Colectomy for Porto-Systemic Encephalopathy: Is it Still Topical?
A 59-year-old man, was diagnosed as having cirrhosis of unknown etiology since 1993. Viral serology and auto-antibodies were negative. There was neither iron overload, nor alcohol consumption, nor signs of metabolic syndrome. Copper tests were normal. He had 3 episodes of esophageal bleeding between 1993 and 2003 treated successfully with sclerotherapy. The Child-Pugh score was A.\nIn 2003, he had an uncontrolled variceal bleeding refractory to medical and endoscopic therapy. As TIPS was not available, salvage therapy with DSRS was performed in emergency. There was no post-operative complications. Until 2010, he had no recurrent variceal bleeding, no HE and no ascitis.\nIn 2010, he developed a slight tremor of the upper extremities, predominating on the right, but he did not consult.\nOne year later, he presented recurrent episodes of agitation, disorientation, amnesia, stupor and slurred speech. Physical examination disclosed asymmetrical tremor predominating on the right and temporo-spatial disorientation. There was neither focal deficit, nor extra-pyramidal rigidity, nor muscular incoordination. Biochemical analysis revealed normal glycemia and renal function, absence of severe liver dysfunction, no thyroidal disorders, no hypoxia. Ammonemia was not available in our unit. Cerebral computed tomography (CT) scan showed no abnormalities whereas magnetic resonance (MR) disclosed hyperintensity of the globus pallidus on T1 (). Electroencephalogram demonstrated slowing of alpha rhythm. abdominal doppler ultrasound and CT scan showed patency of the spleno-renal shunt, partial portal thrombosis, no signs of hepato-cellular carcinoma.\nThis picture led to the diagnosis of HE associated with porto-systemic shunt. Treatment with lactulose and rifaximin allowed transient resolution of disorientation but tremor persisted. Anticoagulation for portal thrombosis was not effective and dangerous because of mistakes in doses.\nA few weeks later, he presented recurrent episodes of gross disorientation, ideo-motor apraxia and loss of consciousness requiring repeated admissions in intensive care unit. He never became free from symptoms. Lactulose, rifaximin and flumazenil allowed only temporary improvement.\nWe were in front of a persistent and severe HE refractory to medical therapy. The risk of rebleeding after reduction of the shunt was obvious, hepatic transplantation was not available, so we performed a subtotal colectomy with ileo-rectal anastomosis in order to reduce ammonia production. The patient was operated by laparotomy, without intra-operative bleeding. Post-operative course was uneventful, in particular, no postoperative ascites and no need for transfusion. Neurological examination disclosed normal consciousness, intellectual function and behavior while a slight tremor persisted in the right hand. Ten days after the operation, the patient was able to be discharged home.\nIn the follow-up, 17 months later, he was still improved, keeping only the slight tremor of the right hand, and liver function did not worsen. He did not have a recurrence of disorientation, intellectual function and behavior remained normal.