Correspondence

Pseudoxanthoma Elasticum

N Engl J Med 1994; 330:793March 17, 1994

Article

To the Editor:

In their Brief Report on pseudoxanthoma elasticum in patients with premature cardiovascular disease, Lebwohl et al. (Oct. 21 issue)1 provide no data to support one of their conclusions. Their Patient 1 had recurrent angina several months after undergoing revascularization with multiple vein grafts and an internal thoracic-artery graft. They did not report any attempt to determine the patency of the bypass grafts by angiography, and there is no evidence to suggest that the thoracic-artery graft failed because of latent arterial graft disease. Their conclusion that “arterial grafts should not be used for coronary artery bypass surgery in patients with pseudoxanthoma elasticum because of possible calcification of the internal elastic laminae” has no basis whatsoever and is misleading.

Eugene A. Grossi, M.D.
Peter Pasternack, M.D.
New York University Medical Center, New York, NY 10016

1 References

1. 1

   Lebwohl M, Halperin J, Phelps RG. Occult pseudoxanthoma elasticum in patients with premature cardiovascular disease. N Engl J Med 1993;329:1237-1239
   Full Text | Web of Science | Medline

Author/Editor Response

The authors reply:

To the Editor: Pseudoxanthoma elasticum is a systemic disease of elastic tissue. Its effects are not limited to the elastic fibers of one area of the skin or to only one or two coronary arteries. Calcification of the internal elastic laminae of arteries is responsible for the bleeding and vascular narrowing that occur and has been associated with myocardial infarction, even in children and adolescents with this disorder1,2. Observations of this sort are the basis for our concern about the selection of disease-prone arteries for myocardial revascularization.

In addition, pseudoxanthoma elasticum preferentially involves traumatized tissue3. The use of the internal thoracic artery for coronary bypass grafting in patients with pseudoxanthoma elasticum means that one not only selects a vessel with an inherited tendency to become occluded, but also subjects this vessel to surgical trauma, which may induce the changes of pseudoxanthoma elasticum.

In the patient referred to by Grossi and Pasternack, calcification of the internal elastic lamina of the redirected internal thoracic artery was found on histologic sections taken at the time of coronary bypass surgery. Although the thoracic artery may represent a superior vessel for coronary bypass grafting in patients with atherosclerotic coronary disease who do not have pseudoxanthoma elasticum, surgeons should consider the potential disadvantages of myocardial revascularization with a traumatized vessel that has an inherited tendency to become occluded. This risk does not apply to vein grafts, which are devoid of internal elastic laminae.

Mark Lebwohl, M.D.
Robert Phelps, M.D.
Jonathan Halperin, M.D.
Mount Sinai Medical Center, New York, NY 10029-6574

3 References

1. 1

   Schachner L, Young D. Pseudoxanthoma elasticum with severe cardiovascular disease in a child. Am J Dis Child 1974;127:571-575
   Web of Science | Medline

2. 2

   Wilhelm K, Paver K. Sudden death in pseudoxanthoma elasticum. Med J Aust 1972;2:1363-1365
   Web of Science | Medline

3. 3

   Lebwohl M, Phelps RG, Yannuzzi L, Chang S, Schwartz I, Fuchs W. Diagnosis of pseudoxanthoma elasticum by scar biopsy in patients without characteristic skin lesions. N Engl J Med 1987;317:347-350
   Full Text | Web of Science | Medline