Cargando…
Sporadic Peutz–Jeghers syndrome: a rare cause of intussusception in a toddler with no medical history
Peutz–Jeghers syndrome (PJS) is an unusual hamartomatous polyposis of the gastrointestinal tract associated with melanocytic mucocutaneous hyperpigmentation. This research paper examines the case of an 18-month-old Syrian female who had been diagnosed with intussusception. The patient underwent lapa...
Autores principales: | , , , , |
---|---|
Formato: | Online Artículo Texto |
Lenguaje: | English |
Publicado: |
Oxford University Press
2019
|
Materias: | |
Acceso en línea: | https://www.ncbi.nlm.nih.gov/pmc/articles/PMC6600125/ https://www.ncbi.nlm.nih.gov/pubmed/31281661 http://dx.doi.org/10.1093/omcr/omz051 |
Sumario: | Peutz–Jeghers syndrome (PJS) is an unusual hamartomatous polyposis of the gastrointestinal tract associated with melanocytic mucocutaneous hyperpigmentation. This research paper examines the case of an 18-month-old Syrian female who had been diagnosed with intussusception. The patient underwent laparotomy, and multiple small bowel polyps were found to act as the lead point. For this reason, small bowel resection (~15 cm), with end-to-end anastomosis, were performed. Although PJS diagnosis was histopathologically confirmed, the patient had no pigmented lesions on the face, the lower lip or the buccal mucosa and neither had any history of hospitalization or family history of the disease. This case was examined and is reported in the present study because PJS is rarely present at this early age when significant medical history is lacking. |
---|