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2000
Volume 2, Issue 1
  • ISSN: 0250-6882
  • E-ISSN: 0250-6882

Abstract

is a rare congenital disorder characterized by the absence of skin and adjacent tissue that usually affect the scalp. A large scalp defect in ACC with adjacent tissue anomalies is associated with high morbidity and mortality. The management of these lesions can be challenging. The treatment can be either conservative, surgical, or combined.

A full-term newborn girl delivered with a huge scalp defect. The dura mater was exposed but intact and there was no Cerebrospinal Fluid (CSF) leakage. She also had bilateral talipes equinovarus and terminal reduction of multiple toes in the left foot. Genetic testing was positive for . Conservative medical management was initiated at birth and continued until discharge at 4 weeks of age. The patient had a regular follow-up with plastic surgery and neurosurgery teams. At 11 months old, the defect became smaller, hairless with good granulation tissue, but still soft and compressible.

Large scalp midline is a rare, but potentially devastating disease when involved adjacent tissue. The choice of treatment is not straightforward and it is mainly based upon the size, depth, and the risk of pre and post-treatment complications. Conservative management still is an option with very complex .

© 2021 The Author(s). Published by Bentham Science Publisher. This is an open access article published under CC BY 4.0 https://creativecommons.org/licenses/by/4.0/legalcode
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2020-05-18
2025-03-13
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References

  1. CordonM. Extrait du¨ne lettre au sujet de trois enfants de la meme mere nex avec partie des extremmites denuee de peau.J Med Chir Pharm176726556557
    [Google Scholar]
  2. CampbellW. Case of congenital ulcer on the cranium of a fetus.1826
    [Google Scholar]
  3. Martinez-RegueiraS. Vazquez-LopezM.E. Somoza-RubioC. Morales-RedondoR. Gonzalez-GayM.A. Aplasia cutis congenita in a defined population from northwest Spain.Pediatr. Dermatol.200623652853210.1111/j.1525‑1470.2006.00303.x17155992
    [Google Scholar]
  4. FröjdV. MalteseG. KölbyL. TarnowP. Conservative healing of an 11 × 9-cm aplasia cutis congenita of the scalp with bone defect.J. Neurol. Surg. Rep.2014752e220e22310.1055/s‑0034‑138719525485218
    [Google Scholar]
  5. DudoignonB. HuberC. MichotC. Di RoccoF. GirardM. LyonnetS. RioM. RabiaS.H. DaireV.C. BaujatG. Expanding the phenotype in Adams-Oliver syndrome correlating with the genotype.Am. J. Med. Genet. A.20201821293710.1002/ajmg.a.6136431654484
    [Google Scholar]
  6. BhartiG. GrovesL. DavidL.R. SangerC. ArgentaL.C. Aplasia cutis congenita: Clinical management of a rare congenital anomaly.J. Craniofac. Surg.201122115916510.1097/SCS.0b013e3181f7393721187755
    [Google Scholar]
  7. BurkheadA. PoindexterG. MorrellD.S. 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.200929858258410.1038/jp.2008.25019638992
    [Google Scholar]
  8. Maillet-DeclerckM. VinchonM. GuerreschiP. PasquesooneL. DhellemmesP. Duquennoy-MartinotV. PellerinP. Aplasia cutis congenita: review of 29 cases and proposal of a therapeutic strategy.Eur. J. Pediatr. Surg.2013232899322903250
    [Google Scholar]
  9. HarveyG. SolankiN.S. AndersonP.J. CarneyB. SnellB.J. Management of aplasia cutis congenita of the scalp.J. Craniofac. Surg.20122361662166410.1097/SCS.0b013e31826542de23147310
    [Google Scholar]
  10. Betancourth-AlvarengaJ.E. Vázquez-RuedaF. Vargas-CruzV. Paredes-EstebanR.M. Ayala-MontoroJ. Surgical management of aplasia cutis congenita.An. Pediatr. (Barc.)201583534134510.1016/j.anpedi.2015.02.00525804551
    [Google Scholar]
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