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Year : 2020  |  Volume : 9  |  Issue : 1  |  Page : 63-68

Clinical pattern of colonic atresia, management, and outcome in an indian tertiary Care Center

Department of Pediatric Surgery, SVP Postgraduate Institute of Pediatrics, SCB Medical College, Cuttack, Odisha, India

Date of Submission23-Aug-2019
Date of Acceptance01-Oct-2019
Date of Web Publication29-Jan-2020

Correspondence Address:
Dr. Prasanta Kumar Tripathy
Arunodaya Nagar, Link Road, Cuttack - 753 012, Odisha
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/jcn.JCN_92_19

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Introduction: Colonic atresia (CA) is an important cause of congenital intestinal obstruction and frequently associated with other comorbidities. Colon is the least common site of gastrointestinal atresias, and no large series can be documented because of the rarity of the anomaly. Historically, majority of the patients were being managed by staged approach; initial ostomy formation followed by delayed repair. Objective: The objective of this study was to analyze the demographic pattern, management approach in our set up and to compare the outcome with similar available studies. Materials and Methods: This is a retrospective study on infants with CAs managed between October 2016 and March 2019. Results: CAs constituted 10% of all gastrointestinal atresias managed in our study and females were more commonly affected. Atresias were located most commonly in sigmoid colon (7 cases), and Type III was the most common anomaly. Five patients had associated anomalies such as gastroschisis, malrotation, and anorectal malformation (ARM). The initial diversion was done in four cases, and primary repair was done in eight cases. Two neonates died postoperatively due to septicemia. Conclusion: CAs are rare anomalies and often associated with other congenital malformations. Its association should be kept in mind during colostomy formation for high ARM. CAs can be managed successfully by primary anastomosis regardless of the location of atresia. Early diagnosis and intervention are essential for the survival of these neonates.

Keywords: Anorectal malformation, gastroschisis, intestinal obstruction, malrotation

How to cite this article:
Tripathy PK, Jena PK, Mohanty HK. Clinical pattern of colonic atresia, management, and outcome in an indian tertiary Care Center. J Clin Neonatol 2020;9:63-8

How to cite this URL:
Tripathy PK, Jena PK, Mohanty HK. Clinical pattern of colonic atresia, management, and outcome in an indian tertiary Care Center. J Clin Neonatol [serial online] 2020 [cited 2020 Jul 14];9:63-8. Available from: http://www.jcnonweb.com/text.asp?2020/9/1/63/277234

  Introduction Top

Colonic atresias (CAs) are rare congenital anomalies causing intestinal obstruction in neonates and infants.[1],[2] The incidence of CA is reported as 1:66,000 live births.[3] They account for 1.8%–15% of all gastrointestinal atresias with an increased incidence among males.[4],[5] The etiology of CA is most commonly attributed to intrauterine vascular insult.[5],[6] Although CA may be an isolated disorder, other congenital anomalies are associated in 47% of cases.[7] Controversy still exists regarding initial operative management of CA: primary repair versus initial diversion.[8] It is a challenge for pediatric surgeons because of its rarity, unusual presentation, and association with other comorbidities.

The aim of this study was to find the demographic pattern of CAs, associated anomalies and to highlight the rarity of this disorder. The management approach in our set up was reviewed and the outcome compared with other available studies.

  Materials and Methods Top

This retrospective study was conducted in the Department of Pediatric Surgery, where the records of the patients with CA managed between October 2016 and March 2019 were reviewed. The study was conducted in accordance with the ethical standards and with the revised Helsinki Declaration 2000. The demographic data such as age, sex, weight at presentation, symptoms, type of presentation, and associated anomalies were noted. Infants were resuscitated immediately after admission with nasogastric tube, intravenous fluids, antibiotics, and oxygen as per requirement. They were investigated with routine hemogram, serum electrolytes, urea, creatinine, and X-ray abdomen in the erect posture. Patients were operated after correction of fluid and electrolyte deficits. Intraoperative findings such as site of atresia, type of atresia, and associated anomalies were recorded. The classification system described by Grosfeld et al. was followed in our institution; Type I: Mucosal atresia with intact bowel wall, Type II: Fibrous cord separating two blind-ending bowel ends, Type IIIa: Bowel ends separated by V-shaped mesenteric defect, Type IIIb: Apple-peel atresia, and Type IV: Multiple atresias.[9] The operative procedure, postoperative complications, duration of hospitalization, and outcome were noted. Statistical analysis of the available data was performed. Continuous data were represented as mean ± standard deviation, whereas categorical data were represented as percentage. Comparison of categorical data was made using Chi-square analysis. P < 0.05 was considered statistically significant.

  Results Top

Twelve cases of CAs were operated at our institution over a period of 2½ years. During this period, a total of 120 cases of gastrointestinal atresias were operated. Hence, CA constituted 10% of all gastrointestinal atresias in our study. Abdominal distension, bilious vomiting, and nonpassage of meconium were common presenting symptoms. The age range of neonates varied from 1 day to 5 days with a mean age of 3.2 ± 1.1 (confidence interval = 0.76–1.93). One infant presented at the age of 2 months, but intermittent vomiting and constipation was present before 1 month of age. Females (7 cases) outnumbered male infants (5 cases). The mean body weight was 2.6 ± 0.61 kg at presentation with a range from 2.1 kg to 4 kg (confidence interval = 0.43–1.03). Neonatal intestinal obstruction was the most common type of presentation followed by abdominal wall defect [Table 1]. Erect abdominal X-ray showed dilated bowel loops and multiple air-fluid levels. During surgery, the sigmoid colon was the most common site of atresia (58% cases). Transverse colon and ascending colon were affected in 25% and 17% cases, respectively. Type IIIa was the most common anomaly detected in seven cases (58%) followed by Type I and II [Figure 1]. Associated anomalies were detected in five cases (42%). Gastroschisis (2 cases), malrotation (2 cases), and high anorectal malformation (ARM) were associated with CA in our study.
Table 1: Demographic characteristics of infants with colonic atresia

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Figure 1: (a) Intraoperative photograph of a newborn with sigmoid colon atresia. (b) Colonic atresia affecting distal sigmoid colon along with anorectal malformatioin as associated anomaly. Primary anastomosis of colonic atresia and distal descending colostomy was done. Perineum showing a prominent anal dimple, but anal opening was not present. (c) Follow-up at 6 weeks with colostomy in situ. (d) Distal cologram 10 weeks postoperatively, showing good caliber of rectum and sigmoid colon. Patient was planned for posterior sagittal anorectoplasty

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Diverting colostomy was done in four cases (33%) during the initial hospitalization [Table 2]. Primary anastomosis was done in eight cases (67%) and it was added with proximal covering stoma in four cases (33%). Ladd's procedure was added in two patients with malrotation. The patient with CA and high ARM was operated in three stages; initial colostomy followed by posterior sagittal anorectoplasty and colostomy closure [Figure 2]. Postoperatively, complications such as colostomy stenosis wound dehiscence and wound infection were adequately managed. The average duration of hospital stay was 7.7 days. Two patients (16.7%) died postoperatively during initial hospitalization due to septicemia. Diverting colostomies during initial hospitalization and covering colostomies done along with primary anastomosis were reversed in all surviving patients. None of the patients died during hospitalization for final surgery or follow-up. The overall survival rate was 83.3% in this study. All the surviving patients were followed from 6 months to 2 years. They are asymptomatic, gaining weight, and parents are satisfied regarding the quality of life of their children.
Table 2: Surgical management and outcome of colonic atresia

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Figure 2: (a) Explorative laparotomy in a female neonate with colonic atresia. (b) Double-barrel colostomy was performed. (c) Intraoperative photograph of a male infant with Type I colonic atresia and malrotation. Caecum was mobile and found in upper abdomen. (d) Intraoperative picture of enterotomy and excision of web

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  Discussion Top

The colon is the least common site of atresia accounting for <10% of gastrointestinal atresias.[8],[10] Although Benninger described a case in 1673, the first infant with CA survived after about two and half centuries as reported by Gaub in 1922.[11] It was a case of sigmoid colon atresia and managed by colostomy. The first primary repair was done by Potts in 1947 in a patient of transverse colon atresia.[7] However, very few case series are published since then with the variable outcome and about half of these cases are associated with other congenital malformations.[8] The classical etiology responsible for most of the cases of CAs are mesenteric vascular accident inutero, which may be extrinsic or intrinsic. The extrinsic causes are internal hernias, intussusception, volvulus, and strangulation in tight gastroschisis. The intrinsic factor includes thromboembolic event originating from placenta followed by fetal circulation and causing intraluminal mesenteric vascular occlusion.[5],[7],[8] Other causes such as the failure of recanalization during gut development, maternal use of drugs, and genetic/familial factors are proposed.

An average of 1–2 cases of CA are reported annually in pediatric surgical centers worldwide.[2],[4] However, we have managed five cases of CA per year during the study. An increased male predominance is reported by most of the case series on CA,[4],[7],[8],[12] but females were more commonly (58% cases) affected in our study. Type III anomaly is reported as the most common variety in different studies followed by Type I and Type II in order.[4],[7],[12],[13] This finding was also revealed true in our cohort as well. Type III variety was most frequently detected (58%) in our series. Type I and II anomalies were detected in 25% and 17% cases, respectively. In one of the largest literature reviews, majority of CA were found to be located in the right colon (122 cases) than in the left colon (48 cases).[7] However, left colon was more commonly affected in our study in 58% of cases. It was also reported by most of the authors that CA proximal to splenic flexure are more commonly type III variety and atresias distal to this point are more frequently Type I or Type II.[7],[12],[13] In our study, 71% of Type III atresias were located proximal to splenic flexure and all the cases of Type I and II atresias were distal to splenic flexure. Hence, our findings regarding site of atresias corroborated with previously reported literatures.

One important aspect of CA is its association with other congenital anomalies. Associated anomalies are reported in 47.3% of cases in one of the largest literature surveys, of which the majority were gastrointestinal anomalies.[7] Gastrointestinal tract malformations were also major associated anomalies detected in our series. Hirschsprung's disease is associated with about 2% of cases of CA.[14] CA was found in 2.5% cases in a review of 199 newborns with gastroschisis.[15] Malrotation and gastroschisis are also described among associated comorbidities in CA. Both the diseases were detected individually in 17% cases in our series. These findings are similar to the study published by Etensel et al. and Dassinger et al.[7],[13] A remarkable feature in our study is that one of the male newborns presented as high ARM and during laparotomy for colostomy, a Type II sigmoid colon atresia was detected. Later on, distal cologram did not reveal any fistula to urinary tract, which is usually associated with 95% of males with high ARM. To the best of our knowledge, the coexistence of CA with ARM is rare and reported only in eight cases.[6],[14],[16] Hence, the association of a case of Type II CA and high ARM without fistula in our series, is an extremely rare finding. The diagnosis of CA is usually delayed in comparison to proximal small intestinal atresias. By this time, neonate has received several feedings in a setting of failure to pass meconium until gross abdominal distension and feculent vomiting predominates. The clinical picture becomes more complicated due to dehydration, electrolyte imbalance, and sepsis. The closed-loop obstruction caused by these large bowel atresias in the presence of an intact ileocecal valve subjects the infant to an increased risk of colonic perforation.[7]

Operative management of CA has been précised over the decades. Conventionally, primary anastomosis for CA proximal to the splenic flexure and initial colostomy followed by delayed repair for atresias distal to this point was suggested. In the largest literature survey, staged surgery was performed (n: 132) more commonly than primary repair (n: 45).[7] Hence, staged surgical approach was preferred in the past decades to avoid risks of primary bowel anastomosis.[5],[12] Primary repair regardless of the location has been suggested by some surgeons with variable outcome.[3],[13] Primary anastomosis becomes technically difficult due to gross size difference between proximal and distal bowel ends and loss of length due to the excision of the dilated proximal colon.[17] Although the debate is still continuing, the latter approach was preferred in our center. Primary repair was performed in 67% of our cases and diverting colostomy in 33% cases. The bulbous proximal end was excised in type III atresias before anastomosis. Another remarkable feature is that primary anastomosis being protected with a proximal diversion was rarely preferred in the past; three such cases in a review of 177 cases of CA.[7] However, we have performed proximal diversion in 50% of our cases with primary anastomosis.

Postoperative complications such as wound dehiscence, stoma stenosis, and septicemia found in our series were similar to other reports.[4],[8],[12] Saha et al. reported an average duration of hospitalization of 3.5 days in patients who underwent initial diversion and 21 days for patients with primary anastomosis.[4] Although the mean duration of hospital stay was not reported in many series, it was found to be 7 days in patients with initial diversion and 8 days in patients with primary repair in our study. Two patients with ascending colon atresias, who underwent primary repair died postoperatively due to septicemia, of which one patient had gastroschisis as an associated anomaly. However, there is no statistically significant difference in mortality rate between patients with initial diversion and those with primary anastomosis (P = 0.51) [Figure 3]. Although, both the deaths occurred in patients with right CAs, there is no significant difference in death rate between the right colon and left CAs (P = 0.15). Again, both of our deaths occurred in type III atresias and no mortality in Type I and II atresias [Table 3]. The association with other anomalies did not result in a significant difference in mortality rate (P = 1.00). The overall mortality in our study was 16.7%. Mirza et al. and El-Asmar et al. reported 27% and 25% mortalities in their respective series and sepsis was the cause of death.[2],[8] Gobran et al. reported 17.2% mortality caused by sepsis and cardiac anomaly in two of their cases.[12] However, Etensel et al. detected 25.7% mortality in a literature survey of 214 cases of CA.[7] Saha et al. also reported 22.2% mortalities in their series due to anastomotic leak and sepsis.[4] The improved survival noted in our series is attributed to early referral from periphery hospitals in recent years and urgent surgical intervention.
Figure 3: Comparison of survival between two modalities of management in colonic atresia

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Table 3: Comparison between morphological patterns of colonic atresia and outcome

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Limitations of the study

A study having large number of the patient population was not possible due to rarity of the disease.

However, during this short-term study, an increased annual incidence and female preponderance were found in our series as compared to similar studies. A trend toward more frequent involvement of the left colon was also marked. The occurrence of high ARM along with CA is an extremely unusual association. Primary repair was preferred modality of management in our set up.

  Conclusion Top

CAs are rare anomalies and frequently associated with other congenital malformations. Although association with ARM is extremely rare, it should be kept in mind while performing colostomy in high ARM, and the distal bowel should be inspected for any pathology. CA can be managed successfully by primary colo-colonic anastomosis regardless of the location of atresia. Protective colostomy should be considered depending on the intraoperative pathology and condition of the newborn. Early recognition and surgical intervention are required for better survival of these tiny neonates.

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Conflicts of interest

There are no conflicts of interest.

  References Top

Hsu CT, Wang SS, Houng JF, Chiang PJ, Huang CB. Congenital colonic atresia: Report of one case. Pediatr Neonatol 2010;51:186-9.  Back to cited text no. 1
Mirza B, Iqbal S, Ijaz L. Colonic atresia and stenosis: Our experience. J Neonatal Surg 2012;1:4.  Back to cited text no. 2
Davenport M, Bianchi A, Doig CM, Gough DC. Colonic atresia: Current results of treatment. J R Coll Surg Edinb 1990;35:25-8.  Back to cited text no. 3
Saha H, Ghosh D, Ghosh T, Burman S, Saha K. Demographic study and management of colonic atresia: Single-center experience with review of literature. J Indian Assoc Pediatr Surg 2018;23:206-11.  Back to cited text no. 4
[PUBMED]  [Full text]  
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;133:490-6.  Back to cited text no. 5
Derenoncourt MH, Baltazar G, Lubell T, Ruscica A, Sahyoun C, Velcek F, et al. Colonic atresia and anorectal malformation in a Haitian patient: A case study of rare diseases. Springerplus 2014;3:203.  Back to cited text no. 6
Etensel B, Temir G, Karkiner A, Melek M, Edirne Y, Karaca I, et al. Atresia of the colon. J Pediatr Surg 2005;40:1258-68.  Back to cited text no. 7
El-Asmar KM, Abdel-Latif M, El-Kassaby AA, Soliman MH, El-Behery MM. Colonic atresia: Association with other anomalies. J Neonatal Surg 2016;5:47.  Back to cited text no. 8
Grosfeld JL, Ballantine TV, Shoemaker R. Operative mangement of intestinal atresia and stenosis based on pathologic findings. J Pediatr Surg 1979;14:368-75.  Back to cited text no. 9
Karnak I, Ciftci AO, Senocak ME, Tanyel FC, Büyükpamukçu N. Colonic atresia: Surgical management and outcome. Pediatr Surg Int 2001;17:631-5.  Back to cited text no. 10
Gaub OC. Congenital stenosis and atresia of intestinal tract above the rectum: with a report of an operated case of atresia of the sigmoid colon in an infant. Trans Am S A 1922;40:582-638.  Back to cited text no. 11
Gobran TA, Khalifa M, Kamal MK. Different varieties of colonic atresia in a series of 13 patients: A single-centre experience. Ann Pediatr Surg 2013;9:20-4.  Back to cited text no. 12
Dassinger M, Jackson R, Smith S. Management of colonic atresia with primary resection and anastomosis. Pediatr Surg Int 2009;25:579-82.  Back to cited text no. 13
Arca MJ, Oldham KT. Atresia, stenosis and other obstructions of the colon. In: Coran AG, Adzick NS, Krummel TM, Laberge J, Shaberger RC, Caldamone AA, editors. Pediatric Surgery. 7th ed. Philadelphia: Elsevier Saunders; 2012. p. 1247-53.  Back to cited text no. 14
Snyder CL, Miller KA, Sharp RJ, Murphy JP, Andrews WA, Holcomb GW 3rd, et al. Management of intestinal atresia in patients with gastroschisis. J Pediatr Surg 2001;36:1542-5.  Back to cited text no. 15
Tripathy PK, Jena PK. Type II colonic atresia and high anorectal malformation: a rare coexistence and literature review. Indian J Neonat Med Res 2017;5:PC04-6.  Back to cited text no. 16
Chouikh T, Charieg A, Mrad C, Ghorbel S, Saada S, Benkhalifa S, et al. Congenital colonic atresia: 4 case reports. J Pediatr Neonatal Care 2014;1:00018. DOI:10.15406/jpnc.2014.01.00018  Back to cited text no. 17


  [Figure 1], [Figure 2], [Figure 3]

  [Table 1], [Table 2], [Table 3]


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