁰ Firstborn status is also recognized as an associated risk factor, which may be attributable to space constraints in utero. This hypothesis is further supported by the relative DDH-protective effect of prematurity and low birth weight. Other potential risk factors include advanced maternal age, birth weight that is high for gestational age, decreased hip abduction, and joint laxity. However, the majority of patients with hip dysplasia have no identifiable risk ­factors.^3,5,9,11,12

Swaddling, which often maintains the hips in an adducted and/or extended position, has also been strongly associated with hip dysplasia.^5,13 Multiple organizations, including the AAOS,AAP, POSNA, and the International Hip Dysplasia Institute, have developed or endorsed hip-healthy swaddling recommendations to minimize the risk for DDH in swaddled infants.^13-15 Such practices allow the infant’s legs to bend up and out at the hips, promoting free hip movement, flexion, and abduction.^13,15 Swaddling has demonstrated multiple benefits (including improved sleep and relief of excessive crying¹³) and continues to be recommended by many US providers; however, those caring for infants at risk for DDH should avoid traditional swaddling and/or practice hip-healthy swaddling techniques.^10,13,14 Early diagnosis starts with the clinician’s knowledge of DDH risk factors and the recommended screening protocols. The presence of multiple risk factors will increase the likelihood of this condition and should lower the clinician’s threshold for ordering additional screening, regardless of hip exam findings.

PHYSICAL EXAM

Both AAP and AAOS guidelines recommend clinical screening for DDH with physical exam in all newborns.^1,3 A head-to-toe musculoskeletal exam is warranted during the initial evaluation of every newborn in order to assess for any known DDH-associated conditions, which may include neuromuscular disorders, torticollis, and metatarsus adductus.⁵

Initial evaluation of an infant with DDH may reveal nonspecific findings, including asymmetric skin folds and limb-length inequality. The Galeazzi sign should be sought by aligning flexed knees with the child in the supine position and assessing for uneven knee heights (see Figure 2). Unilateral posterior hip dislocation or femoral shortening represents a positive Galeazzi sign.¹⁶ Joint laxity and limited hip abduction have also been associated with DDH.^1,10

Barlow and Ortolani exams are more specific to DDH and should be completed at newborn screening and each subsequent well-baby exam.¹ The Barlow maneuver is a provocative test with flexion, adduction, and posterior pressure through the infant’s hip (Figure 3). A palpable clunk during the Barlow maneuver indicates positive instability with posterior displacement. The Ortolani test is a reductive maneuver requiring abduction with posterior pressure to lift the greater trochanter (Figure 4). A clunk sensation with this test is positive for reduction of the hip.

The infant’s diaper should be removed during the hip evaluation. These exams are more reliable when each hip is evaluated separately with the pelvis stabilized.¹⁰ All physical exam findings must be carefully documented at each encounter.^1,17

It is critical for the examiner to understand the appropriate technique and potential results when conducting each of these specialized hip exams. A true positive finding is the clunking sensation that occurs with the dislocation or relocation of the affected hip; this finding is better felt than heard. In contrast, a benign hip click with these maneuvers is a more subtle sensation—typically, a soft-tissue snapping or catching—and is not diagnostic of DDH. A click is not a clunk and is not indicative of DDH.^1,3

DDH may present later in infancy or early childhood; therefore, DDH should remain within the differential diagnosis for gait asymmetry, unequal hip motion, or limb-length discrepancy. It may be beneficial to continue to evaluate for these developments during routine exams as part of a thorough pediatric musculoskeletal assessment, particularly in patients with documented risk factors for DDH.^1,3,4 Delay in diagnosis of DDH, it should be noted, is a relatively common complaint in pediatric medical malpractice lawsuits; until the early 2000s, this condition represented about 75% of claims in one medical malpractice database.The decrease in claims has been attributed to better awareness and earlier diagnosis of DDH. ¹⁷

Continue for the diagnosis >>

DIAGNOSIS

A positive Ortolani or Barlow sign is diagnostic and warrants prompt orthopedic referral (Figure 5). If physical examination results are equivocal or inconclusive, follow-up at two weeks is recommended, with continued routine follow-up until walking is achieved. Patients with persistent equivocal findings at the two-week follow-up warrant ultrasound at age 3 to 4 weeks or orthopedic referral. Infants with significant risk factors, particularly breech presentation at birth, should also undergo imaging.¹⁸ AAP recommends ultrasound at age 6 weeks or radiograph after 4 months of age.^1,18 AAOS recommends performing an imaging study before age 6 months when at least one of the following risk factors is present: breech presentation, positive family history of DDH, or previous clinical instability (moderate level of evidence).³

IMAGING

Ultrasound is the diagnostic test of choice for infants because radiographs have limited value until the femoral heads begin to ossify at age 4 to 6 months.¹⁸ Ultrasonography allows for visualization of the cartilaginous portion of the acetabulum and femoral head.¹ Dynamic stressing is performed during ultrasound to assess the level of hip stability. A provider trained in ultrasound will measure the depth of the acetabulum and identify any potential laxity or instability of the hip joint. Accuracy of these findings is largely dependent on the experience and skill of the examiner.

Ultrasound evaluation is not recommended until after age 3 to 4 weeks. Earlier findings may include mild laxity and immature morphology of the acetabulum, which often resolve spontaneously.^1,18 Use of ultrasound is currently recommended only to confirm diagnostic suspicion, based on clinical findings, or for infants with significant risk factors.¹⁸ Universal ultrasound screening in newborns is not recommended and would incur unnecessary costs.^1,3,9 Plain radiographs are used after age 4 months to confirm a diagnosis of DDH or to assess for residual dysplasia.^3,18

Continue for management >>

MANAGEMENT

Once hip dysplasia is suggested by physical exam or imaging study, the child’s subsequent care should be provided by an orthopedic specialist with experience in treating this condition. Treatment is preferably initiated before age 6 weeks.¹² The specifics of treatment are largely based on age at diagnosis and the severity of dysplasia.

The goal of treatment is to maintain the hips in a stable position with the femoral head well covered by the acetabulum. This will improve anatomic development and function. Early clinical diagnosis is often sufficient to justify initiating conservative treatment; additionally, early detection of DDH can considerably reduce the need for surgical intervention.¹² Although the potential for spontaneous resolution is high, the consequences associated with delay in care can be significant.

Preferred initial management, which can be initiated before confirmation of DDH by ultrasound, involves implementation of soft abduction support.¹⁹ The Pavlik harness is the support design of choice (Figure 6).¹² This harness maintains hip flexion and abduction, creating concentric reduction of the femoral head. The brace is highly successful when its use is initiated early. Treatment in a Pavlik harness requires nearly full-time wear and close monitoring by a clinician. Unlikely potential risks associated with this treatment include avascular necrosis and femoral nerve palsy.⁴

Ultrasonography is used to further monitor treatment and to determine length of wear. Long-term results suggest a success rate exceeding 90%.^20,21 However, this rate may be falsely elevated due to the number of hips that likely would have improved spontaneously without treatment.^6,19

The Pavlik harness becomes less effective with increasing age, and a more rigid abduction brace may be considered in infants older than 6 months.²⁰ Overall outcomes improve once the femoral head is consistently maintained in the acetabulum. Delay in treatment is associated with an increase in the long-term complications associated with residual hip dysplasia.²²

Once an infant is undergoing treatment for DDH in a Pavlik harness, there is no need for primary care providers to continue to perform provocative testing, such as the Ortolani or Barlow test, at routine well-baby checks. Unnecessary stress to the hips is not beneficial, and any new results will not change the treatment being provided by the orthopedic specialist. Adjustments to the fit of the harness should be made only by the orthopedist, unless femoral nerve palsy is noted on exam. This development warrants immediate discontinuation of harness use until symptoms resolve.²¹

Abduction bracing may not be suitable for all cases of hip dysplasia. Newborns with irreducible hips, more advanced dysplasia, or associated neuromuscular or syndromic disorder may require closed versus open reduction and casting. More invasive surgical options may also be considered in advanced dysplasia in order to reshape the joint and improve function.^20,22

Continue for patient education >>

PATIENT EDUCATION

Parents should be fully educated on the options for managing hip dysplasia. Once DDH is diagnosed, prompt referral to an orthopedic specialist is critical in order to weigh the treatment options and to develop the appropriate individualized plan for each child. Once treatment is initiated, parental compliance is essential; frequent meetings between parents and the specialist are important.

Parents of infants with known risk factors for and/or suspicion of hip dysplasia should also be educated on hip-healthy swaddling to allow for free motion of the hips and knees.^10,13 Advise them that some commercial baby carriers and slings may maintain the hips in an undesirable extended position. In both swaddling and with baby carriers, care should be taken to allow for hip abduction and flexion. Caution should also be taken during diaper changes to avoid lifting the legs and thereby causing unnecessary stress to the hips.

CONCLUSION

Developmental dysplasia of the hip can be a disabling pediatric condition. Early diagnosis improves the likelihood of successful treatment during infancy and can prevent serious complications. If untreated, DDH can lead to joint degeneration and premature arthritis. Recognition and treatment within the first six weeks of life is crucial to the overall outcome.

The role of a primary care provider is to identify hip dysplasia risk factors and recognize associated physical exam findings in order to refer to an orthopedic specialist in a timely manner. Guidelines from the AAP, POSNA, and AAOS help direct this process in order to effectively identify infants at risk and in need of treatment.

REFERENCES
1. American Academy of Pediatrics. Committee on Quality Improvement, Subcommittee on Developmental Dysplasia of the Hip. Clinical practice guideline: early detection of developmental dysplasia of the hip. Pediatrics. 2000;105(4 pt 1):896-905.
2. Schwend RM, Schoenecker P, Richards BS, et al. Screening the newborn for developmental dysplasia of the hip: now what do we do? J Pediatr Orthop. 2007;27(6):607-610.
3. Mulpuri K, Song KM, Goldberg MJ, Sevarino K. Detection and nonoperative management of pediatric developmental dysplasia of the hip in infants up to six months of age. J Am Acad Orthop Surg. 2015;23(3):202-205.
4. Thomas SRYW. A review of long-term outcomes for late presenting developmental hip dysplasia. Bone Joint J. 2015;97-B(6):729-733.
5. Loder RT, Skopelja EN. The epidemiology and demographics of hip dysplasia. ISRN Orthop. 2011;2011:238607.
6. US Preventive Services Task Force. Screening for developmental dysplasia of the hip: recommendation statement. Pediatrics. 2006;117(3):898-902.
7. Shorter D, Hong T, Osborn DA. Screening programmes for developmental dysplasia of the hip in newborn infants. Cochrane Database Syst Rev. 2011;(9):CD004595.
8. Loder RT, Shafer C. The demographics of developmental hip dysplasia in the Midwestern United States (Indiana). J Child Orthop. 2015;9(1):93-98.
9. Paton RW, Hinduja K, Thomas CD. The significance of at-risk factors in ultrasound surveillance of developmental dysplasia of the hip: a ten-year prospective study. J Bone Joint Surg Br. 2005;87(9):1264-1266.
10. Alsaleem M, Set KK, Saadeh L. Developmental dysplasia of hip: a review. Clin Pediatr (Phila). 2015;54(10):921-928.
11. Chan A, McCaul KA, Cundy PJ, et al. Perinatal risk factors for developmental dysplasia of the hip. Arch Dis Child. 1997;76(2):F94-F100.
12. Godley DR. Assessment, diagnosis, and treatment of developmental dysplasia of the hip. JAAPA. 2013;26(3):54-58.
13. Van Sleuwen BE, Engelberts AC, Boere-Boonekamp MM, et al. Swaddling: a systematic review. Pediatrics. 2007;120(4):e1097-e1106.
14. American Academy of Orthopaedic Surgeons, American Association of Orthopaedic Surgeons. Position statement: swaddling and developmental hip dysplasia. www.aaos.org/uploadedFiles/PreProduction/About/Opinion_Statements/position/1186%20Swaddling%20and%20Developmental%20Hip%20Dysplasia.pdf. Accessed January 22, 2016.
15. Clarke NM. Swaddling and hip dysplasia: an orthopaedic perspective. Arch Dis Child. 2014;99(1):5-6.
16. Storer SK, Skaggs DL. Developmental dysplasia of the hip. Am Fam Physician. 2006;74(8):1310-1316.
17. McAbee GN, Donn SM, Mendelson RA, et al. Medical diagnoses commonly associated with pediatric malpractice lawsuits in the United States. Pediatrics. 2008;122(6):e1282-e1286.
18. Imrie M, Scott V, Stearns P, et al. Is ultrasound screening for DDH in babies born breech sufficient? J Child Orthop. 2010;4(1):3-8.
19. Chen HW, Chang CH, Tsai ST, et al. Natural progression of hip dysplasia in newborns: a reflection of hip ultrasonographic screenings in newborn nurseries. J Pediatr Orthop B. 2010;19(5):418-423.
20. Gans I, Flynn JM, Sankar WN. Abduction bracing for residual acetabular dysplasia in infantile DDH. J Pediatr Orthop. 2013;33(7):714-718.
21. Murnaghan ML, Browne RH, Sucato DJ, Birch J. Femoral nerve palsy in Pavlik harness treatment for developmental dysplasia of the hip. J Bone Joint Surg Am. 2011;93(5):493-499.
22. Dezateux C, Rosendahl K. Developmental dysplasia of the hip. Lancet. 2007;369(9572):1541-1552.

IN THIS ARTICLE

• Diagnosis
• Management
• Newborn hip evaluation algorithm

Developmental dysplasia of the hip (DDH), previously known as congenital dislocation of the hip, follows a spectrum of irregular anatomic hip development spanning from acetabular dysplasia to irreducible dislocation at birth. Early detection is critical to improve the overall prognosis. Prompt diagnosis requires understanding of potential risk factors, proficiency in physical examination techniques, and implementation of appropriate screening tools when indicated. Although current guidelines direct timing for physical exam screenings, imaging, and treatment, it is ultimately up to the provider to determine the best course of action on a case-by-case basis. This article provides a review of these topics and more.

CURRENT GUIDELINES

In 2000, the American Academy of Pediatrics (AAP) developed guidelines for detection of hip dysplasia, including recommendation of relevant physical exam screenings for all newborns.¹ In 2007, the Pediatric Orthopaedic Society of North America (POSNA) encouraged providers to follow the AAP guidelines with a continued recommendation to perform newborn screening for hip instability and routine follow-up evaluations until the child achieves walking.² The American Academy of Orthopaedic Surgeons (AAOS) also established clinical guidelines in 2014 that are endorsed by both AAP and POSNA.³ These guidelines support routine clinical screening; research evaluated infants up to 6 months old, however, limiting the recommendations to that age-group.

Failure to treat DDH early has been associated with serious negative sequelae that include chronic pain, degenerative arthritis, postural scoliosis, and early gait disturbances.⁴ Primary care providers are expected to perform thorough newborn hip exams with associated specialized tests (ie, Ortolani and Barlow, which are discussed in “Physical exam”) at each routine follow-up. Heightened clinical suspicion and risk factor awareness are key for primary care providers to promptly identify patients requiring orthopedic referral. With early diagnosis, a removable soft abduction brace can be applied as the initial treatment. When treatment is delayed, however, closed reduction under anesthesia or complex surgical intervention may be required.

EPIDEMIOLOGY

The etiology for DDH remains unknown. Hip dysplasia typically presents unilaterally but can also occur bilaterally. DDH is more likely to affect the left hip than the right.⁵

Reported incidence varies, ranging from 0.06 to 76.1 per 1,000 live births, and is largely affected by race and geographic location.⁵ Incidence is higher in countries where routine screening is required, by either physical examination or ultrasound (1.6 to 28.5 and 34.0 to 60.3 per 1,000, respectively), compared with countries not requiring routine screening (1.3 per 1,000). This may suggest that the majority of hip dysplasia cases are transient and resolve spontaneously without treatment.^6,7

RISK FACTORS AND PATIENT HISTORY

Known risk factors for DDH include breech presentation (see Figure 1), positive family history, and female gender.^5,8-10 Female infants are eight times more likely than males to develop DDH.¹⁰ Firstborn status is also recognized as an associated risk factor, which may be attributable to space constraints in utero. This hypothesis is further supported by the relative DDH-protective effect of prematurity and low birth weight. Other potential risk factors include advanced maternal age, birth weight that is high for gestational age, decreased hip abduction, and joint laxity. However, the majority of patients with hip dysplasia have no identifiable risk ­factors.^3,5,9,11,12

Swaddling, which often maintains the hips in an adducted and/or extended position, has also been strongly associated with hip dysplasia.^5,13 Multiple organizations, including the AAOS,AAP, POSNA, and the International Hip Dysplasia Institute, have developed or endorsed hip-healthy swaddling recommendations to minimize the risk for DDH in swaddled infants.^13-15 Such practices allow the infant’s legs to bend up and out at the hips, promoting free hip movement, flexion, and abduction.^13,15 Swaddling has demonstrated multiple benefits (including improved sleep and relief of excessive crying¹³) and continues to be recommended by many US providers; however, those caring for infants at risk for DDH should avoid traditional swaddling and/or practice hip-healthy swaddling techniques.^10,13,14 Early diagnosis starts with the clinician’s knowledge of DDH risk factors and the recommended screening protocols. The presence of multiple risk factors will increase the likelihood of this condition and should lower the clinician’s threshold for ordering additional screening, regardless of hip exam findings.

PHYSICAL EXAM

Both AAP and AAOS guidelines recommend clinical screening for DDH with physical exam in all newborns.^1,3 A head-to-toe musculoskeletal exam is warranted during the initial evaluation of every newborn in order to assess for any known DDH-associated conditions, which may include neuromuscular disorders, torticollis, and metatarsus adductus.⁵

Initial evaluation of an infant with DDH may reveal nonspecific findings, including asymmetric skin folds and limb-length inequality. The Galeazzi sign should be sought by aligning flexed knees with the child in the supine position and assessing for uneven knee heights (see Figure 2). Unilateral posterior hip dislocation or femoral shortening represents a positive Galeazzi sign.¹⁶ Joint laxity and limited hip abduction have also been associated with DDH.^1,10

Barlow and Ortolani exams are more specific to DDH and should be completed at newborn screening and each subsequent well-baby exam.¹ The Barlow maneuver is a provocative test with flexion, adduction, and posterior pressure through the infant’s hip (Figure 3). A palpable clunk during the Barlow maneuver indicates positive instability with posterior displacement. The Ortolani test is a reductive maneuver requiring abduction with posterior pressure to lift the greater trochanter (Figure 4). A clunk sensation with this test is positive for reduction of the hip.

The infant’s diaper should be removed during the hip evaluation. These exams are more reliable when each hip is evaluated separately with the pelvis stabilized.¹⁰ All physical exam findings must be carefully documented at each encounter.^1,17

It is critical for the examiner to understand the appropriate technique and potential results when conducting each of these specialized hip exams. A true positive finding is the clunking sensation that occurs with the dislocation or relocation of the affected hip; this finding is better felt than heard. In contrast, a benign hip click with these maneuvers is a more subtle sensation—typically, a soft-tissue snapping or catching—and is not diagnostic of DDH. A click is not a clunk and is not indicative of DDH.^1,3

DDH may present later in infancy or early childhood; therefore, DDH should remain within the differential diagnosis for gait asymmetry, unequal hip motion, or limb-length discrepancy. It may be beneficial to continue to evaluate for these developments during routine exams as part of a thorough pediatric musculoskeletal assessment, particularly in patients with documented risk factors for DDH.^1,3,4 Delay in diagnosis of DDH, it should be noted, is a relatively common complaint in pediatric medical malpractice lawsuits; until the early 2000s, this condition represented about 75% of claims in one medical malpractice database.The decrease in claims has been attributed to better awareness and earlier diagnosis of DDH. ¹⁷

Continue for the diagnosis >>

DIAGNOSIS

A positive Ortolani or Barlow sign is diagnostic and warrants prompt orthopedic referral (Figure 5). If physical examination results are equivocal or inconclusive, follow-up at two weeks is recommended, with continued routine follow-up until walking is achieved. Patients with persistent equivocal findings at the two-week follow-up warrant ultrasound at age 3 to 4 weeks or orthopedic referral. Infants with significant risk factors, particularly breech presentation at birth, should also undergo imaging.¹⁸ AAP recommends ultrasound at age 6 weeks or radiograph after 4 months of age.^1,18 AAOS recommends performing an imaging study before age 6 months when at least one of the following risk factors is present: breech presentation, positive family history of DDH, or previous clinical instability (moderate level of evidence).³

IMAGING

Ultrasound is the diagnostic test of choice for infants because radiographs have limited value until the femoral heads begin to ossify at age 4 to 6 months.¹⁸ Ultrasonography allows for visualization of the cartilaginous portion of the acetabulum and femoral head.¹ Dynamic stressing is performed during ultrasound to assess the level of hip stability. A provider trained in ultrasound will measure the depth of the acetabulum and identify any potential laxity or instability of the hip joint. Accuracy of these findings is largely dependent on the experience and skill of the examiner.

Ultrasound evaluation is not recommended until after age 3 to 4 weeks. Earlier findings may include mild laxity and immature morphology of the acetabulum, which often resolve spontaneously.^1,18 Use of ultrasound is currently recommended only to confirm diagnostic suspicion, based on clinical findings, or for infants with significant risk factors.¹⁸ Universal ultrasound screening in newborns is not recommended and would incur unnecessary costs.^1,3,9 Plain radiographs are used after age 4 months to confirm a diagnosis of DDH or to assess for residual dysplasia.^3,18

Continue for management >>

MANAGEMENT

Once hip dysplasia is suggested by physical exam or imaging study, the child’s subsequent care should be provided by an orthopedic specialist with experience in treating this condition. Treatment is preferably initiated before age 6 weeks.¹² The specifics of treatment are largely based on age at diagnosis and the severity of dysplasia.

The goal of treatment is to maintain the hips in a stable position with the femoral head well covered by the acetabulum. This will improve anatomic development and function. Early clinical diagnosis is often sufficient to justify initiating conservative treatment; additionally, early detection of DDH can considerably reduce the need for surgical intervention.¹² Although the potential for spontaneous resolution is high, the consequences associated with delay in care can be significant.

Preferred initial management, which can be initiated before confirmation of DDH by ultrasound, involves implementation of soft abduction support.¹⁹ The Pavlik harness is the support design of choice (Figure 6).¹² This harness maintains hip flexion and abduction, creating concentric reduction of the femoral head. The brace is highly successful when its use is initiated early. Treatment in a Pavlik harness requires nearly full-time wear and close monitoring by a clinician. Unlikely potential risks associated with this treatment include avascular necrosis and femoral nerve palsy.⁴

Ultrasonography is used to further monitor treatment and to determine length of wear. Long-term results suggest a success rate exceeding 90%.^20,21 However, this rate may be falsely elevated due to the number of hips that likely would have improved spontaneously without treatment.^6,19

The Pavlik harness becomes less effective with increasing age, and a more rigid abduction brace may be considered in infants older than 6 months.²⁰ Overall outcomes improve once the femoral head is consistently maintained in the acetabulum. Delay in treatment is associated with an increase in the long-term complications associated with residual hip dysplasia.²²

Once an infant is undergoing treatment for DDH in a Pavlik harness, there is no need for primary care providers to continue to perform provocative testing, such as the Ortolani or Barlow test, at routine well-baby checks. Unnecessary stress to the hips is not beneficial, and any new results will not change the treatment being provided by the orthopedic specialist. Adjustments to the fit of the harness should be made only by the orthopedist, unless femoral nerve palsy is noted on exam. This development warrants immediate discontinuation of harness use until symptoms resolve.²¹

Abduction bracing may not be suitable for all cases of hip dysplasia. Newborns with irreducible hips, more advanced dysplasia, or associated neuromuscular or syndromic disorder may require closed versus open reduction and casting. More invasive surgical options may also be considered in advanced dysplasia in order to reshape the joint and improve function.^20,22

Continue for patient education >>

PATIENT EDUCATION

Parents should be fully educated on the options for managing hip dysplasia. Once DDH is diagnosed, prompt referral to an orthopedic specialist is critical in order to weigh the treatment options and to develop the appropriate individualized plan for each child. Once treatment is initiated, parental compliance is essential; frequent meetings between parents and the specialist are important.

Parents of infants with known risk factors for and/or suspicion of hip dysplasia should also be educated on hip-healthy swaddling to allow for free motion of the hips and knees.^10,13 Advise them that some commercial baby carriers and slings may maintain the hips in an undesirable extended position. In both swaddling and with baby carriers, care should be taken to allow for hip abduction and flexion. Caution should also be taken during diaper changes to avoid lifting the legs and thereby causing unnecessary stress to the hips.

CONCLUSION

Developmental dysplasia of the hip can be a disabling pediatric condition. Early diagnosis improves the likelihood of successful treatment during infancy and can prevent serious complications. If untreated, DDH can lead to joint degeneration and premature arthritis. Recognition and treatment within the first six weeks of life is crucial to the overall outcome.

The role of a primary care provider is to identify hip dysplasia risk factors and recognize associated physical exam findings in order to refer to an orthopedic specialist in a timely manner. Guidelines from the AAP, POSNA, and AAOS help direct this process in order to effectively identify infants at risk and in need of treatment.

REFERENCES
1. American Academy of Pediatrics. Committee on Quality Improvement, Subcommittee on Developmental Dysplasia of the Hip. Clinical practice guideline: early detection of developmental dysplasia of the hip. Pediatrics. 2000;105(4 pt 1):896-905.
2. Schwend RM, Schoenecker P, Richards BS, et al. Screening the newborn for developmental dysplasia of the hip: now what do we do? J Pediatr Orthop. 2007;27(6):607-610.
3. Mulpuri K, Song KM, Goldberg MJ, Sevarino K. Detection and nonoperative management of pediatric developmental dysplasia of the hip in infants up to six months of age. J Am Acad Orthop Surg. 2015;23(3):202-205.
4. Thomas SRYW. A review of long-term outcomes for late presenting developmental hip dysplasia. Bone Joint J. 2015;97-B(6):729-733.
5. Loder RT, Skopelja EN. The epidemiology and demographics of hip dysplasia. ISRN Orthop. 2011;2011:238607.
6. US Preventive Services Task Force. Screening for developmental dysplasia of the hip: recommendation statement. Pediatrics. 2006;117(3):898-902.
7. Shorter D, Hong T, Osborn DA. Screening programmes for developmental dysplasia of the hip in newborn infants. Cochrane Database Syst Rev. 2011;(9):CD004595.
8. Loder RT, Shafer C. The demographics of developmental hip dysplasia in the Midwestern United States (Indiana). J Child Orthop. 2015;9(1):93-98.
9. Paton RW, Hinduja K, Thomas CD. The significance of at-risk factors in ultrasound surveillance of developmental dysplasia of the hip: a ten-year prospective study. J Bone Joint Surg Br. 2005;87(9):1264-1266.
10. Alsaleem M, Set KK, Saadeh L. Developmental dysplasia of hip: a review. Clin Pediatr (Phila). 2015;54(10):921-928.
11. Chan A, McCaul KA, Cundy PJ, et al. Perinatal risk factors for developmental dysplasia of the hip. Arch Dis Child. 1997;76(2):F94-F100.
12. Godley DR. Assessment, diagnosis, and treatment of developmental dysplasia of the hip. JAAPA. 2013;26(3):54-58.
13. Van Sleuwen BE, Engelberts AC, Boere-Boonekamp MM, et al. Swaddling: a systematic review. Pediatrics. 2007;120(4):e1097-e1106.
14. American Academy of Orthopaedic Surgeons, American Association of Orthopaedic Surgeons. Position statement: swaddling and developmental hip dysplasia. www.aaos.org/uploadedFiles/PreProduction/About/Opinion_Statements/position/1186%20Swaddling%20and%20Developmental%20Hip%20Dysplasia.pdf. Accessed January 22, 2016.
15. Clarke NM. Swaddling and hip dysplasia: an orthopaedic perspective. Arch Dis Child. 2014;99(1):5-6.
16. Storer SK, Skaggs DL. Developmental dysplasia of the hip. Am Fam Physician. 2006;74(8):1310-1316.
17. McAbee GN, Donn SM, Mendelson RA, et al. Medical diagnoses commonly associated with pediatric malpractice lawsuits in the United States. Pediatrics. 2008;122(6):e1282-e1286.
18. Imrie M, Scott V, Stearns P, et al. Is ultrasound screening for DDH in babies born breech sufficient? J Child Orthop. 2010;4(1):3-8.
19. Chen HW, Chang CH, Tsai ST, et al. Natural progression of hip dysplasia in newborns: a reflection of hip ultrasonographic screenings in newborn nurseries. J Pediatr Orthop B. 2010;19(5):418-423.
20. Gans I, Flynn JM, Sankar WN. Abduction bracing for residual acetabular dysplasia in infantile DDH. J Pediatr Orthop. 2013;33(7):714-718.
21. Murnaghan ML, Browne RH, Sucato DJ, Birch J. Femoral nerve palsy in Pavlik harness treatment for developmental dysplasia of the hip. J Bone Joint Surg Am. 2011;93(5):493-499.
22. Dezateux C, Rosendahl K. Developmental dysplasia of the hip. Lancet. 2007;369(9572):1541-1552.

IN THIS ARTICLE

• Diagnosis
• Management
• Newborn hip evaluation algorithm

Developmental dysplasia of the hip (DDH), previously known as congenital dislocation of the hip, follows a spectrum of irregular anatomic hip development spanning from acetabular dysplasia to irreducible dislocation at birth. Early detection is critical to improve the overall prognosis. Prompt diagnosis requires understanding of potential risk factors, proficiency in physical examination techniques, and implementation of appropriate screening tools when indicated. Although current guidelines direct timing for physical exam screenings, imaging, and treatment, it is ultimately up to the provider to determine the best course of action on a case-by-case basis. This article provides a review of these topics and more.

CURRENT GUIDELINES

In 2000, the American Academy of Pediatrics (AAP) developed guidelines for detection of hip dysplasia, including recommendation of relevant physical exam screenings for all newborns.¹ In 2007, the Pediatric Orthopaedic Society of North America (POSNA) encouraged providers to follow the AAP guidelines with a continued recommendation to perform newborn screening for hip instability and routine follow-up evaluations until the child achieves walking.² The American Academy of Orthopaedic Surgeons (AAOS) also established clinical guidelines in 2014 that are endorsed by both AAP and POSNA.³ These guidelines support routine clinical screening; research evaluated infants up to 6 months old, however, limiting the recommendations to that age-group.

Failure to treat DDH early has been associated with serious negative sequelae that include chronic pain, degenerative arthritis, postural scoliosis, and early gait disturbances.⁴ Primary care providers are expected to perform thorough newborn hip exams with associated specialized tests (ie, Ortolani and Barlow, which are discussed in “Physical exam”) at each routine follow-up. Heightened clinical suspicion and risk factor awareness are key for primary care providers to promptly identify patients requiring orthopedic referral. With early diagnosis, a removable soft abduction brace can be applied as the initial treatment. When treatment is delayed, however, closed reduction under anesthesia or complex surgical intervention may be required.

EPIDEMIOLOGY

The etiology for DDH remains unknown. Hip dysplasia typically presents unilaterally but can also occur bilaterally. DDH is more likely to affect the left hip than the right.⁵

Reported incidence varies, ranging from 0.06 to 76.1 per 1,000 live births, and is largely affected by race and geographic location.⁵ Incidence is higher in countries where routine screening is required, by either physical examination or ultrasound (1.6 to 28.5 and 34.0 to 60.3 per 1,000, respectively), compared with countries not requiring routine screening (1.3 per 1,000). This may suggest that the majority of hip dysplasia cases are transient and resolve spontaneously without treatment.^6,7

RISK FACTORS AND PATIENT HISTORY

Known risk factors for DDH include breech presentation (see Figure 1), positive family history, and female gender.^5,8-10 Female infants are eight times more likely than males to develop DDH.¹⁰ Firstborn status is also recognized as an associated risk factor, which may be attributable to space constraints in utero. This hypothesis is further supported by the relative DDH-protective effect of prematurity and low birth weight. Other potential risk factors include advanced maternal age, birth weight that is high for gestational age, decreased hip abduction, and joint laxity. However, the majority of patients with hip dysplasia have no identifiable risk ­factors.^3,5,9,11,12

Swaddling, which often maintains the hips in an adducted and/or extended position, has also been strongly associated with hip dysplasia.^5,13 Multiple organizations, including the AAOS,AAP, POSNA, and the International Hip Dysplasia Institute, have developed or endorsed hip-healthy swaddling recommendations to minimize the risk for DDH in swaddled infants.^13-15 Such practices allow the infant’s legs to bend up and out at the hips, promoting free hip movement, flexion, and abduction.^13,15 Swaddling has demonstrated multiple benefits (including improved sleep and relief of excessive crying¹³) and continues to be recommended by many US providers; however, those caring for infants at risk for DDH should avoid traditional swaddling and/or practice hip-healthy swaddling techniques.^10,13,14 Early diagnosis starts with the clinician’s knowledge of DDH risk factors and the recommended screening protocols. The presence of multiple risk factors will increase the likelihood of this condition and should lower the clinician’s threshold for ordering additional screening, regardless of hip exam findings.

PHYSICAL EXAM

Both AAP and AAOS guidelines recommend clinical screening for DDH with physical exam in all newborns.^1,3 A head-to-toe musculoskeletal exam is warranted during the initial evaluation of every newborn in order to assess for any known DDH-associated conditions, which may include neuromuscular disorders, torticollis, and metatarsus adductus.⁵

Initial evaluation of an infant with DDH may reveal nonspecific findings, including asymmetric skin folds and limb-length inequality. The Galeazzi sign should be sought by aligning flexed knees with the child in the supine position and assessing for uneven knee heights (see Figure 2). Unilateral posterior hip dislocation or femoral shortening represents a positive Galeazzi sign.¹⁶ Joint laxity and limited hip abduction have also been associated with DDH.^1,10

Barlow and Ortolani exams are more specific to DDH and should be completed at newborn screening and each subsequent well-baby exam.¹ The Barlow maneuver is a provocative test with flexion, adduction, and posterior pressure through the infant’s hip (Figure 3). A palpable clunk during the Barlow maneuver indicates positive instability with posterior displacement. The Ortolani test is a reductive maneuver requiring abduction with posterior pressure to lift the greater trochanter (Figure 4). A clunk sensation with this test is positive for reduction of the hip.

The infant’s diaper should be removed during the hip evaluation. These exams are more reliable when each hip is evaluated separately with the pelvis stabilized.¹⁰ All physical exam findings must be carefully documented at each encounter.^1,17

It is critical for the examiner to understand the appropriate technique and potential results when conducting each of these specialized hip exams. A true positive finding is the clunking sensation that occurs with the dislocation or relocation of the affected hip; this finding is better felt than heard. In contrast, a benign hip click with these maneuvers is a more subtle sensation—typically, a soft-tissue snapping or catching—and is not diagnostic of DDH. A click is not a clunk and is not indicative of DDH.^1,3

DDH may present later in infancy or early childhood; therefore, DDH should remain within the differential diagnosis for gait asymmetry, unequal hip motion, or limb-length discrepancy. It may be beneficial to continue to evaluate for these developments during routine exams as part of a thorough pediatric musculoskeletal assessment, particularly in patients with documented risk factors for DDH.^1,3,4 Delay in diagnosis of DDH, it should be noted, is a relatively common complaint in pediatric medical malpractice lawsuits; until the early 2000s, this condition represented about 75% of claims in one medical malpractice database.The decrease in claims has been attributed to better awareness and earlier diagnosis of DDH. ¹⁷

Continue for the diagnosis >>

DIAGNOSIS

A positive Ortolani or Barlow sign is diagnostic and warrants prompt orthopedic referral (Figure 5). If physical examination results are equivocal or inconclusive, follow-up at two weeks is recommended, with continued routine follow-up until walking is achieved. Patients with persistent equivocal findings at the two-week follow-up warrant ultrasound at age 3 to 4 weeks or orthopedic referral. Infants with significant risk factors, particularly breech presentation at birth, should also undergo imaging.¹⁸ AAP recommends ultrasound at age 6 weeks or radiograph after 4 months of age.^1,18 AAOS recommends performing an imaging study before age 6 months when at least one of the following risk factors is present: breech presentation, positive family history of DDH, or previous clinical instability (moderate level of evidence).³

IMAGING

Ultrasound is the diagnostic test of choice for infants because radiographs have limited value until the femoral heads begin to ossify at age 4 to 6 months.¹⁸ Ultrasonography allows for visualization of the cartilaginous portion of the acetabulum and femoral head.¹ Dynamic stressing is performed during ultrasound to assess the level of hip stability. A provider trained in ultrasound will measure the depth of the acetabulum and identify any potential laxity or instability of the hip joint. Accuracy of these findings is largely dependent on the experience and skill of the examiner.

Ultrasound evaluation is not recommended until after age 3 to 4 weeks. Earlier findings may include mild laxity and immature morphology of the acetabulum, which often resolve spontaneously.^1,18 Use of ultrasound is currently recommended only to confirm diagnostic suspicion, based on clinical findings, or for infants with significant risk factors.¹⁸ Universal ultrasound screening in newborns is not recommended and would incur unnecessary costs.^1,3,9 Plain radiographs are used after age 4 months to confirm a diagnosis of DDH or to assess for residual dysplasia.^3,18

Continue for management >>

MANAGEMENT

Once hip dysplasia is suggested by physical exam or imaging study, the child’s subsequent care should be provided by an orthopedic specialist with experience in treating this condition. Treatment is preferably initiated before age 6 weeks.¹² The specifics of treatment are largely based on age at diagnosis and the severity of dysplasia.

The goal of treatment is to maintain the hips in a stable position with the femoral head well covered by the acetabulum. This will improve anatomic development and function. Early clinical diagnosis is often sufficient to justify initiating conservative treatment; additionally, early detection of DDH can considerably reduce the need for surgical intervention.¹² Although the potential for spontaneous resolution is high, the consequences associated with delay in care can be significant.

Preferred initial management, which can be initiated before confirmation of DDH by ultrasound, involves implementation of soft abduction support.¹⁹ The Pavlik harness is the support design of choice (Figure 6).¹² This harness maintains hip flexion and abduction, creating concentric reduction of the femoral head. The brace is highly successful when its use is initiated early. Treatment in a Pavlik harness requires nearly full-time wear and close monitoring by a clinician. Unlikely potential risks associated with this treatment include avascular necrosis and femoral nerve palsy.⁴

Ultrasonography is used to further monitor treatment and to determine length of wear. Long-term results suggest a success rate exceeding 90%.^20,21 However, this rate may be falsely elevated due to the number of hips that likely would have improved spontaneously without treatment.^6,19

The Pavlik harness becomes less effective with increasing age, and a more rigid abduction brace may be considered in infants older than 6 months.²⁰ Overall outcomes improve once the femoral head is consistently maintained in the acetabulum. Delay in treatment is associated with an increase in the long-term complications associated with residual hip dysplasia.²²

Once an infant is undergoing treatment for DDH in a Pavlik harness, there is no need for primary care providers to continue to perform provocative testing, such as the Ortolani or Barlow test, at routine well-baby checks. Unnecessary stress to the hips is not beneficial, and any new results will not change the treatment being provided by the orthopedic specialist. Adjustments to the fit of the harness should be made only by the orthopedist, unless femoral nerve palsy is noted on exam. This development warrants immediate discontinuation of harness use until symptoms resolve.²¹

Abduction bracing may not be suitable for all cases of hip dysplasia. Newborns with irreducible hips, more advanced dysplasia, or associated neuromuscular or syndromic disorder may require closed versus open reduction and casting. More invasive surgical options may also be considered in advanced dysplasia in order to reshape the joint and improve function.^20,22

Continue for patient education >>

PATIENT EDUCATION

Parents should be fully educated on the options for managing hip dysplasia. Once DDH is diagnosed, prompt referral to an orthopedic specialist is critical in order to weigh the treatment options and to develop the appropriate individualized plan for each child. Once treatment is initiated, parental compliance is essential; frequent meetings between parents and the specialist are important.

Parents of infants with known risk factors for and/or suspicion of hip dysplasia should also be educated on hip-healthy swaddling to allow for free motion of the hips and knees.^10,13 Advise them that some commercial baby carriers and slings may maintain the hips in an undesirable extended position. In both swaddling and with baby carriers, care should be taken to allow for hip abduction and flexion. Caution should also be taken during diaper changes to avoid lifting the legs and thereby causing unnecessary stress to the hips.

CONCLUSION

Developmental dysplasia of the hip can be a disabling pediatric condition. Early diagnosis improves the likelihood of successful treatment during infancy and can prevent serious complications. If untreated, DDH can lead to joint degeneration and premature arthritis. Recognition and treatment within the first six weeks of life is crucial to the overall outcome.

The role of a primary care provider is to identify hip dysplasia risk factors and recognize associated physical exam findings in order to refer to an orthopedic specialist in a timely manner. Guidelines from the AAP, POSNA, and AAOS help direct this process in order to effectively identify infants at risk and in need of treatment.

REFERENCES
1. American Academy of Pediatrics. Committee on Quality Improvement, Subcommittee on Developmental Dysplasia of the Hip. Clinical practice guideline: early detection of developmental dysplasia of the hip. Pediatrics. 2000;105(4 pt 1):896-905.
2. Schwend RM, Schoenecker P, Richards BS, et al. Screening the newborn for developmental dysplasia of the hip: now what do we do? J Pediatr Orthop. 2007;27(6):607-610.
3. Mulpuri K, Song KM, Goldberg MJ, Sevarino K. Detection and nonoperative management of pediatric developmental dysplasia of the hip in infants up to six months of age. J Am Acad Orthop Surg. 2015;23(3):202-205.
4. Thomas SRYW. A review of long-term outcomes for late presenting developmental hip dysplasia. Bone Joint J. 2015;97-B(6):729-733.
5. Loder RT, Skopelja EN. The epidemiology and demographics of hip dysplasia. ISRN Orthop. 2011;2011:238607.
6. US Preventive Services Task Force. Screening for developmental dysplasia of the hip: recommendation statement. Pediatrics. 2006;117(3):898-902.
7. Shorter D, Hong T, Osborn DA. Screening programmes for developmental dysplasia of the hip in newborn infants. Cochrane Database Syst Rev. 2011;(9):CD004595.
8. Loder RT, Shafer C. The demographics of developmental hip dysplasia in the Midwestern United States (Indiana). J Child Orthop. 2015;9(1):93-98.
9. Paton RW, Hinduja K, Thomas CD. The significance of at-risk factors in ultrasound surveillance of developmental dysplasia of the hip: a ten-year prospective study. J Bone Joint Surg Br. 2005;87(9):1264-1266.
10. Alsaleem M, Set KK, Saadeh L. Developmental dysplasia of hip: a review. Clin Pediatr (Phila). 2015;54(10):921-928.
11. Chan A, McCaul KA, Cundy PJ, et al. Perinatal risk factors for developmental dysplasia of the hip. Arch Dis Child. 1997;76(2):F94-F100.
12. Godley DR. Assessment, diagnosis, and treatment of developmental dysplasia of the hip. JAAPA. 2013;26(3):54-58.
13. Van Sleuwen BE, Engelberts AC, Boere-Boonekamp MM, et al. Swaddling: a systematic review. Pediatrics. 2007;120(4):e1097-e1106.
14. American Academy of Orthopaedic Surgeons, American Association of Orthopaedic Surgeons. Position statement: swaddling and developmental hip dysplasia. www.aaos.org/uploadedFiles/PreProduction/About/Opinion_Statements/position/1186%20Swaddling%20and%20Developmental%20Hip%20Dysplasia.pdf. Accessed January 22, 2016.
15. Clarke NM. Swaddling and hip dysplasia: an orthopaedic perspective. Arch Dis Child. 2014;99(1):5-6.
16. Storer SK, Skaggs DL. Developmental dysplasia of the hip. Am Fam Physician. 2006;74(8):1310-1316.
17. McAbee GN, Donn SM, Mendelson RA, et al. Medical diagnoses commonly associated with pediatric malpractice lawsuits in the United States. Pediatrics. 2008;122(6):e1282-e1286.
18. Imrie M, Scott V, Stearns P, et al. Is ultrasound screening for DDH in babies born breech sufficient? J Child Orthop. 2010;4(1):3-8.
19. Chen HW, Chang CH, Tsai ST, et al. Natural progression of hip dysplasia in newborns: a reflection of hip ultrasonographic screenings in newborn nurseries. J Pediatr Orthop B. 2010;19(5):418-423.
20. Gans I, Flynn JM, Sankar WN. Abduction bracing for residual acetabular dysplasia in infantile DDH. J Pediatr Orthop. 2013;33(7):714-718.
21. Murnaghan ML, Browne RH, Sucato DJ, Birch J. Femoral nerve palsy in Pavlik harness treatment for developmental dysplasia of the hip. J Bone Joint Surg Am. 2011;93(5):493-499.
22. Dezateux C, Rosendahl K. Developmental dysplasia of the hip. Lancet. 2007;369(9572):1541-1552.

Introduction

Often, it can be difficult for providers to fully understand why children may be presenting with behavioral problems that appear to be occurring principally in specific environments. Parents may bring their children for an evaluation based upon reports they are receiving from teachers and other school personnel who may be observing more prominent oppositionality, social struggles, troubles following instructions, and frustration intolerance than the parents are experiencing in the home. Additionally, youth may begin to display school refusal and voice strong negative feelings about school. Although at face value, these problems may indicate potential diagnoses like oppositional defiant disorder and attention-deficit/hyperactivity disorder, one also should consider other underlying “non–mental health” issues that could greatly influence school success and present with a range of emotional and behavioral struggles.

Case Summary

Brynn is an animated, social, and strong-willed 4 year-old girl who experienced delays in her receptive and expressive language that prompted her engagement in early intervention around 18 months of age. She and her family continued to receive developmental services over the next few years and, at the age of 3 years, because of ongoing speech and language challenges, she was enrolled in a preschool individualized education program. In her program, Brynn participates in an array of specialized instruction, but her educators comment that she is not making expected academic progress and has troubles “holding onto information,” focusing, and participating meaningfully with peers in a consistent manner.

At times, Brynn can be impulsive, aggressive, and volatile – behavioral traits that mom denies are occurring in the home. A diagnosis of an autism spectrum disorder has been ruled out, and Brynn’s hearing and vision are tested to be normal. Brynn’s mother feels confused by the reports she’s getting from school; “I want Brynn to be happy,” she shares. “I needed special help to read in high school, and I worry about her future.”

Discussion

Learning disorders (or learning disabilities, using educational terminology) are defined as neurologically rooted problems that affect academic achievement via the receiving, processing, or communication of information. They occur in 1 in 10 children (Pediatrics. 2007 Feb;119 Suppl 1:S77-83) and can present with specific problems in reading, writing, and mathematics while having considerable influence on related aptitudes in language, social ability, self-help, and motor functioning. Dyslexia – a developmental reading disorder – is the most common type of learning disorder (LD). Although it’s not clear what causes learning disorders, there are several factors that are thought to play a role in their development, including hereditary factors and problems with pregnancy and birth. Having developmental delays also can place children at risk for having later learning problems that may not be identifiable until a child enters a more structured learning environment. At clinical visits during the preschool years, particularly with a child who may have had earlier developmental concerns, the pediatrician should inquire about a range of “warning signs” that may indicate a need for additional screening and evaluation for specific learning issues.

The National Center for Learning Disabilities offers a range of practical tips for pediatricians who may want to further explore parental and teacher concerns by asking questions related to literacy, writing, language, and social-emotional skills, attention, and gross and fine motor movements in a developmentally informed manner. Further exploring Brynn’s mother’s comments in the context of her daughter not progressing in school revealed a history of difficulty retaining new words, troubles learning colors and shapes, challenges remembering rules, and particular difficulties engaging in group play with other 4-year-olds – all potential signals for a learning disorder. This alerted educators that she may indeed be struggling with issues beyond that of an enduring speech-language delay.

With the suspicion that Brynn was presenting with signs and symptoms suggestive of a learning disorder, her family was educated about the Individuals with Disabilities Education Act, and it was recommended she receive a comprehensive psychoeducational evaluation to further assess her intellectual profile, academic achievement, social functioning, and performance in the classroom using standardized tools. These tools, among other objectives, can help the child’s team offer a more definitive LD diagnosis while informing the potential development of special education supports and assessing for an intellectual disability. The DSM 5 indicates that LD diagnostic criteria are to be met based upon a clinical synthesis of history, school reports, and psychoeducational assessment.

©Bigandt_Photography/Thinkstock

It’s been long established that children with learning problems frequently have co-occurring emotional and behavioral troubles (Arch Dis Child. 1974 Apr; 49[4]:249-56), many of which also should be considered as differential diagnoses in a child with school problems – as such, a complicated interplay of learning disorders and internalizing and externalizing conditions is often appreciated in school-age children with academic difficulties. At times, learning disorders can lead to emotional distress and could also be misdiagnosed as primary emotional and behavioral challenges. Specifically, children with learning problems are at risk for struggling with low self-esteem, loneliness, and anxiety, which also can be associated with mood disorders, school dropout, victimization, and engaging in substance use.

In Brynn’s case, the results of the Teacher Report Forms and Child Behavior Checklists were reviewed, revealing some evidence that she was experiencing clinical-level problems with her attention, but a discrepancy was noted between the teacher and parent report (the teachers endorsing more clinically significant symptoms). Although co-occurring attention-deficit/hyperactivity disorder (ADHD) is not uncommon in children with a learning disorder (Pediatrics. 2011 Mar;127[3]:462-70), we did not feel Brynn met criteria for this. We elected not to provide an ADHD diagnosis but are mindful that her attentional concerns should be closely monitored over time; a diagnosis may be more relevant in the future, perhaps influencing Brynn’s eligibility for services and treatment planning. Furthermore, comorbidity with ADHD is predictive of worse mental health outcomes, compared with learning disabilities presenting without ADHD.

Clinical Pearl

Pediatricians should consider the possibility of a child having a learning disorder in youth who display risk factors (family history of learning concerns, atypical development, prematurity, etc.) and have problems at school. Such problems may be presenting with emotional and behavioral symptoms that could mask a child’s primary learning impairments. Learning disorders also frequently co-occur with psychiatric conditions, but engaging children in effective interventions (school-based supports) could potentially attenuate the risk for the development of mental health problems. Also, promoting emotional wellness and fostering self-worth may improve the academic performance of children with learning disorders.

Dr. Dickerson, a child and adolescent psychiatrist, is an assistant professor of psychiatry at the University of Vermont, Burlington. He is the director of the university’s autism diagnostic clinic. Dr. Dickerson said he had no relevant financial disclosures. Contact Dr. Dickerson at pdnews@frontlinemedcom.com.

Introduction

Often, it can be difficult for providers to fully understand why children may be presenting with behavioral problems that appear to be occurring principally in specific environments. Parents may bring their children for an evaluation based upon reports they are receiving from teachers and other school personnel who may be observing more prominent oppositionality, social struggles, troubles following instructions, and frustration intolerance than the parents are experiencing in the home. Additionally, youth may begin to display school refusal and voice strong negative feelings about school. Although at face value, these problems may indicate potential diagnoses like oppositional defiant disorder and attention-deficit/hyperactivity disorder, one also should consider other underlying “non–mental health” issues that could greatly influence school success and present with a range of emotional and behavioral struggles.

Case Summary

Brynn is an animated, social, and strong-willed 4 year-old girl who experienced delays in her receptive and expressive language that prompted her engagement in early intervention around 18 months of age. She and her family continued to receive developmental services over the next few years and, at the age of 3 years, because of ongoing speech and language challenges, she was enrolled in a preschool individualized education program. In her program, Brynn participates in an array of specialized instruction, but her educators comment that she is not making expected academic progress and has troubles “holding onto information,” focusing, and participating meaningfully with peers in a consistent manner.

At times, Brynn can be impulsive, aggressive, and volatile – behavioral traits that mom denies are occurring in the home. A diagnosis of an autism spectrum disorder has been ruled out, and Brynn’s hearing and vision are tested to be normal. Brynn’s mother feels confused by the reports she’s getting from school; “I want Brynn to be happy,” she shares. “I needed special help to read in high school, and I worry about her future.”

Discussion

Learning disorders (or learning disabilities, using educational terminology) are defined as neurologically rooted problems that affect academic achievement via the receiving, processing, or communication of information. They occur in 1 in 10 children (Pediatrics. 2007 Feb;119 Suppl 1:S77-83) and can present with specific problems in reading, writing, and mathematics while having considerable influence on related aptitudes in language, social ability, self-help, and motor functioning. Dyslexia – a developmental reading disorder – is the most common type of learning disorder (LD). Although it’s not clear what causes learning disorders, there are several factors that are thought to play a role in their development, including hereditary factors and problems with pregnancy and birth. Having developmental delays also can place children at risk for having later learning problems that may not be identifiable until a child enters a more structured learning environment. At clinical visits during the preschool years, particularly with a child who may have had earlier developmental concerns, the pediatrician should inquire about a range of “warning signs” that may indicate a need for additional screening and evaluation for specific learning issues.

The National Center for Learning Disabilities offers a range of practical tips for pediatricians who may want to further explore parental and teacher concerns by asking questions related to literacy, writing, language, and social-emotional skills, attention, and gross and fine motor movements in a developmentally informed manner. Further exploring Brynn’s mother’s comments in the context of her daughter not progressing in school revealed a history of difficulty retaining new words, troubles learning colors and shapes, challenges remembering rules, and particular difficulties engaging in group play with other 4-year-olds – all potential signals for a learning disorder. This alerted educators that she may indeed be struggling with issues beyond that of an enduring speech-language delay.

With the suspicion that Brynn was presenting with signs and symptoms suggestive of a learning disorder, her family was educated about the Individuals with Disabilities Education Act, and it was recommended she receive a comprehensive psychoeducational evaluation to further assess her intellectual profile, academic achievement, social functioning, and performance in the classroom using standardized tools. These tools, among other objectives, can help the child’s team offer a more definitive LD diagnosis while informing the potential development of special education supports and assessing for an intellectual disability. The DSM 5 indicates that LD diagnostic criteria are to be met based upon a clinical synthesis of history, school reports, and psychoeducational assessment.

©Bigandt_Photography/Thinkstock

It’s been long established that children with learning problems frequently have co-occurring emotional and behavioral troubles (Arch Dis Child. 1974 Apr; 49[4]:249-56), many of which also should be considered as differential diagnoses in a child with school problems – as such, a complicated interplay of learning disorders and internalizing and externalizing conditions is often appreciated in school-age children with academic difficulties. At times, learning disorders can lead to emotional distress and could also be misdiagnosed as primary emotional and behavioral challenges. Specifically, children with learning problems are at risk for struggling with low self-esteem, loneliness, and anxiety, which also can be associated with mood disorders, school dropout, victimization, and engaging in substance use.

In Brynn’s case, the results of the Teacher Report Forms and Child Behavior Checklists were reviewed, revealing some evidence that she was experiencing clinical-level problems with her attention, but a discrepancy was noted between the teacher and parent report (the teachers endorsing more clinically significant symptoms). Although co-occurring attention-deficit/hyperactivity disorder (ADHD) is not uncommon in children with a learning disorder (Pediatrics. 2011 Mar;127[3]:462-70), we did not feel Brynn met criteria for this. We elected not to provide an ADHD diagnosis but are mindful that her attentional concerns should be closely monitored over time; a diagnosis may be more relevant in the future, perhaps influencing Brynn’s eligibility for services and treatment planning. Furthermore, comorbidity with ADHD is predictive of worse mental health outcomes, compared with learning disabilities presenting without ADHD.

Clinical Pearl

Pediatricians should consider the possibility of a child having a learning disorder in youth who display risk factors (family history of learning concerns, atypical development, prematurity, etc.) and have problems at school. Such problems may be presenting with emotional and behavioral symptoms that could mask a child’s primary learning impairments. Learning disorders also frequently co-occur with psychiatric conditions, but engaging children in effective interventions (school-based supports) could potentially attenuate the risk for the development of mental health problems. Also, promoting emotional wellness and fostering self-worth may improve the academic performance of children with learning disorders.

Dr. Dickerson, a child and adolescent psychiatrist, is an assistant professor of psychiatry at the University of Vermont, Burlington. He is the director of the university’s autism diagnostic clinic. Dr. Dickerson said he had no relevant financial disclosures. Contact Dr. Dickerson at pdnews@frontlinemedcom.com.

Introduction

Often, it can be difficult for providers to fully understand why children may be presenting with behavioral problems that appear to be occurring principally in specific environments. Parents may bring their children for an evaluation based upon reports they are receiving from teachers and other school personnel who may be observing more prominent oppositionality, social struggles, troubles following instructions, and frustration intolerance than the parents are experiencing in the home. Additionally, youth may begin to display school refusal and voice strong negative feelings about school. Although at face value, these problems may indicate potential diagnoses like oppositional defiant disorder and attention-deficit/hyperactivity disorder, one also should consider other underlying “non–mental health” issues that could greatly influence school success and present with a range of emotional and behavioral struggles.

Case Summary

Brynn is an animated, social, and strong-willed 4 year-old girl who experienced delays in her receptive and expressive language that prompted her engagement in early intervention around 18 months of age. She and her family continued to receive developmental services over the next few years and, at the age of 3 years, because of ongoing speech and language challenges, she was enrolled in a preschool individualized education program. In her program, Brynn participates in an array of specialized instruction, but her educators comment that she is not making expected academic progress and has troubles “holding onto information,” focusing, and participating meaningfully with peers in a consistent manner.

At times, Brynn can be impulsive, aggressive, and volatile – behavioral traits that mom denies are occurring in the home. A diagnosis of an autism spectrum disorder has been ruled out, and Brynn’s hearing and vision are tested to be normal. Brynn’s mother feels confused by the reports she’s getting from school; “I want Brynn to be happy,” she shares. “I needed special help to read in high school, and I worry about her future.”

Discussion

Learning disorders (or learning disabilities, using educational terminology) are defined as neurologically rooted problems that affect academic achievement via the receiving, processing, or communication of information. They occur in 1 in 10 children (Pediatrics. 2007 Feb;119 Suppl 1:S77-83) and can present with specific problems in reading, writing, and mathematics while having considerable influence on related aptitudes in language, social ability, self-help, and motor functioning. Dyslexia – a developmental reading disorder – is the most common type of learning disorder (LD). Although it’s not clear what causes learning disorders, there are several factors that are thought to play a role in their development, including hereditary factors and problems with pregnancy and birth. Having developmental delays also can place children at risk for having later learning problems that may not be identifiable until a child enters a more structured learning environment. At clinical visits during the preschool years, particularly with a child who may have had earlier developmental concerns, the pediatrician should inquire about a range of “warning signs” that may indicate a need for additional screening and evaluation for specific learning issues.

The National Center for Learning Disabilities offers a range of practical tips for pediatricians who may want to further explore parental and teacher concerns by asking questions related to literacy, writing, language, and social-emotional skills, attention, and gross and fine motor movements in a developmentally informed manner. Further exploring Brynn’s mother’s comments in the context of her daughter not progressing in school revealed a history of difficulty retaining new words, troubles learning colors and shapes, challenges remembering rules, and particular difficulties engaging in group play with other 4-year-olds – all potential signals for a learning disorder. This alerted educators that she may indeed be struggling with issues beyond that of an enduring speech-language delay.

With the suspicion that Brynn was presenting with signs and symptoms suggestive of a learning disorder, her family was educated about the Individuals with Disabilities Education Act, and it was recommended she receive a comprehensive psychoeducational evaluation to further assess her intellectual profile, academic achievement, social functioning, and performance in the classroom using standardized tools. These tools, among other objectives, can help the child’s team offer a more definitive LD diagnosis while informing the potential development of special education supports and assessing for an intellectual disability. The DSM 5 indicates that LD diagnostic criteria are to be met based upon a clinical synthesis of history, school reports, and psychoeducational assessment.

©Bigandt_Photography/Thinkstock

It’s been long established that children with learning problems frequently have co-occurring emotional and behavioral troubles (Arch Dis Child. 1974 Apr; 49[4]:249-56), many of which also should be considered as differential diagnoses in a child with school problems – as such, a complicated interplay of learning disorders and internalizing and externalizing conditions is often appreciated in school-age children with academic difficulties. At times, learning disorders can lead to emotional distress and could also be misdiagnosed as primary emotional and behavioral challenges. Specifically, children with learning problems are at risk for struggling with low self-esteem, loneliness, and anxiety, which also can be associated with mood disorders, school dropout, victimization, and engaging in substance use.

In Brynn’s case, the results of the Teacher Report Forms and Child Behavior Checklists were reviewed, revealing some evidence that she was experiencing clinical-level problems with her attention, but a discrepancy was noted between the teacher and parent report (the teachers endorsing more clinically significant symptoms). Although co-occurring attention-deficit/hyperactivity disorder (ADHD) is not uncommon in children with a learning disorder (Pediatrics. 2011 Mar;127[3]:462-70), we did not feel Brynn met criteria for this. We elected not to provide an ADHD diagnosis but are mindful that her attentional concerns should be closely monitored over time; a diagnosis may be more relevant in the future, perhaps influencing Brynn’s eligibility for services and treatment planning. Furthermore, comorbidity with ADHD is predictive of worse mental health outcomes, compared with learning disabilities presenting without ADHD.

Clinical Pearl

Pediatricians should consider the possibility of a child having a learning disorder in youth who display risk factors (family history of learning concerns, atypical development, prematurity, etc.) and have problems at school. Such problems may be presenting with emotional and behavioral symptoms that could mask a child’s primary learning impairments. Learning disorders also frequently co-occur with psychiatric conditions, but engaging children in effective interventions (school-based supports) could potentially attenuate the risk for the development of mental health problems. Also, promoting emotional wellness and fostering self-worth may improve the academic performance of children with learning disorders.

Dr. Dickerson, a child and adolescent psychiatrist, is an assistant professor of psychiatry at the University of Vermont, Burlington. He is the director of the university’s autism diagnostic clinic. Dr. Dickerson said he had no relevant financial disclosures. Contact Dr. Dickerson at pdnews@frontlinemedcom.com.

Rheumatologists already are voicing concerns regarding a new proposal to test adjustments to how drugs administered in a physician’s office are paid for.

That proposal, published March 11 in the Federal Register, would test a change to the current reimbursement of average sales price plus 6% for Part B drugs with a lower add-on percentage of 2.5% plus $16.50.

In a fact sheet highlighting the proposals, the Centers for Medicare & Medicaid Services said the change to a lower percentage plus a flat fee “will cover the cost of any drug paid under Medicare Part B.”

However, there are already questions about that.

“While this may seem the way in which CMS will control costs, they fail to recognize the cost to facilities in obtaining approval for these treatments, receiving and storing, and ultimately safely administering these therapies in an environment that provides the best outcomes for patients,” Dr. Norman B. Gaylis, a rheumatologist in private practice in Aventura, Fla., said.

In fact, Dr. Gaylis adds that the focus on lowering drug expenses in the Part B space could have the unintended consequence of raising these expenses because it will force a change of venue.

“It has become so prohibitive that ultimately many patients will be referred to more expensive, less efficient outpatient facilities with, in fact, an increase in overall costs,” he said.

More than 300 provider and patient groups covering a range of specialties and including the American College of Rheumatology, the Coalition of State Rheumatology Organizations, and a number of state rheumatology organizations, are calling on Congress to ask CMS to withdraw the proposal.

In a March 17 letter to the majority and minority leaders in both chambers, the group is challenging the CMS assertion in the proposed rule that the current 6% add-on “may encourage the use of more expensive drugs because the 6% add-on generates more revenues for more expensive drugs.”

“This assumption fails to take into account the fact that providers’ prescribing decisions depend on a variety of factors, including clinical characteristics and the complex needs of the Medicare population,” the letter states. “Most importantly, there is no evidence indicating that the payment changes contemplated by the model will improve quality of care, and may adversely impact those patients that lose access to their most appropriate treatments.”

CMS offered two other pricing models that would be tested: indications-based pricing and reference pricing. The former would set payment rates based on the clinical effectiveness of a drug, while the latter would test the impact of setting a benchmark price for a group of drugs in a similar therapeutic class. Related to that is a proposal that CMS enter into voluntary risk-sharing agreements with drug manufacturers to link outcomes with price adjustments.

Dr. Gaylis suggested that CMS is going after the wrong party if cost containment is the ultimate goal here and should be focusing its efforts on the prices of the drugs themselves rather than how much they spend on physician reimbursement.

“Ironically, the major expense, i.e., the cost of drugs themselves, continues to spiral in the absence of any legitimate mechanism between CMS and pharma to contract prices that could save health care billions of dollars,” he said. “Ultimately, in my opinion, the solution rests in creating a fair and equal price for facilities administering these therapies and creating a pass-through where the drugs are not part of the physician’s risk, cost, or benefit and all payers, including CMS, can negotiate drug costs directly with the manufacturer.”

As part of the proposed rule, CMS also is considering creating feedback and decision-support tools to help, such as offering best practices for prescribing certain medications or providing feedback on prescribing patterns relative to local, regional, and national trends.

On the patient side, CMS is proposing to eliminate any patient cost sharing for office-administered drugs.

Comments on the proposals are due May 9.

gtwachtman@frontlinemedcom.com

Rheumatologists already are voicing concerns regarding a new proposal to test adjustments to how drugs administered in a physician’s office are paid for.

That proposal, published March 11 in the Federal Register, would test a change to the current reimbursement of average sales price plus 6% for Part B drugs with a lower add-on percentage of 2.5% plus $16.50.

In a fact sheet highlighting the proposals, the Centers for Medicare & Medicaid Services said the change to a lower percentage plus a flat fee “will cover the cost of any drug paid under Medicare Part B.”

However, there are already questions about that.

“While this may seem the way in which CMS will control costs, they fail to recognize the cost to facilities in obtaining approval for these treatments, receiving and storing, and ultimately safely administering these therapies in an environment that provides the best outcomes for patients,” Dr. Norman B. Gaylis, a rheumatologist in private practice in Aventura, Fla., said.

In fact, Dr. Gaylis adds that the focus on lowering drug expenses in the Part B space could have the unintended consequence of raising these expenses because it will force a change of venue.

“It has become so prohibitive that ultimately many patients will be referred to more expensive, less efficient outpatient facilities with, in fact, an increase in overall costs,” he said.

More than 300 provider and patient groups covering a range of specialties and including the American College of Rheumatology, the Coalition of State Rheumatology Organizations, and a number of state rheumatology organizations, are calling on Congress to ask CMS to withdraw the proposal.

In a March 17 letter to the majority and minority leaders in both chambers, the group is challenging the CMS assertion in the proposed rule that the current 6% add-on “may encourage the use of more expensive drugs because the 6% add-on generates more revenues for more expensive drugs.”

“This assumption fails to take into account the fact that providers’ prescribing decisions depend on a variety of factors, including clinical characteristics and the complex needs of the Medicare population,” the letter states. “Most importantly, there is no evidence indicating that the payment changes contemplated by the model will improve quality of care, and may adversely impact those patients that lose access to their most appropriate treatments.”

CMS offered two other pricing models that would be tested: indications-based pricing and reference pricing. The former would set payment rates based on the clinical effectiveness of a drug, while the latter would test the impact of setting a benchmark price for a group of drugs in a similar therapeutic class. Related to that is a proposal that CMS enter into voluntary risk-sharing agreements with drug manufacturers to link outcomes with price adjustments.

Dr. Gaylis suggested that CMS is going after the wrong party if cost containment is the ultimate goal here and should be focusing its efforts on the prices of the drugs themselves rather than how much they spend on physician reimbursement.

“Ironically, the major expense, i.e., the cost of drugs themselves, continues to spiral in the absence of any legitimate mechanism between CMS and pharma to contract prices that could save health care billions of dollars,” he said. “Ultimately, in my opinion, the solution rests in creating a fair and equal price for facilities administering these therapies and creating a pass-through where the drugs are not part of the physician’s risk, cost, or benefit and all payers, including CMS, can negotiate drug costs directly with the manufacturer.”

As part of the proposed rule, CMS also is considering creating feedback and decision-support tools to help, such as offering best practices for prescribing certain medications or providing feedback on prescribing patterns relative to local, regional, and national trends.

On the patient side, CMS is proposing to eliminate any patient cost sharing for office-administered drugs.

Comments on the proposals are due May 9.

gtwachtman@frontlinemedcom.com

Rheumatologists already are voicing concerns regarding a new proposal to test adjustments to how drugs administered in a physician’s office are paid for.

That proposal, published March 11 in the Federal Register, would test a change to the current reimbursement of average sales price plus 6% for Part B drugs with a lower add-on percentage of 2.5% plus $16.50.

In a fact sheet highlighting the proposals, the Centers for Medicare & Medicaid Services said the change to a lower percentage plus a flat fee “will cover the cost of any drug paid under Medicare Part B.”

However, there are already questions about that.

“While this may seem the way in which CMS will control costs, they fail to recognize the cost to facilities in obtaining approval for these treatments, receiving and storing, and ultimately safely administering these therapies in an environment that provides the best outcomes for patients,” Dr. Norman B. Gaylis, a rheumatologist in private practice in Aventura, Fla., said.

In fact, Dr. Gaylis adds that the focus on lowering drug expenses in the Part B space could have the unintended consequence of raising these expenses because it will force a change of venue.

“It has become so prohibitive that ultimately many patients will be referred to more expensive, less efficient outpatient facilities with, in fact, an increase in overall costs,” he said.

More than 300 provider and patient groups covering a range of specialties and including the American College of Rheumatology, the Coalition of State Rheumatology Organizations, and a number of state rheumatology organizations, are calling on Congress to ask CMS to withdraw the proposal.

In a March 17 letter to the majority and minority leaders in both chambers, the group is challenging the CMS assertion in the proposed rule that the current 6% add-on “may encourage the use of more expensive drugs because the 6% add-on generates more revenues for more expensive drugs.”

“This assumption fails to take into account the fact that providers’ prescribing decisions depend on a variety of factors, including clinical characteristics and the complex needs of the Medicare population,” the letter states. “Most importantly, there is no evidence indicating that the payment changes contemplated by the model will improve quality of care, and may adversely impact those patients that lose access to their most appropriate treatments.”

CMS offered two other pricing models that would be tested: indications-based pricing and reference pricing. The former would set payment rates based on the clinical effectiveness of a drug, while the latter would test the impact of setting a benchmark price for a group of drugs in a similar therapeutic class. Related to that is a proposal that CMS enter into voluntary risk-sharing agreements with drug manufacturers to link outcomes with price adjustments.

Dr. Gaylis suggested that CMS is going after the wrong party if cost containment is the ultimate goal here and should be focusing its efforts on the prices of the drugs themselves rather than how much they spend on physician reimbursement.

“Ironically, the major expense, i.e., the cost of drugs themselves, continues to spiral in the absence of any legitimate mechanism between CMS and pharma to contract prices that could save health care billions of dollars,” he said. “Ultimately, in my opinion, the solution rests in creating a fair and equal price for facilities administering these therapies and creating a pass-through where the drugs are not part of the physician’s risk, cost, or benefit and all payers, including CMS, can negotiate drug costs directly with the manufacturer.”

As part of the proposed rule, CMS also is considering creating feedback and decision-support tools to help, such as offering best practices for prescribing certain medications or providing feedback on prescribing patterns relative to local, regional, and national trends.

On the patient side, CMS is proposing to eliminate any patient cost sharing for office-administered drugs.

Comments on the proposals are due May 9.

gtwachtman@frontlinemedcom.com