Urethral Duplication With Single Bladder and Multiple Genitourinary Abnormalities: An Example of Continent Urinary Diversion By Andrew
6. Pint&
and Andreas
P. Farkas
P&s, Hungary 0 A 13-year-old girl with multiple genitourinary malformations, incomplete bladder exstrophy, urethral duplication with single bladder, septate vagina, and total urinary incontinence is presented. Prior to admission she had undergone surgery for teratoma and calculi of the bladder and partial fecal incontinence. A continent urinary diversion was done by bladder augmentation using a cecalcolonic segment and by surgical closure of the bladder neck. The distal end of the appendix was brought to the skin as an inconspicuous, easily catheterizable, watertight stoma, our modification of the Mitrofanoff procedure. Excision of the vaginal septum, creation of an introitus. and unification of the split clitoris improved the cosmetic appearance of the external genitalia and improved the outlook for a normal sexual life and pregnancy. o 1990 by W-5. Saunders Company. INDEX WORDS: diversion.
A
Urethral duplication; continent urinary
CASE IS presented with serious multiple mations of the genitourinary tract. CASE
malfor-
REPORT
A girl was born after an uneventful pregnancy as a premature first child, weighing 1,440 g at birth. The following abnormalities were detected at birth. (1) An incomplete form of bladder exstrophy, wide symphysiolysis, cleft clitoris, diastasis of the rectus abdominis below the umbilicus, the bladder was covered with thin skin and fascia only. (2) A 2 x 2 cm surface area on the perineum, bordered by the cleft clitoris, the antepositioned anus, and the pubic bones, was covered with mucous membrane. On its upper part there were two urethral orifices, side by side in the frontal level, discharging urine continuously. Both urethras measured 5 to 6 mm in length, without a detectable sphincter mechanism. Below them, there were two other narrow openings of the septated vagina (Fig IE). At the age of 3 years two stones (Fig 1A) were detected in the bladder and removed. During cystotomy a cystic-cartilaginous tumor the size of a hen’s egg attached to the anterior wall of the bladder was found and excised. Histology demonstrated adult-type teratoma. In the same year her perineal anus was retroposed and 3 years later levatorplasty and Dacron collar implantation for fecal incontinence were done. At the age of 13 years she was referred to us because of total urinary incontinence and genital malformations. The following x-ray examinations were made: (1) intravenous urography: normal kidneys and ureters, small bladder of abnormal shape (Fig 1B); and (2) genitography: a duplicated (septate) vagina without communication between the two parts; no filling of the uterus on either side (Fig 1C). Our plan of treatment was to establish continent urinary diversion, and the construction of a vagina to make possible a sexual life and delivery.
Technical Details of the Surgery Behind the small, thin-walled bladder a bicornuate uterus with normal ovaries was found. The bladder was opened and both bladder necks were closed surgically. For bladder augmentation a coloniccecal segment was taken, and for continent diversion the appendix
Journal
of Pediatric
Surgery,
Vol 25, No 12 Khcember),
was taken using our modification of the Mitrofanoff principle.’ Twenty centimeters of the ascending colon was taken with the cecum and appendix, as was 15 cm of the terminal ileum. In order to disrupt the peristalsis, which could generate pressure in the reservoir, the large bowel was reconfigurated according to Heineke-Mikulicz, as advocated for this purpose by Rowland and associates* (Fig 1D). For continent diversion use of the ileal segment was planned (Indianatype diversion)‘; however, during surgery the original plan was changed and the 8- to 9-cm-long appendix was used for cutaneous diversion. The ileal end was closed and saved for a possible later diversion in case of failure of the appendiceal diversion. The cecal part of the pouch was then opened and anastomosed to the bladder (Fig 1D). The distal end of the appendix was brought to the skin as an inconspicuous stoma in the lower left quadrant of the abdominal wall so that the patient could reach it easily, although it was hidden by the underwear (Fig 1 H). During the early postoperative period a suprapubic Foley catheter through the colonic tenia and a fine silastic tube via the appendix were left in the augmented bladder to provide free run-off of urine and prevent leakage. To avoid accumulation of mucous produced by the large bowel the augmented bladder was irrigated every 4 hours in the first 6 postoperative weeks. The patient was able to start self-catheterization on the 10th postoperative day. The capacity of the low-pressure bladder soon reached 200 to 250 mL, which ensured dry periods of at least 3 hours in the daytime and required one catheterization during the night. Filling up the augmented bladder with contrast material demonstrated a large reservoir, which became nearly empty following catheterization (Fig 1 F). Four months after establishing the continent urinary diversion the septum dividing the vagina into two parts was resected, the split clitoris was unified, and one vaginal introitus of normal appearance was formed (Fig 1G). One year after the establishment of urinary diversion the patient is free from complaints, socially fully accepted, and engages in outdoor games. DISCUSSION
Recently, Psihramis et al3 have reviewed 16 cases with complete patent duplication of the urethra, seen over 40 years, and found only one female case. Double urethras with single bladder are extremely rare.4 Grob and Grob-Vontobe15 have published a series of incomplete exstrophies of the bladder, including closed bladders as in the present case. Partial failure of the mtillerian ducts to fuse would result in a septum either in the uterus or in the vagina.6 A continent urinary diversion was done by bladder augmentation using a
From the Surgical Unit of the University, Children’s Hospital, Pk. Hungary. Address reprint requests to Andrew B. Pint&r. MD, University Medical School of Pks. Children’s Hospital. Pk. Jbzsef A u 7, H-7423 Hungary. o 1990 by W.B. Saunders Company. 0022-3468/90/2512-0030$03.00/O
1990: pp 1285-1286
1285
Fig 1. (A) Plane abdominal x-ray: small (arrow) and large stone in bladder. (B) Intravenous urography: small bladder of abnormal shape. (Cl Genitography: septate vagina without communication between the two parts. (D) Bladder (arrow) augmentation using a cecsl-colonic segment reconfigurated according to Heineke-Mikulicz. (E) Cleft clitoris (1). urethral orifices (2). and narrow vaginal introituses (3) are shown. (FI Augmented bladder filled up with contrast material. (G) Unification of split clitoris and creation of one vaginal introitus of normal appearance. (HI Distal end of appendix forming an inconspicuous, easily catheterizable watertight stoma.
cecal-colonic segment. The distal end of the appendix was brought to the skin. The watertight, easily catheterizable urinary diversion performed in this case might mean that there is no need to implant the distal end of the appendix into the bladder or colon in a submucosal nonrefluxing fashion, as recommended by Mitrofanoff. The narrow lumen of the appendix itself probably
provides a closure mechanism in a low-pressure urinary reservoir. Recently, Duckett and Snyder’ have reported various uses of the Mitrofanoff principle. Surgery of the external genitalia not only improved the cosmetic appearance and decreased the psychological burden of the anomaly, but bettered the outlook for normal sexual life and pregnancy.
REFERENCES 1. Mitrofanoff dans le traitment 3051980
P: Cystotomie continente des vessies neurologiques.
tram-appendiculaire Chir Pediatr 21:297-
2. Rowland RG, Mitchell ME, Bihrle R, et al: Indiana urinary reservoir. J Urol 137:1136-1139, 1987
continent
3. Psihramis KE, Colodny AH, Lebowitz RL, et al: Complete patent duplication of the urethra. J Urol 136:63-67, 1986
4. Boissonat P: Two cases of complete double functional urethra with a single bladder. Br J Ural 33:453-462, 1961 5. Grob M, Grob-Vontobel V: Uber inkomplette Formen der Blasenekstrophie. Z Kinderchir 7:559-565, 1969 6. Gilsanz V, Cleveland RH, Reid SB: Duplication of the miillerian ducts and genitourinary malformations. Radiology 144: 797-801, 1982 7. Duckett JW, Snyder HM: Continent urinary diversion: Variations on the Mitrofanoff principle. J Urol 136:58-62,1986
6. Pint&
and Andreas
P. Farkas
P&s, Hungary 0 A 13-year-old girl with multiple genitourinary malformations, incomplete bladder exstrophy, urethral duplication with single bladder, septate vagina, and total urinary incontinence is presented. Prior to admission she had undergone surgery for teratoma and calculi of the bladder and partial fecal incontinence. A continent urinary diversion was done by bladder augmentation using a cecalcolonic segment and by surgical closure of the bladder neck. The distal end of the appendix was brought to the skin as an inconspicuous, easily catheterizable, watertight stoma, our modification of the Mitrofanoff procedure. Excision of the vaginal septum, creation of an introitus. and unification of the split clitoris improved the cosmetic appearance of the external genitalia and improved the outlook for a normal sexual life and pregnancy. o 1990 by W-5. Saunders Company. INDEX WORDS: diversion.
A
Urethral duplication; continent urinary
CASE IS presented with serious multiple mations of the genitourinary tract. CASE
malfor-
REPORT
A girl was born after an uneventful pregnancy as a premature first child, weighing 1,440 g at birth. The following abnormalities were detected at birth. (1) An incomplete form of bladder exstrophy, wide symphysiolysis, cleft clitoris, diastasis of the rectus abdominis below the umbilicus, the bladder was covered with thin skin and fascia only. (2) A 2 x 2 cm surface area on the perineum, bordered by the cleft clitoris, the antepositioned anus, and the pubic bones, was covered with mucous membrane. On its upper part there were two urethral orifices, side by side in the frontal level, discharging urine continuously. Both urethras measured 5 to 6 mm in length, without a detectable sphincter mechanism. Below them, there were two other narrow openings of the septated vagina (Fig IE). At the age of 3 years two stones (Fig 1A) were detected in the bladder and removed. During cystotomy a cystic-cartilaginous tumor the size of a hen’s egg attached to the anterior wall of the bladder was found and excised. Histology demonstrated adult-type teratoma. In the same year her perineal anus was retroposed and 3 years later levatorplasty and Dacron collar implantation for fecal incontinence were done. At the age of 13 years she was referred to us because of total urinary incontinence and genital malformations. The following x-ray examinations were made: (1) intravenous urography: normal kidneys and ureters, small bladder of abnormal shape (Fig 1B); and (2) genitography: a duplicated (septate) vagina without communication between the two parts; no filling of the uterus on either side (Fig 1C). Our plan of treatment was to establish continent urinary diversion, and the construction of a vagina to make possible a sexual life and delivery.
Technical Details of the Surgery Behind the small, thin-walled bladder a bicornuate uterus with normal ovaries was found. The bladder was opened and both bladder necks were closed surgically. For bladder augmentation a coloniccecal segment was taken, and for continent diversion the appendix
Journal
of Pediatric
Surgery,
Vol 25, No 12 Khcember),
was taken using our modification of the Mitrofanoff principle.’ Twenty centimeters of the ascending colon was taken with the cecum and appendix, as was 15 cm of the terminal ileum. In order to disrupt the peristalsis, which could generate pressure in the reservoir, the large bowel was reconfigurated according to Heineke-Mikulicz, as advocated for this purpose by Rowland and associates* (Fig 1D). For continent diversion use of the ileal segment was planned (Indianatype diversion)‘; however, during surgery the original plan was changed and the 8- to 9-cm-long appendix was used for cutaneous diversion. The ileal end was closed and saved for a possible later diversion in case of failure of the appendiceal diversion. The cecal part of the pouch was then opened and anastomosed to the bladder (Fig 1D). The distal end of the appendix was brought to the skin as an inconspicuous stoma in the lower left quadrant of the abdominal wall so that the patient could reach it easily, although it was hidden by the underwear (Fig 1 H). During the early postoperative period a suprapubic Foley catheter through the colonic tenia and a fine silastic tube via the appendix were left in the augmented bladder to provide free run-off of urine and prevent leakage. To avoid accumulation of mucous produced by the large bowel the augmented bladder was irrigated every 4 hours in the first 6 postoperative weeks. The patient was able to start self-catheterization on the 10th postoperative day. The capacity of the low-pressure bladder soon reached 200 to 250 mL, which ensured dry periods of at least 3 hours in the daytime and required one catheterization during the night. Filling up the augmented bladder with contrast material demonstrated a large reservoir, which became nearly empty following catheterization (Fig 1 F). Four months after establishing the continent urinary diversion the septum dividing the vagina into two parts was resected, the split clitoris was unified, and one vaginal introitus of normal appearance was formed (Fig 1G). One year after the establishment of urinary diversion the patient is free from complaints, socially fully accepted, and engages in outdoor games. DISCUSSION
Recently, Psihramis et al3 have reviewed 16 cases with complete patent duplication of the urethra, seen over 40 years, and found only one female case. Double urethras with single bladder are extremely rare.4 Grob and Grob-Vontobe15 have published a series of incomplete exstrophies of the bladder, including closed bladders as in the present case. Partial failure of the mtillerian ducts to fuse would result in a septum either in the uterus or in the vagina.6 A continent urinary diversion was done by bladder augmentation using a
From the Surgical Unit of the University, Children’s Hospital, Pk. Hungary. Address reprint requests to Andrew B. Pint&r. MD, University Medical School of Pks. Children’s Hospital. Pk. Jbzsef A u 7, H-7423 Hungary. o 1990 by W.B. Saunders Company. 0022-3468/90/2512-0030$03.00/O
1990: pp 1285-1286
1285
Fig 1. (A) Plane abdominal x-ray: small (arrow) and large stone in bladder. (B) Intravenous urography: small bladder of abnormal shape. (Cl Genitography: septate vagina without communication between the two parts. (D) Bladder (arrow) augmentation using a cecsl-colonic segment reconfigurated according to Heineke-Mikulicz. (E) Cleft clitoris (1). urethral orifices (2). and narrow vaginal introituses (3) are shown. (FI Augmented bladder filled up with contrast material. (G) Unification of split clitoris and creation of one vaginal introitus of normal appearance. (HI Distal end of appendix forming an inconspicuous, easily catheterizable watertight stoma.
cecal-colonic segment. The distal end of the appendix was brought to the skin. The watertight, easily catheterizable urinary diversion performed in this case might mean that there is no need to implant the distal end of the appendix into the bladder or colon in a submucosal nonrefluxing fashion, as recommended by Mitrofanoff. The narrow lumen of the appendix itself probably
provides a closure mechanism in a low-pressure urinary reservoir. Recently, Duckett and Snyder’ have reported various uses of the Mitrofanoff principle. Surgery of the external genitalia not only improved the cosmetic appearance and decreased the psychological burden of the anomaly, but bettered the outlook for normal sexual life and pregnancy.
REFERENCES 1. Mitrofanoff dans le traitment 3051980
P: Cystotomie continente des vessies neurologiques.
tram-appendiculaire Chir Pediatr 21:297-
2. Rowland RG, Mitchell ME, Bihrle R, et al: Indiana urinary reservoir. J Urol 137:1136-1139, 1987
continent
3. Psihramis KE, Colodny AH, Lebowitz RL, et al: Complete patent duplication of the urethra. J Urol 136:63-67, 1986
4. Boissonat P: Two cases of complete double functional urethra with a single bladder. Br J Ural 33:453-462, 1961 5. Grob M, Grob-Vontobel V: Uber inkomplette Formen der Blasenekstrophie. Z Kinderchir 7:559-565, 1969 6. Gilsanz V, Cleveland RH, Reid SB: Duplication of the miillerian ducts and genitourinary malformations. Radiology 144: 797-801, 1982 7. Duckett JW, Snyder HM: Continent urinary diversion: Variations on the Mitrofanoff principle. J Urol 136:58-62,1986
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