Background:
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.
Patients and Methods:
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.
Results:
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.
Conclusion:
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.
The association of gastroenteropancreatic neuroendocrine tumours (GEP-NETs) with obesity has been reported and researched on. Rendering of a laparoscopic treatment treating these concurring pathologies in unison has not been described. Two morbidly obese patients with duodenal NETs underwent a resectional procedure, with curative intent, in the form of laparoscopic subtotal gastrectomy with roux-en-y gastrojejunostomy with partial duodenectomy and a laparoscopic one-anastomosis gastric bypass-mini gastric bypass with remnant gastrectomy and partial duodenectomy. Both patients had an uneventful convalescence with acceptable weight loss and no evidence of tumour recurrence on follow-up. The indolent nature of NETs, as compared to the morbidity of obesity provides the rationale for treating this particular cohort of patients with a surgical procedure that would serve to remove the tumour and also provide therapeutic benefit for obesity. With experience in advanced laparoscopic procedures, this can be accomplished safely with acceptable results.
The laparoscopic management of hepatobiliary pathology is an established mode of treatment. Incomplete excision of choledochal cyst with the resultant complications is a distinct surgical pathology, the treatment of which can be rendered based on the philosophy of minimally invasive approach which is now an acceptable treatment for the primary condition itself. We describe a case of hepaticojejunostomy site stricture associated with incomplete cyst excision managed laparoscopically. A redo procedure is technically demanding considering the presence of adhesions and a difficult to discern anatomy, but resulted in an excellent outcome. At centres with significant experience in laparoscopic surgery, redo procedures with a favourable impression on pre-operative work-up can be effectively treated with laparoscopy.
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