Saffra Norman A, Emborgo Trisha S, Ranka Milan P, Kirsch David S
Department of Ophthalmology, Maimonides Medical Center, 4802 10th Ave, Brooklyn, NY, 11219, United States.
Department of Ophthalmology, St. John's Episcopal Hospital, 327 Beach 19th St, Far Rockaway, New York, 11691, United States.
Am J Ophthalmol Case Rep. 2023 Jul 20;32:101904. doi: 10.1016/j.ajoc.2023.101904. eCollection 2023 Dec.
We present a case of a 10-year-old boy with Crouzon syndrome that demonstrates progressive myelinated retinal nerve fibers (MRNF).
A 10-year-old boy was referred for ophthalmic examination due to clusters of opaque white fibers around his optic nerve. Past surgical history includes craniofacial surgery at 3 years of age secondary to the deteriorating vision from increased intracranial pressure and papilledema. Upon examination (now 6.5 years post-craniofacial surgery), the patient denied any ocular complaints. The fundus examination showed progressively enlarged myelination of the retinal nerve fiber layer (right eye > left eye). Although previous cases of MRNF with Crouzon syndrome have been reported, our case is unique given its post-operative status with early onset of MRNF.
This case report documents the photographic progression of bilateral myelinated retinal nerve fibers (MRNF) in a pediatric case of Crouzon syndrome post-craniofacial surgery secondary to increased intracranial pressure and papilledema. Based on our patient, craniofacial decompression surgery may not prevent the development of MRNF. The exact mechanisms of MRNF are still being studied. Further investigations correlating craniofacial surgeries, increased intracranial pressure, and progression of myelinated retinal nerve fibers are needed to understand this process.
我们报告一例10岁患有克鲁宗综合征的男孩，其表现为进行性有髓鞘视网膜神经纤维（MRNF）。
一名10岁男孩因视神经周围出现成簇的不透明白色纤维而被转诊进行眼科检查。既往手术史包括3岁时因颅内压升高和视乳头水肿导致视力恶化而进行的颅面手术。检查时（颅面手术后6.5年），患者否认有任何眼部不适。眼底检查显示视网膜神经纤维层的髓鞘化逐渐扩大（右眼>左眼）。虽然之前已有克鲁宗综合征合并MRNF的病例报道，但我们的病例因其术后状态及MRNF的早期发病而独具特点。
本病例报告记录了一名患有克鲁宗综合征的儿童在因颅内压升高和视乳头水肿接受颅面手术后双侧有髓鞘视网膜神经纤维（MRNF）的影像学进展情况。根据我们的患者情况，颅面减压手术可能无法预防MRNF的发生。MRNF的确切机制仍在研究中。需要进一步研究颅面手术、颅内压升高与有髓鞘视网膜神经纤维进展之间的关系，以了解这一过程。
