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Unusual Variety of Posterior Cloaca
0022-5347/04/1713-1291/0 THE JOURNAL OF UROLOGY® Copyright © 2004 by AMERICAN UROLOGICAL ASSOCIATION
Vol. 171, 1291, March 2004 Printed in U.S.A.
DOI: 10.1097/01.ju.0000112981.84342.75
UNUSUAL VARIETY OF POSTERIOR CLOACA I. SAU, S. A. WARNE
AND
D. T. WILCOX*
From the Department of Paediatric Urology, Guy’s Hospital, London, United Kingdom KEY WORDS: cloaca, rectum, abnormalities
Cloaca is a rare anomaly affecting a wide spectrum of anatomy. Posterior cloaca is a variation that involves the cloacal channel ending in the rectum. We describe 2 cases of an unusual variety of posterior cloaca. CASE HISTORIES
Case 1. A female newborn presented with an abdominal mass arising from the pelvis, a normally sited anal opening and a single opening at the introitus. She passed urine and meconium through the 2 openings separately. Imaging revealed a complete duplex system on the left side with a poorly functioning upper moiety draining ectopically. The right side was an incomplete duplex. There was a short sacrum but no cord tethering. Examination with the patient under anesthesia and cystoscopy showed a urogenital sinus with a common channel of 3 cm and a capacious vagina. The vagina was decompressed and the patient was started on clean intermittent catheterization. At cystovaginoscopy the patient had an H-type fistulous connection from the posterior wall of the urogenital sinus to the anal canal. This finding was confirmed at operation (see figure). During surgical reconstruction via the perineal approach the fistula was opened and the vaginal wall was mobilized off the rectum. The common channel was mobilized and then opened onto the perineum, creating separate urethral and vaginal openings. An upper pole duplex ureter also Accepted for publication October 10, 2003. * Correspondence: Department of Paediatric Urology, Great Ormond Street Hospital for Children NHS Trust, London, WC1N 3JH, United Kingdom (telephone: 0044 207 405 9200, ext. 0445; FAX: 0044 2078138260; e-mail: [email protected]).
drained into the upper vagina and was removed by right upper pole heminephrectomy. The patient recovered well and at age 3 years she is dry during the day and in diapers at night. She has regular bowel movements. Case 2. At 26 weeks of gestation a female fetus was diagnosed with left renal agenesis and right hydronephrosis. Serial scans demonstrated increasing right hydronephrosis with a subsequent perinephric urinoma, which was shunted by pelvic amniotic shunt at 31 weeks. The patient was born at 37 weeks by normal vaginal delivery with a turbulent neonatal period, and required peritoneal dialysis and right nephrostomy. Right end ureterostomy was performed. She was noted to have an anterior anal opening and a single opening at the introitus. Cystovaginoscopy revealed a common channel of 1 cm with a small hypoplastic bladder. There were hemivaginae and hemiuteri and rectal duplication, and an H-type fistulous connection between the urogenital sinus and the anterior wall of the rectum with a good external anal sphincter. After renal function stabilized the patient underwent surgery by posterior sagittal approach at age 1 year. The septum between the 2 hemivaginae was divided with mobilization of the short common channel to create separate urethral and vaginal openings. The fistula was excised. Rectal duplication was excised and the neorectum was placed in the middle of the sphincter complex. DISCUSSION
Posterior cloaca differs from classic cloaca in that the anal opening is at a normal or anterior position.1, 2 The urethra and vagina converge into a urogenital sinus that drains posteriorly and opens either at the anterior wall of the anus (type A) or outside just anterior to the anal opening (type B).3 In our patients the urogenital sinus and anus opened separately and were joined by an H-type fistulous connection. These cases further highlight the anatomical complexity seen in cloacal anomalies, and the need for complete renal and urogenital investigation before reconstruction is attempted. These cases have been described to highlight the fact that a female infant with a urogenital sinus and apparently normal anus merits further investigation. We recommend examination with the patient under anesthesia and cystovaginoscopy in all such patients to document the internal anatomy and plan subsequent reconstructive surgery. REFERENCES
Anterior urogenital sinus. Catheter passes from urogenital sinus anteriorly through horizontal limb of H-type fistula and out of rectum posteriorly.
1291
1. Pena, A. and Kessler, O.: Posterior cloaca: a unique defect. J Pediatr Surg, 33: 407, 1998 2. Leditschke, J. F. and Pena, A.: An unusual cloacal anomaly. Late recognition and operative correction. Pediatr Surg Int, 6: 52, 1991 3. Stephens, F. D. and Smith, D. E.: Classification, identification, and assessment of surgical treatment of anorectal anomalies. Pediatr Surg Int, 1: 200, 1986
Vol. 171, 1291, March 2004 Printed in U.S.A.
DOI: 10.1097/01.ju.0000112981.84342.75
UNUSUAL VARIETY OF POSTERIOR CLOACA I. SAU, S. A. WARNE
AND
D. T. WILCOX*
From the Department of Paediatric Urology, Guy’s Hospital, London, United Kingdom KEY WORDS: cloaca, rectum, abnormalities
Cloaca is a rare anomaly affecting a wide spectrum of anatomy. Posterior cloaca is a variation that involves the cloacal channel ending in the rectum. We describe 2 cases of an unusual variety of posterior cloaca. CASE HISTORIES
Case 1. A female newborn presented with an abdominal mass arising from the pelvis, a normally sited anal opening and a single opening at the introitus. She passed urine and meconium through the 2 openings separately. Imaging revealed a complete duplex system on the left side with a poorly functioning upper moiety draining ectopically. The right side was an incomplete duplex. There was a short sacrum but no cord tethering. Examination with the patient under anesthesia and cystoscopy showed a urogenital sinus with a common channel of 3 cm and a capacious vagina. The vagina was decompressed and the patient was started on clean intermittent catheterization. At cystovaginoscopy the patient had an H-type fistulous connection from the posterior wall of the urogenital sinus to the anal canal. This finding was confirmed at operation (see figure). During surgical reconstruction via the perineal approach the fistula was opened and the vaginal wall was mobilized off the rectum. The common channel was mobilized and then opened onto the perineum, creating separate urethral and vaginal openings. An upper pole duplex ureter also Accepted for publication October 10, 2003. * Correspondence: Department of Paediatric Urology, Great Ormond Street Hospital for Children NHS Trust, London, WC1N 3JH, United Kingdom (telephone: 0044 207 405 9200, ext. 0445; FAX: 0044 2078138260; e-mail: [email protected]).
drained into the upper vagina and was removed by right upper pole heminephrectomy. The patient recovered well and at age 3 years she is dry during the day and in diapers at night. She has regular bowel movements. Case 2. At 26 weeks of gestation a female fetus was diagnosed with left renal agenesis and right hydronephrosis. Serial scans demonstrated increasing right hydronephrosis with a subsequent perinephric urinoma, which was shunted by pelvic amniotic shunt at 31 weeks. The patient was born at 37 weeks by normal vaginal delivery with a turbulent neonatal period, and required peritoneal dialysis and right nephrostomy. Right end ureterostomy was performed. She was noted to have an anterior anal opening and a single opening at the introitus. Cystovaginoscopy revealed a common channel of 1 cm with a small hypoplastic bladder. There were hemivaginae and hemiuteri and rectal duplication, and an H-type fistulous connection between the urogenital sinus and the anterior wall of the rectum with a good external anal sphincter. After renal function stabilized the patient underwent surgery by posterior sagittal approach at age 1 year. The septum between the 2 hemivaginae was divided with mobilization of the short common channel to create separate urethral and vaginal openings. The fistula was excised. Rectal duplication was excised and the neorectum was placed in the middle of the sphincter complex. DISCUSSION
Posterior cloaca differs from classic cloaca in that the anal opening is at a normal or anterior position.1, 2 The urethra and vagina converge into a urogenital sinus that drains posteriorly and opens either at the anterior wall of the anus (type A) or outside just anterior to the anal opening (type B).3 In our patients the urogenital sinus and anus opened separately and were joined by an H-type fistulous connection. These cases further highlight the anatomical complexity seen in cloacal anomalies, and the need for complete renal and urogenital investigation before reconstruction is attempted. These cases have been described to highlight the fact that a female infant with a urogenital sinus and apparently normal anus merits further investigation. We recommend examination with the patient under anesthesia and cystovaginoscopy in all such patients to document the internal anatomy and plan subsequent reconstructive surgery. REFERENCES
Anterior urogenital sinus. Catheter passes from urogenital sinus anteriorly through horizontal limb of H-type fistula and out of rectum posteriorly.
1291
1. Pena, A. and Kessler, O.: Posterior cloaca: a unique defect. J Pediatr Surg, 33: 407, 1998 2. Leditschke, J. F. and Pena, A.: An unusual cloacal anomaly. Late recognition and operative correction. Pediatr Surg Int, 6: 52, 1991 3. Stephens, F. D. and Smith, D. E.: Classification, identification, and assessment of surgical treatment of anorectal anomalies. Pediatr Surg Int, 1: 200, 1986