Johnson Rowena, Offiah Amaka, Cohen Marta C
University of Sheffield, Sheffield, UK.
Forensic Sci Med Pathol. 2015 Jun;11(2):243-8. doi: 10.1007/s12024-014-9645-5. Epub 2015 Jan 23.
Aplasia cutis congenita (ACC) is a rare condition that occurs in around 0.01% of births. Characterized by a localized absence of skin, it affects the midline of the scalp in over 80% of cases. We describe the case of an infant born via vaginal spontaneous delivery with ACC affecting the scalp. This extended to the subcutaneous tissue and bone leaving the dura mater exposed. The patient was managed conservatively. At 4 weeks of age, she had a fatal superior sagittal sinus hemorrhage. In a review of the literature, we found ten previous cases of hemorrhage of the superior sagittal sinus complicating an ACC. Including our case, ACC complicated by hemorrhage of the superior sagittal sinus shows a 36% mortality (4/11). An analysis of these cases suggests that this tends to occur between 1 and 3 months of age, though it may occur in younger neonates.
先天性皮肤发育不全（ACC）是一种罕见疾病，发生率约为出生人口的0.01%。其特征为局部皮肤缺失，超过80%的病例累及头皮中线。我们描述了一例经阴道自然分娩出生的婴儿，患有累及头皮的ACC。病变延伸至皮下组织和骨骼，硬脑膜暴露。该患者接受了保守治疗。在4周龄时，她发生了致命的上矢状窦出血。在文献回顾中，我们发现之前有10例上矢状窦出血并发ACC的病例。包括我们的病例在内，ACC并发上矢状窦出血的死亡率为36%（4/11）。对这些病例的分析表明，这种情况倾向于发生在1至3个月龄之间，不过也可能发生在更小的新生儿身上。
