Prostaglandin-induced cortical hyperostosis

Discussion:
  • ECHOCARDIOGRAM: Complex congenital heart disease. Dextrocardia, unbalanced AVSD, hypoplastic RV, pulmonary atresia, small tortuous PDA, unobstructed left aortic arch, superior vena cava duplication with no bridging vein, total anomalous pulmonary venous return.
  • Radiographic, sonographic & echocardiographic features are suggestive of heterotaxy syndrome (right isomerism/asplenia).
  • Prostaglandin E (PGE1 0.05 mic/kg/min) infusion was started within first hour of birth. Case was discussed in multidisciplinary meeting at pediatric cardiac center and offered no cardiac intervention due to complexity of the disease with grave prognosis. Patient was managed conservatively with low dose prostaglandins for three months, which were later on discontinued due to clinical deterioration of the patient. Patient died after one week of discontinuation of prostaglandins.  
  • Based on above mentioned clinical history, prostaglandin therapy, and imaging findings; diffuse periosteal reaction / thickening noted along the long bones of the bilateral upper and lower extremities on 2nd skeletal survey, is suggestive of prostaglandin-induced cortical hyperostosis.
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