Seher KIR, MEHMET FATİH ERMİŞ, Ali KUTLUCAN, Orhan KOCAMAN, Muhammet Ali KAYIKÇI, YUSUF AYDIN

A rare clinical entity: Two cases of retroperitoneal fibrosis with different approach and consequences

Retroperitoneal fibroz, kronik inşamasyon ve belirgin fibroz içeren sıklıkla üreterleri ya da diğer intraabdominal organları çevreleyen retroperitoneal bir dokunun varlığı ile karakterize nadir bir hastalıktır. Erken dönem semptomları karında ve lumbar bölgede rahatsızlık şeklinde nonpesifik özelliktedir. Fibroz ilerledikçe oluşan bası etkisi septomlarda artışı belirler. Nadir bir klinik durum olması ve klinik ve fizik muayene bulgularının nonspesifik olması çoğu zaman teşhisi geciktirir ve kalıcı organ yetmezliği ve mortaliteye yol açar. Biz burada hastalığın farklı evrelerinde tanı almış ve farklı şekillerde sonuçlanmış iki retroperitoneal fibroz olgusunu sunduk. Amacımız erken teşhisin organ fonksiyonlarını korumadaki önemini vurgulamaktır.

Nadir bir klinik durum: Farkl yaklaﬂm ve sonlanmlarla iki retroperitoneal fibrozis olgusu

Retroperitoneal fibrosis is a rare disease, characterized by the presen- ce of a retroperitoneal tissue, consisting of chronic inflammation and marked fibrosis, which often entraps the ureters or other abdominal organs. Early symptoms are nonspecific as abdominal or lumbar dis- comfort. As the fibrosis progresses, the compressive effects determi- ne the symptomatic evolution. Retroperitoneal fibrosis diagnosis is usually delayed, which can result in permanent organ failure and mor- tality. We present herein two cases of retroperitoneal fibrosis diagno- sed in different stages of the disease and resulting in different outco- mes. Our aim is to stress the importance of early diagnosis in preser- ving organ function.

PDF

___

1.Gilkeson GS, Allen NB. Retroperitoneal fibrosis: a true connective tissue disease. Rheum Dis Clin North Am 1996; 22: 23-38.
2.Cronin CG, Lohan DG, Blake MA, Roche C, McCarthy P, Murphy JM. Retroperitoneal fibrosis: a review of clinical features and ima- ging findings. AJR Am J Roentgenol 2008; 191: 423-31.
3.Bouhabel A, Takoucht F, Meier P. Idiopathic retroperitoneal fibro- sis revealed by renal colic. Saudi J Kidney Dis Transplant 2005; 16: 78-80.
4.Pizzini AM, Corrado S, Radighieri E, Ferretti G, Carani C, Papi G. Hashimoto's thyroiditis associated with idiopathic retroperitoneal fibrosis: case report and review of the literature. Int J Clin Pract 2007; 61: 162-4.
5.Hughes D, Buckley PJ. Idiopathic retroperitoneal fibrosis is a mac- rophage-rich process. Implications for its pathogenesis and treat- ment. Am J Surg Pathol 1993; 17: 482-90.
6.Vaglio A, Salvarani C, Buzio C. Retroperitoneal fibrosis. Lancet 2006; 367: 241-51.
7.Lee JE, Han SH, Kim DK, et al. Idiopathic retroperitoneal fibrosis as- sociated with Hashimoto's thyroiditis in an old-aged man. Yonsei Med J 2008; 49: 1032-5.
8.Takashima T, Onoda N, Ishikawa T, et al. Tumor forming idiopat- hic retroperitoneal fibrosis: Report of a case. Surg Today 2004; 34: 374-8.
9.Van Bommel EF. Retroperitoneal fibrosis. Neth J Med 2002; 60: 231-42.
10.Vaglio A, Corradi D, Manenti L, et al. Evidence of autoimmunity in chronic periaortitis: a prospective study. Am J Med 2003; 114: 454-62.
11.Vivas I, Nicolas AI, Velazquez P, et al. Retroperitoneal fibrosis: typi- cal and atypical manifestations. Br J Radiol 2000; 73: 214-22.
12.Kottra JJ, Dunnick NR. Retroperitoneal fibrosis. Radiol Clin North Am 1996; 43: 1259-75.
13.Burn PR, Singh S, Barbar S, et al. Role of gadolinium-enhanced magnetic resonance imaging in retroperitoneal fibrosis. Can Assoc Radiol J 2002; 53: 168-70.
14.Monev S. Idiopathic retroperitoneal fibrosis: prompt diagnosis pre- serves organ function. Cleve Clin J Med 2002; 69: 160-6.
15.Warakulle DR, Prematilleke I, Moore NR. Retroperitoneal fibrosis mimicking retrocrural lymphadenopathy. Clin Radiol 2004; 59: 292-3.