title: Repeat Intracranial Expansion After Skull Regrowth in Hyperostotic Disease: Technical Note.
abstract: OBJECTIVE
  Camurati-Engelmann disease (CED) is a rare, autosomal-dominant genetic disorder resulting in hyperostosis of the long bones and skull. Patients often develop cranial nerve dysfunction and increased intracranial pressure secondary to stenosis of nerve foramina and hyperostosis. Surgical decompression may provide symptomatic relief in select patients; however, a small number of reports document the recurrence of symptoms due to bony regrowth. We present a patient who had been treated previously with bilateral frontal and parietal craniotomy who experienced recurrence of symptoms due to reossification of her cranial bones. This report underscores the progressive nature of CED and its influence on surgical management. Furthermore, we propose a novel surgical approach with multiple craniectomies and titanium mesh cranioplasties that could potentially offer long-term symptomatic relief.
  
  
  METHODS
  A 46-year-old female patient with CED who was treated with ventriculoperitoneal shunting, posterior fossa decompression, and multiple craniotomies 2 decades prior presented with signs and symptoms of increased intracranial pressure. Studies of the skull at presentation demonstrated rethickening of cranial bones that resulted in severely decreased intracranial volume.
  
  
  METHODS
  A radical craniectomy, requiring 4 separate bone flaps made up of bilateral frontal and parietal bones, was performed. The remaining coronal and sagittal bony struts were drilled to approximately 1 cm thick. Cranioplasties with 4 separate titanium meshes were performed to preserve the natural contour of the patient's skull.
  
  
  CONCLUSIONS
  Although surgical decompression could provide some patients with CED symptomatic relief, clinicians should consider managing CED as a chronic condition. To the authors' knowledge, this is one of few case reports documenting the recurrence of symptoms in a patient with CED treated by surgical intervention. Furthermore, we propose that multiple craniectomies with titanium mesh cranioplasties confer more permanent symptomatic control, and, more importantly, lower the risk of recurrence secondary to cranial hyperostosis.
authors.affiliation: Rutgers, Robert Wood Johnson Medical School, New Brunswick, New Jersey, USA. Electronic address: twchunghin@gmail.com.
authors.forename: Timothy
authors.initials: T
authors.lastname: Wong
authors.affiliation: Rutgers, New Jersey Medical School, Newark, New Jersey, USA.
authors.forename: Yehuda
authors.initials: Y
authors.lastname: Herschman
authors.affiliation: Rutgers, New Jersey Medical School, Newark, New Jersey, USA.
authors.forename: Nitesh V
authors.initials: NV
authors.lastname: Patel
authors.affiliation: Rutgers, Robert Wood Johnson University Hospital, New Brunswick, New Jersey, USA.
authors.forename: Tushar
authors.initials: T
authors.lastname: Patel
authors.affiliation: Rutgers, Cancer Institute of New Jersey, New Brunswick, New Jersey, USA.
authors.forename: Simon
authors.initials: S
authors.lastname: Hanft
mesh_terms: D003966:Camurati-Engelmann Syndrome / Q000000981:diagnostic imaging / Q000503:physiopathology / Q000601:surgery*; D003399:Craniotomy / Q000379:methods; D019299:Decompression, Surgical / Q000379:methods; D005260:Female; D006801:Humans; D015576:Hyperostosis / Q000000981:diagnostic imaging / Q000209:etiology / Q000503:physiopathology*; D008875:Middle Aged; D011182:Postoperative Care / Q000379:methods; D011183:Postoperative Complications / Q000209:etiology / Q000503:physiopathology; D012886:Skull / Q000254:growth & development*; D013524:Surgical Flaps; D014057:Tomography, X-Ray Computed; D016896:Treatment Outcome; D017287:Ventriculoperitoneal Shunt / Q000379:methods
keywords: Hyperostosis; Intracranial expansion; Skull Remodeling