ABSTRACT
Peutz-Jeghers Syndrome (PJS) is an infrequent genetic disorder characterized by intestinal hamartomas and characteristic mucocutaneous pigmentation. It is frequently the cause of intususception, intentinal obstruction and/or intestinal bleeding. An 8 y.o. patient is described who presented with bowel obstruction and a history of familiar PJS polyposis. Exploratory laparotomy showed that the obstruction was due to a mass of sizable polyps which obstructed the intestinal lumen in the jejunum and colon. Surgical treatment consisted of double intestinal resection and termino-terminal anastomosis. This procedure resolved the acute intestinal obstruction and saved the patient's life.
El síndrome de Peutz-Jeghers (SPJ) es una enfermedad genética poco frecuente, caracterizada por la presencia de pólipos hamartomatosos intestinales y pigmentaciones mucocutáneas características. Con frecuencia es causal de intususcepción, obstrucción intestinal y/o hemorragias digestivas. Se describe el caso clínico de una paciente de 8 años que tenía el diagnóstico de poliposis familiar de Peutz Jeghers y que presentó obstrucción intestinal. En la laparotomía exploradora se constató que la obstrucción intestinal era secundaria a una masa de grandes pólipos sésiles, que obstruían el lumen intestinal en yeyuno y colon. El tratamiento quirúrgico consistió en doble resección intestinal y anastomosis termino-terminal. Este procedimiento quirúrgico constituyó una solución al cuadro agudo de obstrucción intestinal de la paciente, siendo de vital importancia para mantener su sobrevida.
Subject(s)
Humans , Female , Child , Intestinal Obstruction/surgery , Intestinal Obstruction/etiology , Peutz-Jeghers Syndrome/complications , Anastomosis, Surgical , Intestinal Polyposis/etiology , Treatment OutcomeSubject(s)
Humans , Male , Adolescent , Lip Diseases/diagnosis , Peutz-Jeghers Syndrome , Hyperpigmentation , Intestinal Polyposis/etiologyABSTRACT
Juvenile polyposis located solely on prolapsed rectal mucosa is very unusual. We report the case of a 17-year old boy who presented to us with a history of passage of blood and mucus per rectum of a mass protruding through the anus during defecation. Per rectal and colonoscopic examinations revealed numerous polyps located solely on the prolapsed rectal mucosa. Histopathology was consistent with juvenile polyposis. He was managed with repeated sessions of endoscopic polypectomy. Family screening was negative for colonic polyps.
Subject(s)
Adolescent , Humans , Intestinal Polyposis/etiology , Male , Rectal Prolapse/etiologyABSTRACT
A 13-year-old girl presented with features of intestinal obstruction. At surgery, the terminal 25 cm of ileum, which was resected along with the right colon, showed plexiform neurofibromatosis of the serosa and mesentery, hyperplastic submucosal and myenteric nerve plexuses and proliferation of neural tissue in the lamina propria, which manifested as diffuse polyposis of the ileal mucosa. The patient had a single inconspicuous external neurofibroma and a few café-au-lait spots on the back.