Cystic fibrosis and portal hypertension

Distal splenorenal shunt can prevent the need for future liver transplant

Caroline Lemoine, Joan Lokar, Susanna A McColley, Estella M Alonso, Riccardo A Superina*

*Corresponding author for this work

Research output: Contribution to journalArticle

Abstract

Background: The management of portal hypertension (PHT)in children with well compensated cirrhosis and cystic fibrosis (CF)is controversial. We present our experience with distal splenorenal shunting (DSRS)for the treatment of PHT as an alternative to liver transplantation (LT). Methods: Between 2008 and 2017, 5 CF children underwent a DSRS at a pediatric hepatobiliary and transplantation referral center. LT (n = 9)was reserved for patients with decompensated cirrhosis. Statistical analysis was done using the paired t-test (p < 0.05 considered significant). Results: Mean PELD/MELD score was significantly lower for DSRS patients than LT (3 ± 6 vs 28 ± 4, p < 0.001). All 5 DSRS patients had grade III–IV varices. One bled prior to surgery. After DSRS, spleen size decreased significantly from 8.4 ± 1.5 cm to 4.4 ± 1.8 cm (p = 0.019). Mean platelet count remained stable (87.8 ± 48 to 91.8 ± 35, p = 0.9). There were no postoperative complications. No DSRS patient experienced variceal bleeding following shunt creation. Liver function tests remained stable in the DSRS group, and no patient required a liver transplant (median follow up 4.65 years, range 1.24–7.79). Conclusions: Patients with cystic fibrosis who have well-compensated cirrhosis and symptomatic portal hypertension can be palliated with distal splenorenal shunting and do not need liver transplants. These patients can undergo shunting with minimal morbidity. Type of study: Case series with no comparison group. Level of evidence: IV.

Original languageEnglish (US)
Pages (from-to)1076-1082
Number of pages7
JournalJournal of pediatric surgery
Volume54
Issue number5
DOIs
StatePublished - May 1 2019

Fingerprint

Surgical Splenorenal Shunt
Portal Hypertension
Cystic Fibrosis
Transplants
Liver
Liver Transplantation
Fibrosis
Liver Function Tests
Varicose Veins
Platelet Count
Referral and Consultation
Spleen
Transplantation
Pediatrics
Hemorrhage
Morbidity

Keywords

  • Cystic fibrosis
  • Cystic fibrosis-liver related disease
  • Distal splenorenal shunt
  • Liver transplantation
  • Portal hypertension

ASJC Scopus subject areas

  • Surgery
  • Pediatrics, Perinatology, and Child Health

Cite this

@article{66bfa192c16742948779be98c179f3eb,
title = "Cystic fibrosis and portal hypertension: Distal splenorenal shunt can prevent the need for future liver transplant",
abstract = "Background: The management of portal hypertension (PHT)in children with well compensated cirrhosis and cystic fibrosis (CF)is controversial. We present our experience with distal splenorenal shunting (DSRS)for the treatment of PHT as an alternative to liver transplantation (LT). Methods: Between 2008 and 2017, 5 CF children underwent a DSRS at a pediatric hepatobiliary and transplantation referral center. LT (n = 9)was reserved for patients with decompensated cirrhosis. Statistical analysis was done using the paired t-test (p < 0.05 considered significant). Results: Mean PELD/MELD score was significantly lower for DSRS patients than LT (3 ± 6 vs 28 ± 4, p < 0.001). All 5 DSRS patients had grade III–IV varices. One bled prior to surgery. After DSRS, spleen size decreased significantly from 8.4 ± 1.5 cm to 4.4 ± 1.8 cm (p = 0.019). Mean platelet count remained stable (87.8 ± 48 to 91.8 ± 35, p = 0.9). There were no postoperative complications. No DSRS patient experienced variceal bleeding following shunt creation. Liver function tests remained stable in the DSRS group, and no patient required a liver transplant (median follow up 4.65 years, range 1.24–7.79). Conclusions: Patients with cystic fibrosis who have well-compensated cirrhosis and symptomatic portal hypertension can be palliated with distal splenorenal shunting and do not need liver transplants. These patients can undergo shunting with minimal morbidity. Type of study: Case series with no comparison group. Level of evidence: IV.",
keywords = "Cystic fibrosis, Cystic fibrosis-liver related disease, Distal splenorenal shunt, Liver transplantation, Portal hypertension",
author = "Caroline Lemoine and Joan Lokar and McColley, {Susanna A} and Alonso, {Estella M} and Superina, {Riccardo A}",
year = "2019",
month = "5",
day = "1",
doi = "10.1016/j.jpedsurg.2019.01.035",
language = "English (US)",
volume = "54",
pages = "1076--1082",
journal = "Journal of Pediatric Surgery",
issn = "0022-3468",
publisher = "W.B. Saunders Ltd",
number = "5",

}

TY - JOUR

T1 - Cystic fibrosis and portal hypertension

T2 - Distal splenorenal shunt can prevent the need for future liver transplant

AU - Lemoine, Caroline

AU - Lokar, Joan

AU - McColley, Susanna A

AU - Alonso, Estella M

AU - Superina, Riccardo A

PY - 2019/5/1

Y1 - 2019/5/1

N2 - Background: The management of portal hypertension (PHT)in children with well compensated cirrhosis and cystic fibrosis (CF)is controversial. We present our experience with distal splenorenal shunting (DSRS)for the treatment of PHT as an alternative to liver transplantation (LT). Methods: Between 2008 and 2017, 5 CF children underwent a DSRS at a pediatric hepatobiliary and transplantation referral center. LT (n = 9)was reserved for patients with decompensated cirrhosis. Statistical analysis was done using the paired t-test (p < 0.05 considered significant). Results: Mean PELD/MELD score was significantly lower for DSRS patients than LT (3 ± 6 vs 28 ± 4, p < 0.001). All 5 DSRS patients had grade III–IV varices. One bled prior to surgery. After DSRS, spleen size decreased significantly from 8.4 ± 1.5 cm to 4.4 ± 1.8 cm (p = 0.019). Mean platelet count remained stable (87.8 ± 48 to 91.8 ± 35, p = 0.9). There were no postoperative complications. No DSRS patient experienced variceal bleeding following shunt creation. Liver function tests remained stable in the DSRS group, and no patient required a liver transplant (median follow up 4.65 years, range 1.24–7.79). Conclusions: Patients with cystic fibrosis who have well-compensated cirrhosis and symptomatic portal hypertension can be palliated with distal splenorenal shunting and do not need liver transplants. These patients can undergo shunting with minimal morbidity. Type of study: Case series with no comparison group. Level of evidence: IV.

AB - Background: The management of portal hypertension (PHT)in children with well compensated cirrhosis and cystic fibrosis (CF)is controversial. We present our experience with distal splenorenal shunting (DSRS)for the treatment of PHT as an alternative to liver transplantation (LT). Methods: Between 2008 and 2017, 5 CF children underwent a DSRS at a pediatric hepatobiliary and transplantation referral center. LT (n = 9)was reserved for patients with decompensated cirrhosis. Statistical analysis was done using the paired t-test (p < 0.05 considered significant). Results: Mean PELD/MELD score was significantly lower for DSRS patients than LT (3 ± 6 vs 28 ± 4, p < 0.001). All 5 DSRS patients had grade III–IV varices. One bled prior to surgery. After DSRS, spleen size decreased significantly from 8.4 ± 1.5 cm to 4.4 ± 1.8 cm (p = 0.019). Mean platelet count remained stable (87.8 ± 48 to 91.8 ± 35, p = 0.9). There were no postoperative complications. No DSRS patient experienced variceal bleeding following shunt creation. Liver function tests remained stable in the DSRS group, and no patient required a liver transplant (median follow up 4.65 years, range 1.24–7.79). Conclusions: Patients with cystic fibrosis who have well-compensated cirrhosis and symptomatic portal hypertension can be palliated with distal splenorenal shunting and do not need liver transplants. These patients can undergo shunting with minimal morbidity. Type of study: Case series with no comparison group. Level of evidence: IV.

KW - Cystic fibrosis

KW - Cystic fibrosis-liver related disease

KW - Distal splenorenal shunt

KW - Liver transplantation

KW - Portal hypertension

UR - http://www.scopus.com/inward/record.url?scp=85061660092&partnerID=8YFLogxK

UR - http://www.scopus.com/inward/citedby.url?scp=85061660092&partnerID=8YFLogxK

U2 - 10.1016/j.jpedsurg.2019.01.035

DO - 10.1016/j.jpedsurg.2019.01.035

M3 - Article

VL - 54

SP - 1076

EP - 1082

JO - Journal of Pediatric Surgery

JF - Journal of Pediatric Surgery

SN - 0022-3468

IS - 5

ER -