Home Print this page Email this page Small font sizeDefault font sizeIncrease font size
Users Online: 365
About us Editorial board Search Ahead of print Current issue Archives Submit article Instructions Subscribe Contacts Advertise Login 

 Table of Contents  
Year : 2019  |  Volume : 8  |  Issue : 4  |  Page : 245-247

Congenital pyloric atresia with multiple intestinal atresia: Indicators of this rare association

Department of Pediatric Surgery, PGIMER, Chandigarh, India

Date of Submission30-May-2019
Date of Decision19-Sep-2019
Date of Acceptance23-Sep-2019
Date of Web Publication04-Oct-2019

Correspondence Address:
Dr. Shailesh Solanki
Block 3A, Room No. 3103, Advance Pediatric Centre, PGIMER, Sector 12, Chandigarh - 160 012
Login to access the Email id

Source of Support: None, Conflict of Interest: None

DOI: 10.4103/jcn.JCN_66_19

Rights and Permissions

Congenital pyloric atresia (CPA) is one of the rare anomalies of the gastrointestinal tract, and when it is associated with other anomalies such as multiple intestinal atresia (MIA), the prognosis becomes poor. It is always difficult to know the associated intestinal condition before surgery. We here present a case of CPA with MIA with a review of pertinent literature. There are some pointers such as presentation with jaundice, history of MIA in the previous child, calcification on X-ray, and dilated common bile duct on investigation indicate toward this complex association.

Keywords: Congenital bile duct dilatation, congenital pyloric atresia, multiple intestinal atresia

How to cite this article:
Gunasekaran V, Solanki S, Menon P. Congenital pyloric atresia with multiple intestinal atresia: Indicators of this rare association. J Clin Neonatol 2019;8:245-7

How to cite this URL:
Gunasekaran V, Solanki S, Menon P. Congenital pyloric atresia with multiple intestinal atresia: Indicators of this rare association. J Clin Neonatol [serial online] 2019 [cited 2022 Aug 9];8:245-7. Available from: https://www.jcnonweb.com/text.asp?2019/8/4/245/268590

  Introduction Top

Congenital pyloric atresia (CPA) is a rare anomaly of the gastrointestinal (GI) tract with an incidence of 1 in 100,000 live births.[1] Isolated variant carries a better prognosis than those associated with other anomalies such as multiple intestinal atresia (MIA) and epidermolysis bullosa.[2] Management of CPA associated with other anomalies is difficult; preoperative knowledge of associated anomalies not only suggests prognosis but also helps in proper planning and management. We here describe a case of CPA associated with MIA.

  Case Report Top

A 4-day-old female neonate with a birth weight of 2.7 kg presented to the Emergency Department with the complaint of feed intolerance, nonbilious vomiting, and not passed stool since birth. The child was first in birth order to nonconsanguineous parents. An antenatal ultrasound at the third trimester was done, which was suggestive of dilated stomach, polyhydramnios, and subhepatic cystic lesion. On physical examination, the neonate was dehydrated and icteric with abdominal distension, and on insertion of a nasogastric tube, aspirate was nonbilious.

Abdominal X-ray revealed a dilated stomach with a single bubble and no distal bowel aeration. Ultrasonography showed dilated stomach and fusiform dilatation of the common bile duct (CBD) with a diameter of 7.4 mm along with dilatation of the intrahepatic biliary radicles, suggested the possibility of choledochal cyst. Upper GI contrast study [Figure 1]a revealed dilatation of the stomach with no distal passage of contrast beyond pylorus. Contrast-enhanced computed tomography of the abdomen showed a dilated stomach, duodenum, CBD [Figure 1]b and [Figure 1]c, and collapsed distal bowel. Blood investigation showed a total bilirubin of 12.9 mg/dl with a direct bilirubin of 7 mg/dl.
Figure 1: Contrast study shows dilated stomach with no contrast (black arrow) distally (a). Contrast-enhanced computed tomography shows (b and c) dilated stomach (red arrow), dilated common bile duct (white arrow), and dilated duodenum (black arrow). Intraoperative picture (d) of multiple intestinal atresias gives a string of beads appearance

Click here to view

On laparotomy, there was gross dilatation of the stomach and mild dilatation of the duodenum and proximal jejunum (20 cm from duodenojejunal junction [DJ]), and the rest entire small bowel was collapsed. The CBD was also uniformly dilated. There were indentation and thickening at the pylorus. On pylorotomy, a pyloric web (diaphragm with no hole) was present. Excision of the web and Heineke–Mikulicz pyloroplasty were done. Distally, 20 cm from the DJ, there was Type 1 jejunal atresia, it was opened, and distal intestinal patency was checked with saline infusion. There were multiple ileal atresias located at 70 cm distal to DJ till ileocecal junction and were presented almost after every few centimeters, giving “string of beads” appearance [Figure 1]d. The terminal ileal segment (approximately 20 cm) containing MIAs was resected [Figure 1]d and ileostomy was made at 70 cm from the DJ flexure after repairing the jejunal atresia with enteroplasty (Heineke–Mikulicz type). Histopathology of the resected segment showed denuded epithelium with focal ulceration composed of fibrocollagenous exudate with histiocytic collection and calcification. Inflammatory infiltrate with the area of hemorrhage was present in the mucosa and submucosa, and neuromatoid hyperplasia was present in the serosa. During the postoperative period, the neonate was managed with total parenteral nutrition. Even after 20 days of surgery, ileostomy was not functioning, and the parents refused to continue the further treatment.

  Discussion Top

CPA can be classified according to the defect: Type 1 – pyloric membrane (57%), Type 2 – pyloric atresia without a gap (34%), and Type 3 – pyloric atresia with a gap between the stomach and the duodenum.[2] CPA may manifest as isolated or along with other genetic disorders such as epidermolysis bullosa, aplasia cutis congenital, or as a part of hereditary MIA (HMIA). The CPA with HMIA is the most unusual form of this rare congenital malformation.[2],[3] The different proposed theories for etiology of these MIA are prenatal vascular insufficiency, an in utero inflammatory/infectious process, failure of recanalization, and abnormal epithelial ingrowth.[3]

Most of these pyloric atresias can be diagnosed antenatally with ultrasonography, demonstrating polyhydramnios and dilated stomach.[3] Postnatally, CPA with MIA presents with nonbilious vomiting, upper abdominal distension, and jaundice, as it was in the index case. Jaundice occurs due to a closed blind loop obstruction between the proximal atretic pylorus and the distal atretic duodenum or proximal jejunum.[4] This blind loop prevented the bile drainage, which resulted in extrahepatic obstructive cholestasis with the dilatation of intrahepatic and extrahepatic bile ducts which gives the appearance of choledochal cyst on ultrasonography and also on CT scan, as it was present in the index case.[5],[6]

The surgical management of CPA depends on its type. Simple excision and Heineke–Mikulicz pyloroplasty are enough for pyloric diaphragm (Type 1), as it was done in the index case. For Type 3 pyloric atresia, gastroduodenostomy is required to establish bowel continuity.[4] The patency of the distal intestines must be checked. In the index case, the distal intestine was unused, thick, and less distensible. During distal intestinal patency test, saline was not going freely, and there was some resistance during saline infusion. Reported literature mentioned sieve-like intestine in cases of HMIA, but in our case, neither it was apparent macroscopically nor pathologically. The possible surgical options for patients with multiple short segment intestinal atresia include multiple resection anastomosis, proximal and distal controlled fistulae with the possibility of subsequent reconstruction, bowel lengthening procedures, or bowel transplantation at a later stage.[2]

In the index case, after restoring intestinal continuity, the peristaltic activity did not appear even after 20 days. Reported literature suggested that in HMIA, bowel motility remains abnormal because of the continuous ongoing inflammatory process since the antenatal period.[3]

Cole et al. also described two cases of CPA with HMIA; in one case, there were multiple colonic atresias, giving the string of beads appearance. Both children died at around 3 months of age.[3] In the index case also, the intestine had the similar (string of beads) type of appearance, but it was in the ileum, and the rest of the colon was normal. Martin reported that the presence of calcification on plain abdominal radiography should raise the possibility of associated HMIA, but it was not present in the index case.[7] Snyder et al. proposed the characteristic features of HMIA and X-ray features of pyloric or duodenal atresia with the intraoperative finding of multiple atresias from the stomach to the rectum, along with dilatation of the bile ducts and the possibility of immunodeficiency and fatal outcome. The index case fulfilled many of the above-mentioned features such as pyloric atresia, jaundice, and dilated CBD along with MIA, and also, the rest of the intestine was not normal in terms of motility. In literature, there are few case reports showed an association of dilated CBD and even choledochal cyst with MIA in the case of CPA.[8],[9] Prognosis of these neonates is usually fatal.

  Conclusion Top

CPA is a rare anomaly and carries a poor prognosis when associated with MIAs. The combination of gastric outlet obstruction and CBD dilatation on investigations, point toward the associated MIA and consideration of this complex pattern gives more insight information in the case of CPA.

Declaration of patient consent

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

Financial support and sponsorship


Conflicts of interest

There are no conflicts of interest.

  References Top

Ksia A, Zitouni H, Zrig A, Laamiri R, Chioukh F, Ayari E, et al. Pyloric atresia: A report of ten patients. Afr J Paediatr Surg 2013;10:192-4.  Back to cited text no. 1
  [Full text]  
Al-Salem AH. Congenital pyloric atresia and associated anomalies. Pediatr Surg Int 2007;23:559-63.  Back to cited text no. 2
Cole C, Freitas A, Clifton MS, Durham MM. Hereditary multiple intestinal atresias: 2 new cases and review of the literature. J Pediatr Surg 2010;45:E21-4.  Back to cited text no. 3
Bawazir OA, Al-Salem AH. Congenital pyloric atresia: Clinical features, diagnosis, associated anomalies, management and outcome. Ann Pediatr Surg 2017;13:188-93.  Back to cited text no. 4
Al-Salem AH, Abdulla MR, Kothari MR, Naga MI. Congenital pyloric atresia, presentation, management, and outcome: A report of 20 cases. J Pediatr Surg 2014;49:1078-82.  Back to cited text no. 5
Parelkar SV, Kapadnis SP, Sanghvi BV, Joshi PB, Mundada D, Shetty S, et al. Pyloric atresia – Three cases and review of literature. Afr J Paediatr Surg 2014;11:362-5.  Back to cited text no. 6
[PUBMED]  [Full text]  
Martin CE, Leonidas JC, Amoury RA. Multiple gastrointestinal atresias, with intraluminal calcifications and cystic dilatation of bile ducts: A newly recognized entity resemblin “a string of pearls”. Pediatrics 1976;57:268-71.  Back to cited text no. 7
Snyder CL, Mancini ML, Kennedy AP, Amoury RA. Multiple gastrointestinal atresias with cystic dilatation of the biliary duct. Pediatr Surg Int 2000;16:211-3.  Back to cited text no. 8
Raj P, Sinha SK, Ramji S, Sarin YK. Hereditary multiple gastrointestinal atresia associated with choledochal cyst: A rare entity with management dilemma. J Neonatal Surg 2014;3:39.  Back to cited text no. 9


  [Figure 1]


Similar in PUBMED
   Search Pubmed for
   Search in Google Scholar for
 Related articles
Access Statistics
Email Alert *
Add to My List *
* Registration required (free)

  In this article
Case Report
Article Figures

 Article Access Statistics
    PDF Downloaded151    
    Comments [Add]    

Recommend this journal