Al-Mefty O, Fox J L, Al-Rodhan N, Dew J H
Department of Neurosurgery, University of Mississippi Medical Center, Jackson.
J Neurosurg. 1988 Jan;68(1):80-4. doi: 10.3171/jns.1988.68.1.0080.
Osteopetrosis is a rare disorder characterized by generalized increased skeletal density with abnormalities of bone modeling. The skull base is usually involved. Loss of vision with optic nerve atrophy is the most common neurological finding and is traditionally believed to be the result of optic nerve compression within the compromised optic canal. However, retinal degeneration has recently been described and is hypothesized to be the etiology of the visual loss (thus challenging the value of surgical decompression). The authors report their experience with six children suffering from osteopetrosis and severe visual loss. All six patients underwent bilateral microsurgical optic nerve decompression through a supraorbital craniotomy. Improvement in visual acuity occurred postoperatively in five patients, and none had complications. Two technical points are emphasized: 1) optic nerve decompression should be wide and include not only unroofing of the canal but also drilling along both sides of the optic nerve, and 2) the thick, irregular, and highly domed orbital roof should be smoothed down by high-speed drilling to facilitate surgical exposure with minimal retraction of the frontal lobe.
骨质石化症是一种罕见的病症，其特征为全身骨骼密度增加以及骨重塑异常。颅底通常会受累。视神经萎缩导致的视力丧失是最常见的神经学表现，传统上认为这是由于视神经在狭窄的视神经管内受压所致。然而，最近有报道称存在视网膜变性，并推测这是视力丧失的病因（从而对手术减压的价值提出了挑战）。作者报告了他们对六名患有骨质石化症且视力严重丧失的儿童的治疗经验。所有六名患者均通过眶上开颅术接受了双侧显微视神经减压术。五名患者术后视力得到改善，且无一例出现并发症。强调了两个技术要点：1）视神经减压范围应广泛，不仅要去除神经管顶，还应沿视神经两侧钻孔；2）应通过高速钻孔将厚的、不规则的和高度隆起的眶顶磨平，以便在额叶最少牵拉的情况下便于手术暴露。
