Introduction:
Posterior Urethral Valve (PUV), the etiology of which is not fully understood, is considered the most prevalent congenital cause of bladder outlet obstruction in male children, with an incidence of 1 in 3800.1–3
PUV can lead to enuresis, urosepsis, chronic kidney disease, and even death. It is very rare for it to present as macroscopic hematuria.4,5
In 40% of cases, PUV is associated with other malformations including aneuploidy, cardiac anomalies, and gastrointestinal anomalies.1 Diagnosis can be made prenatally through sonography.3,5 However, some cases are detected postnatally during investigations for other causes.5The median age at which patients present is 5 months; presentations at older ages are considered unusual.4
The primary management focuses on protecting the kidneys through transurethral or suprapubic catheterization for drainage. After that, the main treatment is primary valve ablation through transurethral incision. If there is difficulty in removing the valve, vesicostomy, ureterocutaneostomy or nephrostomy can be performed. Complications include irreversible hypoplastic and dysplastic kidneys as well as lifelong bladder dysfunction and chronic kidney disease.3
It is rare to have follow-up cases as the majority of patients believe that valve ablation is a complete solution.4
What makes this reported case unique is the age at which the patient presented and the main complaint of seizures. During evaluation, an insidious progressive chronic renal failure was observed.
Case History and Examination:
A 5-year-old boy from consanguineous parents presented to the emergency room of Children’s Hospital in Damascus with a chief complaint of a seizure episode. According to the parents, the child had two episodes of limb shaking that lasted for minutes and were consistent with Tonic-Clonic seizures. These episodes were considered benign febrile seizures as they occurred during periods of hyperthermia following pharyngitis and flu. The last episode lasted for 2 minutes and the patient lost consciousness afterward. The patient was admitted to the emergency room for evaluation.
Upon arrival, the patient had woken up and vital signs were within normal ranges for his age. Medical history revealed a slight delay in motor and language development and a history of surgically treated undescended testes at the age of 1 year.
Physical examination showed ascites and tenderness in the right iliac region. Additionally, enlarged lymph nodes were observed in the neck, axilla, and groin.