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ABSTRACT Aplasia Cutis Congenita (ACC) is a rare condition characterized by the absence of a portion of skin at birth. Skin defects are usually small (0.5 to 3 cm) and located on the scalp.
Although there can be other physical or genetic abnormalities, ACC is most often a benign isolated condition. Rarely is an underlying bony defect present, and this association increases the
rate of complications. We report a case of a newborn male with ACC of the entire crown and vertex scalp, non-ossified parietal skull and dysplastic corpus callosum. The patient's skull
and skin defects were treated non-surgically, and he recovered well. Access through your institution Buy or subscribe This is a preview of subscription content, access via your institution
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about institutional subscriptions * Read our FAQs * Contact customer support SIMILAR CONTENT BEING VIEWED BY OTHERS ADDITIONAL FINDINGS OF TIBIAL DYSPLASIA IN A MALE WITH OROFACIODIGITAL
SYNDROME TYPE XVI Article Open access 31 March 2022 TYPE 2 CONGENITAL GENERALIZED LIPODYSTROPHY WITH A HETEROZYGOUS MISSENSE NOTCH2 MUTATION Article 18 January 2022 _BCL11B_-RELATED DISEASE:
A SINGLE PHENOTYPIC ENTITY? Article Open access 03 March 2025 REFERENCES * Cordon M . Extrait düne lettre au sujet de trois enfants de la meme mere nex avec partie des extremmites denuee de
peau. _J Med Chir Pharm_ 1767; 26: 556–557. Google Scholar * Frieden IJ . Aplasia cutis congenita: a clinical review and proposal for classification. _J Am AcadDermatol_ 1986; 14 (4):
646–660. Article CAS Google Scholar * Spraker MK, Garcia-Gonzalez E, Sanchez LT . Aplasia cutis congenita. In: Schachner LA, Hansen RC (eds). _Pediatr Dermatol_, 2nd edn., vol. 2.
Churchill Livingstone: New York, 1995, pp 905–907. Google Scholar * Iwayama H, Hosono H, Yamamoto H, Oshiro M, Ueda N . Aplasia Cutis Congenita with skull defect in a monozygotic twin after
exposure to methimazole in utero. _Birth Defects Res Clin Mol Teratol_ 2007; 79 (10): 680–684. Article CAS Google Scholar * Azad S, Falder S, Harrison J, Graham K . An adherent dressing
for aplasia cutis congenita. _Br J Plast Surg_ 2005; 58 (8): 1159–1161. Article CAS Google Scholar * Kantor J, Yan A, Hivnor C, Honig P, Kirschner R . Extensive Aplasia Cutis Congenita
and the Risk of Sagittal Sinus Thrombosis. _Arch Dermatol_ 2005; 141: 554–556. Article Google Scholar * Ploplys E, Muzaffar A, Gruss J, Ellenbogen R . Early composite cranioplasty in
infants with severe aplasia cutis congenita: a report of two cases. _Cleft Palate—Craniofac J_ 2005; 42 (4): 442–447. Article Google Scholar * Smartt J, Kim E, Tobias A, Yan A, Kirschner R
. Aplasia cutis congenita with calvarial defects: A simplified management strategy using acellular dermal matrix. _Plast Reconstr Surg_ 2008; 121 (4): 1224–1229. Article CAS Google
Scholar * Amor D, Leventer R, Hayllar S, Bankier A . Polymicrogyria associated with scalp and limb defects: Variant of Adams-Oliver Syndrome. _Am J Med Genet_ 2000; 93 (4): 328–334. Article
CAS Google Scholar * Rajabian M, Aghaei S . Adams-Oliver Syndrome and isolated aplasia cutis congenita in two siblings. _Dermatol Online J_ 2006; 12 (6): 17. PubMed Google Scholar * Ng
P, Lee C, To K, Fok T, So K, Wong W _et al_. Severe congenital absence of skin in a preterm infant. _J Paediat Child Health_ 1999; 35 (3): 306–308. Article CAS Google Scholar * Beekmans
S, Don Griot J, Mulder J . Split rib cranioplasty for aplasia cutis congenital and traumatic skull defects: more than 30 years of follow-up. _J Craniofac Surg_ 2007; 18 (3): 594–597. Article
Google Scholar * Tosun Z, Ozkan A, Savaci N . Is surgery always necessary in the treatment of aplasia cutis congenita? _Plast Reconstr Surg_ 2006; 117 (4): 1355–1356. Article CAS Google
Scholar * Rhee S, Colville C, Buchman S, Muraszko K . Complete osseous regeneration of a large skull defect in a patient with cutis aplasia: a conservative approach. _J Craniofac Surg_
2002; 13 (4): 497–500. Article Google Scholar * Saraiya H . Management of aplasia cutis congenita of the scalp: a continuing enigma. _Br J Plast Surg_ 2000; 53 (7): 619–622. Article
Google Scholar * Yang J, Yang W . Large scalp and skull defect in aplasia cutis congenita. _Br J Plast Surg_ 2000; 53 (7): 619–622. Article CAS Google Scholar * Benjamin L, Trowers A,
Schachner L . Giant aplasia cutis congenita without associated anomalies. _Pediatr Dermatol_ 2004; 21 (2): 150–153. Article Google Scholar * Kim C, Tatum S, Rodziewicz G . Scalp aplasia
cutis congenita presenting with sagittal sinus hemorrhage. _Arch Otolaryngol—Head Neck Surg_ 2001; 127 (1): 71–74. Article CAS Google Scholar * Bernbeck B, Schwabe J, Groninger A, Schaper
J, Messing-Junger H, Mayatepek E _et al_. Aplasia cutis congenital of the scalp: how much therapy is necessary in large defects? _Acta Paediatrica_ 2005; 94 (6): 758–760. Article CAS
Google Scholar * Muakkassa K, King R, Stark D . Nonsurgical approach to congenital scalp and skull defects. _J Neurosurg_ 1982; 56 (5): 711–715. Article CAS Google Scholar * Smith C,
Zeeman B . Cutis aplasia. _Plast Reconstr Surg_ 1992; 90 (2): 336. Google Scholar * Theile R, Lanigan M, McDermant G . Reconstruction of aplasia cutis congenita of the scalp by split rib
cranioplasty and a free latissimus dorsi muscle flap in a nine month old infant. _Br J Plast Surg_ 1995; 48 (7): 507–510. Article CAS Google Scholar Download references AUTHOR INFORMATION
AUTHORS AND AFFILIATIONS * University of North Carolina School of Medicine, Chapel Hill, NC, USA A Burkhead * Department of Dermatology, University of North Carolina, Chapel Hill, NC, USA G
Poindexter & D S Morrell Authors * A Burkhead View author publications You can also search for this author inPubMed Google Scholar * G Poindexter View author publications You can also
search for this author inPubMed Google Scholar * D S Morrell View author publications You can also search for this author inPubMed Google Scholar CORRESPONDING AUTHOR Correspondence to A
Burkhead. RIGHTS AND PERMISSIONS Reprints and permissions ABOUT THIS ARTICLE CITE THIS ARTICLE Burkhead, A., Poindexter, G. & Morrell, D. A case of extensive Aplasia Cutis Congenita with
underlying skull defect and central nervous system malformation: discussion of large skin defects, complications, treatment and outcome. _J Perinatol_ 29, 582–584 (2009).
https://doi.org/10.1038/jp.2008.250 Download citation * Received: 05 July 2008 * Revised: 21 November 2008 * Accepted: 08 December 2008 * Published: 29 July 2009 * Issue Date: August 2009 *
DOI: https://doi.org/10.1038/jp.2008.250 SHARE THIS ARTICLE Anyone you share the following link with will be able to read this content: Get shareable link Sorry, a shareable link is not
currently available for this article. Copy to clipboard Provided by the Springer Nature SharedIt content-sharing initiative KEYWORDS * aplasia cutis congenita * bony defect * sagittal sinus
hemorrhage