Al-Shanafey Saud, Harvey John
Department of Surgery, The Children's Hospital at Westmead, Sydney, Australia.
J Pediatr Surg. 2008 Apr;43(4):597-601. doi: 10.1016/j.jpedsurg.2007.12.001.
The diagnosis of long gap esophageal atresia (LGEA) may preclude immediate primary anastomosis. We reviewed our experience with this entity for a period of 10 years.
A retrospective review was undertaken of the medical records of all patients managed for esophageal atresia (EA)/tracheoesophageal fistula (TEF) during the period from 1991 to 2001 at the Children's Hospital at Westmead, Sydney, Australia. Esophageal atresia was defined as long gap when primary repair was considered technically impossible by the surgeon. Also, a questionnaire was sent to all the general pediatric surgeons in Australia to explore their attitude toward LGEA management.
One hundred three patients with EA were managed for that period, 17 (16%) of them were defined as LGEA, with mean gap of 5 cm (SD, 1cm). Eight patients (47%) had TEF. Sixteen patients had gastrostomy tube (GT) insertion at a mean age of 4 days. Six patients had esophagostomy at a mean age of 27 days. Thirteen patients had EA repair at a mean age of 146 days. Four patients died before repair and 2 after repair secondary to associated anomalies. Fifty percent of Australian Pediatric Surgeons (APS) responded to the questionnaire. Forty percent defined LGEA as a gap more than 3 to 4 vertebral bodies, whereas 24% considered the absence of TEF as an indication of LGEA. Fifty-six percent of APS will measure the gap on preoperative chest x-rays, and 80% will assess the gap at thoracotomy for ligation of TEF. Ninety-two will measure the gap by inserting a bougie into the upper pouch and into the lower esophagus via the GT. If LGEA was diagnosed, all APS will perform GT with delayed repair. Seventy-two percent of APS will attempt delayed primary repair within 3 to 6 months of age. Seventy-six percent will perform hiatal dissection, and 48% will use upper pouch myotomies. Forty-eight percent will perform gastric pull up, and 32% will use gastric tube for esophageal replacement.
Long gap esophageal atresia represents a surgical challenge. Mortality rate is high secondary to associated anomalies. There is no consensus among APS regarding the definition of LGEA. In general, the consensus of APS would be that the preservation of the patient's own esophagus should be attempted before considering the use of an esophageal replacement.
长段食管闭锁（LGEA）的诊断可能排除立即进行一期吻合术的可能性。我们回顾了我们在10年期间处理该疾病的经验。
对1991年至2001年期间在澳大利亚悉尼韦斯特米德儿童医院接受食管闭锁（EA）/气管食管瘘（TEF）治疗的所有患者的病历进行回顾性研究。当外科医生认为一期修复在技术上不可能时，食管闭锁被定义为长段。此外，还向澳大利亚所有普通儿科外科医生发送了一份问卷，以探讨他们对LGEA治疗的态度。
在此期间共治疗了103例EA患者，其中17例（16%）被定义为LGEA，平均间隙为5cm（标准差，1cm）。8例（47%）有TEF。16例患者平均在4天时插入胃造瘘管（GT）。6例患者平均在27天时进行食管造口术。13例患者平均在146天时进行EA修复。4例患者在修复前死亡，2例在修复后因相关畸形死亡。50%的澳大利亚儿科外科医生（APS）回复了问卷。40%将LGEA定义为间隙超过3至4个椎体，而24%认为无TEF是LGEA的一个指标。56%的APS会在术前胸部X光片上测量间隙，80%会在开胸结扎TEF时评估间隙。92%会通过经GT将探条插入上袋和下食管来测量间隙。如果诊断为LGEA，所有APS都会进行GT并延迟修复。72%的APS会在3至6个月大时尝试延迟一期修复。76%会进行裂孔解剖，48%会进行上袋肌切开术。48%会进行胃上提术，32%会使用胃管进行食管替代。
长段食管闭锁是一个手术挑战。由于相关畸形，死亡率很高。APS之间对于LGEA的定义没有共识。一般来说，APS的共识是在考虑使用食管替代物之前应尝试保留患者自身的食管。
