Paliga James Thomas, Goldstein Jesse A, Storm Phillip B, Taylor Jesse Adam
Division of Plastic Surgery, The Perelman School of Medicine at theUniversity of Pennsylvania, The Children's Hospital of Philadelphia, Philadelphia, Pennsylvania 19104, USA.
J Craniofac Surg. 2013 Mar;24(2):596-8. doi: 10.1097/SCS.0b013e318280233d.
Treatment of the Apert syndrome phenotype aims to correct airway obstruction, exorbitism, elevated intracranial pressure, midface hypoplasia, and malocclusion. Cranial vault expansion prevents elevated intracranial pressure, normalizes head shape, and protects the globes, but variation exists in surgical timing and osteotomy to treat the midface. We present the case of an 11-year-old female patient with Apert syndrome and no prior surgical interventions who presented with severe turribrachycephaly, exorbitism, severe midface retrusion, and apertognathia. A monobloc distraction with simultaneous Le Fort II distraction was planned using computer-aided design and modeling (CAD/CAM) techniques to provide for concurrent distraction of the segments in independent vectors without bony interferences.Monobloc minus Le Fort II distraction was performed without intraoperative complications. Surgical time was 340 minutes with an estimated blood loss of 1100 mL. Distraction began on postoperative day 5 at a rate of 1.5 mm/day for the Le Fort II via an external Halo distractor and 1 mm/day for the monobloc segment via internal distractors anchored bitemporally. The monobloc was distracted a total of 17 mm in a horizontal vector, while the Le Fort II segment was distracted 18 mm horizontally and 5 mm inferiorly. The Halo distractor was removed 3 months following the procedure and the internal distractors 1 month later. Monobloc minus Le Fort II distraction enables correction of the Apert phenotype with a single-stage approach, potentially decreasing the burden of care with improved results. Utilization of CAD/CAM modeling allows for accurate planning of multisegment distraction in independent vectors without concerns for bony interferences.
Apert综合征表型的治疗旨在纠正气道阻塞、眼球突出、颅内压升高、面中部发育不全和咬合不正。颅骨穹窿扩张可预防颅内压升高、使头型正常化并保护眼球，但在治疗面中部的手术时机和截骨术方面存在差异。我们报告了一例11岁的Apert综合征女性患者，此前未接受过手术干预，表现为严重的短头畸形、眼球突出、严重的面中部后缩和开颌。计划采用计算机辅助设计和建模（CAD/CAM）技术进行整块牵张并同时进行Le Fort II型牵张，以在无骨干扰的情况下在独立向量中同时牵张各节段。进行了整块减Le Fort II型牵张，术中无并发症。手术时间为340分钟，估计失血量为1100毫升。牵张于术后第5天开始，通过外部Halo牵张器以每天1.5毫米的速度对Le Fort II型进行牵张，通过双侧颞部固定的内部牵张器以每天1毫米的速度对整块节段进行牵张。整块节段在水平向量上总共牵张了17毫米，而Le Fort II型节段在水平方向上牵张了18毫米，在下方牵张了5毫米。术后3个月移除Halo牵张器，1个月后移除内部牵张器。整块减Le Fort II型牵张能够通过单阶段方法纠正Apert综合征表型，可能减轻护理负担并改善治疗效果。利用CAD/CAM建模可在无骨干扰的情况下对独立向量中的多节段牵张进行精确规划。
