We report a two years old girl who was born preterm at 36 weeks with a birth weight of 2.6 kg to a primi mother by emergency cesarean section due to uncontrolled hypertension. Mother’s age was 24 years and she was hypothyroid, hypertensive and had Type 2 diabetes mellitus. She was on thyroxine, nifedipine and oral hypoglycemics, which was changed to insulin during pregnancy.

Child was put on propranolol and was investigated for secondary causes of hypertension. Renal function test, plasma adrenaline, noradrenaline; urine 24 hours metanephrine, 24 hours VMA; serum cortisol, aldosterone, and renin were within normal limits. CT abdomen showed normal sized kidneys and normal appearing liver and spleen. Echocardiography was with in normal limits. CT renal angiography showed single renal artery on both sides with no coarctation or aneurysm, single renal veins on both sides, abnormal large spenorenal shunt between splenic vein and left renal vein, left renal vein dilated measuring 1.1 cm, shunt measured 0.6 cm, portal vein narrowed to 0.2 cm (Fig.1).

Fig.1 CT renal angiography showing large splenorenal shunt.

Presently the child is on propranolol 5 mg twice a day, and L-thyroxine 50 µg daily. Her growth and milestones are with in normal limits. Her BP and thyroid status is with in range.

Our dilemma was whether hypertension is due to the splenorenal shunt and the future management of this shunt. Pulmonary hypertension has been reported in cases with congenital porto-systemic venous shunt but we could not get any literature reporting systemic hypertension in this condition. Extremely hypoplastic kidneys have been reported in an aborted fetus with congenital splenorenal venous shunt. But ultrasonography shows bilateral normal size kidneys in this child.

It is reported that patients with congenital splenorenal shunt may be asymptomatic in the initial years but develop portosystemic encephalopathy at later stage. Hepatic cirrhosis has also been reported. We are following her with serial ammonia levels which is with in normal limits. CT guided liver biopsy shows normal histology.

1. Ohno T, Muneuchi J, Ihara K, Yuge T, Kanaya Y, Yamaki S, et al. Pulmonary hypertension in patients with congenital portosystemic venous shunt: a previously unrecognized association. Pediatrics. 2008;121:e892-9.

2. Ji EK, Yoo SJ, Kim JH, Cho KS. Congenital splenorenal venous shunt detected by prenatal ultrasonography. J Ultrasound Med. 1999;18:437-9.

3. Ishii Y, Inagaki Y, Hirai K, Aoki T. Hepatic encephalopathy caused by congenital extrahepatic portosystemic venous shunt. J Hepatobiliary Pancreat Surg. 2000;7:524-8.

4. Yamagami T, Nakamura T, Iida S, Kato T, Tanaka O, Matsushima S, et al. Hepatic encephalopathy secondary to intrahepatic portosystemic venous shunt: balloon-occluded retrograde transvenous embolization with n-butyl cyanoacrylate and microcoils. Cardiovasc Intervent Radiol. 2002;25:219-21.

5. Ishii Y, Inagaki Y, Hirai K, Aoki T. Hepatic encephalopathy caused by congenital extrahepatic portosystemic venous shunt. J Hepatobiliary Pancreat Surg. 2000;7:524-8.