A 6-year-old boy presents with an acute onset of left flank pain associated with nausea and vomiting. The pain started as a dull ache and escalated into colicky severe left flank and abdominal pain. He has never had any prior episodes. There have been no other health issues. He was born full term. There is no significant past general medical or genitourinary history. Physical exam reveals that he is in moderate distress. His vital signs are stable and he is afebrile. The only pertinent physical exam finding is that he has severe left CVA tenderness and left-sided abdominal pain. Laboratory analysis showed a normal white blood cell count, normal urine analysis, and normal basic metabolic panel. An ultrasound was obtained (Figure 1 and Figure 2). An IVP (Figure 3) was obtained which led to an MR Urogram (Figure 4).

• 1.Detrusor hyperactivity
• 2.Stress urinary incontinence
• 3.Voiding dysfunction with constipation
• 4.Areflexic bladder
• 5.Late presentation of a primary obstructing megaureter (POM)

Correct!

Megaureter, the accepted term for the dilated ureter, is divided into primary (congenital) and secondary categories. Each category is further subdivided into (1) refluxing nonobstructing and (2) nonrefluxing obstructing. These distinctions are based on radiographic and clinical findings. The incidence is 1:10,000. The male-to-female ratio ranges from 1.2:1.0 to 4.8:1.0. The left-to-right ratio ranges from 1.7:1.0 to 4.5:1.0. Obstruction is bilateral in 10-20% of cases. Primary obstructing megaureter (POM) is caused by a structural alteration in the muscular layers of the distal ureter, which is characterized by diminished or absent longitudinal muscle fibers, hypertrophied or hyperplastic circular muscle fibers, or increased connective tissue deposition. These changes are defined pathologically and may represent either an arrest of normal development or a response of the ureter to ongoing obstruction. A partial obstruction in the abnormal distal segment of the ureter leads to progressive dilatation. Complete obstructions are rare and usually associated with a poorly functioning renal unit at diagnosis. Children with an obstructed megaureter may present with vague abdominal pain, hematuria, UTI, fever of unknown origin, or an abdominal mass. The primary presentation, antenatal hydronephrosis, is diagnosed through prenatal ultrasonography. The present case is a very rare entity because most POMs resolve by 1 year of age. Typically, if a POM becomes symptomatic, it would present earlier than 6 years of age.

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