Paucity of evidence for urinary tract outcomes in closed spinal dysraphism: a systematic review

Abstract

Objectives• To describe the long-term upper (UUT) and lower urinary tract (LUT) outcomes in patients with closed spinal dysraphism (CSD). • CSD differs from open spinal dysraphism (OSD) by its long asymptomatic course and consequent later diagnosis. The outcome of UUT and LUT function in adults with CSD is relatively unknown. Patients and Methods• A systematic review was performed following the standards of the Preferred Reporting Items for Systematic Reviews and Meta-Analyses (PRISMA) guidelines. An extensive search was made of PubMed and EMBASE.• Included were papers on adults with any form of primary CSD that described bladder and/or kidney function. Only English and Dutch language papers were included. Excluded were papers on patients aged <18 years and patients with secondary tethered cord following childhood OSD repair. International Continence Society terminology was used to describe LUT outcomes. Results• Eventually, only seven studies (90 patients) were included and none of these described renal outcomes. Five of the seven papers were on outcomes after untethering surgery during adulthood.• Urological complaints were present in 54/79 (68.4%) patients.• Urodynamic studies (48 patients available) revealed detrusor underactivity in 46.5% of the evaluated cases, detrusor overactivity in 32.6% and normal findings in 16.3% of the studied patients.• Symptomatic improvement after surgery for tethered cord occurred in 33.3-90.0%, depending on the subgroup studied. Urodynamic improvement rates ranged from 11.1% to 54.5% (but based on three studies with only 24 patients). Success of surgery depended on the time between onset of symptoms and operation, and (sometimes) type of lesion. Conclusions• Few data are available on long-term urological outcomes in adult patients with CSD.• More extensive research on follow-up, including the functional status of the UUT, is recommended.• Based upon the little evidence available, we think life-long follow-up (from birth into adulthood) of those with CSD and neurogenic bladder is advisable.

Comparison of neurogenic lower urinary tract dysfunctions in open versus closed spinal dysraphism: A prospective cross‐sectional study of 318 patients

“…NLUTD is a well‐established leading cause of death, with a major impact on quality of life in patients with myelomeningocele, and has been extensively studied in this population . Conversely, NLUTD in patients with CSD remain elusive, partly because the lack of a consensual definition may have hampered research in this field . Current knowledge of CSD in general is also less advanced (vs OSD) due to lack of appropriate animal models that permit detailed analyses of underlying embryological mechanisms .…”

“…but the pathogenesis of neurological defects in these patients remain uncertain and so does the neuropathophysiology of their NLUTD. Several intrinsic mechanisms have been proposed to explain the neurological dysfunction resulting from CSD such as dysplasia, mass effect and mechanical compression but neurological defects may also arise from an associated tethered cord syndrome which interferes with the normal postnatal ascent of the conus medullaris and by mechanical traction which reduces spinal cord blood flow, leading to impairments in oxidative metabolism . This role of growth and of repeated microtraumata throughout life (ie, during spinal flexion) in the genesis of neurological damage could explain how this congenital condition can be associated with symptoms onset during adulthood.…”

“…This role of growth and of repeated microtraumata throughout life (ie, during spinal flexion) in the genesis of neurological damage could explain how this congenital condition can be associated with symptoms onset during adulthood. An alternative explanation for the worsening of LUTD in adults with CSD might be the effect of ageing on the lower urinary tract as in any healthy individuals but with greater impact than in this latter population as it may disrupt a fragile balance in CSD patients . However, the significant associations between ASIA subscores and several surrogates of NLUTD (ie, Qualiveen, urinary incontinence, self‐catheterization dependence) favor the hypothesis of a neurologically driven lower urinary tract dysfunction in adult CSD patients.…”

Lack of efficacy of an intradural somatic-to-autonomic nerve anastomosis (Xiao procedure) for bladder control in children with myelomeningocele and lipomyelomeningocele: results of a prospective, randomized, double-blind study

“…5 Clean intermittent catheterization (CIC), the use of bladder-active medications (BAMs), and close urological follow-up can mitigate many of the potentially life-threatening complications of neurogenic bladder dysfunction, but patients' quality of life (QOL) is considerably affected by the lifelong burden of these interventions, the lack of control of their ability to empty their bladders, and by incontinence and sexual dysfunction. 4,5,9,10,13,15,21,25,[28][29][30] For more than 100 years, researchers and clinicians have pursued a myriad of surgical remedies to allow reinnervation of the urinary bladder, attempting a variety of nerve repair and transfer techniques in experimental animals and in humans. 11 While there has been great success in the clinical use of nerve transfer techniques to reinnervate peripheral nerves of the extremities, 3 there is no surgical nerve transfer or repair procedure that is widely used in patients with neurogenic bladder dysfunction.…”

Urological presentations of adult primary tethered cord syndrome

“…Our findings on detrusor contractility and bladder compliance explaining the tendency of an unfavorable condition in the earlier symptom onset group align with the results of the study by Keating et al that analyzed 40 children with occult spinal lesions. They described that as the children grew older, the bladder showed a complete denervation pattern like acontractile detrusor . When the detrusor becomes areflexic by parasympathetic decentralization, the detrusor might be transformed to a characteristic histological structure, resulting in a stiffer bladder .…”