British Journal of Radiology (2005) 78, 1047-1049
© 2005 British Institute of Radiology
doi: 10.1259/bjr/25000606
Colo-colonic intussusception caused by a solitary Peutz-Jeghers polyp
J L Jaremko, MD, PhD and
B Rawat, MD, FRCPC
Department of Radiology, 2nd Floor, University of Alberta Hospital, 8440 112 Street, Edmonton, Alberta, Canada, T6G 2B7
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Abstract
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A case of acute large bowel obstruction by colo-colonic intussusception in a healthy 19-year-old man is presented. The lead point of the intussusception is a rare solitary colonic Peutz-Jeghers polyp in the descending colon of a man without the full Peutz-Jeghers syndrome. The clinical presentation, imaging findings on plain radiographs, single contrast enema and CT, and findings at colonoscopy and surgery are correlated with pathology results and a brief review of the literature.
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Clinical presentation
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A previously healthy 19-year-old man presented to the emergency room with a 1 week history of bloody diarrhoea and increasing crampy abdominal pain. There was no recent travel, antibiotic use or weight loss, no prior surgery and no family history of bowel disease. On examination he had a tender distended abdomen without peritoneal signs, fever or adenopathy. Aside from mild acne, the patient's skin including the perioral area was unremarkable. Oral mucosa appeared normal. Bright red blood was present on an otherwise normal rectal examination. A complete blood count was normal.
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Imaging findings
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An initial plain film of the abdomen (Figure 1
) revealed faeces in the ascending colon, air in the distended transverse colon and an abrupt, convex cutoff just below the splenic flexure, with no air or stool beyond to the rectum.
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Figure 1. Initial radiograph demonstrates distended ascending and transverse colon, with a rounded soft tissue density in the proximal descending colon.
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Colonoscopy revealed a large intraluminal mass-like abnormality in the descending colon covered in blood clot, with hyperaemic mucosa on one side, suspicious for an intussusception. Unable to proceed beyond this point due to discomfort, the patient was referred for a single contrast enema. The enema demonstrated a large smooth filling defect occluding almost the entire lumen of the descending colon. A thin rim of contrast passed beyond the defect to the normal-appearing splenic flexure (Figure 2). A subsequent CT scan of the abdomen showed features typical of colo-colonic intussusception (Figure 3).
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Figure 2. (a) Lateral fluoroscopic spot view of a single-contrast enema shows a tortuous filling defect in the descending colon (arrow) with a thin rim of contrast passing upwards anteriorly. (b) Anteroposterior fluoroscopic spot view reveals thin crescents of contrast with a "whirled" appearance in the descending colon (arrow) with a normal appearing splenic flexure above.
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Figure 3. (a) Axial CT image augmented with intravenous contrast near the splenic flexure shows a "target" shape of colon within colon, with entrapped mesentery (thick arrow). A rim of contrast from the recent enema is also seen (thin arrow). (b) Axial CT image near the distal tip of the intussusceptum shows wall thickening and oedema (arrow).
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Treatment and outcome
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At surgery, the greater omentum was found to be telescoped into the colon causing partial obstruction and haemorrhage. Due to omental involvement up to the splenic flexure, a subtotal colectomy with ileosigmoid anastomosis was performed. A small dark hyperaemic mass was found at the lead point of the intussusception (Figure 4). This mass proved to be a solitary Peutz-Jeghers polyp without malignant features. The patient recovered uneventfully from surgery and remained well 6 months later. Consent to present the case was obtained from the patient and his mother.
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Figure 4. Gross pathology specimen shows typical appearance of a Peutz-Jeghers polyp at the lead point (arrow).
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Discussion
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Intussusception occurs when one loop of bowel (intussusceptum) telescopes into an adjacent segment (intussuscipiens). It is common in children, but rare in adults, representing just 1% of adult bowel obstructions [1]. Intussusception in an adult is often associated with a malignant lead point, in contrast to childhood intussusception which is often traced to a benign cause such as enlarged lymphoid tissue due to viral infection [2]. A 40 year institutional review at Massachusetts General Hospital found that 93% of their series of 58 cases of adult intussusception were due to an intestinal lesion, approximately half of those being malignant [3]. Colo-colonic intussusception in particular is strongly associated with malignancy, with nearly two-thirds having a malignant lead point [2]. Since the incidence of most bowel lesions increases with age, the mean age for adult intussusception is typically in the fifth to sixth decade [1, 3, 4].
Intussusception is less frequent in the colon than in the longer, more mobile small intestine. A comprehensive review (n=745) found that only 17% of adult intussusceptions were colo-colonic [5]. Recent smaller case series have found similar proportions: 14/58 were colonic at the Massachusetts General Hospital [3], while at the Mount Sinai Hospital, 5/27 were colonic, three of which were at the caecum and none were distal to the transverse colon [4]. The descending colon is a particularly uncommon site for intussusception, since it lies in a fixed retroperitoneal position.
The most common non-malignant lead point in colo-colonic intussusceptions is an adenomatous polyp, which is a pre-malignant lesion [2]. Peutz-Jeghers polyps, which are a common benign cause of intussusception in the small bowel, are a virtually unrecognized cause of colo-colonic intussusception. Our patient had a single hamartomatous polyp, a non-neoplastic developmental anomaly of the intestinal mucosa. The microscopic appearance was diagnostic of a Peutz-Jeghers polyp. Peutz-Jeghers syndrome is a rare autosomal dominant disorder consisting of multiple (at least two) benign hamartomatous polyps throughout the gastrointestinal tract, often with associated mucocutaneous pigment lesions (darkening of the skin and perioral mucosa) [6]. The polyps are benign, although patients with the syndrome are at increased risk of colon cancer, perhaps due to eventual dysplastic transformation of some polyps. To our knowledge, our patient had only a single Peutz-Jeghers polyp, not the full syndrome. No other polyps were visible in the sigmoid colon on colonoscopy or in the surgically resected ascending, transverse and descending colon. No pigmented perioral or facial lesions were observed, and the family history was negative. No investigations of the stomach or small bowel were performed to exclude other polyps. However, the absence of skin lesions and of a family history still precludes a diagnosis of Peutz-Jeghers syndrome in this patient [7].
Solitary Peutz-Jeghers polyps are extremely rare, with an estimated incidence of 1:120 000 [8]. In a recent series of eight patients with these polyps, five were found incidentally in the colon and the other three in the stomach or duodenum. No further polyps grew in an average of 11 years of follow-up per patient [7]. The authors concluded that solitary Peutz-Jeghers polyps are benign, but suggested that it would nevertheless be prudent to continue periodic surveillance in these patients. Other case reports describe these polyps in the duodenum [9, 10], the small bowel [11] and the rectum [12]. No intussusception was described in these cases.
Colo-colonic intussusception is an unusual cause of large bowel obstruction in a young adult, and is even more rarely associated with a benign lead point such as a solitary Peutz-Jeghers polyp. This case highlights a rare benign aetiology of intussusception at an uncommon site in the young adult. To our knowledge, no such case has been reported.
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Acknowledgments
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Thanks to Dr L Russell of the Department of Pathology and to Dr Drew Sutherland of the Department of Surgery, University of Alberta Hospital, for their assistance with this case.
Received for publication February 23, 2005.
Revision received April 7, 2005.
Accepted for publication April 22, 2005.
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References
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- Oncel M, Remzi FH, Church JM, Goldblum JR, Zutshi M, Fazio VW. Course and follow-up of solitary Peutz-Jeghers polyps: a case series. Int J Colorectal Dis 2003;18:33–5.[Medline]
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- Tanaka H, Iida M, Kohrogi N, Matsui T, Yasunami Y, Yao T, et al. Endoscopic removal of solitary hamartomatous polyps of the duodenum. Gastrointest Endosc 1990;36:640–2.
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- Hanna RM, Dahniya MH, Seddiq MA, Kamel H. Case report: a case of solitary Peutz-Jegher's hamartoma in the small bowel with angiographic evaluation. Br J Radiol 1994;67:897–9.[Abstract]
- Nakayama H, Fujii M, Kimura A, Kajihara H. A solitary Peutz-Jeghers-type hamartomatous polyp of the rectum: report of a case and review of the literature. Jpn J Clin Oncol 1996;26:273–6.[Abstract/Free Full Text]
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Abstract
Clinical presentation
