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Congenital dislocation of the hip in Ehlers-Danlos syndrome
O Badelon1, H Bensahel, Z Csukonyi
1Department of Pediatric Orthopaedic Surgery, Hospital Robert Debré, Bichat University Medical School, Paris, France.
Insights
Congenital hip dislocation in Ehlers-Danlos syndrome (EDS) patients is challenging. Surgical intervention, including osteotomies, is often required for successful treatment and stabilization.
Area of Science:
- Orthopedics
- Genetics
- Pediatric Surgery
Background:
- Ehlers-Danlos syndrome (EDS) is a group of inherited connective tissue disorders.
- Congenital dislocation of the hip (CDH) is a complex orthopedic condition.
- EDS can complicate the management of CDH due to tissue fragility.
Purpose of the Study:
- To evaluate the treatment outcomes for congenital dislocation of the hip (CDH) in patients with Ehlers-Danlos syndrome (EDS).
- To identify challenges and necessary surgical approaches for managing CDH in this specific patient population.
Main Methods:
- Retrospective analysis of nine patients diagnosed with EDS and bilateral CDH.
- Initial treatment involved closed reduction for eight patients, with subsequent surgical interventions.
- Total of 42 surgical procedures performed across all patients.
Main Results:
- Avascular necrosis of the femoral head occurred in five hips, predominantly after closed reduction.
- Clinical success was achieved in 12 of 16 hips, while radiographic success was noted in only six.
- Significant surgical interventions, including femoral and innominate osteotomies, were required for stabilization.
Conclusions:
- Managing CDH in EDS patients presents significant challenges requiring specialized surgical techniques.
- Femoral and innominate osteotomies are crucial for achieving and maintaining hip reduction in EDS patients with CDH.
- Early and aggressive surgical management may be necessary to improve outcomes.
Abstract:
Nine patients with Ehlers-Danlos syndrome (EDS) were treated for bilateral congenital dislocation of the hip (CDH). CDH was diagnosed at six months of age in two patients and at two to five years of age in the remaining seven patients. The diagnosis of EDS, based on clinical criteria, was established considerably later. One patient with multiple deformities was only observed; the remaining eight patients were initially treated by closed reduction. Due to difficulties encountered in reduction and especially in stabilization, all hips were subsequently treated surgically, with a total of 42 procedures. Avascular necrosis of the femoral head developed in five hips, four of which had been treated with closed reduction with the patients under general anesthesia. At the follow-up evaluation, six patients had reached adulthood. Clinically, satisfactory results were obtained in 12 of 16 hips but roentgenographically in only six hips. Both femoral and innominate osteotomies are necessary to achieve and maintain the reduction in EDS patients with CDH.
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