What are the rare complications of Peutz-Jeghers syndrome (PJS)?

Updated: Oct 11, 2018
  • Author: Buu Anh T To, MD; Chief Editor: Praveen K Roy, MD, AGAF  more...
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Answer

Other rare reported complications include gastrointestinal obstruction and vomiting secondary to gastric polyps. [16, 63, 64] Extraintestinal polyps are also reported, although they are rare; they include nasal polyps, [65] gall bladder polyps, [66] ureteric polyps, [67] and respiratory tract polyps. [68]

The principal causes of morbidity in Peutz-Jeghers syndrome are owing to the intestinal location of the polyps (ie, small intestine, colon, stomach). Morbidity includes small intestinal obstruction and intussusception (43%), abdominal pain (23%), hematochezia (14%), and prolapse of a colonic polyp (7%) [69] ; these typically occur in the second and third decades of life.

Intestinal obstruction can occur in about 50% of patients, and it is usually localized in the small bowel. Obstruction can be complete or incomplete and is caused by the polyp itself or by the subsequent intussusception that may occur, as seen in the images below.

This barium enema radiograph reveals intussuscepti This barium enema radiograph reveals intussusception in the descending colon.
The computed tomography scan demonstrates the clas The computed tomography scan demonstrates the classic yin-yang sign of an intussusceptum inside an intussuscipiens.
Abdominal ultrasonography reveals the classic targ Abdominal ultrasonography reveals the classic target sign of an intussusceptum inside an intussuscipiens.
This postevacuation image is from part of a double This postevacuation image is from part of a double-contrast barium enema study in a 47-year-old man presenting with features of small-bowel obstruction. The image shows a coiled-spring appearance in the region of the cecum suggestive of an intussusception. At laparotomy, an ileocecal intussusception was found in association with a carcinoid tumor of the terminal ileum.

 

In a retrospective study that examined the incidence of sporadic Peutz-Jeghers syndrome polyps over a 22-year period, Burkhart et al reported that although sporadic Peutz-Jeghers syndrome polyps are very rare, individuals who may have even a single Peutz-Jeghers syndrome polyp may have an accumulative lifetime risk of cancer that is similar to those with the syndrome. [70]


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