Continence outcomes in children with isolated primary tethered cord release
Sahar Eftekharzadeh, MD, MPH, Dawud Hamdan, BS, Christopher J. Long, MD, Jason Van Batavia, MD, Stephen A. Zderic, MD, Dana A. Weiss, MD.
The Children's Hospital of Philadelphia, Philadelphia, PA, USA.
Background: Primary isolated tethered cord (pTC) may be diagnosed at birth due to a sacral dimple or may be identified only later if a child develops neurologic symptoms. pTC may result in urological issues including neurological bladder dysfunction. We hypothesized that there is large variability in the urologic evaluation and outcomes in pTC that may be related to the age at initial surgery. Methods: An IRB-approved single institutional database of patients who have undergone detethering surgery for pTC between 2010 and 2019 was reviewed. Patients who had any more extensive forms of spinal dysraphism (lipomeningocele, myelomeningocele), caudal regression, or any associated anorectal malformations were excluded from the cohort. Only patients who were seen by a urologist during their care were included in the present analysis. The electronic health record was queried for demographics, radiographic studies, urodynamics, and long-term urologic clinical outcomes for patients older than 3 years. Furthermore, multivariate regression was used to identify factors that might correlate with long-term continence. Results: A total of 187 patients with pTC who had undergone detethering were identified; 32 (17%) patients were excluded due to associated anorectal malformation and 73/187 (39%) had never been seen in Urology and so no continence data was available. The remaining 82 patients (35 (43%) females) who had detethering surgery were followed for a median of 3.9 (IQR 1.3, 7.2) years after detethering. The age at detethering was significantly higher in this group compared to those who were not seen by urology (1.8 vs 0.7 years, p<0.001). Thirty (37%) patients were seen by urology pre- and post-detethering, while 8 (10%) have been seen only pre-detethering and 44 (54%) were only seen post-detethering. At least one urological evaluation (i.e. ultrasound, VUDS, VCUG, Uroflowmetry) was obtained for 65/82 (79%) at some point during their care and 6/82 (7%) underwent bladder surgeries after detethering (Table 1). Volitional voiding was present in 69 (84%) patients at the most recent follow-up, while 8 (10%) patients were on clean intermittent catheterization, 1 (1%) had a vesicostomy and the voiding status in the remaining 4 (5%) is unknown. Among patients who were older than 3 years, 34/58 (59%) have a dry interval of longer than 3 hours. Multivariate analysis showed that age at first detethering surgery, age at first urology visit, and the duration of follow-up did not predict urinary continence status. Conclusion: While isolated pTC can lead to neurogenic bladder, the majority of patients in this cohort are continent with volitional voiding. Moreover, there were a large number of patients who were not referred to urology for evaluation, especially when the pTC was released at an early age. Age at detethering was not a predictor of bladder function in those seen in Urology cohort, however further data is needed to know if there are specific features that may predict who will develop a neurogenic bladder after release of pTC.
|Table1: Clinical and imaging details of patients with pTC who were seen by urology (N=82)|
|Pre-detethering urology visit||38 (46%)|
|Post-detethering urology visit||74 (90%)|
|Pre- and post- detethering urology visit||30 (37%)|
|Only seen at urology once||8 (10%)|
|Age at detethering in years, Median (IQR)||1.8 (0.8, 5.9)|
|Age at most recent urology visit in years, Median (IQR)||7.4 (4.2, 11.7)|
|Duration of follow-up by urology in years, Median (IQR)||3.5 (0.4, 6.1)|
|Any urological evaluations||65 (79%)|
|RBUS pre-detethering||11 (13%)|
|Thickened/Trabeculated bladder||0 (0%)|
|Urinary Tract Dilation||1 (9%)|
|RBUS post-detethering||46 (56%)|
|Thickened/Trabeculated bladder||11 (24%)|
|Urinary Tract Dilation||6 (13%)|
|Bladder surgery†||6 (7%)|
|* 1 patient had VUDS performed both pre- and post-detethering. † 1 patient had only vesicostomy, 1 patient had only APV, 2 patients underwent APV and Botox® injection, 1 patient underwent APV with bladder augmentation and Botox® injection, and 1 patient had a bladder augmentation and Botox® injection.|
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