Hersh David S, Hoover-Fong Julie E, Beck Natalie, Dorafshar Amir H, Ahn Edward S
Department of Neurosurgery, University of Maryland School of Medicine; and.
McKusick-Nathans Institute of Genetic Medicine.
J Neurosurg Pediatr. 2017 Jul;20(1):91-98. doi: 10.3171/2017.2.PEDS16710. Epub 2017 May 5.
OBJECTIVE Recent reports have described early endoscopic suturectomy as a treatment option for patients with syndromic craniosynostosis, but such patients often require subsequent calvarial remodeling. The authors describe their experience with this patient population and seek to identify predictors of sufficiency of endoscopic surgery alone. METHODS The medical records of patients with syndromic craniosynostosis who underwent endoscopic repair were retrospectively reviewed. Demographic data, operative details, and follow-up data were collected. RESULTS A total of 6 patients with syndromic craniosynostosis underwent endoscopic surgery followed by helmet therapy during the study period. Of these, 3 patients were male. The involved syndromes included Crouzon, Pfeiffer, Jackson-Weiss, Muenke, Saethre-Chotzen, and craniosynostosis-3 (n = 1 each). The patients underwent endoscopic surgery at a median age of 2.1 months (range 0.9-4.1 months). The median estimated blood loss was 30 ml (range 20-100 ml), with 2 patients requiring a transfusion. The median length of stay in the hospital was 1.5 days (range 1-4 days), and the median follow-up was 29.0 months (range 16.8-81.7 months), with 1 patient (16.7%) requiring an open revision. Three patients (50%) were classified as Whitaker Category I at the last follow-up. The patients for whom additional open surgery was performed or recommended (Whitaker Category IV) were the oldest patients in the cohort, ranging from 2.6 to 4.1 months at the time of surgery. CONCLUSIONS This series demonstrates that endoscopic surgery can be sufficient to treat syndromic craniosynostosis without subsequent open calvarial remodeling over a median follow-up period of at least 2 years. The findings suggest that younger age at the time of endoscopic surgery may be an important factor in determining the sufficiency of this procedure. Even among patients who require subsequent open calvarial remodeling, early endoscopic surgery may allow for growth and development of the brain and skull while delaying the need for open remodeling until the patient is older and can better tolerate the procedure.
目的 近期报告描述了早期内镜下缝扎切除术可作为综合征性颅缝早闭患者的一种治疗选择,但此类患者通常需要后续的颅骨重塑。作者介绍了他们在这一患者群体中的经验,并试图确定仅通过内镜手术即可充分治疗的预测因素。方法 对接受内镜修复的综合征性颅缝早闭患者的病历进行回顾性分析。收集人口统计学数据、手术细节和随访数据。结果 在研究期间,共有6例综合征性颅缝早闭患者接受了内镜手术,随后进行头盔治疗。其中3例为男性。所涉及的综合征包括克鲁宗综合征、菲佛综合征、杰克逊-韦斯综合征、蒙克综合征、塞特勒-乔岑综合征和颅缝早闭-3型(各1例)。患者接受内镜手术的中位年龄为2.1个月(范围0.9 - 4.1个月)。估计中位失血量为30毫升(范围20 - 100毫升),2例患者需要输血。中位住院时间为1.5天(范围1 - 4天),中位随访时间为29.0个月(范围16.8 - 81.7个月),1例患者(16.7%)需要进行开放性翻修手术。在最后一次随访时,3例患者(50%)被归类为惠特克I类。需要进行或建议进行额外开放性手术的患者(惠特克IV类)是该队列中年龄最大的患者,手术时年龄在2.6至4.1个月之间。结论 本系列研究表明,在内镜手术至少2年的中位随访期内,内镜手术足以治疗综合征性颅缝早闭,无需后续的开放性颅骨重塑。研究结果表明,内镜手术时年龄较小可能是决定该手术是否充分的一个重要因素。即使在需要后续开放性颅骨重塑的患者中,早期内镜手术也可能有助于大脑和颅骨的生长发育,同时将开放性重塑的需求推迟到患者年龄较大且能更好耐受该手术时。