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Year : 2018  |  Volume : 23  |  Issue : 4  |  Page : 206-211

Demographic study and management of colonic atresia: Single-center experience with review of literature

Department of Paediatric Surgery, Nil Ratan Sircar Medical College and Hospital, Kolkata, West Bengal, India

Date of Web Publication4-Oct-2018

Correspondence Address:
Prof. Dipak Ghosh
Orbit Sky View, 1/1A, Umakanta Sen Lane, Block 4, Flat-4b, Kolkata - 700 030, West Bengal
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/jiaps.JIAPS_219_17

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Context and Aim: The aim of this study is to highlight the rarity of this disorder and its associated anomalies and our objective was to review our experience in the management of colonic atresia (CA) with respect to staged surgery versus one-step procedure for a better outcome of the disease.
Settings and Design: A randomized, controlled, single-center study conducted over a period of 4 years from October 2013 to 2017.
Subjects and Methods: Nine cases were operated for CA of which three underwent primary anastomosis and six underwent initial colostomy followed by definitive surgery. Age, sex, body weight, clinical presentation, type of atresia, site, time interval of operation, associated anomalies, initial procedure, postoperative complications, final procedure, biopsy, hospital stay, and outcome were noted.
Statistical Analysis Used: Microsoft Excel was used for statistical analysis.
Results: Out of 9 patients, 6 were males and 3 were females, 7 patients survived and 2 patients died (mortality 22.2%), of which one patient with primary anastomosis had leak and sepsis and one patient with primary anastomosis had associated Hirschsprung's disease (HD). Mean time gap for operation was 12.8 h and mean hospital stay was 3.5 days for initial colostomy and 21 days for primary anastomosis. Wound dehiscence occurred in 4 patients. Malrotation was found in 3 patients, HD in 2 patients, cardiac anomaly in 2, Meckel's diverticulum in 1, and cleft lip in 1 patient. Type IIIa atresia was found in in 6 patients, Type I in 2, and Type II in 1 patient. The most common site was transverse colon (n = 5).
Conclusion: Diagnosis and management of CA is a challenge. Early presentation and diagnosis should be prompt. Staged procedure with initial colostomy followed by definitive procedure is the preferred choice. Associated anomaly HD must be ruled out.

Keywords: Colonic atresia, colostomy, Hirschsprung's disease, rare

How to cite this article:
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

How to cite this URL:
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 [serial online] 2018 [cited 2021 May 13];23:206-11. Available from: https://www.jiaps.com/text.asp?2018/23/4/206/242712

   Introduction Top

Colonic atresia (CA) is a rare cause of neonatal intestinal obstruction with an average of 1–2 reported cases per year in a tertiary care center worldwide. The incidence is approximately 1.8%–5% of intestinal atresia with an increased preponderance in males.[1],[2],[3],[4],[5] Mortality and morbidity depends on early diagnosis and urgent management. It may be isolated or associated with other congenital anomalies such as malrotation, atresia of small bowel, cleft lip, Meckel's diverticulum, and Hirschsprung's disease (HD).[1],[2],[3],[4],[5] Till date, there have been many studies but management of CA is challenging due to its rare and unusual presentation.

The purpose of this study was to highlight the rarity of this disorder and its associated anomalies and our objective was to review our experience in the management of CA with respect to staged surgery versus one step procedure for a better outcome of the disease.

   Subjects and Methods Top

The study was performed in the Department of Paediatric Surgery, Nil Ratan Sircar Medical College, Kolkata, India, from October 2013 to 2017. A total of 11 cases of CA were referred to this center, of which 9 cases were operated. Complete demographic data including age on presentation, sex, body weight, associated anomalies, and intraoperative findings including type of atresia, site of atresia, procedure performed colostomy or resection anastomosis, postoperative complications, hospital stay, and outcome were noted. Clinical examination was complete and routine blood investigations including complete hemogram, renal function test, liver function test, serology, straight X-ray abdomen, echocardiography, and contrast enema was done. Patients with severe sepsis or unfit for anesthesia were excluded from the study.

Age at presentation was considered at the time when the baby reached our referral center. Associated anomalies were noted by clinical examination, investigations, and intraoperative findings. Intraoperative finding of CA was classified according to the Grosfeld Classification: Type I atresia with a mucosal defect, Type II fibrous cord connecting the atretic ends, Type IIIa atresia with a V-shaped mesenteric gap defect, Type IIIb apple-peel deformity, and Type IV multiple atresia.[6],[7]

All the neonates that reached our center were immediately resuscitated with intravenous fluid, antibiotics, and nasogastric suction. Electrolyte imbalance, metabolic status, and dehydration were corrected before operation. Out of 9 patients, 2 patients presented on the 4th day with features of systemic inflammatory response syndrome and hence were excluded from contrast enema. While remaining 7 patients contrast enema was performed. Contrast enema revealed distal microcolon and the site of atresia as cutoff sign [Figure 1]c and [Figure 1]d. Plain abdominal X-ray revealed multiple air-fluid level and dilated bowel loops indicating a distal intestinal obstruction [Figure 1]a and [Figure 1]b. Echocardiography revealed associated cardiac anomalies.
Figure 1: Plain abdominal X-ray (a) multiple air-fluid levels, (b) dilated bowel loops, (c and d) contrast enema-cutoff sign of distal colon at atretic site

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After complete resuscitation, the patient was shifted to operation theater usually within 24 h of admission. Laparotomy was performed through right upper transverse incision. Out of 9 CA, six patients underwent initial double-barrel colostomy or ileostomy and biopsy from the distal colon and rectum was done and planned for definitive surgery after 4 weeks based on the biopsy report. Three cases underwent primary resection anastomosis and biopsy of the distal colon and rectum was done.[8],[9],[10],[11] Biopsy was taken as full thickness up to 1 cm of the distal colon and resection up to 10 cm proximal dilated colon was done. Patency of the distal bowel was checked with normal saline. The time interval from reporting to our hospital to time of surgical intervention defined as time gap in hours was also noted [Table 1].
Table 1: Management and outcome of colonic atresia

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Postoperative patient was maintained on nasogastric suction, intravenous fluid, nutrition, and antibiotics. For those patients with a diverting stoma, feeding was started within 24 h. Patients with primary anastomosis feeding were delayed until there was passage of stool. The length of hospital was 12–27 days for those with primary anastomosis. Parenteral nutrition was added from day 2 for patient with primary anastomosis.

   Results Top

A total of 11 cases were referred to our center over 4 years which were included in the study. Two cases of suspected CA based on radiological finding and clinical examination were excluded due to severe sepsis or unfit for anesthesia. Out of 9 cases, 6 were male and 3 were female (ratio 2:1). Clinical presentation was similar in all cases with characteristics radiological findings of distal microcolon and site of atresia in contrast enema and multiple air-fluid levels in plain abdominal X-ray. Mean age at presentation was 2 days and mean body weight was 2.1 kg [Table 2].
Table 2: Demographic data of different types of colonic atresia

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Associated anomalies are listed in [Table 2]. Most common findings were malrotation cardiac anomalies and HD. There were 3 cases of malrotation and 2 cases of HD and patient with atrial septal defect. Meckel's diverticulum was seen in 1 case [Figure 1]d and unilateral cleft lip in 1 case. Operative findings and classification of atresia are shown in [Table 2]. Most common site of atresia in our series was in the transverse colon followed by ascending colon and sigmoid colon. Type IIIa was seen as the most common type of presentation. Type I atresia were seen in 2 cases and Type II atresia in 1 case [Table 2].

The decision of single-step procedure or initial stoma was made during the operation based on the patient condition and intraoperative findings. Three patients underwent primary resection and anastomosis after confirming the distal patency without any diverting stoma. Out of the remaining 6 patients, 5 underwent double-barrel colostomy and 1 underwent ileocolostomy irrespective of distal patency. Patient in which ileocolostomy was done had an ileal perforation which was detected intraoperative [Figure 2]d. The mean time interval from reporting to the time of operation was 12.8 h.
Figure 2: (a) Sigmoid colon atresia Type 1, (b) sigmoid colon atretic portion exposed revealed web, (c) transverse colon atresia (hepatic flexure) Type IIIa, (d) ascending colon atresia Type 1 with ileal perforation and Meckel's diverticulum

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Out of 9 cases, 2 cases showed the absence of ganglion cells with hypertrophied nerve bundles both in biopsy from the distal colon and rectum. Of these 2 cases, 1 had initial double-barrel colostomy and further underwent endorectal pull-through procedure (ERPT). Remaining 1 patient with initial resection anastomosis had anastomotic leak, underwent diverting colostomy but failed to survive due to sepsis. Closure of stoma was performed for patient with negative biopsy report after performing a distal colostogram. In one case with Type II sigmoid atresia, the distal rectum was inadequate for anastomosis and hence underwent ERPT procedure [Table 1].

Overall average hospital stay was 3.5 days for patients underwent initial colostomy and 21 days for patients underwent primary resection anastomosis. Postoperative complications included mainly wound dehiscence in 4 patients and anastomotic leak and sepsis in 2 patients [Table 1].

   Discussion Top

CA has been a rare disorder since Binninger first reported a case of CA in 1673. The first survivor was a case of sigmoid atresia reported in 1922 by Gaub.[5],[6],[12] Since then, several studies have been published regarding type of atresia, associated anomalies, management with primary anastomosis, or staged procedure to reduce the mortality and morbidity. However, none could withstand a definitive guideline.

The cause of CA has been attributed to intrauterine mesenteric vascular accident secondary to thromboembolic event, volvulus, and strangulation of small bowel or lack of recanalization of the solid cord stage of intestinal development. CA as a result of choledochal cyst due to extrinsic mesenteric pressure effect has been reported earlier.[12],[13],[14],[15] In our study, we did not find any intrauterine pressure effect as etiology to CA. CA may be an isolated anomaly or associated with other congenital anomalies association with malrotation, anorectal malformation, gastroschisis, HD, limb malformation, and choledochal cyst has been reported.[1],[2],[3],[4],[5],[14],[15],[16],[19] In our study, we found association with malrotation in 3 cases, HD in 2 cases, unilateral cleft lip in 1, and cardiac anomaly 2 patient and Meckels diverticulum in 1 patient. Presence of anomalies alters overall mortality. In our study, we had one death due to postoperative anastomotic leak and sepsis and 1 neonate with associated HD and cardiac anomaly died secondary-to-postoperative sepsis.

Previous case series have reported Type III CA as the most common type and the right colon as the most common location.[1],[2],[3],[4],[5],[6] The findings corroborated with our study, we found Type IIIa atresia in 6 cases (66.5%) and Type I in 2 cases (22.2%) and Type II in one case. Furthermore, most of the atresia was found proximal to splenic flexure [Figure 2].

The diagnosis of CA has been dicey with patients without abdominal distension and without multiple air-fluid levels in X-ray. Moreover, delay in diagnosis increased the risk of mortality.[1],[2],[15],[19] Hence, a high index of suspicion and contrast enema is essential for an early diagnosis of CA for patient who failed to pass meconium within 48 h. We performed contrast enema in seven patients with CA and found characteristics cutoff sign [Figure 1]c and [Figure 1]d. However, in four patients, this investigation was avoided due to sepsis and also their classical features of intestinal obstruction could be revealed clinically and in plain abdominal X-ray.

The time of presentation has been important for CA till the time of intervention. This has been highlighted in several series and case reports earlier.[1],[2] In our study, we found two patients which presented on 6th day and was in severe sepsis and unfit for anesthesia. Plain abdominal X-ray could only be obtained suggestive of distal intestinal obstruction. Average age of presentation was 2 days and mean interval for intervention was 12.8 h from time of presentation. Survival rate in our study was 77.7%.

Primary colo-colic anastomosis for atresia in the right colon and diverting colostomy for the left colon has been suggested by many studies earlier.[1],[3],[4],[5],[16],[17],[18],[19],[20],[21],[22],[23],[24] Dalla Vecchia et al. reported null mortality in 21 CA and in 3 out of 21 with primary one-stage anastomosis approach to CA.[15] Dassinger et al. found null mortality in 10 out of 12 cases with primary anastomosis.[18] Karnak et al. reported failure in three cases of primary anastomosis.[2] Both staged procedure and primary anastomosis was described by Pohlson et al.[17] In our experience, we performed primary anastomosis in three cases and postoperative leak was found in two patients. These patients were later diagnosed as HD in one and another had leak probably due to poor nutritional status and sepsis or delayed return of bowel activity. Several reports of intestinal atresia stated that proximal dilated bowel lack interstitial cells of Cajal and abnormality of intestinal musculature due to dilation results in delayed return of bowel activity.[25],[26] In our study, postoperative recovery in one patient with primary anastomosis was delayed by 21 days. However, patients with primary diverting stoma survived and were discharged early. Furthermore, there was no mortality seen in these patients who followed up for definitive surgery.

In our study, overall mortality was 22.2%. Etensel et al. in review of literature in 214 cases found an overall mortality rate 25.6% and in case of CA, HD must be ruled out.[1] Mortality is also higher with delayed presentation and associated anomalies. Hence, we support the formal dictum as early presentation of CA within 72 h reduces the mortality rate by more than 50%. Park in his case report stated that association of CA and HD is a diagnostic challenge and attempt to primary anastomosis results in failure with leakage and sepsis.[19] In our study, wound dehiscence was noted in four patients who may be probably due to prematurity and poor nutritional status.

   Conclusion Top

CA is a very rare disorder that possesses a great challenge in diagnosis and management. Awareness for the condition is of immense importance. Contrast enema is a key to the diagnosis and must not be delayed where there is a doubt and the patient is stable. Associated anomalies must be given priority to avoid mortality. Staged procedure with initial colostomy and followed by definitive procedure is the preferred choice irrespective of location of atresia. All cases of CA must be biopsied from distal part of the colon and rectum to rule out HD as an associated anomaly. Further detailed studies are required based on histology and immunohistochemistry of intestine to decide the level of resection of the proximal dilated colon and distal colon to achieve early bowel activity and reduce the morbidity.

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   References Top

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  [Figure 1], [Figure 2]

  [Table 1], [Table 2]


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