Kluth Type I-2 Variant of Pure Esophageal Atresia - First Case Report and Challenges in its Management.

This is the first case report of the Kluth type I-2 variant of esophageal atresia. The peculiar anatomy of this variant does not suit (1) esophageal substitution via posterior mediastinal route, (2) esophageal lengthening for preserving native esophagus and (3) distal esophageal stump stoma for gastric feeds. Copyright:

INTRODUCTION

Pure Esophageal atresia (PEA) is a rare anomaly with an incidence of 6%.[1] We report a case of one such rare variant, Kluth type I-2 and the challenges faced in its management. This is the first-ever report of this rare variant, to the best of our knowledge, after its first description by Brodie in 1810.[2]

CASE REPORT

A 5-month-old male child presented to us with cervical esophagostomy and gastrostomy done elsewhere for Gross type-A esophageal atresia (EA). The history revealed delivery by cesarean section at 37 weeks with a birth weight of 2.8 kg. Antenatal ultrasonography had shown polyhydramnios and absent fetal stomach bubble. Postnatally, the APGAR score was 8 and 9 at 1 and 5 min, respectively; there was frothing of saliva from the mouth and a nasogastric tube could not be passed beyond 11 cms from the incisor teeth. X-ray chest showed gasless abdomen with the tip of the nasogastric tube at the level of T-4 vertebra. There were no other associated anomalies.

He was thriving well on oral sham feeds followed by gastrostomy feeds. An esophageal replacement, preferably gastric transposition via posterior mediastinal route, was planned at 1 year of age. A contrast study done at 10 months via gastrostomy showed a good caliber stomach and the lower esophageal pouch was not opacified [Figure 1a]. Intraoperatively, to our surprise, lower esophageal pouch was absent [Figure 1b] with no esophageal hiatus in the diaphragm to guide the creation of posterior mediastinal space. Persistence to create posterior mediastinal route would have placed adjacent structures, particularly aorta and inferior vena cava at risk of injury, so it was abandoned. As the length of the stomach was adequate, the stomach was pulled up via the retrosternal route after closing the gastrostomy; a feeding jejunostomy was also placed for early enteral feeds during the early postoperative period. The postoperative course was uneventful and he was shifted on oral feeds after 2 weeks of jejunostomy feeds. At 9 months follow-up, the child is tolerating oral feeds well and has achieved normal milestones with adequate height and weight above the 50th centile of the growth chart.

Figure 1

(a) Contrast study via gastrostomy typically shows nonopacification of lower esophagus even on completely filled stomach. (b) Intraoperative photograph of mobilized stomach with absent lower esophageal stump

DISCUSSION

In clinical practice, the Gross classification for EA is one of the most frequently used.[1] There are many variants among these types highlighted by Kluth who divided EA into 10 broad types and 96 subtypes based upon various anatomical characteristics.[2] We encountered this very rare Kluth type I subtype 2 variant with a proximal blind-ending upper esophagus without any recognizable lower esophagus above the diaphragm. This was first described by Brodie in 1810 as mentioned in Kluth's compilation of variants of EA.[2] This anomaly was an intraoperative surprise for us. Although in retrospect, an important finding in the preoperative contrast study done via gastrostomy points toward this variant. The nonopacification of the lower esophagus, despite the stomach being completely filled with contrast, corresponds with our intraoperative finding.

Cervical esophagostomy and feeding gastrostomy are the most common procedures done initially for PEA, especially in the Indian subcontinent.[3] Authors prefer gastric transposition via posterior mediastinum being the shortest route. But, in this variant, due to absent lower esophageal pouch and diaphragmatic esophageal hiatus, there was no natural anatomical plane to create posterior mediastinal space for the gastric pull-up. Blind dissection would have put the diaphragm, major vessels (inferior vena cava and aorta), azygous and Hemi-azygous vein, vagus nerves, and thoracic duct at significant risk of injury. Since the stomach was adequate in size, the retrosternal route was chosen for gastric transposition after closing the gastrostomy. It is interesting to note that there was no additional suture line, other than the gastrostomy site in the stomach transposed in the mediastinum, due to closure of the lower esophageal stump as it was absent. Alternative surgical options for esophageal replacement in this variant can be colonic or jejunal interposition via retrosternal route.

The nonavailability of the lower esophagus in this variant is a limitation for various esophageal lengthening procedures.[1] Bhatnagar et al. recommended an abdominal esophagostomy in the left upper abdominal wall utilizing the lower esophageal stump in cases of long gap EA enlisting its advantages over the conventional Stamm gastrostomy in the anterior wall of the stomach;[4] such a procedure will be not possible in Kluth type I-2 variant of PEA.

CONCLUSION

Kluth type-1 subtype-2 is an extremely rare subtype of EA. To the best of our knowledge, this is the first case report of such a variant after its initial description by Brodie in 1810[2]

On radiology, nonvisualization of the lower esophagus despite contrast completely filling the stomach raises a strong suspicion regarding the presence of this rare variant

Its anatomy extends an advantage, during gastric transposition, due to the absence of an additional suture line on the stomach from the excision of the remnant lower esophageal stump

The surgical options for esophageal substitution in this variant are (1) if stomach size is adequate either gastric transposition or gastric tube via retrosternal route, (2) if the stomach is small, then colonic or jejunal interposition

This variant is a contraindication for the following procedures (1) esophageal substitution via posterior mediastinal route, (2) esophageal lengthening to preserve native esophagus, and (3) stoma using distal esophageal stump for gastric feeds.

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Conflicts of interest

There are no conflicts of interest.