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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
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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
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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
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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.