An 18 yr old female referred to us as hypertrophic obstructive cardiomyopathy with bicuspid aortic valve. On Initial history and examination patient also had primary amenorrhoea, differential cyanosis in lower limbs, differential clubbing, polydactyly, syndactyly, high arched foot, pectus carinatum and scoliosis. Oxygen saturation was 94% at room air and complete blood count was suggestive of p...

Many debates surrounded the most appropriate approach of mullerian anomalies diagnosis. In congenital uterine anomalies 3D ultrasound is critical in reaching the correct diagnosis, by means of assessing the coronal plane of the uterus. Canalization defects reduce fertility and increase rates of miscarriage and preterm delivery. Unification defects seem to preserve fertility, but some are associ...

Many debates surrounded the most appropriate approach of mullerian anomalies diagnosis. In congenital uterine anomalies 3D ultrasound is critical in reaching the correct diagnosis, by means of assessing the coronal plane of the uterus. Canalization defects reduce fertility and increase rates of miscarriage and preterm delivery. Unification defects seem to preserve fertility, but some are associ...

Developmental anomalies of the mullerian ducts represent some of the most fascinating disorders that the obstetricians and gynecologists encounter. Incidence varies between0.110%.Our objective is to study the incidence and reproductive outcome in women with uterine anomalies. Reproductive performance of 74 women with uterine anomalies delivered in Modern Government and Maternity Hospital (MGMH)...

Congenital uterine malformations are known to have a higher incidence of infertility, repeated first trimester spontaneous miscarriages, fetal intrauterine growth restriction, fetal malposition, preterm labor, retained placenta and increased cesarean section rate. The actual incidence of uterine malformation is unknown, since many women do not have any symptoms. They are estimated to occur in 0...

Herlyn-Werner-Wunderlich (HWW) syndrome is a rare developmental anomaly that includes uterus didelphys with obstructed hemivagina and ipsilateral renal agenesis. A 13-year-old girl presented with chronic abdominal pain. Magnetic resonance imaging revealed uterus didelphys, hematometrocolpos and renal agenesis on the right side with imperforate hymen. Subsequently the patient was found to have M...

Neonatal hydrometrocolpos (HMC) is a rare Mullerian duct anomaly with an incidence of 0.006%. It occurs due to blockage of the vagina with accumulation of mucus secretions proximal to the obstacle. These secretions are secondary to intrauterine and postnatal stimulation of uterine and cervical glands by maternal estrogens. A triad of congenital HMC, polydactyly, and cardiac anomalies are the ca...

Mayer-Rokitansky-Kuster-Hauser (MRKH) is a characteristic syndrome in which the Mullerian structures are absent or rudimentary. It is also associated with anomalies of the genitourinary and skeletal systems. Its association with gonadal dysgenesis is extremely rare and appears to be fortuitous, independent of chromosomal anomalies. We report such a case in a 21-year-old girl who presented prima...

46,XX gonadal dysgenesis is a rare genetically heterogeneous disorder characterized by underdeveloped ovaries with consequent, impuberism, primary amenorrhea, and hypergonadotropic hypogonadism. Mullerian agenesis or Mayer-Rokitansky-Kuster-Hauser (MRKH) syndrome is characterized by congenital aplasia of the uterus and the upper part (2/3) of the vagina in a woman with normal development of sec...