Hart Justin, Lu Stephen, Gasteratos Konstantinos, Chaiyasate Kongkrit
Plastic and Reconstructive Surgery, Beaumont Hospital, Royal Oak, Mich.
Plastic and Reconstructive Surgery, Papageorgiou General Hospital, Thessaloniki, Greece.
Plast Reconstr Surg Glob Open. 2021 Nov 2;9(11):e3869. doi: 10.1097/GOX.0000000000003869. eCollection 2021 Nov.
Crouzon syndrome (CS) is a rare form of craniosynostosis characterized by bicoronal craniosynostosis and facial features including severe midface hypoplasia, exophthalmos, and hypertelorism. Most patients are diagnosed and treated in early childhood; however, there are a few reports of Crouzon patients treated as adults with monobloc facial advancement. To our knowledge, this is the first report of a family affected by CS treated sequentially with monobloc facial advancement using combined internal and external distraction osteogenesis (rigid external distraction).
We present a family from Jamaica (mother 47 years old, older daughter 17, and younger daughter 9) who were brought to our craniofacial clinic with stigmata of CS and no previous surgical intervention. Patients had bicoronal craniosynostosis and exorbitism, with varying severity, sequelae, and comorbidities. Here, we delineate our technique of monobloc distraction osteogenesis with advancement osteotomies using dual "push-pull" method, elevation of a split anteriorly based tunneled pericranial flap to seal off nasal cavity, and internal and external distraction.
Our patients had favorable outcomes after reconstruction to reduce ocular symptoms and improve midface hypoplasia and aesthetic appearance. No intracranial injury, hardware/soft-tissue infection, hardware failure, or (new) loss of vision were encountered in 10 months follow-up.
Dual "push-pull" monobloc distraction is safe and effective for a range of ages in CS; it allows good vector control, accommodates patient compliance, and allows early rigid external distraction device removal with sufficient time for consolidation. This surgery can be performed with highly satisfactory results.
克鲁宗综合征（CS）是一种罕见的颅缝早闭形式，其特征为双侧冠状缝颅缝早闭以及面部特征，包括严重的面中部发育不全、眼球突出和眼距增宽。大多数患者在幼儿期被诊断和治疗；然而，有少数关于成年克鲁宗患者接受整块面部前移治疗的报道。据我们所知，这是首例报道的一个受CS影响的家族，该家族依次接受了使用内外联合牵引成骨术（坚固外牵引）的整块面部前移治疗。
我们介绍一个来自牙买加的家族（母亲47岁，大女儿17岁，小女儿9岁），他们因CS体征被带到我们的颅面诊所，且之前未接受过手术干预。患者有双侧冠状缝颅缝早闭和眼球突出，严重程度、后遗症及合并症各不相同。在此，我们描述了使用双“推 - 拉”法进行整块牵引成骨术并进行截骨前移、掀起基于前方劈开的隧道式颅骨膜瓣以封闭鼻腔以及内外牵引的技术。
我们的患者在重建后取得了良好的效果，减轻了眼部症状，改善了面中部发育不全和美观外观。在10个月的随访中未出现颅内损伤、硬件/软组织感染、硬件故障或（新的）视力丧失。
双“推 - 拉”整块牵引术对不同年龄段的CS患者安全有效；它能实现良好的矢量控制，适应患者的依从性，并能在有足够时间进行骨痂形成的情况下早期拆除坚固外牵引装置。该手术可取得非常令人满意的结果。
