Servelle-Martorell syndrome, also known as angio-osteohypotrophic syndrome, is a rare disorder characterized by limb hypertrophy due to extensive venous anomalies such as venous dilatations, venous ectasia, venous malformation with absent valves, and/or hypoplastic veins. Rarely, arterial anomalies are also implicated 3.
Clinical presentation
The affected limb is hypertrophied compared to the contralateral side 3. Additionally, there is often significant skin discolouration and there may be focal neurological deficits 3.
Pathology
Intraosseous venous malformation frequently results in bone hypoplasia and joint destruction 1.
Treatment and prognosis
The prognosis for this condition is uncertain. Management is predominantly conservative. In cases of aneurysmal complications or severe shunting, surgery may be necessary 2.
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