title: Difficulties of delayed treatment of craniosynostosis in a patient with Crouzon, increased intracranial pressure, and papilledema.
abstract: 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.
authors.affiliation: Department of Plastic and Reconstructive Surgery, Wake Forest University School of Medicine, Winston-Salem, North Carolina 27157-1075, USA.
authors.forename: Christian N
authors.initials: CN
authors.lastname: Kirman
authors.forename: Bryant
authors.initials: B
authors.lastname: Tran
authors.forename: Claire
authors.initials: C
authors.lastname: Sanger
authors.forename: Silvia
authors.initials: S
authors.lastname: Railean
authors.forename: Steven S
authors.initials: SS
authors.lastname: Glazier
authors.forename: Lisa R
authors.initials: LR
authors.lastname: David
mesh_terms: D020341:Absorbable Implants; D001860:Bone Plates; D003393:Cranial Sutures / Q000002:abnormalities; D003394:Craniofacial Dysostosis / Q000150:complications*; D003398:Craniosynostoses / Q000150:complications / Q000601:surgery*; D003399:Craniotomy / Q000379:methods; D005260:Female; D005500:Follow-Up Studies; D005624:Frontal Bone / Q000002:abnormalities; D006801:Humans; D007223:Infant; D019586:Intracranial Hypertension / Q000150:complications*; D008493:Medical Missions; D009915:Orbit / Q000601:surgery; D010211:Papilledema / Q000150:complications*; D010294:Parietal Bone / Q000002:abnormalities; D019651:Plastic Surgery Procedures / Q000379:methods; D013997:Time Factors; D017287:Ventriculoperitoneal Shunt; D014786:Vision Disorders / Q000150:complications