RARE PEUTZ JEGHERS SYNDROME IN PEDIATRIC AGE: MERELY ON UPPER GI WITH ADENOMATOUS CHANGE

Ferdous Ara Begum*, Md Nazmul Hassan and Khan Lamia Nahid

ABSTRACT

Peutz-Jeghers Syndrome (PJS) is a rare, hereditary, and autosomal dominant disorder usually presented with mucocutaneous pigmentation and gastrointestinal (GI) polyps. GI polyps usually hamartomatous polyps located throughout the gastrointestinal tract and may be presented with bleeding and small bowel intussusception, potentially leading to the need for emergency surgery. We represent a case of Peutz-Jeghers syndrome in a 05-year-old female child who presented with abdominal pain, vomiting, and upper GI bleeding. This syndrome is a rare entity that required regular follow-up with screening for other malignancies due to increased chances of intestinal and extra-intestinal malignancy.

Keywords: Hamartomatous polyp, Melanoma cutaneous pigmentation, Peutz-Jeghers syndrome, Intestinal polyps, Child polyposis.