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Purpose: Closed spina bifida (CSB) is rare in prenatal literature, and various lesions are grouped under this broad nosological entity CSB, leading to confusing and misleading prognostic conclusions. Methods: This is a retrospective observational cohort study of prenatally detected CSB cases using two-dimensional ultrasound, complemented by three-dimensional ultrasonography and foetal MRI in indicated cases, from October 2014 to October 2021 in a tertiary-level single centre. Results: The most common upper vertebral level of CSB was lumbar in 66.6% (10/15). The sub-classification of lesions based on prenatal ultrasound showed an agreement in 53% of the cases. Sixty percent had associated abnormalities identified postnatally, the most common being anorectal malformation seen in 33.3%. On postnatal follow-up, 46.6% had bowel incontinence and bladder dysfunction, and 33.3% developed lower limb deformities. Conclusions: All CSBs do not have a uniformly favourable prognosis. The prognosis of CSB depends on the pathological type, the presence of associated abnormalities and the management. [ABSTRACT FROM AUTHOR]

The incidence and severity of urinary tract infections (UTIs) due to spina bifida is poorly understood in Malaysia. Tethering of the spinal cord is a pathological fixation of the cord in the vertebral column that can result in neurogenic bladder dysfunction and other neurological problems. It occurs in patients with spina bifida, and the authors of this study sought to investigate the impact of untethering on the urological manifestations of children with a tethered cord, thereby consolidating a previously known understanding that untethering improves bladder and bowel function. Demographic and clinical data were collected via an online questionnaire and convenient sampling techniques were used. A total of 49 individuals affected by spina bifida participated in this study. UTIs were reported based on patients' observation of cloudy and smelly urine (67%) as well as urine validation (60%). UTI is defined as the combination of symptoms and factoring in urine culture results that eventually affects the UTI diagnosis in spina bifida individuals irrespective of CISC status. Furthermore, 18% of the respondents reported being prescribed antibiotics even though they had no history of UTI. Therefore, indiscriminate prescription of antibiotics by healthcare workers further compounds the severity of future UTIs. Employing CISC (73%) including stringent usage of sterile catheters (71%) did not prevent patients from getting UTI. Overall, 33% of our respondents reported manageable control of UTI (0–35 years of age). All individuals below the age of 5 (100%, n = 14) were seen to have improved urologically after the untethering surgery under the guidance of the Malaysia NTD support group. Improvement was scored and observed using KUB (Kidneys, Ureters and Bladder) ultrasound surveillance before untethering and continued thereafter. Spina bifida individuals may procure healthy bladder and bowel continence for the rest of their lives provided that neurosurgical and urological treatments were sought soon after birth and continues into adulthood. [ABSTRACT FROM AUTHOR]

Purpose: Controversy exists regarding the role preoperative urodynamic study for asymptomatic closed spinal dysraphism as it has failed to reveal the benefit in surgical decision and expectation of urological outcomes. We explore the relationship between preoperative videourodynamic study and postoperative urological outcomes after toilet training completed, focusing on their capability of spontaneous voiding. Methods: We retrospectively reviewed the data of 181 patients who underwent preventive spinal cord untethering and followed at least till the completion of toilet training. Before untethering, patients underwent preoperative videourodynamic study. Postoperative voiding function was evaluated in 3 phases: (1) till postoperative 6 months, (2) till the completion of toilet training, and (3) 2 years after toilet training. Changing distribution of emptying pattern at each period was described. Also, relevance to preoperative urodynamic parameters on spontaneous voiding and urinary continence after toilet training was assessed. Results: Spinal lipoma and low lying conus were found in 145 (80%) and 128 patients (70.7%), respectively. Spontaneous voiding was found in 125 (69.1%), 164 (90.6%), and 162 patients (89.5%) at postoperative 6 months, till the toilet training, and 2 years after toilet training, respectively. Videourodynamics helped to clarify the presence of vesicourethral synergy. This was correlated with spontaneous voiding at postoperative 6 months and better urinary continence after 2 years of toilet training. Conclusions: Eventual spontaneous voiding was achieved till toilet training in 90% patients following preventive untethering. Those showing preoperative vesicourethral synergy was associated with faster achievement of spontaneous voiding and better urinary continence when they enter elementary school. [ABSTRACT FROM AUTHOR]

Purpose: Secondary tethered cord syndrome (TCS) can be diagnosed with signs of progressive deterioration in urological or neuro‐orthopedic systems following primary untethering surgery. Though urological deterioration is a common secondary TCS manifestation, a paucity of diagnostic criteria makes diagnoses challenging. A detailed description of urological deterioration may help diagnose secondary TCS. Thus, the clinical and urodynamic features of the current secondary TCS cases were described. Materials and Methods: Fifty‐one patients who had undergone reuntethering for secondary TCS experienced improvement or stabilization of progressive problems. Moreover, their clinical and videourodynamic changes were longitudinally described. Results: Loss of postoperative spontaneous voiding was the first urological secondary TCS sign for those who could void spontaneously. Urological problems mostly occurred during elementary school (6–12 years). Major urological presentations were recalcitrant urinary tract infection or urinary incontinence. Follow‐up videourodynamic studies revealed typical changes, from acontractile bladder to overactive and low‐complaint bladders. While detrusor overactivity did not always occur during the progression, detrusor sphincter dyssynergia was always present in all patients with urological deterioration. All patients postoperatively showed significant urodynamic improvement regardless of preoperative bladder dysfunction. This included four cases of restoring spontaneous voiding. Nine patients experienced newly appearing nonprogressive neuro‐orthopedic complications despite their urological improvement. Conclusions: Urological deterioration should prompt secondary TCS suspicion, and changes in clinical patterns and videourodynamic studies helped diagnose it. However, reuntethering can effectively address urological problems at the cost of some neuro‐orthopedic functions in some patients. [ABSTRACT FROM AUTHOR]

Purpose: To compare the short- and long-term outcomes after surgical treatment of tethered cord syndrome with and without electrophysiological monitoring. Method: We collected the preoperative data of 102 tethered cord surgeries of 91 patients. We compared the outcomes regarding the presence of intraoperative electrophysiology, the types of surgeries and the preoperative neurological condition. We also analysed the long-term outcomes in the cases of 69 patients. Results: We found that intraoperative electrophysiology can reduce the perioperative surgical risk significantly (from 9.4 to 2.9%, p < 0.001), and electrophysiology is also beneficial in avoiding long-term progression in 88.7% (p = 0.03341). Conclusion: Tethered cord surgeries are safe and effective. With the use of intraoperative electrophysiology, the risk of postoperative worsening is as low as 2.9%, and long-term progression can be avoided in the majority of the patients. [ABSTRACT FROM AUTHOR]

Introduction: We conducted a retrospective study of children with lumbosacral lipomas treated in the Department of Pediatric Neurosurgery hospital Timone Enfants in the last 20 years. We selected patients with lipomas of the conus medullaris who underwent preventive surgery. 86 ,4 % of the patients remained asymptomatic during the follow-up. Worse results were observed in children older than one year old at surgery with transitional lipoma, with the conus medullaris was below L5 and when the resection had been partial. In view of the results, the authors recommend the preventive surgery before the first year of life under neurophysiologic monitoring in order to perform a total near-total resection with a neural plate reconstitution. [ABSTRACT FROM AUTHOR]

Background: Tethered cord syndrome occurs when there is abnormal tension on the distal spinal cord, which limits its elevation as patients grow. This results in stretching of the neural elements and microvasculature, resulting in both direct and ischemic injury.[7] Animal studies suggest that impairment of oxidative metabolic pathways may contribute to neuronal injury.[7] Associated conditions include myelomeningocele, lipomyelomeningocele, intraspinal lipomas, diastematomyelia, thickened/fatty filum terminale, and trauma.[2] Tethering may be asymptomatic or result in a variety of symptoms including lower extremity weakness/sensory deficits, bowel/bladder dysfunction, scoliosis, pes cavus, and back/leg pain.[6] Early surgical intervention has been shown to improve outcomes and may be performed prophylactically or to prevent symptom progression.[1,3] More specifically, retrospective studies demonstrate that surgical intervention in patients under the age of 2 years is associated with improved outcomes.[5] In some cases, detethering may result in clinical improvement. Case Description: We present a case of a 6-month-old male with a low-lying conus medullaris, lumbar syrinx, mildly abnormal urodynamic studies, and asymmetric utilization of his lower extremities observed during the evaluation of a Y-shaped gluteal cleft. He underwent elective spinal cord detethering via the safe and effective, minimally invasive technique described in the video. The patient's parents gave informed consent for treatment and video recording. Institutional review board approval was deemed unnecessary. Conclusion: Given the variety of surgical techniques used for cord detethering, this video may assist other surgeons in developing techniques that require little to no compromise of the developing bony spinal column while achieving sufficient release of the spinal cord. [ABSTRACT FROM AUTHOR]

Objective: An objective clinical assessment tool whose accuracy and reproducibility can be validated is essential for the initial evaluation, selection for surgery and surveillance of children with lipomyelomeningocele (LMMC). The aim of this study was to analyse the large number of such tools presently in use and recommend an alternative that could lead to greater uniformity between different series and greater consistency in the assessment of individual patients. Methods: A systematic review of the literature between January 1980 and December 2010 was undertaken and details of how the children in each series were assessed and the degree to which age was taken into account recorded. Results: Thirty-six different assessment tools were used in 40 different publications. None was validated in all aspects. Objective measures were used most in urological assessments but rarely in other domains. Age-specific assessments were used in only 10 % of publications. Conclusion: This study confirmed that the assessment tools for evaluation of children with LMMC are inconsistent, often vague and poorly validated. This compromises the ability of clinicians who care for them to compare studies across centres for both treated and untreated children. We have sought to highlight those criteria which are relevant, measurable and reproducible and which might be combined into an easily applied assessment. [ABSTRACT FROM AUTHOR]

Tethered cord syndrome with spinal lipoma is the most common form of occult spinal dysraphism. For the symptomatic patients, surgical treatment is recommended; however, there are many patients who have not been encouraged to seek medical attention until adulthood, since their symptoms are not severe enough to interfere with their daily activities. We performed pedicle subtraction osteotomy (PSO) to achieve indirect untethering and neural decompression in two senior patients with tethered cord syndrome, who showed deteriorating neurological condition due to coexisting lumbar canal stenosis. Here we report two patients (aged 56 and 60 years) who underwent PSO of L3 or L4. The pain disappeared and the bladder dysfunction recovered significantly after surgery. Complete bone union and untethering were achieved in both patients. PSO is an alternative surgical technique for senior patients with tethered cord syndrome caused by lumbosacral spinal lipoma, when the syndrome occurs along with lumbar canal stenosis. [ABSTRACT FROM AUTHOR]