CORR Insights®: Iatrogenic Hip Instability Is a Devastating Complication After the Modified Dunn Procedure for Severe Slipped Capital Femoral Epiphysis

Abstract Background Most patients with congenital adrenal hypoplasia (AHC) develop symptoms during infantile and juvenile periods, with varying clinical manifestations. AHC is a disease that is easily misdiagnosed as Addison’s disease or congenital adrenal hyperplasia (CAH). There was also a significant time difference between the age at which patients developed symptoms and the age at which they were diagnosed with AHC. Most patients showed early symptoms during infantile and juvenile periods, but were diagnosed with AHC many years later. Case presentation We are currently reporting a male patient who developed systemic pigmentation at age 2 and was initially diagnosed with Addison’s disease. At 22 years of age, he experienced a slipped capital femoral epiphysis (SCFE), a disease mostly seen in adolescents aged 8–15 years, an important cause of which is endocrine disorder. Testes evaluated using color Doppler Ultrasonography suggested microcalcifications. Further genetic testing and auxiliary examinations revealed that the patient had hypogonadotropic hypogonadism (HH) and DAX-1 gene disorders, at which time he was diagnosed with AHC complicated by HH. He was given hormone replacement therapy, followed by regular outpatient review to adjust the medication. Conclusions The typical early symptoms of AHC are hyperpigmentation and ion disturbance during infantile and juvenile periods, while few patients with AHC develop puberty disorders as early symptoms. AHC is prone to being misdiagnosed as Addison’s disease, and then gradually develops the symptoms of HH in adolescence. The definitive diagnosis of AHC ultimately is based on the patient’s clinical presentation, laboratory results and genetic testing results.

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Slipped Capital Femoral Epiphysis

Journal of Pediatric Orthopaedics B ◽

10.1097/01202412-199801000-00008 ◽

1998 ◽

Vol 7 (1) ◽

pp. 43-52 ◽

Cited By ~ 53

Author(s):

R. Velasco ◽

P. A. Schai ◽

G. U. Exner

Keyword(s):

Slipped Capital Femoral Epiphysis

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Estimated effect of secondary screening for hip dislocation

Archives of Disease in Childhood ◽

10.1136/archdischild-2020-319250 ◽

2020 ◽

Vol 105 (12) ◽

pp. 1175-1179

Author(s):

Daniel Wenger ◽

Carl Johan Tiderius ◽

Henrik Düppe

Keyword(s):

Birth Cohort ◽

Absolute Risk ◽

Hip Dislocation ◽

Absolute Risk Reduction ◽

Age At Diagnosis ◽

Hip Instability ◽

Primary Screening ◽

Surrogate Variable ◽

Secondary Screening ◽

Child Healthcare

ObjectivesTo quantify the effect of secondary screening for hip dislocations.DesignRetrospective analysis of hospital files from participants in a prospectively collected nationwide registry.SettingChild healthcare centres and orthopaedic departments in Sweden.ParticipantsOf 126 children with hip dislocation diagnosed later than 14 days age in the 2000–2009 birth cohort, 101 had complete data and were included in the study.InterventionsThe entire birth cohort was subject to clinical screening for hip instability at 6–8 weeks, 6 months and 10–12 months age. Children diagnosed through this screening were compared with children presenting due to symptoms, which was used as a surrogate variable representing a situation without secondary screening.Main outcome measuresAge at diagnosis and disease severity of late presenting hip dislocations.ResultsChildren diagnosed through secondary screening were 11 months younger (median: 47 weeks) compared with those presenting with symptoms (p<0.001). Children diagnosed through secondary screening had 11% risk of having a high (severe) dislocation, compared with 38% for those diagnosed due to symptoms; absolute risk reduction 27% (95% CI: 9.7% to 45%), relative risk 0.28 (95% CI: 0.11 to 0.70). Children presenting due to symptoms had OR 5.1 (95% CI: 1.7 to 15) of having a high dislocation, and OR 11 (95% CI: 4.1 to 31) of presenting at age 1 year or older, compared with the secondary screening group. The secondary screening was able to identify half of the children (55%, 95% CI: 45% to 66%) not diagnosed through primary screening.ConclusionsSecondary screening at child healthcare centres may have substantially lowered the age at diagnosis in half of all children with late presenting hip dislocation not diagnosed through primary screening, with the risk of having a high dislocation decreased almost to one-quarter in such cases.

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