Obstructed Hemivagina and Ipsilateral Renal Anomaly (OHVIRA) Syndrome, also known as Herlyn-Werner-Wunderlich Syndrome, is a rare congenital anomaly of the female urogenital tract. OHVIRA syndrome results from abnormal development of the Mullerian duct during weeks 10-12 of gestation. OHVIRA syndrome is characterized by a classic triad of uterine didelphys, obstructed hemivagina, and ipsilateral renal anomaly. Clinical presentation of OHVIRA syndrome depends on the degree of hemivaginal obstruction, with Type 1 defined as complete obstruction and Type 2 characterized by incomplete obstruction. In patients with complete hemivagina obstruction, OHVIRA syndrome may manifest in adolescence with pelvic distention and cyclic pain secondary to hematometrocolpos. Several renal anomalies have been described in OHVIRA syndrome including renal agenesis, multicystic dysplastic kidney, and duplicated collecting system. In this report, we present two cases of OHVIRA syndrome with different manifestations of unilateral renal anomaly. The first case features a 12-year-old female who presented with heavy menstrual bleeding. Transpelvic ultrasound demonstrated possible uterine septation and a dilated cervix containing heterogeneous avascular material. Subsequent pelvic MRI revealed duplicated uterine horns with two separate cervices, consist with uterus didelphys. On MRI, the left cervix was distended with non-enhancing debris, suggestive of obstructed left hemivagina. No left kidney was identified in the left renal fossa or elsewhere in the abdomen or pelvis on renal ultrasound. Multiple non-communicating cysts were seen in the left abdomen, raising suspicion for multidysplastic left kidney with arrested ascent. The second case involves a 10-year-old prepubertal female who presented from an outside hospital for evaluation of a uterine anomaly, which was incidentally seen on MRI pelvis performed for characterization of sacrococcygeal teratoma. Transpelvic ultrasound and pelvic MRI at our institution showed duplicated uterine horns with limited visualization of the cervices due to small size. Differential considerations included uterus didelphys and bicornuate uterus, and follow-up imaging was recommended after puberty for definitive diagnosis. MRI pelvis obtained 4 years later demonstrated complete duplication of the uterus, cervix, and vagina with obstruction of the right hemivagina. MRI pelvis also demonstrated right renal agenesis, consistent with OHVIRA syndrome. Read More

Meeting name: SPR 2025 Annual Meeting , 2025

Authors: Merriam Caroline, Ehrlich Lauren

Keywords: Mullerian Duct Anomalies, Congenital

Scimitar syndrome, also known as congenital pulmonary venolobar syndrome, is a rare variant of partial anomalous pulmonary venous return (PAPVR), typically involving the right-sided pulmonary veins. Scimitar syndrome is usually characterized by drainage of the right lung by an anomalous right pulmonary vein, which may empty into the IVC, azygos vein, hepatic vein, coronary sinus, or right atrium. On frontal chest radiograph, the anomalous right pulmonary vein appears as a curvilinear density in the right lung, similar to the shape of a Turkish scimitar sword. Associated imaging features of Scimitar syndrome are variable, but often include right lung hypoplasia and systemic arterial supply to the abnormal right lung segments. Left-sided Scimitar syndrome is extremely rare, with only a handful of cases reported in the literature. In this report, we present a case of left scimitar syndrome with anomalous left pulmonary vein draining into the left renal vein. A 3-year-old female twin, born at term, presented to pediatric cardiology for evaluation of an asymptomatic murmur detected during a routine well-child check. Physical exam revealed a benign Still's murmur. However, ECG demonstrated right ventricular hypertrophy, warranting further evaluation with echocardiogram. Echocardiogram showed moderate left pulmonary artery hypoplasia and poor delineation of the left pulmonary veins. Subsequent cardiac MRI demonstrated absence of normal left pulmonary veins draining into the left atrium and Qp:Qs of 1.5, raising concern for a left-to-right shunt in the setting of anomalous left pulmonary venous return. Further evaluation with cardiac CTA delineated drainage of the entire left lung by a single vertical anomalous left pulmonary vein which coursed below the diaphragm into the left renal vein. The left renal vein then emptied into a moderately dilated IVC. Normal right sided pulmonary venous return into the left atrium was noted. No fissure was visualized in the left lung, which appeared to contain a single lobe. No definite systemic arterial supply to the left lung was identified on cardiac CTA. Imaging findings were compatible with left-sided scimitar syndrome. After discussion of the case at multidisciplinary conference, surgical management was deferred, as the patient was asymptomatic. The patient will be followed with yearly echocardiogram and ECG to monitor for developing stenosis of the anomalous left pulmonary veins. Read More

Meeting name: SPR 2025 Annual Meeting , 2025

Authors: Merriam Caroline, Ehrlich Lauren

Keywords: Congenital Heart, Anomalies