Diffuse Venous Malformations of the Upper Extremity (Bockenheimer disease): Diagnosis and Management.

PURPOSE: Diffuse venous malformations (VMs) that involve all tissues in the upper limb and ipsilateral chest wall are known as "phlebectasia of Bockenheimer." We describe our experience with management of this uncommon vascular anomaly.

METHODS: Our Vascular Anomalies Center registry comprises 18,766 patients over a 40-year period. This review identified 2,036 patients with venous malformations of the extremities (10.8%), of whom only 80 (0.43%) had Bockenheimer disease. We retrospectively analyzed patient characteristics, diagnostics, treatments, and complicati ons.

RESULTS: The VM was first noted at birth or within the first few years of life with slow and gradual progression. Pain was related to engorgement of the limb. Thromboses and phleboliths were common, but diffuse intravascular coagulopathy occurred in only twelve patients (15%). Skeletal involvement was demonstrated as lytic lesions, cortical scalloping, osteopenia, and pathologic fractures. Management included compression garments (100%), sclerotherapy (27.5%), and resection of symptomatic areas in 35% of patients. Adjunctive pharmacologic medication was given in 7.5%. Following resection, 17 patients (60%) had one or more complications: hematoma, wound dehiscence, flap loss, contracture, and psychosis. There were no deaths. Symptoms improved in all patients with useful functional outcomes.

CONCLUSIONS: The decision to pursue compression, sclerotherapy, pharmacologic treatment or resection alone or in combination was by an interdisciplinary team. Although extensive VMs cannot be completely ablated, debulking of symptomatic regions, resection of neuromas and non-invasive treatments improve the quality of life. Despite the bulk and weight of the arm, forearm and hand, and ominous appearance on MRI scans, these patients remain functional.