Orbital Edema Secondary to a Sphenoidal Mass as the Presenting Symptom of High-Risk Precursor B-Cell Acute Lymphoblastic Leukemia.

Introduction: Acute lymphoblastic leukemia (ALL) is the most common childhood malignancy, known to present with ocular manifestations in rare cases. Case Presentation: We describe the case of a 9-year-old previously healthy male who developed a 2-day history of periocular swelling and was found on MRI to have a large sphenoidal mass. Further work up showed involvement of the spinal cord, iliac crests, and kidneys. His initial blood work showed no hematological abnormalities. A bone marrow biopsy taken from the iliac crest demonstrated >90% B lymphoblasts and flow cytometry was positive for CD19. Overall, his investigations were consistent with a diagnosis of precursor B-cell ALL (pre B-ALL). His neuro-ophthalmic exam showed right-sided subtle periocular edema, decreased palpebral fissure height, and proptosis. Posterior exam showed mild nasal elevation of the right optic disc without vessel obscuration and mild tortuosity of the peripheral vessels. He otherwise had no overt signs of afferent or efferent dysfunction despite the proximity of the mass to his optic nerve and globe. Conclusion: This case demonstrates that high-risk pre B-ALL, a childhood cancer not commonly associated with orbital manifestations, can present with orbital edema and normal leukocyte count in an otherwise healthy child.

Endoscopic goniotomy: early clinical experience in congenital glaucoma.

PURPOSE: To review the technique and early outcomes of endoscopic goniotomy (EG) in children with opaque corneas and primary congenital glaucoma (PCG) or developmental glaucoma with ocular or systemic anomalies (DG). METHODS: EG was performed for approximately 300 degrees of the angle through temporal and superonasal corneal incisions. We retrospectively reviewed consecutive cases from 2003 to 2007. Primary outcome was intraocular pressure (IOP) change from baseline to last postoperative visit. Success was defined as IOP < or=21 mm Hg with or without medication but no further surgical intervention. RESULTS: Fourteen eyes of 8 patients (4 PCG, 4 DG) were included. Mean age at surgery was 3.88+/-3.72 months. The reduction in IOP from baseline to the last follow-up visit was -16.7+/-16.7 mm Hg. Success was achieved in 6 of the 16 eyes (Total=43%, PCG=50%, DG=30%). Corneal diameter and axial length remained stable in all eyes. Two patients with DG needed additional surgery after 8 to 9 months. Complications included cataract and zonular dialysis both in 2 patients with aniridia. CONCLUSIONS: This pilot study indicates that EG shows reasonable potential for IOP control in congenital glaucoma. The safety and efficacy of EG needs to be further studied with a larger sample size and comparison to other angle surgery techniques.