Bhojwani Rajesh, Jain Nikhil
Department of Surgical Gastroenterology and Minimal Invasive Surgery, Santokba Institute of Digestive Surgical Sciences, Santokba Durlabhji Memorial Hospital Cum Medical Research Center, Jaipur, Rajasthan, India.
J Minim Access Surg. 2021 Jul-Sep;17(3):395-398. doi: 10.4103/jmas.JMAS_313_20.
Heterotaxy syndrome is associated with a plethora of cardiovascular and other multi-system anomalies with a high childhood mortality. A dilated azygos vein as part of the polysplenia variant of heterotaxy syndrome may cause oesophageal stenosis owing to a prolonged compression. We describe our technique of extramediastinal oesophago-oesophagostomy in the prone position for this rare congenital syndromic malformation with an excellent outcome.
A 17-year-old boy with heterotaxy syndrome presented with intermittent dysphagia and postprandial emesis with failure to thrive. Despite the presence of diverse anatomic abnormalities, it was only his symptom of dysphagia due to oesophageal stricture that merited surgical intervention. He underwent an azygos-preserving extramediastinal oesophago-oesophagostomy in the prone position without segmental resection with the establishment of continuity using a modified Collard-type anastomosis.
The patient had an uneventful convalescence, with imaging after 1 year showing no re-stenosis. After a follow-up of 3 years, the patient is free of symptoms and has gained weight.
Oesophageal stenosis may result from prolonged compression by anomalous vasculature. An isolated correctable anatomic derangement, young age with good functional reserve, other associated anomalies not causing any symptoms, the physiological advantages of executing the surgery in a prone position and availability of expertise in minimally invasive surgery ensured excellent outcomes. The hitherto unreported technique may open up avenues for further research regarding the behaviour of the oesophageal muscular tube with transection and re-anastomosis for rare benign abnormalities.
内脏反位综合征与大量心血管及其他多系统异常相关，儿童死亡率很高。作为内脏反位综合征多脾变异型一部分的奇静脉扩张，可能因长期压迫导致食管狭窄。我们描述了在俯卧位进行纵隔外食管-食管吻合术的技术，用于这种罕见的先天性综合征畸形，效果良好。
一名17岁患有内脏反位综合征的男孩，出现间歇性吞咽困难、餐后呕吐且发育不良。尽管存在多种解剖异常，但只有因食管狭窄导致的吞咽困难症状值得进行手术干预。他在俯卧位接受了保留奇静脉的纵隔外食管-食管吻合术，未进行节段性切除，采用改良的科拉德式吻合术建立连续性。
患者恢复顺利，1年后的影像学检查显示无再狭窄。随访3年后，患者无症状且体重增加。
食管狭窄可能由异常血管的长期压迫引起。孤立的可纠正解剖紊乱、功能储备良好的年轻患者、其他未引起任何症状的相关异常、在俯卧位进行手术的生理优势以及微创手术专业知识的可得性确保了良好的结果。这种迄今未报道的技术可能为进一步研究食管肌性管在横断和重新吻合治疗罕见良性异常时的行为开辟途径。
