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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...
Autores principales: | , , , |
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Formato: | Online Artículo Texto |
Lenguaje: | English |
Publicado: |
Wolters Kluwer - Medknow
2023
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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. |
format | Online Article Text |
id | pubmed-10305953 |
institution | National Center for Biotechnology Information |
language | English |
publishDate | 2023 |
publisher | Wolters Kluwer - Medknow |
record_format | MEDLINE/PubMed |
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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