Your browser does not support javascript:   Search for gard hereSearch for news-and-events here.

Diseases

Genetic and Rare Diseases Information Center (GARD)

Aplasia cutis congenita


Other Names for this Disease
  • Aplasia cutis congenita nonsyndromic
  • Congenital defect of skull and scalp
  • Scalp defect congenital
See Disclaimer regarding information on this site. Some links on this page may take you to organizations outside of the National Institutes of Health.

Treatment

Newline Maker

How might aplasia cutis congenita be treated?

The management of aplasia cutis congenita of the scalp is controversial.[1]; both surgical and conservative treatment modalities have their proponents and opponents.[2] The decision to use medical, surgical, or both forms of therapy in aplasia cutis congenita depends primarily on the size, depth, and location of the skin defect.[3]

Local therapy includes gentle cleansing and the application of bland ointment or silver sulfadiazine ointment to keep the area moist. Antibiotics may be utilized if overt signs of infection are noted. In many cases, other treatment is not necessary because the erosions and the ulcerations almost always heal on their own. Recently, a variety of specialized dressing materials have been developed and used.[3]

Surgical repair is not usually indicated if the defect is small. Recovery is generally uneventful, with gradual epithelialization and formation of a hairless, atrophic scar over several weeks. Small underlying bony defects usually close spontaneously during the first year of life. Surgical repair of large or multiple scalp defects may require excision with primary closure, if feasible, or the use of tissue expanders and rotation of a flap to fill the defect. On occasion, skin and bone grafts may also be required.[3]

    Last updated: 2/2/2010

    References
    1. Santos de Oliveira R, Barros Juca CE, Lopes Lins-Neto A, Aparecida do Carmo Rego M, Farina J, Machado HR. Childs Nerv Syst. 2006; http://www.ncbi.nlm.nih.gov/pubmed/16639628. Accessed 1/19/2010.
    2. Basterzi Y, Bagdatoglu C, Sari A, Demirkan F. J Craniofac Surg. 2007; http://www.ncbi.nlm.nih.gov/pubmed/17414296. Accessed 1/19/2010.
    3. Crowe MA. Aplasia Cutis Congenita: Treatment & Medication. eMedicine. 2010; http://emedicine.medscape.com/article/1110134-treatment. Accessed 1/19/2010.


    Clinical Trials & Research for this Disease

    • The Centers for Mendelian Genomics program is working to discover the causes of rare genetic disorders. For more information about applying to the research study, please visit their website.
    Other Names for this Disease
    • Aplasia cutis congenita nonsyndromic
    • Congenital defect of skull and scalp
    • Scalp defect congenital
    See Disclaimer regarding information on this site. Some links on this page may take you to organizations outside of the National Institutes of Health.