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Infundibulopelvic stenosis in children
Insights
Infundibulopelvic stenosis is a rare kidney malformation in children. This study found it has characteristic radiographic features and a good prognosis for most affected patients.
Area of Science:
- Pediatric Radiology
- Nephrology
- Congenital Malformations
Background:
- Infundibulopelvic stenosis is a rare renal malformation.
- This condition is characterized by caliceal dilatation, infundibular stenosis, and renal pelvis hypoplasia or stenosis.
Observation:
- Excretory urography identified three cases of infundibulopelvic stenosis in 11,500 children over 17 years.
- Renal sonography revealed caliceal dilatation without pelvic dilatation; voiding cystourethrograms were normal.
- Retrograde ureteropyelography confirmed the diagnosis in one patient.
Findings:
- Two patients with infundibulopelvic stenosis remained asymptomatic with normal renal function and stable sonographic findings over 12-18 months.
- The contralateral kidney was absent in two cases and normal in one.
- The third patient died from an unrelated cause.
Implications:
- Infundibulopelvic stenosis exhibits distinct radiographic features.
- The prognosis for most children with infundibulopelvic stenosis is favorable.
- Early diagnosis through imaging modalities like sonography and urography is crucial.
Abstract:
Of 11,500 children who underwent excretory urography during a 17-year period, three were found to have the rare renal malformation infundibulopelvic stenosis, characterized by caliceal dilatation, infundibular stenosis, and hypoplasia or stenosis of the renal pelvis. The contralateral kidney was absent in two cases and normal in the other. Voiding cystourethrograms were normal in all three. Renal sonography showed a variable degree of caliceal dilatation without associated pelvic dilatation. The diagnosis was confirmed by retrograde ureteropyelography in one case. Two patients were followed for 12 and 18 months, respectively; both remained asymptomatic with normal renal function, and sequential sonographic examinations of their kidneys have shown no significant changes. The third patient died of an unrelated condition. Infundibulopelvic stenosis has highly characteristic radiographic features, and prognosis is good for most affected patients.
