peutz - jeghers is a rare autosomal dominant disorder characterized by hamartomatous polyps and discoloration of mucosal membranes .
the polyps can occur anywhere in the gastrointestinal tract and can grow large enough to cause bowel obstructions .
a 16-year - old male presented to the emergency department with signs and symptoms of an acute bowel obstruction .
he had a previous history of a colonoscopy with polypectomy at age 4 , and hyperpigmentation of his mucous membranes .
an exploratory laparoscopy found an intussusception of the mid jejunum . a laparoscopic - assisted small bowel resection was performed .
colonoscopy and upper endoscopy revealed 5 more polyps in the stomach and colon that were removed .
the treatment of obstruction in these patients is to remove the offending hamartomatous polyp(s ) .
the rest of the intestine needs to be examined and those polyps found should be removed .
peutz - jeghers syndrome ( pjs ) is a rare autosomal dominant condition characterized by hamartomatous polyps and mucocutaneous pigmentation of the lips , buccal mucosa , and digits .
polyps can occur anywhere in the gastrointestinal tract and can grow large enough to cause bowel obstructions .
a 16-year - old male presented to the emergency department with signs and symptoms of an acute bowel obstruction .
he had a previous history of a colonoscopy with polypectomy at age 4 , had hyperpigmentation of his mucous membranes , and his mother and maternal grandfather had a history of gastrointestinal polyps .
the patient underwent an exploratory laparoscopy and was found to have an intussusception of the mid jejunum ( figure 1 ) .
pathology showed a 5-cm polyp that had acted as a lead point for the intussusception ( figure 2 ) .
the patient had an uncomplicated postoperative course and was discharged home on postoperative day 3 .
he is one year out from surgery and has resumed his normal activities with no evidence of recurrence .
pjs was first described in 1921 by peutz and subsequently elaborated upon by jeghers in 1949 .
germline defects in the tumor suppressor gene serine / threonine kinase 11 ( stk11 ) are implicated in this rare autosomal dominant inherited disease .
its incidence is calculated in 1 in 200,000 liveborns , and its mean age of onset is 25.2 years .
the most common location of the hamartomatous polyps is the small bowel ( 78% ) , followed by the colon ( 42% ) , stomach ( 38% ) , and rectum ( 28% ) .
these polyps can cause obstruction in up to 43% of cases and rectal bleeding in up to 14% of patients .
the syndrome is associated with a 2% to 10% increased risk of cancer of the intestinal tract , from the stomach to the rectum .
there is also an increased risk of extraintestinal malignancies , including cancer of the breast , ovary , cervix , fallopian tubes , thyroid , lung , gallbladder , bile ducts , pancreas , and testes .
however , recurrence of intussusception episodes occurs in at least 10% of cases , resulting in repeated surgical intervention .
thus , the rest of the intestine needs to be examined , and those polyps found should be removed .
once the gastrointestinal tract has been cleared of polyps , the recommended interval of small bowel follow - through is from 2 years to 3 years .
the presence of polyps larger than 1.5 cm in diameter mandates another complete gastrointestinal evaluation with endoscopic removal of polyps .
patients should also be screened periodically for malignancies of the breast , cervix , ovary , testis , stomach , and pancreas . to our knowledge
, there are at least 2 other published case reports regarding the laparoscopic management of bowel obstructions in pjs . the ideal way to remove a pedunculated polyp acting as a lead point
, laparoscopy offers a safe and effective method for surgical management with reduction of the intussusception and small bowel resection .
the diagnosis of pjs should be considered in patients presenting with a clinical picture of bowel obstruction and mucocutaneous hyperpigmentation .
if the diagnosis is made preoperatively , optimal management should include laparoscopic treatment of the bowel obstruction and intraoperative enteroscopy .
if the diagnosis is made after the operation , the patient needs complete evaluation of their gastrointestinal tract .
 introduction : peutz - jeghers is a rare autosomal dominant disorder characterized by hamartomatous polyps and discoloration of mucosal membranes . 
 the polyps can occur anywhere in the gastrointestinal tract and can grow large enough to cause bowel obstructions.case report : a 16-year - old male presented to the emergency department with signs and symptoms of an acute bowel obstruction . 
 he had 2 days of abdominal pain , obstipation , and vomiting . 
 he had a previous history of a colonoscopy with polypectomy at age 4 , and hyperpigmentation of his mucous membranes.results:computed tomographic ( ct ) scan revealed an intussusception of the small intestine . 
 an exploratory laparoscopy found an intussusception of the mid jejunum . a laparoscopic - assisted small bowel resection was performed . 
 pathology showed a 5-cm polyp that acted as a lead point for the intussusception . 
 colonoscopy and upper endoscopy revealed 5 more polyps in the stomach and colon that were removed.conclusion:small bowel obstructions can be managed successfully with minimally invasive approaches . 
 the treatment of obstruction in these patients is to remove the offending hamartomatous polyp(s ) . 
 the rest of the intestine needs to be examined and those polyps found should be removed . 
 this can be done intraoperatively with laparoscopic - assisted enteroscopy and colonoscopy . 