Servelle-Martorell Syndrome (SMS)
Servelle-Martorell syndrome, also known as Servelle-Martorell angiodysplasia or phlebectatic osteohypoplastic angiodysplasia, involves partial or a complete lack of valves in the deep venous system and intra-osseous vascular malformations. It is an extremely rare condition with characteristics including:
- Venous malformations
- Bone hypoplasia
- Limb (usually upper extremity) hypertrophy
Findings may also include venous thrombosis as well as consumptive coagulopathy.
Diagnosis:
You/your child will meet with the Vascular Anomalies Center team during the initial clinic visit for a comprehensive review of the patient’s medical history, any imaging studies and/or laboratory tests that have been performed, and a complete physical examination. The medical specialists will then confer and diagnose the condition and propose a treatment plan. Diagnosis is based on:
- Clinical features
- Plain radiograph of upper extremity (which show phleboliths and bony hypoplasia)
- MRI (to discern from other vascular anomalies)
Treatment:
There is no cure for patients with this condition. Conservative management may include external compression garments to help alleviate pain as well as protect the affected region from external trauma. Wearing a compression garment has no effect on the ultimate size of the limb.
Treatment modalities may involve sclerotherapy. As the extent of the venous malformations can be extensive, complete excision of the affected vessels is generally not possible. Symptomatic varicosities or localized venous malformations can be removed in some patients with good results provided that there is a functioning deep vein system. Patients with recurrent attacks of cellulitis may benefit from prophylactic antibiotic therapy. Anticoagulants are indicated after deep vein thrombosis or pulmonary embolus. Patients with recurrent superficial thrombophlebitis frequently require daily administration of aspirin. Pain can be managed with oral anti-inflammatory medication, such as ibuprofen.
