Volume 24, Issue 105, September - October, 2020

Report of Peutz-Jeghers syndrome in two sisters with intussusception at the age of 10 and 12 year old

Mohammad Solaiman Mohammad Alnoaiji^1♦, Asmaa Salah Ghmaird², Ahmad Khaled Aleliwi³, Tahani Nasser M Alrashidi⁴

¹Consultant Pediatric Surgeon and head division of pediatric surgery department at King Salman Armed Forces Hospital, Tabuk, Saudi Arabia
²Assistant professor pediatric department at the University of Tabuk, Tabuk, Saudi Arabia
³Consultant Pediatric Surgeon at King Salman Armed Forces Hospital, Tabuk, Saudi Arabia
⁴MBBS, University of Tabuk, Tabuk, Saudi Arabia

^♦Corresponding author
Consultant Pediatric Surgeon and head division of pediatric surgery department, King Salman Armed Forces Hospital, Tabuk, Saudi Arabia; Email: drmsnoaiji@gmail.com

ABSTRACT

Peutz-Jeghers syndrome (PJS) is an inherited autosomal dominant condition that is characterized by various hamartomatous polyps in the gastrointestinal tract (GIT) and hyperpigmentation of the skin and mucous membranes. PJS increased risk for developing intestinal malignancy. Cases presentation: we report two sisters with intussusception at the age of 10 and 12 year old presented to the emergency department (ED) with features of intestinal obstruction. After thoroughly investigating, they diagnosed intussusception. Intraoperative was found intestinal polyps and by history, intraoperative findings, and histopathology, they confirmed diagnosis of PJS. These cases are reported because of the rarity of PJS and rarity of intussusception occurrence at 10 and 12 years. Conclusions: PJS is a rare familial disease. Early detection, treatment, and closely monitored can improve the prognosis of PJS and prevent complications related to polyps. PJS should be included as a differential diagnosis if intussusception happens at an atypical age.

Keywords: Peutz-jeghers syndrome; Intussusception; Hyperpigmentation; hamartomatous polyps; Intestinal malignancy

Medical Science, 2020, 24(105), 3077-3082

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