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388  Intestinal Atresia and Stenosis
                            Prevention                         Table 63.1: Evidence-based research.
        Unfortunately, there are no options at present for prevention because   Title  Jejuno-ileal atresia and stenosis
        these anomalies are usually not recognized prior to birth.
                                                                  Authors   Rode H, Millar AJW
                           Ethical Issues                         Institution  Red Cross Children’s Hospital, Cape Town, South Africa
        In  resource-poor  regions  without  recourse  to  intensive  care  and  par-  Reference  In: Prem Puri, ed. Newborn Surgery, 2nd ed. Arnold, 2003,
        enteral  nutrition,  infants  with  ultra-short  bowel  resulting  from  con-  Pp 445–456
        genital  atresia  may  have  to  be  managed  conservatively.  Discussion   Problem  Late presentation of jejunoileal atresia and stenosis in Africa.
        around  parental  expectations  and  centre  outcomes  should  be  part  of   Intervention  Surgery is curative if presentation is early enough.
        the informed consent. Nursing staff and other caregivers should also   Comparison/  Before 1952, the mortality for jejunoileal atresia alone was
        be party to the decision-making process. Withdrawal of treatment that   control   90%. Between 1952 and 1955, the mortality was 80%
        is thought to be futile is often difficult to institute. If there are choices   (quality of   when primary anastamosis was performed without bowel
        to be made based on allocation of limited resources, then infants with   evidence)  resection. Between 1955 and 1958, the mortality decreased
                                                                            to 22% due to liberal resection of the dilated loop and
        the potential for good outcomes may be given preference for meagre   primary anastomosis. From 1959 to 2000, the mortality has
        resources. However, it is only the infrequent case of intestinal atresia   decreased to 10%. Factors contributing to mortality were type
        that develops intestinal failure, and with prompt operation and preser-  III atresia, proximal bowel infarction, peritonitis, anastomotic
        vation of as much functioning bowel as possible, prognosis should be   leaks, missed distal atresias, short bowel syndrome, sepsis,
                                                                            and human immunodeficiency virus (HIV) infection.
        excellent.
                                                                  Outcome/  The survival rate is more than 90% in well-resourced
                    Evidence-Based Research                       effect    countries, but 40–50% in Africa, where no nutritional support
        Table 63.1 presents a study of the change in mortality rates from jeju-  is available and presentation is late.
        noileal atresia and stenosis over a nearly 50-year period.  Historical   No advancement on vascular theory for aetiology. A variant of
                                                                  significance/   multiple intestinal atresias may have a familial/genetic cause.
                                                                  comments



                                                  Key Summary Points
            1.  Intestinal atresia may occur at any level of the gastrointestinal   5.  The primary surgery for intestinal atresia consists of a
              tract.                                              generous back resection of the bulbous blind end and an end-
            2.  Small bowel atresia in most cases is due to an antenatal   to-end anastomosis.
              ischaemic insult to a segment of intestine. Resorption of the   6.  Outcomes are generally good if sufficient bowel length
              infarcted segment leads to occlusion of the lumen, with a   remains.
              varying degree of dilatation of the proximal blind end.
                                                               7.  Stomas should be avoided.
            3.  One-third of infants with intestinal atresia are born prematurely.  8.  The mortality rate depends on birth weight, residual bowel
            4.  Differential diagnoses include midgut volvulus, meconium ileus,   length, the degree of dysmotility, associated anomalies, and
              extensive aganglionosis, and intussusception.       septic complications.




                                                        References

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            3.   Barrack SM, Kyambi JM, Ndungu J, Wachira N, Anangwe G,   7.   Surana R, Puri P. Small intestinal atresia: effect on fetal nutrition. J
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               at Kenyatta National Hospital, Nairobi. East Afr Med J 1993;   8.   Millar AJW, Rode H, Cywes S. Intestinal atresia and stenosis. In:
               70:558–564.                                         Ashcraft KW, Holder TM, eds. Pediatric Surgery, Saunders, 2000,
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