Intestinal Obstruction as a Late Complication of Fibrocystic Disease of the Pancreas

The features of fibrocystic disease of the pancreas are well recognized following the description by Andersen in 1938 of three main clinical forms. The first is that of intestinal obstruction in the first few days of life due to meconium ileus. About a fifth of all cases present with failure to pass meconium which is followed by vomiting and abdominal distension. It results from inspissation of meconium in the small bowel and at operation or necropsy the upper part of the small bowel is seen to be distended with gas and fluid. The lower part is filled with hard inspissated meconium and the bowel wall hypertrophies and tapers into an unexpanded ribbon-fike colon. Perforation of the bowel wall may occur causing meconium peritonitis while volvulus and sometimes atresia of the small bowel may result. Bodian (1952) describes three instances of meconium retention in which spontaneous recovery occurred a few days after birth although all finally succumbed to the disease. The second form is that of respiratory disorders which is marked by a distressingly persistent and spasmodic cough which simulates pertussis and is associated with episodes of respiratory infection usually due to Staphylococcus aureus. Respiratory distress with an increased respiratory rate, signs of infection and emphysema may be noted. Bronchiectatic changes are common and finger clubbing and even cor pulmonale may develop. The third form is that of the coeliac syndrome in which failure to thrive in spite of an excellent appetite is followed by loose offensive stools, gaseous abdominal distension, wasting and stunting. Meconium ileus is usually fatal though immediate survival has fcllowed surgical intervention but later features of the disease soon supervene. Numerous variations and combinations of symptoms may confuse the clinical picture but, despite fluctuations, the course of the disease is invariably fatal. In fibrocystic disease of the pancreas, intestinal obstruction after the neonatal period due to inspissated small bowel contents with recovery is considered worth while reporting.