ESPE Abstracts

Introduction: Prostaglandin E1 (PGE1) is a drug used for ductal patency in cyanotic congenital heart disease, We are reporting an infant with cortical hyperostosis secondary to prolonged use of PGE1, with typical and extensive radiological findings.

Case Report: A 2-month-old girl, born at 37 weeks with APGAR scores of 9 & 10 at 1 & 5 min, and birth weight = 2.9 kg. She failed Critical Congenital Heart Defects Screening Methods at 12 hrs. of age with pre- and post-ductal oxygen saturation in the 80% with no respiratory distress. Echocardiogram revealed a complex congenital heart disease (Tetralogy of Fallot, complete atrioventricular canal defect, and pulmonary atresia). The patient was started on PGE1 infusion at 0.03 mcg/kg/min. Her oxygen saturation remained between 85: and 93 % in room air. She was feeding well and gaining weight appropriately for her age. A palliative PDA stenting was planned but delayed because of social factors and the baby remained on PGE1 infusion. By the age of two months, the baby was noted to have diffuse upper and lower limb tenderness with swelling, irritability, and intense crying with minimal handling. The radiography of the large bones revealed an intense periosteal reaction with bilateral corticoperiosteal thickening of the diaphysis in clavicles, femur, tibia, humerus, radius, and ulna. The lesions were distributed along the middle portion of the long bones, except for the humerus, where the affection was distributed more distally. Laboratory findings showed raised alkaline phosphatase (ALP) (713 U/L) with normal serum calcium and phosphorus. The dose of the PGE1 was reduced to 0.02 and then 0.01 mcg/kg/min gradually while monitoring for PDA patency and oxygen saturation over 10 days. The infant did not tolerate the reduction of the drug, experiencing a rapid decrease in arterial saturation, and was eventually taken for PDA stenting on DOL 72. After 10 days, the swelling of all limbs and pain and tenderness disappeared and ALP decreased.

Discussion: Prostaglandin-induced cortical hyperostosis appears to be linked to the duration or dosage of the PGE1. It can occur as early as 9-11 days after PGE1 infusion. The percentage of patients developing it increases from 42% at <30 days to 100% at >60 days. Elevated serum ALP level helps in the diagnosis and follow-up of cortical hyperostosis.

Conclusion: Early and frequent radiological investigations should be made in patients undergoing intravenous PGE1 treatment for more than 7 days.