Prepubertal presentation of Herlyn-Werner-Wunderlich syndrome: a case report

Sanghvi, Yogendra; Shastri, Pankaj; Mane, Sushant; Dhende, Nitin P.

doi:10.1016/j.jpedsurg.2011.02.067

Cited by 31 publications

(34 citation statements)

References 14 publications

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“…Urogenital sinus develops and lack of exposure to androgens allows regression of wolffian ducts and further development of m€ ullerian ducts [12][13][14] M€ ullerian ducts fuse with urogenital sinus [15][16][17][18][19][20][21][22][23][24][25][26] Cephalic growth of sinovaginal bulb occurs [15][16][17][18][19][20][21][22][23][24][25][26] After cephalic growth occurs, fusion of sinovaginal bulb will help form the vaginal plate. Subsequently, canalization of the vagina occurs.…”

Section: Primary Amenorrhea Due To An Obstructed Anomalymentioning

confidence: 99%

Obstructive Reproductive Tract Anomalies

Dietrich

Millar

Quint

2014

Journal of Pediatric and Adolescent Gynecology

130

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Section: Primary Amenorrhea Due To An Obstructed Anomalymentioning

confidence: 99%

Obstructive Reproductive Tract Anomalies

Dietrich

Millar

Quint

2014

Journal of Pediatric and Adolescent Gynecology

130

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“…Pelvic pain, dysmenorrhea, and palpable mass due to hematocolpos or hematometra are the most common findings [2,20]. Primary amenorrhea, dyspareunia, urinary retention, spontaneous rupture of the hematocolpos, infertility, and obstetric complications are less common manifestations [13,21,22].…”

Section: Clinical Presentationmentioning

confidence: 99%

“…Hematocolpos and hematometra are more prevalent at the right side and result from retained menstrual flow. These manifestations are more painful when the vaginal septum fuses with the vaginal wall, leading to a complete obstruction [1,2,20,25]. If there is not a complete obstruction, symptoms may delay or be only mild [12].…”

Section: Clinical Presentationmentioning

confidence: 99%

“…Nonetheless, some studies like that of Tong et al [1] showed that the incidence of primary infertility is not increased among women with HWWS. Actually, infertility is a potential complication of HWWS when the diagnosis is delayed and treatment is inappropriate, and appears to be more related to endometriosis and infectious complications than to uterus didelphys [20,23,29].…”

Section: Complicationsmentioning

confidence: 99%

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Herlyn–Werner–Wunderlich syndrome: pre- and post-surgical MRI and US findings

Dias

Jogo

2015

Abdom Imaging

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Herlyn-Werner-Wunderlich syndrome (HWWS) is a rare congenital anomaly of the female urogenital tract that associates Mu¨llerian 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.

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“…HWW sendromu genellikle pubertal dönemde menarşı takiben hematokolposa sekonder gelişen şiddetli pelvik ağrı ve pelvik kitle ile kendini göstermektedir (11,12). Ancak prepubertal dönemde de saptanan olgular bildirilmiştir (13). Piyohematokolpos, piyosalpenks, piyoperitonit gibi enfektif süreçler, retrograd menstruasyona sekonder endometriozis ve pelvik adezyonlar karşılaşılabilecek komplikasyonlar arasındadır (11,12,(16)(17)(18).…”

Section: Olguunclassified