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Small Bowel Intussusception due to Solitary Peutz–Jeghers Jejunal Polyp: A Rare Entity

Peutz–Jeghers syndrome (PJS) is characterized by mucocutaneous pigmentation with multiple hamartomatous polyps of the gastrointestinal tract and/or a familial history with autosomal dominant inheritance pattern having incomplete penetrance, with some cases arising from spontaneous mutations. We pres...

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Detalles Bibliográficos
Autores principales: Kamath, Bharat, Doddamallappa, Samprathi, Roy, Arpit, Dhobale, Sudhir
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Wolters Kluwer - Medknow 2023
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC10305953/
https://www.ncbi.nlm.nih.gov/pubmed/37389396
http://dx.doi.org/10.4103/jiaps.jiaps_160_22
Descripción
Sumario:Peutz–Jeghers syndrome (PJS) is characterized by mucocutaneous pigmentation with multiple hamartomatous polyps of the gastrointestinal tract and/or a familial history with autosomal dominant inheritance pattern having incomplete penetrance, with some cases arising from spontaneous mutations. We present this case of a 12-year-old female who presented with jejunojejunal intussusception and on exploration revealed a polypoidal mass around 50 cm from duodenojejunal flexure acting as a lead point. A segmental jejunal resection with anastomosis was performed and histopathology confirmed a solitary Peutz–Jeghers (PJ) hamartomatous polyp. She neither had mucocutaneous pigmentation nor family history of PJS or any other polyps anywhere in the gut on further evaluation by endoscopies. A solitary PJ polyp in the jejunum is a rare entity, and to the best of our knowledge, only around 13 cases have been reported in world literature so far. Regular follow-up is essential in young children, so that future manifestations of the PJS are not missed.