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Key Clinical Message: Early detection of occult spinal dysraphism, such as diastematomyelia with tethered cord, is vital to prevent neurological damage. Though rarely diagnosed in the neonatal period, cutaneous stigmata can aid early identification. Utilizing neonatal ultrasound enables timely management and multidisciplinary intervention. [ABSTRACT FROM AUTHOR]

Objective: To compare cases of dysraphism with ventral tethering of cord with those with dorsal tethering and to find out any differences in the outcome of surgery in them. Methods: We collected the data of 188 consecutively operated tethered cord patients at our institute in the past 7 years and divided them into ventral tethering and dorsal tethering groups. Those that we felt had both dorsal and ventral tethering were excluded. Their preoperative clinical, radiological, and baseline neurophysiological parameters as well as postoperative clinical and radiological parameters were analyzed in a retrospective study. Results: Among the 188 tethered cord patients, 52 (28%) had ventral tethering and 136 (72%) had posterior tethering. Preoperative neurodeficit and cord signal changes as well as absent baseline MEP (of any one muscle) were significantly more associated with ventral tethered cord than the dorsal tethered cord. The neurological deterioration after surgery occurred significantly in the ventral tethered cord group than in the dorsal tethered cord group. Also, the postoperative MRI had more incomplete detethering cases in the ventral group than in the dorsal tethered cord group. Conclusion: Ventral tethered cord is more likely to present with preoperatively neurological deficits. It should be carefully identified in the preoperative MRI, so that the intraoperative difficulties in complete detethering and postoperative deterioration can be anticipated. [ABSTRACT FROM AUTHOR]

After myelomeningocele (MMC) repair, a secondary tethered spinal cord occurs in almost all patients. The tethered spinal cord may result in progressive neurological deterioration and walking disability. This retrospective cohort study aimed to highlight the walking recovery one year after tethered cord release and its relation to the preoperative conus level. We reviewed the medical records at our university hospital from January 2014 to December 2022. The patients who underwent spinal cord untethering following lumbosacral MMC repair were included. We assessed the walking recovery one year after cord release using the modified Benzel scale. Thirty-seven patients met our selection criteria. There were 19 girls (51.4%) and 18 boys (48.6%). Their mean age at presentation was 8.6 years. The preoperative conus vertebral levels ranged between L4 and S3. One year after spinal cord release, 37.8% of the patients regained their walking ability. All the patients whose preoperative conus level was at S2 or S3 regained their walking ability. In contrast, all the patients with preoperative conus levels at L4 or L5 didn't regain their ability to walk. One-third (33.3%) of patients whose conus was at the S1 level regained their walking ability one year after cord release. One year after tethered cord release, 37.8% of the patients regained their walking ability. We found that the walking recovery was statistically associated with the preoperative conus level. A multicenter prospective study is required to support the results of this study. [ABSTRACT FROM AUTHOR]

Spinal ultrasound (US) is an invaluable tool in screening the newborn for congenital spine abnormalities and characterizing them when present. If unrecognized, spinal anomalies can lead to devastating neurological consequences. Knowledge of spine US indications is essential to maximize its utility, and proper technique is critical in obtaining diagnostic images. Knowledge of normal anatomy, pathologic findings, and anatomic variants is paramount in interpretation of images. In this review, we discuss indications for screening US, imaging technique, and illustrate the normal anatomy on US, abnormal findings, and nonpathologic anatomic variants. • Sonographic spine imaging allows visualization of the neonatal spinal cord. • Knowledge of spine ultrasound indications is essential to maximize its utility. • Proper sonographic technique is critical in obtaining diagnostic images. • Early identification of congenital abnormalities can improve patient outcomes. [ABSTRACT FROM AUTHOR]

Key Clinical Message Early detection of occult spinal dysraphism, such as diastematomyelia with tethered cord, is vital to prevent neurological damage. Though rarely diagnosed in the neonatal period, cutaneous stigmata can aid early identification. Utilizing neonatal ultrasound enables timely management and multidisciplinary intervention.

Occult spinal dysraphisms (OSDs) are caused by various defects in the embryogenesis of the spinal cord and represent an obstacle to the ascent of the conus, which allows the conus to pass from the lower levels of the spinal canal to the final position between L1 and L2 during normal foetal life. When an OSD tethers the spinal cord at the lower levels, it can lead to neurological symptoms, better known as tethered cord syndrome. Surgical treatment of OSD is primarily aimed at untethering the spinal cord. In asymptomatic patients, this can protect against the long-term development of neurological deficits. In symptomatic patients, this can halt or limit the progression of existing symptoms. The aim of this study is to examine all paediatric and adult patients diagnosed with OSD and treated in the Department of Neurosurgery at the University Medical Centre Ljubljana during the 5-year period of 2016–2021. All patients diagnosed with OSD during this period were included in the study. Patient characteristics, treatment modalities and outcomes were studied with the aim of describing the differences between the paediatric and adult population and defining the rationality of treating these pathological conditions. We included in the study 52 patients with 64 occult dysraphic lesions. Adults (>18 years old) represented 15/52 (28.8%) of all patients, while 37/52 (71.8%) were children. The most common OSDs were conus lipomas, followed by dermal sinus tracts, filum terminale lipomas and split cord malformations. Surgical treatment was performed in 35/52 (67.3%) cases, while conservative management was chosen in 17/52 (32.6%) cases. The preoperative presence of symptoms was statistically higher in adults than in children (p = 0.0098). Surgery on complex spinal cord lipomas was statistically related to a higher rate of postoperative neurological complications (p = 0.0002). The treatment of OSD is complex and must be based on knowledge of the developmental anomalies of the spine and spinal cord. Successful surgical treatment relies on microsurgical techniques and the use of neuromonitoring. Successful treatment can prevent or limit the occurrence of neurological problems. [ABSTRACT FROM AUTHOR]

Key Clinical Message: Diastematomyelia, tethered cord, intradural extramedullary dermoid tumor and lipomyelomeningocele such disease entities themselves are rare in their own form and concurrent presentation of all those pathological states in a single individual can be considered one of the rarest forms of spinal dysraphism globally. Moreover for prompt management with optimal prognosis needs refined neurosurgical intervention guided by intraoperative neuromonitoring so as to bring about the best quality of life in the patient. [ABSTRACT FROM AUTHOR]

The exoscope has emerged as an efficacious microscope in adult spinal neurosurgery providing improved operative field visibility and surgeon ergonomics. However, outcome data and feasibility are underrepresented in the pediatric literature. We present the largest case series aimed at assessing operative and clinical outcomes in pediatric patients undergoing various exoscope-assisted spinal surgeries. A retrospective review was conducted on all consecutive pediatric (age <18 years) spinal surgeries performed with the use of an exoscope by 3 senior surgeons at a single institution from 2020–2023. Demographics and clinical and operative outcomes were reviewed and analyzed. Ninety-six exoscope-assisted pediatric spine surgeries were performed on 89 unique patients, 41 (42.7%) of which were male. The mean age at surgery was 12 (±5.3) years. Spinal cord detethering (55.8%) was the most common procedure performed. The overall mean operative time for all procedures was 155 (±86) minutes, and the mean estimated blood loss was 18 (±41) mL. The mean length of stay was 5.4 (±6.5) days. There were 14 (14.6%) patients with complications in this cohort. At final follow-up, 64 (83.1%) of symptomatic patients reported neurologic symptom improvement. Using the exoscope in a variety of pediatric spinal surgeries resulted in an acceptable average operative time, estimated blood loss, length of stay, and rate of neurologic symptom improvement. The exoscope appears to be an efficacious option for pediatric neurosurgical spinal procedures. [ABSTRACT FROM AUTHOR]

Total/near-total resection (TR/NTR) of complex lumbosacral lipomas (CSL) is reported to be associated with better long-term functional outcomes and lower symptomatic re-tethering rates. We report our institutional experience for CSL resection in affected children. This is a single-institution, retrospective study. Inclusion criteria consist of patients with CSL with dorsal, transitional and chaotic lipomas based on Pang et al's classification. The study population is divided into 2 groups: asymptomatic patients with a normal preoperative workup referred to as 'prophylactic intent' and 'therapeutic intent' for those with pre-existing neuro-urological symptoms. Primary aims are to review factors that affect post-operative clean intermittent catheterization (CIC), functional outcomes based on Necker functional score (NFS), and re-tethering rates. 122 patients were included from 2000 to 2021. There were 32 dorsal lipomas (26.2 %), 74 transitional lipomas (60.7 %), and 16 chaotic lipomas (13.1 %). 82 % patients achieved TR/NTR. Favourable NFS at 1-year was 48.2 %. The re-tethering rate was 6.6 %. After multivariable analysis, post-operative CIC was associated with median age at surgery (p = 0.026), lipoma type (p = 0.029), conus height (p = 0.048) and prophylactic intent (p < 0.001). Next, extent of lipoma resection (p = 0.012) and the post-operative CSF leak (p = 0.004) were associated with re-tethering. Favourable NFS was associated with lipoma type (p = 0.047) and prophylactic intent surgery (p < 0.001). Our experience shows that TR/NTR for CSL is a feasible option to prevent functional deterioration and re-tethering. Efforts are needed to work on factors associated with post-operative CIC. • Complex lumbosacral spinal cord lipomas (CSL) are challenging conditions. • The role of radical resection of CSL in asymptomatic children remains uncertain. • There is a paucity of data on CSL management from our region of Southeast Asia. • Global collaborative research in CSL is strongly advocated. [ABSTRACT FROM AUTHOR]

Charcot arthropathy is a rare disease in clinic, which is easy to be misdiagnosed and delayed diagnosis. Imaging examination plays a key role in the diagnosis of Charcot arthropathy. It is important to improve the early diagnosis rate and strive for early treatment to improve the quality of life of these patients. Here we reported a rare case of charcot knee (CK) accompanied by tethered cord syndrome and lumbosacral fur sinus, who presented with joint destruction, joint deformity and multiple free bodies and received joint free bodies removal and joint replacement surgery with acceptable short and midterm follow-up results.

Key Clinical Message Diastematomyelia, tethered cord, intradural extramedullary dermoid tumor and lipomyelomeningocele such disease entities themselves are rare in their own form and concurrent presentation of all those pathological states in a single individual can be considered one of the rarest forms of spinal dysraphism globally. Moreover for prompt management with optimal prognosis needs refined neurosurgical intervention guided by intraoperative neuromonitoring so as to bring about the best quality of life in the patient.

Dermal sinus tracts (DSTs) are congenital lesions that connect the cutaneous ectoderm with the underlying neuroectodermal tissues. They are typically midline, solitary lesions. Multiple, and atypically located DSTs have been only rarely described. We present two cases of multiple and laterally located DSTs. The first presented with bacterial meningitis and two tracts in the right buttock, one of which entered the spinal canal through the S3 neural foramen. The second child had three midline lumbar DSTs, one subcutaneous dermoid cyst and one intradural epidermoid cyst. Complete surgical excision was achieved in both cases with good late follow up. Multiple or atypically located DSTs appear to carry the same risks of infection as the more common, midline, single tracts. Complete surgical excision is recommended to avoid the risks of neurological deterioration, in particular due to infection. Incomplete disjunction is the proposed developmental anomaly for DSTs, however the location of the cases presented here requires an alternative explanation.

Occult spinal dysraphisms (OSDs) are caused by various defects in the embryogenesis of the spinal cord and represent an obstacle to the ascent of the conus, which allows the conus to pass from the lower levels of the spinal canal to the final position between L1 and L2 during normal foetal life. When an OSD tethers the spinal cord at the lower levels, it can lead to neurological symptoms, better known as tethered cord syndrome. Surgical treatment of OSD is primarily aimed at untethering the spinal cord. In asymptomatic patients, this can protect against the long-term development of neurological deficits. In symptomatic patients, this can halt or limit the progression of existing symptoms. The aim of this study is to examine all paediatric and adult patients diagnosed with OSD and treated in the Department of Neurosurgery at the University Medical Centre Ljubljana during the 5-year period of 2016–2021. All patients diagnosed with OSD during this period were included in the study. Patient characteristics, treatment modalities and outcomes were studied with the aim of describing the differences between the paediatric and adult population and defining the rationality of treating these pathological conditions. We included in the study 52 patients with 64 occult dysraphic lesions. Adults (>18 years old) represented 15/52 (28.8%) of all patients, while 37/52 (71.8%) were children. The most common OSDs were conus lipomas, followed by dermal sinus tracts, filum terminale lipomas and split cord malformations. Surgical treatment was performed in 35/52 (67.3%) cases, while conservative management was chosen in 17/52 (32.6%) cases. The preoperative presence of symptoms was statistically higher in adults than in children (p = 0.0098). Surgery on complex spinal cord lipomas was statistically related to a higher rate of postoperative neurological complications (p = 0.0002). The treatment of OSD is complex and must be based on knowledge of the developmental anomalies of the spine and spinal cord. Successful surgical treatment relies on microsurgical techniques and the use of neuromonitoring. Successful treatment can prevent or limit the occurrence of neurological problems.

Diastematomyelia is a rare congenital anomaly in which the spinal cord splits into 2 lateral halves. Spinal dermoid cysts are uncommon lesions. Hereby authors present a case of type 2 diastematomyelia in a 5-year-old male patient with concurrent distal cord intramedullary dermoid cyst, diagnosed by MRI and treated surgically., (© 2024 The Authors. Published by Elsevier Inc. on behalf of University of Washington.)

Charcot arthropathy is a rare disease in clinic, which is easy to be misdiagnosed and delayed diagnosis. Imaging examination plays a key role in the diagnosis of Charcot arthropathy. It is important to improve the early diagnosis rate and strive for early treatment to improve the quality of life of these patients. Here we reported a rare case of charcot knee (CK) accompanied by tethered cord syndrome and lumbosacral fur sinus, who presented with joint destruction, joint deformity and multiple free bodies and received joint free bodies removal and joint replacement surgery with acceptable short and midterm follow-up results., Competing Interests: The authors declare that they have no known competing financial interests or personal relationships that could have appeared to influence the work reported in this paper., (© 2024 The Authors.)

Objective: Among patients with a history of prior lipomyelomeningocele repair, an association between increased lumbosacral angle (LSA) and cord retethering has been described. The authors sought to build a predictive algorithm to determine which complex tethered cord patients will develop the symptoms of spinal cord retethering after initial surgical repair with a focus on spinopelvic parameters., Methods: An electronic medical record database was reviewed to identify patients with complex tethered cord (e.g., lipomyelomeningocele, lipomyeloschisis, myelocystocele) who underwent detethering before 12 months of age between January 1, 2008, and June 30, 2022. Descriptive statistics were used to characterize the patient population. The Caret package in R was used to develop a machine learning model that predicted symptom development by using spinopelvic parameters., Results: A total of 72 patients were identified (28/72 [38.9%] were male). The most commonly observed dysraphism was lipomyelomeningocele (41/72 [56.9%]). The mean ± SD age at index MRI was 2.1 ± 2.2 months, at which time 87.5% of patients (63/72) were asymptomatic. The mean ± SD lumbar lordosis at the time of index MRI was 23.8° ± 11.1°, LSA was 36.5° ± 12.3°, sacral inclination was 30.4° ± 11.3°, and sacral slope was 23.0° ± 10.5°. Overall, 39.6% (25/63) of previously asymptomatic patients developed new symptoms during the mean ± SD follow-up period of 44.9 ± 47.2 months. In the recursive partitioning model, patients whose LSA increased at a rate ≥ 5.84°/year remained asymptomatic, whereas those with slower rates of LSA change experienced neurological decline (sensitivity 77.5%, specificity 84.9%, positive predictive value 88.9%, and negative predictive value 70.9%)., Conclusions: This is the first study to build a machine learning algorithm to predict symptom development of spinal cord retethering after initial surgical repair. The authors found that, after initial surgery, patients who demonstrate a slower rate of LSA change per year may be at risk of developing neurological symptoms.

Objective: Spina bifida represents one of the most common birth defects, occurring in approximately 1-2 children per 1000 live births worldwide. The functional level of patients with spina bifida is highly variable and believed to be correlated with the anatomical level of the lesion. The variable clinical picture is well established, but the correlation with anatomical level and intraoperative neuromonitoring (IONM) data has not been investigated. Furthermore, the potential for preserving function beyond the apparent clinical level has also not been investigated. The objective of this research was to determine the presence and level of intraoperative transcranial motor evoked potential (tcMEP) and triggered electromyography (tEMG) responses, and the association of these responses with preoperative clinical function and radiographic data in pediatric cases of complex tethered cord release reoperations., Methods: A single-center retrospective review of pediatric patients with complex spinal dysraphism undergoing detethering reoperations was conducted. Preoperative demographic and clinical data, including the radiographic and clinical level of dysraphism, were collected. IONM, including tcMEPs and tEMG responses, were obtained and compared with preoperative clinical data. Descriptive analysis was performed, by patient for demographics and by case for surgeries performed., Results: In 100% of 21 cases of complex detethering reoperations, representing 20 patients, intraoperative tcMEPs could be generated at (4.8%) or below (95.2%) the level of clinical function. Compared with the preoperative clinical examination, 5 cases (23.8%) demonstrated tcMEP responses that were 1 level below the clinical function level, 11 cases (52.4%) were 2 levels below, and 4 cases (19.0%) were 3 levels below. Overall, 18 of 21 cases showed tEMG responses at or below the level of clinical function; of these, 7 cases (33%) were 1 level below and 3 (14%) were ≥ 2 levels below the clinical function level., Conclusions: The presence of positive stimulation potentials below the level of clinical function in patients with complex spinal dysraphism undergoing detethering reoperations indicates a degree of preserved neuronal connectivity. These findings suggest novel future treatment approaches for these patients, including using devices targeted to stimulation of these neurological pathways.