AbouZeid Amr Abdelhamid, Mohammad Shaimaa Abdelsattar, Abolfotoh Mohammad, Radwan Ahmed Bassiouny, Ismail Mohamed Mohamed ElSayed, Hassan Tarek Ahmed
Pediatric Surgery Department, Faculty of medicine, Ain-Shams University.
Radiodiagnosis Department, Faculty of medicine, Ain-Shams University.
J Pediatr Surg. 2017 Aug;52(8):1260-1268. doi: 10.1016/j.jpedsurg.2016.12.010. Epub 2016 Dec 27.
We report our experience in managing a group of patients with Currarino syndrome, highlighting diagnostic challenges, surgical techniques, in addition to a review of current neurosurgical options.
The study included patients with Currarino syndrome who presented to our pediatric surgery department during the period 2010 through 2016. The 'sacral scimitar' in plain X-ray provided the clue for the diagnosis; while MRI examination was essential to define the nature of the presacral mass and associated spinal anomalies.
The study included 17 patients (13 girls and 4 boys). Their age at presentation ranged from 7months to 10years. We used posterior sagittal approach to correct anorectal anomalies, and excise presacral cysts that were subjected to histopathological examination. Two cases presented with a pelvic abscess (infected presacral dermoid cyst), which were initially drained followed by excision. The presacral mass consisted of either lipomyelocele (6), lipomyelomeningocele (3), or a developmental (dermoid) cyst (8). Tethering of the spinal cord was a common association (70%) CONCLUSION: Apart from diagnostic challenges, the management of Currarino syndrome is similar to the usual management of ARM regarding the surgical approach and probably the prognosis that mainly depends on degree of associated sacral dysplasia.
This is a case series with no comparison group (level IV).
我们报告了管理一组Currarino综合征患者的经验，重点介绍了诊断挑战、手术技术，并对当前的神经外科治疗选择进行了综述。
该研究纳入了2010年至2016年期间到我们儿科外科就诊的Currarino综合征患者。X线平片中的“骶弯刀征”为诊断提供了线索；而MRI检查对于明确骶前肿块的性质及相关脊柱异常至关重要。
该研究纳入了17例患者（13例女孩和4例男孩）。他们就诊时的年龄范围为7个月至10岁。我们采用后矢状入路纠正肛门直肠畸形，并切除接受组织病理学检查的骶前囊肿。2例患者出现盆腔脓肿（感染性骶前皮样囊肿），最初进行了引流，随后切除。骶前肿块包括脂肪脊髓膨出（6例）、脂肪脊髓脊膜膨出（3例）或发育性（皮样）囊肿（8例）。脊髓栓系是常见的伴随情况（70%）。结论：除了诊断挑战外，Currarino综合征的治疗在手术方法以及可能的预后方面与肛门直肠畸形的常规治疗相似，预后主要取决于相关骶骨发育不良的程度。
这是一个无对照组的病例系列（IV级）。
