Bajpai Minu, Pal K
Department of Paediatric Surgery, All India Institute of Medical Sciences, New Delhi, India.
J Pediatr Surg. 2003 Feb;38(2):e4. doi: 10.1053/jpsu.2003.50064.
Aplasia cutis cerebri with partial acrania is a rare anomaly that can involve the epidermis, dermis, and subcutaneous tissues of the scalp with significant bone defect.
The authors present the first successful report of providing one-stage complete cover in a case of aplasia cutis cerebri with major skull defect by using local rotational flaps. Also presented is a review of literature.
The newborn had a receding forehead and a large scalp defect from frontal to occipital bone measuring 10 x 8 cm2 involving full thickness of cranium centrally, thereby exposing the dura. Local rotation flaps were raised from the scalp to cover the 10- x 8-cm2 defect. The edges of the defect were showing granulation tissue with ingrowth of epithelium. Partial-thickness skin grafts were used to cover the raw area left at the nape of the neck after raising the rotation flaps. The child was discharged on the 19th postoperative day.
Aplasia cutis cerebri with partial acrania, as in our case, has a high mortality rate secondary to infection or to hemorrhage from ulceration of the sagittal sinus. The successful outcome of our one-stage local rotational scalp flap technique provided complete cover to this major scalp defect. This technique will definitely improve the management of this complex disorder keeping the morbidity to the minimum.
脑性皮肤发育不全伴部分颅骨缺失是一种罕见的先天性畸形，可累及头皮的表皮、真皮及皮下组织，并伴有明显的骨缺损。
作者报道了首例应用局部旋转皮瓣一期完全覆盖脑性皮肤发育不全伴严重颅骨缺损病例的成功案例，并对相关文献进行了综述。
该新生儿前额后缩，头皮存在巨大缺损，从前额至枕骨，面积为10×8 cm²，颅骨中央全层受累，硬脑膜暴露。自头皮掀起局部旋转皮瓣覆盖10×8 cm²的缺损区。缺损边缘可见肉芽组织及上皮向内生长。掀起旋转皮瓣后，取刃厚皮片覆盖颈后部的创面。患儿术后第19天出院。
如我们病例中的脑性皮肤发育不全伴部分颅骨缺失，因感染或矢状窦溃疡出血导致的死亡率较高。我们的一期局部旋转头皮皮瓣技术成功为这一巨大头皮缺损提供了完全覆盖。该技术必将改善这种复杂疾病的治疗，将发病率降至最低。
