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Congenital megaureter

MedGen UID:
451022
•Concept ID:
C0266324
•
Congenital Abnormality
Synonyms: Congenital dilatation of ureter; Congenital megaloureter
SNOMED CT: Congenital dilatation of ureter (718485003); Congenital megaloureter (718485003); Congenital megaureter (718485003)
 
HPO: HP:0008676

Definition

A developmental disturbance with extreme ureteral dilatation. [from HPO]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVCongenital megaureter

Conditions with this feature

Congenital anomalies of kidney and urinary tract 2
MedGen UID:
1804316
•Concept ID:
C5574705
•
Disease or Syndrome
Congenital anomalies of the kidneys and urinary tract (CAKUT) encompasses a spectrum of developmental disorders of the urinary tract that can range from mild vesicoureteral reflux to severe renal agenesis. Other phenotypes include renal duplication, small kidneys, ureteropelvic junction obstruction, hydronephrosis, and renal dysplasia. These abnormalities can result in kidney damage, and possibly renal failure (summary by Vivante et al., 2015). For a discussion of genetic heterogeneity of CAKUT, see 610805.
See: Condition Record
Congenital anomalies of kidney and urinary tract 2

Professional guidelines

PubMed

British Association of Paediatric Urologists consensus statement on the management of the primary obstructive megaureter.
Farrugia MK, Hitchcock R, Radford A, Burki T, Robb A, Murphy F; British Association of Paediatric Urologists
J Pediatr Urol 2014 Feb;10(1):26-33. Epub 2013 Oct 16 doi: 10.1016/j.jpurol.2013.09.018. PMID: 24206785
Role of prenatal diagnosis in the treatment of congenital obstructive megaureter in a solitary kidney.
Caione P, Patricolo M, Lais A, Capitanucci ML, Capozza N, Ferro F
Fetal Diagn Ther 1996 May-Jun;11(3):205-9. doi: 10.1159/000264303. PMID: 8739588

Recent clinical studies

Etiology

A Single-Center Study of the Efficacy of Transvesicoscopic Ureterovesical Reimplantation: With or Without Ureteral Tailoring in Children with Congenital Megaureter.
Chu H, Cao YS, Deng QF, Mao CK
J Endourol 2023 Aug;37(8):889-894. Epub 2023 Jul 3 doi: 10.1089/end.2022.0834. PMID: 37265149
Rare Single Nucleotide and Copy Number Variants and the Etiology of Congenital Obstructive Uropathy: Implications for Genetic Diagnosis.
Ahram DF, Lim TY, Ke J, Jin G, Verbitsky M, Bodria M, Kil BH, Chatterjee D, Piva SE, Marasa M, Zhang JY, Cocchi E, Caridi G, Gucev Z, Lozanovski VJ, Pisani I, Izzi C, Savoldi G, Gnutti B, Capone VP, Morello W, Guarino S, Esposito P, Lambert S, Radhakrishnan J, Appel GB, Uy NS, Rao MK, Canetta PA, Bomback AS, Nestor JG, Hays T, Cohen DJ, Finale C, Wijk JAEV, La Scola C, Baraldi O, Tondolo F, Di Renzo D, Jamry-Dziurla A, Pezzutto A, Manca V, Mitrotti A, Santoro D, Conti G, Martino M, Giordano M, Gesualdo L, Zibar L, Masnata G, Bonomini M, Alberti D, La Manna G, Caliskan Y, Ranghino A, Marzuillo P, Kiryluk K, Krzemień G, Miklaszewska M, Lin F, Montini G, Scolari F, Fiaccadori E, Arapović A, Saraga M, McKiernan J, Alam S, Zaniew M, Szczepańska M, Szmigielska A, Sikora P, Drożdż D, Mizerska-Wasiak M, Mane S, Lifton RP, Tasic V, Latos-Bielenska A, Gharavi AG, Ghiggeri GM, Materna-Kiryluk A, Westland R, Sanna-Cherchi S
J Am Soc Nephrol 2023 Jun 1;34(6):1105-1119. Epub 2023 Mar 30 doi: 10.1681/ASN.0000000000000132. PMID: 36995132Free PMC Article
Pure laparoscopic and robot-assisted laparoscopic reconstructive surgery in congenital megaureter: a single institution experience.
Fu W, Zhang X, Zhang X, Zhang P, Gao J, Dong J, Chen G, Xu A, Ma X, Li H, Shi L
PLoS One 2014;9(6):e99777. Epub 2014 Jun 12 doi: 10.1371/journal.pone.0099777. PMID: 24924420Free PMC Article
Congenital megaureter in adults: endoscopic management with holmium: YAG laser--preliminary experience.
Biyani CS, Powell CS
J Endourol 2001 Oct;15(8):797-9. doi: 10.1089/089277901753205771. PMID: 11724117
Antenatal renal pelvis dilatation: a predictor of vesicoureteral reflux?
Walsh G, Dubbins PA
AJR Am J Roentgenol 1996 Oct;167(4):897-900. doi: 10.2214/ajr.167.4.8819378. PMID: 8819378

Diagnosis

Rare Single Nucleotide and Copy Number Variants and the Etiology of Congenital Obstructive Uropathy: Implications for Genetic Diagnosis.
Ahram DF, Lim TY, Ke J, Jin G, Verbitsky M, Bodria M, Kil BH, Chatterjee D, Piva SE, Marasa M, Zhang JY, Cocchi E, Caridi G, Gucev Z, Lozanovski VJ, Pisani I, Izzi C, Savoldi G, Gnutti B, Capone VP, Morello W, Guarino S, Esposito P, Lambert S, Radhakrishnan J, Appel GB, Uy NS, Rao MK, Canetta PA, Bomback AS, Nestor JG, Hays T, Cohen DJ, Finale C, Wijk JAEV, La Scola C, Baraldi O, Tondolo F, Di Renzo D, Jamry-Dziurla A, Pezzutto A, Manca V, Mitrotti A, Santoro D, Conti G, Martino M, Giordano M, Gesualdo L, Zibar L, Masnata G, Bonomini M, Alberti D, La Manna G, Caliskan Y, Ranghino A, Marzuillo P, Kiryluk K, Krzemień G, Miklaszewska M, Lin F, Montini G, Scolari F, Fiaccadori E, Arapović A, Saraga M, McKiernan J, Alam S, Zaniew M, Szczepańska M, Szmigielska A, Sikora P, Drożdż D, Mizerska-Wasiak M, Mane S, Lifton RP, Tasic V, Latos-Bielenska A, Gharavi AG, Ghiggeri GM, Materna-Kiryluk A, Westland R, Sanna-Cherchi S
J Am Soc Nephrol 2023 Jun 1;34(6):1105-1119. Epub 2023 Mar 30 doi: 10.1681/ASN.0000000000000132. PMID: 36995132Free PMC Article
Diagnosis of megaureter by point-of-care ultrasound.
Mackenzie DC, Sajed D, Noble VE
J Emerg Med 2014 Jul;47(1):e1-3. Epub 2013 Nov 5 doi: 10.1016/j.jemermed.2013.08.039. PMID: 24199726
Magnetic resonance urography and X-ray urography findings of congenital megaureter.
Li TR, Du XK, Huo TL
Chin Med Sci J 2011 Jun;26(2):103-8. doi: 10.1016/s1001-9294(11)60028-x. PMID: 21703118
Morphological study of congenital megaureter.
Vlad M, Ionescu N, Ispas AT, Ungureanu E, Stoica C
Rom J Morphol Embryol 2007;48(4):381-90. PMID: 18060188
Congenital megaureter.
Lockhart JL, Singer AM, Glenn JF
J Urol 1979 Sep;122(3):310-4. doi: 10.1016/s0022-5347(17)56380-6. PMID: 470000

Therapy

Antegrade MDCT pyelography for the evaluation of patients with obstructed urinary tract.
Ghersin E, Brook OR, Meretik S, Kaftori JK, Ofer A, Amendola MA, Engel A
AJR Am J Roentgenol 2004 Dec;183(6):1691-6. doi: 10.2214/ajr.183.6.01831691. PMID: 15547210

Prognosis

Rare Single Nucleotide and Copy Number Variants and the Etiology of Congenital Obstructive Uropathy: Implications for Genetic Diagnosis.
Ahram DF, Lim TY, Ke J, Jin G, Verbitsky M, Bodria M, Kil BH, Chatterjee D, Piva SE, Marasa M, Zhang JY, Cocchi E, Caridi G, Gucev Z, Lozanovski VJ, Pisani I, Izzi C, Savoldi G, Gnutti B, Capone VP, Morello W, Guarino S, Esposito P, Lambert S, Radhakrishnan J, Appel GB, Uy NS, Rao MK, Canetta PA, Bomback AS, Nestor JG, Hays T, Cohen DJ, Finale C, Wijk JAEV, La Scola C, Baraldi O, Tondolo F, Di Renzo D, Jamry-Dziurla A, Pezzutto A, Manca V, Mitrotti A, Santoro D, Conti G, Martino M, Giordano M, Gesualdo L, Zibar L, Masnata G, Bonomini M, Alberti D, La Manna G, Caliskan Y, Ranghino A, Marzuillo P, Kiryluk K, Krzemień G, Miklaszewska M, Lin F, Montini G, Scolari F, Fiaccadori E, Arapović A, Saraga M, McKiernan J, Alam S, Zaniew M, Szczepańska M, Szmigielska A, Sikora P, Drożdż D, Mizerska-Wasiak M, Mane S, Lifton RP, Tasic V, Latos-Bielenska A, Gharavi AG, Ghiggeri GM, Materna-Kiryluk A, Westland R, Sanna-Cherchi S
J Am Soc Nephrol 2023 Jun 1;34(6):1105-1119. Epub 2023 Mar 30 doi: 10.1681/ASN.0000000000000132. PMID: 36995132Free PMC Article
Robot-assisted tapered ureteral reimplantation for congenital megaureter.
Goh AC, Link RE
Urology 2011 Mar;77(3):742-5. doi: 10.1016/j.urology.2010.05.051. PMID: 20801491
Congenital megaureter in adults: endoscopic management with holmium: YAG laser--preliminary experience.
Biyani CS, Powell CS
J Endourol 2001 Oct;15(8):797-9. doi: 10.1089/089277901753205771. PMID: 11724117
Antenatal renal pelvis dilatation: a predictor of vesicoureteral reflux?
Walsh G, Dubbins PA
AJR Am J Roentgenol 1996 Oct;167(4):897-900. doi: 10.2214/ajr.167.4.8819378. PMID: 8819378
Congenital megaureter.
Lockhart JL, Singer AM, Glenn JF
J Urol 1979 Sep;122(3):310-4. doi: 10.1016/s0022-5347(17)56380-6. PMID: 470000

Clinical prediction guides

Totally laparoscopic repair of primary obstructive megaureter with pyeloplasty, complete excisional tailoring and nonrefluxing ureteral reimplantation.
Almeida GL, Busato WF, de Cobelli O
Actas Urol Esp 2014 Mar;38(2):127-32. Epub 2013 Aug 1 doi: 10.1016/j.acuro.2013.04.011. PMID: 23910727
Magnetic resonance urography and X-ray urography findings of congenital megaureter.
Li TR, Du XK, Huo TL
Chin Med Sci J 2011 Jun;26(2):103-8. doi: 10.1016/s1001-9294(11)60028-x. PMID: 21703118
Antenatal renal pelvis dilatation: a predictor of vesicoureteral reflux?
Walsh G, Dubbins PA
AJR Am J Roentgenol 1996 Oct;167(4):897-900. doi: 10.2214/ajr.167.4.8819378. PMID: 8819378
Role of prenatal diagnosis in the treatment of congenital obstructive megaureter in a solitary kidney.
Caione P, Patricolo M, Lais A, Capitanucci ML, Capozza N, Ferro F
Fetal Diagn Ther 1996 May-Jun;11(3):205-9. doi: 10.1159/000264303. PMID: 8739588

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