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      Congenital multiple colonic atresias with intestinal malrotation: a case report

      case-report

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          Abstract

          Background

          Congenital intestinal atresia develops in 1 in 1500 to 20,000 births. Colonic atresia, which accounts for 1.8–15% of intestinal atresia cases, is accompanied by other gastrointestinal atresias such as small intestinal atresia, gastroschisis, imperforate anus, and intestinal malformation in 47–80%. Although a report shows that patients with multiple colonic atresias are 8.9% of those with colonic atresia.

          Case presentation

          A male infant did not have the first bowel movement within 36 h of birth and had abdominal distention/vomiting. Radiography showed significant dilation of the intestinal tract. A contrast enema examination at 3 days of age showed a microcolon and disruption in the descending colon. We performed an emergency decompressive loop enterostomy in the distended segment. At the age of 7 months, imaging from the stoma showed disruption of the contrast medium in the intestinal tract at the right lower abdomen, and the continuity of the intestinal tract was not clarified. Intestinal malrotation was found during the second surgery, and the enterostomy was located in the ileum proximal to Bauhin’s valve. Continuity of the intestinal serosal surface was maintained. However, multiple membranous obstructions (three atresias and one stenosis) were observed in the distal segment of the bowel, which was penetrated by intraluminal advancement of a urethral catheter. Therefore, he was diagnosed with multiple colonic atresias. The intestinal tract was longitudinally incised, and membranectomy and mucosal/lateral suture were performed.

          Conclusions

          It is important for neonates with intestinal atresia to evaluate and prepare for distal patency of the colon before radical anastomosis. In addition, anomalies associated with colon atresia should also be assessed.

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          Most cited references16

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          Intestinal atresia and stenosis: a 25-year experience with 277 cases.

          To evaluate the causes, clinical presentation, diagnosis, operative management, postoperative care, and outcome in infants with intestinal atresia. Retrospective case series. Pediatric tertiary care teaching hospital. A population-based sample of 277 neonates with intestinal atresia and stenosis treated from July 1, 1972, through April 30, 1997. The level of obstruction was duodenal in 138 infants, jejunoileal in 128, and colonic in 21. Of the 277 neonates, 10 had obstruction in more than 1 site. Duodenal atresia was associated with prematurity (46%), maternal polyhydramnios (33%), Down syndrome (24%), annular pancreas (33%), and malrotation (28%). Jejunoileal atresia was associated with intrauterine volvulus, (27%), gastroschisis (16%), and meconium ileus (11.7%). Patients with duodenal obstruction were treated by duodenoduodenostomy in 119 (86%), of 138 patients duodenotomy with web excision in 9 (7%), and duodenojejunostomy in 7 (5%) A duodenostomy tube was placed in 3 critically ill neonates. Patients with jejunoileal atresia were treated with resection in 97 (76%) of 128 patients (anastomosis, 45 [46%]; tapering enteroplasty, 23 [24%]; or temporary ostomy, 29 [30%]), ostomy alone in 25 (20%), web excision in 5 (4%), and the Bianchi procedure in 1 (0.8%). Patients with colon atresia were managed with initial ostomy and delayed anastomosis in 18 (86%) of 21 patients and resection with primary anastomosis in 3 (14%). Short-bowel syndrome was noted in 32 neonates. Morbidity and early and late mortality. Operative mortality for neonates with duodenal atresia was 4%, with jejunoileal atresia, 0.8%, and with colonic atresia, 0%. The long-term survival rate for children with duodenal atresia was 86%; with jejunoileal atresia, 84%; and with colon atresia, 100%. The Bianchi procedure (1 patient, 0.8%) and growth hormone, glutamine, and modified diet (4 patients, 1%) reduced total parenteral nutrition dependence. Cardiac anomalies (with duodenal atresia) and ultrashort-bowel syndrome (<40 cm) requiring long-term total parenteral nutrition, which can be complicated by liver disease (with jejunoileal atresia), are the major causes of morbidity and mortality in these patients. Use of growth factors to enhance adaptation and advances in small bowel transplantation may improve long-term outcomes.
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            Congenital intestinal atresia; observations on its origin.

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              Atresia of the colon.

              Colonic atresia (CA) is one of the rarest causes of neonatal intestinal obstructions, and no large series can be reported. Therefore, we did perform a retrospective clinical trial to delineate our CA cases and carry out a literature survey. We reviewed the charts of CA cases treated in our center between 1992 and 2002. We aimed to collect all reported cases in Medline, and personal communications with the authors of published series were used to reach the missing data. The chart review revealed 9 newborns with CA treated in our center (6 cases of type III, 2 cases of type II, and 1 case of type IV). These accounted for 3.7% of all gastrointestinal atresias managed in our center. Of the CA cases, 3 were isolated and 6 had at least one or more associated congenital anomalies. The preferred surgical technique at the initial treatment of CA was performing a proximal stoma and distal mucous fistula in an average of postnatal 59.4 hours. The literature survey enabled us to reach 224 cases of CA, including our cases. Because of the low incidence of CA, delay in diagnosis and treatment may occur. The mortality is statistically higher when the surgical management is performed after 72 hours of age. However, the prognosis of CA is satisfactory if diagnosis and surgical management could be made promptly and properly.
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                Author and article information

                Contributors
                d-ishii@asahikawa-med.ac.jp
                miyagi@mvj.biglobe.ne.jp
                hirasawa@asahikawa-med.ac.jp
                miyamoto@asahikawa-med.ac.jp
                Journal
                Surg Case Rep
                Surg Case Rep
                Surgical Case Reports
                Springer Berlin Heidelberg (Berlin/Heidelberg )
                2198-7793
                30 March 2020
                30 March 2020
                December 2020
                : 6
                : 60
                Affiliations
                GRID grid.252427.4, ISNI 0000 0000 8638 2724, Department of Pediatric Surgery, , Asahikawa Medical University, ; 2-1-1, Midorigaoka-higashi Asahikawashi, Hokkaido, 078-8510 Japan
                Article
                822
                10.1186/s40792-020-00822-z
                7105550
                32232592
                6119092e-b780-4efa-b201-619d39802d13
                © The Author(s) 2020

                Open AccessThis article is licensed under a Creative Commons Attribution 4.0 International License, which permits use, sharing, adaptation, distribution and reproduction in any medium or format, as long as you give appropriate credit to the original author(s) and the source, provide a link to the Creative Commons licence, and indicate if changes were made. The images or other third party material in this article are included in the article's Creative Commons licence, unless indicated otherwise in a credit line to the material. If material is not included in the article's Creative Commons licence and your intended use is not permitted by statutory regulation or exceeds the permitted use, you will need to obtain permission directly from the copyright holder. To view a copy of this licence, visit http://creativecommons.org/licenses/by/4.0/.

                History
                : 23 November 2019
                : 19 March 2020
                Categories
                Case Report
                Custom metadata
                © The Author(s) 2020

                colonic atresia,multiple,intestinal malrotation,microcolon

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