The coexistence of primary megaureter and reflux

Primary megaureter is a common cause of obstruction of the urinary tract in children. Vesicoureteric reflux is a common lower-tract abnormality. The seemingly paradoxical coexistence of these entities was found to occur nine times over the 10-year period, 1973 to 1983 (in about 2800 children with reflux and about 75 children with primary megaureter). This coexistence should be suspected on voiding cystourethrography when the juxtavesical segment of the megaureter is normal in caliber, the refluxed contrast agent is diluted by urine that is trapped in the ureter, and drainage of refluxed contrast agent into the bladder is impeded. The diagnosis can be confirmed by excretory urography with a catheter draining the bladder to temporarily prevent the reflux. The surgical treatment consists of resection of the distal aperistaltic segment and reimplantation of the ureter.     

先天性巨输尿管症多层螺旋CT诊断

目的:探讨先天性巨输尿管症的多层螺旋CT(MSCT)表现及MSCT对其诊断的价值,提高诊断水平。方法:回顾性分析9例经手术证实的先天性巨输尿管症的CT表现和临床资料。结果:先天性巨输尿管症的MSCT表现为患侧输尿管不同程度的扩张。扩张的输尿管末端可呈"杵状"或"蛇头状"。有3例可观察到输尿管末端正常部分,4例发现肾结石,1例发现输尿管开口位置异常。结论:多层螺旋CT在先天性巨输尿管症及其并发症的诊断、鉴别诊断方面具有重要价值。     

成人先天性巨输尿管症的临床研究

 目的探讨本病的病因、病理、诊断及治疗.方法对10例成人先天性巨输尿管病人的临床资料进行分析总结.结果10例12侧成人先天性巨输尿管,其中左侧6例,右侧2例,双侧2例,8例行输尿管裁剪成形和再植术;2例保守观察定期复查随访,取得较好效果.结论引起成人先天性巨输尿管原因少数是单一的,多数常常与几种因素有关.治疗可根据不同的情况选择不同的治疗方法.     

先天性巨输尿管症的X线与超声诊断(附19例分析)

目的：探讨X线与超声对先天性巨输尿管症的诊断价值。方法：回顾性分析了19例先天性巨输尿管症的静脉肾盂造影(IVP)、尿路逆行造影及超声影像资料。19例先天性巨输尿管症均进行了尿路造影和超声检查。其中3例术前做了CT检查，本组病例均经手术及病理证实。结果：巨输尿管症以其下端管腔无机械性梗阻的相对狭窄和狭窄段以上显著性扩张为特征。成人的扩张多不延及肾盂肾盏，小儿多数病例有肾盂肾盏扩张积水表现。早期扩张的下段可呈“纺锤状”或“杵状”。尿路造影透视和超声实时观察时，可见扩张输尿管蠕动频率减慢、幅度增大、向下传递间断。结论：超声因其具有诸多优点，可作为诊断巨输尿管症的首选的筛查方法。尿路造影检查，正确诊断率高，为本症术前的必查项目。两种检查方法联合应用可优势互补。     

先天性巨输尿管症的影像学表现(附9例分析)

目的：探讨先天性巨输尿管症的影像学表现。方法：运用X线平片、尿路造影，B超对9例先天性巨输尿管症进行了影像学检查，检查采用大剂量静脉尿路造影，造影时早期解除腹压带，增加延迟摄片，并加照膀胱斜位点片，动态观察且与手术病理对照。结果：①单或双侧输尿管扩张始于盆腔段，且呈上行渐进性发展，造成肾盂肾盏扩张，扩张的输尿管可发生迂曲改变，而扩张输尿管末端临近膀胱入口处管径正常，为功能性梗阻段，长度0．5—3cm；②除功能性梗阻外，其余段输尿管的蠕动一般正常；③扩张的输尿管排空延迟；④无膀胱输尿管返流。结论：本病对影像学检查方法的敏感性各不相同，但综合影像学检查可取长补短，相互印证，可提高对先天性巨输尿管症的诊断率。     

Uriniferous perirenal pseudocyst in a neonate with primary obstructive megaureter

The case of a newborn infant with a high-grade ureterovesical junction obstruction--a primary obstructive megaureter--who presented with a perirenal pseudocyst (urinoma) is discussed. Radionuclide studies allowed recognition of this collection and led to the discovery of the ureteral obstruction. The rarity of urinomas in neonates and the scintigraphic appearance of urinomas are emphasized.     

Giant ureteral stone in association with primary megaureter presenting as an acute abdomen.

A 20-year-old woman presented with abdominal pain of 4-h duration and of sudden onset. A plain abdominal radiograph showed a giant ureteral stone measuring 12 cm causing ureteral obstruction. Abdominal ultrasound revealed severe dilatation of the two upper thirds of the left ureter and a hydronephrotic ipsilateral kidney. Subsequent renal scan demonstrated that it was a non-functional kidney while the contralateral kidney was normal. A left nephroureterectomy was performed.     

Primary megaureter in a mother and daughter

Primary megaureter in a mother and daughter is reported. This is the first well-documented familial occurrence of this entity in the English literature.     

The coexistence of ureteropelvic junction obstruction and reflux

Since ureteropelvic junction obstruction is the most common upper urinary tract problem in children, and vesicoureteral reflux the most common lower tract problem, it is not surprising that these entities sometimes coexist in the same child. Over a 10 year period this uncommon phenomenon has been noted 21 times (in about 2,800 children with reflux and 200 children with ureteropelvic junction obstruction). Significant ureteropelvic junction obstruction in association with mild reflux can mimic severe reflux, but the operation needed is not reimplantation but pyeloplasty. Conversely, when significant ureteropelvic junction obstruction coexists with significant reflux, both operations may be necessary, but the order in which they are done (pyeloplasty first) seems to be crucial. Voiding cystography with appropriate postvoid drainage films, excretory urography, often with a catheter draining the bladder to prevent reflux, and provocative diuretic excretory urography and/or renography can determine that ureteropelvic junction obstruction does coexist and quantitate the severity of each problem.     

Pertechnetate scintigraphy in primary congenital hypothyroidism.

Primary congenital hypothyroidism (PCH) is currently detected effectively by heel-stick screening. When elevated thyrotropin (TSH) and/or decreased T4 are found in the blood of neonates, they are recalled, values are confirmed in venous blood and thyroxine replacement therapy (TRT) is immediately instituted, thus cretinism or severe retardation is prevented. However, in a significant percentage of neonates with abnormal blood levels of T4 or TSH, the disorder is transient. To help determine the exact cause of PCH and the possibility of transient PCH, pinhole thyroid imaging is performed 30 min after an intravenous injection of 18.5 MBq (500 microCi) 99mTc-pertechnetate (TcPT). Patients with a nonvisualized gland or patients with images suggesting dyshormonogenesis are reevaluated at age 3-4 y to exclude transient PCH. METHODS: To define the role of TcPT imaging in determining the exact etiology of PCH and the possibility of its being transient, we reviewed data from 103 neonates with PCH who had scintigraphy in our laboratory between 1970 and 1996 and we correlated the results with clinical outcome. RESULTS: Four patterns of thyroid scintigrams were recognized and these determined patient classification: (a) normal in 7 patients with false-positive heel-stick screening but normal venous blood hormone levels; (b) hypoplasia-ectopia in 32 patients requiring lifelong TRT; (c) nonvisualization in 35 patients-32 with agenesis requiring lifelong TRT and 3 with fetal thyroid suppression by maternal antibodies whose TRT was discontinued at a later age; and (d) dyshormonogenesis (markedly increased TcPT concentration) in 29 patients-25 with permanent PCH requiring lifelong TRT and 4 with transient PCH in whom TRT was discontinued. Of the 25 patients with dyshormonogenesis, 12 belonged to five families with two or three siblings having the same disorder. CONCLUSION: TcPT thyroid scintigraphy in the neonate with PCH provides a more specific diagnosis, is useful for selecting patients for re-evaluation to uncover transient PCH and discontinue TRT and defines dyshormonogenesis, which is familial and requires genetic counseling. It is also cost-effective.     

Primary megaureter in infants and children: A review

Primary megaureter is a common cause of obstructive uropathy in children. The imaging studies and records of 75 infants and children with primary megaureter seen at Children’s Hospital were reviewed. We describe our findings and illustrate the clinical presentations, diagnosis, and treatment of this entity.     

The coexistence of obstruction at the ureteropelvic and ureterovesical junctions

Obstructions at the ureteropelvic junction and ureterovesical junction were found to coexist in 14 children seen over an 11-year period. The coexistence of these two conditions made the diagnosis of each difficult. In only three children were both diagnoses made preoperatively; in the others, the second diagnosis was either suspected at surgery or was made on postoperative uroradiologic studies. Of five children in whom the initial diagnosis was obstruction at the ureteropelvic junction, only two have needed surgery for obstruction at the ureterovesical junction. Seven children had an initial diagnosis of obstruction at the ureterovesical junction, and all required subsequent pyeloplasty to relieve coexisting obstruction at the ureteropelvic junction. Therefore, our experience suggests that when both obstructions are known to exist or are suspected preoperatively, it is advisable to repair the stenosis at the ureteropelvic junction first, as distal ureteral surgery may not be necessary.     

Radionuclide imaging in primary permanent congenital hypothyroidism.

Thyroid scintigraphy was performed in 69 consecutive infants with permanent primary congenital hypothyroidism (CH) detected by systematic neonatal thyroid screening using a rectilinear scanner or a gamma camera 15 minutes after the intravenous injection of 250 microCi Tc-99m sodium pertechnetate, immediately after the infants were fed. Immobilization of the infant in a supine position with the neck in hyperextension was achieved by a vacuum cushion without any sedation. Thyroid scintigraphy showed thyroid ectopia in 43 of the 69 CH infants, thyroid agenesis in 19, and goiter in the last 7 infants, respectively. The images were easily interpretable in all cases, even in very small ectopic glands in the lingual area, as there was no interference with extra-thyroidal uptake of the tracer, especially by the salivary glands. Our experience indicates that thyroid scintigraphy in the CH newborns can be performed immediately at the time of diagnosis of CH without technical difficulties. As thyroid scintigraphy is the only procedure allowing the distinction between thyroid agenesis and ectopia, and as the neurointellectual prognosis might be lower in the former than in the latter situation, thyroid scintigraphy in CH newborns contributes not only to the diagnosis but also the prognosis of the disorder.     

Primary megaureter in infants and children: a review.

Primary megaureter is a common cause of obstructive uropathy in children. The imaging studies and records of 75 infants and children with primary megaureter seen at Children's Hospital were reviewed. We describe our findings and illustrate the clinical presentations, diagnosis, and treatment of this entity.     

超声诊断左侧巨输尿管1例

患者女,77岁。反复阵发性左侧腰痛1年余,加重1月,体温38.2℃,尿频7天。B超:左肾实质变薄,左肾重度积水,左侧输尿管全程不规则扩张,最宽处约为3.4cm,内透声差,走行迂回扭曲,膀胱壁间段明显变窄,约为0.4cm,内未见明显异常结石及其他囊实性包块回声(图1,2);动态观察可见蠕动;静脉尿路造影:左侧肾盂肾盏明显扩张,输尿管全程扩张,膀胱壁间段呈鸟嘴状狭窄。超声诊断:考虑左侧巨输尿管。