今日の臨床サポート 今日の臨床サポート

著者: 野口満 佐賀大学医学部泌尿器科講座

監修: 中川昌之 公益財団法人 慈愛会 今村総合病院 泌尿器科顧問

著者校正/監修レビュー済:2024/05/29
患者向け説明資料

改訂のポイント:
  1. 定期レビューを行った。
  1. 本疾患の外科的治療は、単一尿管か重複腎盂尿管で分類し、さらに症状の有無、膀胱尿管逆流、所属腎機能を踏まえて術式選択を行い、両疾患の治療方針は多く共通する。
  1. 有熱性尿路感染症では、グラム染色等で起炎菌を推定し、それに対応した抗菌薬加療を計2週間程度行う。

概要・推奨   

  1. 本疾患は、有熱性の尿路感染、尿失禁、腎尿路の異常が契機で診断される(推奨度2)
  1. 本疾患は、排尿時膀胱造影、膀胱鏡検査、腎シンチグラフィーは必須の検査である(推奨度1)
  1. グラム染色の結果でグラム陽性球菌なら腸球菌を想定しペニシリン系(アンピシリン)、グラム陰性桿菌で大腸菌想定であれば第1~3世代セフェム系、緑膿菌想定であればセフタジジムを投与します(推奨度1)
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病態・疫学・診察 

疾患情報(疫学・病態)  
  1. 先天性の膀胱尿管接合部異常で、胎生期の尿管芽および中腎管の分化・消退過程での異常による。
  1. 発症頻度には性差があり、両疾患とも女児に多く男児の3~7倍である。
  1. 尿管瘤では、左側が60%で右側より頻度が高い。
  1. 両疾患とも単一腎盂尿管で発生するもの、重複腎盂尿管で発生するものがある。
  1. 原発性膀胱尿管逆流症や腎盂尿管移行部狭窄症と違い、自然軽快・治癒は望めない。
  1. 尿管瘤では、完全重複尿管の症例(疾患の所属尿管は上半腎由来)であることが70~80%と多く、異所性尿管では、単一腎盂尿管が多い。
 
完全重複尿管での尿管瘤

左腎は完全重複腎盂尿管であり、上半腎由来の尿管が膀胱内尿管瘤を呈している。

 
  1. 異所性尿管では発症側に左右差はなく、10%程度は両側性である。
  1. 異所性尿管は膀胱頚部や尿道への尿路への異所開口と、精路、腟、腸管になど尿路外開口がある。
 
尿管異所開口の部位

尿路のほか、腸管、腟、子宮、精路に尿管が異所開口することがある。
a:女性
b:男性

 
  1. 尿管瘤は尿管下端が膀胱粘膜と排尿筋との間で嚢状に拡張したものである。
  1. 尿管瘤は瘤が膀胱内に限局した膀胱内尿管瘤(intravesical type)と膀胱頚部・尿道へ伸展した異所性尿管瘤(extravesical type)に分類される。
 
尿管瘤の分類

瘤が膀胱内に限局した膀胱内尿管瘤(a:intravesical type)と膀胱頚部・尿道へ進展した異所性尿管瘤(b:extravesical type)がある。さらに、巨大尿管の膀胱壁外からの膀胱内への圧迫突出(c:偽性尿管瘤)は鑑別として重要である。
a:膀胱内尿管瘤
b:異所性尿管瘤
c:偽性尿管瘤

出典

著者提供
問診・診察のポイント  
  1. 尿路感染症による発熱、下腹部痛・下腹部腫瘤、排尿障害・尿失禁などが症状として挙げられる[1]
  1. 女児の異所性尿管では腟に開口することが多く、この場合dry timeのない持続した尿失禁を認めることから外陰部の観察で本疾患が想定される。

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文献 

Stacy T Tanaka, John W Brock
Pediatric urologic conditions, including urinary infections.
Med Clin North Am. 2011 Jan;95(1):1-13. doi: 10.1016/j.mcna.2010.08.018.
Abstract/Text Genitourinary complaints are common in children, and the busy primary care provider must determine initial treatment and assess need for specialty referral. Many complaints are self-limited, but some represent disorders that can threaten organ function. In this article, an initial approach in the primary care office and a guide to specialty referral for pediatric urologic conditions of the urinary tract, male genitalia, and female genitalia are suggested.

Copyright © 2011 Elsevier Inc. All rights reserved.
PMID 21095407
G G Mackie, F D Stephens
Duplex kidneys: a correlation of renal dysplasia with position of the ureteral orifice.
J Urol. 1975 Aug;114(2):274-80.
Abstract/Text A study was made of 51 duplex kidneys, ureters and bladders to assess the types of abnormalities seen in duplex kidneys. The abnormalities affected ectopic and orthotopic segments equally. The occurrence of renal abnormality was closely correlated to the abnormal locations of the segment's ureteral orifice. When the orifice was displaced either cranially (orifice positions B, C and D) or caudally (orifices G and H) the orthotopic and ectopic segments, respectively, showed more severe hypoplasia and dysplasia. Faulty embryogenesis is proposed to explain these findings and the importance of endoscopic positioning of the ureteral orifice is emphasized in determining whether heminephrectomy will be necessary.

PMID 1171997
Katherine C Hubert, Jeffrey S Palmer
Current diagnosis and management of fetal genitourinary abnormalities.
Urol Clin North Am. 2007 Feb;34(1):89-101. doi: 10.1016/j.ucl.2006.10.002.
Abstract/Text Prenatal assessment with ultrasonography provides excellent imaging of fluid-filled structures (eg, hydronephrosis, renal cysts, and dilated bladder) and renal parenchyma. This information allows for the generation of a differential diagnosis, identification of associated anomalies, and assessment of the prenatal and postnatal risks of a given anomaly. This enhances parental education and prenatal and postnatal planning. This article discusses the current methods of diagnosis and management of fetal genitourinary anomalies, and also the postnatal evaluation and treatment of these conditions.

PMID 17145364
Seyedmehdi Payabvash, Abdol-Mohammad Kajbafzadeh, Parisa Saeedi, Zhina Sadeghi, Azadeh Elmi, Mehrzad Mehdizadeh
Application of magnetic resonance urography in diagnosis of congenital urogenital anomalies in children.
Pediatr Surg Int. 2008 Sep;24(9):979-86. doi: 10.1007/s00383-008-2196-7. Epub 2008 Jul 31.
Abstract/Text Magnetic resonance urography (MRU) has become a useful adjuvant in evaluating urogenital anomalies. In present study, we evaluated the ability of MRU in diagnosis of different congenital urogenital anomalies when the results of conventional imaging modalities were inconclusive. A total of 90 children were included in this series. The children were evaluated with T2-weighted and contrast-enhanced T1-weighted MRU sequences. The results were compared with findings obtained with ultrasonography, intravenous urography, renal nuclide scan, and voiding cystourethrography. MRU was requested in these children because conventional imaging modalities were equivocal or a co-existing urogenital anomaly was suspected. Only those cases that underwent surgery were included in this study and the surgical findings were set as the reference standard in statistical evaluation. The records of 61 boys with mean (range) age of 2.3 years (2 months-12 years) and 29 girls with mean (range) age of 3.3 years (3 months-12 years) were reviewed. The final diagnosis was ureteropelvic junction obstruction (n = 25), vesicoureteral junction obstruction (n = 16), ureterocele (n = 19), ectopic kidney (n = 11), posterior urethral valve (n = 17), and polycystic kidney (n = 2). The overall sensitivity of MRU, intravenous urography, renal nuclide scan, ultrasonography, and voiding cystourethrography in diagnosis of the aforementioned anomalies were 86, 63, 50, 44, and 41%, respectively. MRU was much more sensitive than other imaging modalities in diagnosis of end-ureteral dilation (100%) and ureterocele (89%). MRU provides a reliable noninvasive technique for imaging of the congenital anomalies in the urinary tract of children with T2-weighted MRU sequences providing unenhanced static-water images of the urinary tract as well as depicting adjacent soft-tissue lesions, and T1-weighted MRU technique imitating conventional intravenous urography. Both MRU sequences can be combined for a comprehensive examination of the urinary tract.

PMID 18668256
Ming-Dar Lee, Chun-Chen Lin, Brian Pin-Hsuan Chang, Pei-Yi Lin, Jeng-Daw Tsai
Pseudoureterocele.
Pediatr Neonatol. 2018 Feb;59(1):104-105. doi: 10.1016/j.pedneo.2017.09.002. Epub 2017 Sep 6.
Abstract/Text
PMID 28941655
金子一成:小児の尿路感染症up-to-date. 小児感染免疫。2021. 33巻(1):58-65.
Ottavio Adorisio, Antonio Elia, Luca Landi, Maria Taverna, Valeria Malvasio, Alfredo D Danti
Effectiveness of primary endoscopic incision in treatment of ectopic ureterocele associated with duplex system.
Urology. 2011 Jan;77(1):191-4. doi: 10.1016/j.urology.2010.02.061. Epub 2010 Dec 18.
Abstract/Text OBJECTIVES: To report our experience with the endoscopic treatment of ectopic ureterocele to demonstrate its long-term effectiveness. Endoscopic treatment is often recommended as the initial and definitive treatment in patients with ureterocele.
METHODS: A total of 46 children with ectopic ureterocele in a duplex system underwent primary endoscopic incision from January 1998 to January 2006. The mean follow-up was 3.8 years. Of the 46 children, 35 had been diagnosed prenatally and 11 had been diagnosed after birth because of a urinary tract infection. Low-dose antibiotic prophylaxis was administered to all children and was maintained until voiding cystourethrography showed no reflux. The pre- and postoperative evaluation included clinical assessment, ultrasound evaluation, diethylenetriaminepentacetic acid renography, and cyclic voiding cystourethrography.
RESULTS: Ureterocele decompression was achieved in 43 patients (93%). Three patients required additional surgery for persistent ureterocele (1 underwent ureteroureterostomy and 2 ureteropyelostomy). None of our patients showed deterioration of renal function after the procedures. Vesicoureteral reflux was seen in the lower moiety of the ipsilateral kidney in 14 patients (30%). Of the 14 patients with vesicoureteral reflux, 10 had spontaneous resolution. The remaining 4 underwent endoscopic correction. Five patients (10%) developed de novo vesicoureteral reflux in the ipsilateral ureterocele moiety. Of these 5 patients, 3 were treated with endoscopic injection and 2 had spontaneous resolution after 6 months of follow-up.
CONCLUSIONS: Our data have shown that primary endoscopic puncture of a ureterocele is a simple, long-term, effective, and safe procedure, avoiding complete reconstruction in most patients.

Copyright © 2011 Elsevier Inc. All rights reserved.
PMID 21168903
Paolo Caione, Simona Gerocarni Nappo, Giuseppe Collura, Ennio Matarazzo, Maida Bada, Laura Del Prete, Michele Innocenzi, Ermelinda Mele, Nicola Capozza
Minimally Invasive Laser Treatment of Ureterocele.
Front Pediatr. 2019;7:106. doi: 10.3389/fped.2019.00106. Epub 2019 Apr 8.
Abstract/Text Introduction: Ureterocelemay cause severe pyelo-ureteral obstruction with afebrile urinary tract infections in infants and children. Early decompressive treatment is advocated to reduce the risk of related renal and urinary tract damage. Endoscopic techniques of incision have been offered utilizing diathermic electrode. We adopted laser energy to release the obstruction of the ureterocele and reduce the need of further surgery. Our technique is described and results are presented, compared with a group of matched patients treated by diathermic energy. Materials and methods: Decompression was performed by endoscopic multiple punctures at the basis of the ureterocele. Holmium YAG Laser was utilized with 0.5-0.8 joule energy, through 8-9.8F cystoscope under general anesthesia. The control group received ureterocele incision by diathermic energy through pediatric resettoscope. Foley indwelling catheter was removed after 18-24 h. Renal ultrasound was performed at 1, 3, 6, and 12 months follow-up. Voiding cysto-urethrogram and radionuclide renal scan were done at 6-18 months in selected cases. Statistical analysis was utilized for data evaluation. Results: From January 2012 to December 2017, 64 endoscopic procedures were performed: 49 were ectopic and 15 orthotopicureteroceles. Fifty-three were in duplex systems, mostly ectopic. Mean age at endoscopy was 6.3 months (1-168). Immediate decompression of the ureterocele was obtained, but in five cases (8%) a second endoscopic puncture was necessary at 6-18 months follow-up for recurrent dilatation. Urinary tract infections and de novo refluxes occurred in 23.4 and 29.7% in the study group, compared to 38.5 and 61.5% in the 26 controls (p < 0.05). Further surgery was required in 12 patients (18%) at 1-5 years follow-up (10 in ectopic ureteroceles with duplex systems): seven ureteral reimplantation for reflux, five laparoscopic hemy-nephro-ureterectomy. Orthotopic ureteroceceles had better outcome. Secondary surgery was necessary in 13 patients (50.0%) of control group (p < 0.05). Conclusions: Early endoscopic decompression should be considered first line treatment of obstructing ureterocele in infants and children. Multiple punctures at the basis of the ureterocele, performed by low laser energy, is resulted a really minimally invasive treatment, providing immediate decompression of the upper urinary tract, and reducing the risk of further aggressive surgery.

PMID 31024867
R Pearce, R Subramaniam
Partial nephroureterectomy in a duplex system in children: the need for additional bladder procedures.
Pediatr Surg Int. 2011 Dec;27(12):1323-6. doi: 10.1007/s00383-011-2968-3. Epub 2011 Aug 30.
Abstract/Text INTRODUCTION AND OBJECTIVES: Heminephroureterectomy (HN) is our treatment of choice in a duplex system with non-functioning moiety. We examined the need for endoscopic incision (EI)/bladder reconstructive surgery (BRS) and whether ureteroceles and/or vesicoureteric reflux (VUR) influenced management options.
METHODS: Retrospective study of patients undergoing HN by a single surgeon (2003-2008). Patients were classified according to the presence (Group 1) or absence (Group 2) of ureterocele. The groups were subdivided with coexisting dilating VUR (a) or not (b). Statistical analysis included Fisher's exact test.
RESULTS: Thirty-one children were identified. Seventeen (54.8%) had ureterocele (Group 1) and 14 patients had no ureterocele (Group 2). Group 1 had eight with VUR (1a) and nine without (1b). Group 2 had seven with VUR (2a) and seven without (2b). Significantly more patients with ureterocele required EI/BRS (p = 0.006). Five (29%) in Group 1 required BRS versus none in Group 2 (p = 0.04). Six (75%) in Group 1a underwent EI/BRS versus three (33%) in Group 1b (p = 0.15). Significantly more in Group 1a required EI prior to HN versus Group 1b (p = 0.04). Similar numbers of patients required BRS in Groups 1a and 1b (p = 0.61).
CONCLUSIONS: In the absence of ureterocele, there is minimal likelihood of requiring surgery apart from HN, independent of VUR. Presence of ureterocele is an indicator for additional procedure within the bladder. There is a higher incidence of EI when ureterocele co-exists with dilating VUR.

PMID 21877243
Wein: Campbell-Walsh Urology, 12th. Ed.
薬剤監修について:
オーダー内の薬剤用量は日本医科大学付属病院 薬剤部 部長 伊勢雄也 以下、渡邉裕次、井ノ口岳洋、梅田将光および日本医科大学多摩永山病院 副薬剤部長 林太祐による疑義照会のプロセスを実施、疑義照会の対象については著者の方による再確認を実施しております。
※薬剤中分類、用法、同効薬、診療報酬は、エルゼビアが独自に作成した薬剤情報であり、 著者により作成された情報ではありません。
尚、用法は添付文書より、同効薬は、薬剤師監修のもとで作成しております。
※同効薬・小児・妊娠および授乳中の注意事項等は、海外の情報も掲載しており、日本の医療事情に適応しない場合があります。
※薬剤情報の(適外/適内/⽤量内/⽤量外/㊜)等の表記は、エルゼビアジャパン編集部によって記載日時にレセプトチェックソフトなどで確認し作成しております。ただし、これらの記載は、実際の保険適応の査定において保険適応及び保険適応外と判断されることを保証するものではありません。また、検査薬、輸液、血液製剤、全身麻酔薬、抗癌剤等の薬剤は保険適応の記載の一部を割愛させていただいています。
(詳細はこちらを参照)
著者のCOI(Conflicts of Interest)開示:
野口満 : 特に申告事項無し[2024年]
監修:中川昌之 : 特に申告事項無し[2024年]

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