Takano S, Enomoto T, Onizuka H, Nose T
Neurol Med Chir (Tokyo). 1989 Aug;29(8):746-52. doi: 10.2176/nmc.29.746.
Two cases of cloverleaf skull syndrome are reported. The patients were born with trilobed deformity of the skull associated with synostosis of the lambdoidal, coronal, and sagittal sutures in Case 1 and of the lambdoidal suture in Case 2. Both patients had hydrocephalus, low-set ears, and ocular hypertelorism. Ventriculoperitoneal shunting had been performed for hydrocephalus in both cases in early infancy. Thereafter, the skull anomaly caused mental and physical retardation and elevated intracranial pressure. In both cases, surgery was successful in terms of cosmetic and neurological improvement. Both cases involved prominent midline crista formation of the skull over the posterior fossa, which steadily progressed during the first year of life and produced hydrocephalus, apparently by compressing or obstructing the aqueduct and/or the ostia of the fourth ventricle. Twenty-four cases of surgery for cloverleaf skull syndrome have been reported to date. Only Partington's types II and III were treated surgically, and patients with type III had more favorable outcomes than those with type II. However, these reports provide no clear-cut data that surgery significantly influences the outcome. The experience with the two cases reported here suggests the benefits of early shunting followed later by radical surgery.
本文报告了两例三叶草颅骨综合征病例。病例1患者出生时颅骨呈三叶形畸形，伴有人字缝、冠状缝和矢状缝早闭；病例2患者仅有人字缝早闭。两名患者均患有脑积水、低位耳和眼距过宽。两例患者均在婴儿早期因脑积水接受了脑室腹腔分流术。此后，颅骨异常导致智力和身体发育迟缓以及颅内压升高。在这两个病例中，手术在改善外观和神经功能方面均取得了成功。两例病例均出现后颅窝上方颅骨中线嵴突出，在生命的第一年稳步进展，并显然通过压迫或阻塞导水管和/或第四脑室的开口导致脑积水。迄今为止，已报道了24例三叶草颅骨综合征手术病例。仅对帕廷顿II型和III型进行了手术治疗，III型患者的预后比II型患者更好。然而，这些报告没有提供明确的数据表明手术对预后有显著影响。本文报告的两例病例的经验表明，早期分流随后进行根治性手术是有益的。
