Pathological causes of sudden death in autopsied children with reference to peculiar findings: An Egyptian perspective.

Childhood is a long period extending up to the age of 18 years. Childhood encompasses different developmental stages; each stage has specific characteristics. This 5-year study included 244 autopsied children who died unexpectedly due to natural causes. This study was conducted in the forensic pathology unit of the Egyptian Forensic Medicine Authority (EFMA). Pathological causes of death were diagnosed in 181 cases, representing nearly three-quarters (74.2 %) of cases. Males represented 60.8 % of these cases. More than half (51.4 %) of deaths due to natural disease occurred within the first month of life. The diagnosis was established for the first-time during autopsy in 58 % of cases. Prematurity complications and infections were the cause of death in 35.9 % and 30.4 % of deaths attributed to natural pathologies, respectively. Prematurity complications are the most common cause of death in perinatal (63.6) and neonatal (71.4 %) periods. Whereas infection is the most common cause of death during infancy (55 %), childhood (52.8 %), and adolescence (43.8 %). Pneumonia was the most common infection (61.8 %). This study highlighted cases with peculiar pathologies that include cardiomyopathies (idiopathic dilated cardiomyopathy (DCM), hypertrophic cardiomyopathy (HCM), and Arrhythmogenic right ventricular cardiomyopathy (ARVD)), Waterhouse-Friderichsen syndrome (WFS), ruptured cerebral cavernous hemangioma, and cerebellar medulloblastoma.

Sengers syndrome: a rare case of cardiomyopathy combined with congenital cataracts in an infant: post-mortem case report.

Sengers syndrome is a rare autosomal recessive disorder caused by a mutation in the Acylglycerol Kinase (AGK) gene with subsequent mitochondrial dysfunction. It is a combination of cardiomyopathy, skeletal myopathy, congenital bilateral cataracts, and lactic acidosis following exertion. The current study describes an extremely rare case of Sengers syndrome that is diagnosed during post-mortem examination. A four-month-old female child underwent sudden cardiac arrest immediately following the induction of general anesthesia for lens extraction surgery. This infant was the first child born to healthy, non-consanguineous, Egyptian parents. There were unremarkable medical problems either during pregnancy or during delivery. This infant was born at term with a normal APGAR score and weight. She had congenital bilateral cataracts since birth, easily-fatigued, and delayed milestones for her age. A post-mortem examination revealed a hypertrophied heart that weighed 96.6 grams with left ventricular hypertrophy. The left ventricle free wall thickness was 1cm, and an interventricular septum thickness of 1.3 cm. Histopathologically, the myocardium showed myocytes disarray. Also, the myocytes were hypertrophied, and vacuolated. The Gömöri trichrome stain revealed vacuolated cardiomyocytes surrounded by collagenous connective tissue, and the Periodic Acid Schiff (PAS) stain excluded that these vacuolations were related to glycogen storage disease. Focal lymphocytic infiltrations were observed within the myocardium. Thus, Sengers syndrome should be taken into consideration as a possible diagnosis whenever a congenital cataract is present for clinical and medicolegal cases.