Bilateral Endoscopic Endonasal Optic Nerve Decompression in an Infant with Osteopetrosis: A Case Report

TL;DR

A 7-month-old infant with osteopetrosis underwent a rare endoscopic surgery to relieve optic nerve compression, showing the procedure's viability in very young patients.

Contribution

This case report presents one of the youngest patients to undergo endoscopic endonasal optic nerve decompression for osteopetrosis.

Findings

Bilateral endoscopic endonasal decompression was successfully performed on a 7-month-old infant with osteopetrosis.

The surgical approach provided direct access to the optic canal with minimal morbidity.

This case highlights the potential for early surgical intervention to improve visual outcomes and quality of life in infants with osteopetrosis.

Abstract

Osteopetrosis is a rare genetic disorder characterized by abnormal bone density and structure, often leading to vision loss due to optic canal stenosis and consequent nerve compression. Early intervention is critical to prevent irreversible damage. This case report discusses the management of bilateral optic nerve compression in an infant with osteopetrosis.

A 7-month-old male with a family history of osteopetrosis presented with hepatosplenomegaly. The infant was diagnosed with osteopetrosis based on radiological findings and genetic testing. Ophthalmologic examination and magnetic resonance imaging showed evidence of bilateral optic nerve compression. Endoscopic transcaruncular optic nerve decompression was not attainable The patient underwent a bilateral expanded endoscopic endonasal medial orbital wall and optic canal decompression.

This is one of the few reported cases of endoscopic endonasal optic nerve decompression surgery on an infant. Endoscopic endonasal optic nerve decompression surgery is a viable and effective treatment option for optic nerve compression in infants with osteopetrosis, especially in cases where cost of surgery is a limiting factor for patients. This approach provides direct access to the optic canal with minimal morbidity, offering significant potential for visual recovery, and an improved quality of life. Our patient represents the youngest reported infant in the literature, demonstrating the potential for undergoing this surgical approach at the earliest possible age to aid with his prognosis.

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