Boybeyi Ozlem, Karnak Ibrahim, Orhan Diclehan, Ciftci Arbay Ozden, Tanyel F Cahit, Kale Gülsev, Senocak Mehmet Emin
Department of Pediatric Surgery, Faculty of Medicine, Hacettepe University, Ankara, Turkey.
J Pediatr Surg. 2008 Nov;43(11):1985-9. doi: 10.1016/j.jpedsurg.2008.04.006.
Cystic nephroma (CN) and localized renal cystic disease (LRCD) are rare benign renal entities that can occur in children. A retrospective evaluation of the experience with these conditions was performed to emphasize the importance of differential diagnosis, recognition, and selection of the proper mode of treatment.
The medical records of patients treated for CN (n = 4) and LRCD (n = 1) between 1980 and 2007 were retrospectively evaluated. The age, sex, family history of renal cystic disease, presenting symptoms, physical examination findings, diagnostic radiologic imaging used, operative findings, details of the surgical procedure, histopathologic features, and outcomes were evaluated.
There were 4 cases of CN and 1 case of LRCD without a positive family history. The age and sex of patients with CN were two 1-year-old girls and two 1.5-year-old boys. The patient with LRCD was a 4.5-year-old girl. The presenting symptoms were palpable mass in patients with CN and urinary tract infection in the patient with LRCD without impairment of renal function. Intravenous pyelography (n = 2), abdominal ultrasonography (n = 4), and computed tomography (n = 4) were used. All patients underwent an operation. Nephrectomy was performed in all 4 CN cases, and total excision of the cyst was performed in the LRCD case.
Pediatric surgeons should be familiar with the clinical, radiologic, surgical, and histopathologic characteristics of CN and LRCD. The presumptive diagnosis of CN prompts surgical intervention. Enucleation or partial nephrectomy is the treatment of choice in suitable cases. Nephrectomy should be reserved for CN, which totally replaces the parenchyma. Conservative treatment with follow-up imaging may be considered in selected cases of LRCD that have characteristic computed tomographic criteria. Operative intervention is indicated in symptomatic cases and should be limited to simple excision of the cysts.
囊性肾瘤（CN）和局限性肾囊性疾病（LRCD）是罕见的儿童期良性肾脏病变。对这些疾病的诊疗经验进行回顾性评估，以强调鉴别诊断、识别及选择合适治疗方式的重要性。
回顾性评估1980年至2007年间接受治疗的CN患者（n = 4）和LRCD患者（n = 1）的病历。评估患者的年龄、性别、肾囊性疾病家族史、临床表现、体格检查结果、诊断性放射影像学检查、手术所见、手术过程细节、组织病理学特征及治疗结果。
有4例CN和1例LRCD，均无阳性家族史。CN患者中，年龄和性别分别为两名1岁女童和两名1.5岁男童。LRCD患者为一名4.5岁女童。CN患者的临床表现为可触及肿块，LRCD患者为尿路感染，且肾功能未受损。使用了静脉肾盂造影（n = 2）、腹部超声（n = 4）和计算机断层扫描（n = 4）。所有患者均接受了手术。4例CN患者均行肾切除术，LRCD患者行囊肿全切术。
小儿外科医生应熟悉CN和LRCD的临床、放射学、手术及组织病理学特征。CN的初步诊断提示需进行手术干预。在合适的病例中，剜除术或部分肾切除术是首选治疗方法。对于完全替代肾实质的CN，应保留肾切除术。对于具有特征性计算机断层扫描标准的LRCD特定病例，可考虑采用随访影像学检查的保守治疗。有症状的病例应进行手术干预，且应限于囊肿的简单切除。
