Abstract
Persistent cloaca involves fusion of the bladder, vagina, and rectum into a single duct called the common duct. Although its pathogenesis remains unclear, it has been associated with hyperchloremic metabolic acidosis. Herein, we present the case of a neonatal girl with high-confluence type variant of persistent cloaca treated with vesicostomy (Blocksom) for refractory metabolic acidosis. She was diagnosed with persistent cloaca before birth; colostomy was performed and a urinary catheter was placed in the bladder. Voiding cystourethrography on day 19 after birth showed that most of the contrast material leaked into the rectum; hence, the urinary catheter was removed. On day 27, hyperchloremic metabolic acidosis was detected and treatment with oral sodium bicarbonate was initiated; however, the infant showed no response. Because hyperchloremia occurred after removal of the urinary catheter, continuous urine retention in the colon through the common duct was believed to have caused the progression of hyperchloremic metabolic acidosis through transporters in the intestinal mucosa. As reinstallation of a urinary catheter was technically difficult, vesicostomy was performed on day 29, after which the metabolic acidosis improved. This report suggests vesicostomy as an effective treatment for refractory hyperchloremic metabolic acidosis associated with high-confluence type persistent cloaca.