Department of Maxillofacial Surgery, Clocheville Hospital, Centre Hospitalier Universitaire de Tours, Tours, France; Université of Medecine François Rabelais, Tours, France.
Department of Maxillofacial Surgery, Clocheville Hospital, Centre Hospitalier Universitaire de Tours, Tours, France; Université of Medecine François Rabelais, Tours, France.
World Neurosurg. 2019 Sep;129:1-4. doi: 10.1016/j.wneu.2019.05.207. Epub 2019 May 29.
Patients with Crouzon syndrome are mainly treated in childhood by frontofacial monobloc advancement to avoid ophthalmic, neurologic, and maxillary complications. There is no reported case of surgery on adult patients with Crouzon syndrome in the literature. However, when faced with 2 cases of adult patients showing severe quality of life deterioration, our team decided to make an attempt using monobloc advancement technique.
Two women aged 41 and 56 presented with untreated Crouzon syndrome and suffered from exorbitism, intracranial hypertension with chronic headaches, and hypoplastic maxillary. We decided to perform frontofacial monobloc advancement with internal distraction despite their advanced age using planned surgery and cutting guides. Distraction began 7-10 days after surgery and was of 15 mm. Distractors were taken off at 6 months. Surgical treatment corrected chronic headaches, ocular symptoms due to exorbitism, and hypoplastic maxillary. Patients were satisfied with the functional and aesthetic results. We noticed that this heavy surgery was more difficult to bear by these adults than children.
Adults with craniofacial malformations have a lower self-esteem, lower quality of life, and less satisfaction with their facial look as compared with individuals without facial malformations. There is also an increased risk of psychosocial problems. Despite postoperative difficulties and minor complications, our 2 patients were satisfied with the functional and aesthetic results. This led to the conclusion that surgically addressing adult patients with Crouzon syndrome via monobloc advancement is appropriate and secure when performed by a trained team.
Crouzon 综合征患者主要在儿童期通过额眶一体推进术进行治疗，以避免眼部、神经和上颌并发症。文献中尚无 Crouzon 综合征成年患者手术的报道。然而，当面对 2 例生活质量严重恶化的成年患者时，我们团队决定尝试使用单块推进技术。
2 名 41 岁和 56 岁女性患有未治疗的 Crouzon 综合征，表现为眼球突出、颅内压增高伴慢性头痛和上颌发育不良。尽管年龄较大，我们仍决定使用计划手术和切割引导器进行额眶一体推进内牵引。术后 7-10 天开始牵引，牵引距离为 15mm。6 个月后取出牵引器。手术治疗纠正了慢性头痛、眼球突出引起的眼部症状和上颌发育不良。患者对功能和美学效果满意。我们注意到，与儿童相比，这种大手术对这些成年人来说更难承受。
与无面部畸形的个体相比，颅面畸形的成年人自尊心较低、生活质量较低、对面部外观的满意度较低，并且发生心理社会问题的风险增加。尽管术后困难和轻微并发症，我们的 2 名患者对功能和美学效果满意。因此得出结论，由经过培训的团队通过单块推进术治疗 Crouzon 综合征成年患者是合适且安全的。
