Kirman Christian N, Tran Bryant, Sanger Claire, Railean Silvia, Glazier Steven S, David Lisa R
Department of Plastic and Reconstructive Surgery, Wake Forest University School of Medicine, Winston-Salem, North Carolina 27157-1075, USA.
J Craniofac Surg. 2011 Jul;22(4):1409-12. doi: 10.1097/SCS.0b013e31821cc50c.
Crouzon syndrome is an autosomal dominant disorder characterized by cranial synostosis, hypertelorism, orbital proptosis, parrot-beaked nose, short upper lip, hypoplastic maxilla, and a relative mandibular prognathism, without extremity involvement. Surgical intervention should occur at the onset of progressive craniosynostosis to treat or to prevent intracranial hypertension and visual impairment. Throughout developing countries, early treatment is often not a viable option. Often, the only option for treatment is through humanitarian missions. Appropriate preselection of surgical patients is essential, as is having a multidisciplinary team and a well-equipped hospital and staff to perform the operations and to care for the postsurgical patient. This type of humanitarian care benefits the patient selected to receive the intense logistical and financial effort when there is no possibility of timely care in their own countries. This clinical report describes a patient with Crouzon syndrome brought to the United States from a developing country through humanitarian efforts. She presented at 19 months of age with bicoronal and sagittal synostosis and advanced visual impairment and papilledema. Surgical intervention included cranial expansion and reconstruction with a multidisciplinary team. This case illustrates not only the difficulty of delayed treatment but also some of the issues arising from this type of humanitarian medical care.
克鲁宗综合征是一种常染色体显性疾病，其特征为颅骨缝早闭、眼距过宽、眼球突出、鹦鹉嘴样鼻、上唇短、上颌骨发育不全以及相对的下颌前突，不累及四肢。手术干预应在进行性颅骨缝早闭开始时进行，以治疗或预防颅内高压和视力损害。在整个发展中国家，早期治疗往往不是一个可行的选择。通常，唯一的治疗选择是通过人道主义医疗任务。对手术患者进行适当的预选至关重要，拥有一个多学科团队以及设备齐全的医院和工作人员来进行手术并护理术后患者也同样重要。当患者在本国无法获得及时治疗时，这种人道主义护理对被选中接受大量后勤和资金投入的患者有益。本临床报告描述了一名通过人道主义努力从发展中国家带到美国的克鲁宗综合征患者。她19个月大时出现双冠状缝和矢状缝早闭以及严重视力损害和视乳头水肿。手术干预包括由多学科团队进行颅骨扩张和重建。该病例不仅说明了延迟治疗的困难，还展示了这种人道主义医疗护理引发的一些问题。
