Improved severe hepatopulmonary syndrome after liver transplantation in an adolescent with end-stage liver disease secondary to biliary atresia
A 16 years-old female was born with biliary atresia, and she underwent porto-enterostomy (Kasai's procedure) on the 55th day after birth. She had intermittent attacks of cholangitis during her childhood. She had been pretty good and developed normally until the age of 15 years. Although she had hepatosplenomegaly, there was no overt symptom. However, she had progressed liver failure aggressively for 6 months before LT. In addition, she was on a bed ridden status because of dyspnea for last 2 months before LT. Both fingers showed clubbing (), ankles were swollen and lips were cyanotic, those were suspected as secondary changes of chronic respiratory insufficiency. Her chest X-ray showed no abnormal findings, except elevated left diaphragm due to splenomegaly. However, her partial pressure of arterial oxygen and arterial oxygen saturation was markedly decreased (PaO2 54.5 mmHg, O2sat 84.2%). Her hepatic and renal profiles were as follows: total bilirubin 42.3 mg/dL, albumin 2.5 g/dL, AST 241 U/L, ALT 93 U/L, INR 2.39, ammonia 2.54 µg/mL and creatinine 0.5 mg/dL. Her Child-Turcotte-Pugh classification was C and MELD (Model of End-Stage Liver Disease) score was 30. The transesophageal echocardiography (TEE) with agitated saline bubble showed an extracardiac right to left shunt, which suggested intrapulmonary arterio-venous shunt ().\nShe underwent orthotopic LT donated from a 13-year old deceased donor, in February 2010. For the liver transplant procedure, there shows marked adhesion of entire perihepatic area, and 1.5 cm sized two gallstones in the Roux-limb of jejunal loop, just above the jejunal stenosis, which was removed by jejunotomy. Otherwise, there was no notable event during a routine orthotopic LT procedure. The first PaO2/O2sat level of postoperative 2nd day was 51.3 mmHg/88.0%; however, she was free from ventilator on the morning of that day. In spite of supplying oxygen with a mask, the lower partial pressure of arterial oxygen was persisted, ranging from 36.2 to 53.9 mmHg during the 48 hours after extubation. Although her PaO2 and O2sat level were shown to be very low, her breathing and physical activity with consciousness was rapidly recovered and very stable. She was transferred from the ICU to the isolation ward, on postoperative day 17. Bile leakage was developed from the enterotomy site to remove the gallstones, which was detected by DISIDA scan; it was healed spontaneously by percutaneous drainage and conservative management. Her partial pressure of arterial oxygen remained as low as 55-60 mmHg during a month after surgery. She was allowed to go home on postoperative day 75.\nRoutine regimen for immunosuppression was applied. Steroid and Simulect® (basiliximab) were administered during anhepatic period, and Prograf® (tacrolimus) and Cellcept® (mycophenolate mofetil) were added on postoperative day 2 and was followed by a routine protocol of our institution. The steroid was tapered until postoperative month 3 and mycophenolate mofetil was used for postoperative 6 months. Tacrolimus monotheray has been maintained since postoperative month 6.\nThe partial pressure of arterial oxygen improved progressively during the postoperative follow up period and on postoperative month 11, the level was normalized (PaO2 118 mmHg). Follow-up transthoracic echocardiography (TTE) with agitated saline bubble on postoperative 11th months did not show pulmonary arteriovenous shunt any more (). Clubbing of both fingers also recovered to normal shape in postoperative month 11 (). The patient recovered successfully, not only physically, but also pychosocial activity, including school performance during the 48-months follow-up period.