Serena Fragiotta , Massimiliano Sepe , Andrea Perdicchi , Luca Scuderi , Maria Trani & Gianluca Scuderi
A 62-year-old white woman presented with a diagnosis of blue rubber bleb nevus syndrome (BRBNS). The right eye appeared enophthalmic, yet the patient complained of episodes of right proptosis on bending forward. The remainder of the examination was unremarkable. Orbital ultrasound (US) in an upright posture revealed a single low reflectivity cavity (4.27 mm x 2.82 mm) of uncertain interpretation. In a forward-leaning posture the lesion increased in size (maximum thickness of 13.72 mm), demonstrating multiple low reflectivity spaces with highly reflective septae. This case first reports the use of US with postural changes to assess the presence of orbital venous malformation in BRBNS. The expansile nature upon postural changes supports the venous origin of the orbital lesion.
Pandit, Saagar A.; Godfrey, Kyle J.; Dunbar, Kristen E.; Campbell, Ashley A.; Kazim, Michael Less
A 15-month-old male was referred for biopsy of presumed rhabdomyosarcoma in the setting of rapidly progressing left-sided proptosis. Examination revealed left periorbital edema and left hypoglobus. MRI revealed a soft-tissue density mass within the left lateral retrobulbar space. Several days later, he developed acute periorbital ecchymosis and increasing edema. With high suspicion for a vascular lesion, a CT scan was performed with dynamic arterial and venous imaging. Central filling was noted in the lateral retrobulbar component with increased enhancement on delayed venous imaging in the middle cranial fossa component favoring the diagnosis of a low-flow orbital venous malformation. In the setting of spontaneous orbital hemorrhage and risk of future vision loss, the decision was made to proceed with a combined neurosurgical approach treating the intracranial component and debulking the orbital component. This case highlights the importance of thorough radiographic evaluation prior to proceeding with a surgical procedure in the appropriate clinical context.
Evan Kalin-Hajdu, John B. Colby, Oluwatobi Idowu, F. Lawson Grumbine, Jessica M. Kang, Kristin S. Hirabayashi, Christine M. Glastonbury, M. Reza Vagefi, Robert C. Kersten
To compare the diffusion-weighted imaging of nonthrombosed distensible venous malformations of the orbit with that of other histologically-proven orbital tumors.
Retrospective case-control study.
Patients with nonthrombosed distensible venous malformations of the orbit and patients with other histologically-proven orbital tumors were selected for chart review. The main outcome measure was the apparent diffusion coefficient of these lesions.
Sixty-seven patients qualified for chart review; 9 patients had nonthrombosed distensible venous malformations and 58 patients had other histologically-proven tumors. Three of the 9 patients with nonthrombosed distensible venous malformations were initially misdiagnosed as having had solid orbital tumors. The mean apparent diffusion coefficient of distensible venous malformations was 2.80 ± 0.48 × 10−3 mm2/s, whereas the mean apparent diffusion coefficient of other histologically-proven tumors was 1.18 ± 0.39 × 10−3 mm2/s (P < .001). The mean apparent diffusion coefficient ranged from 2.42 to 3.94 × 10−3 mm2/s in the distensible venous malformation group, whereas other histologically-proven tumors ranged from 0.53 to 2.08 × 10−3 mm2/s. Therefore, in this single-institution series, a threshold value of 2.10 × 10−3 mm2/s was 100% sensitive and 100% specific for distensible venous malformations.
Certain nonthrombosed distensible venous malformations can evade diagnostic suspicion and mimic solid orbital tumors on standard magnetic resonance imaging sequences. In this single-institution series, diffusion-weighted imaging effectively distinguished these nonthrombosed distensible venous malformations from other orbital tumors.
Galindo-Ferreiro, Alicia; Alkatan, Hind M.; ElKhamary, Sahar M.; AlDosairi, Saif; Cruz, A. Augusto V.
An 8-year-old boy initially presented with an orbitopalpebral mass diagnosed clinically and radiologically as a low-flow diffuse venous lymphatic malformation involving the left upper eyelid and orbit. Over 8 months of follow up, he had 2 acute episodes of severe orbital inflammation that warranted hospitalization and treatment with intravenous antibiotic, steroids and surgical debulking. After a third surgical excision, the lesion remained clinically stable.