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Journal of Indian Association of Pediatric Surgeons
     Journal of Indian Association of Pediatric Surgeons
Official journal of the Indian Association of Pediatric Surgeons         
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Year : 2014  |  Volume : 19  |  Issue : 2  |  Page : 112-114

Pyloric stenosis in a patient with pure esophageal atresia: A difficult diagnosis

Consultant Pediatric Surgeon, Department of Pediatric Surgery, Columbia Asia Hospitals, Kolkata, India

Date of Web Publication29-Mar-2014

Correspondence Address:
Anindya Chattopadhyay
A-807, Siddha Pines, Rajarhat Main Road, Kolkata - 700136
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/0971-9261.129610

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Hypertrophic pyloric stenosis is brought to attention by its characteristic non-bilious vomiting. In a patient with pure esophageal atresia and a feeding gastrostomy, the symptoms were modified and the diagnosis was delayed. This case report highlights the clinical features of this rare combination, whose diagnosis was easily established once the entity was considered.

Keywords: Esophageal atresia, hypertrophic pyloric stenosis, retching

How to cite this article:
Chattopadhyay A. Pyloric stenosis in a patient with pure esophageal atresia: A difficult diagnosis. J Indian Assoc Pediatr Surg 2014;19:112-4

How to cite this URL:
Chattopadhyay A. Pyloric stenosis in a patient with pure esophageal atresia: A difficult diagnosis. J Indian Assoc Pediatr Surg [serial online] 2014 [cited 2020 Sep 25];19:112-4. Available from: http://www.jiaps.com/text.asp?2014/19/2/112/129610

   Introduction Top

The association of pyloric stenosis and esophageal atresia with tracheoesophageal fistula, although rare, is well documented. [1],[2] However, pyloric stenosis in a patient with pure esophageal atresia having both gastrostomy and esophagostomy produced puzzling symptoms and signs, thereby delaying the diagnosis. The case report highlights the clinical features of pyloric stenosis in a child with gastrostomy.

   Case Report Top

A two-day-old, 1.8-kg preterm newborn was admitted with a history of frothing from the mouth and a failure to pass a nasogastric tube into the stomach. On examination the neonate was active, pink, and icteric. His vitals were stable, and his chest was clear. A No. 10 red rubber catheter could not be negotiated beyond 10 cm from the alveolar margin, confirming the diagnosis of esophageal atresia. A chest and abdominal X-ray revealed a gasless abdomen and no other abnormalities. Echocardiogram was normal. His hemogram and blood biochemistry were normal. The child was taken up for surgery and feeding gastrostomy and cervical esophagostomy were performed, as the parents of the child were unwilling to attempt a delayed primary esophageal repair.

The postoperative course was benign and the child was discharged on the seventh postoperative day with gastrostomy feeds (30 ml every 2 h) and sham feeding by mouth. Screening for VACTERL anomalies was done and the result was negative. Over the next four weeks, the feeds were gradually increased to 45 ml every 2 h and the child weighed 3 kg.

In the next week, whenever the parents attempted to increase the feed to more than 45 ml, the infant had a retching response, which settled over the next 15-20 minutes. This occurred over two days, and the parents reported that the child had become lethargic. Suspecting sepsis, the child was readmitted and a sepsis screening was performed; the child was started on broad spectrum antibiotics, the feeds continued, and the retching response observed and documented. The sepsis screening was negative; moreover, the retching stopped spontaneously and the child's activities became normal. No blood gas analysis was done at this time. The child was discharged after four days, and the parents were asked to continue feeds at 40-45 ml. At the next follow-up visit after one week, the parents reported that the retching persisted, and occasionally there was a gush of residual milk from the gastrostomy prior to feeds. The infant had lost weight and now weighed 2.75 kg. There was no visible gastric peristalsis, nor was there any mass palpable per abdomen. However, suspecting a gastric outlet obstruction, an ultrasonography (USG) and barium meal were ordered. The USG showed a pyloric length of 20 mm and a thickness of 4 mm [Figure 1], while barium was also suggestive of pyloric stenosis [Figure 2]. The child's hemogram, electrolytes, and acid-base analysis were normal and the child was taken up for surgery. A fresh transverse incision was made and the pylorus delivered after releasing adhesions with the liver. The findings confirmed pyloric stenosis, and a pyloromyotomy was done. The postoperative course was uneventful and the child was restarted on feeds after 24 hours, which rapidly progressed to 50-ml feeds every 2 h without any retching. The child gained weight and recently underwent an uneventful gastric pull-up, weighing 8 kg at 8 months of age.
Figure 1: USG showing thickened pyloric musculature

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Figure 2: Barium meal: Note the string sign and reflux into the distal blind esophagus

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   Discussion Top

Infantile pyloric stenosis (IHPS) has been reported to occur more frequently in patients with esophageal atresia and tracheoesophageal fistula (OA TEF) as compared to the general population. [1],[2] A review in 1986 reported two such cases and calculated that 1-10% of patients with OA TEF develop pyloric stenosis. [1] Out of 371 patients, Deurloo et al. calculated a 6% incidence of IHPS in all OA with TEF survivors, with a 13% incidence in patients with pure atresia. [2] Vagal injury, performance of a gastrostomy, and transpyloric feeding tubes have been implicated in the increased incidence. [2],[3],[4] Interestingly, no such reports are available from India.

The chief symptom of pyloric stenosis is non-bilious vomiting, and in patients with repaired OA TEF, such vomiting is well known as a result of reflux or anastomotic stricture, leading to a delay in the diagnosis. [5],[6] In the presence of a gastrostomy and esophagostomy, however, vomiting cannot occur, and this neonate had pronounced retching whenever a large feed was delivered. The response was thought to be due to overfill of the stomach and reflux into the lower esophagus, along with a centrally mediated emesis reflex. Since the esophagus was disconnected in the patient, this resulted in the strange "retching" type response in the patient. In addition, there was the sudden backflow of the previously administered feed reminiscent of projectile vomiting, which led us to think of pyloric stenosis. Once suspected, the diagnosis was easily established by standard radiological investigations and treatment was relatively straightforward.

As stated before, although pyloric stenosis occurs more frequently in patients of OA and TEF, it is relatively rare. There has been only one report of pyloric stenosis in an H-type TEF, [7] and one in a patient with membranous esophageal atresia [8] ; we believe this report is the first to describe the condition in a patient with both esophagostomy and gastrostomy. Awareness of the relationship and its presentation should make early diagnosis feasible.

   References Top

1.Magilner AD. Esophageal atresia and hypertrophic pyloric stenosis: Sequential coexistence of disease (case report). AJR Am J Roentgenol 1986;147:329-30.  Back to cited text no. 1
2.Deurloo JA, Ekkelkamp S, Schoorl M, Heij HA, Aronson DC. Esophageal atresia: Historical evolution of management and results in 371 patients. Ann Thorac Surg 2002;73:267-72.  Back to cited text no. 2
3.Nasr A, Ein SH. Postoperative pyloric stenosis in the newborn: A forgotten problem. J Pediatr Surg 2007;42:1409-11.  Back to cited text no. 3
4.Czernik J, Raine PA. Esophageal atresia and pyloric stenosis: An association. Eur J Pediatr Surg 1982;35:18-20.  Back to cited text no. 4
5.Latchow LA, Jacir NN, Harris BH. The development of pyloric stenosis during transpyloric feedings. J Pediatr Surg 1989;24:823-4.  Back to cited text no. 5
6.Qvist N, Rasmussen l, Hansen LP, Pederson SA. Development of infantile hypertrophic pyloric stenosis in patients treated for oesophageal atresia: A case report. Acta Chir Scand 1986;152:237-8.  Back to cited text no. 6
7.Oðuzkurt P, Tanyel FC, Haliloðlu M, Hicsönmez A. An uncommon association of H-type tracheoesophageal fistula with infantile pyloric stenosis. Turk J Pediatr 1999;41:143-6.  Back to cited text no. 7
8.Chuang JH, Chen MJ. Membranous atresia of esophagus associated with pyloric stenosis. J Pediatr Surg 1987;22:988-90.  Back to cited text no. 8


  [Figure 1], [Figure 2]


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