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Complex juxtapapillary capillary hemangioma: a case report.

Sherrol A Reynolds¹, Diana Shechtman, Laura Falco

¹Nova Southeastern University, College of Optometry, Ft. Lauderdale, Florida 33328, USA. sreynold@nova.edu

Optometry (St. Louis, Mo.)

|August 30, 2008

Summary

Juxtapapillary capillary hemangiomas, a condition linked to von Hippel-Lindau (VHL) disease, can cause vision loss. Early diagnosis and treatment are crucial for better outcomes in patients with these tumors.

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Area of Science:

Ophthalmology
Medical Genetics

Background:

Juxtapapillary capillary hemangiomas are vascular tumors located on or near the optic disc.
These tumors can be isolated or a manifestation of von Hippel-Lindau (VHL) disease.
Management involves monitoring, with treatment initiated upon tumor progression or vision compromise.

Observation:

A 14-year-old male presented with blurry vision due to a juxtapapillary capillary hemangioma.
Initial observation showed 20/25 visual acuity in the affected eye; no immediate treatment was given.
The patient was lost to follow-up and returned a year later with vision deteriorated to 20/400.

Findings:

The hemangioma enlarged, causing macular exudation and subretinal fluid.
Treatment with photodynamic therapy led to complications including vitreous hemorrhage and retinal detachment.
Subsequent surgeries improved vision to 20/60, but a cataract developed.
Genetic testing confirmed a VHL gene deletion; brain MRI was normal.

Implications:

The clinical course of juxtapapillary capillary hemangiomas is unpredictable and can lead to severe ocular complications.
No established treatment guidelines exist, and interventions carry risks due to tumor location.
Screening for VHL disease in patients and families is vital, as this hemangioma can be the sole presenting sign.
Prompt diagnosis and management are essential for improving visual and systemic prognoses.