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Complete second branchial cleft fistula in a fifteen-year-old boy: A case report.
Int J Surg Case Rep
January 2025
King Edward Medical University Lahore, Pakistan.
Introduction And Importance: The branchial or pharyngeal apparatus, crucial in embryological development, consists of clefts, arches, pouches, and membranes. Anomalies arising from this apparatus particularly involving the second branchial arch, are rare. Among these anomalies, complete second branchial cleft fistulas, with both external and internal openings, are exceptionally uncommon.
View Article and Find Full Text PDFLiver Transplant Outcome in Chanarin-Dorfman Syndrome: A Rare Lipid Storage Disease.
Exp Clin Transplant
December 2024
>From the Department of Anesthesia and Intensive Care, Faculty of Medicine, Mashhad University of Medical Sciences, Mashhad, Iran.
Chanarin-Dorfman syndrome is a multisystem inherited metabolic disorder characterized by congenital ichthyosis and lipid droplet accumulation in various organs, including the liver, muscles, and skin. The accumulation of lipids in the liver can lead to cirrhosis, liver failure, and even hepatocellular carcinoma. Here, we present a 17-year-old girl who underwent a deceased donor liver transplant to treat uncompensated cirrhosis due to Chanarin-Dorfman syndrome.
View Article and Find Full Text PDFA Delicate Balance: Anesthetic Management for Neonatal Congenital Diaphragmatic Hernia Repair.
Cureus
December 2024
Anesthesiology, Emirates Health Services, Sharjah, ARE.
Congenital diaphragmatic hernia (CDH) presents significant challenges in neonatal management, particularly in the context of anesthesia. This case report details the successful anesthetic management of a five-day-old neonate with left-sided CDH requiring thoracoscopic repair. A five-day-old neonate, delivered via emergency cesarean section due to breech presentation, presented with severe respiratory distress and was diagnosed with left-sided CDH.
View Article and Find Full Text PDFPulmonary Atresia with Ventricular Septal Defect without Major Aorto-Pulmonary Collateral Arteries: Echocardiography and the Role of Computed Tomography and Magnetic Resonance Imaging.
World J Pediatr Congenit Heart Surg
January 2025
Department of Pediatrics, Inova Health System, Falls Church, VA, USA.
Pulmonary atresia with ventricular septal defect (PA-VSD) is usually diagnosed by transthoracic or fetal echocardiography, with the prenatal diagnosis being feasible and accurate if fetal cardiology services are available. The limitations of transthoracic echocardiography (TTE) in the evaluation of PA-VSD include the complete evaluation of the pulmonary arteries and patent ductus arteriosus, quantitative evaluation of the right ventricle size and function, and delineation of associated cardiac anomalies such as coronary artery anomalies, anomalies of systemic or pulmonary venous return, and complex arch anomalies. Echocardiography also has limitations in evaluating hemodynamics such as flow volumes, shunts, and regurgitant fraction.
View Article and Find Full Text PDFBackground: Vaginal atresia (VA) is a rare female genital tract malformation characterized by the absence or incomplete development of the vagina, often leading to cyclic abdominal pain and menstrual blood retention in adolescent patients. Vaginal atresia is often accompanied by multiple organ malformations. The condition poses significant challenges in diagnosis and management, requiring a multidisciplinary approach.
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