Low-Flow Orbital Venous Malformation Masquerading as Rhabdomyosarcoma

Area of Science:

• Ophthalmology
• Pediatric Neurosurgery
• Vascular Malformations

Background:

• Orbital masses in children can present with rapidly progressing symptoms like proptosis and edema.
• Rhabdomyosarcoma is a common differential diagnosis for pediatric orbital tumors, necessitating accurate diagnostic workup.

Observation:

• A 15-month-old male presented with left-sided proptosis, periorbital edema, and hypoglobus.
• Initial MRI suggested a soft-tissue mass in the retrobulbar space, raising suspicion for rhabdomyosarcoma.
• Subsequent CT with dynamic imaging revealed characteristics of a low-flow orbital venous malformation with intracranial extension.

Findings:

• The orbital mass demonstrated central filling and delayed venous enhancement, indicative of a vascular lesion.
• The patient experienced spontaneous orbital hemorrhage, increasing the risk of vision loss.
• A combined neurosurgical approach was employed to address both the intracranial and orbital components.

Implications:

• This case underscores the importance of advanced imaging techniques in differentiating orbital masses.
• Accurate radiographic evaluation is critical for appropriate surgical planning and management of pediatric orbital vascular malformations.
• Timely intervention in cases of orbital venous malformation with hemorrhage can prevent irreversible vision impairment.