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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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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
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author Kamath, Bharat
Doddamallappa, Samprathi
Roy, Arpit
Dhobale, Sudhir
author_facet Kamath, Bharat
Doddamallappa, Samprathi
Roy, Arpit
Dhobale, Sudhir
author_sort Kamath, Bharat
collection PubMed
description 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.
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spelling pubmed-103059532023-06-29 Small Bowel Intussusception due to Solitary Peutz–Jeghers Jejunal Polyp: A Rare Entity Kamath, Bharat Doddamallappa, Samprathi Roy, Arpit Dhobale, Sudhir J Indian Assoc Pediatr Surg Case Report 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. Wolters Kluwer - Medknow 2023 2023-05-02 /pmc/articles/PMC10305953/ /pubmed/37389396 http://dx.doi.org/10.4103/jiaps.jiaps_160_22 Text en Copyright: © 2023 Journal of Indian Association of Pediatric Surgeons https://creativecommons.org/licenses/by-nc-sa/4.0/This is an open access journal, and articles are distributed under the terms of the Creative Commons Attribution-NonCommercial-ShareAlike 4.0 License, which allows others to remix, tweak, and build upon the work non-commercially, as long as appropriate credit is given and the new creations are licensed under the identical terms.
spellingShingle Case Report
Kamath, Bharat
Doddamallappa, Samprathi
Roy, Arpit
Dhobale, Sudhir
Small Bowel Intussusception due to Solitary Peutz–Jeghers Jejunal Polyp: A Rare Entity
title Small Bowel Intussusception due to Solitary Peutz–Jeghers Jejunal Polyp: A Rare Entity
title_full Small Bowel Intussusception due to Solitary Peutz–Jeghers Jejunal Polyp: A Rare Entity
title_fullStr Small Bowel Intussusception due to Solitary Peutz–Jeghers Jejunal Polyp: A Rare Entity
title_full_unstemmed Small Bowel Intussusception due to Solitary Peutz–Jeghers Jejunal Polyp: A Rare Entity
title_short Small Bowel Intussusception due to Solitary Peutz–Jeghers Jejunal Polyp: A Rare Entity
title_sort small bowel intussusception due to solitary peutz–jeghers jejunal polyp: a rare entity
topic Case Report
url 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
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