Total spine MRI for the preoperative evaluation of adolescent idiopathic scoliosis: Part 2 - spinal cord tumors, dysraphisms, diastematomyelia, and vertebral anomalies.

Adolescent idiopathic scoliosis is a commonly encountered condition often diagnosed on screening examination. Underlying, asymptomatic neural axis abnormalities may be present at the time of diagnosis. At certain institutions, total spine MRI is obtained preoperatively to identify these abnormalities. We provide a framework for the radiologist to follow while interpreting these studies. In part 1, we discuss Arnold Chiari malformations, syringomyelia, and the tethered cord. In part 2, we focus on spinal cord tumors, dysraphisms, to include diastematomyelia, and vertebral anomalies.

Endoscopic spinal cord untethering using a 1 cm skin incision technique in pediatrics: a technical case report.

BACKGROUND: Spinal cord untethering by sectioning the filum terminale is commonly performed in tethered cord syndrome patients with minor abnormalities such as filar lipoma, thickened filum terminale, and low conus medullaris. Our endoscopic surgical technique, using the interlaminar approach, allows for sectioning the filum terminale through a very small skin incision. To our knowledge, this procedure has not been previously reported. This is the first case report involving a 1 cm skin incision. CASE PRESENTATION: A 9-month-old male patient was referred to our neurosurgical department due to a coccygeal dimple. MRI revealed a thickened fatty filum. After considering the treatment options for this patient, the parents agreed to spinal cord untethering. A midline 1 cm skin incision was made at the L4/5 vertebral level. Untethering by sectioning the filum terminale was performed by full endoscopic surgery using the interlaminar approach. The procedure was uneventful and there were no postoperative complications. CONCLUSIONS: In terms of visibility and minimizing invasiveness, our surgical technique of using the interlaminar approach with endoscopy allows for untethering by sectioning the filum terminale through a very small skin incision.

Surgical technique of spine-shortening vertebral osteotomy for adult tethered cord syndrome: a case report and review of the literature.

BACKGROUND: Miyakoshi et al. reported three cases of tethered cord syndrome treated by spine-shortening vertebral osteotomy, which provided relief of the patients' symptoms with no complications. Although the details of these cases were described in a previous report, the surgical technique was not thoroughly explained. In the present report, we describe the details of our procedure with reference to a fourth case. CASE PRESENTATION: A 47-year-old Asian woman was admitted to our hospital with a 1-year history of worsening leg numbness and urinary dysfunction. Magnetic resonance imaging revealed a low-lying conus medullaris extending to the level of S2 and surrounded by fat tissue at that level. We diagnosed her condition as adult tethered cord syndrome, and spine-shortening vertebral osteotomy was planned. The target level for the osteotomy was L2. Bilateral pedicle screw implants were placed at L1 and L3 using an anterior-posterior image intensifier. In this procedure, it is essential to use monoaxial screws inserted exactly parallel to the rostral endplates of each vertebral body; this ensures appropriate alignment between the L1 caudal endplate and the L2 osteotomy surface. The upper one-third of the lamina of L2 was resected, and the bilateral two-thirds of the pedicle of L2 was removed with a surgical air drill. After exposure of the lateral side of the L1-2 disc, discectomy was performed with a knife and curette. Following complete discectomy of L1-2, the upper vertebral body of L2 was removed with a surgical air drill. After complete removal of the vertebral body, a straight rod was connected to two screws and applied pressure between the screws. Two polyethylene tapes were applied to the L2 lamina and bilateral rods. CONCLUSION: Spine-shortening osteotomy that preserves the caudal one-third of the pedicle and lamina with one-above and one-below instrumentation successfully reduced the spinal cord tension without causing neural damage.

Syndrome of anterior neural stalk, vertebral abnormality, enteric duplication cyst, and diaphragmatic hernia related to persistent ventral neurenteric canal: report of two cases.

PURPOSE: Abnormalities in notochordal development can cause a range of developmental malformations, including the split notochord syndrome and split cord malformations. We describe two cases that appear related to unusual notochordal malformations, in a female and a male infant diagnosed in the early postnatal and prenatal periods, which were treated at our institution. These cases were unusual from prior cases given a shared constellation of an anterior cervicothoracic meningocele with a prominent "neural stalk," which coursed ventrally from the spinal cord into the thorax in proximity to a foregut duplication cyst. METHODS: Two patients with this unusual spinal cord anomaly were assessed clinically, and with neuroimaging and genetics studies. RESULTS: We describe common anatomical features (anterior neural stalk arising from the spinal cord, vertebral abnormality, enteric duplication cyst, and diaphragmatic hernia) that support a common etiopathogenesis and distinguish these cases. In both cases, we opted for conservative neurosurgical management in regards to the spinal cord anomaly. We proposed a preliminary theory of the embryogenesis that explains these findings related to a persistence of the ventral portion of the neurenteric canal. CONCLUSION: These cases may represent a form of spinal cord malformation due to a persistent neurenteric canal and affecting notochord development that has rarely been described. Over more than 1 year of follow-up while managed conservatively, there was no evidence of neurologic dysfunction, so far supporting a treatment strategy of observation.

Role of Magnetic Resonance Imaging in the Screening of Closed Spinal Dysraphism.

Closed spinal dysraphism (CSD) encompasses a heterogeneous group of spinal cord deformities, which can be accompanied by several types of skin stigmata. These skin stigmata may include inconspicuous features, such as sacral dimples and deformed gluteal clefts, but the association between such mild skin stigmata and CSD is uncertain. This study aimed to reevaluate the indication for magnetic resonance imaging (MRI) in patients with skin stigmata while considering the indication for surgery. A retrospective analysis was conducted on magnetic resonance images of 1255 asymptomatic children with skin stigmata between 2003 and 2015. Skin stigmata classification was based on medical chart data. All subtypes of CSDs except for filum terminale lipomas (FTL), FTL thicker than 2 mm or with low conus medullaris, were considered to meet the surgical indication. CSD prevalence was estimated while considering the surgical indications and assessed after excluding all FTL cases. Skin stigmata were classified into seven types, dimple, deformed gluteal cleft, hair, subcutaneous mass, appendage, discoloration, and protruding bone, and included 1056 isolated and 199 complex ones. The prevalence of CSD was 19.5%, 6.8%, and 0.5% among patients with isolated dimples (n = 881) and 13.9%, 5.8%, and 0.7% among those with isolated deformed gluteal clefts (n = 136) for all cases, surgical indications, and patients without FTL, respectively. Dimples and deformed gluteal clefts had a low prevalence of CSD requiring surgical intervention, and cases without FTL were rare. Asymptomatic patients with mild skin stigmata may not require immediate MRI.

Junctional Neural Tube Defect (JNTD): A Rare and Relatively New Spinal Dysraphic Malformation.

Junctional neurulation completes the sequential embryological processes of primary and secondary neurulation as the intermediary step linking the end of primary neurulation and the beginning of secondary neurulation. Its exact molecular process is a matter of ongoing scientific debate. Abnormality of junctional neurulation-junctional neural tube defect (JNTD)-was first described in 2017 based on a series of three patients who displayed a well-formed secondary neural tube, the conus, that is physically separated by a fair distance from its companion primary neural tube and functionally disconnected from rostral corticospinal control. Several other cases conforming to this bizarre neural tube arrangement have since appeared in the literature, reinforcing the validity of this entity. The clinical, neuroimaging, and electrophysiological features of JNTD, as well as the hypothesis of its embryogenetic mechanism, will be described in this chapter.

Prenatal Diagnosis of Diastematomyelia: a Case Report and Literature Review.

Diastematomyelia is a type of closed spinal dysraphism in which there is splitting of the spinal cord. It is a rare entity that accounts for less than 3% of closed spinal dysraphisms and affects females 1.3 to 6 times more frequently than males. Lesions are usually found in the lower thoracic and upper lumbar regions. It is characterised by two hemicords separated by a bony or cartilaginous spur. In most cases, it is an isolated malformation with a favourable prognosis. However, it may be associated with other abnormalities and sonography is the imaging test par excellence for early prenatal diagnosis. We report a case of diastematomyelia diagnosed by prenatal sonography at 24 weeks' gestation. Amniotic fluid alpha-fetoprotein (AF-AFP) was normal, while amniotic fluid acetylcholinesterase (AF-AChE) was positive. After birth, the diagnosis was confirmed with magnetic resonance imaging (MRI). The anomaly was associated with a spinal lipoma, tethered cord and dermal sinus. A review of all the cases described in the literature to date is carried out.

Dorsal bony spur in pediatric split cord malformations: eight-year experience from a tertiary care hospital.

INTRODUCTION: Dorsal spurs in Type I split cord malformations (SCM-I) are infrequent findings. The pathogenesis of the same is debatable. The objective of this study is to analyze our experience with SCM-I patients having dorsal bony spurs. METHODOLOGY: Retrospective analysis of SCM patients operated from 2010 to 2017 was performed. Their demographic profile, clinic-radiological features, operative findings, and outcome following surgery were recorded. RESULTS: Twenty-four cases of Type I SCM harboring dorsal bony spurs were identified with mean age of 4.96 years. The commonest split site was lumbar, documented in 62.5%. Scoliosis was observed in 58.3%. Pre-operative neurological deficits were seen in 66.6% cases with asymmetric weakness of limbs seen in 16.6%. There was no new neurological deficit observed post-operatively. CONCLUSIONS: This is the largest series of dorsal spurs occurring in SCM, reported in literature so far. Meticulous pre-operative evaluation and imaging are important to identify dorsal spurs for appropriate management and good clinical outcome. Differentiating dorsal spur from ventral spur is important as it has a bearing on surgical approach.

A clinical clue toward recognition of split cord malformation type-I resembling meningocele in neonates.

This is a letter to share the clinical experience we had with neonates born with a specific feature of spina bifida. The lesion is like a meningocele containing skim amount of fluid, with a tiny dimple around the center of the cutaneous lesion which causes inward retraction of the dysplastic coverage. This feature is in favor of accompanied split cord malformation (SCM) type I, and the central nidus is in continuous with the dural sleeve around the bony septum of SCM. By recognition of this clinical clue, surgical repair can be planned to perform with appropriate provision, particularly foreseeing the amount of intraoperative bleeding and duration of anesthesia in the newborn patients.

Demographics, referral patterns, and outcome of patients with neural tube defects in southwestern Uganda.

OBJECTIVE: In Uganda, the burden of neural tube defects (NTDs) poses a serious neurosurgical and public health challenge; however, published data on this patient population are lacking. The authors sought to characterize the population of patients with NTDs, maternal characteristics, and referral patterns of these patients, and to quantify the burden of NTDs in southwestern Uganda. METHODS: A retrospective neurosurgical database at a referral hospital was reviewed to identify all patients with NTDs treated between August 2016 and May 2022. Descriptive statistics were used to characterize the patient population and maternal risk factors. A Wilcoxon rank-sum test and chi-square test were used to determine the association between demographic variables and patient mortality. RESULTS: A total of 235 patients were identified (121 male, 52%). The median age at presentation was 2 days (IQR 1-8 days). A total of 87% of patients with NTDs presented with spina bifida (n = 204) and 31 presented with encephalocele (13%). The most common location of dysraphism was lumbosacral (n = 180, 88%). Of all patients, 80% were delivered vaginally (n = 188). Overall, 67% of patients were discharged (n = 156) and 10% died (n = 23). The median length of stay was 12 days (IQR 7-19 days). The median maternal age was 26 years (IQR 22-30 years). The majority of mothers received only primary education (n = 100, 43%). The majority of mothers reported prenatal folate use (n = 158, 67%) and regular antenatal care (n = 220, 94%), although only 23% underwent an antenatal ultrasound (n = 55). Mortality was associated with younger age at presentation (p = 0.01), need for blood transfusion (p = 0.016) and oxygen supplementation (p < 0.001), and maternal education level (p = 0.001). CONCLUSIONS: To the authors' knowledge, this is the first study to describe the population of patients with NTDs and their mothers in southwestern Uganda. A prospective case-control study is necessary to identify unique demographic and genetic risk factors associated with NTDs in this region.

Prevention of retethering in untethering surgery of a tight filum terminale.

OBJECTIVE: Untethering surgery for a tight filum terminale is a common treatment with considerable efficacy and safety. On the other hand, retethering has been reported to occur. One of the major mechanisms of retethering is adherence of the cut end of the sectioned filum to the midline dorsal dural surface. To prevent retethering, the authors sectioned a filum terminale at the rostral level to the dural incision to keep the distance between the cut end of the sectioned filum and the dural incision and investigated whether this procedure decreased the occurrence of retethering. METHODS: Among the patients who underwent untethering surgery for a tight filum terminale between 2012 and 2016, patients followed up more than 5 years were included in the study. Symptoms, comorbid malformations, preoperative imaging, surgical details, perioperative complications, and long-term outcomes were reviewed retrospectively. RESULTS: Retrospective data for a total of 342 cases were included. The median age at surgery was 11 months (range 3-156 months). Preoperative MRI revealed 254 patients (74.3%) had a low-set conus. There were 142 patients (41.5%) with filar lipoma and 42 patients (12.3%) with terminal cyst. Syringomyelia was found in 29 patients (8.5%). In total, 246 patients (71.9%) were symptomatic and 96 patients (28.1%) were asymptomatic. There were no perioperative complications that required surgical intervention or prolonged hospitalization. The mean postoperative follow-up was 88 months (range 60-127 months). There were 4 patients (1.2%) with retethering who presented with bladder and bowel dysfunction. The mean time from initial untethering to retethering was 54 months (range 36-80 months). All 4 patients underwent untethering surgery, and preoperative symptoms resolved in 3 patients. CONCLUSIONS: The retethering rate after untethering surgery for a tight filum terminale in our series was lower than those in previously reported studies. Sectioning the filum terminale at the rostral level to the dural incision was considered an effective way to prevent retethering.

Is Intervertebral Disc Degeneration a Compensatory Mechanism in Adult Tethered Cord Syndrome?

OBJECTIVE: The purpose of the present study was to evaluate the influence of high nerve tension on lumbar disc degeneration and sagittal morphologies. MATERIALS AND METHODS: A total of 50 young and middle-aged patients (mean age 32.1 ± 7.4 years, 22 men and 28 women) who suffered from tethered cord syndrome (TCS) were retrospectively assessed by two observers. Demographic and radiological data were recorded, including lumbar disc degeneration, disc height index and lumbar spine angle, and were compared with 50 patients (mean age 29.7 ± 5.4 years, 22 men and 28 women) without spinal cord abnormalities. Statistical associations were assessed by student's t-test and chi-square test. RESULTS: Our results showed patients with TCS had a significantly higher rate of lumbar disc degeneration in L1/2, L2/3, L4/5 and L5/S1 than in those without TCS (P < 0.05). Moreover, the rates of multilevel disc degeneration and severe disc degeneration in TCS group were significantly higher than those in control group (P < 0.01). The mean disc height index of L3/4 and L4/5 in TCS group was significantly lower than that in control group (P < 0.05). The mean lumbosacral angle of TCS patients was significantly higher than that of patients without TCS (38.4 ± 3.5°vs. 33.7 ± 5.9°, P < 0.01). CONCLUSIONS: We found a certain correlation between TCS and lumbar disc degeneration and lumbosacral angle enlargement, suggesting that the spine reduces the high tension of the spinal cord through disc degeneration. Therefore, it is speculated that there is a "compromised regulation" mechanism in the body under the condition of neurological abnormalities.

Ventral anchoring of the conus medullaris: a new surgical technique preventing the radiographic recurrence of retethering after surgery for tethered spinal cord.

OBJECTIVE: Retethering is not an unusual operation for a congenital lumbosacral dysraphic spinal lesion. The present study aimed to assess a new surgical technique for preventing retethering. SURGICAL TECHNIQUE: After untethering the spinal cord, the pia mater or scar tissue at the caudal end of the conus medullaris is anchored to the ventral dura mater loosely using 8-0 thread, and the dura mater is closed directly. This technique is called ventral anchoring. RESULTS: Ventral anchoring was performed in 15 patients (aged 5 to 37 years old, average age: 12.1 years old) between 2014 and 2021. All but one patient showed improvement or stabilization of the preoperative symptoms. No complication directly related to the procedure was observed. Postoperative MRI demonstrated that the dorsal subarachnoid space was restored in 14 patients but was undetectable or absent in three patients on follow-up MRI. No patients have experienced a recurrence of the tethered cord syndrome during the follow-up period. CONCLUSION: Ventral anchoring is effective for restoring the dorsal subarachnoid space after untethering the spinal cord. This preliminary study suggested that ventral anchoring has the potential to prevent the postoperative radiographic recurrence of tethered spinal cord in patients with a congenital lumbosacral dysraphic spinal lesion.

The significance of hindbrain herniation reversal following prenatal repair of neural tube defects.

OBJECTIVE: The aim of this study was to determine whether reversal of hindbrain herniation (HBH) on MRI following prenatal repair of neural tube defects (NTDs) is associated with reduced rates of ventriculoperitoneal (VP) shunt placement or endoscopic third ventriculostomy (ETV) within the 1st year of life. METHODS: This is a secondary analysis of prospectively collected data from all patients who had prenatal open repair of a fetal NTD at a single tertiary care center between 2012 and 2020. Patients were offered surgery according to inclusion criteria from the Management of Myelomeningocele Study (MOMS). Patients were excluded if they were lost to follow-up, did not undergo postnatal MRI, or underwent postnatal MRI without a report assessing hindbrain status. Patients with HBH reversal were compared with those without HBH reversal. The primary outcome assessed was surgical CSF diversion (i.e., VP shunt or ETV) within the first 12 months of life. Secondary outcomes included CSF leakage, repair dehiscence, CSF diversion prior to discharge from the neonatal intensive care unit (NICU), and composite neonatal morbidity. Demographic, prenatal sonographic, and operative characteristics as well as outcomes were assessed using standard univariate statistical methods. Multivariate logistic regression models were fit to assess for independent contributions to the primary and secondary outcomes. RESULTS: Following exclusions, 78 patients were available for analysis. Of these patients, 38 (48.7%) had HBH reversal and 40 (51.3%) had persistent HBH on postnatal MRI. Baseline demographic and preoperative ultrasound characteristics were similar between groups. The primary outcome of CSF diversion within the 1st year of life was similar between the two groups (42.1% vs 57.5%, p = 0.17). All secondary outcomes were also similar between groups. Patients who had occurrence of the primary outcome had greater presurgical lateral ventricle width than those who did not (16.1 vs 12.1 mm, p = 0.02) when HBH was reversed, but not when HBH was persistent (12.5 vs 10.7 mm, p = 0.49). In multivariate analysis, presurgical lateral ventricle width was associated with increased rates of CSF diversion before 12 months of life (adjusted OR 1.18, 95% CI 1.03-1.35) and CSF diversion prior to NICU discharge (adjusted OR 1.18, 95% CI 1.02-1.37). CONCLUSIONS: HBH reversal was not associated with decreased rates of CSF diversion in this cohort. Predictive accuracy of the anticipated benefits of prenatal NTD repair may not be augmented by the observation of HBH reversal on MRI.

Prevalence of neural tube defects among pregnant women in Addis Ababa: a community-based study using prenatal ultrasound examination.

PURPOSE: The primary aim of this study was to estimate the prevalence of NTDs at ultrasound examination in communities of Addis Ababa and secondarily to provide a description of the dysmorphology of the NTD cases. METHODS: We enrolled 958 pregnant women from 20 randomly selected health centers in Addis Ababa during the period from October 1, 2018, to April 30, 2019. Of these 958 women, 891 had an ultrasound examination after enrollment, with a special focus on NTDs. We estimated the prevalence of NTDs and compared it with previously reported hospital-based birth prevalence estimates from Addis Ababa. RESULTS: Among 891 women, 13 had twin pregnancies. We identified 15 NTD cases among 904 fetuses, corresponding to an ultrasound-based prevalence of 166 per 10,000 (95% CI: 100-274). There were no NTD cases among the 26 twins. Eleven had spina bifida (122 per 10,000, 95% CI: 67-219). Among the 11 fetuses with spina bifida, three had a cervical and one had a thoracolumbar defect while the anatomical site for 7 was not registered. Seven of the 11 spina bifida defects had skin covering, while two of the cervical lesions were uncovered. CONCLUSION: We report a high prevalence of NTDs among pregnancies in communities of Addis Ababa based on screening by ultrasound. The prevalence was higher than in previous hospital-based studies in Addis, and the prevalence of spina bifida was particularly high.

Comparison of Ultrasonographic Findings between Patients with Tethered Cord Syndrome and Healthy Children.

Background: Tethered cord syndrome (TCS) is a type of occult spinal dysraphism, which necessitates early detection as an essential component of patient management in reducing complications. This study aimed to compare the findings of spinal cord ultrasonography between TCS patients and healthy individuals. Methods: The current study is a case-control study of patients who were admitted to the Akbar and Ghaem Hospitals (Mashhad, Iran) in 2019. The study population comprised 30 children with TCS aged under two years old, and the control group included 34 healthy peers of the same age. The maximum distance of the spinal cord from the posterior canal wall was measured in millimeters using ultrasonography. Demographic and sonographic findings of each participant were recorded in checklists, which were then entered into SPSS software. P values less than 0.05 were considered statistically significant. Results: The study included 30 children with TCS and 34 healthy individuals with a mean age of 7.67±6.39 months. TCS patients had a significantly shorter maximum distance of the spinal cord from the posterior wall of the spinal canal than the control group (1.75±0.62 mm vs. 2.79±0.76, P<0.001). After performing corrective surgery, the TCS patients indicated significant improvement in this interval (1.57±0.54 mm to 2.95±0.49 mm, respectively, P=0.001). Conclusion: In comparison to children without TCS, the spinal cord was substantially closer to the posterior canal wall in TCS patients. However, these outcomes were improved significantly in patients after surgery.