08_cc_sindrome_de_he.. - revista mexicana de urología

Clinical case
Herlyn-Werner-Wunderlich syndrome:
a case report and literature review
Osornio-Sánchez Víctor, Santana-Ríos Zae, Fulda-Graue Santiago D, Pérez-Becerra Rodrigo, Urdiales-Ortiz
Aejandro, Martínez Ángel, Fernández-Noyola Gerardo, Ahumada-Tamayo Samue, Camacho-Castro Alberto,
Muñoz-Ibarra Erik, García-Salcido Francisco, Garza-Sainz Gerardo, Mayorga Édgar, Martínez-Arroyo Carlos,
Cantellano-Orozco Mauricio, Morales-Montor Gustavo, Pacheco-Gahbler Carlos
•Abstract
•Resumen
Introduction: Uterus didelphys with blind hemivagina
and ipsilateral renal agenesis (Herlyn-WernerWunderlich syndrome) is a rare congenital anomaly.
There is severe dysmenorrhea and palpable mass due
to unilateral hematocolpos. A case of Herlyn-WernerWunderlich syndrome with left ureteric bud and bladder
diverticulum is presented here.
Introducción: El útero bicorne con hemivagina en fondo de saco y agenesia renal ipsilateral (síndrome de Herlyn
Werner Wünderlich), es una anomalía congénita poco frecuente. Existe dismenorrea severa y masa palpable, por
hematocolpos unilateral. Presentar el caso de síndrome de
Herlyn Werner Wünderlich, con yema ureteral izquierda y
divertículo vesical.
Clinical case: Patient is an eighteen-year-old female
who experienced menarche at twelve years of age.
Menstrual cycles lasted twenty-eight days with
menstrual bleeding for four days and dysmenorrhea.
Left salpingo-oophorectomy was carried out three years
prior due to hematosalpinx and ovarian cysts. Disease
onset presented with dysuria, cloudy and fetid urine,
and recurrent urinary infection symptoms that improved
with antimicrobial treatment. Physical examination
revealed Tanner IV, normal external genitals, and
septate vagina. Imaging studies showed left renal
agenesis, left ureterocele, and bladder diverticulum.
Cystoscopy revealed left ureterocele and was deroofed
by endoscopy. Retrograde pyelography revealed left
ureteric bud. Open procedures of left ureterectomy and
bladder diverticulectomy were later carried out.
Department of Urology, Hospital General “Dr. Manuel Gea González”,
Mexico City, Mexico.
Caso clínico: Femenino de 18 años de edad. Menarca a los
12 años. Ciclos menstruales 28 por 4, con dismenorrea. Salpingooforectomía izquierda hace tres años, por hematosalpinx y quistes de ovario. Inició su padecimiento con disuria, orina turbia y fétida, cuadros repetitivos de infección
urinaria, presentando mejoría con tratamiento antimicrobiano. A la exploración física se encuentra con Tanner IV,
genitales externos normales, vagina septada. Por imagen,
existía agenesia renal izquierda, ureterocele izquierdo y divertículo vesical. Por cistoscopia se encontró ureterocele izquierdo, destechándose endoscópicamente. La pielografia
ascendente mostró yema ureteral izquierda, posteriormente
se realizó ureterectomía izquierda y diverticulectomía vesical
abierta.
Corresponding author: Dr. Víctor Osornio-Sánchez. Calle 9na Oriente
N°11. Col. Isidro Fabela. C.P. 14030. Delegación Tlalpan, México
D.F., México. Telephone: (55) 5424 5078. Cell phone: (044)
5554066358. Email: vickos103@msn.com
Rev Mex Urol 2012;72(1):31-34
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Osornio-Sánchez V, et al. Herlyn-Werner-Wunderlich syndrome: a case report and literature review
Discussion: The Wolffian ducts give rise to the kidneys
and induce Müllerian duct fusion; abnormality in Wolffian
duct caudal development is the cause of unilateral renal
agenesis. The Müllerian duct on the same side as the
absent Wolffian duct is laterally displaced without fusing
with the contralateral duct. This results in bicornuate
uterus and there is no contact with the central urogenital
sinus. The contralateral Müllerian duct gives rise to a
vagina and the displaced component forms a blind sac.
Conclusions: It is important to detect these types of rare
abnormalities that cause precocious symptomatology,
pelvic endometriosis, and collection infections.
Keywords: Renal agenesis, unilateral hematocolpos,
bicornuate uterus, Mexico.
Discusión: Los conductos wolffianos dan origen a
los riñones, son inductores de la fusión de los conductos müllerianos. La anomalía en el desarrollo caudal de los
conductos de wolff, es la causa de agenesia renal unilateral. En el lado del conducto de wolf ausente, el conducto de
müller está desplazado lateralmente sin fusionarse con el
conducto contralateral, resultando útero bicorne sin contactar el seno urogenital central. El conducto mülleriano
contralateral da origen a una vagina, y el componente desplazado forma un saco ciego.
Conclusiones: Es importante detectar este tipo de anomalías infrecuentes, que causan sintomatología precoz,
endometriosis pélvica e infección de colecciones.
Palabras clave: Agenesia renal, hematocolpos unilateral,
útero bicorne, México.
•Introduction
Herlyn-Werner-Wunderlich syndrome is a rare
congenital malformation of the Müllerian ducts. It
is thought to be a developmental abnormality of the
Wolffian ducts as well as of the Müllerian ducts. It is
also known today as uterine didelphys associated with
obstructed hemivagina and ipsilateral renal anomaly
(OHVIRA), a broader term, since it includes other
types of renal abnormalities. These alterations are
significantly more frequent on the right side and there is
no clear explanation for this fact. The disease is rare and
prevalence is undetermined. In the general population,
unilateral renal agenesis is estimated to be from 1 in
every 600-1200 individuals. In women, the prevalence
of genital abnormalities associated with kidney
anomalies is estimated between 25-89%. The alterations
of the urinary tract most frequently associated with
developmental abnormalities of the Müllerian ducts are
renal agenesis, double collecting system, double kidney,
and horseshoe kidney. 1-6
•Case presentation
Patient is an 18-year-old woman. She began menarche
at 12 years of age. Menstrual cycles lasted for 28 days
and bleeding lasted for 4 days with dysmenorrhea. Left
salpingo-oophorectomy was carried out three years
prior due to hematosalpinx and multiple ovarian cysts.
Three years earlier patient presented with dysuria,
32
Rev Mex Urol 2012;72(1):31-34
Figure 1. Excretory urography at 25 minutes with no evidence of
left kidney. Adequate contrast medium elimination in right kidney.
Filling defect in left lateral wall of the bladder.
Osornio-Sánchez V, et al. Herlyn-Werner-Wunderlich syndrome: a case report and literature review
A
B
C
D
Figure 2. Abdominopelvic CAT scan. A and B: Left renal agenesis. C and D: left ureterocele.
cloudy and fetid urine, and recurrent urinary tract
infection symptoms that mildly improved with
antimicrobial treatment. Due to persistence of
symptomatology, patient was sent to the authors’
unit for evaluation. She continued to have the same
symptomatology and urine culture was positive
for Escherichia coli. Physical examination revealed
Tanner IV, normal external genitals, and gynecological
examination showed unaltered vaginal introitus and
septate vagina.
Kidney and pelvic ultrasound revealed one
hypertrophied right kidney with regular edges, left renal
agenesis, left ureterocele, and bicornuate uterus.
Excretory urography (Figure 1) showed: Right
kidney, enlarged kidney silhouette, adequate uptake and
elimination of contrast medium, tortuous distal third of
right ureter, and no evidence of obstructive pathology.
Left kidney was not visible.
Abdominopelvic computed tomography (CT) scan
(Figure 2) showed 13 x 6.5 x 6.3 cm right kidney,
adequate uptake and elimination of contrast medium,
left renal agenesis, left ureterocele, and bicornuate
uterus.
Cystourethrography (Figure 3) showed left ureteral
reflux and ectasia and bladder diverticulum in left lateral
wall.
Cystoscopy reported 350 mL bladder capacity
and orthotopic and ejaculating right ureteral meatus.
Extrinsic mass was found in left lateral wall compatible
with ureterocele, which was deroofed through
endoscopy with exit of chocolate-brown matter and
Rev Mex Urol 2012;72(1):31-34
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Osornio-Sánchez V, et al. Herlyn-Werner-Wunderlich syndrome: a case report and literature review
A
B
C
Figure 3. Filling and emptying urethrocystography. A and B: Left ureteral reflux. C: Bladder diverticulum
coagulates. Retrograde pyelography showed incomplete
left ureteric bud (up to mid-third), and unaltered right
ureter.
After
retrograde
pyelography
findings,
the
open procedures of left ureterectomy and bladder
diverticulectomy were carried out.
Patient is currently asymptomatic and being
evaluated by the Department of Gynecology, where she
is not considered to be a candidate for another surgical
treatment at this time. Patient should remain under
surveillance.
•Discussion
The Wolffian ducts, in addition to giving rise to the kidneys,
are factors that induce adequate fusion of the Müllerian
ducts. Therefore, abnormality in the development of the
caudal portion of the Wolffian ducts can be the cause of
unilateral renal agenesis associated with imperforated
hemivagina. Left renal agenesis presented in the patient
described here. The Müllerian duct is laterally displaced
on the side in which the Wolffian duct is absent and
cannot fuse with the contralateral duct, resulting in
a bicornuate uterus with no central urogenital sinus
contact. The contralateral Müllerian duct gives rise to
the vagina, while the displaced component forms a blind
sac, the obstructed or imperforated hemivagina. In the
case described here, the patient presented with septate
vagina. The vaginal introitus is not involved because it
arises from the urogenital sinus. 3
34
Rev Mex Urol 2012;72(1):31-34
•Conclusions
In clinical practice it is important to detect this type of
abnormality, despite its rareness, because it is the cause
of precocious symptomatology and facilitates early pelvic
endometriosis and collection infections (pyocolpos,
pyometra, or pyosalpinx). Perhaps the present patient’s
hematosalpinx was an early disease manifestation
due to endometriosis associated with these types of
cases, but this could not be demonstrated because it
did not happen when the patient was at the authors’
institution. Simple surgical procedures (septum excision
and collection drainage) provide satisfactory results in
relation to pain suppression as well as to reproductive
capacity. Because these patients have only one kidney,
urinary infection prevention is very important.
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