Complete rectal prolapse in a patient with Hirschsprung disease: a clinical case

The disease of Mya-Hirschsprung (HD) it's rare and congenital, usually diagnosed in child age, but that it can also remain unknown until the adult age. It's associated to genetic mutations and it can involve other pathologies and malformations. The variability of the anatomopathological and clinical phenomena is correlated to the length of the aganglionic segment. The Authors describe the clinical case of one young female patient, who presented chronic constipation (less than 3 evacuations to week), tenesm, meteorism, abdominal pain. To the inspection of the anal region after the evacuation the presence of complete rectal prolapse (3) cylinders of the length of 30 cm was appraised, with presence of ulcerations of the mucosa. The patient came subordinate to diagnostic study that they demonstrated the presence of HD. The Authors operated the patient with the technique of Frykman and Goldberg. In the follow-up the patient had the complete remission of the symptomatology and resumption of the rectoanal inhibitory reflex, remarkable diminished in the preoperative manometric examination. The pathogenesis of the association of HD and rectal prolapse goes searched in the presence of ultrashort HD (aganglionic defect of a limited segment of rectum), pathological variety somewhat rare that can determine subocclusive phenomena and that it favors the prolapse of the rectum for the continuous evacuating strains.