V05-03 LAPAROSCOPIC NEPHRON-SPARING TREATMENT OF FRALEY’S SYNDROME IN A PAEDIATRIC PATIENT

You have accessJournal of UrologyPediatrics (V05)1 May 2024V05-03 LAPAROSCOPIC NEPHRON-SPARING TREATMENT OF FRALEY'S SYNDROME IN A PAEDIATRIC PATIENT Javier Ruiz, Juan Pablo Corbetta, Santiago Weller, Felicitas Lopez Imizcoz, Luciana Diaz Zabala, Cristian Sager, Carol Burek, Nicolas Rosiere, Yesica Gomez, and Otilia Blain Javier RuizJavier Ruiz , Juan Pablo CorbettaJuan Pablo Corbetta , Santiago WellerSantiago Weller , Felicitas Lopez ImizcozFelicitas Lopez Imizcoz , Luciana Diaz ZabalaLuciana Diaz Zabala , Cristian SagerCristian Sager , Carol BurekCarol Burek , Nicolas RosiereNicolas Rosiere , Yesica GomezYesica Gomez , and Otilia BlainOtilia Blain View All Author Informationhttps://doi.org/10.1097/01.JU.0001009516.84627.21.03AboutPDF ToolsAdd to favoritesDownload CitationsTrack CitationsPermissionsReprints ShareFacebookLinked InTwitterEmail Abstract INTRODUCTION AND OBJECTIVE: Fraley's syndrome is a rare cause of extrinsic compression of the upper pole infundibulum secondary to a renal vascular variant. Surgical treatment with nephron-sparing procedures is indicated only in symptomatic cases and only few cases have been published in the literature. The aim of this video is to illustrate in a step-by-step fashion the diagnosis and minimally invasive management of this rare entity in a paediatric patient. METHODS: We present a 4-year-old boy with a 6-month history of recurrent left flank pain and isolated upper calix dilation on ultrasound. Nuclear Scan demonstrated tracer retention in the left upper pole and CT scan showed anterior and posterior branches of the apical renal artery. Patient was positioned in a Galdakao-modified supine Valdivia position. A retrograde pyelogram confirmed an obstructed and long upper pole infundibulum. A transperitoneal laparoscopic caliconeopyelostomy was performed ventrally to the upper pole vessels. RESULTS: Operative time was 90 minutes. Patient was discharge after 48 h. Double-J ureteral stent was removed after 1 month. There were no complications with reduction of the upper pole dilation on ultrasound and resolution of symptoms during follow-up. CONCLUSIONS: Fraley's syndrome is rare cause of upper pole obstruction and should be suspected in the presence of recurrent flank pain, haematuria, or urinary tract infection with an isolated upper calix dilatation on images. Laparoscopic nephron-sparing surgery is a feasible option and should be the first line of treatment in paediatric patients. Source of Funding: None © 2024 by American Urological Association Education and Research, Inc.FiguresReferencesRelatedDetails Volume 211Issue 5SMay 2024Page: e268 Advertisement Copyright & Permissions© 2024 by American Urological Association Education and Research, Inc.Metrics Author Information Javier Ruiz More articles by this author Juan Pablo Corbetta More articles by this author Santiago Weller More articles by this author Felicitas Lopez Imizcoz More articles by this author Luciana Diaz Zabala More articles by this author Cristian Sager More articles by this author Carol Burek More articles by this author Nicolas Rosiere More articles by this author Yesica Gomez More articles by this author Otilia Blain More articles by this author Expand All Advertisement PDF downloadLoading ...