Arch Dis Child. Published Online First: 31 May 2006. doi:10.1136/adc.2006.093773
Original articles |
Does splenectomy in cystic fibrosis related liver disease improve lung function and nutritional status? - A case series
1 Royal Children's Hospital, Melbourne, Australia
* To whom correspondence should be addressed. E-mail: barry.linnane{at}rch.org.au.
Accepted 22 May 2006
Abstract
Objective: 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 2 years pre-splenectomy. This contrasts with the
increase in FEV1% predicted of 2 ± 16% in the 2 years
post-splenectomy (p = 0.05). The cumulative gain in WAZ
score (
WAZ pre) over the two years prior to
splenectomy of 0.045 ± 0.69 was not significantly
different from the cumulative gain in WAZ score (
WAZ 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 of 983 grms (SD = 414grms). There were no deaths
associated with splenectomy. The median length of follow
up post-splenectomy was 6.0 years, (range 0.7 to 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.
Keywords: Cystic fibrosis, Nutrition, Portal hypertension, Respiratory function tests, Splenectomy
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Arch. Dis. Child. 2006 91: e5.[Extract] [Full Text] [PDF]
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Van Biervliet, S., Robberecht, E.
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