Hepatopulmonary Syndrome in a Nigerian Man with Decompensated Alcoholic Liver Cirrhosis
Chinwe Philomena Onyia
Gastroenterology Unit, Department of Medicine, University of Nigeria Teaching Hospital, Ituku-Ozalla, Enugu, Nigeria.
Olive Obienu
Gastroenterology Unit, Department of Medicine, University of Nigeria Teaching Hospital, Ituku-Ozalla, Enugu, Nigeria.
Promise Asogwa
Gastroenterology Unit, Department of Medicine, University of Nigeria Teaching Hospital, Ituku-Ozalla, Enugu, Nigeria.
Winifred Adiri
Gastroenterology Unit, Department of Medicine, University of Nigeria Teaching Hospital, Ituku-Ozalla, Enugu, Nigeria.
Ugochukwu Nwoko
Gastroenterology Unit, Department of Medicine, University of Nigeria Teaching Hospital, Ituku-Ozalla, Enugu, Nigeria.
Uchenna Nkemdilim Ijoma *
Gastroenterology Unit, Department of Medicine, University of Nigeria Teaching Hospital, Ituku-Ozalla, Enugu, Nigeria and Department of Medicine, Faculty of Medical Sciences, College of Medicine, University of Nigeria, Ituku-Ozalla Campus, Enugu, Nigeria.
Sylvester Chuks Nwokediuko
Gastroenterology Unit, Department of Medicine, University of Nigeria Teaching Hospital, Ituku-Ozalla, Enugu, Nigeria and Department of Medicine, Faculty of Medical Sciences, College of Medicine, University of Nigeria, Ituku-Ozalla Campus, Enugu, Nigeria.
*Author to whom correspondence should be addressed.
Abstract
Hepatopulmonary syndrome is a rare complication of liver disease and portal hypertension. It has not been previously described in medical literature in Nigeria. We report this case of a 64-year-old man who presented with a 3 months’ history of recurrent breathlessness, platypnoea and leg swelling. He had been diagnosed with liver cirrhosis and had variceal band ligation in 2018, and had been managed for 2 episodes of hepatic encephalopathy prior to referral. Physical examination showed he was in respiratory distress, centrally cyanosed, had grade 4 digital clubbing, tachypnoea, platypnoea, orthodeoxia and bibasal coarse crepitations He also had a non-tender hepatomegaly, splenomegaly and ascites with no leg edema. Investigations showed negative screening for hepatitis B and C, elevated AST and GGT, conjugated hyperbilirubinemia, hypoalbuminemia, negative COVID-19 test, polycythemia, thrombocytopenia, prolonged INR and coarse enlarged liver with irregular margins on ultrasound. Gastroscopy revealed Grade 3 oesophageal varices, Forrest 3 pre-pyloric ulcer and portal hypertensive gastropathy. Spirometry demonstrated a restrictive pattern of airflow limitation. Contrast echocardiography demonstrated intrapulmonary shunt. Alpha-1- anti-trypsin was not deficient. The peripheral cyanosis resolved with oxygen therapy. He was initially commenced on pentoxyfylline but terminated therapy due to side effects.
The relevant literature was reviewed. Liver transplantation is the only effective therapy for HPS and prompt recognition and referral for transplant is key.
We therefore present the first documented and published case of hepatopulmonary syndrome in Nigeria and the West African sub-region.
Keywords: Hepatopulmonary syndrome, Nigeria, alcoholic liver cirrhosis