Acalvaria

Case report

Ammar H. HawasliDepartment of Neurosurgery and

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Thomas L. BeaumontDepartment of Neurosurgery and

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Timothy W. VogelDivision of Pediatric Neurosurgery, University of Cincinnati, Ohio

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Albert S. WooDivision of Plastic and Reconstructive Surgery, Washington University School of Medicine, St. Louis, Missouri; and

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Jeffrey R. LeonardDepartment of Neurosurgery and

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Acalvaria is a rare congenital malformation characterized by an absence of skin and skull. The authors describe a newborn at an estimated 38 weeks gestational age who was delivered via cesarean section from a 32-year-old mother. Upon delivery, the child was noted to have a frontal encephalocele and an absence of calvaria including skull and skin overlying the brain. A thin membrane representing dura mater was overlying the cortical tissue. After multiple craniofacial operations, including repair of the encephalocele and application of cultured keratinocytes over the rostral defect, the patient demonstrated significant closure of the calvarial defect and was alive at an age of more than 17 months with near-average development.

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  • 1

    Gaynor BG, , Benveniste RJ, , Lieberman S, , Casiano R, & Morcos JJ: Acellular dermal allograft for sellar repair after transsphenoidal approach to pituitary adenomas. J Neurol Surg B Skull Base 74:155159, 2013

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  • 2

    Gupta V, & Kumar S: Acalvaria: a rare congenital malformation. J Pediatr Neurosci 7:185187, 2012

  • 3

    Harris CP, , Townsend JJ, & Carey JC: Acalvaria: a unique congenital anomaly. Am J Med Genet 46:694699, 1993

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    Kurata H, , Tamaki N, , Sawa H, , Oi S, , Katayama K, & Mochizuki M, et al.: Acrania: report of the first surviving case. Pediatr Neurosurg 24:5254, 1996

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    Sittitavornwong S, & Morlandt AB: Reconstruction of the scalp, calvarium, and frontal sinus. Oral Maxillofac Surg Clin North Am 25:105129, 2013

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