Transjugular renal biopsy in a case of nephrotic syndrome with extrahepatic portal venous obstruction
A 25-year-old male patient, a known case of extrahepatic portal venous obstruction and portal hypertension, was detected to have nephrotic syndrome during his pre-anesthetic evaluation for hydrocele surgery. He was told that his surgery was deferred till his kidney disease was evaluated and treated. It was felt that the worsening ascites and subsequent scrotal swelling was aggravated by the presence of his nephrotic state, and hence correction of his nephrotic state will reduce post-operative complications and recurrence of his scrotal swelling. He was subsequently referred to our hospital for his renal evaluation and management.\nAt the age of 12 years, he had an episode of hematemesis and was evaluated and found to have esophageal varices. Evaluation of his portal hypertension revealed the presence of extrahepatic portal venous obstruction. He was managed with oral propranolol. He stayed asymptomatic except for minimal ascites. His liver functions on regular follow-up stayed normal.\nThree months prior to this admission, he started developing scrotal swelling, abdominal distension and pedal edema for which he consulted a surgeon. Physical examination revealed pallor, pedal oedema, tense ascites, splenomegaly and bilateral hydrocele. Laboratory investigations showed evidence of anemia, thrombocytopenia, renal insufficiency hypoalbuminemia, and normal liver enzymes. His coagulation work up was normal. His cardiac evaluation did not reveal any abnormality. His urine analysis revealed the presence of proteinuria. Ultrasonography of the abdomen showed massive splenomegaly, ascites and no evidence of liver cirrhosis. Liver biopsy showed normal liver architecture with no abnormality. Adiagnosis of nephrotic syndrome was made and the bilateral hydrocele repair was postponed, and the patient was referred to the Nephrology services of our hospital for the management of nephrotic syndrome and renal insufficiency.\nThe laboratory investigations in our hospital are shown in .\nA renal biopsy was advised for histopathological diagnosis. However, due to pancytopenia and tense ascitis, a percutaneous renal biopsy was deferred and open renal biopsy was considered. An open surgical biopsy was also considered risky as the anatomical position of his kidneys necessitated 12th rib resection for renal access. The case was discussed with the interventional radiologists whose opinion was sought for management of hypersplenism. It was felt that during his partial splenic embolisation via the transjugular route, a renal biopsy can be performed. Transjugular renal biopsy along with splenic embolization was thus advised and after optimisation with blood and blood products the patient underwent both the procedures in the same sitting. For the transjugular renal biopsy, 8 Fr guiding sheath was guided into the lower pole of right (R) renal vein. The long transjugular liver biopsy needle (Cook USA) was used to get a core biopsy. The needle tip was protected with a 5 Fr catheter. Under fluoroscopic control, the needle was positioned deep in the vein, allowing the tip to protrude into the renal cortex []. No post procedure complications like drop in hematocrit, gross hematuria or perirenal hematoma were encountered.\nPost procedure his platelet counts improved to 120,000/μL (vs. 77,000) and WBC count to 16,300, three days after the splenic embolization. However, the biopsy specimen was inadequate for an opinion and a re-biopsy through the transjugular approach was done with no complications []. Biopsy was suggestive of minimal change disease. He was started on oral steroids. His condition gradually improved with resolution of pallor and pedal edema and ascites. Pre-steroid urine protein/creatinine ratio was 11.67 which improved to 3.02 after 1week of steroids and ACE inhibitors. His haemoglobin and albumin levels improved subsequently. He underwent bilateral hydocele repair successfully with no complications. Predischarge he received his pneumococcal vaccination. He is now asymptomatic and under regular follow-up. At 6-months post follow-up, he was in clinical and biochemical remission for his nephrotic syndrome and had normal renal functions.