Park Seong-Cheol, Kim Seung-Ki, Cho Byung-Kyu, Kim Hyun Jib, Kim Jeong Eun, Phi Ji Hoon, Kim In-One, Wang Kyu-Chang
Department of Neurosurgery, Seoul National University College of Medicine, Seoul, Republic of Korea.
J Neurosurg Pediatr. 2009 Dec;4(6):536-42. doi: 10.3171/2009.7.PEDS0994.
Sinus pericranii (SP) is a rare venous varix in an extracranial location connected to the intracranial venous system. The aim of this retrospective study was to report on 16 pediatric cases of SP with consideration of the preoperative evaluation of surgical risk.
The study population consisted of 10 patients who had undergone surgery for SP and 6 patients with concomitant craniosynostosis and SP. The mean age of the patients at presentation was 3.7 years. To identify characteristics of SP with high operative risk, 8 cases in this report and 11 previously reported cases of SP with sufficient information were categorized on the basis of the number and size of SP, the number and size of transcranial channels, the venous drainage type, and the amount of blood loss. Hemorrhage amounts were classified into 3 grades based on the description of intraoperative blood loss.
Sinus pericranii not associated with craniosynostosis were resected without any postoperative morbidity. Sinus pericranii associated with craniosynostosis were preserved. After craniofacial reconstruction, 2 cases of SP with craniosynostosis regressed, completely in one patient and partially in another. These 2 patients with SP were confirmed to have compromised intracranial sinus before craniofacial reconstruction. Among a total of 19 patients, multiplicity or size (> 6 cm) of SP (p = 0.036) and multiplicity (> 3) or size (> 3 mm) of transcranial channels (p = 0.004) was associated with more severe hemorrhage grade. Sinus pericranii with peripheral venous drainage (drainer type) was not associated with hemorrhage grade after classification into 3 grades (p = 0.192). However, all 3 cases of SP with massive Grade 3 hemorrhage were the drainer type. Hemorrhage grade was correlated with the number of risk factors for SP (r = 0.793, p < 0.001).
Three risk factors of SP and the presence of compromised intracranial sinus are markers for highrisk SP. "Squeezed-out sinus syndrome" is suggested as a concept for SP associated with compromised intracranial sinus, mainly caused by craniosynostosis. Sinus pericranii in squeezed-out sinus syndrome probably serves as a crucial alternative to venous drainage of the brain with intracranial venous compromise. Conservative treatment for such patients with SP is recommended.
颅骨膜窦（SP）是一种罕见的位于颅外并与颅内静脉系统相连的静脉血管畸形。本回顾性研究的目的是报告16例儿童颅骨膜窦病例，并对手术风险进行术前评估。
研究对象包括10例行颅骨膜窦手术的患者和6例合并颅缝早闭及颅骨膜窦的患者。患者就诊时的平均年龄为3.7岁。为确定手术风险高的颅骨膜窦的特征，根据本报告中的8例及之前报告的11例信息充分的颅骨膜窦病例，按照颅骨膜窦的数量和大小、经颅通道的数量和大小、静脉引流类型及失血量进行分类。根据术中失血量的描述，将失血量分为3级。
不合并颅缝早闭的颅骨膜窦切除后无任何术后并发症。合并颅缝早闭的颅骨膜窦予以保留。颅面重建后，2例合并颅缝早闭的颅骨膜窦有所消退，1例完全消退，另1例部分消退。这2例颅骨膜窦患者在颅面重建前被证实存在颅内窦受损。在总共19例患者中，颅骨膜窦的多发性或大小（>6 cm）（p = 0.036）以及经颅通道的多发性（>3个）或大小（>3 mm）（p = 0.004）与更严重的出血分级相关。分为3级后，外周静脉引流型（引流器类型）的颅骨膜窦与出血分级无关（p = 0.192）。然而，所有3例3级大量出血的颅骨膜窦病例均为引流器类型。出血分级与颅骨膜窦的风险因素数量相关（r = 0.793，p < 0.001）。
颅骨膜窦的三个风险因素及颅内窦受损是高风险颅骨膜窦的标志。“挤压出窦综合征”被提出作为与颅内窦受损相关的颅骨膜窦的一个概念，主要由颅缝早闭引起。挤压出窦综合征中的颅骨膜窦可能是颅内静脉受损时大脑静脉引流的关键替代途径。建议对这类颅骨膜窦患者进行保守治疗。
