Clarke D B, Farmer J P, Montes J L, Watters G V, Rouleau G
Division of Neurosurgery, Montreal Children's Hospital, McGill University, Quebec, Canada.
Can J Neurol Sci. 1994 Feb;21(1):64-6. doi: 10.1017/s0317167100048800.
The authors report, for the first time, the finding by magnetic resonance imaging of a neurofibroma at the craniocervical junction with upper cervical cord and lower brainstem compression causing complete apnea from birth. Subsequent subtotal resection of the neurofibroma resulted in the successful extubation of a previously ventilator-dependent patient. After a two month period of breathing spontaneously, the newborn developed an upper respiratory tract infection and was reintubated. The patient, unable to be weaned off of the respirator, was extubated and expired shortly thereafter, at the age of five months. The authors suggest that in newborns with unexplained apnea, MRI of the cranio-cervical junction is indicated. Certain patients may be discovered who have less compromised cervico-medullary function and are afflicted by less aggressive forms of neurofibromatosis type 1. These patients may benefit permanently from a surgical decompression.
作者首次报告了通过磁共振成像发现颅颈交界处有神经纤维瘤，该神经纤维瘤压迫颈上段脊髓和脑桥下部，导致患儿自出生起即出现完全性呼吸暂停。随后对神经纤维瘤进行次全切除，使一名此前依赖呼吸机的患者成功脱机。在自主呼吸两个月后，该新生儿患上了上呼吸道感染，再次接受插管。该患者无法撤掉呼吸机，拔管后不久，于五个月大时死亡。作者建议，对于原因不明的新生儿呼吸暂停患者，应进行颅颈交界处的磁共振成像检查。可能会发现某些患者的颈髓功能受损较轻，且患有侵袭性较小的1型神经纤维瘤病。这些患者可能会从手术减压中永久受益。
