Nodular regenerative hyperplasia: a controversial indication for orthotopic liver transplantation

Abstract

Nodular regenerative hyperplasia of the liver is an uncommon cause of portal hypertension. Patients with nodular regenerative hyperplasia have signs and symptoms of portal hypertension, without evidence of hepatocellular failure or encephalopathy. We report the case of a 44-year-old woman with recurrent esophageal bleeding and refractory ascites who had a history of hemosiderosis, hepatitis C, and chronic renal allograft rejection. Our preoperative diagnosis was cirrhotic end-stage liver disease and end-stage renal disease for which the patient underwent combined hepatic and renal transplantation. Her portal hypertension symptoms resolved, and her renal function has been normal for 18 months of follow-up. Histologic examination of the liver revealed nodular regenerative hyperplasia, and a review of the literature regarding the surgical management of patients with nodular regenerative hyperplasia revealed that various shunting procedures are generally recommended. After the failure of medical management in patients with nodular regenerative hyperplasia, portosystemic shunting may be indicated before proceeding to hepatic transplantation.

Orthotopic Liver Transplantation for Idiopathic Portal Hypertension: Indications and Outcome

“…In our patients, as in previously reported patients, OLT was decided on because of the rapidly progressive, life-threatening complications of portal hypertension and liver disease and the inef cacy of surgical or radiological porto-systemic shunting, as attempted in three of our patients. In two of our patients, combined liver and renal transplantation was performed because of associated severe renal failure; this association has been reported in two previous cases (10,14). In our series, the delay between the initial diagnosis and OLT was short: 4.5 years (range 3-10).…”

“…The histopathological diagnosis was NRHL in eight patients (7)(8)(9)(10)(11)14), ISC in two (12,13) and partial nodular transformation of the liver in one (14). In our patients, as in previously reported patients, OLT was decided on because of the rapidly progressive, life-threatening complications of portal hypertension and liver disease and the inef cacy of surgical or radiological porto-systemic shunting, as attempted in three of our patients.…”

“…Such complications are usually controlled by medical treatment and rarely require surgical portosystemic shunting (3)(4)(5)(6). In a very few patients, orthotopic liver transplantation has been required because of the occurrence of life-threatening complications of portal hypertension or severe hepatocellular insuf ciency (7)(8)(9)(10)(11)(12)(13)(14).…”

Early Hepatic Nodular Hyperplasia and Submicroscopic Fibrosis Associated With 6-Thioguanine Therapy in Inflammatory Bowel Disease

“…26 Clinically, NH may be without signs or symptoms, may be associated with biochemical abnormalities in the absence of physical signs, and may also be the cause of profound portal hypertension. 21,22,26,28,32,35,55,65 There is a long list of associated conditions, 22,55,66 and the presence of portal hypertension in a patient with one of these disorders should suggest the possibility of NH.…”

Spectrum of Idiopathic Noncirrhotic Portal Hypertension

“…In these patients the complications of portal hypertension are sufficiently severe to warrant liver transplantation. [12][13][14][15][16][17] In a recent study, Saigal et al 7 have demonstrated that small fraction of patients with NCPF (INCPH) (∼5%) do develop ascites, jaundice, and hepatic encephalopathy making it difficult to differentiate it from cirrhosis of liver. The diagnosis of NCPF (INCPH) was missed prior to the liver transplant and had a pre-transplant diagnosis of cirrhosis of unknown or some known etiology.…”