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Successful treatment of an extensive form of aplasia cutis congenita by dermis grafts

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Summary

This is a report of an extensive form of aplasia cutis congenita in a male newborn with normal family history, normal birth and no other anomalies. The deformity was located on the lower and lateral part of the thorax extending to the axillae and including the whole abdominal wall. A small skin area of 2 cm radius around the umbilicus and 2 cm wide on each side of the lumbar spine were normal. This entire defect was covered by a transparent membrane through which the movement of the lungs, the liver and the bowel could be observed. The segmental intercostal vessels and nerves were visible. The transparent membrane consisted of two very thin layers; the inner one was thought to be the peritoneum, and the outer one showed malformed epithelium and a few muscles under the electronmicroscope. A few smaller lesions were found on the hairy scalp on both sides of the midline and on both thighs. Surgical treatment was performed with multiple dermis grafts taken from different parts of th body sutured together to form one piece and inserted between the two layers of the lesion. In two weeks time complete epithelialisation occured except over one small granulating defect which was covered with a mesh graft. There were no postoperative complications and the infant showed normal development with normal weight gain and growth. No scar contracture has been observed and even signs of some muscle activity in the abdominal wall can be detected.

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Anderl, H., Frisch, H. & Hussl, H. Successful treatment of an extensive form of aplasia cutis congenita by dermis grafts. Chir Plastica 5, 183–190 (1980). https://doi.org/10.1007/BF00264789

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Key words

  • Extensive aplasia cutis of thorax and abdomen
  • Dermis Grafts