Aplasia Cutis Congenita, most commonly affects the scalp, with a variable penetrance ranging from a small (<2 cm) area of missing skin to large defects characterized by absent skin, subcutaneous tissue, calvarium, and dura.

Patients with small defects respond well to conservative management. However, larger defects often can pose management and technical challenge.

Calvarial reconstruction in ACC can be challenging. Due to exposed neurologic structures, in large defects, ACC has a high mortality rate.

A stable reconstruction is optimally achieved shortly after birth to minimize complications.

Mericli et al. present a case of a neonate with an extensive (4.5 × 7 cm) cutis aplasia defect associated with absent skin, subcutaneous tissue, calvarium, dura, and with exposed cortical surface and sagittal sinus. This defect was successfully reconstructed in a single stage with the use of an acellular dermal matrix/skin graft construct. The acelluar dermal matrix served as a scaffold for tissue ingrowth, promoting regeneration of the bony calvarium as well as soft tissue. At 18-month follow-up, the patient exhibits a 50% smaller calvarial defect as well as stable skin coverage ¹⁾.

Successful management of a large, composite aplasia cutis congenita defect using Integra dermal regeneration matrix is presented. At 16 months, the patient showed a stable and supple scalp with evidence of calvarial regeneration ²⁾.

Two cases of cutis aplasia congenita with large skull defects (6 x 10 cm, 8 x 8 cm) of superficial layers, skull, and dura are presented. In each case, composite reconstruction was undertaken before 2 weeks of age with restoration of bony and soft tissue coverage through autologous, full-thickness cranial bone grafts and scalp flaps. Both children have been followed up over 2 years with clinical examination and computed tomography (CT) scans.

In both cases, defects were completely repaired postoperatively and remained closed 2 years later. Complete regeneration of calvarial bone graft donor sites were documented by CT scan. Head shape and circumference were normal at 2-year follow up ³⁾.

Komuro et al. report a case of aplasia cutis congenita of the scalp associated with bilateral coronal synostosis. Simultaneous fronto-orbital advancement and skull reconstruction for large defect at the fontanelle were performed in the initial operation. A tissue expander made it possible to resect most of the hairless scar and totally cover the reconstructed defect and skull. During the second stage, the residual hairless scar was completely covered with hair-bearing scalp by tissue expansion and the residual skull defects were successfully reconstructed with split calvarial bone grafting ⁴⁾.

A case is reported of early repair of a cranial defect associated with aplasia cutis congenita using a full-thickness autologous calvarial graft. New bone regenerated to fill the defect at the donor site, such that by 6 weeks of life no calvarial defects remained. This simple technique is recommended as an alternative to a delayed acrylic or split cranial bone cranioplasty as a second operation. A similar technique could be considered for other congenital cranial defects, such as those associated with encephaloceles or bilateral parietal foramina ⁵⁾.