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 language | English (US) |
|---|---|
| Pages (from-to) | 1076-1082 |
| Number of pages | 7 |
| Journal | Journal of pediatric surgery |
| Volume | 54 |
| Issue number | 5 |
| DOIs | |
| State | Published - May 1 2019 |
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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
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Cystic fibrosis and portal hypertension : Distal splenorenal shunt can prevent the need for future liver transplant. / Lemoine, Caroline; Lokar, Joan; McColley, Susanna A; Alonso, Estella M; Superina, Riccardo A.
In: Journal of pediatric surgery, Vol. 54, No. 5, 01.05.2019, p. 1076-1082.Research output: Contribution to journal › Article
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 -