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.
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Frieden IJ (1986) Aplasia cutis congenita: a clinical review and proposal for classification. Acad Dermatol 14:646–660
Sybert VP (1985) Aplasia cutis congenita: a report of 12 new families and review of the literature. Pediatric Dermatol 3:1–14
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 121:21–50
Abbott R, Cutting CB, Wisoff JH, Thorne CH, Epstein FJ (1991–1992) Aplasia cutis congenita of the scalp: issues in its management. Pediatr Neurosurg 17:182–184
Schneider BM, Berg RA, Kaplan AM (1980) Aplasia cutis congenita complicated by sagittal sinus hemorrhage. Pediatrics 66: 948–950
Wexler A, Harris M, Lesavoy M (1990) Conservative treatment of cutis aplasia. Plast Reconstr Surg 86:1066–1071
Vinocur CD, Weintraub WH, Wilensky RJ (1976) Surgical management of aplasia cutis congenita. Arch Surg 111:1160–1164
O'Brien BMcC, Drake JE (1960) Congenital defects of skull and scalp. Br J Plast Surg 13:102–109
Kosnik EJ, Sayers MP (1975) Congenital scalp defects: aplasia cutis congenita. J Neurosurg 42:32–36
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 451–454
Muakassa KF, King RB, Stark DB (1982) Neurosurgical approach to congenital scalp and skull defects. J. Neurosurg 56: 711–715
Aknin J, Seguin P, Brunon J, Oulhchane M (1992) Aplasie cutanée du vertex. Rev Stomatol Chir Maxillofac 93:267–272
Spear SL, Mickle JP (1983) Simultaneous cutis aplasia congenita of the scapl and cranial stenosis. Plast Reconstr Surg 71: 413–417
Arand AG, Ball WS, Crone KR, (1991–1992) Congenital scalp defects: Adams-Oliver syndrome. Pediatr Neurosurg 17:203–207
Pozzati E, Padovanie R, Frank F, Gaist G (1983) Leptomeningeal angiomatosis and aplasia congenita of the scalp: case report.J Neurosurg 58:937–940
Vasconez LO (1973) Congenital defect of the skull and scalp due to an arteriovenous malformation. Plast Reconstr Surg 51:692–695
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 31:99–115
Smit CSF, Zeeman BJR (1992) Cutis aplasia. Plast Reconstr Surg 90:336
Croce EJ, Purohit RC, Janovski NA (1973) Congenitals absence of the skin (aplasia cutis congenita). Arch Surg 106:732–734
Stephan MJ, Smith DN, Ponzi JW, Alden ER (1982) Origin of scalp vertex aplasia cutis. J Pediatr 101:850–853
Argenta LC, Dingman RO (1986) Total reconstruction of aplasia cutis congenita involving scalp, skull and dura. Plast Reconstr Surg 77:650–653
Irons GB, Olson RM (1980) Aplasia cutis congenita. Plast Reconstr Surg 66:199–203
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Leboucq, N., Montoya y Mártínez, P., Montoya-Vigo, F. et al. Aplasia cutis congenita of the scalp with large underlying skull defect: a case report. Neuroradiology 36, 480–482 (1994). https://doi.org/10.1007/BF00593689
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DOI: https://doi.org/10.1007/BF00593689