Conclusion:
Although PJ polyps are unusual in children, they should be considered as
a potential pathological lead points in case of recurrent jejunal
intussusceptions, especially in presence of characteristic dark
muco-cutaneous macula, or familial history. Besides the risk of serious
gastro-intestinal polyp-related complications, PJS is associated to an
increased risk of cancers. Early identification of this condition is
then important for symptomatic or at risk patients, as it permits to
establish a strict and regular systemic surveillance program, which can
considerably improve quality of life and prognosis of these individuals.