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                                                                       Intestinal Atresia and Stenosis  387

             At  exploration,  the  site  of  the  most  proximal  atresia  is  readily
          identified as the site of marked change in intestinal calibre. The outer
          wall  of  the  intestine  at  the  site  of  obstruction  may  appear  intact  or
          there may be an associated defect in continuity of the intestine and the
          mesentery (Figure 63.4). Generally, surgical treatment requires excision
          of the ends of the intestine involved in the atresia. It is also important
          to look for distal sites of obstruction, which can occur in up to 20%
          of patients and may not be immediately obvious due to lack of calibre
          change beyond the proximal atresia. These distal points of obstruction
          can be identified by flushing the distal intestinal lumen with saline to
          confirm intestinal continuity to the level of the rectum.
             After  resection  of  the  atretic  segment,  the  surgeon  is  faced  with
          the  difficult  task  of  re-establishing  continuity  between  intestinal
          segments with marked size discrepancies. Another consideration is the
          potential dysmotility of the proximal markedly dilated segment, which
          may result in delayed intestinal function and problems with bacterial
          overgrowth. Therefore, in patients with a relatively short segment of   Figure 63.4: Type I jejunal atresia. Membrane occlusion without mesenteric
          severely  dilated  proximal  intestine,  resection  of  the  dilated  segment   defect or loss of intestinal length.
          with  re-establishment  of  continuity  by  end-to-end  anastomosis  is  a
          good  option.  However,  in  patients  with  long  segments  of  proximal
          intestine that are significantly dilated, resection of the whole involved
          segment may result in inadequate remaining intestinal length to allow
          absorption of enteric nutrients (i.e., short bowel syndrome). Therefore,
          these patients frequently are treated by either imbrication or tapering
          enteroplasty of the proximal dilated segment. To date, no randomized
          studies have compared the outcomes for patients with intestinal atresias
          with or without the addition of an enteroplasty or plication. In patients
          for  whom  the  atresia  is  just  distal  to  the  duodenojejunal  flexure,  it
          may be advantageous to resect the dilated bowel, derotate, and taper
          the duodenum with primary anastomosis. This facilitates passage of a
          transanastomotic feeding tube and early restoration of foregut function.
          The total residual length of bowel should be measured with a tape and
          recorded, as this gives some guidance as to prognosis.
             Patients  who  have  multiple  atresias  (type  IV)  or  an  apple-peel
          deformity  (type  IIIb)  present  particularly  challenging  management
          problems (Figures 63.5 and 63.6). These patients may require multiple
          anastomoses and frequently will experience long-term delays in return   Figure 63.5: Type IIIb atresia (apple peel deformity). Note proximal jejunal atresia,
          of intestinal function. In addition, many of these patients will have short   malrotation, and mesenteric defect with a single artery of supply from the middle and right
          bowel syndrome due to inadequate residual intestinal length. In general,   colic vessels with significant loss of intestinal length.
          the formation of stomas is unnecessary and should be avoided because
          dilated bowel does not reduce in caliber, and fluid and electrolyte losses
          may be severe.
                     Postoperative Complications
          The most common postoperative complication is a functional obstruc-
          tion at the site of anastomosis. Unfortunately, this complication may be
          due to the underlying intestinal dysmotility associated with this anoma-
          ly and may not be preventable by changes in surgical technique. Other
          less commonly observed complications include anastomotic leak and
          adhesive obstructions. Obstructions due to missed distal unrecognized
          atresias should not occur and can be prevented by proper evaluation at
          the time of the initial operation.
                      Prognosis and Outcomes
          Most patients with intestinal atresia do not have associated life-threat-
          ening anomalies. Therefore, the primary factor that impacts mortality
          is the ability to support the nutritional needs of the patient during the
          postoperative period while awaiting adequate bowel function to allow
          enteral alimentation. In centres where parenteral nutritional support is
          feasible, these patients can be supported for prolonged periods of time
          while awaiting gastrointestinal function. However, in centres without
          these resources, patient mortality will be higher and primarily attribut-
          able to malnutrition. The judicious use of nasojejunal or gastrostomy   Figure 63.6: Type IIIb atresia with antenatal volvulus of the “apple peel” leading
                                                                 to short bowel.
          transanastomotic feeding tubes for enteral feeding may be life saving.
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