Eur J Pediatr Surg 2014; 24(04): e1-e2
DOI: 10.1055/s-2007-965474
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Georg Thieme Verlag KG Stuttgart · New York

Paediatric Surgical Complications of Ehlers-Danlos Syndrome

B. Amjad
1  Royal Hospital for Sick Children, Yorkhill, Glasgow, United Kingdom
,
C. A. Hajivassiliou
1  Royal Hospital for Sick Children, Yorkhill, Glasgow, United Kingdom
,
F. M. Pope
2  School of Health and Life Sciences, University of London, London, United Kingdom
› Author Affiliations
Further Information

Publication History

Publication Date:
07 July 2009 (eFirst)

Introduction

Ehlers-Danlos syndrome (EDS) is a group of inherited disorders characterised by abnormalities of the skin, joints and other connective tissues. The cardinal features include cutaneous fragility and hyperextensibility, dystrophic scarring, joint hypermobility and connective tissue weakness. The clinical features of this condition were first described by Van Meek'ren in 1668 and Tschernogubow in 1891. Edward Ehlers thereafter in 1899 and Henri-Alexandre Danlos in 1906 independently reported the classic triad of lax digits, fragile skin and recurrent haematomas. EDS is very heterogeneous on genetic, molecular and clinical grounds. Precise clinical subclassification is important, particularly as some variants have life-threatening associations, requiring appropriate management. EDS type IV is of particular importance to surgeons as it is associated with arterial rupture and visceral perforation, with grave consequences. We present a case in whom the potential association of spontaneous bleeding, albeit in a less likely subtype, was not considered, leading to an unnecessary operation.