Idiopathic Infantile Arterial Calcification: A Case Report

Idiopathic Infantile Arterial Calcification (IIAC) is a rare autosomal recessive inherited disorder that influences the metabolism of pyrophosphate and leads to in-utero calcium deposition with fibrotic proliferation in the walls of the large and medium-sized arteries. IIAC may present antenatally with polyhydramnios with or without arterial calcification, and may present postnatally if intractable systemic hypertension and/or heart failure from systemic hypertension and or ischemic coronary artery disease. Diagnosis of IIAC is usually confirmed by the genetic study. It has a poor prognosis, with mortality been reported by the age of 4-6 months. Increasing awareness about IIAC facilitates early diagnosis, early management, family counseling for prognosis and future conceptual plans, as early diagnosis helps in the decision of pregnancy termination.