EHLER DANLOS SYNDROME- ARTHROCHALASIS TYPEJournal: University Journal of Surgery and Surgical Specialities (Vol.5, No. 1)
Publication Date: 2019-01-22
Authors : DINESH PRABHU S;
Page : 22-23
Keywords : EDS; ARTHROCHALASIS; HYPER MOBILITY.;
The Ehlers-Danlos syndromes (EDS) form a clinically and genetically heterogeneous group of inherited connective-tissue disorders characterized by joint hypermobility, tissue fragility and skin abnormalities. Six subtypes have been well characterized based on clinical features and molecular genetic abnormalities. The arthrochalasia type EDS (formerly type VIIa and VIIb) is a extremely rare condition characterized by severe generalized joint hypermobility with multiple dislocations including congenital bilateral dislocation of the hips, muscular hypotonia and distinct dysmorphic features. We report a 7 year old girl who presented with joint hyper mobility and bilateral dislocation of hips. This was a case of EDS- ARTHROCHALASIS type. This case is being reported for its rarity. Till date only 30 cases have been reported in literature worldwide.
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Last modified: 2019-02-05 19:08:03