RESUMO
@#Obstructed hemivagina and ipsilateral renal anomaly (OHVIRA), or Herlyn–Werner–Wunderlich syndrome, is a rare Müllerian duct anomaly that is characterized by a triad of uterus didelphys, unilateral obstructed vagina, and ipsilateral renal agenesis. Patients with this anomaly usually present after menarche with abdominal pain or a pelvic mass which worsens over some time. Herein, presented is a case of a 12‑year‑old nulligravid with cyclic pelvic pain and palpable pelvic mass after her menarche. A series of diagnostic tests were done which were compatible with the diagnosis of OHVIRA with an associated finding of ectopic ureteral insertion into the obstructed hemivagina and nonfunctional urinary bladder. The patient underwent diagnostic hysteroscopy, cystoscopy, and full resection of the longitudinal vaginal septum as well as drainage of hematocolpos, which are the main treatment of the patient with OHVIRA.
Assuntos
Humanos , Ureterocele , Vagina , ÚteroRESUMO
Herlyn-Werner-Wunderlich syndrome (HWWS) is a very rare congenital anomaly of the urogenital tract involving Mullerian ducts and Wolffian ducts, and is characterized by the triad of uterine didelphys, unilateral obstructed hemivagina and ipsilateral renal agenesis. Generally it is diagnosed at puberty after menarche due to recurrent pelvic pain or abdominal mass. We report 2 cases of female newborns whose fetal ultrasonography (USG) showed unilateral renal agenesis and were diagnosed with HWWS by postnatal evaluation. Both cases were female newborns who were born at term by vaginal delivery. They had no perinatal problems except suspicious findings of unilateral renal agenesis from fetal USG. Abdominal ultrasonography and pelvic MRI were performed after birth, and they were diagnosed with HWWS. The potential complications of this syndrome such as pyosalpinx, pelvic adhesions and increased risk of abortion or infertility can occur, but without complication, the prognosis is very good with simple surgical treatment. If renal agenesis is detected in a fetus or a newborn, possible anomalies of urogenital tract such as HWWS should be considered; and postnatal evaluation should be performed, as a simple surgical treatment before menarche can prevent unnecessary complications of disease.
Assuntos
Feminino , Humanos , Recém-Nascido , Anormalidades Congênitas , Feto , Infertilidade , Rim , Nefropatias , Menarca , Ductos Paramesonéfricos , Parto , Dor Pélvica , Prognóstico , Puberdade , Ultrassonografia Pré-Natal , Anormalidades Urogenitais , Ductos MesonéfricosRESUMO
Seminal vesicle cysts have been rarely detected. Most of them are caused congenitally, and two- thirds of them are accompanied with ipsilateral renal agenesis or dysplasia. They are usually present with dysuria, urinary frequency, perineal pain, epididymitis, pain after ejaculation, scrotal pain or infertility in the second to fourth decade of patient's life. Occasionally cysts are palpable by digital rectal examination, but radiologic imaging study is necessary to diagnose. We report a case of an infant with seminal vesicle cyst accompanied with ipsilateral renal agenesis detected incidentally in postnatal sonogram. The infant's right side of kidney was diagnosed as antenatally multicystic dysplastic kidney.
Assuntos
Humanos , Lactente , Masculino , Anormalidades Congênitas , Exame Retal Digital , Disuria , Ejaculação , Epididimite , Infertilidade , Rim , Nefropatias , Rim Displásico Multicístico , Glândulas SeminaisRESUMO
Uterine malformations consist of a group of miscellaneous congenital anomalies of the female genital system, and these anomalies are the result of major disturbances in the development, such as formation or fusion of the Mullerian or paramesonephric ducts during fetal life. The Mullerian anomalies have been estimated to occur in up to 0.001~10%, and these anomalies may be associated with numerous congenital anomalies, and ipsilateral renal anomalies especially are the most common. The uterine didelphys with obstructed hemivagina and ipsilateral renal agenesis clinically may result in dysmenorrhea, low abdominal pain, endometriosis or pelvic mass after menarche. Furthemore the delayed diagnosis causes infertility and poor pregnancy outcome due to continued retrograde menstruation which leads to endometriosis and distorted pelvic anatomy. These anomalies could be diagnosed with ultrasonography and MRI that have the high sensitivity and specificity. Excision of vaginal septum and marsupialization are appropriate treatments to relieve symptoms and reserve fertility. We report 7 cases of uterine didelphys with obstructed hemivagina and ipsi-unilateral renal agenesis with a brief review of relevant literatures to help understand these anomalies.