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398  Gastrointestinal Duplications
                                                               Ultrasonography
                                                               US is the most common modality used and should be the first choice.
                                                               It typically shows a double-layered wall (inner echogenic mucosa and
                                                               outer  sonoluscent  muscular  layer). When  this  double-layered  pattern
                                                               is present on US, a GID is confirmed and there is no need for further
                                                               radiologic evaluation. 25,26
                                                               Plain X-Rays
                                                               A plain chest x-ray (plain abdominal/lateral) will be able to detect fore-
                                                               gut duplications in the chest in up to 90% of cases. Plain abdominal
                                                               x-ray may show evidence of intestinal obstruction.
        Figure 66.1: Spherical duplication of the ileocaecal area in an 8-month-old child.
                                                               Contrast Medium Studies
                                                               Contrast medium studies may reveal compression or displacement of
        in  the  small  and  large  bowels.  The  lining  mucosa  is  usually  the   the adjacent organ. Rarely, it will show communication with the adja-
        same  as  the  adjacent  normal  bowel,  but  can  be  heterotopic,  such  as   cent native organ, but it does not specify the nature of the duplication.
        gastric,  squamous,  transitional,  ciliated  columnar  mucosa,  pancreas,   CT Scan or MRI
        lymphoid aggregates resembling Peyer’s patches, and ganglion cells.   A CT scan or MRI is employed in difficult cases. It is noninvasive and
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        Others include heterotopic lung tissue or thyroid stroma.  However,   has the advantage of demonstrating the exact location and relationship
        heterotopic  gastric  and  pancreatic  tissues  are  the  ones  of  significant   to adjacent normal structures. It may also reveal other duplications. It is
        clinical importance due to the risk of peptic ulceration and pancreatitis.   particularly useful in thoracic, pelvic, and the rare large retroperitoneal
        Even though GID in children is benign, malignant transformation has   duplication cysts. A spinal MRI will outline the relationship of the cyst
        been described in adults. 21
                                                               with the spinal column and spinal canal.
                             Diagnosis                         Technetium 99m Pertechnetate Scintigraphy Scan
        Clinical Features                                      This scan indicates the definite existence of GID when it contains ecto-
        There is no common clinical pattern of signs and symptoms of duplica-  pic gastric mucosa. This is especially useful in oesophageal, duodenal,
        tions. They present with a variety of symptoms or sometimes as masses   and tubular small bowel duplications with a high incidence of hetero-
        found  incidentally  during  routine  examinations  or  investigations,  or   tropic gastric mucosa.
        they are encountered during an operation for other problems.  Laparoscopy
           Most patients present before the age of 2 years, 11,12  but presentation   Laparoscopy is useful in cases when all the above investigations are
        during adulthood has been described. 4,22  The clinical presentation also   not conclusive.
        varies according to the age of the patient, location of duplication, type
        of mucosal lining, duration of disease, and presence of complications.     Treatment
        The  clinical  presentation  may  be  due  to  the  pressure  effect  of  the   The goal is to make a prompt diagnosis and provide treatment before
        duplication. Feeding difficulties are associated with masses in the floor   the onset of symptoms or the development of complications. The ideal
        of the mouth. In thoracic duplications, this leads to respiratory distress   treatment for GID is complete excision. However, GID in children is
        or dysphagia. Other symptoms in the chest include recurrent pneumonia   a benign disease, and any treatment should not be more radical than to
        and failure to thrive. In the abdomen, GID causes intestinal obstruction   eliminate the patient’s complaints and prevent further recurrence.
        but  may  also  cause  ureteric,  biliary,  or  even  vena  caval  obstruction.   Important points to be considered in the surgical treatment of GID
        Pancreatitis can arise from pressure on the pancreas. Duplications in the   include:
        abdomen commonly present with pain, vomiting, and abdominal mass.  1. the nature of the blood supply shared between the duplication and
           The clinical presentation may also be secondary to complications   native bowel;
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        of  the  duplications.  These  include  intussusception,   volvulus,    2. the presence of heterotopic gastric mucosa, which will negate
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                 23
        perforation,   bleeding  (related  to  ectopic  gastric  mucosa),  peptic   internal drainage due to the risk of peptic ulceration; and
        oesophageal stricture, and malignant transformation, as seen in adults. 21
                                                               3. the relationship with adjacent structures, such as the biliary tract in
        Prenatal Diagnosis                                     duodenal duplications.
        Prenatal  diagnosis  of  GIDs  is  becoming  widespread  in  the  Western   The  treatment  of  GID  is  best  considered  by  location  of  the
        world. This ability to accurately identify GID has provided an opportu-  duplication.  However,  in  selected  cases,  an  intraoperative  frozen
        nity to intervene. In cases of nonimmune hydrops in thoracic duplica-  section  may  give  further  information  on  the  absence  or  presence  of
        tions, thoracoamniotic shunting is carried out in utero in some centres   heterotopic components.
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        with  experience  in  foetal  treatment.  This  is  done  in  the  immediate   Oropharynx
        postnatal period before the onset of symptoms or the development of
                   2
        complications.                                         Oropharyngeal  duplications  are  rare  and  constitute  less  than  1%  of
                                                               duplications.  They  may  contain  ectopic  gastric  or  colonic  mucosa.
                          Investigations                       These cysts are excised by an intraoral incision.
        In  the  management  of  GID,  accurate  preoperative  diagnosis  is  dif-  Oesophagus
        ficult. This is because GID is very rare, and the clinical presentation   Oesophageal duplications are related to the right side of the oesophagus
        is nonspecific.                                        and  are  best  approached  through  a  right  posterolateral  thoracotomy.
           In  Africa,  where  resources  are  limited,  the  more  expensive   A  supraclavicular  approach  is  used  for  those  located  in  the  cervical
        investigations, such as computed tomography (CT) scan and magnetic   region.  These  lesions  should  be  completely  excised  due  to  the  high
        resonance  imaging  (MRI),  should  be  reserved  for  the  very  difficult   incidence  of  gastric  heterotopia  in  this  location. Where  facilities  are
        cases. The most common investigations carried out are ultrasonography   available, a thoracoscopic approach is preferable for isolated lesions.
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        (US) and contrast medium examinations.
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