Chronic pancreatitis in muscular cytochrome c oxidase deficiency.

Chronic pancreatitis is described in a 10-year-old boy with mitochondrial myopathy due to cytochrome c oxidase (complex IV) deficiency. There have been few reports of chronic pancreatitis associated with congenital metabolic diseases. Marked pancreatic calcifications and pseudocysts of the pancreatic head and tail were demonstrated by computed tomography of the abdomen and by endoscopic retrograde pancreatography, which were thought to be useful methods for morphological examination of the pancreas. In the patient, no possible etiological factors were identified, including anomalous pancreatobiliary ductal junction or biochemical abnormalities. The association between cytochrome c oxidase deficiency and chronic pancreatitis remains to be established.