Department of Neurosurgery, National Institute of Neurology"Mongi Ben Hmida", Tunis Faculty of Medicine, University of Tunis El Manar, Tunis, Tunisia.
Reconstructive & Aesthetic Surgery Department, Trauma & Burns Center, Ben Arous, Tunisia.
Childs Nerv Syst. 2024 Feb;40(2):285-292. doi: 10.1007/s00381-023-06190-x. Epub 2023 Oct 22.
Aplasia cutis congenita (ACC) of the vertex with bone defect is a rare and begnin anomaly that can involve the epidermis, dermis, and subcutaneous tissues of the scalp with significant bone defect Bajpai and Pal (J Pediatr Surg 38(2):e4, 2003). When associated with skull defect, this rare malformation carries the risk of severe complications such as rupture of the superior sagittal sinus or infections.
We report a case of aplasia cutis congenita of the scalp with skull defect measuring 9 × 10 cm and an exposed sagittal sinus in a newborn. Both conservative and surgical methods have been proposed to treat this condition. In our case, conservative treatment was planned led to complete epithelization and the patient was healing well at 5 years of follow-up.
ACC of the vertex with a large scalp defects present a management dilemma Rocha et al. (Clin Case Rep 3(10):841-4, 2015). Based on a review of the literature, we report this case to demonstrate that even for the largest skin and bone defects, an initial conservative approach may allow for complete wound closure without the need for early surgical intervention.
头皮先天性表皮发育不全（ACC）伴骨缺损是一种罕见的良性病变，可累及头皮表皮、真皮和皮下组织，伴显著骨缺损。Bajpai 和 Pal（2003 年《儿外科杂志》38 卷 2 期，e4）曾报道过此类病例。当伴有颅骨缺损时，这种罕见的畸形可能会导致严重并发症，如矢状窦破裂或感染。
我们报告了一例新生儿头皮先天性表皮发育不全伴颅骨缺损（大小为 9×10cm）和暴露的矢状窦的病例。目前已经提出了保守和手术治疗这一疾病的方法。在我们的病例中，计划进行保守治疗，导致完全上皮化，患者在 5 年的随访中恢复良好。
头皮先天性表皮发育不全伴较大的头皮缺损存在管理难题。Rocha 等人曾对此进行过综述（2015 年《临床病例报告》3 卷 10 期，841-4）。基于文献复习，我们报告了这一病例，以表明即使对于最大的皮肤和骨缺损，初始保守方法也可能允许完全伤口闭合，而无需早期手术干预。
