Difficulties of Delayed Treatment of Craniosynostosis in a Patient With Crouzon, Increased Intracranial Pressure, and Papilledema

作者：Kirman Christian N; Tran Bryant; Sanger Claire; Railean Silvia; Glazier Steven S; David Lisa R^*

来源：Journal of Craniofacial Surgery, 2011, 22(4): 1409-1412.

DOI：10.1097/SCS.0b013e31821cc50c

• Crouzon syndrome
• medical missions
• humanitarian collaboration
• cranial vault remodeling

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.

• 出版日期2011-7