Francis P M, Beals S, Rekate H L, Pittman H W, Manwaring K, Reiff J
Division of Neurological Surgery, Barrow Neurological Institute, Phoenix, Ariz 85013.
Pediatr Neurosurg. 1992;18(4):202-6. doi: 10.1159/000120663.
Patients born with craniofacial syndromes such as Crouzon's syndrome will often develop hydrocephalus after their initial craniofacial reconstructive procedures. We have treated 10 patients with Crouzon's syndrome; 5 patients required a shunting procedure after cranial remodeling. Each of these 5 shunted patients later demonstrated chronic tonsillar herniation on magnetic resonance imaging studies. One of these patients exhibited signs of pseudotumor cerebri and 1 had a spastic quadriparesis. Of the 5 patients who did not require a shunt, none displayed chronic tonsillar herniation. Our evidence suggests that jugular foramen stenosis produces an increased cerebral venous turgor that leads to a cerebrospinal fluid absorption defect and hydrocephalus. After the hydrocephalus is treated the increased venous turgor remains and provides the driving force for the development of chronic tonsillar herniation.
患有颅面综合征（如克鲁宗综合征）的患者在初次颅面重建手术后常发生脑积水。我们治疗了10例克鲁宗综合征患者；5例患者在颅骨重塑后需要进行分流手术。这5例接受分流手术的患者后来在磁共振成像研究中均显示出慢性扁桃体疝。其中1例患者出现了假性脑瘤的症状，1例出现痉挛性四肢瘫。在5例不需要分流的患者中，没有一例出现慢性扁桃体疝。我们的证据表明，颈静脉孔狭窄会导致脑静脉压力升高，进而导致脑脊液吸收缺陷和脑积水。在脑积水得到治疗后，升高的静脉压力仍然存在，并为慢性扁桃体疝的发展提供了驱动力。
