Tan Yu Meng, Ooi London Lucien
Hepatobiliary Unit, Department of Surgery, Singapore General Hospital and Department of Surgical Oncology, National Cancer Center, 11 Hospital Drive, Singapore.
ANZ J Surg. 2004 Aug;74(8):653-7. doi: 10.1111/j.1445-1433.2004.03112.x.
The majority of patients afflicted with adult polycystic liver disease (APLD) are asymptomatic. For those who are symptomatic, there are a variety of treatment procedures that have been proposed but these lack verification through long-term studies with respect to safety and long-term effectiveness. Choice of surgical procedure is related to the severity of APLD and morphology of the cysts within the liver. The aim of the present study was to analyse the immediate and long-term results of fenestration and combined resection-fenestration at Singapore General Hospital.
A retrospective analysis of clinical, operative, imaging and follow-up data was carried out for 12 patients (10 women and two men) with symptomatic APLD who underwent surgery from January 1992 to December 2000. The primary outcome measures assessed were postoperative alleviation of symptoms, performance status, complications, mortality and long-term recurrence of symptoms.
Nine patients underwent 12 fenestration procedures and three patients had combined resection-fenestration. Fenestration was carried out for eight of nine patients with a dominant cyst morphology and combination resection-fenestration was carried out for those three patients with diffuse cyst morphology. There was no operative mortality and all patients were discharged from hospital free of their preoperative symptoms. Overall morbidity rate was 58%. The mean follow up for the present cohort was 29.3 months. Only two patients had recurrence of symptoms. One patient with dominant cyst morphology who underwent laparoscopic fenestration had recurrence at 26 and 43 months but this was successfully treated finally with open fenestration. The other patient had diffuse cyst morphology and was treated with fenestration for recurrent cyst infection that recurred 1 month postoperatively. This required subsequent intravenous antibiotics and percutaneous drainage for resolution of symptoms.
Treatment for symptomatic APLD should be based on the morphology of the liver cysts. Fenestration is a safe and acceptable procedure for patients with a dominant cyst pattern where liver size can be reduced after the cysts collapse. A combination of resection-fenestration is suitable for those with a diffuse cyst pattern where grossly affected segments are resected in combination with fenestration to allow for reduction in liver size.
大多数成年多囊肝病（APLD）患者无症状。对于有症状的患者，已提出多种治疗方法，但这些方法缺乏关于安全性和长期有效性的长期研究验证。手术方式的选择与APLD的严重程度及肝脏内囊肿的形态有关。本研究的目的是分析新加坡总医院开窗术及联合切除术-开窗术的近期和长期结果。
对1992年1月至2000年12月期间接受手术的12例有症状的APLD患者（10例女性和2例男性）的临床、手术、影像学及随访数据进行回顾性分析。评估的主要结局指标包括术后症状缓解情况、功能状态、并发症、死亡率及症状长期复发情况。
9例患者接受了12次开窗手术，3例患者接受了联合切除术-开窗术。9例囊肿形态以单个为主的患者中8例行开窗术，3例囊肿形态为弥漫性的患者行联合切除术-开窗术。无手术死亡病例，所有患者出院时术前症状均消失。总体发病率为58%。本队列的平均随访时间为29.3个月。仅2例患者症状复发。1例囊肿形态以单个为主的患者接受腹腔镜开窗术后，分别于26个月和43个月复发，但最终通过开放开窗术成功治疗。另1例患者囊肿形态为弥漫性，因复发性囊肿感染行开窗术，术后1个月复发。这需要随后静脉使用抗生素及经皮引流以缓解症状。
有症状的APLD的治疗应基于肝脏囊肿的形态。对于囊肿形态以单个为主且囊肿塌陷后肝脏大小可减小的患者，开窗术是一种安全且可接受的手术。联合切除术-开窗术适用于囊肿形态为弥漫性的患者，即切除严重受累节段并结合开窗术以减小肝脏大小。
