Congenital multiple colonic atresias with intestinal malrotation: a case report

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.

Hereditary Multiple Intestinal Atresia: A Case Report and Review of the Literature

“…Based on the pathological finding, the suggested cause of HMIA is the malformation of the gastrointestinal tract, rather than the presence of ischemic changes, which is usually associated with mesenteric defects caused by vascular insults [ 4 , 8 ]. The most likely explanation for the disease is autosomal recessive transmission, as suggested by Bilodeau et al [ 4 ] and Ishii et al [ 9 ].…”

Short-term curative effect of laparoscopic assisted surgery on patients with congenital intestinal malrotation and its effect on gastrointestinal function in very low birth weight infants.

“…According to the random residue grouping method, they were divided into a control group and an observation group with 50 cases each. Indications for laparoscopic surgery: (1) No serious heart and lung function damage. (2) The coagulation function is normal.…”

The twists and turns of left-right asymmetric gut morphogenesis

“…As the number and curvature of gut loops is dependent not only on the rate of gut lengthening, but also on the radius of the gut and the length/thickness of the mesentery (Nerurkar et al, 2017), any perturbation that alters the geometric and/or physical properties of different segments of the gut tube and/or the attached DM might influence the mechanics of looping and 'rotation'. For example, defects in the radial patterning and diameter of the gut tube itself may affect its mechanical properties and 'loopability'; indeed, intestinal narrowing (stenosis and/or atresia) has been associated with malrotation in humans (Adams and Stanton, 2014;Chinya et al, 2019;Ishii et al, 2020;Morikawa et al, 2009). Interestingly, these narrowing defects are often attributed to 'vascular accidents' that inhibit blood flow during gut development (Adams and Stanton, 2014;Ishii et al, 2020;Martin and Shaw-Smith, 2010).…”

“…For example, defects in the radial patterning and diameter of the gut tube itself may affect its mechanical properties and 'loopability'; indeed, intestinal narrowing (stenosis and/or atresia) has been associated with malrotation in humans (Adams and Stanton, 2014;Chinya et al, 2019;Ishii et al, 2020;Morikawa et al, 2009). Interestingly, these narrowing defects are often attributed to 'vascular accidents' that inhibit blood flow during gut development (Adams and Stanton, 2014;Ishii et al, 2020;Martin and Shaw-Smith, 2010). Thus, the DM, which is crucial for development of the gut vasculature (Sivakumar et al, 2018), may play a key role in asymmetric intestine morphogenesis simply by supporting proper gut tube growth and/or elongation.…”