Pediatria pre prax 2/2024

Anorectal malformations – part 2: ARM in females

Anorectal malformations belong to frequent congenital disorders in children, in which there is absent anal opening or one that is in the wrong place. These anomalies are a spectrum of disorders involving anus, rectum and frequently the urinary and gynecologic system (1). Rectum ends higher, than normally and continues distally as a thin channel – fistula, which can open at the perineum or to the genitourinary tract, only rarely ends blindly and is without any fistula. In majority of patients ARM is diagnosed postnatally, while it is important to set the diagnose of the specific anorectal malformation and associated defects. Anorectal malformations are different in both sexes what is the result of anatomic differences in small pelvis in males and females. The most frequent anorectal malformations in girls are recto-vestibular and recto-perineal fistula. In ARM is important not just the specific type of malformation, but as well how high is rectum localised, the quality of sacrum and anomalies of spine, which are the predictors of prognosis and continence in patients with ARM. According to the findings from the diagnostics, the next therapeutic strategy and timing of the surgery is set up. Surgical options in patients with ARM include two main surgical procedures – primary anorectoplasty by a method PSARP = posterior sagital anorectoplasty (one step surgical procedure), or initial creation of a colostomy – duplex descendentostomy, followed by the next surgery – anorectoplasty and the third surgical procedure is colostomy closure. (three-step surgical procedure). In the case of complex anorectal malformation, for example a cloaca, there are usually more reconstructive surgical procedures. Current surgical trends are miniinvasive forms of anorectoplasty, in which by shorter cut in perineum and sparing of the sphincter we are improving the prognosis – voluntarily bowel movements in patients with ARM.

Keywords: anorectal malformations, rectum, perineum, anorectoplasty, voluntarily bowel movements