Areses Trapote R, Urbieta Garagorri M A, Ubetagoyena Arrieta M, Alzueta Beneite M T, Arruebarrena Lizarraga D, Eizaguirre Sexmilo I, Rodríguez Mazorriaga F, Emparanza Knorr J I
Sección de Nefrología Pediátrica, Instituto Oncológico de Guipúzcoa, Hospital Donostia, San Sebastián, España.
An Pediatr (Barc). 2007 Aug;67(2):123-32. doi: 10.1016/s1695-4033(07)70572-6.
Most primary non-refluxing megaureters resolve spontaneously and the indications for surgery are not sufficiently well established.
To analyze the clinical features, treatment and outcome of asymptomatic primary non-refluxing megaureter.
We retrospectively studied 58 infants with primary non-refluxing megaureter. The diagnostic methods used were renal ultrasound, renal isotopic renogram, DMSA scan, and evaluation of renal function.
The mean age at postnatal diagnosis was 24 days. The mean follow-up was 4 years. Sixty-nine percent of the patients were male. Fifty-seven percent were left megaureters and 22% were bilateral (71 affected renal units). Eleven percent of megaureters were grade I, 48% were grade II, and 41% were grade III. Only nine patients (15%) received surgical treatment. The indications for surgical treatment were severe megaureter (3/9 patients; 33%), prolonged T1/2 (3/9 patients; 33%), reduced function (1/9 patients; 11%), prolonged T1/2 plus reduced function (1/9 patients; 11%) and increased dilation (1/9 patients; 11%). The mean age at surgery was 7 months. Outcomes in the non-surgical group (85% of the patients; 60 renal units) were as follows: 90% of megaureters were corrected or improved on ultrasound scan and 10% showed no change. In the first renogram, function was low in 4/60 kidneys (7%) and T1/2 was prolonged in 3/60 (5%). Finally, all kidneys in the non-surgical group had normal function, except one, which was injured from the beginning. T1/2 was normal in all kidneys. Outcomes in the surgical group (10 megaureters) were as follows: 80% of megaureters were corrected or improved after surgical intervention and 20% showed no change. At diagnosis, 4/10 kidneys (40%) had reduced function. In the post-surgical renogram 2/10 kidneys (20%) continued to show reduced function, 1/10 kidney (10%) showed restored renal function, and 1/10 kidney (10%) was nephrectomized. Initial T1/2 was prolonged in 4/10 patients (40%), and after surgery T1/2 was normal in all patients. The final DMSA scan showed 5/71 kidneys (7%) with irreversible damage (one slightly injured, two moderately injured, and three severely injured). The remaining 66 kidneys were normal. Overall renal function and blood pressure were normal in all patients.
Primary non-refluxing megaureter is usually a functional and benign congenital malformation that resolves during the first months of life. Although the malformation can persist, only a few patients require surgical treatment. Most authors agree that initial treatment should not be surgical and that surgery should be reserved for patients who develop ureteral dilation, a decrease in differential renal function, and/or severe symptoms during follow-up. In a few patients (7% of our series), the renal unit belonging to the megaureter shows irreversible congenital injury. In these patients, surgery is not useful.
大多数原发性无反流巨输尿管会自行缓解,手术指征尚未充分明确。
分析无症状原发性无反流巨输尿管的临床特征、治疗方法及预后。
我们回顾性研究了58例原发性无反流巨输尿管患儿。所采用的诊断方法包括肾脏超声、肾脏同位素肾图、二巯基丁二酸(DMSA)扫描及肾功能评估。
出生后诊断的平均年龄为24天。平均随访时间为4年。69%的患者为男性。57%为左侧巨输尿管,22%为双侧(71个受累肾单位)。11%的巨输尿管为I级,48%为II级,41%为III级。仅9例患者(15%)接受了手术治疗。手术治疗的指征为重度巨输尿管(3/9例患者;33%)、T1/2延长(3/9例患者;33%)、功能降低(1/9例患者;11%)、T1/2延长加功能降低(1/9例患者;11%)及扩张增加(1/9例患者;11%)。手术的平均年龄为7个月。非手术组(85%的患者;60个肾单位)的预后如下:90%的巨输尿管在超声检查时得到纠正或改善,10%无变化。在首次肾图检查中,4/60个肾脏(7%)功能低下,3/60个肾脏(5%)T1/2延长。最后,非手术组除1个一开始就受损的肾脏外,所有肾脏功能均正常。所有肾脏的T1/2均正常。手术组(10个巨输尿管)的预后如下:80%的巨输尿管在手术干预后得到纠正或改善,20%无变化。诊断时,4/10个肾脏(40%)功能降低。术后肾图检查中,2/10个肾脏(20%)继续显示功能降低,1/10个肾脏(10%)肾功能恢复,1/10个肾脏(10%)被切除。4/10例患者(40%)初始T1/2延长,术后所有患者T1/2均正常。最终的DMSA扫描显示71个肾脏中有5个(7%)存在不可逆损伤(1个轻度损伤,2个中度损伤,3个重度损伤)。其余66个肾脏正常。所有患者的总体肾功能和血压均正常。
原发性无反流巨输尿管通常是一种功能性良性先天性畸形,在出生后的头几个月内会自行缓解。尽管这种畸形可能持续存在,但只有少数患者需要手术治疗。大多数作者一致认为,初始治疗不应采用手术,手术应保留给在随访期间出现输尿管扩张、分肾功能降低和/或严重症状的患者。在少数患者(我们系列中的7%)中,属于巨输尿管的肾单位显示出不可逆的先天性损伤。在这些患者中,手术无效。