A 10-year-old presented to the emergency room after having emesis of bright red blood 3 times. Hematocrit on admission was 15. The patient received 20 mL/kg of packed red blood cells and was booked for an emergency diagnostic esophagogastroduodenoscopy with banding of varices. Pertinent past medical history reveals that he was diagnosed at age 6 months with cystic fibrosis (CF). He has had banding of esophageal varices 3 times in the past. He also has chronic lung disease requiring chest physical therapy (PT), a cough assist vest, inhaled tobramycin, and DNase, fluticasone, and albuterol inhalers. He just finished a course of IV antibiotics last week for increased pulmonary secretions and presumed pneumonia.
Upper gastrointestinal bleeding can originate from any area proximal to the ligament of Treitz. Most commonly, any bleeding that occurs distally will present as melena. The most common differential diagnosis of upper gastrointestinal bleeding in children includes gastritis, peptic ulcer disease, Mallory-Weiss syndrome, and esophageal varices. Given this patient’s history, the most likely cause is variceal bleeding.
Cystic fibrosis is a genetic disorder associated with chronic lung disease and pancreatic insufficiency. Pulmonary disease is responsible for more than 90% of the morbidity in these patients. Classically, patients are diagnosed by performing a sweat test. Chloride in sweat will be markedly increased. These patients can present at birth with meconium ileus with rare perforation. They may also present with severe pulmonary manifestations; thick secretions or mucus plugs, leading to multiple infections; or severe persistent episodes of obstructive airway disease. This may eventually lead to pulmonary hypertension and cor pulmonale. They may also present with malnutrition or diabetes resulting from exocrine or endocrine pancreatic dysfunction. They may also present with liver cirrhosis. They can develop upper gastrointestinal bleeding from esophageal varices secondary to portal hypertension and also have coagulopathy from liver failure or cirrhosis and malnutrition.
Thoroughly assess the severity of pulmonary disease in order to better plan intraoperative and postoperative management; oxygen should be available as well as biphasic positive airway pressure if it is used preoperatively
Humidify gases. Frequent suctioning or lavage of secretions may be necessary.
Assess the degree of pancreatic involvement and manage diabetes if present; frequent blood glucose monitoring is indicated.
Sinus involvement may act as a bacterial reservoir and also trigger bronchospasm.
Upper gastrointestinal bleeding may be severe, and adequate volume resuscitation as well as preparation for possible blood transfusion should be made.
Manage coagulopathy, if present.
Have blood products available.
Early extubation should be the goal with all CF patients, as prolonged intubation may increase morbidity and mortality. Early chest PT and mobilization should be encouraged.