Open and Endoscopic Endonasal Optic Nerve Decompression for Craniofacial Fibrous Dysplasia in an Adolescent: 2-Dimensional Operative Video.

Craniofacial fibrous dysplasia (CFFD) is a benign, bony disease that may affect the skull base.1,2 Most cases are asymptomatic and observed; however, advanced disease can present with cranial neuropathy or craniofacial deformity requiring intervention.3-5 A 16-year-old adolescent girl with known CFFD involving the sphenoid and frontal bones with severe bilateral optic canal narrowing developed progressive right eye visual decline and frontal cosmetic deformity. Visual acuity worsened oculus dextrus (OD) to 20/30 with a new superior nasal scotoma and 20% loss in the retinal nerve fiber layer and remained oculus sinister (OS) 20/20. The patient was recommended a staged subfrontal craniotomy for right optic decompression and simultaneous correction cosmetic deformity followed by endonasal right optic decompression. On postoperative day one, visual acuity OD improved to 20/20; however, she developed OS visual decline to 20/800. Curiously, there were no episodes of intraoperative hypotension or additional iatrogenic compression. Use of methylprednisolone led to improvement OS 20/400. Given persistent visual decline, urgent second stage endonasal bilateral optic nerve decompression, rather than unilateral, was performed. Postoperatively, vision improved to OS 20/200. At one month, her vision improved to OD 20/15 and OS returned to 20/20 with a paracentral scotoma and 29% decline in left retinal nerve fiber layer with further improvement anticipated. This video describes a multidisciplinary, multistaged approach in treatment of optic nerve compression due to CFFD in addition to the management of unanticipated contralateral visual decline. The patient consented to the procedure and publication of her image. No Institutional Review Board/ethics committee approval was necessary for this case report.

Cataract associated with type-1 diabetes mellitus in the pediatric population.

PURPOSE: To report 14 cases (28 eyes) of cataract associated with type-1 diabetes mellitus in young children and adolescents. METHODS: Retrospective review of the medical records of 14 patients from seven institutions. All patients under the age of 18 years who met the inclusion criteria of type-1 diabetes mellitus and cataract were included. RESULTS: Mean age at the time of diabetes diagnosis was 9.8 years (range, 0.5-14 years), and mean age at cataract diagnosis was 11.7 years (range, 5-16 years). Two patients presented with cataracts one month before the diagnosis of diabetes; seven after the diagnosis of diabetes; and in five patients the cataract was found at the time the diabetes was diagnosed by the pediatrician. Nineteen out of 23 operated eyes had a best corrected post operative visual acuity of 20/40 or better. Two patients (4 eyes) developed diabetic retinopathy postoperatively. CONCLUSIONS: Although cataract formation in type-1 diabetes is rare, it is treatable and potentially sight-saving in young children and adolescents.