Velho Vernon, Naik Harish, Survashe Pravin, Guthe Sachin, Bhide Anuj, Bhople Laxmikant, Guha Amrita
Department of Neurosurgery, JJ Group of Hospitals and Grant Medical College, Mumbai, Maharashtra, India.
Department of Radiology, JJ Group of Hospitals and Grant Medical College, Mumbai, Maharashtra, India.
Asian J Neurosurg. 2019 Jul-Sep;14(3):718-724. doi: 10.4103/ajns.AJNS_139_17.
Encephalocele is defined as herniation of cranial contents beyond the normal confines of the skull through a defect in the calvarium either along the midline or at the base of skull. These anomalies should be repaired in the first few months of life to prevent neurological deficits and facial disfigurement. The aim of the surgery is water tight dural closure at the level of internal defect, closure of skull defect, and reconstruction of external bony deformity.
Fifty-four cases of encephalocoeles were studied in our hospital over a 6-year period from 2010 to 2016. Computed tomography (CT) and magnetic resonance imaging (MRI) brain were performed to delineate the bony defect and associated anomalies. Reconstruction of the bony defect was done using autologous calvarial bone graft, Osteopore polycaprolactone (PCL) bone scaffold filler and titanium mesh.
In our study, 54 patients (34 boys and 20 girls) whose age varied between 2 months and 14 years were evaluated. Frontoethmoidal (44.5%) and occipital encephaloceles (25.9%) were the most frequently seen varieties. Repair of the dural defect either primarily or using pericranium was done in all cases. Closure of the bony defect was done using autologous calvarial bone graft in 12 (22.22%) patients. Titanium mesh was used in eight and Osteopore PCL bone scaffold filler in four children. Cranioplasty was not done in remaining thirty children because of the small bone defect. Overall, 80% had no postoperative problem and were discharged between 7 and 10 days of surgery. Cerebrospinal fluid leak was the most frequent postoperative complication, noted in five patients. Re-exploration with repair was done in one and remaining four were managed conservatively. Overall, cosmetic results were acceptably good, with parents judging the cosmetic outcome as good to excellent in 70%, satisfactory in 18%, and poor in 3% at the last follow-up.
Our study demonstrated that encephaloceles are associated with complex deformities and pose a technical challenge to the neurosurgeon. A multidisciplinary approach is necessary to manage these cases. MRI brain and three dimensional CT aids in evaluating the deformity better and surgical correction should be performed as soon as possible to prevent a further neurological deficit. Repair of dural defect and reconstruction of the skull defect results in a good long-term outcome. We present our experience on 54 cases of cranial encephaloceles managed surgically over a period of only 6 years which is one of the largest series reported from Asia.
脑膨出被定义为颅骨内容物通过颅骨缺损处疝出至颅骨正常边界之外，缺损部位可位于中线或颅底。这些畸形应在出生后的头几个月内修复，以防止神经功能缺损和面部畸形。手术的目的是在内部缺损处实现硬膜水密性闭合、闭合颅骨缺损并重建外部骨畸形。
2010年至2016年的6年间，我院对54例脑膨出病例进行了研究。进行了头颅计算机断层扫描（CT）和磁共振成像（MRI）以明确骨缺损及相关畸形情况。使用自体颅骨骨移植、聚己内酯（PCL）骨支架填充物（Osteopore）和钛网对骨缺损进行重建。
在我们的研究中，评估了54例年龄在2个月至14岁之间的患者（34例男孩和20例女孩）。额筛型（44.5%）和枕型脑膨出（25.9%）是最常见的类型。所有病例均采用一期修复硬膜缺损或使用帽状腱膜进行修复。12例（22.22%）患者使用自体颅骨骨移植闭合骨缺损。8例儿童使用钛网，4例使用Osteopore PCL骨支架填充物。其余30例儿童因骨缺损较小未进行颅骨成形术。总体而言，80%的患者术后无问题，在术后7至10天出院。脑脊液漏是最常见的术后并发症，5例患者出现此情况。1例患者进行了再次探查修复，其余4例采用保守治疗。总体而言，美容效果良好，在最后一次随访时，家长将美容效果评为良好至优秀的占70%，满意的占18%，差的占3%。
我们的研究表明，脑膨出与复杂畸形相关，给神经外科医生带来了技术挑战。处理这些病例需要多学科方法。头颅MRI和三维CT有助于更好地评估畸形情况，应尽快进行手术矫正以防止进一步的神经功能缺损。硬膜缺损修复和颅骨缺损重建可带来良好的长期效果。我们介绍了在仅6年时间内手术治疗54例颅脑膨出病例的经验，这是亚洲报道的最大系列之一。
