Intestinal obstruction due to dual gastrointestinal atresia in infants: diagnosis and management of 3 cases
© Chen et al.; licensee BioMed Central Ltd. 2014
Received: 9 January 2014
Accepted: 5 June 2014
Published: 13 June 2014
Several types of congenital lesions can cause complete or incomplete obstruction of the intestine. Our purpose is to present 3 neonates with dual intestinal type I atresia, i.e., simultaneous obstructive lesions at 2 locations in which the atresia manifested as diaphragm-like tissue.
All 3 cases were female infants ranging in age from 2 to 14 months. The common symptom in all cases was intermittent persistent vomiting. In some cases the vomitus was bilious, and other symptoms included abdominal distention and delayed meconium passage. Prior surgeries at another hospital were unsuccessful at relieving the symptoms in one case. One case had dual lesions in the colon, one dual lesions in the duodenum, and one atresia at both the distal portion of the ileum and the descending colon. Surgical exploration and removal of the lesions at our hospital was successful in all cases, and the infants were discharged in good condition.
Type I atresia can manifest as a diaphragm-like tissue obstructing the continuity of gastrointestinal tract, and in rare cases multiple areas may be present. Base on the intermittent nature of the associated symptoms, diagnosis can be difficult and is often delayed. Physicians should be aware of this condition during the work-up of an infant with persistent intermittent vomiting.
KeywordsType I Atresia Gastrointestinal diaphragm Congenital Intestinal obstruction Infant
Several types of congenital lesions can cause complete or incomplete obstruction of the intestines and atresia, failure of the lumen to canalize, accounts for approximately 95% of obstructions . The spectrum of abnormalities causing obstruction includes imperforate and perforate diaphragms of variable thickness within the intestines. Imperforate diaphragms cause complete obstruction, whereas those with central perforations cause incomplete obstruction.
Intestinal atresia can occur anywhere along the GI tract, and the anatomic location of the obstruction and the degree of blockage determine the clinical manifestations . Characteristic clinical features of duodenal atresia are repetitive bilious vomiting, with or without subtle upper abdominal distension. The recognition of partial obstructions may be delayed if the obstruction is of a relatively minor degree. The pertinent signs of jejunoileal atresia include bilious emesis, abdominal distension, jaundice, and failure to pass meconium on the first day of life.
Small intestine atresia accounts for more than 90% of all cases of infants with congenital occlusions, while colon occlusion due to variable septa is rare, with only 6 reports since 1966. Though intestinal atresia usually occurs in a single location (>90% of cases), 6-20% of cases have multiple areas of atresia , and more often involve the proximal jejunum. Nevertheless, concurrent type I atresia in both the small intestine and colon have never been reported. We herein present 3 cases of dual intestinal type I atresia located in both the small intestine and colon.
All three patients had signed informed consent, and the project was approved by the Medical Ethical Committee, The First Affiliated Hospital, Sun Yat-sen University. The details of each GI atresia conditions were described below.
Her postoperative course was uneventful, and the symptoms did not recur. At 1 year postoperatively, her growth and development were within the normal range.
Discussion and conclusions
This report presented 3 cases of dual intestinal obstruction in neonates as a result of type I atresia with central perforation of the obstructing diaphragm. As a congenital disease, type I atresia is not a rare abnormality; however, in all of the 3 cases the infants had concurrent atresia along the GI track, which is uncommon among type I atresia patients and complicated arriving at a correct diagnosis.
The differential diagnosis of neonatal upper GI obstruction includes esophageal atresia, malrotation with midgut volvulus, pyloric stenosis, intestinal atresia and stenosis, annular pancreas, preduodenal portal vein, duodenal duplication, foreign body obstruction, Hirschsprung disease, and gastroesophageal reflux . Atresia in the intestines of newborns is a congenital abnormality, which occurs most frequently in the duodenum [5, 6] and less frequently in the jejunoileal region . The etiologies of duodenal obstruction and jejunum obstruction are different . On the other hand, congenital colonic atresia is rare [8, 9], and should be differentiated from Hirschsprung disease, small left colon syndrome, obturation obstruction, and meconium ileus and meconium plug . Most newborns with intestinal obstruction present with bilious emesis, and in the neonate this should be considered secondary to a mechanical obstruction until proven otherwise and emergent evaluation is warranted . Intestinal atresia is not commonly observed in children, especially those occured at multiple sites .
Intestinal atresia may take the form of a transverse diaphragm of tissue with or without a perforation obstructing the continuity of GI tract (type I atresia). The perforation within the diaphragm is usually singular and centrally located within the lumen of the intestine. An intestinal diaphragm may be accompanied with other malformations of the digestive tract such as an annular pancreas . A windsock abnormality is a thin diaphragm that has ballooned distally as a result of peristalsis . Multiple areas of diaphragmatic atresia are only noted in 6-20% of cases , and diaphragms located in both the small intestine and colon have never been reported before.
The characteristic clinical feature of almost all intestinal diaphragms is repetitive vomiting. In cases of a perforate diaphragm, the diameter of the opening directly determines the degree of obstruction, and therefore is inversely related to the level of symptoms. Nevertheless, in cases of colonic diaphragmatic atresia, vomiting typically occurs after a full stomach and stools tend to be unformed or thin strip-shaped . In case 2, the patient did not manifest symptoms until 3 months after birth. In that case, successful extrusion of air through the occluded intestine suggests a larger perforation in the diaphragm, which explains the delayed symptoms. These findings may also have contributed to the misdiagnosis prior to admission to our institution. In general, manifestations of colonic type I atresia as seen in cases 2 and 3 are similar to those of congenital megacolon [11, 12]. However, when given the routine treatment for congenital megacolon (return-flow enema) the symptoms were not alleviated. Additionally, a large amount of gas had also accumulated in the small intestine, which is not seen in cases of congenital megacolon. A review of 118 cases colonic atresia found the lesion occurred in the ascending colon in 28% of cases, the hepatic flexure in 3%, the transverse colon in 23%, the splenic flexure in 25%, and in the descending and sigmoid in 20% .
It is difficult to distinguish type I atresia from other causes of vomiting by clinical manifestations alone. GI radiography and endoscopy are the best methods to diagnose the obstruction before surgery . However, misdiagnosis may still occur. Even intraoperatively, it is challenging to determine the existence of an obstructing diaphragm of tissue by visual observation or palpation . In case 2, a duodenal septum was confirmed intraoperatively and a jejuno-duodenostomy was performed before the patient was admitted to our institution. Unfortunately, obstructive symptoms remained which suggested the presence of a persistent obstruction proximal to the anastomosis. This obstruction was determined to be an intestinal transverse diaphragm during surgery at our hospital. On the other hand, cases 1 and 3 both received barium enema examinations which identified obstructions in the colon. Thus, consideration was given to this specific observation before surgery.
To avoid misdiagnosis, a few key points should be noted before surgery. Surgeons should observe the barium enema being performed which will help them to distinguish abnormalities from normal intestinal movement. In addition, surgeons should fully understand the patient’s prior surgeries and pathogenesis to avoid misdiagnosis of an intestinal diaphragmatic atresia.
Written informed consents were obtained from the patients’ parents or guardians for publication of the case report and any accompanying images. Copies of the written consents are available for review by the journal editor.
- Rescorla FL, Grosfeld JL: Intestinal atresia and stenosis: analysis of survival in 120 cases. Surgery. 1985, 98: 668-676.PubMedGoogle Scholar
- Kimura K, Loening-Baucke V: Bilious vomiting in the newborn: rapid diagnosis of intestinal obstruction. Am Fam Physician. 2000, 61: 2791-2798.PubMedGoogle Scholar
- Grosfeld JL, O’Neill JA, Fonkalsrud EW, Coran AG: Pediatric Surgery. 2006, Philadelphia: Mosby-Elsevier, 1275, 6Google Scholar
- Reyes HM, Meller JL, Loeff D: Neonatal intestinal obstruction. Clin Perinatol. 1989, 16: 85-96.PubMedGoogle Scholar
- Nixon HH, Tawes R: Etiology and treatment of small intestinal atresia: analysis of a series of 127 jejunoileal atresias and comparison with 62 duodenal atresias. Surgery. 1971, 69: 41-51.PubMedGoogle Scholar
- Escobar MA, Ladd AP, Grosfeld JL, West KW, Rescorla FJ, Scherer LR, Engum SA, Rouse TM, Billmire DF: Duodenal atresia and stenosis: long-term follow-up over 30 years. J Pediatr Surg. 2004, 39: 867-871. 10.1016/j.jpedsurg.2004.02.025.View ArticlePubMedGoogle Scholar
- Dalla Vecchia LK, Grosfeld JL, West KW, Rescorla FJ, Scherer LR, Engum SA: Intestinal atresia and stenosis: a 25-year experience with 277 cases. Arch Surg. 1998, 133: 490-496.View ArticlePubMedGoogle Scholar
- Davenport M, Bianchi A, Doig CM, Gough DC: Colonic atresia: current results of treatment. J R Coll Surg Edinb. 1990, 35: 25-28.PubMedGoogle Scholar
- Juang D, Snyder CL: Neonatal bowel obstruction. Surg Clin North Am. 2012, 92: 685-711. 10.1016/j.suc.2012.03.008. ix-xView ArticlePubMedGoogle Scholar
- Chen JY: The value of digital gastrointestinal diagnostics on children digestive tract diaphragm stenosis. Chin J Composite Clin Med. 2002, 3: 303-304.Google Scholar
- Applebaum H, Lee SL, Puapong DP: Duodenal Atresia and Stenosis. Grosfeld Pediatric Surgery. Edited by: Grosfeld JL, O'Neill JAJr, Fonkalsrud EW, Coran AG. 2006, Philadelphia, PA: Mosby Elsevier, 1260-1268.View ArticleGoogle Scholar
- Guo ZP, Wang JF, Lee ZK: Diagnosis and treatment of colon diaphragm stenosis. Tianjin Med J. 2002, 30: 758-759.Google Scholar
- Gupta AK, Guglani B: Imaging of congenital anomalies of the gastrointestinal tract. Indian J Pediatr. 2005, 72: 403-414. 10.1007/BF02731737.View ArticlePubMedGoogle Scholar
- The pre-publication history for this paper can be accessed here:http://www.biomedcentral.com/1471-230X/14/108/prepub
This article is published under license to BioMed Central Ltd. This is an Open Access article distributed under the terms of the Creative Commons Attribution License (http://creativecommons.org/licenses/by/2.0), which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly credited. The Creative Commons Public Domain Dedication waiver (http://creativecommons.org/publicdomain/zero/1.0/) applies to the data made available in this article, unless otherwise stated.