Puigvert's disease: A rare case
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Calyx dilation
Renal ultrasound
Diuretic Renogram

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Guerrero Tinoco GA, Moreno Maya DP. Puigvert’s disease: A rare case. Rev. Colomb. Nefrol. [Internet]. 2024 Jan. 24 [cited 2024 Feb. 26];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.

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