Abstract

Newborn infants suffering from severe generalized neuromuscular disorders at birth usually represent a diagnostic dilemma for the clinician. Not uncommonly they are given low Apgar scores and develop early signs of respiratory failure requiring ventilatory assistance. Frequently, the floppiness and muscle weakness is attributed initially to birth asphyxia. In this study we report our experience with 7 neonates diagnosed as having generalized muscle disorder - Congenital Muscular Dystrophy (3), Congenital Myotonic Dystrophy (1), Myotubular Myopathy (2), Undefined Congenital Myopathy (1) - in whom the chest x-ray revealed markedly thin ribs as a striking and consistent finding. This finding was not present in term infants with severe hypotonia as the result of acute hypoxia at birth. Normal values for rib width were established for infants of different birth weights in an attempt to provide an objective means of defining thin ribs. Based on the consistency of this finding in severe cases of neuromuscular disorder we consider this as a useful radiological sign in differentiating muscle disease from other causes of neonatal hyptonia and respiratory insufficiency while waiting for more definitive studies.

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