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Laparoscopic Splenectomy with Pericardial Devascularization for Hypersplenism and Esophageal Variceal Hemorrhage Due to Portal Hypertension
Published on: November 15, 2024
Does splenectomy in cystic fibrosis related liver disease improve lung function and nutritional status? A case series
B Linnane1, M R Oliver, P J Robinson
1Department of Respiratory Medicine, Royal Children's Hospital, Melbourne, Australia. barry.linnane@rch.org.au
Insights
Total splenectomy may improve lung function in children with cystic fibrosis related liver disease (CFLD) and portal hypertension. This surgery appears safe, with no reported deaths or sepsis in this study.
Area of Science:
- Pediatric Surgery
- Pulmonology
- Hepatology
Background:
- Cystic Fibrosis Related Liver Disease (CFLD) can lead to portal hypertension in children.
- Portal hypertension often necessitates surgical intervention, including splenectomy.
- The impact of splenectomy on lung function and nutritional status in CFLD patients is not well-established.
Purpose of the Study:
- To evaluate the effect of total splenectomy on lung function and nutrition in pediatric CFLD patients.
- To document surgical indications and assess short- and long-term risks of splenectomy in this population.
Main Methods:
- Retrospective review of medical records for nine pediatric patients with CFLD who underwent splenectomy.
- Data collected over a 25-year period (1980-2005) at a single tertiary pediatric hospital.
- Analysis of lung function (FEV1% predicted) and nutritional status (WAZ score) pre- and post-splenectomy.
Main Results:
- Lung function (FEV1% predicted) showed a trend towards improvement in the two years post-splenectomy (2+/-16%) compared to the pre-splenectomy period (-16+/-11%, p=0.05).
- Nutritional status (WAZ score) did not significantly change post-splenectomy (0.15+/-0.36) compared to pre-splenectomy (0.045+/-0.69, p=0.65).
- Splenectomy was performed at an average age of 14.8 years, with no associated mortality or episodes of severe infection (bacterial peritonitis, overwhelming sepsis).
Conclusions:
- Splenectomy may offer a potential benefit to lung function in pediatric CFLD patients, possibly manifesting in the second year post-surgery.
- Total splenectomy appears to be a safe procedure for managing CFLD with associated portal hypertension in children.
Aims:
To review the effect of total splenectomy on lung function and nutrition in children with cystic fibrosis related liver disease (CFLD) and associated portal hypertension. The stated indications for surgery and the short and long term risks of the procedure were also documented.
Method:
Over a 25 year period from January 1980 to June 2005, approximately 650 patients with cystic fibrosis (CF) were treated at the Royal Children's Hospital, Melbourne, Australia. Nine patients with CFLD who underwent a splenectomy during that time were identified and their medical records were reviewed.
Results:
FEV1% predicted dropped by -16+/-11% in the two years pre-splenectomy. This contrasts with the increase in FEV1% predicted of 2+/-16% in the two years post-splenectomy (p = 0.05). The cumulative gain in WAZ score (DeltaWAZ pre) over the two years prior to splenectomy of 0.045+/-0.69 was not significantly different from the cumulative gain in WAZ score (DeltaWAZ post) for the two years after splenectomy of 0.15+/-0.36 (p = 0.65). The average age at splenectomy was 14.8 years (SD = 3 years). The average weight of an excised spleen was 983 g (SD = 414 g). There were no deaths associated with splenectomy. The median length of follow up post-splenectomy was 6.0 years (range 0.7-15.8). There were no episodes of bacterial peritonitis or overwhelming sepsis.
Conclusions:
Splenectomy may have a beneficial effect on lung function although this may not manifest itself until the second year post-splenectomy. Splenectomy in patients with CFLD appears to be a safe procedure.
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