Mericli Alexander F, Chen Kevin, Murariu Daniel, Jane John A, Lin Kant Y
*Department of Plastic Surgery, University of Virginia Health System †Department of Neurosurgery, University of Virginia Health System, Charlottesville, VA.
J Craniofac Surg. 2015 Sep;26(6):1960-2. doi: 10.1097/SCS.0000000000002024.
Aplasia cutis congenital (ACC) is a rare congenital anomaly, most commonly affecting 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. 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. Herein the authors 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.
先天性皮肤发育不全(ACC)是一种罕见的先天性异常,最常累及头皮,其外显率各异,范围从小的(<2厘米)皮肤缺失区域到以皮肤、皮下组织、颅骨和硬脑膜缺失为特征的大缺损。ACC的颅骨重建具有挑战性。由于神经结构暴露,在大的缺损中,ACC的死亡率很高。在出生后不久进行稳定的重建可最佳地减少并发症。在此,作者介绍了一例患有广泛(4.5×7厘米)皮肤发育不全缺损的新生儿病例,该缺损伴有皮肤、皮下组织、颅骨、硬脑膜缺失,且皮质表面和矢状窦暴露。使用脱细胞真皮基质/皮肤移植构建物在一期成功重建了该缺损。脱细胞真皮基质作为组织向内生长的支架,促进了颅骨和软组织的再生。在18个月的随访中,患者的颅骨缺损缩小了50%,皮肤覆盖也稳定。