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Extreme aplasia cutis congenita involving the skull

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Abstract

Aplasia cutis congenita (ACC) is a rare congenital malformation of primarily the skin; it is most commonly seen on the scalp but can occur anywhere on the body. The exact etiology is still unclear but there are many suggested causes. Classification systems have been proposed to help categorize patients and assist with treatment. Treatment options are controversial and range from conservative to surgical interventions. We report an extreme case of ACC that included a significant part of the skull. We discuss this case and review salient literature. Although such cases of ACC with bony involvement are rare, this aspect of the pathology should be kept in mind when treating or imaging such patients.

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Authors and Affiliations

  1. Department of Anatomical Sciences, St. George’s University, St. George, Grenada

    Sebastian Shrager & R. Shane Tubbs

  2. Seattle Science Foundation, 550 17th Ave, James Tower, Suite 600, Seattle, WA, 98122, USA

    Vlad Voin, Joe Iwanaga & R. Shane Tubbs

  3. Pediatric Neurosurgery, Children’s of Alabama, Birmingham, AL, USA

    James Johnston

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  1. Sebastian Shrager
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  2. Vlad Voin
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  3. Joe Iwanaga
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  4. R. Shane Tubbs
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  5. James Johnston
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Correspondence to Vlad Voin.

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On behalf of all authors, the corresponding author states that there is no conflict of interest.

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Shrager, S., Voin, V., Iwanaga, J. et al. Extreme aplasia cutis congenita involving the skull. Childs Nerv Syst 33, 1395–1398 (2017). https://doi.org/10.1007/s00381-017-3426-x

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  • DOI: https://doi.org/10.1007/s00381-017-3426-x

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