2025 Poster Presentations
P046: ENDOSCOPIC ENDONASAL OPTIC NERVE DECOMPRESSION IN AN INFANT WITH OSTEOPETROSIS: A CASE REPORT
Zeina Korban, MD; Nader Zalaquet; Hussein Darwich; Yara Yammine; Rita Maria Jalkh; American University of Beirut Medical center
Background: Osteopetrosis is a rare genetic disorder characterized by abnormal bone density and structure, often leading to various complications, including 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 optic nerve compression in an infant with osteopetrosis through a bilateral endoscopic endonasal optic nerve decompression.
Case Presentation: 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 revealed esotropia and temporal pallor. MRI showed narrowing of the optic canals, confirming the presence of bilateral optic nerve compression.
Management and Outcome: Endoscopic transcaruncular optic nerve decompression was not possible. In collaboration between rhinology and neurosurgery, the patient underwent endoscopic endonasal bilateral medial orbital wall and optic canal decompression. There were no intraoperative or postoperative complications.
Conclusion: This is one of the few reported cases of endoscopic endonasal optic nerve decompression surgery on an infant. Endoscopic endonasal optic nerve decompression surgery without navigation is a viable and effective treatment option for optic nerve compression in infants with osteopetrosis, especially in a third world country where cost of surgery is a limiting factor for patients. This minimally invasive approach provides direct access to the optic canal with minimal morbidity, offering significant potential for visual recovery, prevention of worsening and improved quality of life. Early diagnosis and intervention are essential in preventing permanent visual loss in affected patients. Our patient is the youngest reported in the literature, demonstrating the potential for undergoing such surgeries at the earliest possible age to help with prognosis and early intervention with bone marrow transplantation.