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Objectives: Amiodarone represents one of the oldest, widely used and most effective drugs in the treatment of atrial and ventricular arrhythmias. Both are a frequently encountered clinical challenge in patients with congenital heart disease. The current case report outlines the rare complication of blue-gray skin pigmentation and pulmonary mass after long-term amiodarone treatment and is the first report of these adverse effects of amiodarone in a child.
Case Presentation: A 13-year-old male patient with congenital Ebsteiǹs anomaly was referred to our center for cardiac surgery. Due to atrial flutter he was treated with amiodarone (6mg/kg/d) since the age of 7 years. On admission he presented with a blue-gray facial skin pigmentation and preoperative X-ray revealed several ominous pulmonary nodules. Bronchoscopy was performed which histopathologically showed numerous macrophages with foamy cytoplasm, presumably in the context of lipid sediment and confirmed the suspicion of a correlation to the chronic amiodarone intake. Amiodarone was discontinued after successful cardiac surgery. Follow up after 1year showed thorough disappearance of blue-gray skin pigmentation and there was no relapse of atrial arrhythmia. Noteworthy, the pulmonary nodules showed only incomplete regression after 2years follow-up.
Discussion: Many adverse effects of amiodarone are reported but blue-gray skin discoloration (4–9%) and pulmonary toxicity (1–17%) are rather rare.The exact pathomechanism is still controversial. Theories include drug-induced lipidosis, photosensitivity reaction to ultraviolet light or leukocytoclastic vasculitis. Amiodarone induced pulmonary mass represents a subtype of the rather common phenomenon of amiodarone induced pulmonary toxicity (AIPT) and is thought to be due to a selective accumulation of the drug in a prior lung lesion. On biopsy, the presence of foamy macrophages confirms exposure to amiodarone. Most patients respond well to withdrawal of amiodarone and prednisolone can be added to promote healing.
Conclusion: This is the first report of amiodarone induced blue-gray skin syndrome and pulmonary mass in a child. Chronic administration of amiodarone can cause severe, partly reversible side effects in children even if an approved dosage is prescribed. Discontinuation of amiodarone reverses symptoms in the majority of patients.