Skip Navigation LinksHome > February 2013 - Volume 56 - Issue 2 > Peutz-Jegher Syndrome in Childhood: Need for Updated Recomm...
Journal of Pediatric Gastroenterology & Nutrition:
doi: 10.1097/MPG.0b013e318271643c
Original Articles: Gastroenterology

Peutz-Jegher Syndrome in Childhood: Need for Updated Recommendations?

Goldstein, Stephanie A.*; Hoffenberg, Edward J.

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Background and Aims: We reviewed our institution's experience with Peutz-Jegher syndrome (PJS) in children to determine whether current recommendations on timing of screening and follow-up should be modified.

Methods: We reviewed the charts of all of the children with a diagnosis of PJS at our institution from 2000 to 2011 abstracting data on intussusceptions events, polyp characteristics, Sertoli cell (SC) tumors, family history, imaging, and interventions.

Results: Of 14 children identified, 10 were boys. Median age at first clinical evaluation was 4.5 years, and family history and/or mucocutaneous pigmentation were the 2 most common factors stimulating screening. Median age at first screening test was 5 years (range 1–16), and at first polyp identification, 5 years (range 1 to 18). There were 7 intussusception events in 5 children, with median age of 10 and range 5 to 16 for first event. Two boys had SC tumors at 8 and 11 years. Polyps were identified during initial screening in 9 of 14 patients. Polyps were found in the stomach or duodenum in 5 (36%), small bowel in 7, (50%) and colon in 3 (21%) children. Large polyps were identified in 9 children at median age of 7 years.

Conclusions: Polyps causing significant clinical consequences can occur frequently in children with PJS younger than 8 years. Revised guidelines should consider initial screening at age 4 to 5 with capsule endoscopy and upper and lower endoscopy as well as evaluation for SC tumors and re-evaluation whenever symptoms suggest polyp-associated complications.

Copyright 2013 by ESPGHAN and NASPGHAN


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