Peña A, Filmer B, Bonilla E, Mendez M, Stolar C
Long Island Jewish Medical Center, Schneider Children's Hospital, New Hyde Park, NY 11042.
J Pediatr Surg. 1992 Jun;27(6):681-5. doi: 10.1016/s0022-3468(05)80090-9.
The treatment of the urogenital sinus with normal rectum still represents a challenge. A perineal approach with or without a skin flap seems to be effective for those patients with a low implantation of the vagina. However, in patients with a high vaginal implantation, this treatment frequently fails to provide a good, functional vagina due to a narrow, strictured vaginal opening. Based on previous experience in the treatment of more than 80 patients with a persistent cloaca, a posterior sagittal transanorectal approach with a protective colostomy was performed in three patients with urogenital sinus and normal rectum. The pelvis was approached through a midsagittal posterior incision; the coccyx was split and the entire anorectal sphincteric mechanism was divided in the midline. The rectum was bivalved in the midline including both posterior and anterior rectal walls. This provided excellent exposure to the urogenital sinus. The vagina was then fully separated from the urogenital sinus (as described in cases of persistent cloacas), and then mobilized and sutured to the perineum. The rectum and sphincteric mechanism were meticulously reconstructed. A midline incision assures the preservation of anorectal innervation, and provides excellent exposure to the pelvis. Anal dilatations are not necessary to maintain a patent and supple anorectal opening because the rectum has two suture lines, one in front of the other. After the colostomy was closed, all patients had appropriate bowel control for their age; two of them are fully continent for urine and the third one still has a suprapubic cystostomy tube waiting for a repair of an additional urethral malformation.
对于伴有正常直肠的泌尿生殖窦的治疗仍然是一项挑战。对于阴道低位植入的患者，采用或不采用皮瓣的会阴入路似乎是有效的。然而，对于阴道高位植入的患者，由于阴道口狭窄、闭锁，这种治疗常常无法提供一个良好的、功能正常的阴道。基于之前治疗80余例持续性泄殖腔患者的经验，对3例伴有泌尿生殖窦且直肠正常的患者采用了带保护性结肠造口术的后矢状经肛门直肠入路。通过后正中矢状切口进入骨盆；劈开尾骨，在中线处将整个肛门直肠括约肌机制分开。在中线处将直肠分为两半，包括直肠后壁和前壁。这为暴露泌尿生殖窦提供了极佳的视野。然后将阴道与泌尿生殖窦完全分离（如在持续性泄殖腔病例中所述），然后游离并缝合至会阴。精心重建直肠和括约肌机制。正中切口可确保保留肛门直肠神经支配，并能很好地暴露骨盆。由于直肠有两条缝线，一前一后，因此无需进行肛门扩张来维持肛门直肠开口的通畅和柔软。结肠造口关闭后，所有患者的排便控制能力与其年龄相符；其中2例排尿完全自控，第3例仍有耻骨上膀胱造瘘管，等待修复额外的尿道畸形。
