Servelle-Martorell syndrome with extensive upper limb involvement: A case report

Introduction: Angio-osteohypotrophic syndrome is also known as Servelle-Martorell angiodysplasia. It is characterized by venous or, rarely, arterial malformations, which may result in limb hypertrophy and bony hypoplasia. Extensive involvement of the upper limb is a rare feature of Servelle-Martorell syndrome. Cases with minimal upper limb involvement have been described in the literature. Case presentation: A young man presented with multiple separate swollen areas over the right upper limb and functional difficulty since birth. The arm muscles and muscles of the limb girdle were atrophic. The forearm and hand bones were hypoplastic and tender. Conclusion: We report a case of Servelle-Martorell syndrome with extensive involvement of the entire upper limb and periscapular region. Servelle-Martorell syndrome is highlighted as one of the causes of angiodysplastic limb hypertrophy. © 2008 Karuppal et al; licensee BioMed Central Ltd.