Herlyn-Werner-Wunderlich syndrome, characterized by uterus didelphys with blind hemivagina and ipsilateral renal agenesis, is a rare Mullerian duct anomaly. This case series shows a wide spectrum of the condition, one presenting in an adolescent, managed conservatively and the other in the perimenopausal age group given a more definitive management. The first case is an 18-year- old nulligravid who manifested with progressive dysmenorrhea and foul smelling vaginal discharge a few years after menarche. She subsequently underwent vaginal septotomy followed by diagnostic hysteroscopy. On the other hand, the second case is a 46- year-old nulligravid whose chief complaint is a foul smelling vaginal discharge and consequently went through a total abdominal hysterectomy with salpingo-oophorectomy. To our knowledge, the second case is the only patient diagnosed in the perimenopausal stage and underwent a total hysterectomy. Ultrasound is the first-line imaging modality used in both cases and its merits are highlighted to prove its importance and diagnostic value in the workup of this condition.
Mullerian aplasia ; Mullerian Ducts ; Urogenital Abnormalities

Transverse vaginal septum is a rare Mullerian anomaly which is related to vertical fusion disorder. It is an uncommon anomaly that is not associated with a well-defined inheritance pattern. The vase majority of these obstructed mullerian duct abnormalities are first seen at puberty. Hematocolpos, hematometra and hematometrocolpos are some of its most common presentations. Some authors have suggested that retrograde flow through the uterus and falopian tubes could have occured earlier. It may even present before puberty. Transverse vaginal septum is infrequently associated with genitourinary tract, gastrointestinal tract, musculoskeletal, and cardiac malformations. However, it is still prudent to work up patients of the possibility of having any concomitant anomaly. The differential diagnosis of transverse vaginal septum includes imperforate hymen, Mayer-Rokitansky-Kuster-Hauser syndrome and vaginal agenesis. The complex anatomy of mullerian duct anomalies requires complete evaluation before surgical repair which would entail the use of magnetic resonance imaging, pelvic sonographic and computed tomography. In a study by Thabet, et al. in 2002, a newer modality, sonocolpography can be used to evaluate transverse vaginal septum and other allied conditions. Treatment is surgical. This presentation aims to refocus attention to the disease by reviewing the case of a 14 year old female who presented with cyclical pain and primary amenorrhea.
Human ; Female ; Adolescent ; MULLERIAN DUCTS ; ABNORMALITIES ; ENDOMETRIOSIS ;

OBJECTIVE: To study the etiology of primary amenorrhea in 19-aged woman. MATERIALS ANDMETHODS: Case report. RESULTS: I found that there were not gonads and organ of mullerian ducts origin on diagnostic laparoscopy. She was normal 46XX on chromosomal study and normal external genitalia except short vaginal canal. CONCLUSION: It is very rare that absent gonads and lack of mullerian ducts with 46XX female and primary amenorrhea.
Amenorrhea* ; Female ; Genitalia ; Gonads* ; Humans ; Laparoscopy ; Mullerian Ducts*

Ovarian malignancy can rarely present as a colonic mass and simulate colon cancer. Mullerian anomalies are congenital anatomic abnormalities of the female internal genitalia, of which a unicornuate uterus is a rare subtype. Several cases of gynecologic malignancies arising in Mullerian anomalies have been described. However, an ectopic ovarian malignancy in conjunction with a unicornuate uterus has never been reported. We report a case of ectopic ovarian mixed epithelial tumor, a very rare subtype of ovarian epithelial tumor, which presented as a colonic mass simulating colon cancer in a woman with a unicornuate uterus.
Colon ; Colonic Neoplasms ; Female ; Genitalia ; Humans ; Mullerian Ducts ; Ovary ; Uterus

Ovarian malignancy can rarely present as a colonic mass and simulate colon cancer. Mullerian anomalies are congenital anatomic abnormalities of the female internal genitalia, of which a unicornuate uterus is a rare subtype. Several cases of gynecologic malignancies arising in Mullerian anomalies have been described. However, an ectopic ovarian malignancy in conjunction with a unicornuate uterus has never been reported. We report a case of ectopic ovarian mixed epithelial tumor, a very rare subtype of ovarian epithelial tumor, which presented as a colonic mass simulating colon cancer in a woman with a unicornuate uterus.
Colon ; Colonic Neoplasms ; Female ; Genitalia ; Humans ; Mullerian Ducts ; Ovary ; Uterus

The diagnosis of the Herlyn-Werner-Wunderlich syndrome is frequently misleading, not only to underreporting of cases, but, more seriously, to suboptimal treatment that can be detrimental to the reproductive potential of the affected woman. The case of a 14-year old girl with the HWW syndrome is presented. Although the diagnosis was suggested by imaging modalities such as ultrasound and magnetic resonance imaging, the use of endoscopic procedures such as laparoscopy and hysteroscopy, provided additional vital information that allowed an accurate assessment of her condition and a definitive, minimally-invasive management that was protective of her reproductive function.
Human ; Female ; Adolescent ; MULLERIAN DUCTS ; HEREDITARY RENAL AGENESIS

A case of a 14 years old student diagnosed with congenital mullerian anomaly.
Human ; Female ; Adolescent ; VACTERL ASSOCIATION ; MULLERIAN DUCTS ; ABNORMALITIES

Herlyn-Werner-Wunderlich (HWW) syndrome is a rare, congenital genitourinary anomaly involving the Müllerian and Wolffian structures, and is characterized by the triad of uterine didelphys, obstructed hemivagina, and ipsilateral renal agenesis. It usually presents in adolescent girls in whom hematometrocolpos produces a pronounced mass effect and pain on the side of the obstructed hemivagina. Accurate diagnosis and surgical treatment can be delayed for several months or even years. Here, we report a case of a 12-year-old girl who presented to the emergency department with lower abdominal pain and mass that had lasted for 2 weeks. After the confirmation of HWW syndrome with magnetic resonance imaging, hysteroscopic septostomy was carried out as a definitive treatment. When we evaluate adolescent girls with lower abdominal pain and mass, we should consider the possibility of HWW syndrome.
Abdominal Pain ; Adolescent ; Child* ; Diagnosis ; Emergency Service, Hospital ; Female* ; Hematocolpos ; Humans ; Magnetic Resonance Imaging ; Mullerian Ducts ; Wolffian Ducts

The Currarino triad is a unique complex of congenital caudal anomalies, including anorectal malformation, sacral bony defect and presacral mass. This triad may be associated with Mullerian duct anomalies, such as duplication of the vagina and uterus. Each of these diseases has a familial tendency and sometimes coexist within families. But, when coexisting in familial cases, nearly all reported cases revealed mutations of the motor neuron and pancreas homeobox 1 (MNX1) gene. Familial cases of Currarino triad combined with Mullerian duct anomaly without MNX1 gene mutation are very rare. Here we report cases of mother and daughter, who had Currarino triad and Mullerian duct anomaly without MNX1 gene mutation, along with a brief literature review.
Genes, Homeobox ; Humans ; Mothers* ; Motor Neurons ; Mullerian Ducts ; Nuclear Family* ; Pancreas ; Uterus ; Vagina

A small number of patients have been described in whom normal male development of the external genitalia has occurred but whom the Mullerian ducts persist. The retention of Mullerian structures can be ascribed to the failure of the Sertoli cells to synthesize Mullerian duct inhibitory factor or to a defect in the response of the duct to that factor. We experienced a case of persistent Mullerian duct syndrome. which is phenotypically normal male with bilateral undescended testes, bilateral fallopian tubes and a uterus. Herein we report a case with brief review of the literatures.
Cryptorchidism ; Fallopian Tubes ; Female ; Genitalia ; Humans ; Male ; Mullerian Ducts ; Sertoli Cells ; Uterus