Reparation of a severe case of aplasia cutis congenita with engineered skin

Aplasia cutis congenita. (ACC) is an uncommon congenital malformation. It i s characterized by defects of the skin that occur most frequently on the sc alp along the midline, but can also be localized on the trunk, face and lim bs, usually with a symmetrical distribution. When it is localized in the sk ull it can extend to the dura mater, leaving only the thin pia mater to pro tect the brain. The most common complications related to this disorder are infection, hemorrhage, and, in defects localized on the vertex, meningitis and bleeding from the sagittal sinus can occur with dramatic consequences. In those cases some authors suggest the use of local flaps even if this imp lies a surgical procedure. In this case a 2.540-kg baby was delivered at 40 weeks of gestation be eutocic delivery, and good adaptation to extrauterin e life. At birth the baby showed a large cutaneous and osseous defect at th e vertex measuring 68 cm(2), equal to almost one third of the calvarial sur face, and extended to the dura mater through which it was possible to see t he sagittal sinus and the brain surface with its vessels. Skull X-rays show ed loss of normal radioopacity of the cranial vault with lack of ossificati on especially at the parietal level. In our patient we therefore decided to use a composite graft of cultured autologous fibroblasts and keratinocytes to provide coverage, avoiding any surgical procedure and patient morbidity . This technique consists first in an autograft of cultured fibroblasts whi ch has proved to promote the production of type IV collagen, fibronectin an d laminin whereby creating an ideal bed for the taking of the graft of cult ured keratinocytes, to be put in place a week later. The use of a composite graft with both the derma-like and epithelial components has been also sug gested to diminish scarring. Two months after the last graft area was compl etely closed. Copyright (C) 2001 S. Karger AG, Basel.