Nishant Kumar

Dandy-Walker Malformation is characterized by triad of Hypoplasia of vermis, Fourth ventricular cystic dilatation and tentorium cerebelli elevation. It is a rare anomaly of cerebellum which is commonly associated with other chromosomal disorder like Trisomy 13. The ultrasound is very critical and diagnostic imaging modality for diagnosis during antenatal period. We report a case of Dandy-Walker malformation with association of Cleft lip/Cleft palate; posterior spinal defect with myelomeningocele, colpocephaly; and other congenital defects that was diagnosed during a first routine prenatal ultrasound at 21 weeks of gestation. Keywords: Dandy-Walker Malformation, Antenatal ultrasonography, Posterior fossa, Cyst herniation, Occipital meningoencephalocele, Posterior spinal defect with myelomeningocele, Cleft lip/palate

The Pericardium is a membrane like structure which envelops the heart and the proximal great vessels from all around. In normal physiological conditions, there is ~ 10 – 50 ml of pericardial fluid present. But, significant accumulation of pericardial fluid can occur in certain conditions like inflammation or infection of the pericardium and adjacent surrounding structures. This condition can remain asymptomatic for significant time period and incidentally can be found after any imaging investigations for another disease process, or patient can present with shock like symptoms due to cardiac tamponade. Here, one point is of significant notification that it is the rate of accumulation of the fluid around pericardium, and not the absolute size of the heart, that primarily determines the symptoms that occur in the pericardial effusion. Meanwhile the clinical suspicion may be introduced by the history of the patient, examination of the patient, electrocardiogram investigation, or chest X-ray. In all these, the echocardiogram is the basic and mainstay to confirm the diagnosis. Options for treatment depends on the etiology and hemodynamics consequences of the heart. This article mainly focuses on the etiology, pathophysiology, investigation, chest X-Ray, CT, and management of the pericardial effusion. Keywords: Pericardial effusion, Chest X-Ray, Moneybag appearance of Heart, Hypertension, CKD Grade V.

ABSTRACTHereditary hemorrhagic telangiectasia, also known as Osler-Weber-Rendu syndrome is a rare inherited disorder which shows autosomal dominant inheritance pattern with variable sensitivity primarily affecting the blood vessels and is characterized by abnormal formation of blood vessels in skin, mucous membranes, and organs like lungs, liver and central nervous system. It is seen to involve the long arm of chromosome 9. It affects both sexes equally and is characterized by multiple telangiectasias and Arteriovenous malformation that causes direct connections between arteries and veins which bypasses intermediate capillaries. The most marked sites where telangiectasia occurs are lips, tongue, face, nasal mucosa, oral cavity and gastrointestinal tract. The main complications of telangiectasias are easily rupture and bleeding which occurs due to the thin walls, narrow tortuous course, and in close proximity to skin surface and mucus membrane. The symptoms are obvious and are classified according to the involvement of organ which includes repeated epistaxis, gastrointestinal hemorrhage, hemoptysis, skin lesions, and sometimes leads to dreadful condition like stroke. Complications do occur in Hereditary hemorrhagic telangiectasia and those includes bleeding from various sites, reduced hemoglobin, followed by stroke, pulmonary AVM, Transient Ischemic Attack (TIA), hypovolemic shock which occurs due to severe uncontrolled bleeding, which eventually leads to cardiac failure. Present case is of 40 year old male who presented with complaints of shortness of breath, epistaxis, non-productive, yellow mucoid cough sometime associated with hemoptysis, GERD, skin thickening with finger-tip ulcers and nail changes. Keywords: Osler-Weber-Rendu syndrome, Finger-tip ulcers