M.M.Shahin-Ul-Islam
Peutz-Jeghers syndrome(PJS) is an autosomal dominant syndrome characterized by multiple hamartomatous polyps in the gastrointestinal tract, mucocutaneous pigmentation and an increased risk of gastrointestinal cancer. Polyps are found throughout the gastrointestinaltract but commonly in the small bowel (60-90%), colon (50-64%), stomach (15-30%) and presents with gastrointestinal bleeding, anaemia, abdominal pain due to intussusceptions, obstruction of small intestine or infarction. PJS as a cause of gastric outlet obstruction is rare.A young female of 13 years presented with recurrent attack of abdominal pain, vomiting with significant weight loss for last 4 years. Pain was severe, colicky, in epigastric, periumbilical region aggravated after taking food, relieved with vomiting (spontaneous and induced), vomitous contained 2-3 previous days undigested food. She lost 8 kg weight in last 1 year. She underwent appendicectomy 3 years back. There was no history of fever, jaundice, haematemesis, melaena, alteration of bowel habit. She was moderately anaemic, nonicteric, BMI 15 kg/m2with black pigmentation in lower lip, mucous membrane, at finger tips and sole of foot(figure:1) with an ill-defined lump in epigastric region. Other systemic examination revealed normal. Her haemoglobin was 9 gm/dl, ESR, CRP, LFT, creatinine, CXR were normal with
