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 Table of Contents  
CASE REPORT
Year : 2018  |  Volume : 6  |  Issue : 3  |  Page : 100-103

Two different presentations of intestinal duplication cyst in pediatric age group


1 Intern, College of Medicine and Surgery, Umm Al-Qura University, Makkah, Saudi Arabia
2 Department of Pediatric Surgery, King Abdulaziz Medical City, Jeddah, Saudi Arabia
3 Department of Pediatric Surgery, King Faisal Specialist Hospital and Research Center, Jeddah, Saudi Arabia
4 Department of Pediatric Surgery, Maternity and Children Hospital, Makkah, Saudi Arabia

Date of Web Publication10-Sep-2018

Correspondence Address:
Dr. Ameera Almatrfi
Department of Pediatric Surgery, King Abdulaziz Medical City, Jeddah
Saudi Arabia
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/ssj.ssj_72_17

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  Abstract 

Intestinal duplication cyst (IDC) is a rare congenital anomaly where there is an abnormal portion of intestine attached to or intrinsic with the normal bowel. This condition was first reported in 1733 by Calderin followed by Fitz in 1884 and finally popularized by Ladd in 1937. This study described two cases with different presentations of IDC. The first one was a 6-month-old female presented with bleeding per rectum for 2 days. The second one was a 7-year-old boy presented with constipation for 3 years and abdominal swelling in the last 3 months. Both cases were investigated clinically and radiologically and showed different findings. Laparotomy was performed for both cases and the diagnosis of IDC was made, and the management was achieved by resection with primary anastomosis for both cases. The cyst was located at jejunoileal in the first case, and in the second case, it was found in the distal descending and sigmoid colon – both were communicating and tubular type.

Keywords: Distal descending, intestinal duplication cyst, jejunoileal, sigmoid colon, tubular duplication


How to cite this article:
Neazy A, Almatrfi A, Alharbi W, Abokrecha A. Two different presentations of intestinal duplication cyst in pediatric age group. Saudi Surg J 2018;6:100-3

How to cite this URL:
Neazy A, Almatrfi A, Alharbi W, Abokrecha A. Two different presentations of intestinal duplication cyst in pediatric age group. Saudi Surg J [serial online] 2018 [cited 2021 Jul 30];6:100-3. Available from: https://www.saudisurgj.org/text.asp?2018/6/3/100/240914


  Introduction Top


Intestinal duplication cyst (IDC) is a rare congenital anomaly where there is an abnormal portion of intestine attached to or intrinsic with the normal bowel and can involve any part of gastrointestinal (GI) tract from the mouth to the anus. This condition had incidence of two or three cases per year in pediatric referral centers with estimated incidence thought to be 1 in 4500 births which represent its rarity, with slight male predominance. This anomaly most commonly affects the small bowel with around 44%. IDC can be misdiagnosed as bowel intussusception or Meckel's diverticulum.


  Case Reports Top


Case 1

A 6-month-old female child presented with bleeding per rectum for 2 days before admission. She was pallor for the last 2 months and she had one episode of dark-colored stool 3 months back. Stool characteristic was semi-solid, mixed with light red-to-dark-colored blood. Unremarkable medical history revealed no obvious associated anomalies.

On examination, the patient's weight was 6 kg, pale looking, and lying comfortably in the bed; her vital signs included pulse 175 beats/min, respiratory rate 40 breaths/min, blood pressure 85/45 mmHg, and temperature 37°C. Locally, the abdomen was soft and lax with no tenderness, and per-rectum examination showed blood-stained stool.

Laboratory investigations: Hemoglobin: 3.4, Hematocrit: 11.9, prothrombin time: 11.5, partial thromboplastin time: 37.7, international normalized ratio: 1.01.

The managment sarted immediately with intravenous fluids and blood transfusion. Patient was investigated with abdominal X-ray [Figure 1] and ultrasound abdomen which was unremarkable, followed by Meckel's scan which revealed the presence of ectopic gastric mucosa in abnormal convoluted tubular structure in the left lower part of the abdomen and the impression of the Radiologist was: query Meckel's diverticulum or Small bowel duplication. The patient was planned for assisted laparoscopic exploration, which revealed communicated jejunoileal duplication cyst. Resection of the segment which is 30 cm was done with primary anastomosis [Figure 2]. Postoperative period was uneventful, feeding resumed 4th day post operatively.
Figure 1: Abdominal X-ray

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Figure 2: Jejunoileal duplication cyst resected segment

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Case 2

A 7-year-old Pakistani boy presented with constipation for 3 years and abdominal swelling in the last 3 months. Habitual constipation treated with frequent enemas along with laxatives, with some response to medications. The constipation was sufficient to cause significant abdominal discomfort and fullness. Medical history is not significant with no associated anomalies. On examination, the patient was conscious, cooperative with average body weight. His vital signs included pulse 93 beats/min, temperature 36.9°C, respiratory rate 18 breaths/min, and blood pressure 110/65 mmHg. Locally, the abdomen was soft lax with mild distension at the lower abdomen, with no tenderness, palpable well-defined mass about 15–20 cm, extending from the right iliac fossa to the left lumber region, firm, not tender with well-defined borders, lobulated, indentation of surface on palpation, and mobile. Per-rectum examination: Unremarkable.

The management started with plain abdominal X-ray control film which revealed soft-tissue mass in lower abdomen and pelvis. Contrast enema revealed non distended rectum or sigmoid colon and no out pouching of the descending colon [Figure 3]. CT scan abdomen and pelvis without contrast revealed the same finding of contrast enema. We diagnosed the patient as a case of tubular duplication of the colon with proximal communication with normal bowel. Exploratory laparotomy revealed duplication of the distal descending and sigmoid colon, so the resection of the duplicated colon with end-to-end anastomosis using circular stapler was done [Figure 4]. Smooth postoperative course with no complications and the patient was discharged home on the 6th postoperative day.
Figure 3: Contrast enema

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Figure 4: Duplication of the distal descending and sigmoid colon resected part

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  Discussion Top


IDC is a rare congenital anomaly where there is an abnormal portion of intestine attached to or intrinsic with the normal bowel and can involve any part of GI tract from the mouth to the anus.[1],[2] This condition may be associated with other abnormalities, such as complete colonic duplication, gastric diverticulum, and neurenteric cysts. The most common cell type lining this condition is gastric mucosa, accounting for 50% of cases followed by intestinal, pancreatic, or respiratory epithelium.[3],[4],[5] This condition was first reported in 1733 by Calder followed by Fitz in 1884 and finally popularized by Ladd in 1937 as a tubular or spherical shaped anomaly that is attached or adherent to the normal alimentary tract and share the similar phenotypic characteristics with the three following properties: (a) the cyst is surrounded by smooth muscle, (b) the cyst must contain the GI system mucosa from which it takes its own origin, and (c) the cyst must have a wall in common with the anatomic region in which it is found.[6],[7] This condition had incidence of two or three cases per year in pediatric referral centers with estimated incidence thought to be 1 in 4500 births which represent its rarity, with slight male predominance.[8],[9] This anomaly most commonly affects the small bowel with around 44%. The percentages were reported in the literature regarding different site as following commonly distal ileum 30% followed by esophagus, colon 5%–15%, duodenum 10%, jejunum 8%, stomach 8%, and rectum 4%–5% of all duplication anomalies. They found either on the mesenteric margin or on the contralateral side.[10] There are two types of intestinal duplication cyst (IDC) in general they can be cystic which accounts of 80% of the cases, they are spherical in shape and not communicated with the bowel lumen or tubular which accounts for 20% of the cases and communicated directly with bowel lumen.[11] Li et al. classified small IDC depending on the blood supply into two types: (a) parallel (Type I) defined as duplication is more toward mesentery, having separate blood supply from native bowel. (b) intramesentric (Type II) defined as duplication is cantered in mesentery having vessels from both side of mesentery [12],[13],[14],[15] This condition is usually present in the first 2 years of life, with an 80% in child <2 years, with different presentations according to the size, shape, and its location along the GI tract. Approximately two-thirds of all intestinal duplications are discovered within the first 2 years of life, with one-third identified in the newborn period.[16] Although many of the duplications are diagnosed incidentally, most patients present with a combination of pain and/or obstructive symptoms. Clinically, intestinal duplication cyst (IDC) may present as an asymptomatic, especially in adults or it can present as occlusive symptoms (volvulus, intussception), the classical presentation seen in children is abdominal pain, abdominal mass, bright red blood per rectum due to ulceration of ectopic gastric mucosa and less commonly it is present with intussception, volvulus and intestinal obstruction. Complication could occur rarely like bleeding into the cyst, volvulus, cyst torsion, cystic rupture, infection of the cyst, urinary or biliary obstruction, or malignancy (3% sarcoma, lymphangiosarcoma) may arise.[17] IDC can be misdiagnosed as bowel intussusception or Meckel's diverticulum and differential diagnosis included in this condition involved mesenteric cyst, omental cyst, ovarian cyst, and pancreatic pseudocyst.[1] The etiology is unknown, but multiple and various theories exist regarding this condition which include the following: (a) abortive twinning theory, (b) split notochord theory, and (c) intrauterine vascular accident theory, but the most accepted explanation is that duplication of the gut occurs due to pinching off of a diverticulum during embryological development.[18] This condition can be suspected prenatally by sonographic demonstration of an intra-abdominal cystic mass. Sonographic findings suggestive of an intestinal origin of the cyst are [1] peristaltic muscular contractions of the cyst wall,[2] double-layered wall, and [3] close contact with the mesenteric border.[9] However, in adults, multiple diagnostic tools are reported to be useful in the investigation of IDC including abdominal ultrasonography, GI endoscopy, and contrast-enhanced CT scan.[19] Ultrasound sonography is an important tool and the most widely used for the diagnosis intestinal duplication cyst (IDC) will be seen as hypoechoic outer muscular layer with an echogenic internal mucosal layer, this was termed as “Muscular rim sign” in addition to barium studies. CT and magnetic resonance imaging scans are considered less necessary.[20] The literature suggested Technetium-99 m pretechnetate scintigraphy to be the first test to be done in order to diagnose IDC.[10] The treatment of choice for enteric duplication cysts is complete surgical excision with reanastomosis even if the cysts are found incidentally.[11] The resection of the adjacent normal bowel wall is required due to the potential complications such as malignant changes, ulceration, and hemorrhage due to ectopic gastric mucosa. However, complication as short bowel syndrome can occur with resection of large tubular duplication cyst, so mucosal stripping offers alternative surgical option in these cases, eliminating the possibility of subsequent peptic ulceration or carcinogenesis.[20],[21],[22] While rare, malignancies arising within duplication cysts typically portend a poor prognosis, as most are diagnosed at advanced stages with widespread metastases. Reported cases have included squamous cell carcinoma, adenocarcinoma, and carcinoid tumors. Twenty-three percent of IDC in adults were found to be ileal cancer.[23] The cyst must be adherent to some part of the gastrointestinal tract, contain smooth muscle in the wall, and have an internal lining of alimentary epithelium, which is not necessarily that of the adjacent segment of the gastrointestinal tract. Ectopic tissue, such as gastric, squamous, transitional, and ciliated mucosa, and pancreas, can be found in these lesions at all levels of the GI tract.[16]


  Conclusion Top


IDC presented with different signs and symptoms and should be considered in differential diagnosis of pediatric surgical emergencies.

Declaration of patient consent

The authors certify that they have obtained all appropriate patient consent forms. In the form the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.

 
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    Figures

  [Figure 1], [Figure 2], [Figure 3], [Figure 4]



 

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