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“Endemic” idiopathic portal hypertension: Report on 32 patients with non-cirrhotic portal fibrosis

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Abstract

The clinical features, surgical management, and long term follow up of 32 patients from Iran with idiopathic portal hypertension are reported. Many features of the disease are similar to those reported from India and Japan. The unsuspected finding was a 46% history of marked pica of clay (geophagia) in a subset of 26 patients. In addition, 81% of our patients had a prolonged prothrombin time, despite otherwise normal to minimally abnormal liver function tests. Liver biopsies revealed intrahepatic periportal fibrosis with subintimal thickening of terminal branches, and in many specimens a striking peri-ductular fibrosis was seen in the adjacent bile ducts. The spleen was very large with a dilated artery (external diameter: 11 mm to 15 mm). Portal venous pressure (PVP) was measured intra-operatively before and after clamping the splenic artery (SA). Clamping the SA consistently caused a decreased in PVP which ranged from 2.0 to 18.2 cm water with the mean ± SEM of 9.7 ± 1.5 cm water (p < 0.001, paired t-test). It was equivalent to 32.3 ± 3.6% decrease in PVP. Fifteen selected patients (Group I) were managed with splenectomy with excellent short and long term results. The selection criteria for splenectomy included a decrease in PVP to <24 cm of water after clamping the SA. Three patients from this group were re-examined 10 to 12 years following splenectomy. Cirrhosis had not developed, but the minimal abnormalities in the liver function tests had persisted. Of 17 patients (Group II) requiring a shunting procedure, none of those who survived surgery developed bleeding during the 12 years follow up, however, 2 (12.5%) patients developed encephalopathy.

Résumé

Les données médicales, chirurgicales et les résultats à distance de 32 patients Iraniens ayant une hypertension portale idiopathique ont été analysées. Beaucoup d'aspects sont similaries aux cas rencontés au Japon et en Inde. Une découverte inattendue a été l'existence d'une géophagie argile chez 12 des 26 patients interrogés (46%). Un temps de prothrombine allongé a été trouvé chez 81% de nos patients en dépit de tests fonctionnels hépatiques normaux ou presque normaux. La biopsie hépatique a montré une fibrose périportale intrahépatique avec épaississement de la couche sous intimale des branches termnales (des veines portes), et, dans plusieurs biopsies, une fibrose péricanalaire a été mis en évidence an niveau des canalicules biliaires adjacents. La rate était très hypertrophiée avec une artère splénique dilatée (diamètre externe: 11 à 15 mm). La pression veinuse portale (PVP) a été musurée en peropératoire avant et après le clampage de l'artère splénique (AS). Le clampagne de l'AS a provoqué une chute de la PVP de 2 à 18.2 cm d'H2O: (moyenne ± déviation standard de la moyenne: 9.7 ± 1.5 cm d'H2O (p < 0.001 par le test de Student appartié). Celle-ci correspondait à une chute de 32.3 ± 3.6% de la PVP. Quinze patients sélectionnés (groupe I) ont été traités par splénectomic avec d'excellents résultats à court et à long terme. Le choix de la splénectomie s'est fait chez les patients qui présentaient une diminution nette de la PVP après clampagne de l'AS. Trois de ces patients ont été examinés 10 à 12 ans après la splénectomie. Aucun n'avait de cirrhose, mais des anomalies minimes de la fonction hépatique persistaient. Dix sept patients (groupe II) ont eu une dérivation portosystémique. Aucun patient ayant survécu à la chirurgie n'a resaigné. Cependant, deux patients (12.5%) ont développé une encéphalopathie.

Resumen

Se presentan las características clínicas, el manejo quirúrgico y et seguimiento a largo plazo de 32 pacientes con hipertensión portal idiopática en Irán. Muchas características de la enfermedad demostraron ser similares a las informades en la India y Japón. El hallazgo inesperado fue la historia de geofagia en 46% (12/26) de nuestros pacientes. Además, 81% exhibió un tiempo de trotomina prolongado, con pruebas de función hepática por lo demás mormales o mínimanente alteradas. Las biopsias hepáticas revelaron fibrosis periportal intrahepática con engrosamiento subendothelial de las ramas terminales, y en muchos especímenes una marcada fibrosis peri-ductal en los canalículos biliares adyacentes. El bazo apareció muy aumentado de tamaño con dilatación de la arteria esplénica (diámetro externo: 11–15 mm). La presión venosa portal (PVP) fue medida intra-operatoiramente antes y después de ocluir la arteria esplénica (AE); la oclusión de la AE causó una disminución de la PVP, que exhibió un rango de 2.0 a 18.2 cm de agua con una media ± DME de 9.7 ± 1.5 cm de agua (p < 0.001), equivalente a una disminución de 32.3 ± 3.6%. Quince pacientes seleccionados (Grupo I) fueron manejados con esplenectomía con excelente resultados a corto y largo plazo. Los criterios de selección para esplenectomía incluyeron una determinada disminución de la PVP después de la oclusión de la AE. Tres pacientes de este grupo fueron reexaminados 10 a 12 años después de la esplenectomía; no se encontró cirrosis, pero persistian las mínimas alteraciones de las pruebas de función hepática. De 17 pacientes (Grupo II) que requirieron un “shunt”, ninguno de los que sobrevivió la cirugía desarrolló hemorragia en el lapso de los 12 años de seguimiento; sin embargo dos pacientes (12.5%) desarrollaron encefalopatía.

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Authors and Affiliations

  1. Departments of Surgery and Medicine, Harvard Medical School, Boston, Massachusetts, USA

    Cyrus Vakili M.D., Mohammad J. Farahvash M.D. & T. Edward Bynum M.D.

  2. Division of Gastroenterology and Endoscopy Unit, Tehran University Hospital, Tehran, Iran

    Cyrus Vakili M.D., Mohammad J. Farahvash M.D. & T. Edward Bynum M.D.

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  1. Cyrus Vakili M.D.
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Vakili, C., Farahvash, M.J. & Bynum, T.E. “Endemic” idiopathic portal hypertension: Report on 32 patients with non-cirrhotic portal fibrosis. World J. Surg. 16, 118–124 (1992). https://doi.org/10.1007/BF02067126

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