2007
DOI: 10.1002/ppul.20713
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Partial splenectomy for portal hypertension in cystic fibrosis related liver disease

Abstract: Our results show that PS is a reliable and well-tolerated technique. Therefore, it is a therapeutic option for the management of PHT in CF patients with a preserved liver function. It can prevent and significantly delay a liver transplantation and its constraints.

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Cited by 26 publications
(15 citation statements)
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References 28 publications
(48 reference statements)
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“…Since this latter is mostly performed in children, with specific technical issues associated with patient small size and spleen big size, a comparison with partial splenectomy in adults is difficult. In fact, partial splenectomy in children is reported to have longer operative time [21,26] than in adults [18], both when performed by laparotomy and laparoscopy. Surprisingly, the reported morbidity of partial splenectomy in young patients is very low and not severe [5,29], whereas major complications are reported in adult patients undergoing partial splenectomy for splenic cysts, including splenic hemorrhage or infarction requiring total splenectomy [11,16,26].…”
Section: Discussionmentioning
confidence: 98%
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“…Since this latter is mostly performed in children, with specific technical issues associated with patient small size and spleen big size, a comparison with partial splenectomy in adults is difficult. In fact, partial splenectomy in children is reported to have longer operative time [21,26] than in adults [18], both when performed by laparotomy and laparoscopy. Surprisingly, the reported morbidity of partial splenectomy in young patients is very low and not severe [5,29], whereas major complications are reported in adult patients undergoing partial splenectomy for splenic cysts, including splenic hemorrhage or infarction requiring total splenectomy [11,16,26].…”
Section: Discussionmentioning
confidence: 98%
“…In fact, partial splenectomy in children is reported to have longer operative time [21,26] than in adults [18], both when performed by laparotomy and laparoscopy. Surprisingly, the reported morbidity of partial splenectomy in young patients is very low and not severe [5,29], whereas major complications are reported in adult patients undergoing partial splenectomy for splenic cysts, including splenic hemorrhage or infarction requiring total splenectomy [11,16,26]. In this light, our technique, which implies the meticulous dissection and selective clamping of the polar vascular pedicle in order to identify the limits between vascularized and non-vascularized parenchyma, seems to reduce to a minimum the risk of a postoperative splenic infarction; moreover, the use of new stapling devices/hemostatic agents may be supposed to allow avoiding intra-and post-operative hemorrhage.…”
Section: Discussionmentioning
confidence: 98%
See 1 more Smart Citation
“…Partial splenectomy gives an alternative solution for patients not only with sickle cell disease but also for other hemoglobinopathies [3], hereditary spherocytosis [8], and portal hypertension in cystic fibrosis-related liver disease [9]. Partial splenectomy has also been performed for children with massive splenomegaly and congenital hemolytic anemias [10].…”
Section: Discussionmentioning
confidence: 99%
“…Symptomatic portal hypertension has been successfully treated with TIPS in long-term follow-up (70 months) without clinical signs of hepatic encephalopathy [31]. Splenic artery embolisation or (partial) splenectomy in children with portal hypertension and splenomegaly can stabilise lung function and delay progression of portal hypertension [32]. Liver cirrhosis can adversely affect respiratory function secondary to organomegaly, ascites and hepatopulmonary syndrome (intrapulmonary shunts).…”
Section: Transjugular Intrahepatic Portosystemic Stent-shunt (Tips) Amentioning
confidence: 99%