Objective: To investigate distensible venous malformations of the orbit (DVMO) 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 four were combined distensible venous-lymphatic vascular malformations. Distensible lesions were defined as those showing clinical or radiographic expansion with Valsalva maneuver or when the head was placed in a dependent position. These lesions were then classified as superficial (anterior to the equator of the globe), deep (posterior to the globe's equator), combined (deep and superficial), or complex (with intracranial or major extraorbital involvement).
Intervention: Surgery was performed on 15 patients (50%), mainly for pain or for cosmetic indications. Six patients underwent this new technique, which involved intraoperative direct venography with control of outflow via pressure 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 medial orbital involvement was most common. Three cases (10%) were classified as superficial, and 13 (43%) as deep. Six patients (20%) had combined superficial and deep components. Eight (27%) had major extraorbital involvement (4 intracranial, 2 facial, and 2 paranasal sinus). Direct venography demonstrated complex multichannel anomalies draining to various sites, including the face and pterygopalatine fossa, without necessarily having a direct connection to the major orbital venous circulation.
Conclusions: Distensible venous malformations of the orbit are part of a spectrum of developmental venous malformations that may be localized to the orbit or involve it as part of a more extensive lesion. The authors describe their clinical and radiologic features and report a new technique of management for selected cases. This method of vascular isolation and embolization of lesions may greatly facilitate excision.