TY - JOUR
T1 - Herlyn-Werner-Wunderlich syndrome: pre- and post-surgical MRI and US findings
AU - Dias, João Carlos Fernandes Lopes
N1 - PMID: 25852048
WOS:000361835700059
PY - 2015/1
Y1 - 2015/1
N2 - Herlyn-Werner-Wunderlich syndrome (HWWS) is a rare congenital anomaly of the female urogenital tract that associates Mullerian duct anomalies with mesonephric duct anomalies. The triad of uterus didelphys, obstructed hemivagina, and ipsilateral renal agenesis characterizes this syndrome. Patients generally present with non-specific symptoms after menarche. Pelvic pain, dysmenorrhea, and palpable mass due to hematocolpos or hematometra are the most common findings. Pyohematocolpos and pyosalpinx may appear as acute complications, while endometriosis and pelvic adhesions constitute potential long-term complications. When a prenatal diagnosis of unilateral renal agenesis in newborn girls is known, a gynecological imaging study should be performed to exclude uterine and vaginal abnormalities. These patients should be followed up to ensure that a timely surgical correction is performed. The diagnosis of HWWS is difficult due to the lack of specific symptoms or findings upon physical examination. An accurate imaging description of these congenital anomalies is crucial to guide patients toward surgical treatment, relieving acute complications, and preserving the normal fertility. The authors provide a pictorial review of the magnetic resonance imaging and ultrasonography findings of the HWWS with correlation to embryological, clinical, and surgical features.
AB - Herlyn-Werner-Wunderlich syndrome (HWWS) is a rare congenital anomaly of the female urogenital tract that associates Mullerian duct anomalies with mesonephric duct anomalies. The triad of uterus didelphys, obstructed hemivagina, and ipsilateral renal agenesis characterizes this syndrome. Patients generally present with non-specific symptoms after menarche. Pelvic pain, dysmenorrhea, and palpable mass due to hematocolpos or hematometra are the most common findings. Pyohematocolpos and pyosalpinx may appear as acute complications, while endometriosis and pelvic adhesions constitute potential long-term complications. When a prenatal diagnosis of unilateral renal agenesis in newborn girls is known, a gynecological imaging study should be performed to exclude uterine and vaginal abnormalities. These patients should be followed up to ensure that a timely surgical correction is performed. The diagnosis of HWWS is difficult due to the lack of specific symptoms or findings upon physical examination. An accurate imaging description of these congenital anomalies is crucial to guide patients toward surgical treatment, relieving acute complications, and preserving the normal fertility. The authors provide a pictorial review of the magnetic resonance imaging and ultrasonography findings of the HWWS with correlation to embryological, clinical, and surgical features.
KW - OBSTRUCTED HEMIVAGINA
KW - FEMALE GENITAL-TRACT
KW - CLINICAL CHARACTERISTICS
KW - VAGINAL SEPTUM
KW - DIAGNOSIS
KW - Uterus didelphys
KW - GARTNER DUCT PSEUDOCYST
KW - UTERUS DIDELPHYS
KW - UTERINE ANOMALIES
KW - MULLERIAN ANOMALY VARIANT
KW - Mullerian duct anomalies
KW - Renal agenesis
KW - Herlyn-Werner-Wunderlich syndrome
KW - Mesonephric duct anomalies
KW - IPSILATERAL RENAL AGENESIS
KW - Hematocolpos
U2 - 10.1007/s00261-015-0421-0
DO - 10.1007/s00261-015-0421-0
M3 - Article
C2 - 25852048
SN - 0942-8925
VL - 40
SP - 2667
EP - 2682
JO - Abdominal Imaging
JF - Abdominal Imaging
IS - 7
ER -