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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
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

Recent clinical studies

Etiology

Lower Urinary Tract Obstruction in Newborns.
Flores-Torres J, Sanchez-Valle A, Duncan JR, Panzarino V, Rodriguez JM, Kirby RS
Adv Pediatr 2023 Aug;70(1):131-144. Epub 2023 May 15 doi: 10.1016/j.yapd.2023.03.001. PMID: 37422291
Dysuria: Evaluation and Differential Diagnosis in Adults.
Michels TC, Sands JE
Am Fam Physician 2015 Nov 1;92(9):778-86. PMID: 26554471
Female epispadias.
Shetty MV, Bhaskaran A, Sen TK
Afr J Paediatr Surg 2011 May-Aug;8(2):215-7. doi: 10.4103/0189-6725.86066. PMID: 22005369
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
Anorectal malformations.
Peña A
Semin Pediatr Surg 1995 Feb;4(1):35-47. PMID: 7728507

Diagnosis

Lower Urinary Tract Obstruction in Newborns.
Flores-Torres J, Sanchez-Valle A, Duncan JR, Panzarino V, Rodriguez JM, Kirby RS
Adv Pediatr 2023 Aug;70(1):131-144. Epub 2023 May 15 doi: 10.1016/j.yapd.2023.03.001. PMID: 37422291
Dysuria: Evaluation and Differential Diagnosis in Adults.
Michels TC, Sands JE
Am Fam Physician 2015 Nov 1;92(9):778-86. PMID: 26554471
Antenatally detected hydronephrosis.
Piepsz A
Semin Nucl Med 2007 Jul;37(4):249-60. doi: 10.1053/j.semnuclmed.2007.02.008. PMID: 17544625
Posterior urethral valves.
Manzoni C, Valentini AL
Rays 2002 Apr-Jun;27(2):131-4. PMID: 12696266
Incontinence.
Edwards LE
Practitioner 1975 Jan;214(1279):46-55. PMID: 1096112

Therapy

Acute Uncomplicated UTIs in Adults: Rapid Evidence Review.
Kurotschka PK, Gágyor I, Ebell MH
Am Fam Physician 2024 Feb;109(2):167-174. PMID: 38393801
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
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
The vesical calculus.
Schwartz BF, Stoller ML
Urol Clin North Am 2000 May;27(2):333-46. doi: 10.1016/s0094-0143(05)70262-7. PMID: 10778475
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
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
Female epispadias.
Shetty MV, Bhaskaran A, Sen TK
Afr J Paediatr Surg 2011 May-Aug;8(2):215-7. doi: 10.4103/0189-6725.86066. PMID: 22005369
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
Anorectal malformations.
Peña A
Semin Pediatr Surg 1995 Feb;4(1):35-47. PMID: 7728507

Clinical prediction guides

Ontogeny of estrogen receptors in human male and female fetal reproductive tracts.
Cunha GR, Li Y, Mei C, Derpinghaus A, Baskin LS
Differentiation 2021 Mar-Apr;118:107-131. Epub 2020 Oct 17 doi: 10.1016/j.diff.2020.10.001. PMID: 33176961Free PMC Article
Fetal bladder outflow obstruction: Interventions, outcomes and management uncertainties.
Farrugia MK
Early Hum Dev 2020 Nov;150:105189. Epub 2020 Sep 10 doi: 10.1016/j.earlhumdev.2020.105189. PMID: 32978001
MRI of the Male Pelvic Floor.
Lakhoo J, Khatri G, Elsayed RF, Chernyak V, Olpin J, Steiner A, Tammisetti VS, Sundaram KM, Arora SS
Radiographics 2019 Nov-Dec;39(7):2003-2022. doi: 10.1148/rg.2019190064. PMID: 31697623
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
Environmental factors and testicular function.
Giwercman A, Giwercman YL
Best Pract Res Clin Endocrinol Metab 2011 Apr;25(2):391-402. doi: 10.1016/j.beem.2010.09.011. PMID: 21397206

Recent systematic reviews

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
Surgical management of functional bladder outlet obstruction in adults with neurogenic bladder dysfunction.
Utomo E, Groen J, Blok BF
Cochrane Database Syst Rev 2014 May 24;2014(5):CD004927. doi: 10.1002/14651858.CD004927.pub4. PMID: 24859260Free PMC Article
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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