[CASE OF HYPEROSTOSIS GENERALISATA].
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Two small neonates, one with hypoplastic right heart syndrome, and the other with tetralogy of Fallot and pulmonary atresia, were treated with intravenous prostaglandin E1 (Prostyn VR, Upjohn, Kalamazoo, Michigan) for 96 and 33 days, respectively. They developed limited cortical hyperostosis, but marked soft tissue swellings in all extremities. The changes were noted after PGE1 treatment for four weeks and one week, respectively. A skin biopsy was performed in one patient and revealed edematous changes and arteriolar wall abnormalities. We add this peripheral hard edema to the list of complications of PGE1 therapy, and speculate as to its pathophysiology.
Cortical hyperostoses are found to be side effects after therapy in early infancy with prostaglandin E1 and E2. Correlation seems to exist between dosage and duration of therapy. Radiologically the lesions cannot be differentiated from Caffey's disease. Pathogenetic relationship of the two diseases is discussed; 62 cases of newborn children with ductal related vitium cordis, who were treated for a short time or for longer with prostaglandin E2 are demonstrated.
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Prostaglandin E1 (PGE1) is an essential drug for infants with ductal-dependent congenital heart disease. Cortical hyperostosis of the long bones is one of the complications during and after PGE1 therapy and should be considered in the differential diagnosis of periostitis in the infant.
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