腹膜后纤维化三例及三十余年国内文献分析

Retroperitoneal fibrosis: report of 3 cases and reviews of the literature in recent thirty years

目的总结了北京协和医院３例和国内１９６３年以来报道的共４４例腹膜后纤维化，旨在提高对该病的警惕和早期诊断率。方法和结果（１）临床表现：该病发病男性略高（１．４：１）。首发症状虽可各异，多以腰背痛、尿闭和腹痛为首发症状，常伴下肢水肿和腹水的体征。（２）诊断：对腹膜后占位性病变，ＣＴ检出的阳性率（８８．９％）较Ｂ超（２０．６％）高，而Ｂ超和静脉肾盂造影对肾盂和输尿管上段病变敏感，其阳性率分别为８６．２％和６９．２％。如疑为下输尿管病变则应考虑做逆行肾盂造影，其阳性率为８４．２％。在腹膜后纤维化中，特发与继发之比为２．１：１，在继发患者中合并消化道肿瘤占３５．７％（５／１４）。（３）治疗：内科治疗目前仍以激素为主，新近应用他莫西芬，其疗效有待进一步观察。但晚期需外科治疗。结论腹膜后纤维化确诊需经手术和病理。影像学检查对腹膜后纤维化的诊断和鉴别诊断较为重要。对继发腹膜后纤维化应警惕是否合并消化道肿瘤。早期与中期以内科治疗，而晚期则需外科手术治疗。

Objective A total of 44 cases of retroperitoneal fibrosis (RPF) reported in the literature in recent 30 years were reviewed, including 3 cases from Peking Union Medical College Hospital and 41 cases from other hospitals. The purpose of this paper is to analyze the clinical manifestations, so that diagnosis may be made and treatment instituted as early as possible. Methods and Results (1) Clinical symptoms and signs: The incidence of RPF in male was slightly higher than that in female (1.4∶1). The initial symptom of RPF was quite variable, but back or flank pain, abdominal pain and oliguria with physical signs of either swollen leg or ascites were most commonly seen. (2) Diagnosis: For masses in retroperitoneal space, CT is considered as the diagnostic method of first choice, because the diagnostic rate of CT (88.9%) is much higher than that of B mode ultrasonography (20.6%). However, B mode ultrasonography (86.2%) and intravenous pyelography (69.2%) were more sensitive methods for the involvement of renal pelvis and upper ureter. For lesions in lower ureter, retrograde pyelography (84.6%) should be first considered. The ratio of idiopathic RPF and secondary RPF is about 2.1∶1. (3) Treatment: Use of cortisone was essential in longterm control. Tamoxifen has also been used recently. Conclusion The final diagnosis of RPF depends on pathology and surgery. Image examination is quite important in diagnosis and differential diagnosis. It is suggested that presence of gastrointestinal tumor should be noted in case of secondary RPF. Drug therapy is only used in the early or middle stage of RPF, while surgical therapy should be resorted to in the late stage of RPF.