Kluth Type IV3 Membranous Esophageal Atresia at Middle One-third of Esophagus: An Extremely Rare Entity.

We herein present an extremely rare case of an isolated membranous atresia causing near-complete obstruction of the esophagus. The neonate presented with drooling of saliva and frothing from the mouth. A red rubber catheter met with an obstruction at 12 cm from the gum margins. Radiograph showed paucity of gas in the abdomen. Thoracotomy revealed external continuity of esophagus; dilated and elongated proximal segment constituting upper half of esophagus, and a membrane with tiny opening in the center, at its junction with narrow distal segment. Excision was performed. A high index of suspicion for membrane should be kept in dealing with esophageal obstruction beyond 10 cm from the gum margins, particularly those with paucity of abdominal gas pattern or gasless abdomen.

INTRODUCTION

Esophageal obstruction in the newborn is usually related to esophageal atresia (EA) with trachea-esophageal fistula (TEF).[1] Congenital membranous esophageal obstruction is rare anomaly.[23] We herein present an extremely rare case of an isolated membranous atresia with tiny opening communicating proximal and distal segments, causing near-complete obstruction of the esophagus, and mimicking EA with TEF.

CASE REPORT

A 1-day-old, low birth weight (1.8 kg) female neonate presented to us with drooling of saliva and frothing from the mouth. The patient was premature with 36 weeks' gestational age. On examination, the patient was stable, respiratory rate - 62/min, and pulse rate - 160/min. There was absence of abdominal distension. Perineal examination revealed vestibular fistula. After oropharyngeal suctioning, a soft, 10F red rubber catheter was passed in the esophagus, but met with an obstruction at 12 cm from the gum margins suggesting the presence of EA. A radiograph with red rubber catheter in place assured EA. It also showed the absence of gastric shadow and paucity of gas in the abdomen. Right posterolateral extrapleural thoracotomy was performed through the 5th intercostal space. The distal esophagus was mobilized and the presence of distal TEF was ruled out [Figure 1]. The proximal segment (upper half) of esophagus was dilated similar to that seen in proximal pouch of EA and was also elongated. External continuity of esophagus was demonstrated [Figure 1]. The distal segment of esophagus was narrow and continuous with proximal segment at posteromedial aspect [Figure 1]. An esophagotomy was performed; a membrane with tiny opening in the center was identified [Figure 2]. Excision of short segment with the web was done and end-to-end esophageal anastomosis was performed. Postoperative dye study was normal. There were no postoperative complications. The patient was passing meconium stools from the vestibular fistula and decision was taken to perform primary repair in the subsequent admission.

Figure 1

Peroperative photograph showing upper pouch with red rubber catheter in situ (green arrow) and narrow distal segment (white arrow) in the sling. Inset image (on right) showing external continuity of esophagus with dilated and elongated proximal segment of esophagus (black arrow) constituting its upper half and also narrow distal segment (white arrow) of esophagus continuous with proximal segment at posteromedial aspect

Figure 2

Diagrammatic representation of membranous esophageal atresia at middle one-third of esophagus, with a tiny opening in the center of the membrane is present

DISCUSSION

Tenon was first to describe the “membranous obstruction of the gullet in its upper part” in 1791.[23] Rossi described the first case of distal esophageal membrane in 1826.[23] Abel and Schwartz described membranous obstruction in middle-third of the esophagus.[23] Kate in the year 1952 described a case where the membrane did not occlude the lumen completely and there was fistulous tract between the proximal and distal segments; the membrane was present in the upper esophagus.[4]

Kluth in 1976 divided EA into ten large classifications and ninety-six small types based on the presence or location and number of TEF, the gap distance, and shape of the upper pouch and the presence of cyst, stenosis, strands, membranes, duplications, tracheal abnormalities, esophagobronchial communication, and fissure.[5] Membranous atresia is type IV and is further divided into 7 types from IV1 to IV7 on the basis of associated anomalies, with or without fistulae at various sites.[5] Sharma et al. divided type IV into subtypes A and B on the basis of absence and presence of associated TEF, respectively.[3] We believe that our case is similar to that described by Kate and classified by Kluth as type IV3, with a small difference that in our case, (a) the membrane was present at middle of the esophagus and (b) tiny opening in the center of the membrane. Our case is similar to the case described by Goldenberg and Smith in 1961, who reported a neonate with midesophageal web with pinpoint central opening.[6] Authors believe that it would be inappropriate to classify the present case into stenosis of the esophagus (Kluth type VIII2) as pathology in membranous obstruction is intraluminal rather than intramural or external in case of stenosis.

The presentation of congenital membranous esophageal obstruction is similar to EA with TEF as there is drooling of saliva and frothing from the mouth.[234] Impediment to red rubber catheter may be observed according to the level of membrane.[234] The site of web is usually in the lower one-third followed by middle and upper one-third of the esophagus.[234678] Plain radiography shows gasless abdomen in case of isolated membranous obstruction or subtype A (according to Sharma et al.), EA without TEF and EA with proximal fistula; however, in our case, there was presence of some abdominal gas owing to tiny communication between the upper and lower segments, which is extremely rare.[6] The presence of gas signifies the presence of distal fistulous communication. Our case mimicked EA with TEF. Gastric gas shadow was absent in our case as seen in the preoperative radiograph.

The presence of abdominal gas and level of obstruction (indicated by red rubber catheter in situ or by esophagogram) dictates the surgical approach for excision of membrane. Isolated membranous obstruction of esophagus has been managed in the past primarily by endoscopic method or after performing gastrostomy.[39] In cases with TEF, management is entirely surgical. Right posterolateral thoracotomy approach is ideal for upper and middle esophageal webs and also those associated with TEF; approach is left sided for isolated lower one-third web as both distal esophagus and stomach can be mobilized (if required) and also for the right descending aorta.[710] An esophagotomy incision is given after identification of the obstruction by already placed catheter in the esophageal lumen or by noting the change in caliber of the esophagus; excision of the membrane and closure is done transversely. Excision of short segment along with the web and anastomosis may be performed.[7] Very rarely, in case of isolated webs near the esophagogastric junction, abdominal approach has been performed with favorable outcome.[8]

Thus, in our case, due to the presence of midesophageal obstruction and presence of abdominal gas pattern, thoracotomy was undertaken from right side and repair was performed primarily without attempting gastrostomy. After review of pertinent literature and because of the typical embryology in the formation of esophagus, we recommend surgical management instead of endoscopic treatment in the management of esophageal membrane for favorable outcome.

A high index of suspicion for membrane should be kept in dealing with esophageal obstruction beyond 10 cm from the gum margins, particularly those with paucity of abdominal gas pattern or gasless abdomen.

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Conflicts of interest

There are no conflicts of interest.