Newman K D, Torres V E, Rakela J, Nagorney D M
Department of Surgery, Mayo Clinic, Rochester, Minnesota.
Ann Surg. 1990 Jul;212(1):30-7. doi: 10.1097/00000658-199007000-00005.
Polycystic liver disease (PLD) associated with autosomal dominant polycystic kidney disease is usually well tolerated. However there is a small subset of patients who become incapacitated by massive liver enlargement and for whom effective nonsurgical therapy is limited. Recent surgical advances in the treatment of PLD have raised uncertainties regarding proper management of these highly symptomatic patients. We have reviewed our recent experience with a combined hepatic resection-fenestration procedure to assess its efficacy in nine patients. All patients underwent resection of two or more liver segments and extensive fenestration of residual cysts in the remnant liver. Symptomatic relief and reduction in abdominal girth were obtained in eight surviving patients, persisting for an average follow-up period of 17 months. No progression of cystic disease has been observed clinically or by computed tomography and hepatic function was preserved. Three patients had no complications. Five patients had complications including transient right pleural effusion (3) and thrombosis of an arteriovenous fistula (2). One patient who had a previous hepatic cyst fenestration and a cadaveric renal transplantation died after operation of an intracerebral hemorrhage after experiencing coagulopathy, hyperbilirubinemia, and sepsis. Our results suggest (1) some highly symptomatic patients with massive PLD may benefit from combined hepatic resection and fenestration with acceptable risk, and (2) previous liver surgery and immunosuppressive therapy may increase the risk of such surgery. Longer follow-up is needed in a larger number of patients to determine the duration of benefit from the combined resection-fenestration procedure for highly symptomatic PLD.
与常染色体显性遗传性多囊肾病相关的多囊肝病通常耐受性良好。然而，有一小部分患者因肝脏巨大肿大而丧失能力，且有效的非手术治疗有限。多囊肝病治疗方面的最新手术进展引发了对于这些症状严重患者恰当管理的不确定性。我们回顾了近期采用肝切除联合开窗术治疗9例患者的经验，以评估其疗效。所有患者均接受了两个或更多肝段的切除以及对残余肝脏中残留囊肿的广泛开窗术。8例存活患者症状得到缓解，腹围减小，平均随访期为17个月。临床或通过计算机断层扫描均未观察到囊肿疾病进展，肝功能得以保留。3例患者无并发症。5例患者出现并发症，包括短暂性右侧胸腔积液（3例）和动静脉瘘血栓形成（2例）。1例曾接受肝囊肿开窗术和尸体肾移植的患者，在经历凝血障碍、高胆红素血症和脓毒症后，术后死于脑出血。我们的结果表明：（1）一些症状严重的巨大多囊肝病患者可能从肝切除联合开窗术中获益，且风险可接受；（2）既往肝脏手术和免疫抑制治疗可能增加此类手术的风险。需要对更多患者进行更长时间的随访，以确定症状严重的多囊肝病患者接受切除联合开窗术的获益持续时间。
