Radiation esophagitis is usually diagnosed clinically, based on patient symptoms and radiation dose exposure of the esophagus. Treatment includes acid suppression, diet modification, analgesics,

empiric treatment for candidiasis, and aggressive nutritional support. Esophageal stricture is the most common late effect of radiation on the esophagus, presenting as progressive dysphagia. Endoscopic dilatation is successful therapy in the majority of patients. “
“A 34-year-old Malaysian man presented with esophageal variceal bleeding, which was successfully treated with sclerotherapy. Investigations at that time showed evidence of portal hypertension with prominent portosystemic collateral vessels and splenomegaly. Hepatic synthetic function was normal. A liver biopsy Gemcitabine ic50 revealed minimal fibrosis of nonspecific etiology and a diagnosis of noncirrhotic portal hypertension (NCPH) was made. Five years later he presented with increasing exertional breathlessness. On examination he had developed finger clubbing and was cyanotic. O2 saturation and PaO2 on room air were reduced at 91% and 60 mmHg, respectively. The PO2 rose to 576 mmHg on 100% O2. Pulmonary angiography showed no evidence of discrete AV malformations;

however, hepatic vein wedge pressure was measured at the time and was elevated at 30 mmHg, in keeping with marked portal hypertension. Doppler studies of the hepatic vasculature showed patent vasculature. A repeat liver biopsy revealed hepatoportal

sclerosis, in keeping with the MG132 original diagnosis of NCPH. Subsequently, a contrast-enhanced echocardiogram was performed which demonstrated delayed appearance of echo-contrast in the left atrium indicating an intrapulmonary shunt confirming the diagnosis of Acetophenone hepatopulmonary syndrome (HPS). The patient’s respiratory symptoms insidiously worsened. By 4 years later his PaO2 on room air had fallen to 49 mmHg. A technetium-labeled macroaggregated albumin (99mTcMAA) lung perfusion scan revealed markedly abnormal brain uptake of radioactivity tracer and a shunt fraction of 15.25% (normal <6%) was calculated using standard methods.[1] The patient was listed for orthotopic liver transplantation (OLT) and the surgery was successfully performed 6 months later. Examination of the explanted liver was again consistent with hepatoportal sclerosis with no evidence of cirrhosis or active liver disease. Following OLT his breathlessness improved steadily over several months. By 3 months post-OLT his O2 saturation on room air was 96% and PO2 was 90 mmHg. Three years post-OLT he presented with right upper quadrant discomfort and worsening dyspnea associated with the redevelopment of hypoxia. At rest, in the supine position his O2 saturation on room air was 93% with a PaO2 of 64 mmHg. A liver biopsy revealed no evidence of significant graft pathology and a computed tomography (CT) chest was unremarkable. A 99mTcMAA lung perfusion scan was repeated and indicated 8.