Mullerian anomalies arise from the failure in the development of Mullerian ducts and their associated structures during organogenesis which confers adverse impact in fertility and reproductive health. Presented is a rare case of a 15 year old nulligravid, who presented with a chief complaint of severe cyclic hypogastric pain associated with primary amenorrhea. Complete clinical history, physical examination and sonographic findings pointed to a diagnosis of cervical hypoplasia associated with functioning uterine corpus and an absent vagina. Patient underwent total abdominal hysterectomy with left salpingectomy and bilateral oophorocystectomy, for hematometra, bilateral endometriotic cysts, and hematosalpinx. This case report discusses the management of cervicovaginal agenesis through a multidisciplinary approach by a team composed of an obstetrician-gynecologist, reproductive endocrinologist, pediatrician, and pediatric surgeon for proper evaluation, diagnosis, and management of this case.
Hematometra

Imperforate hymen is a rare congenital malformation. Two cases of imperforate hymen regarded to congenital origin, and formed hematocolpometra. In most instances definite finding is noticed after menarche as retention of menstrual flow results in abdominal pain, distension of the lower abdomen and often in acute urinary retention. Two cases of imperforate hymen associated with hematocolpora, hematometra and abdominal distension at age of 14 and 16 were treated satisfactorily by simple crucial incision of hymen, and drained of about 800 cc and 600 cc of tarry blood respectively. We present two cases of imperforate hymen with review of the literatures.
Abdomen ; Abdominal Pain ; Female ; Hematometra ; Hymen* ; Menarche ; Urinary Retention

Adult ; Female ; Hematometra ; pathology ; Humans ; Uterine Neoplasms ; pathology ; Uterus ; pathology

A 24 year-old patient, single, with cervical agenesis and imperforate hymen is presented. She underwent hymenectomy for hematosalpinx and right oophorectomy for endometriosis, before finally undergoing hysterectomy. Cervical agenesis is a rare anatomic developmental abnormality and proposed guidelines in managing similar conditions are reviewed in this report. The management of choice will defend on the condition of the patient and the availability of resources. The patient underwent multiple surgeries in the past. However, despite all efforts to address her medical condition, due to the complications, the small chance of getting pregnant, and the absence of an experienced surgeon capable of performing a reconstructive procedure, she was eventually convinced to undergo hysterectomy. With cervical agenesis, the patient is faced with psychological difficulties that should be addressed. One can only hope that the future will hold a more optimistic outlook for young girls with this rare congenital abnormality.
Human ; Female ; Young Adult ; ABNORMALITIES ; HEMATOMETRA ; HEMATOCOLPOS ; HYSTERECTOMY

A not so benign disease of imperforate hymen in a 20 year old who presented with acute hypogastric pain will be discussed. The diagnosis of an imperforate hymen should be made during routine neonatal pediatric examination. However, it is not unusual to see a teenage girl present with typical findings of cyclic adominal pain, urinary retention, constipation and a bulging introital mass with hematocolpos and hematometra on ultrasounds examination. Additional findings may include hematosalpinges and formation of endometrial cysts with severe pelvic adgesions. Conservative management generally includes hymenectomy with drainage of the blood and antibiotic therapy. Patients are generally discharged after 24 hours or the procedure is done as an outpatient basis with oral pain medications given. However in our case, the dilemma in terms of management of conservative versus aggressive surgery was encountered. Our patient was only 20 years old presenting with primary amenorrhea and cyclic dysmenorrhea for 7 years with the possibility of a pelvic "clean up" and the problems of premature menopause and infertility were at the back of our minds. A greater awareness of this complication should prompt clinicians to consider this entity when dealing with cases such as this one.
Human ; Female ; Young Adult ; HEMATOCOLPOS ; HEMATOMETRA ; VAGINA ; HYMEN

Transverse vaginal septum, especially completely imperforate condition, is a rare congenital anomaly of female genital tract. The patient was 26 years old and visited for primary amenorrhea and low abdominal pain. Secondary sexual characteristics were all normal, but the depth of the vagina was only about 1cm. Hematometra was suspected and two large cystic structures were detected in the pelvis on ultrasonography. Intravenous pyelography and other laboratory results were normal. Vaginoplasty was needed to drain out the hematometra. We tried a new surgical technique using radial incisions of the closed vaginal end. To prevent postoperative contracture, we used a sterile pen case. and educated the patient to dilatate the vagina with that, daily. Here, we report a case of complete imperforate transverse septum with reviews of literatures.
Abdominal Pain ; Adult ; Amenorrhea ; Contracture ; Female ; Hematometra ; Humans ; Pelvis ; Ultrasonography ; Urography ; Vagina

Unicornuate uterus with a rudimentary horn hematometra and bicornuate uterus with unilateral hematometra is rare congenital anatomic anomalys of the female genital system. these patients present with dysmenorrhea, dyspareunia, pelvic mass, urinary tract anomalies and pelvic endometriosis. We have experienced a case of rudimentary uterine horn hematometra with agenesis of ipsilateral kidney and pelvic endometriosis in a 18 years old girl and a case of bicornuate uterus with unilateral hematometra associated with agenesis of ipsilateral kidney and pelvic endometriosis in a 14 years old girl. and we treated by laparoscopic management. So we report two cases with a brief review of literatures.
Adolescent ; Animals ; Dysmenorrhea ; Dyspareunia ; Endometriosis* ; Female ; Hematometra ; Horns ; Humans ; Kidney* ; Urinary Tract ; Uterus

Double uterus with unilateral hematometra and ipsilateral renal agenesis is a rare Mullerian duct malformation. This complex of anomalies is seen in adolescents and young women with progressive dysmenorrhea, abdominal pain, menstrual irregularities and a pelvic mass. Appropriate preoperative diagnosis and treatment will prevent unnecessary procedure and offer relief of symptoms. We report one case of bicornuated uterus with unilateral cervical atresia and homolateral renal agenesis with a brief review of concerned literatures.
Abdominal Pain ; Adolescent ; Diagnosis ; Dysmenorrhea ; Female ; Hematometra ; Humans ; Unnecessary Procedures ; Uterus*

The incidence of all transverse vaginal septum is 1:2,100 - 1:72,000. The transverse vaginal septum is a developmental defect of vagina which may be fused incompletely between the Mullerian duct component and the urogenital sinus component of vagina. Serious complications may be hematocolpos, hematometra and hematosalpinx. Simple surgical excision or simple incision have been developed to treat congenital transverse vaginal septa, but also caused common complications such as secondary tissue contracture. Garcia technique using eight vaginal mucosa flaps was developed to avoid common complication of secondary vaginal stenosis. Here, we present a case of contracted transverse vaginal septum who has been performed a simple incision 13 years ago, successfully performed the modified Garcia technique without serious postoperative complication.
Constriction, Pathologic ; Contracture ; Female ; Hematocolpos ; Hematometra ; Incidence ; Mucous Membrane ; Postoperative Complications ; Vagina

PURPOSE: To assess the role of MRI in the diagnosis of uterine anomaly. MATERIAL AND METHODS: MRI(n:15), hysterosalpingography(n:7) and ultrasonography(n:7) were performed in 15 patients with suspected MullerJan duct anomaly. Nine cases were proved by operation and six cases were diagnoed with imaging and clinical findings. According to Buttram and Gibbons modified classification, the anomalies were 4 cases of class I, 2 cases of class III, one case of class IV, and 8 cases of class V. RESULTS: MRI enabled accurate diagnoses of anomalies in all cases, but HSG and USG showed wrong diagnoses in 3 of 7 cases and in 1 of 7 cases. CONCLUSION: MRI, especially T2-weighted images parallel to long axis of uterine corpus, was very useful in diagnosis of the Mullerian duct anomaly, because it could depict exactly the external fundal contour, intercornual distance, septum, transverse vaginal septum, and associated abnormalities such as hematocolpos and hematometra.
Axis, Cervical Vertebra ; Classification ; Diagnosis ; Female ; Hematocolpos ; Hematometra ; Humans ; Hylobates ; Magnetic Resonance Imaging*