PER RECTAL ENDOSCOPIC MYOTOMY FOR HIRSHPRUNG'S DISEASE IN RESCUE OF UNSUCCESSFULL PREVIOUS SURGICAL MYOMECTOMY
Dervis J. Bandres*1,3, Carlos E. Prada2, Jose R. Soto4, Marielvis Davila5, Gloria Muñoz2, Maria V. Bandres6, Adriana Peña7, Miguel A. Gonzalez8, Cesar Louis9, FreddyFreddy E. Gamboa2
1Medicine, Centro Medico Docente La Trinidad, Caracas, Miranda, Venezuela, Bolivarian Republic of; 2Pediatric Surgery, Centro Medico docente la Trinidad, Caracas, Miranda, Venezuela, Bolivarian Republic of; 3Gastroenterology, Centro Medico Docente La Trinidad, Caracas, Miranda, Venezuela, Bolivarian Republic of; 4Gastroenterology, Centro Medico Docente La Trinidad, Caracas, Miranda, Venezuela, Bolivarian Republic of; 5Gastroenterology, Centro Medico Docente La Trinidad, Caracas, Miranda, Venezuela, Bolivarian Republic of; 6Medical School, Universidad Central de Venezuela, Caracas, DM, Venezuela, Bolivarian Republic of; 7Pediatric Surgery, Centro Medico Docente La Trinidad, Caracas, Miranda, Venezuela, Bolivarian Republic of; 8Pediatric Surgery, Centro Medico Docente La Trinidad, Caracas, Miranda, Venezuela, Bolivarian Republic of; 9Gastroenterology, Centro Medico Docente La Trinidad, Caracas, Miranda, Venezuela, Bolivarian Republic of
Introduction: Hirschsprung's disease (HD) is an anomaly that is characterized by the absence of ganglionar cells in the myenteric and submucosal plexuses in different parts of the intestine. It generally affects a short segment of the rectosigmoid, and there also have been reported ultra short or long variants. The standard treatment consists in surgical and laparoscopic procedures, but on June 2016 the first per rectal endoscopic myotomy was described as treatment of a short segment of HD in an adult. Clinical case: We present the first successful per rectal endoscopic myotomy (PREM) reported in Latin America. The patient is a female of 9 years old who was diagnosed with HD at 4 years of age, time in which she underwent a myomectomy procedure. Nonetheless due to the persistence of clinical symptoms such as abdominal distension and defecation every 5-7 days (only by making use of laxatives), she came to our health center where we suggested the realization of a PREM which she underwent under general anesthesia in lithotomy position, the rectoscopy was done with a gastroscope that revealed a stenotic ring at 3cm from the pectineal line, at 1,5cm proximal to the pectineal line, in the posterior wall the submucosa was elevated with 0,9% saline solution, manitol and methylene blue; the pediatric surgeon did a transverse incision in the mucosal and a 9,8mm endoscope subsequently a submucosal tunnel was created dissecting the submucosa moving the scope in a rotational motion and inyecting the solution, allowing to reach 5cm proximal to the stenotic area, an incision was made in the muscularis propria with a needle knife and then a longitudinal myotomy was done with an IT Knife and Endocut whilst retrieving the scope until the anal verge of the submucosal tunnel, haemostasis with Coagrasper and the incision was stitched. The patient's clinical outcome has been successful during 8 months of follow-up. Conclusion: The PREM is a therapeutic option in patients with HD; it requires a long-term follow-up to determine the effectiveness of the therapy.
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