The evaluation of patients with primary sclerosing cholangitis-like cholangiogram

Öz

The conditions causing primary sclerosing cholangitis-like cholangiogram are pathologies of the biliary tract due to obstructive, ischemic or toxic events and neoplastic diseases. We aimed to discuss the data of 14 patients with primary sclerosing cholangitis-like cholangiogram in this report. Materials and Methods: In this report, the demographic, clinical and follow-up data of the patients with primary sclerosing cholangitis-like cholangiogram who were either followed in our clinic or were referred to our endoscopic retrograde cholangiopancreatography unit for further investigation between January 2006 and November 2010 are evaluated. Results: The mean age of the 14 patients was 56.36±15.93 years, and 5 (36%) were female. In 12 patients (86%), the underlying causes of the pathology were malignancies with liver metastases; in the remaining 2 patients, the causes were granulomatous hepatitis secondary to tuberculosis (n: 1) and systemic Exophiala dermatitidis infection with biliary involvement (n: 1). In 11 (78.5%) of the patients, the main symptom was icterus, and in 5 of these patients, pruritus and/or pain was accompanying. In 2 patients with malignancy, only biochemical analysis and ultrasonographic findings revealed cholestasis. One patient referred with cholangitis was diagnosed as systemic fungal infection. In all patients with primary sclerosing cholangitis-like cholangiogram, endoscopic biliary drainage was attempted by endoscopic retrograde cholangiography. After endoscopic approach, 7 (58.3%) of the patients with underlying malignancy died due to cholangitis and secondary septic complications, but in the patients with tuberculosis and Exophiala dermatitidis infection, follow-up cholangiograms revealed improvement with medical therapy of the underlying diseases. Conclusions: In patients with liver metastases causing primary sclerosing cholangitis-like cholangiogram, endoscopic drainage by endoscopic retrograde cholangiography worsens the clinical condition. In order to determine the optimal biliary duct suitable for drainage, magnetic resonance cholangiopancreatographic evaluation should be performed. The underlying disease should be treated whatever the cause.

Anahtar Kelimeler

• Primary sclerosing cholangitis-like cholangiogram, endoscopic treatment, course

Primer sklerozan kolanjit benzeri kolanjiyograma sahip hastalarin değerlendirilmesi

Öz

Primer sklerozan kolanjit benzeri kolanjiyograma neden olan durumlar; safra yollarinda obstrüktif, toksik, iskemik patolojilere neden olan durumlar ve neoplastik hastaliklar olarak sayilabilir. Biz burada primer sklerozan kolanjit benzeri kolanjiyograma sahip 14 olguya ait verileri sunmayi amaçladik. Gereç ve Yöntem: Ocak 2006 Kasim 2010 yillari arasinda kliniğimizce takip edilen veya endoskopik retrograd kolanjiyopankreatografi ünitemize değerlendirme amaçli gönderilen primer sklerozan kolanjit benzeri kolanjiyograma sahip 14 hastaya ait bulgular ve seyir değerlendirildi. Bulgular: Hastalarin ortalama yaşi 56.36±15.93 idi ve 5'i (%36) kadindi. 12 hastada (%86) altta yatan malignite söz konusu iken (karaciğere metastaz yapmiş diğer organlara ait malignitesi olan 9 hasta ve primer hepatobiliyer sistem malignitesi olan 3 hasta) diğer 2 hastanin birinde tüberküloza bağli granülomatöz hepatit, diğerinde ise biliyer sistem tutulumunun da eşlik ettiği sistemik Exophiala dermatitidis enfeksiyonu söz konusuydu. Tüm hastalarin 11'inde (%78.5) temel bulgu ikter iken, bunlarin 5'inde iktere ağri ve/veya kaşinti eşlik ediyordu. İki hastada ise malignite söz konusu olup bunlarda yalnizca kan biyokimyasinda ve ultrasonografik değerlendirmede kolestaz lehine bulgular saptanmişti. Kolanjit ile başvuran yalnizca bir hasta mevcuttu ve bu hastaya sistemik mantar enfeksiyonu tanisi konulmuştu. Primer sklerozan kolanjit benzeri kolanjiyogram saptanan tüm hastalara endoskopik retrograd kolanjiyografi ile endoskopik biliyer drenaj tedavileri denenmişti. Malignitesi olan hastalarin 7'si (%58.3) endoskopik girişim sonrasi kolanjit ve buna sekonder gelişen septik komplikasyonlarla kaybedilirken, tüberküloz ve Exophiala dermatitidis enfeksiyonu olan hastalarda altta yatan hastaliğin medikal tedavisi ile kontrol kolanjiogramlarinda belirgin düzelme gözlendi. Sonuç: Sonuçlarimiza göre primer sklerozan kolanjit benzeri kolanjiyogramli hastalarin büyük çoğunluğunu, karaciğere metastaz yapmiş diğer organ malignitelerine sahip hastalar oluşturmaktadir. Karaciğere, primer sklerozan kolanjit benzeri kolanjiograma neden olacak şekilde metastaz yapmiş veya primer hepatobiliyer maligniteli olgularda, endoskopik retrograd kolanjiyografi araciliğiyla yapilacak endoskopik drenaj tedavileri tabloyu daha da kötüleştirebilir. Öncelikle drenaja en uygun safra kanalinin saptanmasi açisindan manyetik rezonans kolanjio- pankreatografik inceleme uygun olabilir.

Anahtar Kelimeler

• Primer sklerozan kolanjit benzeri kolanjiyogram, endoskopik tedavi, seyir

Kaynakça

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