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Treatment of portal hypertension in children

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Abstract

The management of children with portal hypertension (PH) has substantially changed owing to the good results and broader application of both endoscopic sclerotherapy and orthotopic liver transplantation (OLT). Since the introduction of sclerotherapy for the treatment of bleeding esophageal varices, the number of surgical procedures has sharply decreased. Until the early 1980s, however, the treatment of choice of bleeding esophageal varices was based on different variations of two main types of open surgery: devacularization and transection operations and portosystemic shunts. The experience with nonshunt procedures is limited in the pediatric population. Literature reports from the last 25 years have emphasized a number of restrictions related to portosystemic shunts in small subjects. However, portosystemic shunts, selective or not, can be performed even in very young subjects with high rates of success. From 1974 to 1984 the distal splenorenal shunt (DSRS) was the procedure of choice for the treatment of children with variceal bleeding in our institution. Forty-two children underwent DSRS during this period. Since 1985, when endoscopic variceal sclerotherapy (EVS) replaced DSRS as the first therapeutic option in our service, this shunt has been performed in only 8 children in whom EVS has failed, none of them during the last 2 years. In this cohort of 50 cases of DSRS, the shunt patency has increased from 71% in the first 7 patients to 95% thereafter. There has been no perioperative mortality. From 1985 to April 1993, 107 children were submitted to EVS sessions for the treatment of esophageal varices bleeding. Among the first 62 patients, complete obliteration of the varices was obtained in 85%, and the incidence of upper gastrointestinal (GI) hemorrhage before total obliteration of the varices was 53%. In the next 45 children, such indexes changed to 96% and 18%, respectively, due to the shorter interval between EVS sessions applied in this group. Currently, we are conducting a prospective, randomized, trial to evaluate the prophylactic value of EVS: 49 patients have received such prophylaxis, and 28 have not (controls). There has been no statistically significant difference in the incidence of upper GI hemorrhage in the two groups: respectively, 6% and 7%. However, the source of bleeding has been different in each group, being always variceal in the control group and hemorrhagic gastritis in the prophylaxis group. In conclusion: (1) EVS is safe and highly effective for the control of esophageal bleeding from PH in children. It is currently the first option of treatment; (2) DSRS still plays an important role in the management of PH in the pediatric population. It is the procedure of choice in cases of EVS failure; (3) Although prophylactic sclerotherapy can prevent only esophageal varices hemorrhage in children with PH, it has not proved to decrease the incidence of upper GI hemorrhage up to this time; (4) OLT is the only definitive treatment of end-stage liver disease, with or without previous portosystemic shunt.

Résumé

Le traitement des enfants ayant une hypertension portale (HTP) a radicalement changé durant ces dernières années, grâce la fois l'amélioration des résultats et une plus large application des méthodes de sclérothérapie et la transplantation hépatique orthotopique (THO). Depuis l'introduction dans l'arsenal thérapeutique de la sclérothérapie, le nombre d'interventions chirurgicales a fortement diminué. Jusqu'en 1980, cependant, le traitement de choix dans l'hémorragie par rupture de varices avait été soit la transsection (dévascularisation) soit l'anastomose porto-cave. L'expérience des interventions sans anastomose est limitée chez l'enfant. De même, la littérature de ces dernières 25 années a souligné les restrictions concernant la pratique d'anastomoses chez l'enfant. L'anastomose portocave, sélective ou pas, peut cependant être proposée l'enfant, avec un taux de succès non négligeable. Entre 1974 et 1984, l'anastomose splénorénale distale (ASRD) a été le procédé de choix dans notre institution pour les enfants ayant saigné de varices oesophagiennes. Quarante-deux enfants ont eu ce procédé durant cette période. Après 1985, date laquelle la sclérothérapie endoscopique (STE) a été introduite, nous préférons celle-ci et depuis lors, seulement huit anastomoses chirurgicales ont été réalisées, toutes avant 1991, et ceci uniquement après échec de la STE. Dans cette cohorte de 50 cas d'ASRD, la perméabilité des anastomoses est passée de 71% chez les sept premiers patients, 95% après. II n'y a eu aucune mortalité périopératoire. De 1985 Avril 1993, 107 enfants ont eu la STE pour une hémorragie par rupture des varices oesophagiennes. Un oblitération complète des varices a été obtenu chez 85% des 62 premiers patients, l'incidence d'hémorragie avant l'obtention d'oblitération complète par STE a été de 53%. Chez les 45 enfants suivants, ces mêmes chiffres ont été respectivement de 96% et 18%, vraisemblablement en raison de la courte intervalle entre les séances de STE. Nous avons entrepris un essais randomisé et contrôlé pour évaluer le rôle prophylactique de la STE. Quarante-neuf patients on eu cette prophylaxie alors que 28 ne l'ont pas eue (témoins). Le taux d'hémorragie ne diffère pas statistiquement entre les deux groupes, 6 et 7%, respectivement. La source d'hémorragie dans chaque groupe était différente dans chaque groupe, étant toujours d'origine variqueuse dans le groupe contrôle alors qu'elle était d'origine gastritique dans le groupe prophylactique. En conclusion, 1) la STE est sûre et efficace chez l'enfant ayant saigné de varices oesophagiennes et doit être la première modalité thérapeutique mettre en oeuvre. 2) L'ASRD joue encore un rôle dans la thérapeutique chez l'enfant. C'est le procédé de choix en cas d'échec de STE. 3) Alors que la STE semble prévenir l'hémorragie provenant des varices oesophagiennes, elle ne permet pas de diminuer l'incidence d'hémorragie du tube digestif supérieur. 4) La THO semble être le seul traitement définitif des maladies hépatiques terminales, avec ou sans anastomoses portocaves.

Resumen

El manejo de los niños con hipertensión portal (HP) ha cambiado sustancialmente en la última década, en virtud de los buenos resultados y la más amplia aplicación tanto de la escleroterapia endoscópica como del trasplate ortotópico (THO). Desde la introducción de la escleroterapia en el tratemiento de las várices sangrantes, se registra una marcadad disminución en el número de procedimientos quirúrgicos. Hasta el comienzo de los años 1980, sin embargo, el tratamiento de escogencia para las várices sangrante se fundamentaba en las variaciones de dos tipos principales de cirugía abierta, la operación de desvascularización y transección, y los “shunts” portasistémicos. La experiencia con los procedimientos no derivativos es limitada en la población pediátrica. La literatura de los últimos 25 años hace énfasis en las numerosas limitaciones de los “shunts” portasistémicos en los pacientes pediátricos.

Sin embargo, tales “shunts”, sean electivos o no, pueden ser practicados muy exitosamente aún en pacientes de tamaño muy pequeño. Entre 1974 y 1984 el “shunt” espleno-renal distal (SERD) fue el procedimiento de elección en el tratamiento de niños con HP en nuestra institución. Cuarenta y dos niños fueron sometidos a SERD en este periodo, pero desde 1985, cuando la escleroterapia endoscópica de las várices (EEV) sustituyó al SERD como la primera opción terapéutica en nuestro servicio, tal “shunt” únicamente ha sido practicado en 8 niños en quienes hubo falla de la EEV, y en ninguno en los últimos dos años.

En este cohorte de 50 casos de SERD, la tasa de permeabilidad del “shunt” ascendió de 71% en los primero 7 pacientes a 95% en el resto de la serie. No se ha registrado mortalidad perioperatoria.

Entre 1985 y Abril de 1993, 107 niños han sido sometidos a sesiones de EEV como tratamiento de la hemorragia varicosa. En los primeros 62 pacientes se logró la erradicación total de las várices en 85% de ellos, y la incidencia de hemorragia gastrointestinal (GI) antes de la obliteración total fue de 53%. En los siguientes 45 niños, tales cifras cambiaron a 96% y 18% respectivamente, gracias a intervalos menores entre las sesiones de EEV. Actualmente adelantamos un ensayo prospectivo y randomizado para valorar la EEV profiláctrica; 49 pacientes han sido sometidos a EEV, y 28 nó, los cuales sirven como controles. No se registra una diferencia estadísticamente significativa en la incidencia de hemorragia GI entre los dos grupos: 6% y 7% rspectivamente. Sin embargo, el origen de la hemorragia ha sido diferente, siendo siempre de origen varicoso en el grupo control y por gastritis hemorrágica en el grupo profiláctico.

En conclusión: 1) La EEV es un procedimiento seguro y altamente efectivo en el control del sangrado esofágico en niños con HP. En el momento actual representa la primera opción terapéutica. 2) El SERD todavía juega un papel importante en el manejo de la HP en la población pediátrica, y es el procedimiento de escogencia cuando hay falla de la EEV. 3) Aunque la escleroterapia profiláctica puede prevenir solamente la hemorragia por várices en los niños con HP, no ha demostrado que disminuya la incidencia de hemorragia GI superior, hasta el momento. 4) El THO representa el único tratamiento definitivo para la enfermedad hepática terminal, en pacientes que hayan sido sometidos o no a “shunts” portasistémico.

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  1. Division of Pediatric Surgery, Department of Surgery, University of São Paulo Medical School, Av. Dr. Arnaldo, 455-sala 416, 01246-000, São Paulo, S.P., Brazil

    João Gilberto Maksoud M.D. & Manoel Ernesto Peçanha Gonçalves M.D.

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Maksoud, J.G., Peçanha Gonçalves, M.E. Treatment of portal hypertension in children. World J. Surg. 18, 251–258 (1994). https://doi.org/10.1007/BF00294410

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