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Neuroradiology

, 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

Abstract

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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References

  1. 1.
    Frieden IJ (1986) Aplasia cutis congenita: a clinical review and proposal for classification. Acad Dermatol 14:646–660Google Scholar
  2. 2.
    Sybert VP (1985) Aplasia cutis congenita: a report of 12 new families and review of the literature. Pediatric Dermatol 3:1–14Google Scholar
  3. 3.
    Demmel U (1975) Clinical aspects of congenital skin defects. I. Congenital skin defects on the head of the newborn. II. Congenital skin defects on the trunk and extremities of the newborn. III. Causal and formal genesis of congenital skin defects of the newborn. Eur J Pediatr 121:21–50Google Scholar
  4. 4.
    Abbott R, Cutting CB, Wisoff JH, Thorne CH, Epstein FJ (1991–1992) Aplasia cutis congenita of the scalp: issues in its management. Pediatr Neurosurg 17:182–184Google Scholar
  5. 5.
    Schneider BM, Berg RA, Kaplan AM (1980) Aplasia cutis congenita complicated by sagittal sinus hemorrhage. Pediatrics 66: 948–950Google Scholar
  6. 6.
    Wexler A, Harris M, Lesavoy M (1990) Conservative treatment of cutis aplasia. Plast Reconstr Surg 86:1066–1071Google Scholar
  7. 7.
    Vinocur CD, Weintraub WH, Wilensky RJ (1976) Surgical management of aplasia cutis congenita. Arch Surg 111:1160–1164Google Scholar
  8. 8.
    O'Brien BMcC, Drake JE (1960) Congenital defects of skull and scalp. Br J Plast Surg 13:102–109Google Scholar
  9. 9.
    Kosnik EJ, Sayers MP (1975) Congenital scalp defects: aplasia cutis congenita. J Neurosurg 42:32–36Google Scholar
  10. 10.
    Broomhead IW (1988) Congenital defects of the scalp. In Mustardé JC, Jackson IT (eds). Plastic surgery in infancy and childhood 3rd edn. Churchill Livingstone, New York pp 451–454Google Scholar
  11. 11.
    Muakassa KF, King RB, Stark DB (1982) Neurosurgical approach to congenital scalp and skull defects. J. Neurosurg 56: 711–715Google Scholar
  12. 12.
    Aknin J, Seguin P, Brunon J, Oulhchane M (1992) Aplasie cutanée du vertex. Rev Stomatol Chir Maxillofac 93:267–272Google Scholar
  13. 13.
    Spear SL, Mickle JP (1983) Simultaneous cutis aplasia congenita of the scapl and cranial stenosis. Plast Reconstr Surg 71: 413–417Google Scholar
  14. 14.
    Arand AG, Ball WS, Crone KR, (1991–1992) Congenital scalp defects: Adams-Oliver syndrome. Pediatr Neurosurg 17:203–207Google Scholar
  15. 15.
    Pozzati E, Padovanie R, Frank F, Gaist G (1983) Leptomeningeal angiomatosis and aplasia congenita of the scalp: case report.J Neurosurg 58:937–940Google Scholar
  16. 16.
    Vasconez LO (1973) Congenital defect of the skull and scalp due to an arteriovenous malformation. Plast Reconstr Surg 51:692–695Google Scholar
  17. 17.
    Küster W, Lenz W, Kääriäinen H, Majewski F (1988) Congenital scalp defects with distal limb anomalies (Adams-Oliver syndrome): report of ten cases and review of the literature. Am J Med Genet 31:99–115Google Scholar
  18. 18.
    Smit CSF, Zeeman BJR (1992) Cutis aplasia. Plast Reconstr Surg 90:336Google Scholar
  19. 19.
    Croce EJ, Purohit RC, Janovski NA (1973) Congenitals absence of the skin (aplasia cutis congenita). Arch Surg 106:732–734Google Scholar
  20. 20.
    Stephan MJ, Smith DN, Ponzi JW, Alden ER (1982) Origin of scalp vertex aplasia cutis. J Pediatr 101:850–853Google Scholar
  21. 21.
    Argenta LC, Dingman RO (1986) Total reconstruction of aplasia cutis congenita involving scalp, skull and dura. Plast Reconstr Surg 77:650–653Google Scholar
  22. 22.
    Irons GB, Olson RM (1980) Aplasia cutis congenita. Plast Reconstr Surg 66:199–203Google Scholar

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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