Kloakalne malformacije u djece

master's thesis

• Title (english): Cloacal malformations in children
• Author: Mia Lorencin
• Mentor: Tomislav Luetić (mentor)
• Committee member: Ruža Grizelj (predsjednik povjerenstva)
• Committee member: Tomislav Meštrović (član povjerenstva)
• Committee member: Tomislav Luetić (član povjerenstva)
• Granter: University of Zagreb; School of Medicine; (Department of Surgery); Zagreb
• Defense date and country: 2017-07-14, Croatia
• Scientific / art field, discipline and subdiscipline: BIOMEDICINE AND HEALTHCARE; Clinical Medical Sciences; Surgery
• Scientific / art field, discipline and subdiscipline: BIOMEDICINE AND HEALTHCARE; Clinical Medical Sciences; Urology

Abstract

Kloakalne malformacije dio su širokog spektra anorektalnih malformacija. Kongenitalna kloaka i kloakalna ekstrofija predstavljaju najsloženije kongenitalne malformacije urogenitalnog i probavnog sustava. Najnovija istraživanja odbacuju teorije o aktivnoj septaciji kloake spuštanjem urogenitalnog septuma i pružaju dokaze o pasivnoj podjeli kloake procesom transformacije embrija. Kongenitalna kloaka u užem smislu predstavlja spoj urinarnog, genitalnog i probavnog sustava i javlja se samo u djevojčica. Dijagnoza se postavlja nalazom jednog otvora na perineumu kroz koji se drenira zajednički izvodni kanal svih triju sustava, a potvrđuje se slikovnom dijagnostikom i endoskopijom. O duljini zajedničkog izvodnog kanala ovisi način rekonstrukcije, kao i funkcionalna prognoza. Inicijalno liječenje novorođenčeta s kloakalnom malformacijom omogućuje drenažu mokraćnog sustava, hidrokolposa i drenažu debelog crijeva rasteretnom kolostomom. Za definitivnu rekonstrukciju kloakalne malformacije potreban je složen kirurški zahvat i iskusan tim dječjih kirurga. Najčešće se koristi posteriorni sagitalni pristup s totalnom urogenitalnom mobilizacijom. Ishod operacije ovisi o složenosti malformacije, no minucioznom rekonstrukcijom i dobrom postoperativnom njegom funkcionalni i estetski rezultat može biti odličan. Do 50% pacijenata postiže urinarnu kontinenciju, voljno pražnjenje crijeva i sposobnost reprodukcije. Sve pacijente dugoročno mora pratiti multidisciplinarni tim.

Abstract (english)

Cloacal malformations are a part of a large spectrum of anorectal malformations. Congenital cloaca and cloacal exstrophy represent one of the most complex malformations of the urinary, genital and gastrointestinal system. Latest research dismisses the theories of active septation of the cloaca by the descent of the urogenital septum, and provides evidence of passive division through a process called transformation. Congenital cloaca is defined as the confluence of the urethra, vagina, and rectum into a single common channel, and it occurs only in females. The diagnosis is a clinical one, made by identifying a single perineal orifice, and confirmed by endoscopy and imaging technology. The length of the common channel has to be measured, as it will determine the approach to reconstruction. The initial treatment of a neonate with a cloacal malformation includes drainage of the urinary tract / hydrocolpos, and bowel diversion by an ostomy. The definitive reconstruction of a cloacal malformation is a complex surgical procedure that needs to be done by an experienced pediatric surgical team. The most common procedure is the posterior sagittal approach with total urogenital mobilization. The cosmetic and functional result can be excellent, depending on the degree of the initial malformation. Meticulous anatomic reconstruction and comprehensive postoperative care results in as many as 50% of patients having voluntary bowel movements, urinary control, and preserved reproductive capacity. All patients must be followed on a long-term basis by a multidisciplinary team.

• Keywords: kloaka; kloakalna ekstrofija; embriologija; kongenitalna malformacija; rekonstruktivna kirurgija
• Keywords (english): cloaca; cloacal exstrophy; embryology; congenital malformation; reconstructive surgery
• Language: croatian
• URN:NBN: urn:nbn:hr:105:146830
• Study programme: Title: Medicine; Study programme type: university; Study level: integrated undergraduate and graduate; Academic / professional title: doktor/doktorica medicine (doktor/doktorica medicine)
• Type of resource: Text
• File origin: Born digital
• Access conditions: Open access
• Created on: 2017-10-23 10:51:46