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\begin{document}

\title{Unveiling the Menace of Incidental Insidious Chronic Kidney Disease due
to Posterior Urethral Valve - A Case Report}



\author[1]{Omran Janoud}%
\author[1]{Bashar khazaal}%
\author[1]{Marwa Alali}%
\author[1]{Anood Almohammed}%
\author[1]{Hala Wannous}%
\affil[1]{Damascus University Faculty of Medicine}%


\vspace{-1em}



  
  \date{June 18, 2024}


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\textbf{Unveiling the Menace of Incidental Insidious Chronic Kidney
Disease due to Posterior Urethral Valve - A Case Report}

\textbf{Authors:}

Janoud, Omran\textsuperscript{1}; Khazaal, Bashar\textsuperscript{1}; Al
Ali, Marwa\textsuperscript{1}; Almohammed, Anood\textsuperscript{1};
Wannous Hala\textsuperscript{2}

\textsuperscript{1} Damascus University, Faculty of Medicine,~Damascus
University Faculty of Medicine,~Damascus,~SYR.

\textsuperscript{2} Children's University Hospital, Damascus University,
Faculty of Medicine, Damascus, SYR

\textbf{Corresponding Author:}

Janoud; Omran

Postal Address: N/A

E-mail: omran2000janoud@gmail.com

Phone: +963998523327

\textbf{Funding} : We received no funding in any form.

\textbf{Key Clinical Message:}

Posterior Urethral Valve should be considered as a possible diagnosis
for chronic kidney disease in any child, even if their age at
presentation is not typical, and even if the initial diagnostic
assessment was normal.

\textbf{Keywords:}

Posterior Urethral valve, Chronic Kidney Disease, Pediatrics,
Nephrology, Urology.

\textbf{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.\textsuperscript{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.\textsuperscript{4,5}

In 40\% of cases, PUV is associated with other malformations including
aneuploidy, cardiac anomalies, and gastrointestinal
anomalies.\textsuperscript{1} Diagnosis can be made prenatally through
sonography.\textsuperscript{3,5} However, some cases are detected
postnatally during investigations for other
causes.\textsuperscript{5}The median age at which patients present is 5
months; presentations at older ages are considered
unusual.\textsuperscript{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.\textsuperscript{3}

It is rare to have follow-up cases as the majority of patients believe
that valve ablation is a complete solution.\textsuperscript{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.

\textbf{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.

\textbf{Differential diagnosis, investigations and treatment:}

Basic laboratory tests showed abnormal renal function with urea levels
at 60 g/dl and Creatinine levels at 2 g/dl. Full laboratory findings are
available in (Table 1)

Abdominal ultrasound confirmed ascites and revealed second-degree
hydronephrosis in the left kidney and first-degree hydronephrosis in the
right kidney. The diameter of ureter was increased bilaterally with
bilateral loss of corticomedullary differentiation. The bladder on
ultrasound appeared full and enlarged, filling most of the abdomen. Its
walls showed thickness and multiple diverticula along with a classified
polyp. These findings are consistent with neurogenic bladder and lower
urinary tract obstruction. MRI of the spine was performed to determine
the cause but no abnormalities were observed. MCUG confirmed bilateral
hydronephrosis with two large diverticula within the bladder wall
(Unfortunately unavailable). Renogram revealed a glomerular filtration
rate (GFR) of 40 ml/min for right kidney and 24 ml/min for left kidney
which is consistent with insidiously progressive chronic renal failure.
Initial management focused on protecting the kidneys from further damage
by performing ureterocutaneostomy as it was believed that patient had
neurogenic bladder.

After the surgery, urodynamics studies were conducted to rule out any
neurological bladder issues. However, these studies did not find any
evidence of a neurological bladder. As a result, the patient was
referred for a urethroscopy procedure.

During the urethroscopy, it was discovered that the patient had a
posterior urethral valve. The valve was treated by ablation during the
procedure. Subsequently, another urethroscopy was performed to assess
the condition of the urethra (video 1).

\textbf{Outcome and follow-up:}

Based on the findings and treatment provided, it was recommended that
retransplantation of the ureters onto the bladder should be carried out
after three months. During the period of follow up there was not any
complications reported and no urinary infections were observed.

\textbf{Discussion:}

Posterior urethral valves (PUV) are the most common form of lower
urinary tract obstructions (LUTO) found in male pediatric patients. The
embryonic abnormality responsible for PUV formation remains unknown.
Some authors suggest that it may result from the abnormal fusion of the
mesonephric duct into the urethra, while others suggest it is due to the
persistence of the cloacal membrane. The overall incidence of LUTO is
approximately 3.3 per 10,000 pregnancies and 2.3 per 10,000 live births.
The overall prevalence is reported to be 3.34 per 10,000 births, ranging
from 2.95 to 3.72.\textsuperscript{3,6}

About 35\% of cases are diagnosed in utero, and these cases are
suspected when there is bilateral hydronephrosis, megacystis, and
oligohydramnion or anhydramnion. On the other hand, 42\% of cases are
diagnosed during childhood, and the presenting symptoms include signs of
urinary obstruction and infection.\textsuperscript{3} Our patient
presented with loss of consciousness after a seizure; laboratory studies
revealed high levels of creatinine and urea consistent with chronic
kidney disease (CKD). Further assessment showed that the patient had
PUV.

The predominant presentation that persists in almost all patients with
PUV is voiding dysfunction (identified by poor stream, straining, and
dribbling). Additional features may include anemia, bladder distension
palpable above the pubic region, ascites, fever, ballotable kidneys, and
inability to thrive.\textsuperscript{4} Many complications can occur due
to the obstruction induced by PUV and it varies depending on the degree
of obstruction and the patient's age. The obstruction can range from
minimal to severe resulting in bilateral severe hydroureteronephrosis
because of the high pressure above the valve placement, chronic kidney
disease (CKD) which may progress to end-stage kidney disease (ESKD),
diverticular formation in the bladder, and even bladder rupture in
severe cases.\textsuperscript{7,8} Even though PUV manifests with
voiding dysfunction and CKD is considered a complication, our patient
presented with no signs or symptoms of voiding problems, and CKD was
detected during evaluation.

Micturating cysto-urethrogram (MCUG) is the most useful technique for
diagnosis. Ultrasonography is considered an assistive diagnostic method
especially for detecting complications such as diverticular formation in
the bladder and hydroureteronephrosis.\textsuperscript{7,8} In this
case, MCUG was not able to detect the valve which was then ablated
during urethroscopy.

As we have seen in this case report, PUV can lead to an insidious
progressive chronic kidney disease even after years of birth. As we
know, PUV is not uncommon; therefore, practitioners should consider it
as a differential diagnosis for CKD in any child even if their age at
presentation is not typical. Moreover, heigh suspicion is needed if
diagnostic assessment investigations show normal findings as in this
case.

\textbf{List of Abbreviations:}

PUV: Posterior Urethral Valve

MCUG: Micturating cysto-urethrogram

CKD: Chronic kidney disease

ESKD: End-stage kidney disease

LUTO: lower urinary tract obstructions

GFR: Glomerular filtration rate

\textbf{Declarations:}

\textbf{Ethics Approval:}

Ethical approval was taken from Faculty of Medicine at Damascus
University.

\textbf{Informed Consent:}

Written informed consent was obtained from the patient's parents for
their daughter's anonymized information to be published in this article.

\textbf{Availability of Data and Materials:}

The datasets used during the current study are available from the
corresponding author on reasonable request

\textbf{Competing Interests:}

The authors have declared that no competing interests exist.

\textbf{Funding:}

We received no funding in any form.

\textbf{Authors Contributions:}

All authors participated in writing and reviewing the whole paper

\textbf{Acknowledgments:}

Authors want to express their gratitude to all members, managers and
leaders of Scientific Research Team in Damascus who sacrificed their
time for helping in production of this paper. We want to thank them as
they paved our way toward achieving a great success in medical research
and fulfilling our needs in this field.

\textbf{Authorship List:}

Omran Janoud

Data curation, Methodology, Writing -- original draft, Writing -- review
\& editing

Bashar khazaal

Data curation, Writing -- original draft

Marwa Alali

Writing -- original draft

Anood Almohammed

Writing -- original draft

Hala Wannous

Writing -- review \& editing

\textbf{References:}

1. Mohamed AO, Eradi B, Owen A, Rajimwale A. Rare Associations with
Posterior Urethral Valves. \emph{Case Rep Urol} . 2021;2021:6647692.
doi:10.1155/2021/6647692

2. Alavi-Dunn N, Waisanen KM, Marrara JA, et al. Renal-Protective
Urinoma Formation in a Newborn Boy With Posterior Urethral
Valves.\emph{Cureus} . 2023;15(6):e39880. doi:10.7759/cureus.39880

3. Klaus R, Lange-Sperandio B. Chronic Kidney Disease in Boys with
Posterior Urethral Valves-Pathogenesis, Prognosis and
Management.\emph{Biomedicines} . 2022;10(8):1894.
doi:10.3390/biomedicines10081894

4. Talabi A, Sowande O, Etonyeaku A, Salako A, Adejuyigbe O. Posterior
urethral valves in children: Pattern of presentation and outcome of
initial treatment in Ile-Ife, Nigeria. \emph{Niger J Surg} .
2015;21(2):151. doi:10.4103/1117-6806.162591

5. Acharya R, DeMarco R, Upadhyay K. Gross Hematuria as a Presenting
Feature of Posterior Urethral Valves in a Neonate with Normal Antenatal
Sonograms. \emph{Med Basel Switz} . 2020;7(1):5.
doi:10.3390/medicines7010005

6. Malin G, Tonks A, Morris R, Gardosi J, Kilby M. Congenital lower
urinary tract obstruction: a population-based epidemiological
study.\emph{BJOG Int J Obstet Gynaecol} . 2012;119(12):1455-1464.
doi:10.1111/j.1471-0528.2012.03476.x

7. Ikhuoriah T, Oboh D, Abramowitz C, Musheyev Y, Shamsian E. Posterior
urethral valve: A case report in an older Nigerian child. \emph{Radiol
Case Rep} . 2023;18(5):1715-1720. doi:10.1016/j.radcr.2023.01.077

8. Lawal S, Ibinaiye P, Lawal A, Zaria M, Igashi J. Unusual presentation
of a rare case of posterior urethral valves in a nine-year-old
boy.\emph{Arch Int Surg} . 2016;6(3):186. doi:10.4103/2278-9596.202371

Table 1: laboratory findings when the patient was presented to the
emergency room.

Video 1: the second urethroscopy which was performed after removing the
valve in the first one.

\textbf{Hosted file}

\verb`table 1.docx`  available at \url{https://authorea.com/users/771398/articles/853818-unveiling-the-menace-of-incidental-insidious-chronic-kidney-disease-due-to-posterior-urethral-valve-a-case-report}

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