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Exstrophy

MedGen UID:
488777
•Concept ID:
C0015338
•
Congenital Abnormality
SNOMED CT: Exstrophy (110407002)
 
HPO: HP:0100548

Definition

Eversion of a hollow organ and exposure, inside out, and protruded through the abdominal wall. [from HPO]

Professional guidelines

PubMed

Genetic Counseling for Bladder Exstrophy-Epispadias Complex.
Reutter H, Holmdahl G
Eur J Pediatr Surg 2021 Dec;31(6):468-471. Epub 2021 Dec 15 doi: 10.1055/s-0041-1740336. PMID: 34911128
Postoperative Immobilization and Pain Management After Repair of Bladder Exstrophy.
Roth E, Goetz J, Kryger J, Groth T
Curr Urol Rep 2017 Mar;18(3):19. doi: 10.1007/s11934-017-0667-x. PMID: 28233224
Diagnosis and management of epispadias.
Frimberger D
Semin Pediatr Surg 2011 May;20(2):85-90. doi: 10.1053/j.sempedsurg.2011.01.003. PMID: 21453851

Recent clinical studies

Etiology

Genetic Counseling for Bladder Exstrophy-Epispadias Complex.
Reutter H, Holmdahl G
Eur J Pediatr Surg 2021 Dec;31(6):468-471. Epub 2021 Dec 15 doi: 10.1055/s-0041-1740336. PMID: 34911128
Anatomy of Classic Bladder Exstrophy: MRI Findings and Surgical Correlation.
Dunn EA, Kasprenski M, Facciola J, Benz K, Maruf M, Zaman MH, Gearhart J, Di Carlo H, Tekes A
Curr Urol Rep 2019 Jul 8;20(9):48. doi: 10.1007/s11934-019-0916-2. PMID: 31286274
Pelvic Organ Prolapse and Pregnancy in the Female Bladder Exstrophy Patient.
Kaufman MR
Curr Urol Rep 2018 Feb 26;19(3):18. doi: 10.1007/s11934-018-0767-2. PMID: 29479649
2D and 3D MRI features of classic bladder exstrophy.
Tekes A, Ertan G, Solaiyappan M, Stec AA, Sponseller PD, Huisman TA, Gearhart JP
Clin Radiol 2014 May;69(5):e223-9. Epub 2014 Feb 26 doi: 10.1016/j.crad.2013.12.019. PMID: 24581971
The exstrophy-epispadias complex.
Eeg KR, Khoury AE
Curr Urol Rep 2008 Mar;9(2):158-64. doi: 10.1007/s11934-008-0028-x. PMID: 18420001

Diagnosis

Prenatal Diagnosis of Bladder Exstrophy and OEIS over 20 Years.
Lee T, Weiss D, Roth E, Bortnick E, Jarosz S, Eftekharzadeh S, Groth T, Shukla A, Kryger JV, Lee RS, Canning DA, Mitchell ME, Borer JG
Urology 2023 Feb;172:174-177. Epub 2022 Nov 30 doi: 10.1016/j.urology.2022.11.020. PMID: 36460061
Fetal abdominal wall defects.
Prefumo F, Izzi C
Best Pract Res Clin Obstet Gynaecol 2014 Apr;28(3):391-402. Epub 2013 Dec 3 doi: 10.1016/j.bpobgyn.2013.10.003. PMID: 24342556
Spectrum of cloacal exstrophy.
Phillips TM
Semin Pediatr Surg 2011 May;20(2):113-8. doi: 10.1053/j.sempedsurg.2010.12.007. PMID: 21453856
The exstrophy-epispadias complex.
Ebert AK, Reutter H, Ludwig M, Rösch WH
Orphanet J Rare Dis 2009 Oct 30;4:23. doi: 10.1186/1750-1172-4-23. PMID: 19878548Free PMC Article
Cloacal exstrophy.
Hyun SJ
Neonatal Netw 2006 Mar-Apr;25(2):101-15. doi: 10.1891/0730-0832.25.2.101. PMID: 16610483

Therapy

Orthopedic complications after osteotomy in patients with classic bladder exstrophy and cloacal exstrophy: a comparative study.
Sholklapper TN, Crigger C, Haney N, Khandge P, Wu W, Sponseller PD, Gearhart JP
J Pediatr Urol 2022 Oct;18(5):586.e1-586.e8. Epub 2022 Sep 15 doi: 10.1016/j.jpurol.2022.09.005. PMID: 36216696
Ureterosigmoidostomy.
Zimmer V, Lammert F
Dig Liver Dis 2019 Nov;51(11):1618. Epub 2019 Sep 30 doi: 10.1016/j.dld.2019.08.026. PMID: 31582325
Penile ischemic injury in the exstrophy/epispadias spectrum: new insights and possible mechanisms.
Cervellione RM, Husmann DA, Bivalacqua TJ, Sponseller PD, Gearhart JP
J Pediatr Urol 2010 Oct;6(5):450-6. Epub 2010 Jun 11 doi: 10.1016/j.jpurol.2010.04.007. PMID: 20541473
Diagnosis and management of urinary incontinence and functional fecal incontinence (encopresis) in children.
Nijman RJ
Gastroenterol Clin North Am 2008 Sep;37(3):731-48, x. doi: 10.1016/j.gtc.2008.06.007. PMID: 18794006
Pelvic and extremity immobilization after bladder exstrophy closure: complications and impact on success.
Meldrum KK, Baird AD, Gearhart JP
Urology 2003 Dec;62(6):1109-13. doi: 10.1016/s0090-4295(03)00791-x. PMID: 14665365

Prognosis

Abdominal wall defects.
Lakshminarayanan B, Lakhoo K
Early Hum Dev 2014 Dec;90(12):917-20. Epub 2014 Oct 14 doi: 10.1016/j.earlhumdev.2014.09.018. PMID: 25448781
Fetal abdominal wall defects.
Prefumo F, Izzi C
Best Pract Res Clin Obstet Gynaecol 2014 Apr;28(3):391-402. Epub 2013 Dec 3 doi: 10.1016/j.bpobgyn.2013.10.003. PMID: 24342556
The exstrophy-epispadias complex.
Ebert AK, Reutter H, Ludwig M, Rösch WH
Orphanet J Rare Dis 2009 Oct 30;4:23. doi: 10.1186/1750-1172-4-23. PMID: 19878548Free PMC Article
Hypospadias and bladder exstrophy.
Stein R, Thüroff JW
Curr Opin Urol 2002 May;12(3):195-200. doi: 10.1097/00042307-200205000-00003. PMID: 11953673
Female epispadias.
Mollard P, Basset T, Mure PY
J Urol 1997 Oct;158(4):1543-6. PMID: 9302170

Clinical prediction guides

Spectrum of fetal limb anomalies.
Thakur S, Chaddha V, Gupta R, Singh C, Dagar S, Shastri A, Tiwari B, Kavitha, Sethia V, Malik M, Jain P, Kapoor A, Kapoor A, Kapoor T, Kapoor A, Kapoor R, Kumar M, Uppal R
J Clin Ultrasound 2023 Jan;51(1):96-106. Epub 2022 Aug 10 doi: 10.1002/jcu.23273. PMID: 36639848
Epispadias and the associated embryopathies: genetic and developmental basis.
Suzuki K, Matsumaru D, Matsushita S, Murashima A, Ludwig M, Reutter H, Yamada G
Clin Genet 2017 Feb;91(2):247-253. Epub 2016 Oct 10 doi: 10.1111/cge.12871. PMID: 27649475
Pathogenesis of bladder exstrophy: A new hypothesis.
K V SK, Mammen A, Varma KK
J Pediatr Urol 2015 Dec;11(6):314-8. Epub 2015 Jul 30 doi: 10.1016/j.jpurol.2015.05.030. PMID: 26316280
Fetal abdominal wall defects.
Prefumo F, Izzi C
Best Pract Res Clin Obstet Gynaecol 2014 Apr;28(3):391-402. Epub 2013 Dec 3 doi: 10.1016/j.bpobgyn.2013.10.003. PMID: 24342556
Prenatal ultrasound and urological anomalies.
Clayton DB, Brock JW 3rd
Pediatr Clin North Am 2012 Aug;59(4):739-56. Epub 2012 Jun 22 doi: 10.1016/j.pcl.2012.05.003. PMID: 22857826

Recent systematic reviews

Long-term active problems in patients with cloacal exstrophy: A systematic review.
Musleh L, Privitera L, Paraboschi I, Polymeropoulos A, Mushtaq I, Giuliani S
J Pediatr Surg 2022 Mar;57(3):339-347. Epub 2021 Sep 4 doi: 10.1016/j.jpedsurg.2021.08.020. PMID: 34563362
Commentary on long-term active problems in patients with cloacal exstrophy: A systematic review.
Haney NM, Gearhart JP
J Pediatr Surg 2022 Mar;57(3):348-349. Epub 2021 Sep 16 doi: 10.1016/j.jpedsurg.2021.09.004. PMID: 34563356
Substitution Phalloplasty in Patients With Bladder Exstrophy-Epispadias Complex: A Systematic Review of Techniques, Complications and Outcomes.
Berrettini A, Sampogna G, Gnech M, Montanari E, Manzoni G, Di Grazia M, Castagnetti M
J Sex Med 2021 Feb;18(2):400-409. Epub 2020 Nov 20 doi: 10.1016/j.jsxm.2020.10.007. PMID: 33223423
Complete primary repair of bladder exstrophy: a systematic review.
Pathak P, Ring JD, Delfino KR, Dynda DI, Mathews RI
J Pediatr Urol 2020 Apr;16(2):149-153. Epub 2020 Jan 14 doi: 10.1016/j.jpurol.2020.01.004. PMID: 32144016
The Effect of Multiple Surgeries on Psychosocial Outcomes in Pediatric Patients: A Scoping Review.
Hu AC, Bertrand AA, Dang BN, Chan CH, Lee JC
Ann Plast Surg 2020 Nov;85(5):574-583. doi: 10.1097/SAP.0000000000002291. PMID: 32040002

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