Case of the Month March 2014, page 9

Answer: c. This patient suffered from multiple periodical high-grade bowel obstructions due to intussuceptions caused by polp lead-points. Most of her obstructions were of the ileo-ileal variety. On this occasion she presented with ileocolic intussusception.

DISCUSSION:

Peutz-Jegers is syndrome characterized by hamartomatous small bowel polyps, mucocutaneous pigmentation, and melena. The majority (75%) of hamartomatous polyposis cases are non-hereditary. Hereditary hamartomatous polyposis goes by the name of Peutz-Jegers syndrome, and comprises the other 25%. It is autosomal dominant, usually involving a mutation of chromosome 19. In addition to the mentioned findings, patients are at an increased risk for cancer in the GI tract, pancreas, breast, and reproductive organs. Polyps occur from the stomach to rectum, sparing the mouth and esophagus. They appear as clusters of small well circumscribed filling defects on small bowel follow through examinations or CT examinations with oral contrast.

Intussusception is the term used to describe when one loop of bowel “telescopes” into another more distal loop of bowel during peristalsis. Transient self-limited ileoileal intussusceptions are not abnormal, and in fact on occasion transient intussusceptions are imaged in asymptomatic patients undergoing CT for other reasons. However, prolonged intussusception can lead to pain and obstructive symptoms. In adults, non-self-limited intussusception is almost always due to a pathologic lead point. Patients with Peutz-Jegers are at increased risk for intussusception due to the numerous polyps scattered within their enteric tracts. This patient suffered from a number of intussusceptions causing mechanical obstruction. On most occasions, with time and supportive care the intussusceptions resolved, but on many other occasions she required operative intervention to reduce the intussusception.

The appearance of intussuception on imaging has been described by various sign names, for example the “target sign,” “donut sign,” “bulls eye” or “coiled spring.” That is, when the proximal segment (the “intussusceptum”) invaginates into the distal segment (the “intussucipiens”), the stacked cylindrical appearance looks like a rounded concentric structure in cross-section much like a target, donut or spring. Intussusception is well demonstrated by CT, as well as fluoroscopy. Ultrasound evaluation is usually reserved for pediatric patients, as patient size limits visualization of bowel pathology in the adult. 95% of intussusception cases are seen in the pediatric population.

Peutz-Jegers patients suffer from frequent obstructions. In patients not carrying this syndrome, intussusception in the adult should raise alarm bells and malignant leadpoints should be excluded. Colon cancer, metastases and lymphoma can cause intussusception. Other benign causes for intussusception in the adult include leiomyoma and lipoma.


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