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Urethral atresia, female

MedGen UID:
871393
•Concept ID:
C4025890
•
Congenital Abnormality
HPO: HP:0000067

Definition

Congenital anomaly characterized by closure or failure to develop an opening in the urethra in females. [from HPO]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVUrethral atresia, female

Professional guidelines

PubMed

Definition, diagnosis and management of fetal lower urinary tract obstruction: consensus of the ERKNet CAKUT-Obstructive Uropathy Work Group.
Capone V, Persico N, Berrettini A, Decramer S, De Marco EA, De Palma D, Familiari A, Feitz W, Herthelius M, Kazlauskas V, Liebau M, Manzoni G, Maternik M, Mosiello G, Schanstra JP, Vande Walle J, Wühl E, Ylinen E, Zurowska A, Schaefer F, Montini G
Nat Rev Urol 2022 May;19(5):295-303. Epub 2022 Feb 8 doi: 10.1038/s41585-022-00563-8. PMID: 35136187
Dysuria: Evaluation and Differential Diagnosis in Adults.
Michels TC, Sands JE
Am Fam Physician 2015 Nov 1;92(9):778-86. PMID: 26554471
Female urethral catheterization.
Clinical Practice Guidelines Task Force; Society of Urologic Nurses and Associates
Urol Nurs 2006 Aug;26(4):314. PMID: 16939049

Recent clinical studies

Etiology

Fetal biomarkers for lower urinary tract obstruction secondary to posterior urethral valves.
Schanstra JP, Decramer S, Buffin-Meyer B, Klein J, Fossum M, Wu HY
J Pediatr Urol 2024 Jun;20(3):492-496. Epub 2024 Jan 14 doi: 10.1016/j.jpurol.2024.01.011. PMID: 38280830
Dysuria: Evaluation and Differential Diagnosis in Adults.
Michels TC, Sands JE
Am Fam Physician 2015 Nov 1;92(9):778-86. PMID: 26554471
Embryology of anorectal malformations.
Kluth D
Semin Pediatr Surg 2010 Aug;19(3):201-8. doi: 10.1053/j.sempedsurg.2010.03.005. PMID: 20610193
Renal injury in sport.
Holmes FC, Hunt JJ, Sevier TL
Curr Sports Med Rep 2003 Apr;2(2):103-9. doi: 10.1249/00149619-200304000-00010. PMID: 12831667
Genitourinary trauma in children.
Livne PM, Gonzales ET Jr
Urol Clin North Am 1985 Feb;12(1):53-65. PMID: 3883625

Diagnosis

Dysuria: Evaluation and Differential Diagnosis in Adults.
Michels TC, Sands JE
Am Fam Physician 2015 Nov 1;92(9):778-86. PMID: 26554471
Urethral strictures in children.
Kaplan GW
Curr Opin Urol 2012 Nov;22(6):462-6. doi: 10.1097/MOU.0b013e328357bc78. PMID: 22918037
Antenatally detected hydronephrosis.
Piepsz A
Semin Nucl Med 2007 Jul;37(4):249-60. doi: 10.1053/j.semnuclmed.2007.02.008. PMID: 17544625
Urethral valves.
Agarwal S
BJU Int 1999 Sep;84(5):570-8. doi: 10.1046/j.1464-410x.1999.00307.x. PMID: 10510096
Incontinence.
Edwards LE
Practitioner 1975 Jan;214(1279):46-55. PMID: 1096112

Therapy

Definition, diagnosis and management of fetal lower urinary tract obstruction: consensus of the ERKNet CAKUT-Obstructive Uropathy Work Group.
Capone V, Persico N, Berrettini A, Decramer S, De Marco EA, De Palma D, Familiari A, Feitz W, Herthelius M, Kazlauskas V, Liebau M, Manzoni G, Maternik M, Mosiello G, Schanstra JP, Vande Walle J, Wühl E, Ylinen E, Zurowska A, Schaefer F, Montini G
Nat Rev Urol 2022 May;19(5):295-303. Epub 2022 Feb 8 doi: 10.1038/s41585-022-00563-8. PMID: 35136187
Gastric Adenocarcinoma Arising in Gastrocystoplasty.
Tran THT, Melamed J, Deng FM
Urology 2021 Feb;148:270-273. Epub 2020 Jul 16 doi: 10.1016/j.urology.2020.07.003. PMID: 32683064
Postoperative complications after reconstructive surgery for cloacal malformations: a systematic review.
Versteegh HP, Sutcliffe JR, Sloots CE, Wijnen RM, de Blaauw I
Tech Coloproctol 2015 Apr;19(4):201-7. Epub 2015 Feb 22 doi: 10.1007/s10151-015-1265-x. PMID: 25702171Free PMC Article
Urethral strictures in children.
Kaplan GW
Curr Opin Urol 2012 Nov;22(6):462-6. doi: 10.1097/MOU.0b013e328357bc78. PMID: 22918037
Incontinence.
Edwards LE
Practitioner 1975 Jan;214(1279):46-55. PMID: 1096112

Prognosis

Definition, diagnosis and management of fetal lower urinary tract obstruction: consensus of the ERKNet CAKUT-Obstructive Uropathy Work Group.
Capone V, Persico N, Berrettini A, Decramer S, De Marco EA, De Palma D, Familiari A, Feitz W, Herthelius M, Kazlauskas V, Liebau M, Manzoni G, Maternik M, Mosiello G, Schanstra JP, Vande Walle J, Wühl E, Ylinen E, Zurowska A, Schaefer F, Montini G
Nat Rev Urol 2022 May;19(5):295-303. Epub 2022 Feb 8 doi: 10.1038/s41585-022-00563-8. PMID: 35136187
Fetal lower urinary tract obstruction and its management.
Tonni G, Vito I, Ventura A, Grisolia G, De Felice C
Arch Gynecol Obstet 2013 Feb;287(2):187-94. Epub 2012 Nov 9 doi: 10.1007/s00404-012-2615-9. PMID: 23138754
Embryology of anorectal malformations.
Kluth D
Semin Pediatr Surg 2010 Aug;19(3):201-8. doi: 10.1053/j.sempedsurg.2010.03.005. PMID: 20610193
Megacystis in the first trimester.
Sepulveda W
Prenat Diagn 2004 Feb;24(2):144-9. doi: 10.1002/pd.790. PMID: 14974124
Renal injury in sport.
Holmes FC, Hunt JJ, Sevier TL
Curr Sports Med Rep 2003 Apr;2(2):103-9. doi: 10.1249/00149619-200304000-00010. PMID: 12831667

Clinical prediction guides

Fetal biomarkers for lower urinary tract obstruction secondary to posterior urethral valves.
Schanstra JP, Decramer S, Buffin-Meyer B, Klein J, Fossum M, Wu HY
J Pediatr Urol 2024 Jun;20(3):492-496. Epub 2024 Jan 14 doi: 10.1016/j.jpurol.2024.01.011. PMID: 38280830
Prenatal diagnosis of congenital megalourethra with imperforate anus.
Chao AS, Chang YL, Hsieh PC
BMC Pediatr 2019 Apr 23;19(1):123. doi: 10.1186/s12887-019-1510-y. PMID: 31014306Free PMC Article
Congenital urinary tract obstruction.
Cheung KW, Morris RK, Kilby MD
Best Pract Res Clin Obstet Gynaecol 2019 Jul;58:78-92. Epub 2019 Jan 11 doi: 10.1016/j.bpobgyn.2019.01.003. PMID: 30819578
Postoperative complications after reconstructive surgery for cloacal malformations: a systematic review.
Versteegh HP, Sutcliffe JR, Sloots CE, Wijnen RM, de Blaauw I
Tech Coloproctol 2015 Apr;19(4):201-7. Epub 2015 Feb 22 doi: 10.1007/s10151-015-1265-x. PMID: 25702171Free PMC Article
Posterior urethral valves: pre- and postnatal management.
Gatti JM, Kirsch AJ
Curr Urol Rep 2001 Apr;2(2):138-45. doi: 10.1007/s11934-001-0011-2. PMID: 12084283

Recent systematic reviews

Postoperative "complications" following laparoscopic-assisted anorectoplasty: A systematic review.
Pathak M, Saxena AK
Pediatr Surg Int 2020 Nov;36(11):1299-1307. Epub 2020 Sep 26 doi: 10.1007/s00383-020-04748-3. PMID: 32980932
Fetal megacystis: A systematic review.
Taghavi K, Sharpe C, Stringer MD
J Pediatr Urol 2017 Feb;13(1):7-15. Epub 2016 Oct 8 doi: 10.1016/j.jpurol.2016.09.003. PMID: 27889224
Safety of topical corticosteroids in pregnancy.
Chi CC, Wang SH, Wojnarowska F, Kirtschig G, Davies E, Bennett C
Cochrane Database Syst Rev 2015 Oct 26;2015(10):CD007346. doi: 10.1002/14651858.CD007346.pub3. PMID: 26497573Free PMC Article
Prenatal Diagnosis and Perinatal Outcomes of Congenital Megalourethra: A Multicenter Cohort Study and Systematic Review of the Literature.
Moaddab A, Sananes N, Hernandez-Ruano S, Werneck Britto IS, Blumenfeld Y, Stoll F, Favre R, Ruano R
J Ultrasound Med 2015 Nov;34(11):2057-64. Epub 2015 Oct 7 doi: 10.7863/ultra.14.12064. PMID: 26446816
Fetal lower urinary tract obstruction and its management.
Tonni G, Vito I, Ventura A, Grisolia G, De Felice C
Arch Gynecol Obstet 2013 Feb;287(2):187-94. Epub 2012 Nov 9 doi: 10.1007/s00404-012-2615-9. PMID: 23138754

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