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.