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A Rare Case of Perforated Tubular Ileal Duplication in 72-Year-Old Male

Authors:
  • shree krishna hospital karamsad

Abstract

Enteric duplication is a rare anomaly, which is common in pediatric population, and it is very rare in adults. Enteric duplication can occur anywhere from mouth to anus. Ileal duplication is the most common (30 %) followed by duodenum, stomach, jejunum, colon and rectum. Though most cases are presented within the first 2 years of life, some may present at later age. Here we report a case of a 72-year-old male presented to us in emergency as acute abdomen, which was presumptively diagnosed as ca rectosigmoid, but later the patient developed perforative peritonitis. On exploratory laparotomy he was diagnosed as a case of perforated ileal duplication cyst. Though these lesions are very rare, the importance of enteric duplication cysts lies in the fact that they can mimic many disease conditions and may be left untreated.
CASE REPORT
A Rare Case of Perforated Tubular Ileal Duplication in 72-Year-
Old Male
Gajanan Ekbote &Ashish Balwant Pokharkar &
Prashant Moon
Received: 9 June 2011 / Accepted: 11 September 2012 / Published online: 27 September 2012
#Association of Surgeons of India 2012
Abstract Enteric duplication is a rare anomaly, which is
common in pediatric population, and it is very rare in adults.
Enteric duplication can occur anywhere from mouth to anus.
Ileal duplication is the most common (30 %) followed by
duodenum, stomach, jejunum, colon and rectum. Though
most cases are presented within the first 2 years of life, some
may present at later age. Here we report a case of a 72-year-
old male presented to us in emergency as acute abdomen,
which was presumptively diagnosed as ca rectosigmoid, but
later the patient developed perforative peritonitis. On ex-
ploratory laparotomy he was diagnosed as a case of perfo-
rated ileal duplication cyst. Though these lesions are very
rare, the importance of enteric duplication cysts lies in the
fact that they can mimic many disease conditions and may
be left untreated.
Keywords Ileum .Intestinal duplication .Perforation
Introduction
Duplication cysts are a rare group of anomalies which vary
in their presentation. Often they present in a very nonspe-
cific way like vague pain in the abdomen or lump in the
abdomen. Rarely, they present as intestinal obstruction,
perforative peritonitis, malignancy, melena, intussusception
and constipation. There are different theories regarding their
origin but none are proven yet. Enteric duplication can occur
along the entire length of alimentary tract. They are located
along the mesenteric border of the intestine and are lined by
smooth muscle. Heterotopic gastric mucosa often lines
duplication cyst, and its inflammation is responsible for
melena, perforation and intestinal obstruction. Enteric du-
plication can be a diagnostic dilemma for those who are
involved in its management. Often diagnosis is made on the
operating table as in our case.
Case report
A 72-year-old Indian male came to us with history of pain in
the abdomen for 3 months, and vomiting and loose motions
for 10 days. On admission, it was found that the patient had
tenderness in the left iliac fossa and hypogastric region, and
he was febrile with raised leukocyte count. X-ray erect
abdomen was normal. On ultrasonography, it was found that
the patient had thickened wall of the rectosigmoid junction
along with few thickened ileal loops. Patients CT scan
abdomen was done, which showed rectosigmoid wall thick-
ening and the hypoechoic mass attached to the rectum on the
right side. Barium enema showed the apple core sign likely
to be due to malignancy, but on colonoscopy all large colon
found to be normal.
Decision was taken for elective laparotomy, but a day
before elective surgery the patient developed severe pain in
the abdomen with rigidity. X-ray erect abdomen showed air
under the right side of the diaphragm, so emergency lapa-
rotomy was done.
During exploration, we found ruptured small bowel with
inflammatory bowel loops adhered to the rectosigmoid re-
gion in the left iliac fossa. On separating adhesions, we
found interesting finding: a 10 cm duplicated ileal loop,
which was perforated. On opening its anterior wall, it was
found to be communicating both proximally and distally
(Figs. 1,2and 3) Segmented bowel resection and end-to-
end anastomosis was performed. Patients postoperative re-
covery was uneventful.
G. Ekbote :A. B. Pokharkar (*):P. Moon
B. J. Medical College,
Pune, Maharashtra, India
e-mail: ashishpokharkar@rediffmail.com
Indian J Surg (June 2013) 75(Suppl 1):S418S420
DOI 10.1007/s12262-012-0749-4
Discussion
Reginald Filtz [1] first used the term intestinal duplication
in 1844. Intestinal duplication anomalies are generally com-
mon in pediatric population within the first 2 years of life
[2]. About 2/3 cases are detected within the first 2 years.
This finding is very rare in adult population.
Several theories are proposed for the pathogenesis of
intestinal duplication. According to split notochord theory,
splitting of the notochord is a primary event during embry-
ological growth responsible for duplications. Bentley and
Smith in their landmark article described the concept of the
split notochord and found a spectrum of disorders that share
a common embryogenesis, which includes enteric duplica-
tion along with vertebral anomalies [3]. Other theories are
persistent vacuolation theory, faulty separation of the endo-
derm and notochord, localized forms of twinning, etc [4].
Duplication cysts can be found anywhere from mouth to
anus but is most common in the small intestine. Ileal duplica-
tion is the most common (30 %) followed by duodenum,
stomach, jejunum, colon and rectum [5]. There are two forms
of duplications: spherical or cystic duplications and tubular
duplications. Cystic duplications (75 %) are more common
than tubular duplications (25 %) [6]. These hollow structures
possess a well-developed smooth muscle wall and are lined by
intestinal mucosa. Often heterotopic tissues like gastric mu-
cosa or pancreatic tissue are present in the wall of them. The
length of tubular duplications varies from few centimeters up
to as long as the whole length of the small intestine. Tubular
duplication often communicates with the main intestine prox-
imally or distally or at both ends like in our case. These
duplications share common wall and blood supply.
Intestinal duplications are very difficult to diagnose preop-
eratively. They present with myriad of symptoms. The symp-
toms are often related to location of duplication. Most cases
are detected during early years of life; as far as we know there
are very few cases in the literature which are diagnosed at old
age in adults. The most common clinical presentation in adults
is vague abdominal pain and dyspepsia. Other less common
presentations are intestinal obstruction, perforative peritonitis,
malignancy [7], melena [8], intussusception and constipation.
Acid secreting heterotopic gastric mucosa often causes peptic
ulcer disease, which leads to perforation and peritonitis.
Diagnosis of intestinal duplication is often a challenging
task. Most of the time it is not possible to diagnose preop-
eratively or misdiagnosis is made like in our case and
diagnosis is made on the operating table. Various radiolog-
ical modalities like ultrasound and CT scan are helpful in
making diagnosis. Ultrasound is important in case of cystic
duplication but is often inconclusive in tubular duplications
[9,10]. Barium enemas can be helpful in few cases but most
of the time is not much helpful. 99Tcm-pertechnetate scan-
ning is a reliable 99Tcm-pertechnetate, noninvasive method
to diagnose ectopic gastric mucosa [11,12].
Most authors believe that treatment is required in symp-
tomatic patients and those who present with complications.
Intestinal resection and anastomosis is required in all cases
and include adjacent normal intestine because of common
sharing of bowel wall and of blood supply [13,14]. Postop-
erative outcomes are often favorable. Though there are some
Fig. 1 Ileal duplication (arrow) with perforation on exploratory
laparotomy
Fig. 2 Wall of intestinal duplication is cut opened to show common
sharing of wall (arrow) between duplication and normal intestine;
Ryles tube is within normal ileum
Fig. 3 Diagrammatic representation of ileal duplication
Indian J Surg (June 2013) 75(Suppl 1):S418S420 S419
case reports of malignancies in ileal duplication, these find-
ings are rare [2].
To conclude, ileal duplications are rare anomalies in
adults, which are often undiagnosed preoperatively and
can present as perforative peritonitis, intestinal obstruction,
bleeding and malignancy.
References
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S420 Indian J Surg (June 2013) 75(Suppl 1):S418S420
... Duplications of the gastrointestinal tract are rare congenital anomalies that are often found early in life (more than 80 % are diagnosed in children under 2 years of age, and rarely in adults) [1,2]. The preoperative diagnosis of duplication cyst is difficult because the clinical manifestations are highly variable, especially in adults. ...
... Duplication cysts are rare congenital malformations (1/10,000 live birth) that can appear anywhere in the gastrointestinal tract from the oral cavity to the anus [2 -4]. Eighty percent are diagnosed in children below the age of 2 years, with a male predominance [1,2]. The ileum is the most frequently affected site [2]. ...
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Background and study aims Duplication cysts of the ileum are rare and present with non-specific clinical manifestations such as abdominal pain, vomiting, melena, and intussusception. Therefore, preoperative diagnosis is difficult. Here, we report a case of duplication cyst of the small intestine that was diagnosed preoperatively using double-balloon enteroscopy. A 19-year-old man presented with severe iron deficiency anemia, abdominal pain, and exertional dyspnea. Gastroscopy and colonoscopy revealed no remarkable findings. Abdominal computed tomography revealed a cystic structure in the ileum. Therefore, we performed double-balloon enteroscopy via the anal route. The intestinal tract was bifurcated, with one segment ending in a blind sac containing normal villi and an ulceration. Tc-99 m pertechnetate scintigraphy showed no accumulation in the lesion. Accordingly, we diagnosed a duplication cyst and suspected that this was the cause of severe anemia. Following small bowel resection with cyst excision and anastomosis, the anemia and presenting symptoms resolved. This report highlights the usefulness of double-balloon enteroscopy of the small intestine for preoperative diagnosis of the obscure gastrointestinal bleeding, including duplication cysts.
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The ileal duplication, which is a congenital anatomical abnormality of the digestive tract, can lead to the manifestation of the alimentary tract duplication in the small intestine. It is also the rare cause of gastrointestinal bleeding and usually seen in infants, but only rarely in adults. Herein, we describe a case of adult male was suffering from intermittent massive hematochezia for three years, accompanied by abdominal pain, syncope, and palpitations. However, no positive findings were found by gastroscopy and colonoscopy in other hospitals. He visited our hospital owing to the recurrent hematochezia, but re-examination by gastroscopy and colonoscopy indicated no significant abnormality, and hence small intestinal vascular malformation bleeding was considered. Therefore, double-balloon enteroscopy (DBE) examination was conducted and the results revealed a double-lumen opening in the ileum, which was 100 cm from the ileocecal valve. The blind end was observed 6 cm away from one opening along the depth direction, and an ulcer was observed on the intestinal wall of the blind segment, which was considered as an ileal duplication. The patient thereafter underwent surgery subsequently. Postoperative pathology confirmed ileal duplication and that gastrointestinal bleeding was primarily caused by ulcer hemorrhage. The patient had no discomfort after the follow-up. In this report, we have reviewed and summarized the literature to provide references for both diagnosis and treatment of ileal duplication.
... It can be sited on either the mesenteric or the antimesenteric side of the bowel wall, with a mesenteric preponderance, [1] and its length ranges from few centimeters to whole length of the small intestine. [17] Most of the time, it is single, while concurrent multiple cysts are exposed in 10% to 15% of cases. It appears unilocular mostly, but sometimes could be multilocular, or even multilobulated. ...
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... Intralesional calcifications, bony destruction of the coccyx and sacrum, and hypoattenuating fat within the lesions are features of teratomas and were not seen in our case. 22,24,27,28,[31][32][33] Developmental cysts are generally benign but do have the potential to undergo premalignant and malignant transformations. The presence of adenocarcinoma within a completely isolated duplication cyst has been reported. ...
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Duplications of the small bowel are an uncommon group of congenital malformations whose symptoms rarely occur in adults. We report the case of a 21 year-old man who suffered during four years from symptoms related to the ulceration of ectopic gastric mucosa in an ileal duplication (spontaneously resolving peritonitis, pelvic abscess, recurrent gastrointestinal bleeding and chronic abdominal pain). Radiological and endoscopic explorations and a first laparotomy failed to provide the diagnosis. 99m pertechnetate (99mTcO4) scintigraphy demonstrated ectopic gastric mucosa and guided the radiological detection of an ileal ulceration. Injection of 99mTcO4 during a second laparotomy located the ectopic gastric mucosa at the level of a tumefaction found on the mesenteric side of the terminal ileum. Duplication was only recognized at pathological examination of the ileal resection. This case emphasizes the need to evoke a duplication in clinical circumstances like those described in this patient as well as the usefulness of 99mTcO4 in the diagnosis of such a malformation.
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A case is described of a 19-month-old boy who presented with melaena. Intestinal duplication was diagnosed by 99Tcm-pertechnetate scanning and was confirmed at operation 2 years later, when the child was admitted with intestinal perforation. The technique is a reliable, non-invasive method for diagnosing ectopic gastric mucosa and is recommended in all cases of unexplained rectal bleeding in children.
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Alimentary tract duplications are uncommon anomalies that usually present in childhood. They are most common in the ileum but can occur anywhere along the alimentary tract. In this report, we describe an adult with an ileal duplication and review 12 other reported cases of ileal duplication in adults. Abdominal pain, nausea and/or vomiting, weight loss, and hematochezia were the most common symptoms. No patient was correctly diagnosed before operation. Three patients had malignant neoplasm in the resected specimens. Resection of the duplication and adjacent bowel is recommended because of the common blood supply to both, the possibility of malignant degeneration, and the risk of gastrointestinal ulceration and hemorrhage from ectopic gastric mucosa.