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Obstructed Hemivagina Ipsilateral Renal Agenesis (OHVIRA) Syndrome-A Retrospective Cohort Analysis of the Sabah Population

Lim LM, Sivapatham L, Chong ASL, Wahab AVA

J Pediatr Adolesc Gynecol, 2023 Dec;36(6):541-544.
PMID: 37451429 DOI: 10.1016/j.jpag.2023.07.002

OBJECTIVES: The objectives of the study were to compare the demographic characteristics, presenting complaints, timeliness of diagnosis, and treatments given to all patients diagnosed with obstructive hemivagina ipsilateral renal agenesis (OHVIRA) between the adolescent group (age 10-19) and the adult group (age 20 and above) and to propose a feasible screening test consisting of routine bedside ultrasound to detect renal anomalies in addition to pelvic ultrasound during the assessment of females with gynecologic complications in resource-limited settings.
METHOD AND ANALYSIS: We conducted a retrospective cohort analysis of all patients with OHVIRA at our institution, Sabah Women and Children's Hospital, Malaysia, between the 2013 and 2022. Data were collected by reviewing patients' medical notes.
RESULTS: There were a total of 18 patients diagnosed with OHVIRA from 2013 to 2022, aged 10-41 years old. Nine patients (50%) presented during adolescence. Most (88.9%) came with symptoms such as abdominal pain, urinary symptoms, abnormal uterine bleeding, foul-smelling vaginal discharge, and primary infertility, whereas only 2 patients (11.1%) were asymptomatic and diagnosed incidentally. Acute abdomen was more common in the adolescent group (P = .015). No significant difference was found on the side of the renal anomaly.
CONCLUSION: Presenting symptoms vary and are often mimicked other gynecologic or surgical conditions, making the diagnosis difficult and delayed. Some patients were incidentally diagnosed while being managed for other problems, and it was not uncommon to have unnecessary surgery before the correct diagnosis was made. We suggest that all female patients with abdominal and pelvic complaints should be screened for renal anomaly during pelvic scan to improve diagnostic rates.

Matched MeSH terms: Uterus/abnormalities
Case report of ruptured non-communicating right rudimentary horn pregnancy: an acute emergency

Hassan CH, Karim AK, Ismail NA, Omar MH

Acta Medica (Hradec Kralove), 2011;54(3):125-6.
PMID: 22250483

Ruptured pregnancy in the rudimentary horn of women who have had a vaginal delivery is rare and unpredictable. However, when undiagnosed, this condition could lead to maternal morbidity and mortality. We report a pregnancy at 19 weeks gestation presented with acute abdomen and hypovolemic shock. She was initially thought to have an intrauterine pregnancy with the provisional diagnosis of a ruptured uterus. Intraoperatively, a ruptured non-communicating right rudimentary horn with ex utero pregnancy was discovered.

Matched MeSH terms: Uterus/abnormalities*
An unusual cause of antepartum haemorrhage

Soh EB, Lim JM

Aust N Z J Obstet Gynaecol, 1999 Aug;39(3):389-90.
PMID: 10554965

This is a rare case of antepartum haemorrhage arising from the nonpregnant uterus in a woman with uterine didelphys. The bleeding and subsequent passage of a decidual cast did not have any adverse effect on the ongoing pregnancy.

Matched MeSH terms: Uterus/abnormalities*
A case of rudimentary horn pregnancy that reached term

Ngieng LN

Med J Malaya, 1970 Sep;25(1):65-7.
PMID: 4249500
Matched MeSH terms: Uterus/abnormalities
Successful pregnancy outcome following gamete intra-Fallopian transfer in a patient with Müllerian dysgenesis

Lee CS, Lie AT

Reprod Biomed Online, 2012 May;24(5):547-9.
PMID: 22410277 DOI: 10.1016/j.rbmo.2012.01.021

A 29-year-old lady with Müllerian dysgenesis was keen to have a baby. Clinically, she was medium built with well-developed secondary female sexual characteristics. There was a short and blind vagina. She had undergone surgery for an imperforated hymen. Her FSH and LH concentrations were normal. Laparoscopy revealed a patent right Fallopian tube, a rudimentary right uterus and extensive pelvic endometriosis. She subsequently underwent gamete intra-Fallopian transfer (GIFT). Oocyte retrieval was carried out laparoscopically and a total of nine oocytes were retrieved. Four of the oocytes were transferred together with motile spermatozoa into the right Fallopian tube and the remaining five oocytes were inseminated with spermatozoa for IVF. Three embryos resulted and were frozen. She subsequently developed moderate ovarian hyperstimulation syndrome. Serum β-human chorionic gonadotrophin concentration 14 days after GIFT was 1612 IU/l. Her antenatal care was relatively uneventful until 31 weeks of gestation when she was diagnosed to have intrauterine growth retardation and oligohydramnios. She then underwent an emergency Caesarean section at 32 weeks of pregnancy delivering a normal baby. This case study describes a successful pregnancy outcome following gamete intra-Fallopian transfer (GIFT) in a woman with malformation of the vagina (Müllerian dysgenesis). A 29-year-old lady with Müllerian dysgenesis diagnosed at 16 years of age was keen to become pregnant. Upon examination, a decision was made for a William's vulvovaginoplasty but as the patient was indecisive the surgery was deferred. Clinically, she is a medium-built lady with well-developed secondary female sexual characteristics. There was a short and blind vagina. Her serum FSH and LH concentrations were normal. Laparoscopy revealed a patent right Fallopian tube, a rudimentary right uterus and extensive pelvic endometriosis. She subsequently underwent GIFT. Nine oocytes were retrieved through laparoscopy. Four of the oocytes were transferred together with motile sperm into the right Fallopian tube and the remaining five oocytes were inseminated with sperm for IVF. Three embryos resulted and were frozen. Serum β human chorionic gonadotrophin concentration measured 14 days after GIFT was 1612 IU/l. An abdominal ultrasonography performed at 5 weeks showed one intrauterine gestational sac. Her antenatal care was uneventful until 31 weeks of gestation when she developed a deficiency of amniotic fluid in the amniotic sac. She then underwent an emergency Caesarean section at 32 weeks of pregnancy. She delivered a healthy, normal 1.24 kg baby boy. Her post-natal care was uneventful.

Matched MeSH terms: Uterus/abnormalities
Diagnostic pitfalls in the evaluation and management of amenorrhea in adolescents

Azurah AG, Zainuddin AA, Jayasinghe Y

J Reprod Med, 2013 Jul-Aug;58(7-8):324-36.
PMID: 23947083

Amenorrhea is a common menstrual problem seen in adolescents. Amenorrhea has been shown to have a negative impact on adolescents' quality of life. In this paper we discuss the various causes and investigations of amenorrhea in adolescents and address management dilemmas for specific conditions. Specific approaches in dealing with adolescents using the HEADSS (Home, Education, Activity, Drugs, Sexual activity, Suicidal) approach are discussed.

Matched MeSH terms: Uterus/abnormalities