|Year : 2015 | Volume
| Issue : 4 | Page : 1031
Duodenoduodenal intussusception: Report of three challenging cases with literature review
Dinesh Pradhan1, Neeraj Kaur2, Birinder Nagi3
1 Department of Pathology, University of Pittsburgh Medical Center, Pittsburgh, Pennsylvania, India
2 Department of Radiodiagnosis and Imaging, Post Graduate Institute of Medical Education and Research, Chandigarh, India
3 Department of Gastroenterology, Post Graduate Institute of Medical Education and Research, Chandigarh, India
|Date of Web Publication||15-Feb-2016|
660 Maryland Avenue, Pittsburgh, Pennsylvania
Source of Support: None, Conflict of Interest: None
Small bowel intussusception is an uncommon condition with cases of duodenoduodenal intussusception (DDI) being exceptionally rare. Adult intussusception occurs infrequently and differs from childhood intussusception in its presentation, etiology, and treatment. DDI is very unusual due to the fixed position of the duodenum within the retroperitoneum. The lead point usually is hamartomatous polyp, adenoma, or adenocarcinoma. Only few cases of DDI in adults have been reported in the literature. We herein report a series of three cases of DDI encountered in a tertiary level research institute. All cases had underlying abnormality acting as lead point with different etiologies. DDI is a challenging condition due to its rarity and nonspecific presentation and should be considered in the differential diagnosis of gastric outlet obstruction, pancreatitis, and obstructive jaundice. We elaborate this condition with a detailed review of the literature to gain a better understanding of its clinical features and enable early diagnosis.
Keywords: Adenocarcinoma, duodenoduodenal intussusceptions, gastric outlet obstruction, intestinal adenocarcinoma, villous adenoma, tubulovillous adenoma
|How to cite this article:|
Pradhan D, Kaur N, Nagi B. Duodenoduodenal intussusception: Report of three challenging cases with literature review. J Can Res Ther 2015;11:1031
| > Introduction|| |
Intussusception is a condition in which the full thickness of the bowel wall is telescoped into the distal bowel. This condition is uncommon in adults and accounts for less than 0.1% of all adult hospital admissions.  Duodenal intussusception is an extremely rare entity as duodenum assumes a fixed, retroperitoneal position during embryological intestinal rotation and duodenal tumors leading to intussusception are rare.  Hence, intussusception of this organ into itself is extremely unusual. Duodenal intussusception was first described by Sunderlin in 1830 and only 48 cases have been reported till date. Out of these 48 cases of duodenal intussusceptions reported, most of the cases are gastroduodenal or distal duodenojejunal intussusceptions. Duodenoduodenal intussusception (DDI) as such is very rare. Diagnosis of DDI is usually delayed because of its longstanding, intermittent, and nonspecific symptoms and most cases are diagnosed at emergency laparotomy.  We hereby present three cases of DDI in adult patients with a detailed literature review.
| > Case reports|| |
A 16-year-old female patient presented with complaints of vomiting and abdominal pain for last 3-4 years which had aggravated over the last 2 months. Vomiting occurred within 1-2 h of food intake; was non-bilious and projectile. Pain was relieved by vomiting. There was significant weight loss of 15 kg in 3 months along with loss of appetite. Patient had no significant past history of intestinal obstruction or surgical intervention. On examination, patient was emaciated with presence of palpable mass and visible peristalsis in epigastric region. Clinical diagnosis of duodenal stricture was suggested. Abdominal ultrasonography (USG) showed target appearance in right hypochondrium with intussusception extending upto right lumbar region [Figure 1]a and b]. Contrast-enhanced computed tomography (CECT) with neutral oral contrast showed bowel within bowel appearance in proximal duodenum along with 4 × 3 × 3 cm enhancing mass lesion within the intussuceptum [Figure 1]c and d]. Barium examination [Figure 2] showed filling defect in duodenum with pressure effects on duodenum. During laparotomy, stomach was hugely dilated along with dilatation of first and second part of duodenum. DDI was found in which the distalduodenum was acting as the intussuceptum and the proximal duodenum as the intussuscepiens. The intussusception was manually reduced. After successful reduction of the intussusception, a sessile polypoidal mass measuring 5 × 3 cm was found in the posterior wall of second part of the duodenum, 3 cm distal to ampulla. No polyps were seen in the rest of small and large bowel. Polypoidal mass resection along with duodenotomy was done and end-to-end duodenoduodenal anastomosis was performed. The specimens were sent for histopathological examination.
|Figure 1: Sonography images through upper abdomen (axial and longitudinal sections) showing duodenoduodenal intussusception with presence of: (a) "Target sign" (straight arrow) and (b) "sausage sign" (curved arrow), respectively. Contrast-enhanced computed tomography of abdomen showing target appearance with gastric outlet obstruction: (c) There was presence of 4 × 3 × 3 cm enhancing mass in proximal duodenum (white straight arrow); and (d) presence of intervening fat plane (white curved arrow) helped in differentiating intussusception from simple mucosal prolapse associated with tumor|
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|Figure 2: Barium enteroclysis showing polypoidal filling defect with coiled spring appearance (white arrow) in the proximal duodenum|
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Gross examination of the specimen confirmed intussusception along with presence of polypoidal mass lesion in the second part of duodenum. Rest of the bowel was unremarkable. Histopathological examination of the mass revealed features of villous adenoma with areas of high grade dysplasia [Figure 3]. There was no evidence of stalk infiltration. Postoperative period was uneventful and patient was discharged on 15 th postoperative day after she could comfortably take solids. At the 4-month follow-up, she was doing well.
|Figure 3: Photomicrograph of the polypoidal mass revealing villous arrangement of the dysplastic epithelium indicating villous adenoma (hematoxylin and eosin (H and E), ×40)|
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A 59-year-old postmenopausal, hypertensive, and diabetic female, on treatment for last 10 years presented with pain epigastrium for last 1.5 years. Pain was mild to moderate in intensity, intermittent, and colicky. Pain was associated with loss of weight and appetite for last 6 months. On examination, she was pale and emaciated. Barium examination showed delayed passage of contrast across second and third part of duodenum with presence of a polypoidal filling defect in second (D2) and third (D3) part of duodenum. There was also foreshortening of medial wall of D2 and D3 with a longitudinal fold. USG abdomen showed heterogeneous mass in relation to distal D2 and proximal D3 with target appearance. CECT abdomen confirmed DDI along with a heterogeneously enhancing mass in distal D2 and proximal D3 causing telescoping of terminal D2 into D3 segment and giving target appearance. Patient was taken up for surgery. On surgical exploration, the DDI was confirmed along with presence of a mass lesion measuring 5.2 × 4.3 cm. In addition, enlarged lymph nodes were seen in the periduodenal area. The specimens were sent for histopathological examination. Radical gastrojejunostomy was done, followed by chemotherapy.
Gross examination revealed intussusception with presence of friable polypoidal mass lesion. Histological sections from mass lesion revealed features of moderately differentiated adenocarcinoma involving the muscularis and reaching the serosal aspect, stage IIB. Lymph nodes sampled were free from tumor involvement. Patient was disease free on 2 years follow-up.
A 60-year-old male presented in 2005 to liver clinic with long history of progressively increasing jaundice, abdominal discomfort, early satiety, and abdominal distension along with postprandial vomiting for 2 months. On physical examination, patient was icteric with features of cholestatic jaundice. Abdominal examination revealed 7 × 5 × 4 cm palpable mass in epigastrium (in midline and slightly to left) with visible peristalsis. USG showed typical target appearance with findings suggestive of extrahepatic biliary obstruction and pancreatic duct dilatation. Barium studies confirmed intussusception with foreshortening in medial wall of duodenum and large polypoidal filling defect in D3 and fourth (D4) part of duodenum. Patient underwent CECT abdomen which confirmed obstruction of biliary and pancreatic ductal system with D2 intussuscepting into D3 and D4. An enhancing mass lesion was seen acting as lead point. Patient underwent duodenal resection with gastrojejunal anastomosis. Gross examination revealed presence of polypoidal mass lesion measuring 4 × 2 cm. Histopathological sections demonstrated features of tubulovillous adenoma. No dysplastic changes were present.
| > Discussion|| |
Intussusception is defined as invagination of one loop of bowel into the lumen of an adjacent loop, the proximal invaginating segment being called intussuceptum which telescopes into the lumen of the distal segment or intussuscipiens.  It is a common cause of intestinal obstruction in children. Intussusception in adults differs from those in children in various aspects.  In adults, 90% occur in the small or large bowel and, the remaining 10% involve the stomach or a surgically created stoma.  However, DDI is an extremely rare entity as duodenum is a fixed retroperitoneal structure. The exact mechanism of DDI is unknown; however, it is thought that any lesion in theduodenal wall or irritant within the lumen that alters normal peristaltic activity is able to initiate an invagination. The lead points for the intussusception are attributable to benign, malignant, or idiopathic causes. 
The clinical manifestations of DDI are usually nonspecific and commonly presents with obstructive features which may be acute, chronic, or intermittent. Patients may also present with weight loss, fever, and palpable mass. In cases of intussusception involving ampullary region, obstruction of common bile duct and pancreatic duct may occur and patient may present with features of obstructive jaundice or acute/chronic pancreatitis.  DDI tends to occur more frequently in adult female patients. The age of presentation ranges from 10 months to 73 years, with a median of 38 years. The patients in present case were 16-60-year-old with male to female ratio of 1:2.
The diagnostic modalities used most often are USG, upper gastrointestinal series (UGI), CECT, and endoscopy (ES). Radiographs usually are nonspecific, but sonography may show typical "target sign or doughnut sign". CECT is currently considered as the most sensitive radiologic method to make a preoperative diagnosis. It reveals characteristic "bowel within bowel appearance" and "target sign" which consist of outer intussuscipiens, inner intussuceptum, and central fat density formed by the intussuscepted mesenteric fat and mesenteric vessels.  In our study, computed tomography was carried out in all the three patients and it was diagnostic in all the three patients. In addition, in all the three cases, the original lead points were also established on CECT.
According to Lvoff et al.,  and Mateen et al.,  the intussusceptions length less than 3.5 cm is a useful CT quantitative indicator of nonsurgical enteroenteric intussusceptions, which may reduce spontaneously. Sandrasegaran et al.,  suggested that proximal small-bowel lesions were likely to be transient and nonobstructive and were unlikely to have a significant lead point. This is in contrary to what was seen in our series where all the three cases of DDI presented with obstructive symptoms and all required surgical intervention. The choice of the procedure is determined by the size and the site of the lesion, and the presence of complications. However, adult cases of intussusception are usually treated surgically or endoscopically, but never by fluoroscopic reduction as are common in pediatric population. The patients in the present case series underwent open surgery followed by resection and anastomosis. Diagnosing intussusception is important as there are high risk of complications in intussusception such as bowel ischemia, bowel obstruction, and intraluminal bleeding requiring rapid treatment. Diagnosis is usually missed or delayed because of nonspecific and often chronic or recurrent symptoms. Hence, clinicians and surgeons should be suspicious of intussusception if they encounter a clinical picture suggesting intestinal obstruction, gastric outlet obstruction, duodenal stricture, pancreatitis, or obstructive jaundice. The imaging findings may help clinch the diagnosis for better management of these patients.
| > References|| |
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[Figure 1], [Figure 2], [Figure 3]