Background: Persistent urogenital sinus is a rare cloacal anomaly in which the urinary and genital tracts cannot be differentiated during embryonic development. However, the presence of concomitant recto-urogenital sinus or recto-vaginal fistula is much rarer. In the literature, only two cases with accompanying fistula have been presented so far. Case presentation: We present the diagnosis and treatment management of a persistent urogenital sinus case with recto-vaginal fistula. We also aimed to reveal the difference between our patient, who is the 3rd case in the literature, and the other two and the described cloacal anomalies. By performing total urogenital sinus mobilization and fistula repair, the three systems were separated from each other, and both a functional and cosmetic appearance was obtained. Conclusion: It should be kept in mind that there may be different variants other than the defined cloacal malformations. In patients with cloacal malformation, cystoscopy and rectoscopy should be performed to reveal the anatomy before reconstructive surgery in order to avoid any unexpected situation.

Intestinal malrotation is estimated to occur in approximately 1 in 500 live births, although symptomatic cases are considerably less common. Midgut volvulus is a potentially devastating complication of malrotation that requires urgent evaluation because it may rapidly progress to intestinal ischemia and bowel loss. However, plain radiographs may be misleading when proximal obstruction produces a double-bubble-like appearance. We report a term female neonate who developed bilious vomiting on the first postnatal day. Initial radiography suggested duodenal atresia. Clinical deterioration with leukocytosis, elevated C-reactive protein, and rising lactate prompted urgent laparotomy at 48 hours of life. Severe midgut volvulus with three complete turns and near-total small-bowel ischemia was identified. Detorsion and planned second-look laparotomy allowed bowel preservation and completion of the Ladd procedure. Persistent postoperative obstruction later revealed a 30-cm post-ischemic fibrotic stricture requiring resection. Approximately 80 cm of small bowel and the colon were preserved. Midgut volvulus should remain in the differential diagnosis of neonatal bilious vomiting, even when radiography suggests duodenal atresia.