Abstract Book - 26th ESPU Congress, Joint Meeting with ICCS + SPU + AAP/SOU + AAPU + SFU

13:26 - 13:32

ICCS S5-2

(LO)

URODYNAMIC DIFFERENCES OF JARCHO-LEVIN SYNDROME FROM ISOLATED

SPINA BIFIDA APERTA

S. Kerem OZEL

, Ibrahim ALATAS

, Huseyin CANAZ

and Isik KARALOK

1) Istanbul Bilim University Spina Bifida Research Center, Paediatric Urology, Istanbul, TURKEY - 2) Istanbul Bilim

University Spina Bifida Research Center, Paediatric Neurosurgery, Istanbul, TURKEY - 3) Istanbul Bilim University Spina

Bifida Research Center, Paediatric Radiology, Istanbul, TURKEY

PURPOSE

Jarcho-Levin Syndrome (JLS) is a congenital developmental defect associated with costovertebral malformations, spina

bifida and multisystemic anomalies and overall accepted as a severe form of spina bifida. The aim of this study was to

delineate the urological properties in this rare clinical entity.

MATERIAL AND METHODS

The data of spina bifida patients were reviewed retrospectively. Those patients who had the diagnosis of JLS with

radiological evaluation and randomly selected isolated spina bifida aperta (SBA) patients were included. The

urodynamic results were evaluated. Numeric values were evaluated with one way ANOVA test and nominal values were

assessed with chi-square test.

RESULTS

Tha data of 780 patients in our center were evaluated. There were 35 JLS patients, 23 girls and 12 boys and 50 isolated

SBA patients.. The mean age of JLS patients were 67,6±5,8 months and SBA patients 15,7±25,6 months, respectively.

There were no difference in terms of leak point pressures, postvoid residual urine, bladder capacity, compliance and

gender. However, detrusor activity was reduced in 16% of SBA patients and 25% of JLS patients (p=0,016) and

detrusor sphincter dyssynergia (DSD) was observed in all JLS patients whereas 84% of SBA patients (p=0,01).

CONCLUSIONS

JLS is an anomaly mostly seen in girls. This disease has clinical significance due to more detrusor underactivity and DSD

which may denote a more severe neurourological condition . This presented JLS case series is the largest series in the

literature. Recognition of urological problems in these patients may enable proper clinical evaluation.