Goldstein Jesse A, Paliga James Thomas, Bailey Robert L, Heuer Gregory G, Taylor Jesse A
Department of Plastic Surgery, Children's Hospital of Philadelphia, The Perelman School of Medicine at the University of Pennsylvania, Philadelphia, Pennsylvania 19104, USA.
J Craniofac Surg. 2013 Jul;24(4):1263-7. doi: 10.1097/SCS.0b013e318286081f.
Syndromic craniosynostosis (SC) may cause intracranial hypertension, exorbitism, midfacial hypoplasia associated with airway obstruction, and class III malocclusion. Current treatment strategies for SC involve expansion of the cranial vault and orbits, and midfacial advancement to relieve symptoms and sequelae of obstructive sleep apnea. We present a case of a 12-week-old female patient with Saethre-Chotzen syndrome who presented with bicoronal craniosynostosis, severe turribrachycephaly, midface hypoplasia, and a history of obstructive sleep apnea (apnea-hypopnea index = 14). Posterior vault distraction and simultaneous transfacial pin placement under 3D CT surgical navigation was planned to concurrently relieve intracranial pressure and address airway obstruction by distraction of the midface without osteotomy. An uncomplicated surgery was completed in 290 minutes with an estimated blood loss of 340 mL. Midface distraction was initiated on postoperative day 1 at a rate of 1 mm per day for 21 days, which translated to 13 mm of midfacial advancement. Posterior vault distraction was initiated on postoperative day 5 at a rate of 1 mm per day for 25 days with a resultant 25 mm of posterior vault expansion. Transfacial pin and external distractors were removed after 6 weeks, and posterior vault distractors were removed after 8 weeks of consolidation. Craniometric comparison of 3D-CT scan demonstrated an increase in cranial vault volume of 47%, and the patient was able to be weaned from her home oxygen requirement. This is the first report of simultaneous posterior vault distraction with midfacial advancement across open facial sutures using surgical navigation as an initial stage in treating syndromic craniosynostosis.
综合征性颅缝早闭（SC）可能导致颅内高压、眼球突出、与气道阻塞相关的面中部发育不全以及Ⅲ类错牙合畸形。目前SC的治疗策略包括扩大颅穹窿和眼眶，以及面中部前移以缓解阻塞性睡眠呼吸暂停的症状和后遗症。我们报告一例12周龄患有塞-乔综合征的女性患者，其表现为双冠状颅缝早闭、严重的短头畸形、面中部发育不全以及阻塞性睡眠呼吸暂停病史（呼吸暂停低通气指数=14）。计划在3D CT手术导航下进行颅后穹窿牵引并同时经面部置入牵引钉，以通过面中部牵引而不进行截骨术来同时缓解颅内压并解决气道阻塞问题。手术顺利完成，耗时290分钟，估计失血量为340 mL。术后第1天开始进行面中部牵引，每天牵引速度为1 mm，持续21天，相当于面中部前移13 mm。术后第5天开始进行颅后穹窿牵引，每天牵引速度为1 mm，持续25天，颅后穹窿扩张了25 mm。6周后取出经面部牵引钉和外部牵引器，8周巩固期后取出颅后穹窿牵引器。3D-CT扫描的颅骨测量比较显示颅穹窿体积增加了47%，并且患者能够停止家庭吸氧需求。这是首例在治疗综合征性颅缝早闭的初始阶段，利用手术导航同时进行颅后穹窿牵引与经开放面部缝线的面中部前移的报告。
