A pig was confirmed to be a true hermaphrodite on the basis of gross and histomorphological studies of the genital organs. The genitalia was consisted of left ovary, oviduct, two coiled uterine horns, body of uterus alongwith right testis and an epididymis. Vagina and vulva were absent but male urethra with prostate gland was present. Grossly the size of all the genital organs appeared to be normal. Histomorphologically, testis and epididymis were underdeveloped as there was no clearcut spermatogenia and sertoli cells but Leydig cells were normal. The ovary presents normal histological features with some portion of testicular tissue. Degeneration of uterine epithelium was observed along with normal endometrial glands.
Animals ; Female ; Genitalia, Female/abnormalities ; Genitalia, Male/abnormalities ; Hermaphroditism/pathology/*veterinary ; Male ; Swine ; Swine Diseases/*pathology

Unilateral cervicovaginal agenesis in a didelphic uterus is an extremely rare congenital anomaly. Women born with this anomaly present with menarche associated with progressive dysmenorrhea and symptoms of worsening endometriosis. Presented here is a case of a 12 year old nulligravid who presented with progressive cyclic abdominal pain. Physical examination revealed a tender large right adnexal mass and presence of a vaginal canal and cervix tilted to the left. Sonography showed the presence of uterine didelphys, right hematosalpinx and hematocolpos. Preoperative impression was outflow tract obstruction probably due to Herlyn Werner-Wunderlich syndrome. However, intraoperatively, it was discovered this was a case of uterine didelphys with outflow tract obstruction of the right hemiuterus due to unilateral cervicovaginal agenesis and ipsilateral renal agenesis.

Human ; Female ; Congenital Abnormalities ; Genitalia, Female ; Gynecology ; Philippines ; Case Reports

BACKGROUNDFemale genital malformations represent miscellaneous deviations from normal anatomy. This study aimed to explore the clinical characteristics of patients who underwent surgery for genital tract malformations at Peking Union Medical College Hospital (PUMCH) during a 31-year period.

METHODSWe retrospectively reviewed surgical cases of congenital malformation of the female genital tract at PUMCH for a 31-year period, analyzed the clinical characteristics of 1634 hospitalized patients, and investigated their general condition, diagnosis, and treatment process.

RESULTSThe average patient age was 27.6 ± 9.9 years. The average ages of patients who underwent surgery for uterine malformation and vaginal malformation were 31.9 ± 8.8 years and 24.7 ± 9.0 years, respectively; these ages differed significantly (P < 0.01). Among patients with genital tract malformation, the percentages of vaginal malformation, uterine malformation, vulva malformation, cervical malformation, and other malformations were 43.9%, 43.5%, 7.4%, 2.3%, and 2.8%, respectively. Among patients with uterine malformation, 34.5% underwent surgery for the genital tract malformation, whereas in patients with vaginal malformation, the proportion is 70.6%; the difference between the two groups was statistically significant (P < 0.01). The percentage of complications of the urinary system in patients with vaginal malformations was 10.2%, which was statistically significantly higher than that (5.3%) in patients with uterine malformations (P < 0.01).

CONCLUSIONSCompared to patients with uterine malformations, patients with vaginal malformations displayed more severe clinical symptoms, a younger surgical age, and a greater need for attention, early diagnosis, and treatment. Patients with genital tract malformations, particularly vaginal malformations, tend to have more complications of the urinary system and other malformations than patients with uterine malformations.

Abnormalities, Multiple ; surgery ; Adolescent ; Adult ; China ; Female ; Genitalia, Female ; surgery ; Hospitals ; Humans ; Male ; Retrospective Studies ; Urogenital Abnormalities ; surgery ; Uterus ; abnormalities ; surgery ; Vagina ; surgery ; Young Adult

Biopsy ; Child, Preschool ; Clitoris ; pathology ; Disorders of Sex Development ; diagnosis ; pathology ; Female ; Genitalia, Female ; abnormalities ; Humans ; Hypertrophy ; Magnetic Resonance Imaging ; Neurofibromatoses ; diagnosis ; pathology ; Vulvar Neoplasms ; diagnosis ; pathology

PURPOSE: This study aimed to investigate the clinical features of congenital postural deformities and lower extremity asymmetry with respect to the presence of developmental dysplasia of the hip (DDH) in infants with a discrepancy of the limb length referred for suspected DDH. MATERIALS AND METHODS: We retrospectively reviewed the medical records and radiographs of 150 infants who visited Korea University Anam Hospital Orthopedic Clinic for suspected DDH between March 2013 and March 2015. RESULTS: There were greater numbers of infants with a shorter lower extremity on the left side (n=86, 57.3%) than the right. Plagiocephaly was present in 62 infants and trunk curvature in 124 infants (82.7%). Pelvic tilting—indirectly assessed by a skewed direction of the external genitalia in female infants—was present in 62 infants (63.3%). None of the 139 infants with normal physical examination of the hip were diagnosed with DDH. Of those 11 infants with abnormal findings from the hip physical examination, a total of 6 infants were diagnosed with DDH. CONCLUSION: Regardless of the associated findings of congenital postural deformation, all infants diagnosed with DDH had abnormal findings from the physical examination of the hip joint. Thus, we conclude that the hip examination is important as the primary clinical screening in aiding the diagnosis of DDH.
Congenital Abnormalities ; Diagnosis ; Extremities ; Female ; Genitalia ; Hip Joint ; Hip* ; Humans ; Infant* ; Korea ; Lower Extremity* ; Mass Screening* ; Medical Records ; Orthopedics ; Physical Examination ; Plagiocephaly ; Retrospective Studies

PURPOSE: Congenital absence of the vagina is a rare case. It occurs as a result of Mullerian duct aplasia or complete androgen insensitivity syndrome. The reconstructive modality includes skin graft, use of intestine and various methods of flap. We report a patient who underwent vulvoperineal fasciocutaneous flap to reconstruct congenital absence of the vagina, while the external genitalia and ovaries are normal. METHODS: A 26-year-old woman presented with vaginal agenesis. Under general anesthesia, a U-shaped incision was made between the urethral meatus and the anus. The new vaginal pocket was created up to the level of the peritoneal reflection between the urinary structures and the rectum. Next, the vulvoperineal fasciocutaneous flaps were designed in a rectangular fashion. Flap elevation was begun at the lateral margin which the adductor longus fascia was incised and elevated, and the superficial perineal neurovascular pedicle was invested by the fascial layer. The medial border was then elevated. A subcutaneous tunnel was created beneath the inferior of the labia to rotate the flaps. The left vulvoperineal flap was rotated counterclockwise and the right was rotated clockwise. The neovaginal pouch was formed by approximating the medial and lateral borders. The tubed neovagina was then transposed into the cavity. RESULTS: In 3 weeks, the vaginal canal remained supple After 6 weeks, the physical examination showed normal-appearing labia majora and perineum with an adequate vaginal depth. A year after the operation, the patient had a 7 cm vagina of sufficient width with no evidence of contractures nor fibrous scar formation. The patient was sexually active without difficulty. CONCLUSION: Although many methods were described for reconstruction of vaginal absence, there is not a method yet to be approved as a perfect solution. We used the vulvoperineal fasciocutaneous flap to reconstruct a neovagina. This method had a following merits: a single-stage procedure, excellent flap reliability, the potential for normal function, minimal donor site morbidity and no need for subsequent dilatation, stents, or obturators. We thought that this operation has a good anatomic and functional results for reconstruction of the vagina.
Adult ; Anal Canal ; Androgen-Insensitivity Syndrome ; Anesthesia, General ; Cicatrix ; Congenital Abnormalities ; Contracture ; Dilatation ; Fascia ; Female ; Genitalia ; Humans ; Intestines ; Male ; Ovary ; Perineum ; Physical Examination ; Polyenes ; Rectum ; Skin ; Stents ; Tissue Donors ; Transplants ; Vagina

VACTERL association is occuring in conjuction with vertebral anomalies, anal atresia, cardiac abnormalities, tracheoesophageal fistula, renal agenesis and limb defects. Additional abnormalities may include microphthalmia, abnormal lung lobulation and spleen defects. We describe a infant born to consanguineous healthy parents with multiple congenital anomalies of the skeleton and internal organs. A woman at 25 weeks gestaion was transferred to our hospital due to abnormal antenatal sonographic finding(molded calvarium, huge mass in abdomen, hyperechoic intestine and femur bowing). She admitted to our hospital due to decreased fetal movement 3 weeks later. Fetal death in uterus was diagnosed by sonogram. Its phenotype displays imperforated anus, absence of genital organ, absence of utrethral opeining, disunion of maxilla and mandible, varus deformity of bilateral knee joints, widening of interphalangeal space in right foot. The diagnosis was confirmed by clinical features, radiological findings, and autopsy. We reported this case of VACTERL association with review of literatures.
Abdomen ; Anal Canal ; Anus, Imperforate ; Autopsy* ; Congenital Abnormalities ; Diagnosis ; Extremities ; Female ; Femur ; Fetal Death ; Fetal Movement ; Foot ; Genitalia ; Humans ; Infant ; Intestines ; Knee Joint ; Lung ; Mandible ; Maxilla ; Microphthalmos ; Parents ; Phenotype ; Skeleton ; Skull ; Spleen ; Tracheoesophageal Fistula ; Ultrasonography ; Uterus