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                                                                       Gastrointestinal Duplications  399
          Thoracoabdominal                                       The walls of the ducts may be included in the thickness of the wall of
          Thoracoabdominal cysts are not intimately adherent to the oesophagus   the duplication and may also share the same blood supply with the cyst
          but are usually to the posterior right side of the chest and may have com-  (Figure 66.2). In these cases, the options include: 29
          munication through the right crus of the diaphragm to the pylorus, duode-  • partial resection of part of the cyst wall but including the mucosal
          num, jejunum, or ileum. They are often lined by ectopic gastric mucosa.   lining; or
          It is also important to ensure that there is no neuroenteric communication.
          If present, this should be excised in consultation with a neurosurgeon.   • internal drainage through a wide cystoduodenal anastomosis or a
          Incomplete excision will lead to meningitis. The best approach here is the   cystojejunal Roux-en-Y anastomosis.
          use of separate posterolateral thoracotomy and abdominal approaches.   Small Bowel
          Depending on the extent of the lesion and the patient’s condition, it may   The small bowel (jejunum/ileum) is the site of about half of all GIDs.
          be carried out at the same surgery or staged. For isolated lesions, thora-  The  cystic  types  of  duplications  are  sometimes  easily  shelled  out,
          coscopic resection is becoming the preferred treatment.  but most are resected with primary end-to-end anastomosis to restore
          Gastric                                                bowel continuity. A laparoscopic approach may be effective to identify
          Gastric duplications represent 8% of duplications. Unlike other duplica-  the lesion and minimize the abdominal wall incision by lifting up the
                                      28
          tions, these are more common in girls.  Most of these duplications are   affected bowel segment.
          on the greater curvature, but they can rarely be on the lesser curvature   The  tubular  type  of  GID  can  involve  the  whole  ileum.  It  has  an
          or the pylorus. They usually do not communicate with the stomach, and   80% incidence of gastric heterotopia. Extensive resections will lead to
          complete excision is possible. More extensive lesions are excised with   short bowel syndrome, and drainage into the adjacent normal bowel is
          a limited partial gastrectomy.                         not encouraged due to the risk of peptic ulceration. In these cases, the
                                                                                                                   30
          Duodenum                                               multiple stepwise stripping of the mucosa is as described by Wrenn,
                                                                                                                   31
          Duodenal duplications also are rare. The treatment of choice for duo-  but anastomosis of the duplication to the stomach has also been tried.
          denal duplications is complete excision with preservation of the duo-  Colon
          denum. However, this total excision is not always possible if the cyst   Colonic  duplications  rarely  contain  ectopic  gastric  mucosa. The  rare
          is in close proximity to the pancreas or the biliary or pancreatic ducts.   complete  colorectal  duplication  may  be  associated  with  doubling
                                                                                                                   17
                                                                 anomalies of the genitourinary organs, such as the bladder and vagina.
                                                                 Cystic duplications can be shelled out or resected with primary coloco-
                                                                 lostomy. Tubular duplications of the colon have one or more communi-
                                                                 cations with the native bowel. If it is extensive, a distal communication
                                                                 is created with the colon or rectum.
                                                                 Rectum
                                                                 Rectal duplications are usually presacral. In 90% of cases, there is no
                                                                 communication  with  the  rectum.  The  posterior  sagittal  approach  is
                                                                 preferred. It gives good exposure, facilitates safer removal and repair,
                                                                 and prevents entering or compromising the rectal lumen. Laparotomy is
                                                                 preferable, however, for the rare anterior duplications. 32
                                                                              Prognosis and Outcome
                                                                 The outcome of surgical treatment of duplications is good. Poor out-
                                                                 comes are observed when there are associated severe malformations,
                                                                 which in themselves carry a high morbidity and mortality.
                                                                             Evidence-Based Research
                                                                 GIDs  are  very  rare  anomalies,  so  most  large  series  are  accumulated
          Figure 66.2: Communicating cystic duplication of the duodenum. Exposure   over three to four decades from big referral centres. It is difficult to
          after duodenotomy. The upper tube is within the major papilla; the lower one   have randomized studies.
          intubates the cyst.


                                                    Key Summary Points

             1.  Gastrointestinal duplications are rare.         7.  The presence of ectopic gastric mucosa, especially in tubular
                                                                    duplications, should be documented, as it has an impact on the
             2.  GIDs can arise from the mouth to the anus.
                                                                    approach to management.
             3.  The aetiology of gastrointestinal duplications is heterogenous.
                                                                 8.  Ultrasonography should be the first line of investigation.
             4.  About half of all duplications are located in the small bowel.
                                                                 9.  The ideal treatment is total excision; however, in children, GID
             5.  The clinical features of gastrointestinal duplications is   is a benign disease, and therapy should not jeopardise the
                nonspecific; a high index of suspicion is required for prompt   integrity of the adjacent normal bowel.
                diagnosis.
                                                                 10. The results of surgical treatment are good.
             6.  Most patients with gastrointestinal duplications will present
                before 2 years of age, but presentation during adulthood, as
                well as late malignant transformation, has been described.
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