110 Xia et al.
Investigación Clínica 62(2): 2021
system malformation: hydrocephalus, cra-
niostenosis, TC, syringomyelia, intraspinal
mass. Limb malformations: absence of ra-
dius, polydactyly. Genital malformations:
cryptorchidism. Cardiovascular malforma-
tions: dextrocardia, ventricular septal de-
fect. Therefore, other factors apart from the
embryological abnormalities might lead to
multiple malformations.
In our study, three patients with CS had
lipomyelomeningocele and postoperative
VUDS assessment revealed low-risk bladder
in all of them. However, long-term follow-
up of bladder function is required. Other
causes of lower urinary tract dysfunction
may include primary or secondary TC, sacral
nerve development defect, intraspinal mass,
sphincter complex injury during anoplasty,
or nerve injury during neurosurgery.
The present study focuses on the urolog-
ical evaluation and management of patients
with CS. Lee et al. retrospectively analyzed
the urinary symptoms and urodynamics be-
fore and after TC release in 14 patients with
CS (6). Pio et al. reported the clinical symp-
toms, imaging findings, and urodynamics
of 16 patients with CS (7). Our study used
VUDS to evaluate the bladder function of pa-
tients with CS. The risk of bladder function
was classified based on the results of VUDS.
According to the severity of bladder risk, we
performed individualized clinical manage-
ment to protect renal function.
In 2007, Joseph developed a risk index
for bladder based on urodynamic assessment
comprising of DLPP, NDO, and DSD (8).
Tarcan et al. proposed that the predictive
sensitivity of DLPP 20 cm H
2
O for upper uri-
nary tract injury is higher than that of 40 cm
H
2
O (10). However, there are several deter-
minants of the risk of upper urinary tract in-
jury in these patients. The bladder risk classi-
fication proposed by Joseph is an important
tool to guide treatment decision-making for
neurogenic bladder. However, the risk rating
does not incorporate the results of imagen
examination. Therefore, we incorporated the
results of imaging examination in the risk
classification of bladder. For grade I to IV
VUR and/or lack of smoothness of the blad-
der wall, the bladder risk increases by one
level. A high-risk bladder should be defined
when V grade VUR occurs.
Based on our experience, we have de-
veloped a comprehensive multidisciplinary
diagnostic, therapeutic, and long-term fol-
low-up plan for these patients (Table III).
We hope that this plan will provide a viable
treatment for CS and all other diseases that
may lead to neurogenic bladder disease.
Some limitations of this study should
be acknowledged. First of all, CS is a rare
disease, since the spina bifida multidisci-
plinary clinic was established in 2014, lim-
ited sample-sized patients with CS have
received standardized and comprehensive
treatment at our center. This low sample
size has limited us to only perform a cross-
sectional study and no analytical assessment
was possible. So, the proposed interpreta-
tions and approaches are concluded qualita-
tively, as there was a huge heterogeneity in
terms of the differences in the disease mani-
festations, surgeries that patients received,
and their urological managements. Sec-
ondly, patients enrolled in the study did not
undergo systematic genetic studies. Thirdly,
the follow-up time of patients in this study
was relatively short, and the function of the
lower urinary tract of patients with CS may
change over time. Therefore, long-term fol-
low-up is necessary. Finally, our study did not
research the lower urinary tract symptoms.
Since most of the patients were too young
to receive systematic urination training, we
were unable to assess the lower urinary tract
symptoms, such as urgency or urinary incon-
tinence. In addition, most of the symptoms
were reported by the parent or the caregiver,
which may have introduced an element of
bias.
We conclude that CS has a complex
phenotype. The diagnosis and treatment ne-
cessitates multidisciplinary collaboration.
Urologists play an important role in protect-
ing renal function, preventing progression