, Volume 36, Issue 6, pp 480–482 | Cite as

Aplasia cutis congenita of the scalp with large underlying skull defect: a case report

  • N. Leboucq
  • P. Montoya y Mártínez
  • F. Montoya-Vigo
  • P. Castan
Paediatric Neuroradiology


Localised agenesis of the scalp is the most frequent patern in aplasia cutis congenita (ACC), a congenital absence of the skin and occasionally of deeper layers. Several clinical groups are characterised by the location and pattern of skin defects, associated malformations and the mode of inheritance. Death occurs in 20% of cases, secondary to the associated anomalies, to infections or to haemorrhage from ulceration of the sagittal sinus when there is also a defect of the underlying skull. In this latter case, we close the defect by two rotational scalp flaps (Orticochea technique) at birth. A three-dimensional CT study is useful for showing the extent of the skull defect and the deformity of the craniofacial complex and the changes in the bone after treatment.

Key words

Aplasia cutis congenita Scalp defects Congenital anomalies Three-dimensional computed tomography 


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Copyright information

© Springer-Verlag 1994

Authors and Affiliations

  • N. Leboucq
    • 1
  • P. Montoya y Mártínez
    • 2
  • F. Montoya-Vigo
    • 3
  • P. Castan
    • 1
  1. 1.Department of NeuroradiologyCentre Hospitalier UniversitaireMontpellierFrance
  2. 2.Department of Craniofacial Recontructive SurgeryCentre Hospitalier UniversitaireMontpellierFrance
  3. 3.Department of Neonatal PaediatricsCentre Hospitalier UniversitaireMontpellierFrance

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