Tratamiento quirúrgico conservador en un paciente con invaginación yeyuno-yeyunal secundaria a síndrome de Peutz-Jeghers. Reporte de un caso

INTRODUCCION: el sindrome de Peutz-Jeghers es una enfermedad autosomica dominante, poco frecuente, caracterizada por hiperpigmentacion mucocutanea y polipos gastrointestinales; estos ultimos causantes de obstrucciones intestinales recurrentes, secundarias a invaginacion. PRESENTACION DEL CASO: adolescente femenina, de 13 anos, que acudio a urgencias debido a un cuadro de dolor abdominal intenso, con un cuadro de obstruccion intestinal secundario a invaginacion yeyuno-yeyunal. Siete anos antes tuvo un cuadro clinico similar que desaparecio espontaneamente. A la exploracion fisica se observaron lesiones hiperpigmentadas en el labio inferior y anemia microcitica e hipocromica con los que se integro el diagnostico de sindrome de Peutz-Jeghers. Debido a que en la tomografia axial computada de abdomen se evidencio invaginacion intestinal se realizo una laparotomia exploradora, con el hallazgo de dos polipos hamartomatosos de 2 y 3 cm de diametro a 15 cm del ligamento de Treitz. Se trato la invaginacion intestinal y, posteriormente, se efectuaron enterotomia y polipectomia. Mediante enteroscopia transoperatoria se descartaron otras alteraciones. Luego de tratar el cuadro clinico la paciente fue dada de alta del hospital en buenas condiciones. CONCLUSION: la invaginacion intestinal en adolescentes es una situacion excepcional que requiere un alto indice de sospecha para enfermedades poliposicas. Se reporta un caso de invaginacion intestinal que se trato existosamente con cirugia conservadora, sin necesidad de resecciones intestinales que, a largo plazo, condicionan sindrome de intestino corto: uno de los principales problemas a los que se enfrentan esto pacientes. PALABRAS CLAVE: Peutz-Jeghers, polipos gastrointestinales, obstruccion intestinal recurrente, dolor abdominal agudo, yeyuno-yeyunal, adolescentes. Abstract INTRODUCTION: Peutz-Jeghers syndrome is an uncommon autosomal dominant disease, characterized by mucocutaneous hyperpigmentation and gastrointestinal polyps, which are the cause of recurrent intestinal obstructions, secondary to invagination. This case provides evidence to consider more conservative surgical treatments and prevent these patients from being constantly subjected to extensive bowel resections, which in many cases, can condition short bowel disease. CASE PRESENTATION: A 13-year-old female patient who came to the emergency room with acute abdominal pain due to intestinal obstruction secondary to jejuno-jejunal intussusception. 7 years ago, the patient reported similar clinical manifestations that solved spontaneously. Physical examination showed hyperpigmented lesions on the lower lip, and microcytic hypochromic anemia; Peutz-Jeghers syndrome was established. Intestinal intussusception was evidenced by computed axial tomography of the abdomen, so exploratory laparotomy was performed, with the finding of two hamartomatous polyps 2 and 3 cm in diameter at 15 cm of the Treitz ligament. Intestinal intussusception was resolved with subsequently, enterotomy and polypectomy. Other alterations were ruled out by trans-operative enteroscopy. At the resolution of clinical manifestations, the patient was discharged from the hospital in good clinical conditions. CONCLUSION: Intestinal intussusception in adolescents is an exceptional situation, which requires a high index of suspicion for polysposis diseases. We present a case of intestinal intussusception, successfully resolved with conservative surgical treatment, without requiring intestinal resections, which can condition short bowel syndrome in the long-term, one of the major problems these patients face. KEYWORDS: Peutz-Jeghers; gastrontestinal polyps; recurrent intestinal obstructions; acute abdominal pain; jejuno jejunal; adolescents.