Thoracoscopic oesophago-oesophagostomy in the prone position for oesophageal stenosis caused by dilated azygos vein in polysplenia-associated heterotaxy.

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.

INTRODUCTION

Heterotaxy syndrome is rarely observed beyond the paediatric age group because it is characterised by a high childhood mortality due to cardiac malformations. The polysplenia variant of heterotaxy syndrome is associated with less severe cardiac anomalies with survival of 5%-10% of patients into adulthood.[1] Those that survive report to a clinician owing to the extracardiac manifestations that may involve the digestive tract, peripheral vascular system and genitourinary abnormalities along with splenic abnormalities. A 17-year-old boy presented with dysphagia and failure to thrive secondary to oesophageal stenosis owing to a prolonged compression by a dilated azygos vein, with multiple other anatomic abnormalities defining a syndromic possibility not reported so far in the English literature. Despite the presence of diverse anatomic abnormalities, it was only his symptom of dysphagia due to oesophageal stricture that merited surgical intervention. 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 presented to our surgical services with intermittent dysphagia and emesis immediately after oral intake for a duration of 5 years associated with failure to thrive. HHis imaging [Supplementary Figures 1–4), clinical evaluation and biochemistry revealed the following patho anatomic and biochemical abnormalities [Figure 1]:

Figure 1

A contrast-enhanced computed tomography scan showing: (a) oesophageal stenosis (red arrow) with proximal dilatation (black arrow). (b) Annular pancreas (yellow asterisk) and multiple spleens (red asterisk). (c) Hepatic vein draining into the right atrium (black arrow). (d) Post-operative computed tomography scan showing resolution of stenosis with passage of contrast into the distal oesophagus (red arrow)

Mid-oesophageal stenosis

Absent intrahepatic segment of inferior vena cava (IVC)

Hepatic veins draining directly into the right atrium

Continuation of the IVC as a dilated azygos vein causing a persistent compression, leading to a fibrotic stricture of the oesophagus

Aberrant right subclavian artery

Right common carotid artery coursing anterior to the trachea

Complete annular pancreas

Polysplenia

Torticollis

Iron deficiency anaemia possibly owing to malnutrition.

A single previous attempt at endoscopic dilatation had failed because the guide wire could not be passed through the lumen. After approval from the institutional review board, he underwent thoracoscopic correction in the prone position [Figure 2]. After ensuring adequate mobilisation and preserving both the vagi, the two segments after transection were brought heterotopically (without segmental resection) in counter-position to the dilated azygos vein with the intention of performing an azygos-preserving extramediastinal oesophago-oesophagostomy. The oesophagus was stapled-transected just above the fibrotic stricture, and then the back wall of both segments (upper dilated segment and lower collapsed segment below the stricture) was anastomosed with another 45-mm linear stapler (Ethicon Endo-Surgery, Echelon Flex, Cincinnati, OH, USA) that cut through the previous staple lines. The anastomosis performed can be considered modified Collard type,[2] however it was oesophago-oesophageal and the available proximal stump was an already-dilated proximal oesophagus. A 36 F bougie was then passed across the neo lumen, and the closure of the anterior layer entirely hand sewn in two layers was effectively undertaken [Video 1].

Figure 2

The intraoperative pictures showing: (a) a grossly dilated azygos vein compressing the oesophagus (E). (b) The oesophagus has been completely mobilised. (c) A plane has been created between the azygos vein and the oesophagus. (d) The oesophagus is transected with an endostapler. (e) Oesophagotomy made on the anterior wall of both the segments. (f) The limbs of the stapler pass through the enterotomies opposing the back walls to create a modified Collard-type anastomosis. (g) A bougie is passed to calibrate the closure. (h) The anastomosis is completed by hand-sewn anterior closure

RESULTS

The post-operative course was uneventful with initiation of oral feeds on the 6th and discharge on the 8th post-operative days. Contrast study showed no residual or recurrent stenosis after 12 months of follow-up [Figure 3]. At 36 months of follow-up, he is free of symptoms, has gained weight and his haemoglobin has normalised to 12 g/dl.

Figure 3

A barium swallow on follow-up showing smooth passage of contrast with no evidence of re-stenosis

DISCUSSION

Oesophageal stenosis may result from prolonged compression by anomalous vasculature. A strategic surgical plan addressing the affecting factors with the aim of obtaining cure with minimal morbidity needs to be embraced. First, in view of the thick-walled stricture and proximal dilatation of the oesophagus indicating a long-standing unrelenting fibrotic stricture with proximity to major vascular structures, endoscopic therapy was not pursued after the single failed attempt to pass the guide wire in order to avoid any complications amidst the deranged vascular anatomy. Second, the associated vascular anomalies had a bearing on the mode of surgical treatment, and correspondingly a cardio-thoracic surgeon was kept on standby. Third, the extension of the IVC draining the venous return of the entire lower body as the azygos vein, could not have allowed the division with impunity or handling without a veno-venous bypass. To circumvent the need for azygos vein sectioning and a major vascular reconstruction, a vessel-preserving approach was adopted in the form of a thoracoscopic correction to bring the oesophagus in a heterotopic position. To prevent tension and avoid post-operative occurrence of a stricture, specific manoeuvres can be carried out including transecting and re-anastomosing without a segmental resection, followed by restoring the continuity by means of a modified Collard-type anastomosis and closure of the common enterotomy in a calibrated manner over a bougie. Consent had been taken for an Ivor-Lewis procedure and gastro-oesophageal anastomosis in the chest in case the original plan of oesophago-oesophageal anastomosis did not work out.

Numerous anomalies and combinations exist in a patient with polysplenia, with no single anomaly being pathognomonic. Vascular malformations such as an aberrant subclavian artery or double aortic arch,[345] or a dilated azygos vein, can abut or compress the oesophagus, giving rise to occlusive symptomatology. This case demonstrates the co-existence of such anomalies in the form of a dilated azygos vein and an aberrant right subclavian artery, with oesophageal compression caused predominantly by the large and voluminous azygos vein as depicted by the pre-operative imaging and the intraoperative finding. This could represent a different variety of 'dysphagia lusoria' by compressing the oesophagus. Our surgical approach to bring the oesophagus heterotopically by effecting a gastrointestinal reconstruction served the purpose of eliminating these vascular causes of oesophageal compression without having to undertake a complex vascular reconstruction.

An increased flow and subsequent dilatation of azygos or haemiazygos vein can be a result of obstruction of IVC, portal vein hypertension, right heart failure or interruption of IVC in a case of heterotaxy syndrome.[6] Implications of clinical importance include simulating a mediastinal mass or retroperitoneal lymphadenopathy, occurrence of deep-vein thrombosis, respiratory problems due to extrahepatic portocaval shunts, right heart failure due to an increased blood flow and oesophageal compression and resultant stricture, as seen in the present case.

Oesophageal stenosis due to a dilated azygos vein in a patient with polysplenia has not been reported. 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 with excellent outcomes justified the treatment rendered to the index patient.

CONCLUSION

High mortality owing to cardiac abnormalities permits only few children to reach adulthood and the attending patho-anatomic abnormalities come to the clinician very sparingly. Some with polysplenia may have a surgically correctable dysphagia owing to a fibrotic stricture out of a prolonged compression by a dilated azygos vein. An excellent outcome can be achieved with a thoracoscopic procedure after a thorough work-up and pre-operative planning. 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 in adults.

Declaration of patient consent

The authors certify that they have obtained all appropriate patient consent forms. In the form, the legal guardian has given his consent for images and other clinical information to be reported in the journal. The guardian understands that names and initials will not be published and due efforts will be made to conceal identity, but anonymity cannot be guaranteed.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.

Contrast-enhanced computed tomography scan with coronal section of the mediastinum showing the aberrant subclavian artery (blue block arrow), the dilated azygos vein (red block arrow) causing compressive effect over the oesophagus (yellow block arrow)

Sagittal section of a contrast-enhanced computed tomography scan showing the dilated azygos vein (red arrow) as a continuation of the inferior vena cava, entering the superior vena cava (yellow asterisk) and compressing the oesophagus (black arrow) during its course

Axial sections of a contrast-enhanced computed tomography scan showing the aberrant right subclavian artery arising from the aortic arch (red block arrow). Also seen is the dilated azygos vein (yellow block arrow)

Axial sections showing the dilated azygos vein (yellow arrow) causing compressive effect over the oesophagus (red arrow)