Essential timing of orthopaedic treatment in children with Ehlers-Danlos syndrome arthrochalasia type: a case report

M.J. van Rooij, H. Staal, M. Klaassens, L. van Rhijn, C.T.R.M. Stumpel

Research output: Contribution to journalArticleAcademicpeer-review

Abstract

Objective: ?This case report presents the orthopaedic impact of arthrochalasia-type Ehlers-Danlos syndrome (EDS) (former type VIIA and B) in a young girl. Methods: The characteristics of EDSs are skin abnormalities and tissue fragility with orthopaedic consequences including joint hypermobility and dislocations. In EDS arthrochalasia type (former type VIIA and B) severe general hypotonia and congenital bilateral hip dislocation are distinctive symptoms. This type of EDS is less common than other types and just a few cases have been reported. Results: This report describes a case of a 3-year old girl with EDS arthrochalasia type and bilateral high hip dislocation, spontaneous shoulder dislocation and scoliosis as orthopaedic consequences. Conclusions: Orthopaedic consequences in these cases can be severe so conservative or surgical treatment will be necessary to reduce disability in later life. Timing of either conservative or surgical intervention is essential. According to the literature, most conservative treatments fail. Also, surgical treatment is rarely effective in most of the cases. Both conservative and surgical treatment should be considered carefully in the treatment of EDS arthrochalasia type, each of which has pros and cons. The best treatment depends on the prognosis and current situation of the child.
Original languageEnglish
Article number39
JournalF1000Research
Volume3
DOIs
Publication statusPublished - 1 Jan 2014

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