Puigvert's disease: A rare case
PDF (Español)


Calyx dilation
Renal ultrasound
Diuretic Renogram

How to Cite

Guerrero Tinoco GA, Moreno Maya DP. Puigvert’s disease: A rare case. Rev. Colomb. Nefrol. [Internet]. 2024 Jan. 24 [cited 2024 Jul. 18];11(1). Available from: https://revistanefrologia.org/index.php/rcn/article/view/722


Background: Puigvert's disease, or megacaliosis, is a rare congenital disorder of the urinary collecting system, characterized by dilation of the renal calyces without dilation of the urinary tract.

Purpose:  The purpose of this article is to report the case of Puigvert's disease or megacaliosis identified in an infant less than three months of age.

Case presentation: This article reports the case of a minor infant with bilateral megacaliosis in whom a postnatal diagnosis was made, based on a prenatal diagnosis of multicystic kidney.

Discussion and conclusion: This is a rare entity, with few cases reported worldwide, of a benign course, which represents a risk factor for the development of secondary complications, for which early identification is essential, as well as ruling out other differential diagnoses, including hydronephrosis, which was initially suspected in our patient.

In the case of the infant in this report, early diagnosis could be made thanks to prenatal imaging tests that allowed the diagnostic suspicion of a renal abnormality with subsequent postnatal ultrasound confirmation; During follow-up, the patient remained asymptomatic with no evidence of complications.

PDF (Español)


Sosa Barrios RH, Rivera Gorrín ME. Megacalycosis: Sonographic findings of a rare clinical condition. Clin Case Rep. 2020 dic. 26;9(2):1041-2. https://doi.org/10.1002/ccr3.3687

Cho CL, Shiu CK. Megacalycosis: a rare radiological finding. Hong Kong Med J. 2020 dic.;26(6):539.e1-2. https://doi.org/10.12809/hkmj208463

Pieretti-Vanmarcke R, Pieretti A, Pieretti RV. Megacalycosis: a rare condition. Pediatr Nephrol. 2009 my.;24(5):1077-9. https://doi.org/10.1007/s00467-008-1039-z

Houat A, Guimarães C, Takahashi M, Rodi G, Gasparetto T, Blasbalg R, et al. Congenital Anomalies of the Upper Urinary Tract: A Comprehensive Review. RadioGraphics. 2021 en. 29;41(2). https://doi.org/10.1148/rg.2021200078

Parlaktas BS, Erdemir F, Uluocak N. Megacalycosis congénita: una rara anomalía del desarrollo renal. Turco J Med Sci. 2004;34(6):401-3.

Turkmen E, Yildirim T, Ciftci T, Altindal M, Akinci D, Baydar DE, et al. Congenital megacalycosis with IgA nephropathy: a case report and review of the literature. Ren Fail. 2013;35(1):155-8. https://doi.org/10.3109/0886022X.2012.731996

Kasap B, Kavukçu S, Soylu A, Türkmen M, Seçil M. Megacalycosis: report of two cases. Pediatr Nephrol. 2005 jun.;20(6):828-30. https://doi.org/10.1007/s00467-004-1809-1

Kalaitzis C, Patris E, Deligeorgiou E, Sountoulides P, Bantis A, Giannakopoulos S, et al. Radiological findings and the clinical importance of megacalycosis. Res Rep Urol. 2015 oct. 19;7:153-5. https://doi.org/10.2147/RRU.S81519

Bawri B, Puthenveetil RT, Baruah SJ, Barua SK, Bagchi PK. Megacalycosis or Puigvert Disease, a Rare Congenital Calyceal Anomaly: A Report of 3 Cases. UroToday Int J. 2012;5(2). https://doi.org/10.3834/uij.1944-5784.2012.04.01

Vidal Company A, Gonzálvez Piñera J, Ruiz Canoa R, Gutiérrez Junqueraa C, Lillo Lilloa M. Megacaliosis diagnosticada en el estudio de una hidronefrosis prenatal. An Pediatr. 2001;54(1):74-7. https://doi.org/10.1016/S1695-4033(01)78653-5

Castría MA, Biso N, Fasola J. Megacaliosis. Rev Arg Urol Nefrol.1984;50:48-9

O' Connor CJ, Kinnear N, Browne G, Hennessey DB. Bilateral staghorn kidney stones in Megacalycosis: Non operative management of complex kidney stone disease. Urol Case Rep. 2022 jun. 26;44:102146. https://doi.org/10.1016/j.eucr.2022.102146

Creative Commons License

This work is licensed under a Creative Commons Attribution-NonCommercial-NoDerivatives 4.0 International License.




Download data is not yet available.