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Case Report

Transverse Vaginal Septum with Congenital


Vesical-Vaginal Communication and Cyclical
Hematuria
Arnold I. Chin, Matthew Rutman, and Shlomo Raz
Cyclical hematuria is an unusual presentation of a müllerian fusion anomaly. We report a patient with transverse
vaginal septum and menstruation by way of the lower urinary tract because of a vesical-vaginal communication. A
defect of vertical fusion, transverse vaginal septum results from failure of canalization of the vaginal plate. Recon-
struction using a transvaginal and transabdominal approach created a direct anastomosis between the proximal vaginal
segment and the distal vaginal pouch. Previously described cases are reviewed. UROLOGY 69: 575.e5–575.e7, 2007. ©
2007 Elsevier Inc.

T
ransverse vaginal septum, also known as partial urgency. She had had no previous surgeries, irradiation,
vaginal agenesis, is a type of müllerian duct or trauma.
fusion anomaly, which includes vertical and The physical examination revealed a blind-ending va-
lateral fusion defects and complete vaginal agenesis.1 gina approximately 2 cm in length, with the urethral
This rare anomaly carries an incidence of approxi- meatus located on the anterior aspect of the vaginal
mately 1 in 70,000 women.1,2 Patients typically present dimple. The development of the female secondary sexual
with primary amenorrhea and hematocolpos, manifest- characteristics, clitoris, and labia had progressed nor-
ing as abdominal pain from obstruction of the uterine mally. No somatic anomalies were observed.
outflow tract. Associated renal and skeletal abnormal- Cystoscopy performed 2 days after cessation of gross
ities are common. hematuria visualized two areas of bloody discharge at the
We describe an unusual case of transverse vaginal trigone between the ureteral orifices, with surrounding
septum presenting as cyclical gross hematuria. Menstru- squamous metaplasia and an otherwise normal-appearing
ation occurred through the urinary tract as a result of bladder with no evidence of endometriosis (Fig. 1A).
communication between the proximal vagina and blad- Computed tomography of the abdomen and pelvis re-
der. Fourteen similar cases have been previously reported vealed normal kidneys and upper tracts. Magnetic reso-
and are discussed. nance imaging of the pelvis was performed to better
delineate the vaginal canal and showed a normal-appear-
CASE REPORT ing uterus and the presence of ovaries but the notable
A 23-year-old woman experiencing cyclical gross hema- absence of the distal vagina without fluid collections (Fig.
turia was referred to us. The episodes typically lasted 3 to 1B). Although cystoscopy and imaging did not delineate
4 days at 30-day intervals beginning since age 12 and was a distinct fistula tract, possibly because of the timing of
regarded as normal. The patient denied vaginal menstru- the studies, the areas of bloody discharge likely repre-
ation, had never used sanitary pads, nor experienced sented the efflux of menstruation from the proximal
dysmenorrhea or noticed abdominal distension during vagina to the bladder.
menses. In fact, the abnormality was only discovered A transabdominal approach allowed exploration of a
during an initial pelvic examination performed for a potential vesical-vaginal fistula and exposure of the upper
Papanicolaou smear after recent onset of sexual activity. vagina and cervix. Transvesical exploration revealed no
Otherwise, toilet training had occurred at a normal age evidence of a distinct fistula. A dissection between the
with normal voiding patterns and no history of urinary bladder and uterus exposed the proximal vagina and
tract infections or urinary incontinence, frequency, or cervix and freed the subtle communication with the
bladder wall. The anterior vaginal wall at the level of the
cervix was incised to reveal a 0.5-cm-long proximal va-
From the Department of Urology, University of California, Los Angeles, David Geffen
School of Medicine, Los Angeles, California; and Department of Urology, Columbia
gina (Fig. 2A). From the transvaginal perspective, a
University School of Medicine, New York, New York transverse incision made at the apex of the distal vaginal
Address for correspondence: Arnold I. Chin, M.D., Ph.D., Department of Urology, pouch allowed for additional dissection to create a plane
University of California, Los Angeles, David Geffen School of Medicine, 10833
LeConte Avenue, Los Angeles, CA 90095. E-mail: [email protected] between the rectum and urethra to reach the proximal
Submitted: July 31, 2006; accepted (with revisions): January 3, 2007 vagina and cervix (Fig. 2B). Additional mobilization of
© 2007 Elsevier Inc. 0090-4295/07/$32.00 575.e5
All Rights Reserved doi:10.1016/j.urology.2007.01.001
Figure 1. (A) Cystoscopy revealing two areas of bloody
discharge at trigone 2 days after cessation of gross hema-
turia. R ⫽ right side; L ⫽ left side. (B) Magnetic resonance
imaging demonstrating vaginal septum and normal uterus
on sagittal section. Arrows point to vaginal septum, uterus,
and bladder.

the vaginal flaps allowed a direct transvaginal anastomo-


sis of the proximal vaginal edge to the lower vaginal
pouch wall using interrupted sutures, providing continu-
ity to the vaginal canal.
An inflatable Heyer-Schulte vaginal stent with an
internal channel was placed across the anastomosis, and
a nylon suture, securing the stent, was brought through
the abdomen wall to prevent stent migration (Fig. 2C).
The proximal vagina was then closed from the abdomen.
After placement of a suprapubic tube, the bladder was
closed in two layers, with Penrose drainage of the retro- Figure 2. (A) Transabdominal view depicting transverse
pubic space. incision over anterior vaginal wall immediately distal to
Three weeks postoperatively, the patient was exam- cervix. Line drawn over anticipated incision. (B) Transvagi-
nal view showing plane between bladder and rectum before
ined under anesthesia. The stent was removed, and a
creating anastomosis to proximal vagina. (C) Transabdom-
vaginal depth of 12 cm was revealed, with excellent inal view of stent in position after creation of vaginal anas-
healing of the anastomosis. The stent was replaced for 4 tomosis and before closure of anterior vaginal wall. Arrows
more weeks. At the end of this period, the examination point to stent and cervix.
revealed excellent vaginal depth and width, with accom-
modation of a 12-cm dilator. The first vaginal menstru-
ation occurred postoperatively with no further episodes of coital function. She has experienced monthly menstrual
gross hematuria. The patient has been followed up for periods through the vagina, and a recent vaginal exam-
more than 12 months after surgery and has continued ination showed a supple 12-cm vaginal canal without
with the occasional use of the dilator with satisfactory stenosis.

575.e6 UROLOGY 69 (3), 2007


COMMENT genital sinus in 4. Five patients had associated uterine
The embryologic origin of the vagina is derived from the anomalies, four with double apical vaginas. Ten patients
fusion of the müllerian ducts proximally and urogenital had upper tract abnormalities, including solitary kidneys
sinus distally.3 Paired paramesonephric or müllerian ducts in six, duplicated ureters in one, cystic kidneys in two,
arise as coelomic invaginations of the mesonephros and and hypoplastic kidneys in two. Four cases were associ-
fuse caudally to form a midline tubular structure called ated with skeletal malformations.
the uterovaginal primordium. This develops into the Reconstruction for creation of vaginal continuity and
uterus and proximal vagina, and the cranial unfused resection of the fistula is generally planned after puberty
portions of the müllerian ducts develop into the fallopian to allow estrogen stimulation of the tissues.5 Direct anas-
tubes. Endodermal outgrowths from the urogenital sinus, tomosis obviates the need for skin grafts, which may be
termed the sinovaginal bulbs, give rise to the distal va- more prone to stenosis. In at least one case, after recon-
gina. Extension and fusion of the sinovaginal bulb to the struction, the patient conceived and gave birth by cesar-
uterovaginal primordium forms the vaginal plate, a solid ean section.8
cord of epithelial cells. Subsequent canalization of the In the present case of transverse vaginal septum, al-
vaginal plate is thought to occur caudally to cranially and though a delineated vesical-vaginal fistula was not appar-
forms the patent vagina. ent, initial cystoscopy visualized blood emanating from
Incomplete canalization of the vaginal plate leads to the trigone. The absence of subsequent hematuria
defects of vertical fusion such as transverse vaginal sep- pointed to spontaneous closure of the communication
tum and imperforate hymen.1 Other müllerian defects after relief of the vaginal outflow obstruction. Direct
occur with abnormalities of lateral fusion of the müllerian anastomotic reconstruction allowed for good vaginal
ducts or vaginal agenesis with failure of caudal migration depth and width, with minimal stenosis. Pelvic magnetic
of the fused ducts. Müllerian defects are often associated resonance imaging proved beneficial in delineating the
with renal and skeletal abnormalities. Vaginal agenesis, anatomy and location of the obstruction.9,10
relatively more common at 1 in 5000 women, is associ-
ated with a 15% rate of renal anomalies and a 5% to 10% References
rate of skeletal anomalies.1,4 1. Gell JS: Müllerian anomalies. Semin Reprod Med 21: 375–388,
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2. Banerjee R, and Laufer MR: Reproductive disorders associated with
vaginal fistula is a rare variant of an already uncommon pelvic pain. Semin Pediatr Surg 7: 52– 61, 1998.
anomaly. It is unclear whether a true urinary-vaginal 3. Moore KL, and Persaud TVN: The Developing Human: Clinically
fistula is necessary for this condition or whether the Oriented Embryology, 5th ed. Philadelphia, WB Saunders, 1993, pp
fistula results from elevated pressure of the hematocol- 287–289.
pos.5,6 Regardless of its origin, the diversion of menstru- 4. Bryon A, Nigro J, and Counsellor V: One hundred cases of con-
genital absence of the vagina. Surg Gynecol Obstet 88: 79 – 85,
ation reduces the chance of retrograde blood flow, the 1949.
development of hematometra and hematosalphinx, and 5. Genest D, Farber M, Mitchell GW Jr, et al: Partial vaginal agenesis
subsequent risks of infertility.1 with a urinary-vaginal fistula. Obstet Gynecol 58: 130 –134, 1981.
From a literature search, only 14 similar cases have 6. Verhoeven AT: Primary menouria due to congenital vesicovaginal
been previously reported.5–7 Six women presented after fistula. Obstet Gynecol 41: 515–520, 1973.
7. Daskalov I, and Sahpazov M: [Report of a case of congenital
puberty with cyclical hematuria, and four were identified vesico-uterine fistula with vaginal atresia associated with an exte-
postmortem along with multiple congenital anomalies. rior uterine cervix and agenesis of the right kidney]. Jugosl Ginekol
Another four were diagnosed in childhood, all of whom Opstet 22: 78 – 81, 1982.
had a persistent urogenital sinus along with urinary tract 8. Whittemore WS: Pregnancy following operation for congenital
difficulties, including urinary tract infections in two and absence of the vagina. Am J Obstet Gynecol 44: 516 –521, 1942.
9. Hugosson C, Jorulf H, and Bakri Y: MRI in distal vaginal atresia.
incontinence in two. Fistulas between the blind-ending Pediatr Radiol 21: 281–283, 1991.
superior vaginal segment were identified between the 10. Troiano RN, and McCarthy SM: Müllerian duct anomalies: imag-
bladder in 7 and urethra in 3 or with a persistent uro- ing and clinical issues. Radiology 233: 19 –34, 2004.

UROLOGY 69 (3), 2007 575.e7

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