Pediatr Surg Int (2009) 25:579–582 DOI 10.1007/s00383-009-2401-3
ORIGINAL ARTICLE
Management of colonic atresia with primary resection and anastomosis Melvin Dassinger Æ Richard Jackson Æ Samuel Smith
Accepted: 28 May 2009 / Published online: 11 June 2009 Ó Springer-Verlag 2009
Abstract Purpose Historically, the majority of patients with colonic atresia have been managed by ostomy creation at the time of initial operation; conversely, we have tended to treat the atresia with resection and anastomosis. The purpose of this study is to determine the rate of concomitant Hirschsprung’s disease and to identify potential morbidity associated with primary repair. Methods After IRB approval (#105825), charts of patients identified by ICD-9 code as having colonic atresia, who were admitted to our institution between 1993 and 2008 were, retrospectively, reviewed. Particular attention was paid to demographic data, comorbidities, operative therapy, and complications. Continuous variables were compared using an unpaired t test. A P value of \0.05 was considered significant. Results Twelve newborns with colonic atresia were identified. Two babies were managed initially with ostomy, both of whom had gastroschisis; primary repair was performed on ten, with two undergoing simultaneous rectal biopsy. No anastomotic complications occurred and there were no mortalities. One patient experienced self-limited malabsorption symptoms after repair. No cases of Hirschsprung’s disease were detected. Conclusions Uncomplicated colonic atresia can be managed by primary repair with little morbidity.
M. Dassinger (&) R. Jackson S. Smith Arkansas Children’s Hospital, Little Rock, USA e-mail:
[email protected]
Keywords Colonic atresia Neonatal bowel obstruction Gastroschisis Primary repair
Introduction Colonic atresia is a rare cause of neonatal bowel obstruction, accounting for less than\10% of all gastrointestinal atresias [1]. Historically, the majority of patients with colonic atresia have been managed by ostomy creation at the time of initial operation [2]; primary repair has been recommended only after rectal biopsy has excluded Hirschsprung’s disease (HD), a rarely associated comorbidity [3]. Conversely, we have tended to treat the atresia with the resection and primary anastomosis, often without simultaneous rectal biopsy. The purpose of this study is to determine our rate of concomitant Hirschsprung’s disease and to identify the potential morbidity associated with primary repair.
Methods After IRB approval (#105825), charts of patients identified by ICD-9 code as having colonic atresia who were admitted to our institution between 1993 and 2008 were, retrospectively, reviewed. Particular attention was paid to demographic data, method and timing of diagnosis, comorbidities, operative therapy and complications. Continuous variables were compared using an unpaired t test. A P value of\0.05 was considered significant. The type of defect was classified according to Grosfeld’s description: Type I, mucosal atresia with intact bowel wall and mesentery; type II, blind-ending bowel separated by a fibrous cord; type III, blind-ending bowel separated by a V-shaped mesenteric defect; type IV, multiple atresias.
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Results
Discussion
Twelve newborns with colonic atresia were cared for at our institution (Table 1). Three (25%) presented with gastroschisis. These babies had a significantly lower birth weight (1.96 vs. 2.93 kg, P \ 0.05) and gestational age (35 vs. 38.5 weeks, P \ 0.05) when compared with those without gastroschisis. The remaining nine all presented with some degree of abdominal distention, with only two having bilious emesis. Babies in this cohort were diagnosed with colonic atresia on a median of day of life 3 (range 2–4). Barium enema was diagnostic in all cases. Seven (58%) patients had comorbidities: three with gastroschisis, two with malrotation (without gastroschisis), two with orthopedic anomalies, one baby with multiple atresias, and one with an atrial septal defect. The site of atresia was found to be right-sided in seven, transverse in two and distal to the splenic flexure in three. A single type I defect was described, with the remainder being type III. Two babies were managed initially with ostomy, both of whom had gastroschisis; primary repair was performed on ten, including one of the babies with gastroschisis. The patient with the type I defect underwent simple excision of the membranous web. Of the remaining 11 patients, 10 had an ileocolic anastomosis, while one was managed with a colocolostomy. Only two underwent simultaneous rectal biopsy. No cases of Hirschsprung’s disease were detected. No anastomotic complications occurred in whom primary anastomosis was performed. One patient did experience self-limited malabsorption symptoms after repair. Median day of discharge for this group was postoperative day 9 (range 4–25 days). There were no mortalities.
Colonic atresia occurs in roughly 1 in 60,000 live births [2] and accounts for \10% of all gastrointestinal atresias [4]. Whereas prematurity is common in babies with duodenal or jejunoileal atresias, it occurs in less than one-third of babies with colonic atresia [1]. This fact held true in our cohort as well, with only three babies delivered at \37 weeks gestation, all of whom had gastroschisis. Comorbidities occurred in 58% of our patients compared with a rate of 47% published in a recent literature review. As in our group, abdominal wall defects and malrotation were among the more common associated conditions [2]. Neonates with colonic atresia present with a range of symptoms typical for obstruction: abdominal distention, intolerance of feeds, bilious emesis and failure to pass meconium. In our series, distension was the most common symptom with only two babies having bilious emesis. Diagnosis is often delayed several days [2] and is suspected when plain radiographs reveal air-fluid levels and a disproportionately dilated loop of bowel (Fig. 1). Distal contrast enemas are commonly obtained and show the expected microcolon (Fig. 2). Type III defects are the most common form of colonic atresia encountered at laparotomy, with the majority found in the right colon. Type I is the next most common. These defect, however, occur primarily distal to the splenic flexure. All of these trends held true for our cohort as well. Operative therapy for colonic atresia has changed over the years. Initial colostomy was recommended for those lesions in the left colon; primary anastomosis could be performed for those lesions in the right or transverse colon, assuming physiologic stability. Recently, some authors have advocated primary repair regardless of location [5]. We have employed the latter approach at our institution.
Table 1 Characteristics of neonates with colonic atresia Patient #
EGA (weeks)
Weight (kg)
Site
Type
Comorbities
Morbidity
Mortality
1
37
2.9
Right
III
No
None
Alive
2
37
2.55
Right
III
No
Diarrhea
Alive
3
38
3.38
Right
III
Malrotation
None
Alive
4
39
2.8
Transverse
III
No
None
Alive
5
37
2.01
Left
III
ASD
None
Alive
6
41
3.79
Sigmoid
Clubfoot, syndactyly, polydactyly
None
Alive
7
34
1.8
Right
III
Gastroschisis
None
Alive
8
35
1.9
Right
III
Arthrogryposis, gastroschisis, multiple atresias
None
Alive
9
40
2.99
Left
III
None
None
Alive
10
39
3.0
Right
III
Malrotation
None
Alive
11
36
2.2
Right
III
Gastroschisis
None
Alive
12
39
2.95
Transverse
III
None
None
Alive
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Fig. 2 Barium enema demonstrating the typical microcolon and lack of filling of the proximal dilated bowel Fig. 1 Plain abdominal radiograph demonstrating the disproportionately dilated colon proximal to the atresia
When type III defects were found, the bulbous proximal atretic end was resected and anastomosis performed. This operative approach differs from that used in jejunoileal atresias, where tapering of the proximal segment is the rule. However, tapering of the proximal segment has been used in only three recorded cases of colonic atresia [2]. Type I lesions were treated with simple web excision. No subsequent anastomotic complications were seen. Despite the loss of the ileocecal valve in ten patients, only one experienced malabsorptive symptoms, which resolved without intervention. The only patients whom we diverted were those who presented with gastroschisis. If feeding difficulties or anastomotic complications arise after primary repair, Hirschsprung’s disease must be considered. Since Hyde and deLormier first reported a case of coexistent HD and CA in 1968 [6], 26 simultaneous cases have been reported [7, 8]. Only one of these reported cases had the diagnosis of HD made prior to restoration of continuity [9]. Furthermore, the average time to diagnosis of HD in one cohort was 15 months [2]. These delays in diagnoses illustrate the difficulty in predicting based on the gross appearance which patients have HD. Fishman et al. have correlated the lack of distal colonic fixation with the presence of aganglionosis [10]. However, this association has not been consistently seen [10, 11]. The majority of our patients’ charts did not comment on the status of distal colonic fixation. However,
two patients were found at laparotomy to have malrotation which was treated with Ladd’s procedure and three patients had gastroschisis. The association with HD has led to the recommendation of rectal biopsy to rule out aganglionosis prior to the restoration of intestinal continuity in patients with colonic atresia. Although this was not consistently our practice pattern, we may have been fortunate. In 2005, Etensel et al. surveyed the world’s literature, finding 242 reported cases of CA. Since that series was published, Cox et al. reported an additional 14 neonates, none of which had simultaneous HD [12]. Therefore, if our 12 patients are included, the total number of reported cases of CA is 268. Thus, the incidence of concomitant HD is 9.7% (26/268). This figure is almost certainly an overestimate, since many cases go unreported; however, the true incidence may well be higher than the 2% rate of coexistent aganglionosis that is traditionally reported [13]. Colonic atresia is a rare form of neonatal bowel obstruction. Whether managed initially by diverting ostomy or primary repair, mortality rates are low. Our series reinforces the concept that patients with uncomplicated CA can be managed by initial resection and anastomosis, regardless of the location of the atresia, with minimal morbidity. Even though we saw no cases on concomitant HD, the incidence may be higher than previously thought. Thus, rectal biopsy should be strongly considered prior to restoration of intestinal continuity.
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