PATHOLOGY OF IDIOPATHIC INFANTILE ARTERIAL

CALCIFICATION (OF INFANCY)

The main abnormality is intimal fibrous proliferation in arteries with deposition of calcium hydroxyapatite in the internal elastic laminae. The calcification is usually associated with fragmentation of the internal laminae. The degree of calcification is not related to the degree of arterial stenosis (1). Intimal hyperplasia is a fundamental change and plays a major role in the vascular occlusion that results in ischemia and organ dysfunction (1).

The condition develops in utero but usually present in infancy. Most authors believe that abnormal metabolism of elastin is the primary underlying event but altered iron metabolism (2) and pyrophosphate deficiency (3) have also been suggested.

Calcium metabolism is normal but there may be a pyrophosphate deficiency (3).

Pathology (4-18)

• Idiopathic infantile arterial calcification is a diffuse vascular disorder that can affect any artery (4,11).
• Characteristic of the disease process is a patchy destruction or fragmentation of the internal elastic membrane with calcific deposits and intimal proliferation.
• There is disagreement as to whether the calcium deposition or intimal proliferation is the primary pathologic process (5,6,11,18).
• The coronary arteries are the most commonly affected, and death is usually attributed to heart failure secondary to ischemia or infarct due to occlusion of the arteries by intimal thickening (5,8,13,15-18).
• The cerebral arteries are the least commonly affected (9,11).

There has been a case reported where death was attributed to bowel infarction secondary to idiopathic infantile arterial calcification (6).

REFERENCES

1. Thiaville A, Smets A, Clerx A et.al. Idiopathic infantile arterial calcification: a surviving patient with renal artery stenosis. Pediatr Radiol 1994;24:506-508
2. Anderson KA, Burbach JA, Fenton LJ et.al. Idioaphic arterial calcification of infancy in new-born siblings with unusual light electron microscopic manifestations. Arch Pathol Lab Med 1985;109:838-842.
3. Stuart G, Wren C, Bain H Idiopathic infantile arterial calcification in two siblings: failure of treatment with diphosphonate. Br Heart J 1990;64:156-159.
4. Rosenbaum DM, Blumhagen JD: Sonographic recognition of idiopathic arterial calcification of infancy. AJR 146:249-250, 1986.
5. Stanley RJ, Edwards WD, Rommel DA, et al: Idiopathic arterial calcification of infancy with unusual clinical presentations in sisters. Am J Cardiovasc Pathol 2:241-245, 1988.
6. Vade A, Eckner FAO, Rosenthal IM: Computerized tomography in occlusive infantile arteriopathy. Pediatr Cardiol 10:221-224,1989.
7. Barson AJ, Campbell RHA, Langley FA, et al: Idiopathic arterial calcification of infancy without intimal proliferation. Virchows Arch [A] 372:167-173, 1976.
8. Meradji M, de Villeneuve VH, Huber J, et al: Idiopathic infantile arterial calcification in siblings: radiologic diagnosis and successful treatment. J Pediatr 92:401-405,1978.
9. Proesmans W, Dan Dyek M: Correspondence. Pediatr Nephol 5:96,1991.
10. Parker RJ, Smith EH, Stoneman MER: Generalized arterial calcification of infancy. Clin Radiol 22:69-73,1971.
11. Morton R: Idiopathic arterial calcification in infancy. Histopathology 2:423-432,1978.
12. Van Oort AM, Sengers RCA, Stadhoudrs AM, et al: Idiopathic arterial calcification of infancy. Helv Paediat Acta 34:369-374,1979.
13. Kirchner SG, Heller RM, Kasselberg AG: Idiopathic arterial calcification of infancy. South Med J 72:1328-1330,1979.
14. Liu CT, Singer DB, Frates R: Idiopathic arterial calcification in infancy: report of a case in a premature fetus. Arch Pathol Lab Med 104:589-591,1980.
15. Chen H, Fowler M, Yu CW: Generalized arterial calcification of infancy in twins. Birth Defects:Original Article Series 18(3B):67-80,1982.
16. Lie JT: Correspondence. Hum Pathol 16:1178-1179,1985.
17. Anderson KA, Burbach JA, Fenton LJ, et al: Idiopathic arterial calcification of infancy in newborn siblings with unusual light and electon microscope manifestations. Arch Pathol Lab Med 109:838-842,1985
18. Witzleben CL: Idiopathic infantile arterial calcification—a misnomer ? Am J Cardiol 26:305-309,1970.