RARE ENTITY OF TWO-POINT INTUSSUSCEPTIONS IN AN ADOLESCENT WITH PEUTZ-JEGHERS SYNDROME.
Jien Yen SOH, Iqtidaar OARIS , Siti Rahmah HASHIM ISA MERICAN, Zaidi ZAKARIA.
Department of Surgery, Hospital Universiti Sains Malaysia and School of Medical Sciences, Universiti Sains Malaysia, 16150 Kubang Kerian, Kelantan, Malaysia.
Peutz-Jeghers syndrome (PJS) is a rare autosomal dominant disorder characterized by hamartomatous polyposis and mucocutaneous pigmentation. We report a case of a 16-year-old adolescent with chronic abdominal pain and anemia who subsequently developed sudden severe abdominal pain. Clinical examination showed intestinal obstruction, and ultrasound suggested intussusception. He underwent immediate exploratory laparotomy. Intraoperatively, there were two points of jejunal intussusception with intraluminal polyps. Segmental bowel resection was performed with primary anastomosis. The histological examination came back as Peutz-Jegher polyps. We discuss difficulties in diagnosing PJS during the initial presentation, and the diagnosis was only made after the complication occurred. Clinical suspicion with an earlier endoscopic investigation can provide preventive intervention to avoid complication. In the case of intussusception with PJS, a complete assessment of the entire length of the bowel is necessary intraoperatively as there might be additional pathology or additional points of intussusception.
Keywords: Adolescent, Anemia, Intussusception, Peutz-Jegher Syndrome, Polyp, Surgery.
Correspondence: Siti Rahmah Hashim Isa Merican, Master of Medicine (MMED) Surgery USM, Hospital Universiti Sains Malaysia, 16150 Kubang Kerian, Kelantan, Malaysia.
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Brunei Int Med J.2021;17:149-152