P2A291: Delayed Recognition of Posterior Urethral Valves in a Patient with Constipation, Urosepsis, Chronic Kidney Disease and Hypertension: A Case Report

Introduction: Posterior urethral valves (PUV) are growths of membranous folds in the urethra that can cause obstruction, acute urinary retention, and chronic kidney disease (CKD). Symptoms include delayed voiding, urosepsis, and palpable bladder. PUV are typically diagnosed via ultrasound during prenatal screening with signs of bilateral hydroureteronephrosis, dilated urethra, and a distended bladder, but in some cases, they are diagnosed postnatally. There has been an increasing interest in the utility of surgical intervention in an attempt to preserve kidney function. We present a patient with a delayed diagnosis of PUV with evidence of end-organ damage.

Case Description: A 3-year-old boy with a history of constipation presented with abdominal pain and distension. He was managed for these symptoms as an outpatient. On initial presentation to the emergency department, he was febrile, hypertensive, and tachycardic with a soft yet distended abdomen. Abdominal X-rays revealed a moderate amount of stool. He received an enema and was discharged home with outpatient follow-up. He returned with worsening abdominal distension and diffuse abdominal tenderness with guarding. Laboratory studies revealed increased inflammatory markers, urosepsis secondary to E. coli, as well as acute-on-chronic CKD. Abdominal and renal ultrasounds revealed bilateral hydronephrosis, cortical loss, and bladder wall thickening. Patient received appropriate management and was admitted to the pediatric intensive care unit (PICU) for further care of acute pyelonephritis, hydronephrosis, hypertension as well as acute-on-chronic CKD.
PICU evaluation included a multisystem approach. Cardiology workup revealed chronic hypertension and left ventricular hypertrophy. Urology workup included an unsuccessful attempt at voiding cystourethrogram (VCUG) to evaluate for PUV. Patient was taken in for valve ablation to confirm and obliterate the PUV. Pancystoscopy revealed an abnormal bladder with large diverticula and edematous changes in the mucosa. Patient remained in the PICU until clinical improvement and was discharged with appropriate follow-up.

Discussion: Children with PUV will develop CKD in approximately 50% of cases and 20% will develop end-stage renal disease. Due to increased prenatal screening, PUV are typically diagnosed in utero and are oftentimes managed after birth. This patient had a delayed diagnosis of PUV at 3 years old and by that time was already having signs of multi-organ damage. Although he was evaluated by multiple providers, he never received the proper workup likely due to a low index of suspicion. PUV management is centered around managing bladder dysfunction and CKD to slow progression of irreversible kidney damage. This case illustrates the vital role of pediatricians and medical providers in recognizing serious symptoms and recommending further evaluation.

Conclusion: Immediate evaluation and intervention with proper imaging could have prevented this patient’s progression to mild-to-moderate CKD. Due to the high proportion of children with PUV progressing to CKD, the high index of suspicion and use of imaging for patients should be re-established.