Acute pain crisis as a presentation of primary megaureter in children.

PURPOSE: Congenital ureteropelvic junction obstruction may present with flank pain, commonly referred to as a Dietl's crisis. We report on a similar presentation of primary megaureter.

METHODS AND MATERIALS: We identified all patients with any diagnosis of megaureter treated at our institution between 1993 and 2009 (n = 465). We included 103 patients with primary megaureter and, of these, identified patients presenting with pain. All patients with secondary megaureter were excluded.

RESULTS: Seventeen patients (20 megaureters) presented with pain and 10 (13 megaureters) presented with an acute pain crisis. Median age at presentation was 77 months. Seven children initially presented to the emergency department for evaluation and two of them required urgent stent placement for intractable pain. Obstruction was diagnosed in 8 patients. Six patients underwent ureteral reimplantation. Four patients were initially observed: one failed observation at 12 months due to worsening hydronephrosis and required surgery, one was lost to follow up, and two are still successfully being followed. All patients who underwent surgery had pain resolution.

CONCLUSION: In this contemporary cohort, approximately 17% of patients with primary megaureter presented with pain and 10% presented with an acute pain crisis. Most presenting in acute pain required surgery, which resolved presenting symptoms.