Marret Jean-Baptiste, Blanc Thomas, Balaton Andre, La Vignera Sandro, Zanghì Guido, Lottmann Henri Bernard, Bagnara Vincenzo
Department of Paediatric Surgery and Urology, Hôpital Necker Enfants Malades, APHP, Université de Paris, 149 Rue de Sèvres, 75015 Paris, France.
Mechanisms and Therapeutic Strategies of Chronic Kidney Disease, INSERM U1151-CNRS UMR 8253, Institut Necker Enfants Malades, Département "Croissance et Signalisation", Hôpital Necker Enfants Malades, Université de Paris, 149 Rue de Sèvres, 75015 Paris, France.
J Clin Med. 2022 Apr 5;11(7):2035. doi: 10.3390/jcm11072035.
Symptomatic parapelvic cysts (PPC) are rare entities. Our objective is to highlight specific features of PPC to avoid a misdiagnosis of UPJ obstruction.
We retrospectively reviewed the records of children managed between 2012-2017.
All four patients (18 months-8 years) presented with acute renal colic with a large intra-sinusal liquid mass (42-85 mm) on ultrasound, evoking a diagnosis of UPJ obstruction. On preoperative renal scintigraphy ( = 3) there was no dilatation of the renal pelvis and ipsilateral differential function was impaired in 2. Diagnosis of PPC was suspected preoperatively in three children (CT scan ( = 1); MRI ( = 2)) and made peri-operatively ( = 1). Preoperative retrograde pyelography ( = 3) and a further intraoperative retrograde pyelography with methylene blue ( = 1) did not identify communication with the cyst. No renal pelvis was identified in two patients. De-roofing of the cyst was curative in all cases at 5 years mean follow-up (no leakage, cyst recurrence or loss of function) and all 4 patients became asymptomatic after surgery. Histology demonstrated a single flat epithelial cell layer. Renal function normalized in one patient but remained impaired in the other.
In case of symptoms of UPJ obstruction with a medial renal liquid mass on ultrasound, PPC should be considered when no dilatated pelvis on renal scan is identified. In such cases, a complementary imaging work-up is mandatory prior to surgery.
有症状的肾盂旁囊肿（PPC）是罕见的疾病。我们的目的是强调PPC的特定特征，以避免误诊为肾盂输尿管连接部梗阻（UPJ梗阻）。
我们回顾性分析了2012年至2017年间接受治疗的儿童的病历。
所有4例患者（年龄18个月至8岁）均表现为急性肾绞痛，超声检查显示肾窦内有巨大液性肿块（42 - 85毫米），提示诊断为UPJ梗阻。在术前肾闪烁扫描（n = 3）中，肾盂无扩张，2例患侧肾功能受损。3例儿童术前怀疑为PPC（CT扫描（n = 1）；MRI（n = 2）），1例在术中确诊。术前逆行肾盂造影（n = 3）及1例术中用亚甲蓝进行的逆行肾盂造影均未发现囊肿与外界相通。2例患者未发现肾盂。囊肿去顶术在平均5年的随访中对所有病例均有效（无漏尿、囊肿复发或功能丧失），所有4例患者术后均无症状。组织学显示为单层扁平上皮细胞。1例患者肾功能恢复正常，另1例仍受损。
对于超声显示肾内侧有液性肿块且有UPJ梗阻症状的患者，若肾扫描未发现肾盂扩张，则应考虑PPC。在这种情况下，术前必须进行补充影像学检查。
