Hamartomatous Polyps - A rare cause of adult intussusception

Intussusception is defined as telescoping of a segment of the gastrointestinal tract into an adjacent one. It is the leading cause of intestinal obstruction and is one of the commonest causes of abdominal emergency in children [1]. Adult intussusception however is rare with an incidence of 2-3 cases per million per year. An identifiable lead point that often requires definitive treatment is found in 70-90% cases. The preoperative diagnosis can usually be made reliably by non-invasive imaging techniques like barium studies, US and CT. Small bowel intussusceptions in most cases are secondary to benign lesions. We describe a case of jejuno jejunal intussusception in a middle aged female secondary to a multiple polyps in the small & large bowel loops which was detected on CT.

  Case Submitted by Dr. Chetan (DMRD, DNB resident), Dr. Karunakaran N. (consultant), Department of imaging & interventional radiology, Meenakshi Mission Hospital & Research Centre, Madurai 

Case Details  : A 37 year old female presented with complaints of intermittent pain in the epigastric and periumbilical region with significant weight loss for one year with bleeding PR. The physical examination was unremarkable. In Ultrasound abdomen was suggestive of intussusception. Computed tomography of the abdomen showed intussusception with typical target appearance on cross section & sausage shape mass on saggital imaging with a well-defined rounded  polypoid  hypodense mass with smooth margins  at the distal end of intussusceptum.  The diagnosis of enteric intussusception due to pedunculated polyp was made. Polyps were also noted in colon on virtual colonoscopy.

Intraoperative findings confirmed radiographic findings.  Multiple jejunojejunal intussusceptions were noted with a 2 cm intraluminal pedunculated polyp acting as the lead point. Colonic polyps along the entire colon were also noted. Jejunal resection and anastomoses with subtotal colectomy & ileo rectal anastomosis was done. Histopathological examination of the resected mass suggested a hamartomatous polyps; PEUTZ JEGHER’S SYNDROME. The postoperative period was unremarkable.

Discussion : Childhood intussusception is idiopathic in 90% of cases. In contrast, adult intussusception has a demonstrable cause in 70-90% of cases including neoplasms, postoperative changes and adhesions, inflammation and miscellaneous conditions like Meckel's diverticulum and celiac disease [1],[3].

Benign lesions are responsible for approximately 25% of all adult intussusceptions with Peutz-Jeghers polyps and lipomas being common causes of small bowel intussusception (3). Malignant lesions of the small bowel like adenocarcinoma; malignant stromal tumors and metastases, most commonly from malignant melanoma are less common than benign neoplasms and are responsible for approximately 15% cases of small bowel intussusception [4]. Peutz-Jeghers polyps are a rare cause of enteric intussusception in adults. Peutz–Jegher’s syndrome, also known as hereditary intestinal polyposis syndrome, is an autosomal dominant genetic disease characterized by the development of benign hamartomatous polyps in the gastrointestinal tract and hyperpigmented macules on the lips and oral mucosa & surfaces of palm & soles as seen in our patient. Patient is having history of bowel resection of elder sister for unknown cause favouring the autosomal condition of disease.

The classical features of intussusception on plain film like air crescent sign and presence of a soft tissue mass with decreased colonic air are neither sensitive nor specific, however plain radiograph may be useful in assessing signs of bowel compromise like pneumatosis and pneumoperitoneum [3].

The characteristic finding of intussusception on barium studies is the "coiled spring" appearance with a thin central barium stream with or without a leading mass.

Colour Doppler may be useful in determining the degree of vascular compromise of the involved bowel segments [3] .The characteristic doughnut appearance was seen in the present case on transverse scan with a multilayered appearance on longitudinal scan. In addition the leading mass was well demonstrated by US.

On CT a complex small bowel mass is seen with fat and mesenteric vessels seen between the opposing walls of intussusceptum and intussuscipiens.[3], [6],[7],[8].  Abdominal CT has been shown to be the most accurate modality for indentification of intussusception [3],[6],[7].

Magnetic resonance imaging can distinguish between liquid or solid components and ischemic or necrotic bowel can be detected.

To conclude, intussusception in adults occurs rarely and differs from its paediatric counterpart in terms of aetiology, clinical presentation, diagnosis and treatment. Symptoms are usually non specific and of long duration and the condition is rarely suspected on clinical examination. A correct and timely diagnosis is necessary to prevent the complications of bowel infarction and perforation and to resect the underlying leading mass. Imaging thus plays a crucial role in the diagnosis and management of these patients.

Axial section CECT abdomen showing hypodense polyp at the leading point

Coronal CECT abdomen showing sausage shaped bowel mass with fat & mesenteric vessels between opposing walls of intussusceptum & intussucipiens

Polyp in the colon at the site other than intussusception on vitual colonoscopy

Axial section CECT abdomen: Target appearance of intussusception on axial section

Surgical specimen - colectomy done showing multiple polyps

Pigmentation in oral mucosa & sole favouring the diagnosis of Peutz Jegher’s syndome

 

 

   References

 

 

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