Extrahepatic portal venous obstruction (EHPVO) and temporomandibular joint (TMJ) ankylosisis are significant problems in Asian countries. Both EHPVO and bilateral TMJ ankylosis may have rare association due to protein C and S deficiency which may cause hypercoagulability as well as reduced fibrinolytic activity. Ankylosis arising in early childhood is associated with facial asymmetry, feeding difficulty and speech development alterations. It is also associated with great challenges of endoscopic management in extra hepatic portal vein obstruction (EHPVO) with variceal bleed as well as air way management during surgical management and post-operative recovery. Recently a case series had shown association of TMJ ankylosis with EHPVO due to protein C deficiency which might be an etiological factor for both EHPVO as well as TMJ ankylosis. This case report documents a case of 14 year young girl who had TMJ ankylosis due to ear infection and EHPVO with esophageal varices had multiple episodes of upper GI bleed with mild deficiency of protein C and S, successfully managed with proximal splenorenal shunt to prevent further episodes of upper GI bleed, as endoscopic management is not feasible due to TMJ ankylosis.
Keywords: EHPVO; Proximal splenorenal shunt; TMJ ankylosis; Variceal bleed.