Herlyn-Werner-Wunderlich (HWW) syndrome is a rare congenital malformation syndrome that is characterized by a triad of uterine didelphys, blind hemivagina, and ipsilateral renal agenesis. There is a wide variety of phenotypic presentation which is recognized as a spectrum of disease rather than a separate entity. The exact incidence and pathogenesis of HWW syndrome are yet to be investigated. While this disease typically involves adolescent girls who present with abdominal pain or a pelvic mass that is secondary to hematocolpos, nowadays, a majority of potential patients with HWW are being prenatally screened for renal anomalies. Therefore, it is recommended to search for uterovaginal anomalies whenever a multicystic dysplastic kidney or the absence of a kidney is noted in a newborn female, and the role of pediatric nephrologists has become ever more important for early recognition of the disease.
Abdominal Pain ; Adolescent ; Female ; Hematocolpos ; Humans ; Incidence ; Infant, Newborn ; Kidney ; Multicystic Dysplastic Kidney

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

We present a case of complete vaginal stenosis in a woman diagnosed with Sheehan’s syndrome. The patient delivered at full-term 5 months prior, and experienced massive postpartum bleeding at that time. During evaluation of persistent amenorrhea, we found that her vaginal orifice was completely adhesive and obstructed. Prior to corrective surgery, we managed the patient with an oral contraceptive to induce uterine bleeding into the vaginal outflow tract. After three cycles of an oral contraceptive, we could confirm that there was no stenotic lesion in the vaginal cavity as a hematocolpos was created. Adhesiolysis with scar revision for the vaginal stenosis was successfully performed; it was found that the lesion was limited to only the distal part of the vaginal outlet. Complete vaginal stenosis in reproductive age women with hypopituitarism has not been reported. The artificial induction of hematometrocolpos before surgery was useful in determining the extent of the stenotic lesion, and assured safety.
Adhesives ; Amenorrhea ; Cicatrix ; Constriction, Pathologic* ; Female ; Hematocolpos ; Hemorrhage ; Humans ; Hypopituitarism ; Postpartum Period ; Uterine Hemorrhage

We report the case of a 14-year-old girl who visited the emergency room because of suprapubic discomfort and sudden acute urinary retention. She did not have any significant medical and surgical history, and her neurological examinations were all normal. Urinary catheterization led to the passage of 500 mL urine. Abdominal ultrasonography showed a hematocolpos that was compressing the urinary bladder. Gynecologic history taking revealed that the patient has not had menarche yet. Therefore, a cruciate incision was performed and her urination became normal. As the surgical outcome after adequate hymenotomy for imperforate hymen is usually good, the diagnosis of imperforate hymen is important. However, this condition is easily missed in the clinic because the first physician visited by the patient rarely takes a detailed gynecologic history or performs appropriate physical examinations. Although rare, imperforate hymen should be considered as a cause of acute urinary retention in the adolescence period. If an adolescent girl presents with abdominal pain and voiding dysfunction, a detailed gynecologic history and appropriate physical examinations of the genital introitus should be performed.
Abdominal Pain ; Adolescent* ; Diagnosis ; Emergency Service, Hospital ; Female ; Hematocolpos* ; Humans ; Hymen* ; Menarche ; Neurologic Examination ; Physical Examination ; Ultrasonography ; Urinary Bladder ; Urinary Catheterization ; Urinary Catheters ; Urinary Retention* ; Urination

Allogenic peripheral blood stem cell transplantation (Allo-PBSCT) is being used to treat hematological malignancies with increasing frequency. Graft-versus-host disease (GvHD) is a complex complication of PBSCT. A 43-year-old woman came to the gynecology clinic for amenorrhea. She had been diagnosed with acute myeloid leukemia 2 years earlier and treated with induction and consolidation chemotherapy. After developing complete remission, she underwent Allo-PBSCT. When she started chemotherapy, her menstrual cycle completely disappeared. Fourteen months after menopausal hormone replacement therapy, it was discovered that her upper vaginal canal was completely obstructed. The lower vagina had an atrophic appearance. We report a rare case of partial vaginal obstruction as a complication of chronic GvHD and review the literature. We expect that this case report provides an opportunity to remind clinician of the gynecologic complications of GvHD.
Amenorrhea ; Consolidation Chemotherapy ; Female ; Graft vs Host Disease ; Gynecology ; Hematocolpos ; Hematologic Neoplasms ; Hormone Replacement Therapy ; Humans ; Leukemia, Myeloid, Acute ; Menstrual Cycle ; Peripheral Blood Stem Cell Transplantation ; Porphyrins ; Vagina

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

Vaginal agenesis is an uncommon congenital anomaly and such complex malformations are usually incorrectly identified. Deviations from the normal embryologic events in the reproductive system may result to malformations which may exhibit with the same clinical presentation. A complete history and physical examination coupled with appropriate imaging techniques are necessary to arrive at a correct diagnosis. Physicians must be aware of the differential diagnosis and management options available. A correct diagnosis must be made prior to any surgical intervention to prevent inappropriate surgeries with their anticipated complications. In cases with vaginal outlet obstruction, an accurate diagnosis with the adequate treatment reduces the need for re-operations and subsequent complications. Reported are three misdiagnosed cases of distal vaginal agenesis resulting to unwarranted surgical interventions.
Human ; Female ; Adolescent ; ABNORMALITIES ; VAGINA ; HEMATOCOLPOS ; HEMATOMETRA ; ROKITANSKY KUSTER HAUSER SYNDROME

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

Three-dimensional ultrsonography has been applied to diagnose mullerian anomalies. However, in Korea, no such case has been reported in literatures. An adolescent female was evaluated for dysmenorrhea, and a hematocolpos was found at private clinic. We performed three-dimensional ultrsonography and found uterus didelphys by confirming seperated uterine fundi and cervices. Abscence of right kidney was also found by ultrsonography. The diagnosis of Herlyn-Werner-Wunderlich syndrome was made and marsupialization of the right hemivagina was performed, with relief of symptoms.
Adolescent ; Dysmenorrhea ; Female ; Hematocolpos ; Humans ; Imaging, Three-Dimensional ; Kidney ; Korea ; Uterus

The unique clinical syndrome of the uterus didelphys, a unilateral partially or completely obstructed vagina, and ipsilateral renal agenesis is quite rare, and observed on the right side in greater frequency than on the left. The case of fifteen year old girl with uterus didelphys, unilateral partially obstructed vagina and acute renal failure associated with ureteral stone in unilateral kidney is described. Extracorporealshock wave lithotripsy (ESWL) and evacuation of the hematocolpos and excision of the vaginal septum were done. Aware of this relatively rare condition is mainstay of prompt diagnosis which will prevent permanent renal failure and unnecessary and destructive surgery.
Acute Kidney Injury ; Congenital Abnormalities ; Female ; Hematocolpos ; Kidney ; Kidney Diseases ; Lithotripsy ; Renal Insufficiency ; Ureter ; Uterus ; Vagina