Cystic Fibrosis of the Pancreas with Observations on the Roentgen Appearance of the Associated Pulmonary Lesions

Abstract

Listen

Translate article

Cystic fibrosis of the pancreas has been established as a clinical entity affecting infants and young children, by the work of Andersen (1 and 2), Blackfan (3), Parmelee (4), Rauch (5), and others. They have shown, moreover, that the disease is not very uncommon. As Andersen points out, it is the diagnosis and not the disease that is unusual. Reviewing pathological material from 1,000 consecutive autopsies she found that pancreatic sections had been made in 605 cases, 20 of which showed cystic fibrosis with destruction of 90 per cent or more of the acinar tissue. Blackfan and May, studying pathological material from 2,800 autopsies, found 35 cases, all of which were unsuspected during life. The 4 cases reported here were discovered in a series of 125 consecutive autopsies. In only one of the 4 cases was the correct diagnosis suspected from the roentgen and clinical findings. It is not difficult to understand why cystic fibrosis of the pancreas appears seldom to be correctly diagnosed prior to death, and may even be missed at autopsy if sections of the pancreas are not taken for microscopic study. The associated pathological changes involving the lungs overshadow what is probably the fundamental pathological process in the pancreas and offer an obvious, though incorrect, explanation of the illness and death of the patient. It is the purpose of this paper to call attention to some pulmonary changes revealed by roentgen examination which suggest the possibility of cystic fibrosis of the pancreas. It is hoped, moreover, that having had their attention drawn to the roentgenologic aspects of this disease, others may be encouraged to search for additional x-ray evidence of this seldom diagnosed condition. The etiology of the changes in the pancreas has not been definitely established. There exists, moreover, considerable diversity of opinion concerning the relationship between the pancreatic lesion and the associated pathological process in the lungs. Rauch et al., who propose for the disease the name “congenital familial steatorrhea with fibrosis of the pancreas and bronchiolectasis,” believe that the pancreatic and pulmonary lesions are independent but arise from a common congenital factor. It is their belief that there is no cause and effect relation between them. “We are of the opinion,” they state, “that their association is based on a common etiological factor, namely, a congenital anomaly of the lungs and pancreas.” Oppenheimer (6) reported a case in a ten-month-old girl which he believed was “caused by true atresia of the pancreatic duct,” a condition previously reported by Kornblith and Otani (7). He concluded that “congenital atresia of the main pancreatic duct was the basis of the pancreatic lesion. Poor absorption of fats and a deficiency of vitamin A resulted, and death occurred from pulmonary infection secondary to squamous metaplasia of the bronchial epithelium.”