Generalized Cytomegalic Inclusion Disease, with Emphasis on Roentgen Diagnosis

Abstract
Generalized cytomegalic inclusion disease is a systemic disease, primarily of infancy, characterized by the presence of intranuclear and intracytoplasmic inclusion bodies in enlarged cells of a variety of viscera. It has been known also as generalized salivary gland virus disease, inclusion disease, and protozoan cell disease. In 1921 Goodpasture and Talbot recognized the abnormal cells as altered tissue cells rather than protozoan organisms and referred to the changes as “cytomegalia” (1). Apparently identical cells have been encountered incidentally in 10 to 32 per cent of salivary glands (2), and in other organs in 1 to 2 per cent of routine infant autopsies (3). In certain cases, however, a specific salivary gland virus infection exists, which probably originates in utero and mayor may not end fatally (4, 5). Pathology The cellular changes of cytomegalic inclusion disease are distinct from those of other diseases in which inclusions occur. There are two general types of lesions microscopically: (a) the “granulomatous” type, showing areas of necrosis, with infiltration of lymphocytes, mononuclear cells, and large inclusion cells, and (b) inclusion cells with necrosis alone. Any of the organs of the body may be affected, but the more usual sites are the kidneys, liver, lungs, and pancreas, with the brain, endocrine glands, and gastrointestinal tract less frequently involved. Few cases of salivary gland involvement are reported, but these glands are not often examined at autopsy. The intranuclear inclusion appears to have an affinity for epithelium, especially in the renal tubules; also in bile ducts, bronchi, alveoli, and salivary ducts. Lesions in the liver may be associated with jaundice, and cirrhosis may result from periportal fibrosis. Bile stasis and hepatic cellular necrosis often occur. In the lungs the inclusions are usually associated with an interstitial pneumonia, a frequent finding with other viral infections. The brain may reveal evidence of meningo-encephalitis, with areas of necrosis, calcification, and at times hemorrhage. The renal lesions are important clinically for diagnostic purposes, as the inclusion cells, which are located in the tubular epithelium, may be shed into the lumen and appear in the urine. Clinical Aspects Cytomegalic inclusion disease may be divided into four main categories: (a) the subclinical variety found in submaxillary glands and other organs at autopsy on stillborn or newborn infants dying of other causes; (b) poorly understood cases occurring apparently in association with other diseases, such as pertussis (5); (c) postulated cases with mild manifestations and recovery; (d) the systemic or generalized variety, which is usually lethal. In children under two months of age, the condition is usually primary and fatal. In older children it may be primary but more often is associated with another disease and plays a minor role.