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In 1993, we published the results of our first cystic fibrosis (CF) patients who had complications of liver disease and portal hypertension (PHT), and had been operated on by partial splenectomy (PS). We also presented the results of 11 patients at the Jerusalem CF meeting in 1996. Since 1982, we have operated on and followed up 21 patients (aged 8 to 22 years). All patients had a large spleen measuring 15–28 cm in length, oesophageal varices graded 2 to 4 by endoscopy, hypersplenism with a platelet account below 50 000, and a well documented liver disease treated with UDCA.
Surgical procedure consisted of PS with conservation of the upper lobe of the spleen, terminal haemostasis, and suturation of parenchymentous vessels. The whole procedure lasts 3–4 hours. The only postoperative complications consisted of scar rupture in three cases and a painful episode of a few days in two cases. No pulmonary exacerbation occurred after surgery. A speedy normalisation of the haematological profile was observed. Normal function of the remaining upper lobe of the spleen was registered by scintiscan. An important improvement of oesophageal varices was noticed in nine cases out of 11 and a stable condition observed in two cases. The size of the remaining spleen remained stable in nine patients out of the first group who presented in 1996. No deterioration, and even some improvement of hepatic function was observed.
In conclusion, we believe that the risk of PS is worth taking since it appears to be a good option for the treatment of oesophageal varices, which is the main concern, and also might be the cure for hyperslenism. Partial splenectomy is an important alternative to all other procedures in the treatment of PHT. Moreover, it allows a delay of hepatic transplantation and it may even be avoided altogether.
We intend to present our global results in a near future.