Zito Enzo Saverio, Romagnuolo Giuseppe, La Rocca Francesco, Castriconi Maurizio, De Sena Guido, Chianese Francesco, Bartone Giovanni, Giuliano Maria Elena
Azienda Ospedaliera di Rilievo Nazionale "A. Cardarelli", Unità Operativa Complessa di "Chirurgia Generale" del Dipartimento di Emergenza.
Ann Ital Chir. 2005 Jul-Aug;76(4):367-75; discussion 375-6.
The congenital cystic dilatation of the liver and bile ducts (CDB) is a very uncommon disease. It often appears in females and most frequently in paediatric patients. Its etiopathogenesis is not yet completely defined. Its evolution includes, together with several complications, the malignant transformation. The Authors present the outcomes of their experience based on a series of 5 patients observed from 1991 till today. Apart from the rarity of this disease, their series is so interesting because 4 out of 5 are adult patients and the fifth one is over 15. In addition, differentiating from the literature trend, the group included only male patients. Are also discussed the most important aspects referring to nosology, epidemiology, etiopathogenesis, clinical pattern and its evolution of CDB, looking over a wide review too. The Authors extensively examine the diagnostic problems; owing to that the patients were observed in the period 1991-1999 it is necessary to clarify that some imaging methods, i.e. CSTscan and the bile duct MNR, were not yet introduced. The surgical treatment has been investigated as well, developed during its historical evolution until nowadays and our solutions for the 5 cases have been presented and discussed. In their series the Authors haven't ever observed during hospitalisation a malignant transformation.
先天性肝内胆管囊状扩张症(CDB)是一种非常罕见的疾病。它常发生于女性,最常见于儿科患者。其病因尚未完全明确。其病情发展包括多种并发症以及恶变。作者介绍了他们自1991年至今观察的5例患者的治疗结果。除了这种疾病的罕见性外,他们的病例系列很有意思,因为5例中有4例是成年患者,另一例超过15岁。此外,与文献趋势不同的是,该组仅包括男性患者。文中还讨论了CDB在疾病分类学、流行病学、病因学、临床症状及其发展方面的最重要问题,并进行了广泛的综述。作者深入研究了诊断问题;由于这些患者是在1991 - 1999年期间观察的,需要说明的是,当时一些成像方法,如CT扫描和胆管磁共振成像尚未应用。同时也探讨了手术治疗,介绍了其历史发展至今的情况,并展示和讨论了我们对这5例病例的治疗方案。在他们的病例系列中,作者在住院期间从未观察到恶变情况。