Partial splenectomy for portal hypertension in cystic fibrosis related liver disease

• Published in: Pediatric pulmonology Vol. 42; no. 12; pp. 1173 - 1180
• Main Authors: Louis, Dominique, Duc, Mai Linh Pham, Reix, Philippe, Chazalette, Jean-Pierre, Durieu, Isabelle, Feigelson, Jean, Bellon, Gabriel
• Format: Journal Article
• Language: English
• Published: Hoboken Wiley Subscription Services, Inc., A Wiley Company 01-12-2007; Wiley-Liss
• Subjects: Adolescent; Adult; Biological and medical sciences; Child; cystic fibrosis; Cystic Fibrosis - complications; Cystic Fibrosis - surgery; Disease Progression; Errors of metabolism; Female; Follow-Up Studies; Gastroenterology. Liver. Pancreas. Abdomen; General aspects; Humans; Hypertension, Portal - etiology; Hypertension, Portal - physiopathology; Hypertension, Portal - surgery; liver disease; Liver Diseases - complications; Liver. Biliary tract. Portal circulation. Exocrine pancreas; Male; Medical sciences; Metabolic diseases; Miscellaneous hereditary metabolic disorders; Other diseases. Semiology; partial splenectomy; Pneumology; portal hypertension; Retrospective Studies; Severity of Illness Index; Splenectomy - methods; Time Factors; Treatment Outcome; Hepatic fibrosis; Pediatrics; Portal circulation disease; liver disease; Respiratory disease; Metabolic diseases; Cardiovascular disease; Cystic fibrosis; Hepatic disease; partial splenectomy; Genetic disease; Vascular disease; Splenectomy; Treatment; Partial; Surgery; Portal hypertension; Digestive diseases; Pancreatic disease; Pneumology
• ISSN: 8755-6863; 1099-0496
• DOI: 10.1002/ppul.20713

Aims
To review the middle‐ and long‐term effects of partial splenectomy (PS) on portal hypertension (PHT) and its complications in patients with cystic fibrosis (CF) related liver disease risky PHT.
Method
Over a 20 years period, 19 patients aged 7–23 years underwent partial PS for massive splenomegaly, hypersplenism, and / or severe PHT.
Results
In all but three cases, PHT and hypersplenism have improved for long periods. Noticeable improvement of hepatic tests occurred simultaneously. In all patients PS resolved abdominal discomfort. Fifteen patients are alive and a stabilization of the liver disease occurred with a follow‐up of 1–20 years (mean 7.9). One patient died following respiratory insufficiency 10 years after PS although PHT was stable. Manifestations recurred in 2 patients 5 and 6 years after PS. In two patients, the course of the disease evolved to hepatic insufficiency without recurrence of PHT 3 and 8 years after PS. PS did not give the expected results in three cases only, in which PHT was not modified or reoccurred during the following year. No severe complication was observed. Early (three patients) or late (one patient) eventration required surgical procedure.
Conclusions
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. Pediatr Pulmonol. 2007; 42:1173–1180. © 2007 Wiley‐Liss, Inc.