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Adrenoleukodystrophy Presenting as Postperfusion Syndrome

N Engl J Med 1997; 336:731-732March 6, 1997

Article

To the Editor:

The postperfusion syndrome, an uncommon event after open-heart surgery with extracorporeal circulation, is associated with a young age at surgery (less than one year) and bypass lasting longer than 60 minutes.1,2 We report an unusual case of a life-threatening syndrome that occurred after the surgical repair of isolated conal ventricular septal defect.

An 18-year-old man underwent transpulmonary patch repair of ventricular septal defect with cardiopulmonary bypass for 50 minutes. Preoperatively, the patient had normal mental development, a slight gait disorder, and unremarkable electroencephalographic and laboratory findings. Twelve hours after surgery, he had hypotension (blood pressure, 70/30 mm Hg), oliguria (urinary output, <50 ml per hour), and lethargy. Laboratory examination revealed the following values: blood glucose, 32 mg per deciliter; creatinine, 3.3 mg per deciliter; aspartate aminotransferase, 160 U per liter; and creatine kinase, 3690 U per liter. Chest roentgenography showed moderate bilateral pulmonary edema; echocardiography ruled out cardiogenic shock due to residual ventricular septal defect or aortic insufficiency. Bedside right-heart catheterization revealed a normal cardiac index (3.9 liters per minute per square meter) and very low systemic vascular resistance (970 dyn . sec/cm⁵). Pharmacologic support with norepinephrine (0.1 μg per kilogram of body weight per minute) and oxygen (5 liters per minute) and empirical treatment with methylprednisolone (400 mg per day) stabilized the circulatory and metabolic abnormalities within three days. Ten days after discharge, the patient was admitted to the emergency room because of lethargy. Profound hypotension, electrolyte abnormalities, and low serum and urinary cortisol levels suggested the presence of Addison's disease. The gait disturbance had worsened, and ataxia and electroencephalographic abnormalities were present. Magnetic resonance imaging of the head showed multiple areas of high-intensity signals involving the internal and external capsule and the corpus callosum, suggesting demyelination of the white matter (Figure 1Figure 1Magnetic Resonance Images Showing Abnormal Hyperintensity of the Occipital White Matter, the Subcortical White Matter of the Temporal Lobe, and the Corpus Callosum.). The diagnosis of adrenoleukodystrophy was confirmed by the increased plasma levels of very-long-chain saturated fatty acids (C26/C22 = 0.074).3

Adrenoleukodystrophy, a rare neuroendocrine disease causing progressive central demyelination and adrenal cortical insufficiency, may present as Addison's disease in adults.3 The clinical course of our patient suggests that the extracorporeal circulation triggered an addisonian crisis, with multiple-organ dysfunction identical to that seen in the postperfusion syndrome.1,2 These clinical abnormalities resolved with empirical steroid therapy. The only clue to the diagnosis of adrenoleukodystrophy was the discrepancy between the life-threatening postperfusion reaction and the older age of the patient and brief duration of the operation.

Giovanni Battista Luciani, M.D.
Renzo Pessotto, M.D.
Alessandro Mazzucco, M.D.
University of Verona, 37126 Verona, Italy

3 References

1. 1

   Kirklin JK, Westaby S, Blackstone EH, Kirklin JW, Chenoweth DE, Pacifico AD. Complement and the damaging effects of cardiopulmonary bypass. J Thorac Cardiovasc Surg 1983;86:845-857
   Web of Science | Medline

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   Westaby S. Organ dysfunction after cardiopulmonary bypass: a systemic inflammatory reaction initiated by the extracorporeal circuit. Intensive Care Med 1987;13:89-95
   CrossRef | Web of Science | Medline

3. 3

   Moser HW, Moser AE, Singh I, O'Neill BP. Adrenoleukodystrophy: survey of 303 cases: biochemistry, diagnosis, and therapy. Ann Neurol 1984;16:628-641
   CrossRef | Web of Science | Medline

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   C.M. Platis, L. Kachko, E. Peled, J Katz. (2006) Anesthesia for the child with Zellweger syndrome: a case report. Pediatric Anesthesia 16:3, 361-362
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   Antonella Di Biase, Rita Di Benedetto, Serafina Salvati, Lucilla Attorri, Fabiana Leonardi, Donatella Pietraforte. (2005) Effects of L-mono Methyl-arginine, N-Acetyl-cysteine and Diphenyleniodonium on Free Radical Release in C6 Glial Cells Enriched in Hexacosenoic Acid. Neurochemical Research 30:2, 215-223
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   Antonella Di Biase, Rita Di Benedetto, Carla Fiorentini, Sara Travaglione, Serafina Salvati, Lucilla Attorri, Donatella Pietraforte. (2004) Free radical release in C6 glial cells enriched in hexacosanoic acid: implication for X-linked adrenoleukodystrophy pathogenesis. Neurochemistry International 44:4, 215-221
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