 Hello everyone, I am Dr. Garima, I am 2nd year radiology post-graduate student in Chhattarpati Shivaji Subarthi Hospital. The topic for my oral paper presentation is a rare case of neonatal hydrocolpous cough man macausic syndrome. So the aim will be to exhibit the imaging findings of neonatal hydrocolpous macausic syndrome. A one-day old female neonate presented with abdominal distension and urinary retention. On clinical examination an abdominal lump was felt. She also had post-axial polydectyly in both hands and feet. She was diagnosed with mega cystitis antinatally at 33 weeks of gestation. So we can see the photograph of the baby's hand and feet showing post-axial polydectyly. After detailed antinatal history, the patient underwent abdominal radiography, ultrasound and MRI. An abdominal radiograph revealed a large soft tissue density abdominal pelvic mass displacing the bowel loop superiorly and laterally with no obvious signs of bowel distension. As you can see in this abdominal radiograph, this is the soft tissue density mass which is displacing the bowel loop superiorly and laterally. Ultrasonography revealed a large well-defined thin-walled abdominal pelvic cystic lesion with uniform low-level internal egos with fluid debris level within. The lesion was seen posterior to the urinary bladder and extending to the epigestric region superiorly and to the symphysis pubis inferiorly. The uterus was not seen in the pelvis. Mild bilateral hydronephrosis was also noted. On the basis of the ultrasound findings, a provisional diagnosis of hydrometrocallpos was given. So these are the ultrasound images showing the cystic structure with fluid debris level and which this was seen posterior to the urinary bladder. Also there was bilateral hydronephrosis. On MR there was accumulation of fluid intensity i.e. T1 hypointense and T2 hyperintense contents within the vagina. The resulting distended vagina was extending above the level of umbilicus displacing the large bowel loop superiorly and uterus and urinary bladder anteriorly and superiorly. Virtual hydronephrosis was likely secondary to the mass effect from the above structures. Both ovaries were not visualized separately. So this is the T2 axial image showing that cystic area with fluid debris level. On sagittal images, this was found to be distended vagina which is extending above the level of umbilicus and displacing the large bowel loop superiorly and medially and the uterus and urinary bladder anteriorly and superiorly. On exploratory leprotomy, the finding of hydrocholpus was confirmed which was caused by cervical atresia and imperforate hymen. The hypertrophied vagina was excised and vaginal uterine and astromosis and hymenoplasty was done. So MKKS is an autosomal recessive disease and was first described by McCusek in 1964. It occurs due to mutation in the MKKS gene which is present in 20p12 location. Such mutations result in formation of defective protein which is similar to the members of chaperonin family leading to anomalous limbs, heart and rib... And so an abdominal radiograph revealed a large soft tissue density abdominal pelvic mass displacing the bowel loop superiorly and laterally with no obvious signs of bowel distention as you can see in this abdominal radiograph. This is the soft tissue density mass which is displacing the bowel loop superiorly and laterally. Though MKKS is rare in males, if present it is sometimes associated with hypospediasis, cordy and cryptocidism. Polydectily is present in 90% of cases. It is predominantly post-axial and rarely mesoaxial. Syndectily may also be encountered. MKKS shows association with congenital heart defects such as itrioventricular canal defects, ventricular septal defects and hypoblastic left heart from 10 to 20% of cases. Other less commonly associated findings are gastrointestinal abnormalities that consist of imperforate innus, rectovirginal or vesicovirginal fistula, hushbrunks disease and malrotation. Abnormalities of the eyes are also mentioned in literature. Arbitral syndrome also presents with post-axial polydectily and hydrometrocolpus. It is an autosomal recessive disorder characterized by retinal dystrophy or retinitis pigmentosa. Post-axial polydectily, obesity, nephropathy and mental retardation. The diagnosis can only be made if four of the five major manifestations are present in a person and remains a difficult diagnosis in infancy as the appearance of several key features is delayed. Typically MKKS is diagnosed in very young children whereas the diagnosis of bardid vitil is often delayed to the teenage years. Other syndromes like ELIS, WAN, Crevold syndrome characterized with polydectily, acromilia and cardiac anomalies and palistar hall syndrome characterized by facial anomalies, post-axial polydectily, imperforate innus and CNS anomalies like 10 cephalic hematoblastomas may also be considered among the differential diagnosis. These are my references. Thank you.