WordNet

a special situation; "this thing has got to end"; "it is a remarkable thing"
a separate and self-contained entity
an action; "how could you do such a thing?"
an artifact; "how does this thing work?"
an entity that is not named specifically; "I couldnt tell what the thing was"
any attribute or quality considered as having its own existence; "the thing I like about her is ..."
a persistent illogical feeling of desire or aversion; "he has a thing about seafood"; "she has a thing about him"
a special abstraction; "a thing of the spirit"; "things of the heart"
a special objective; "the thing is to stay in bounds"
a statement regarded as an object; "to say the same thing in other terms"; "how can you say such a thing?"
an event; "a funny thing happened on the way to the..."
a bag that fills with air
a distensible membranous sac (usually containing liquid or gas) (同)vesica

PrepTutorEJDIC

〈C〉(形のある)『物』,物体;(生物に対しての)『無生物』 / 〈C〉(形のない)『もの』,事,事柄,でき事 / 《複数形で》事情,事態,状勢,状況 / 《複数形で》(すべての)事物,事柄 / 《通例 the~》(努力・活動などの)目的,目標 / 〈C〉衣服,衣類・《one's things》持ち物,所持品,身の回り品 / 《複数形で》道具,用具・〈C〉《親愛・哀れみ・軽べつなどの感情をこめて》人,者,やつ / 《the~》《話》(…の)流行《+in+名》
《所有・所属》…『の』,…のものである,…に属する・《材料・要素》…『でできた』,から成る・《部分》…『の』[『中の』] ・《数量・単位・種類を表す名詞に付いて》…の・《原因・動機》…『で』,のために(because of) ・《主格関係》…『の』,による,によって・《目的格関係》…『を』,の・《同格関係》…『という』・《関係・関連》…『についての』[『の』],の点で・《抽象名詞などと共に》…の[性質をもつ] ・《『It is』+『形』+『of』+『名』+『to』 doの形で,ofの後の名詞を意味上の主語として》・《分離》…『から』・《起原・出所》…『から』[『の』](out of) ・《『名』+『of』+『a』(『an』)+『名』の形で》…のような・《『名』+『of』+『mine』(『yours, his』など独立所有格)の形で》…の…・《時》(1)《副詞句を作って》…に《形容詞句を作って》…の・《時刻》《米》…前(to,《米》before)
《前述の名詞または文脈でそれと分かる名詞に付けて》『その』,あの,名の / 《唯一の事物に付けて》 / 《方角・自然現象に付けて》 / 《用具・楽器・特定の病気などに付けて》 / 《修飾吾[句]を伴う名詞に付けて》 / 《単数普通名詞に付けて》(1)《種族全体》…『というもの』 / 《複数名詞または集合名詞に付けて》 / 《単位》『…につき;…単位[で]』 / 《身体の一部に付けて,所有格に代わる》 / 《形容詞に付けて》 / 《固有名詞に付けて》 / 《強意用法に》『随一の』,典型的な / 《「the+比較級,the+比較級」の形で》『…すればするほどそれだけ』 / 《比較級の前に》(…につれて,のために)『それだけ』,ますます
(人間・動物の)襄(のう),ぼうこう,(魚の)浮き袋;気胞　嚢に似た物
OLD French古[代]フランス語

Wikipedia preview

出典(authority):フリー百科事典『ウィキペディア（Wikipedia）』「2015/07/08 16:57:38」(JST)

wiki en

Bladder exstrophy (also known as Ectopia vesicae) is a congenital anomaly that exists along the spectrum of the exstrophy-epispadias complex and most notably involves protrusion of the urinary bladder through a defect in the abdominal wall. Its presentation is variable, often including abnormalities of the bony pelvis, pelvic floor, and genitalia. The underlying embryologic mechanism leading to bladder exstrophy is unknown, though it is thought to be in part due to failed reinforcement of the cloacal membrane by underlying mesoderm.^[1]

Incidence

Occurring at a rate between 1 in 10,000 to 1 in 50,000 ^[2] with a male-to-female ratio of 2.3-6:1,^[3]^[4]^[5] bladder exstrophy is relatively rare. For those individuals with bladder exstrophy who maintain their ability to reproduce, the risk of bladder exstrophy in their children is approximately 500-fold greater than the general population.^[3]

Presentation

The classic manifestation of bladder exstrophy presents with:

A defect in the abdominal wall occupied by both the exstrophied bladder as well as a portion of the urethra
A flattened puborectal sling
Separation of the pubic symphysis
Shortening of the pubic ramii
External rotation of the pelvis.

Females frequently have a displaced and narrowed vaginal orifice, a bifid clitoris, and divergent labia.^[6]

Prenatal diagnosis

In a small retrospective study of 25 pregnancies five factors were found to be strongly associated with a prenatal diagnosis of bladder exstrophy:

Inability to visualize the bladder on ultrasound
A lower abdominal bulge
A small penis with anteriorly displaced scrotum
A low set umbilical insertion
Abnormal widening of the iliac crests

While a diagnosis of bladder exstrophy was made retrospectively in a majority of pregnancies, in only three cases was a prenatal diagnosis made.^[7]

Management at birth

Upon delivery, the exposed bladder is irrigated and a non-adherent film is placed to prevent as much contact with the external environment as possible. In the event the child was not born at a medical center with an appropriate exstrophy support team then transfer will likely follow. Upon transfer, or for those infants born at a medical center able to care for bladder exstrophy, imaging may take place in the first few hours of life prior to the child undergoing surgery.^[6]

Primary (immediate) closure is indicated only in those patients with a bladder of appropriate size, elasticity, and contractility as those patients are most likely to develop a bladder of adequate capacity after early surgical intervention.^[8]

Conditions that are absolute contraindications despite bladder adequacy include duplication of the penis or scrotum and significant bilateral hydronephrosis.

Treatment

Modern therapy is aimed at surgical reconstruction of the bladder and genitalia. Throughout time numerous approaches have been developed, but today only two remain with any frequency:

In the Modern Staged Repair of Exstrophy (MSRE) the initial step is closure of the abdominal wall, often requiring a pelvic osteotomy. This leaves the patient with penile epispadias and urinary incontinence. At approximately 6 months of age the patient then undergoes repair of the epispadias after testosterone stimulation. Finally, bladder neck repair usually occurs around the age of 4–5 years, though this is dependent upon a bladder with adequate capacity and, most importantly, an indication that the child is interested in becoming continent.

In the Complete Primary Repair of Exstrophy (CPRE) the bladder closure is combined with an epispadias repair, in an effort to decrease costs and morbidity.^[9] This technique has, however, led to significant loss of penile and corporal tissue, particularly in younger patients.^[10]

Prognosis

The most important criterion for improving long-term prognosis is success of the initial closure.^[11]^[12] If a patient requires more than one closure their chance of continence drops off precipitously with each additional closure - at just two closures the chance of voiding continence is just 17%.^[13]

Complications

Even with successful surgery, patients may have long-term complications.^[14] Some of the most common include:

Vesicoureteral reflux
Bladder spasm
Bladder calculus
Urinary tract infections

References

^ Muecke EC: The role of the cloacal membrane in exstrophy: the first successful experimental study. J Urol 1964; 92:659.
^ Lattimer JK, Smith MJK: Exstrophy closure: a follow up on 70 cases. J Urol 1966; 95:356.
^ ^a ^b Shapiro E, Jeffs RD, Gearhart JP, Lepor H: Muscarinic cholinergic receptors in bladder exstrophy: insights into surgical management. J Urol 1985; 134:309.
^ Ives E, Coffey R, Carter CO: A family study of bladder exstrophy. J Med Genet 1980; 17:139.
^ Lancaster PAL: Epidemiology of bladder exstrophy: a communication from the International Clearinghouse for Birth Defects monitoring systems. Teratology 1987; 36:221.
^ ^a ^b Gearhart JP, Mathews R. Exstrophy-epispadias complex. In: Wein AJ, ed. Campbell-Walsh Urology. 10th ed. Philadelphia, Pa: Saunders Elsevier; 2011:chap 124.
^ Gearhart JP, Ben-Chaim J, Jeffs RD, et al: Criteria for the prenatal diagnosis of classic bladder exstrophy. Obstet Gynecol 1995; 85:961.
^ Gearhart JP, Jeffs RD: The bladder exstrophy-epispadias complex. In: Walsh PC, et al ed. Campbell's urology, 7th ed. Philadelphia: WB Saunders; 1998:1939.
^ Grady, Mitchell, 1999. Grady R, Mitchell ME: Complete repair of exstrophy. J Urol 1999; 162:1415.
^ Husmann, Gearhart, 2004. Husmann DA, Gearhart JP: Loss of penile glans and/or corpora following primary repair of bladder exstrophy using the complete penile disassembly technique. J Urol 2004; 172:1696.
^ The importance of a successful initial bladder closure in the surgical management of classical bladder exstrophy: analysis of 144 patients treated at the Johns Hopkins Hospital between 1975 and 1985. 1987 Feb;137(2):258–62.
^ Closure of the exstrophic bladder: an evaluation of the factors leading to its success and its importance on urinary continence. 1989 Aug;142(2 Pt 2):522–4–discussion542–3.
^ Failed exstrophy closure: management and outcome. 2010 Aug;6(4):381–4.
^ Gargollo PC, Borer JG (2007). "Contemporary outcomes in bladder exstrophy". Current Opinion in Urology 17 (4): 272–80. doi:10.1097/MOU.0b013e3281ddb32f. PMID 17558272.

External links

Association for the Bladder Exstrophy Community

UpToDate Contents

全文を閲覧するには購読必要です。 To read the full text you will need to subscribe.

1. 膀胱外反症を有する乳児の臨床症状および初期マネージメント clinical manifestations and initial management of infants with bladder exstrophy
2. 膀胱外反症を有する小児の外科的マネージメントおよび術後経過 surgical management and postoperative outcome of children with bladder exstrophy
3. 筋層非浸潤性膀胱癌の再発癌あるいは残存癌のマネージメント management of recurrent or persistent non muscle invasive bladder cancer
4. 胎児腹壁破裂および総排泄腔外反症 body stalk anomaly and cloacal exstrophy
5. 非尿路上皮膀胱癌 non urothelial bladder cancer

English Journal

[The German research network CURE-Net : A benefit for patients with rare diseases].

Ebert AK1.
Der Urologe. Ausg. A.Urologe A.2017 May 22. doi: 10.1007/s00120-017-0403-7. [Epub ahead of print]
PMID 28534165

In Vivo Demonstration of PSMA Expression in Adenocarcinoma Urinary Bladder Using 68Ga-PSMA 11 PET/CT.

Roy SG1, Parida GK, Tripathy S, Singhal A, Tripathi M, Bal C.
Clinical nuclear medicine.Clin Nucl Med.2017 May 6. doi: 10.1097/RLU.0000000000001683. [Epub ahead of print]
PMID 28481793

Quality of Life Amongst Women in Bangladesh Following Ileal Conduit Urinary Diversion Operations for Irreparable Vesicovaginal Fistula and Bladder Exstrophy; Observational Study.

Walker SH1, Ambauen-Berger B2, Saha SL3, Akhter S4.
BJOG : an international journal of obstetrics and gynaecology.BJOG.2017 May 3. doi: 10.1111/1471-0528.14721. [Epub ahead of print]
PMID 28467691

Japanese Journal

陰茎欠損・短腸症を伴った外反のない膀胱腸裂の1例

佐藤かおり,東本恭幸,金田英秀,古屋武史,岩井潤
日本小児外科学会雑誌 45(2), 211-214, 2009-04-20
症例は日齢0の男児.在胎32週3日,胎児心音の低下を認め,緊急帝王切開にて出生.外性器異常・鎖肛を認め,当院搬送入院となった.尿膜管より排尿・排便を認めたが,臍帯ヘルニアなどの合併はなく,下腹部は正常皮膚で覆われていた.陰茎欠損と二分陰嚢を認めるも,精巣は陰嚢内に触知可能であった.尿膜管からの造影では膀胱と腸管が描出され外反のない膀胱腸裂と診断し,緊急手術を施行した.開腹所見では,膀胱後壁が回腸に …
NAID 110007161626

Related Pictures

Embryology of the GU system at Temple University School of Medicine - StudyBlue Exstrophy Bladder single stage repair - YouTube huis muur: Bladder exstrophy repair bladder exstrophy - YouTube Bladder exstrophy repair. Causes, symptoms, treatment Bladder exstrophy repair PPT - Bladder Exstrophy PowerPoint Presentation - ID:7932163