Management of large scalp and skull defects in a severe case of Adams-Oliver syndrome

Case report

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  • 1 Departments of Neurosurgery and
  • 2 Radiology, Hackensack University Medical Center, Hackensack, and
  • 3 Section of Pediatric Neurosurgery, Saint Barnabas Medical Center, Livingston, New Jersey;
  • 4 Department of Surgery (Plastic Surgery), Weill Cornell Medical College, New York-Presbyterian Hospital, New York, New York;
  • 5 Boston University, Boston, Massachusetts; and
  • 6 Department of Neurosurgery, Children's Hospital Medical Center, Tehran University of Medical Sciences, Tehran, Iran
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Adams-Oliver syndrome is a rare congenital disorder that includes congenital scalp and skull defects, variable degrees of terminal transverse limb anomalies, and cardiac malformations. Cutis aplasia occurring in 75% of patients is a potentially life-threatening condition. Large skin defects that cannot be closed primarily present a management dilemma, and may require skin grafting or flaps, or a combination of both operative and conservative modalities. The authors' experience in management of huge scalp and bone defects with the Integra Dermal Regeneration Template and regular dressing changes showed good scalp repair and no serious complications attributed to this approach.

Abbreviation used in this paper: AOS = Adams-Oliver syndrome.

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Contributor Notes

Address correspondence to: Farideh Nejat, M.D., Mailbox: F. Nejat, Tehran, 14155-7854 Iran. email: nejat@sina.tums.ac.ir.
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