ISSN: 2754-8880
Published 00 11 0000
OPEN ACCESS
Edited by
A.Hussain
Submitted 05 Oct. 2024
Accepted 27 Nov. 2024
Citation
L Lee, A Faidzal, N
Ramli.Ileal Duplication: A
Rare Entity Uncovered
through Clinical Vigilance -
A Case Study Editorial
article:BJOSS::2025:(5);158-
161
Ileal Duplication: A Rare Entity Uncovered
through Clinical Vigilance - A Case Study
L Lee
1
, A Faidzal
1
, and N Ramli
2
1
Department of Surgery, Kulliyyah of Medicine, International Islamic University Malaysia, Jalan
Sultan Ahmad Shah, 25200 Kuantan, Pahang.
2
Department of Surgery, Hospital Pakar Sultanah Fatimah, Muar.
*
Correspondence author : Lian wei Lee.Email: llwei_0701@hotmail.com
O R IGI N A L
Abstract
Ileal duplication is a rare condition seen which is a rare congenital anatomical abnormality of
alimentary tract. It has an incidence of 1 in 4500 births. This disease can exhibit diverse mani-
festation but the exact etiologies still unclear. Hereby, we reported a 5-year-old boy who was
mistakenly diagnosed with perforated appendicitis and he presented with 5 days history of
right sided abdominal pain with gastrointestinal losses symptoms. He was scheduled for open
appendicectomy with Lanz incision. Intra-operatively, noted gangrenous ileal duplication with
blinded endand he underwent right hemicolectomy. Post-operatively, he is improving well he
was successfully discharged from ward. Histopathology report showed gangrenous tubular small
bowel duplication with extensive area of haemorrhagic necrosis. Gastrointestinal duplication
cysts are uncommon congenital anomalies, 80% of which are diagnosed before 2-years of age.
Clinical presentation depend upon the location, type, size and mucosal lining of the duplication.
Most of the duplications are diagnosed incidentally. The treatment of choice is complete surgical
excision with anastomosis. The resection of adjacent bowel is often recommended due to poten-
tial complications like ulceration, hemorrhage and malignant changes. Ileal duplication presented
with diverse manifestations which can misleading diagnosis and management. More accurate
diagnostic tools are needed to improve our care with early intervention.
Key messages
What is already known on this topic: Ileal duplication is a rare condition frequently encountered
in children, often presenting similarly to appendicitis.
What this study adds: The pre-operative diagnosis of duplicated cysts remains challenging for
pediatric surgeons. The optimal surgical approach—whether simple cyst excision is sufficient
or if a more invasive procedure like right hemicolectomy is necessary to minimize the risk of
reoperation and ensure adequate margins for potential malignancy—is still not standardized.
How this study might affect research, practice, and policy: Based on the findings, right hemicolec-
tomy is recommended due to the uncertainty surrounding the disease. Standardized diagnostic
tools should be implemented to improve patient care and establish optimal treatment protocols.
Introduction
Ileal duplication, a rare congenital anomaly of the alimentary tract, occurs with an incidence of 1 in
4500 births, showing a slight male predominance.(
1
;
2
;
3
) This condition manifests in various ways,
such as bleeding, perforation, and intestinal obstruction, although its precise etiology remains
elusive.(
4
) Despite its rarity, it poses significant diagnostic challenges, as highlighted in the case of
a 5-year-old boy initially misdiagnosed with perforated appendicitis. The complexities of accurate
diagnosis underscore the importance of a thorough understanding of gastrointestinal anomalies,
particularly in pediatric patients, where prompt and precise identification can greatly impact
treatment outcomes and prevent potential complications. Further research into the underlying
mechanisms of ileal duplication is warranted to enhance diagnostic accuracy and inform optimal
management strategies for affected individuals.
Case presentation
We present the case of a 5-year-old boy who presented with a 5-day history of right-sided
abdominal pain accompanied by symptoms of gastrointestinal losses. On clinical examination,
he exhibited signs of sepsis with tenderness over the right side of his abdomen. Laboratory
investigations revealed elevated white blood cell count and serum lactate levels. Consequently,
the decision was made to perform an open appendicectomy with a Lanz incision. Intraoperatively,
gangrenous ileal duplication with a blind end was noted. Following consultation with a pediatric
surgeon, it was determined that a right hemicolectomy was necessary. The patient showed
significant improvement postoperatively and was discharged from the ward on the sixth day
following surgery. Histopathological analysis revealed gangrenous tubular small bowel duplication
with an extensive area of hemorrhagic necrosis. This case underscores the importance of prompt
diagnosis and appropriate surgical intervention in the management of gastrointestinal duplications,
particularly in pediatric patients presenting with acute abdominal symptoms.
Figure 1. Gangrenous duplicated ileal with blinded-end
Figure 2. Presence of 3 lumens within the caecum
159/161
Discussion
Gastrointestinal duplication cysts, though uncommon, represent congenital anomalies typically
diagnosed before the age of two.(
4
) The hallmark of diagnosis lies in the presence of a well-
developed smooth muscle coat and mucosal lining within some segment of the alimentary tract,
often contiguous to any part of it. According to a comprehensive study by Pulgandla et al., the
ileum emerges as the most prevalent site for such duplications, comprising 33.4% of cases, while
colonic, rectal, and pyloroduodenal locations remain exceedingly rare.(4; 5; 6; 7; 2)
Clinical presentations vary depending on factors such as location, type, size, and mucosal lining of
the duplication. Symptoms often include abdominal pain, nausea, vomiting, bleeding, distension,
the presence of an abdominal mass, obstruction, and intussusceptions. Notably, some duplication
cysts can remain asymptomatic until adulthood.(
4
;
7
;
2
) The diagnostic challenge is compounded
by the fact that many duplications are incidentally discovered, while others present with a
combination of pain and obstructive symptoms, often mimicking acute appendicitis, as seen in
the case above.
Preoperative diagnosis remains challenging and often imprecise. Although various imaging modal-
ities such as barium studies, ultrasound, and CT scans are employed, ultrasound is particularly
useful in neonates for identifying the origins of abdominal masses. Antenatal scans play a vital role,
with approximately 30% detection rate of duplication cysts, enabling early intervention to mitigate
potential complications. CT scans offer detailed anatomical insights, while MRI and endoscopic
ultrasonography serve as additional diagnostic tools, although recent research suggests that
ultrasound and CT scans may suffice for preoperative diagnosis.(4; 6; 8)
Upon confirmation of the diagnosis and determination of precise anatomical positioning, urgent
surgical intervention may be warranted due to the risk of complications such as ulceration,
hemorrhage, or bowel perforation from ectopic mucosal secretions.(
4
;
9
) The decision to operate
on asymptomatic patients remains controversial, considering the unknown risk of malignant
transformation and potential complications of bowel resection, such as short bowel syndrome or
anastomotic leak.(9)
Complete surgical excision with anastomosis is the treatment of choice, often necessitating
resection of adjacent bowel to mitigate potential complications like ulceration, hemorrhage, or
malignant changes.(
10
;
8
;
2
;
11
) Histopathological examination is crucial for definitive diagnosis
and ruling out other close differentials, ensuring optimal management and patient outcomes.(3)
Conclusion
Ileal duplication presents a rare pathology with diverse manifestations, often leading to diagnostic
and management challenges. The varied clinical presentations can confound accurate diagnosis
and appropriate management strategies. Early surgical intervention is imperative for patients
presenting with complications such as bleeding, perforation, or obstruction, as prompt treatment
can mitigate potential adverse outcomes. However, the complexity of diagnosing ileal duplication
underscores the need for more accurate diagnostic tools to enhance patient care through timely
intervention. Advancements in diagnostic imaging modalities and surgical techniques hold promise
for improving the detection and management of ileal duplication, ultimately optimizing patient
outcomes and reducing the risk of complications.
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