Obstructed hemivagina and ipsilateral renal anomaly (OHVIRA) is a rare congenital malformation of the female urogenital tract characterized by a triad of uterine didelphys, obstructed hemivagina, and ipsilateral renal anomaly. It was formerly known as Herlyn Werner Wunderlich Syndrome (HWWS). The syndrome usually presents with cyclic pelvic pain following menarche. Endometriosis is a prevalent complication. Magnetic resonance imaging (MRI) helps in diagnosing OHVIRA syndrome and endometriosis due to its high contrast resolution and objectivity. We reported a 13- year-old girl who was evaluated for cyclic pelvic pain after her menarche at 12 years of age. Magnetic resonance imaging (MRI) revealed two separate uterine cavities, services and vaginae, indicating didelphys. The left uterine cavity is filled with fluid, and the left hemivagina is dilated and filled with hyperintense and hypointense fluid on T1 and T2, respectively, indicating blood products. Left hemivagina dilatation implicated the presence of an obstructing vaginal septum. A single left adnexal cyst lesion with blood products was suggestive of an endometriotic cyst. Additionally, the left kidney was absent. A uterine didelphys with left hemivagina obstruction, hematometra, hematocolpos, and the ipsilateral ovarian endometriotic cyst was diagnosed. A final diagnosis of OHVIRA syndrome or HWWS was made, considering that she had no left kidney.MRI is a suitable diagnostic tool for precise anatomical delineation of the uterus, cervix, and vagina in uterovaginal disorders such as OHVIRA syndrome. MRI can also properly evaluate endometriosis and adhesion.
Persistent urogenital sinus (PUGS) is a rare anomaly whereby the urinary and genital tracts fail to separate during embryonic development. We report a three-year-old female child who was referred to the Sabah Women & Children Hospital, Sabah, Malaysia, in 2016 with a pelvic mass. She had been born prematurely at 36 gestational weeks via spontaneous vaginal delivery in 2013 and initially misdiagnosed with neurogenic bladder dysfunction. The external genitalia appeared normal and an initial sonogram and repeat micturating cystourethrograms did not indicate any urogenital anomalies. She therefore underwent clean intermittent catheterisation. Three years later, the diagnosis was corrected following the investigation of a persistent cystic mass posterior to the bladder. At this time, a clinical examination of the perineum showed a single opening into the introitus. Magnetic resonance imaging of the pelvis revealed gross hydrocolpos and a genitogram confirmed a diagnosis of PUGS, for which the patient underwent surgical separation of the urinary and genital tracts.
Male factor infertility which accounts for 30-50% of infertility is a major problem faced by married couples. Congenital absence of the vas deferens, though uncommon, remains the most common abnormality seen in extratesticular ductal and ejaculatory system, accounting for 1-2% of male infertility. It may be unilateral or bilateral. Association with renal abnormality has also been reported with congenital absence of vas deferens (1). The patients are asymptomatic and the congenital abnormality is usually detected when investigation for infertility is carried out. We present a case of an unusual presentation of congenital bilateral absence of vas deferens (CBAVD).
Gastroschisis is often found together with other extra intestinal conditions such as limb, spine, cardiac, central nervous system and genitourinary abnormalities. There are reports of its association with young maternal age. The cases presented here highlight the association of gastroschisis with limbs anomalies and young maternal age.
Transverse vaginal septum (TVS) is a rare obstructive genital tract anomaly. It is associated with primary amenorrhoea and typically presents with cyclical abdominal pain due to obstruction of the menstrual flow. Caudal regression syndrome (CRS) is also a rare congenital anomaly that is frequently associated with anomalies of the neurological, musculoskeletal, cardiac, genitourinary and gastrointestinal systems. Obstructive genital tract anomaly in CRS is exceptionally rare. This report describes the case of a girl in early adolescence with underlying CRS who presented with severe abdominal pain associated with primary amenorrhoea. Clinical and radiological assessment revealed 'haematocolpos' manifesting as a tender 20 weeks' size abdominal mass and an absent vaginal opening. TVS was identified during examination under anaesthesia. The patient subsequently underwent a successful vaginoplasty with no recurrence of symptoms after 2 years.
Variations in the urogenital vascular anomalies in the abdomen are very common. However, they warrant attention due to their importance in operative, diagnostic, and endovascular procedures. During routine dissection of abdomen in a male cadaver, unique urogenital vascular anomalies were observed. On the right side, the right renal artery was found to be originated from the abdominal aorta at the level of L2 and divided into five branches; the right testicular artery and inferior suprarenal artery originated from the lower branch. We also observed, accessory renal artery arising from abdominal aorta at the level of L3 and double renal veins on right side. On the left side, we found left renal artery originating from the abdominal aorta at the level of L2 and divided into two branches. Double testicular (medial and lateral) arteries were also observed. In addition to these vascular variations, bilateral kinking of ureter at the pelviureteric junction was also observed. Although the variations in the origin of urogenital vessels in the abdomen are common, deeper understanding of the urogenital vascular variations and their relations to adjacent structures is significant during surgical and radiological procedures.
Bisphenol A (BPA) is an endocrine disrupting chemical (EDC) that can disrupt the normal functions of the reproductive system. The objective of the study is to investigate the potential protective effects of Tualang honey against BPA-induced uterine toxicity in pubertal rats. The rats were administered with BPA by oral gavage over a period of six weeks. Uterine toxicity in BPA-exposed rats was determined by the degree of the morphological abnormalities, increased lipid peroxidation, and dysregulated expression and distribution of ERα, ERβ, and C3 as compared to the control rats. Concurrent treatment of rats with BPA and Tualang honey significantly improved the uterine morphological abnormalities, reduced lipid peroxidation, and normalized ERα, ERβ, and C3 expressions and distribution. There were no abnormal changes observed in rats treated with Tualang honey alone, comparable with the control rats. In conclusion, Tualang honey has potential roles in protecting the uterus from BPA-induced toxicity, possibly accounted for by its phytochemical properties.
Term, live abdominal pregnancy secondary to rupture of a uterine rudimentary horn is a rare condition. Pregnancies conceived in the rudimentary horn of the uterus usually rupture during early gestation and present as a catastrophic event. However, rarely, after rupture of the uterine horn the foetus may continue to grow in the abdominal cavity and reach term gestation. A primigravida with a term pregnancy was referred to our centre for caesarean section with ultrasonography findings of transverse lie and placenta previa. During surgery, a live baby was extracted from the abdominal cavity, revealing a bicornuate uterus with rupture of the rudimentary horn. The early peroperative diagnosis and prompt control of the bleeding with excision of the rudimentary horn and transfusion of multiple blood products saved the patient's life. The case is presented for its rarity and to highlight the importance of a high index of suspicion in cases presenting with abnormal foetal presentation.
We report a bizarre case of accessory larynx in an infant with OEIS syndrome (omphalocele, cloacal exstrophy, imperforated anus & spinal defects). This is the first reported case in literature of a duplicate accessory larynx which is a mirror image of the true larynx. A congenital duplication of the larynx is a rare anomaly and can present in various forms. In this case, the infant presented with recurrent lung infection and inability to wean off oxygen. Scope revealed severe laryngomalacia in addition to the accessory larynx. Hence, supraglottoplasty was done with aim to resolve the lung and airway problem.