OBSOLETE: Congenital hydronephrosis

Congenital hydronephrosis refers to the dilation of the renal pelvis and calyces of the kidney that is present at birth, resulting from obstruction or impaired drainage of urine from the kidney. This condition is now considered an obsolete entry in Orphanet (ORPHA:2190), as it has been reclassified and incorporated into more specific diagnostic categories such as congenital ureteropelvic junction obstruction, congenital megaureter, or other obstructive uropathies. The condition primarily affects the urinary system, with the kidneys being the main organ involved. When urine cannot drain properly, it accumulates in the kidney, causing swelling that can range from mild to severe. If left untreated, significant hydronephrosis can lead to progressive kidney damage and loss of renal function. Congenital hydronephrosis is frequently detected prenatally during routine ultrasound examinations, making it one of the most commonly identified fetal anomalies. Key symptoms may include flank pain, urinary tract infections, and in severe cases, palpable abdominal mass in newborns. Many mild cases resolve spontaneously without intervention. Treatment depends on the underlying cause and severity, ranging from watchful waiting with serial ultrasound monitoring to surgical intervention such as pyeloplasty (repair of the ureteropelvic junction) in cases with significant obstruction or declining kidney function. Early detection and appropriate management are essential to preserve renal function. Patients are advised to consult current disease classifications for the most specific and up-to-date diagnostic terminology.

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