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Beijing Da Xue Xue Bao. Yi Xue Ban =... Aug 2023To summarize the clinical manifestation, classification, and experience of surgical treatment of primary tethered cord syndrome (TCS) in adults.
OBJECTIVE
To summarize the clinical manifestation, classification, and experience of surgical treatment of primary tethered cord syndrome (TCS) in adults.
METHODS
The authors retrospectively analyzed a series of 171 adult patients with primary TCS who were surgically treated under microscope from March 2007 to October 2019. There were 61 males and 110 females whose ages were 18-65 years, with an average age of (39.02±11.81) years. Clinically, the patients presented with various neurological symptoms and signs including lower back and legs pain, reflex changes, sensory disturbances, muscle weakness, and sphincter problems. They were divided into 5 types by clinical manifestations and neuro-imaging features: (1) filum terminale traction in 69 cases, (2) split cord malformation in 21 cases, (3) myelomeningocele in 20 cases, (4) lipomyelomeningocele in 36 cases, and (5) dermal sinus traction in 25 cases. All the patients underwent microsurgery to untether the spinal cord. The patients kept prone position 7 days postoperatively. The Kirollos grading was used to evaluate the outcome of intraoperative untethering. The visual analogue scale (VAS) was used to evaluate the pain, the score of critical muscle strength was used to evaluate the lower extremity motor function, and the Japanese Orthopaedic Association (JOA) sphincter function score was used to evaluate the bladder function.
RESULTS
All of the 171 patients were treated with microsurgery to release the adhesion and cut off the filum terminalis. 61 cases of them received resection of the lesions according to the etiology. All the tethered spinal cord reached Kirollos grade Ⅰ untethering and the dural sac was reconstructed. Other than 5 patients had cerebrospinal fluid leakage and incision laceration and underwent re-suture, there was no surgical complication. The local pain was relieved, the lower limbs weakness or bowel and bladder dysfunction gradually recovered postoperatively. The period of follow-up ranged from 6 months to 12.5 years with an average of (5.62±2.31) years. The neurological function was improved in 153 cases and stable in 18 cases. There was no recurrence of tethered cord be found during the follow-up period.
CONCLUSION
The primary TCS in adulthood could be classified into 5 types by clinical manifestations and neuro-imaging features and surgical treatment should be undertaken in regard to the classifications including dissection and resection of the lesion detethering the spinal cord and reconstruction of the dura sac under microscope. The outcome of surgical treatment is satisfactory.
Topics: Male; Female; Humans; Adult; Middle Aged; Retrospective Studies; Treatment Outcome; Meningomyelocele; Neural Tube Defects; Pain
PubMed: 37534645
DOI: 10.19723/j.issn.1671-167X.2023.04.012 -
BMC Musculoskeletal Disorders Jul 2023Individuals with myelomeningocele (MMC) exhibit neurological deficits below the lesion level involving both motor and sensory functions. Ambulation and functional...
BACKGROUND
Individuals with myelomeningocele (MMC) exhibit neurological deficits below the lesion level involving both motor and sensory functions. Ambulation and functional outcomes in patients offered orthotic management since childhood were investigated.
METHODS
Physical function, physical activity, pain, and health status were assessed in a descriptive study.
RESULTS
Of 59 adults with MMC, aged 18-33 years, 12 were in the community ambulation (Ca), 19 in the household ambulation (Ha), six in the non-functional (N-f), and 22 in the non-ambulation (N-a) groups. Orthoses were used by 78% (n = 46), i.e., by 10/12 in the Ca, 17/19 in the Ha, 6/6 in the N-f, and 13/22 in the N-a groups. In the ten-metre walking test, the non-orthosis group (NO) walked faster than those wearing ankle-foot orthoses (AFOs) or free-articulated knee-ankle-foot orthoses (KAFO-Fs), the Ca group faster than the Ha and N-f groups, and the Ha group faster than the N-f group. In the six-minute walking test, the Ca group walked farther than the Ha group. In the five times sit-to-stand test, the AFO and KAFO-F groups required longer than the NO group, and the KAFO-F group longer than the foot orthosis (FO) group. Lower extremity function with orthoses was higher in the FO than the AFO and KAFO-F groups, higher in the KAFO-F than the AFO group, and higher in the AFO group than in those using trunk-hip-knee-ankle-foot orthoses. Functional independence increased with ambulatory function. Time spent in physical recreation was higher in the Ha than the Ca and N-a groups. There were no differences between the ambulation groups in rated pain or reported health status.
CONCLUSION
The physical function results in persons with MMC improve our understanding of this population's heterogeneity and shed light on the importance of individualized orthotic management. The similarities between the various ambulatory levels in physical activity, pain, and health status may mirror opportunities to achieve equal results regardless of disability level. A clinical implication of the study is that orthotic management is likely to be beneficial for the patient with MMC of which the majority used their orthoses for most time of the day.
Topics: Humans; Adult; Child; Meningomyelocele; Walking; Orthotic Devices; Foot Orthoses; Pain; Health Status; Gait
PubMed: 37400860
DOI: 10.1186/s12891-023-06673-7 -
Journal of Neurosurgery. Spine Oct 2023The authors analyzed the National Spina Bifida Patient Registry (NSBPR), a national registry that tracks the outcomes for people with various forms of spinal dysraphism,...
OBJECTIVE
The authors analyzed the National Spina Bifida Patient Registry (NSBPR), a national registry that tracks the outcomes for people with various forms of spinal dysraphism, to determine the ongoing longitudinal risk for tethered cord release (TCR) among adults with myelomeningocele who had not previously undergone TCR during childhood. The authors also sought to identify the impact of lesion level, ambulation status, and prior treatments for hydrocephalus or Chiari malformations on TCR rates.
METHODS
Adults in the registry who had not previously undergone TCR during childhood were studied. This group was compared with the remaining adults in the registry. The frequency of first-time TCR and time to TCR (using Kaplan-Meier analysis) were determined independently for males and females. Cox proportional hazards analysis identified correlations between sex, best lesion level and ambulation status prior to TCR, and previous treatments for hydrocephalus and Chiari decompression.
RESULTS
Among 967 adults in the NSBPR (422 [43.6%] males and 545 [56.4%] females) who had not undergone TCR during childhood, the authors identified 47 people (4.9%) who underwent their first TCR during adulthood. This study cohort had significantly better mean functional motor levels and ambulation compared with the remaining adult cohort (both p < 0.001). The study group included 35 females (74.5%) and 12 males (25.5%); this sex distribution was significantly different in comparison with the remaining adult cohort (p = 0.016). The Kaplan-Meier curves for first TCR for females and males were significantly different (p = 0.01, log-rank test). TCR rates were correlated with sex (males had decreased risk; OR 0.31, 95% CI 0.16-0.62, p < 0.001), prior treatment for hydrocephalus (those who underwent prior treatment had decreased risk; OR 0.21, 95% CI 0.20-0.42, p < 0.001), and prior treatment for Chiari malformation (those who underwent prior treatment had greater risk; OR 3.84, 95% CI 1.50-9.88, p = 0.005).
CONCLUSIONS
Adults with myelomeningocele who escape childhood without undergoing TCR have an ongoing, albeit decreased, risk for spinal cord tethering requiring TCR. This risk is obviously not due to spinal column growth and therefore must reflect other factors such as dynamic changes in spinal cord health over time. Among people with MMC who underwent their first TCR as adults, females seemed to be overrepresented. Similar to the authors' prior childhood study, people who underwent previous Chiari decompression seemed to be overrepresented, whereas those who underwent previous treatment for hydrocephalus seem to be underrepresented. These novel findings deserve further study.
Topics: Male; Female; Adult; Humans; Meningomyelocele; Spinal Dysraphism; Neural Tube Defects; Arnold-Chiari Malformation; Hydrocephalus; Registries; Receptors, Antigen, T-Cell
PubMed: 37347622
DOI: 10.3171/2023.5.SPINE2328 -
Urology Journal Dec 2023Myelomeningocele is the most severe form of spina bifida. Management of urologic consequences of spina bifida is life long, demanding and costly for both the patient and... (Observational Study)
Observational Study
Early Onset Clean Intermittent Catheterization May Decrease Prevalence and Severity of Urinary Concentration Defects in Myelomeningocele Patients with Neurogenic Bladder: A Retrospective Cohort Study.
PURPOSE
Myelomeningocele is the most severe form of spina bifida. Management of urologic consequences of spina bifida is life long, demanding and costly for both the patient and the public health system. There is a paucity of data in the literature regarding concentration defects and their consequences on this disease. This paper aims to describe retrospectively the effect of early onset clean intermittent catheterization (CIC) in on the severity of urinary concentration defects in myelomeningocele patients with neurogenic bladder in a retrospective observational study.
MATERIALS AND METHODS
In this 10-year retrospective cohort study, children with myelomeningocele were selected with the Convenience sampling method. Demographic characteristics, polyuria index ratio (PIR) defined as 24 hour urine output of each patient divided by the maximum normal urine output of the same patient in a healthy state, and nocturnal polyuria index (NPI) were compared between early starters (< 2 years old) or late starters (≥ 2 years old) groups.
RESULTS
Seven patients who underwent cystoplasty were excluded and 130 patients (63.8% male, 5.4 ± 3.2 years old, 14.3 ± 2.83 Kg, 28.5% early onset CIC) were investigated. PIR > 1 in inset (1.7 ± 0.2 vs. 2.2 ± 0.5, P = 0.021) and outset (1.5 ± 0.32 vs. 2.5 ± 0.7, P = 0.004) were lower in early starters group than in late starters group. NPI in inset (0.2 ± 0.007 vs. 0.32 ± 0.10, P = 0.018) and outset (0.25 ± 0.15 vs. 0.42 ± 0.095, P = 0.007) were also lower in the early starters group. No further adverse events were reported during the follow-up period.
CONCLUSION
Early onset CIC is more effective than late-onset CIC in preserving the urinary ability of kidneys in myelomeningocele patients.
Topics: Child; Humans; Male; Child, Preschool; Female; Meningomyelocele; Intermittent Urethral Catheterization; Urinary Bladder, Neurogenic; Retrospective Studies; Polyuria; Prevalence; Spinal Dysraphism; Urinary Catheterization
PubMed: 37312599
DOI: 10.22037/uj.v20i.7537 -
Ultrasound in Obstetrics & Gynecology :... Nov 2023Prenatal surgery is offered for selected fetuses with open spina bifida (OSB) to improve long-term outcome. We studied the effect of fetal OSB surgery on brain...
OBJECTIVES
Prenatal surgery is offered for selected fetuses with open spina bifida (OSB) to improve long-term outcome. We studied the effect of fetal OSB surgery on brain development using advanced magnetic resonance imaging (MRI) techniques to quantify the volume, surface area and shape of cerebral structures and to analyze surface curvature by means of parameters that correspond to gyrification.
METHODS
We compared MRI data from 29 fetuses with OSB before fetal surgery (mean gestational age (GA), 23 + 3 weeks) and at 1 and 6 weeks after surgery, with that of 36 GA-matched control fetuses (GA range, 21 + 2 to 36 + 2 weeks). Automated super-resolution reconstruction provided three-dimensional isotropic volumetric brain images. Unmyelinated white matter, cerebellum and ventricles were segmented automatically and refined manually, after which volume, surface area and shape parameter (volume/surface area) were quantified. Mathematical markers (shape index (SI) and curvedness) were used to measure gyrification. Parameters were assessed according to lesion type (myelomeningocele vs myeloschisis (MS)), postoperative persistence of hindbrain herniation (HH) and the presence of supratentorial anomalies, namely partial agenesis of the corpus callosum (pACC) and heterotopia (HT).
RESULTS
Growth in ventricular volume per week and change in shape parameter per week were higher at 6 weeks after surgery in fetuses with OSB compared with controls (median, 2500.94 (interquartile range (IQR), 1689.70-3580.80) mm /week vs 708.21 (IQR, 474.50-925.00) mm /week; P < 0.001 and 0.075 (IQR, 0.047-0.112) mm/week vs 0.022 (IQR, 0.009-0.042) mm/week; P = 0.046, respectively). Ventricular volume growth increased 6 weeks after surgery in cases with pACC (P < 0.001) and those with persistent HH (P = 0.002). During that time period, the change in unmyelinated white-matter shape parameter per week was decreased in OSB fetuses compared with controls (0.056 (IQR, 0.044-0.092) mm/week vs 0.159 (IQR, 0.100-0.247) mm/week; P = 0.002), particularly in cases with persistent HH (P = 0.011), MS (P = 0.015), HT (P = 0.022), HT with corpus callosum anomaly (P = 0.017) and persistent HH with corpus callosum anomaly (P = 0.007). At 6 weeks postoperatively, despite OSB fetuses having a lower rate of change in curvedness compared with controls (0.061 (IQR, 0.040-0.093) mm /week vs 0.094 (IQR, 0.070-0.146) mm /week; P < 0.001), reversing the trend seen at 1 week after surgery (0.144 (IQR, 0.099-0.236) mm /week vs 0.072 (IQR, 0.059-0.081) mm /week; P < 0.001), gyrification, as determined using SI, appeared to be increased in OSB fetuses overall compared with controls. This observation was more prominent in fetuses with pACC and those with severe ventriculomegaly (P-value range, < 0.001 to 0.006).
CONCLUSIONS
Following fetal OSB repair, volume, shape and curvedness of ventricles and unmyelinated white matter differed significantly compared with those of normal fetuses. Morphological brain changes after fetal surgery were not limited to effects on the circulation of cerebrospinal fluid. These observations may have implications for postnatal neurocognitive outcome. © 2023 The Authors. Ultrasound in Obstetrics & Gynecology published by John Wiley & Sons Ltd on behalf of International Society of Ultrasound in Obstetrics and Gynecology.
Topics: Female; Pregnancy; Humans; Spina Bifida Cystica; Meningomyelocele; Brain; Fetus; Gestational Age; Magnetic Resonance Imaging; Retrospective Studies; Ultrasonography, Prenatal
PubMed: 37161647
DOI: 10.1002/uog.26244