Browsing by Author "Mandal, Krishna Deo"
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Publication A Combination Of Vein Of Galen Aneurysmal Malformation And Bovine Aortic Arch In Newborn: A Case Report(Nepal Medical Association, 2025) Mandal, Krishna Deo; Subedi, Kalpana; Amatya, Unnati; Pathak, JanakAbstract Vein of Galen aneurysmal malformation is a rare intracranial arteriovenous malformation, coexistence with aortic arch anomalies is even more uncommon, carry high risk of morbidity and mortality related to endovascular procedure and poor neurological outcome. Vein of Galen aneurysmal malformation is a dilated venous pouch, a persistence of the embryonic median prosencephalic vein of Markowski. Often presents with high output cardiac failure, severe pulmonary hypertension, and systemic steal leading to hemodynamic compromise and multi organ failure. Bicêtre score scale determines the potential treatment option and prognosis of the disease. Aim of the medical treatment is initial stabilization of life threatening condition of severe heart failure followed by the definitive treatment with endovascular embolization. Treatment is a big challenge, specially if coexistence with aortic arch anomalies which increases the morbidity and mortality. We describe a term neonate found to have vein of Galen aneurysmal malformation associated with bovine aortic arch.Publication Carvajal Syndrome- A Variant of Naxos Disease: A Case Report(Nepal Medical Association, 2022) Mandal, Krishna Deo; Shrestha, Pun Narayan; Ghimire, Anjila; Joshi, Prakash; Agrawal, Sumit; Shrestha, PraptiAbstract: Carvajal syndrome is a rare variant of Naxos disease, a recessive mutation of the desmoplakin gene characterized by presence of woolly hair, palmoplantar keratoderma and dilated cardiomyopathy, mainly left ventricular involvement. The main clinical complication is progressive heart disease which may lead to heart failure and sudden cardiac death in childhood and adolescence. Cardiomyopathy is diagnosed by Task Force Criteria. The goal of treatment is to prevent sudden cardiac death by lifestyle modification and regular clinical monitoring with pharmacotherapy. We report a nine years female who had skin and hair abnormality and was admitted with features of heart failure. She was clinically diagnosed as Carvajal syndrome, an under-recognized cardio cutaneous manifestation in children. Clinicians should be aware, if any child present with keratoderma of palm and soles with woolly hair since birth should evaluate for cardiomyopathy. Genetic tests should be done whenever available, for confirming the diagnosis and counseling.