Distensible venous malformations of the orbit - Clinical and hemodynamic features and a new technique of management

Objective: To investigate distensible venous malformations of the orbit (DV MO) as part of a spectrum of orbital vascular malformations, including some that involved periorbital skin, extraorbital sites (central nervous system or nasal sinuses), or combinations of these, The authors also investigated the effectiveness of a new technique of management for selected cases. Design: Retrospective noncomparative case series. Participants: Thirty patients had distensible venous anomalies, of which fo ur were combined distensible venous-lymphatic vascular malformations. Diste nsible lesions were defined as those showing clinical or radiographic expan sion with Valsalva maneuver or when the head was placed in a dependent posi tion. These lesions were then classified as superficial (anterior to the eq uator of the globe), deep (posterior to the globe's equator), combined (dee p and superficial), or complex (with intracranial or major extraorbital inv olvement), Intervention: Surgery was performed on 15 patients (50%), mainly for pain o r for cosmetic indications. Six patients underwent this new technique, whic h involved intraoperative direct venography with control of outflow via pre ssure at the superior or inferior orbital fissure. The venous malformation was then embolized (by use of cyanoacrylate glue) and excised. Results: The mean age at presentation was 28.2 years (range, 8 months to 75 years). Sixty-six percent of cases involved the left orbit. Superior and m edial orbital involvement was most common. Three cases (10%) were classifie d as superficial, and 13 (43%) as deep. Six patients (20%) had combined sup erficial and deep components. Eight (27%) had major extraorbital involvemen t (4 intracranial, 2 facial, and 2 paranasal sinus). Direct venography demo nstrated complex multichannel anomalies draining to various sites, includin g the face and pterygopalatine fossa, without necessarily having a direct c onnection to the major orbital venous circulation. Conclusions: Distensible venous malformations of the orbit are part of a sp ectrum of developmental venous malformations that may be localized to the o rbit or involve it as part of a more extensive lesion. The authors describe their clinical and radiologic features and report a new technique of manag ement for selected cases. This method of vascular isolation and embolizatio n of lesions may greatly facilitate excision.