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Purpose
Children with myelomeningocele (MMC) are at increased risk of developing neuromuscular scoliosis and spinal cord re-tethering (Childs Nerv Syst 12:748-754, 1996; Neurosurg Focus 16:2, 2004; Neurosurg Focus 29:1, 2010). Some centers perform prophylactic untethering on asymptomatic MMC patients prior to scoliosis surgery because of concern th...
Similar publications
Myelomeningocele, characterized by extrusion of the spinal cord through a spinal canal defect, is the most common form of spina bifida, often resulting in lifelong disability and significant orthopaedic issues. A randomized controlled trial (RCT) has shown the efficacy of prenatal repair in decreasing the need for shunting and improving motor outco...
Citations
... However, it can also progress rapidly as a result of a tethered spinal cord [114]. A recently published paper suggested that prophylactic untethering surgery prior to scoliosis correction surgery is associated with increased complications, such as surgical site infection and a higher rate of return to the operating room [119]. ...
Purpose
Myelomeningocele (MMC) is one of the representative anomalies in the field of pediatric neurosurgery. During the 50 years of ISPN history, MMC had a tremendous changes in its incidence, clinical management and outcome with advanced understanding of its pathogenesis. We reviewed the changes in MMC during the period.
Methods
We reviewed the literature review and collected our experiences.
Results
During the 50 years, major changes happened in many aspects of MMC including incidence, pathoembryogenesis, folate deficiency, prevention, prenatal diagnosis, mode of delivery, treatment policy with ethical considerations, clinical treatment including fetal surgery, latex allergy, retethering, management outcome, multidisciplinary team approach, and socioeconomic and family issues.
Conclusions
There was a great advance in the management and research of MMC during the 50 years. It is a monumental achievement of pediatric neurosurgeons and colleagues of the related fields.
... Orthopedic consequences may arise from MMC involving various parts of the body, including feet, spine, and hip [9,10]. Several orthopedic methods are used to manage these disabilities with variable success [11][12][13][14], and we will give a brief introduction regarding them in this "Background" section. ...
... Ten out of the 26 included studies included corrective operations of the spine related to scoliosis in 5 studies [10,13,21,43,51], kyphosis in 3 studies [20,21,47], and lumbar lordosis in 2 studies [20,21]. [20]. ...
... Despite releasing untethered cord procedures, Altiok et al. reported bladder control impairment [51]. According to the study by Goldstein et al., prophylactic spinal cord untethering before scoliosis correction surgeries improved the urologic function of 1% of the MMC patients, which is not promising [10]. Sileo et al. indicated postnatal surgical repairs on neonates with MMC, 10.9% of whom were diagnosed with hydronephrosis at the follow-up, and 81.8% with a neuropathic bladder [57]. ...
Background
Myelomeningocele (MMC) is the most common and severe form of spina bifida and imposes a significant burden on patients and the healthcare system. Recently, the multidisciplinary management of MMC has become popular. Herein, we aimed to review the orthopedic management, outcomes, and complications of the of patients with MMC eyeing a multidisciplinary approach.
Methods
We searched PubMed and EMBASE to find relevant studies published before August 2020. All studies that included clinical management of MMC patients and published earlier than 2000 were considered for review on the condition that they reported at least one orthopedic intervention and the rate of complications. We excluded review articles, case reports, case series, letters, commentaries, editorials, and conference abstracts. The primary and secondary goals of our review were to report the outcomes and complication rates of multidisciplinary management for MMC patients.
Results
Twenty-six studies included data for the management of 229,791 patients with MMC and were selected. Sixteen studies reported multidisciplinary management in addition to orthopedic management. From those, 11 (42.31%) included urologic management, 13 (50%) neurosurgical management, 11 (42.31%) neurologic management, and 5 (19.23%) gastrointestinal management. All studies included postnatal operations and related management. No randomized clinical trial was found in our search.
Conclusion
Orthopedic approaches play a key role in MMC management by alleviating spinal deformities, particularly scoliosis, and hip, foot, and ankle complications. However, the most appropriate management, whether surgical or non-surgical, may vary for different patients, given disease severity and the age of patients.
Graphical abstract
Scoliosis is a common complication of neuromuscular disorders. These patients are frequently recalcitrant to nonoperative treatment. When treated surgically, they have the highest risk of complications of all forms of scoliosis. While recent studies have shown an improvement in the rate of complications, they still remain high ranging from 6.3 to 75% depending upon the underlying etiology and the treatment center (Mohamad et al. in J Pediatr Orthop 27:392–397, 2007; McElroy et al. in Spine, 2012; Toll et al. in J Neurosurg Pediatr 22:207–213, 2018; Cognetti et al. in Neurosurg Focus 43:E10, 2017). For those patients who are able to recover from the perioperative period without major complications, several recent studies have shown decreased long-term mortality and improved health-related quality of life in neuromuscular patients who have undergone spine fusion (Bohtz et al. in J Pediatr Orthop 31:668–673, 2011; Ahonen et al. in Neurology 101:e1787–e1792, 2023; Jain et al. in JBJS 98:1821–1828, 2016). It is critically important to optimize patients preoperatively to minimize the risk of post-operative complications and maximize long-term outcomes. In order to do so, one must familiarize themselves with the common complications and their treatment. The most common complications are pulmonary in nature. With reported rates as high as 23–29%, pre-operative optimization should be employed for these patients to minimize the risk of post-operative complications (Sharma et al. in Eur Spine J 22:1230–1249, 2013; Rumalla et al. in J Neurosurg Spine 25:500–508, 2016). The next most common cause of complications are implant related, with 13–23% of patients experiencing an implant-related complication that may require a second procedure (Toll et al. in J Neurosurg Pediatr 22:207–213, 2018; Sharma et al. in Eur Spine J 22:1230–1249, 2013) Therefore optimization of bone quality prior to surgical intervention is important to help minimize the risk of instrumentation failure. Optimization of muscle tone and spasticity may help to decrease the risk of instrumentation complications, but may also contribute to the progression of scoliosis. While only 3% of patients have neurologic complication, significant equipoise remains regarding whether or not patients should undergo prophylactic detethering procedures to minimize those risks (Sharma et al. in Eur Spine J 22:1230–1249, 2013). Although only 1.8% of complications are classified as cardiac related, they can be among the most devastating (Rumalla et al. in J Neurosurg Spine 25:500–508, 2016). Simply understanding the underlying etiology and the potential risks associated with each condition (i.e., conduction abnormalities in a patient with Rett syndrome or cardiomyopathies patients with muscular dystrophy) can be lifesaving. The following article is a summation of the half day course on neuromuscular scoliosis from the 58th annual SRS annual meeting, summarizing the recommendations from some of the world’s experts on medical considerations in surgical treatment of neuromuscular scoliosis.
Introduction:
It remains unclear whether spinal cord untethering is necessary to reduce the chances of neurologic decline in children with myelomeningocele and complex closed spinal dysraphism who undergo thoracolumbar fusion for scoliosis. We sought to determine the neurologic and functional outcomes of children with spinal dysraphism undergoing spinal fusion for scoliosis with and without prophylactic spinal cord untethering.
Methods:
Retrospective, single center review of patients with spinal dysraphism treated with thoracolumbar fusion over the last ten years (2009-2019) with or without prophylactic spinal cord untethering.
Results:
Seventeen patients with myelomeningocele and complex closed spinal dysraphism underwent spinal fusion for scoliosis. Mean age at time of surgery was 13.9 years. Prophylactic spinal cord untethering was performed in 8/17 (47%) patients. The change in Cobb angle after surgery was similar between the two groups (19.4º untethered versus 19.9º no untethering). The ambulatory status was similar between the groups, with 37% of the untethered cohort and 44% of the non-untethered cohort being community or household ambulators. There were no changes in intraoperative motor or sensory evoked potentials in any patient during fusion surgery. No patient had a change in motor level or ambulatory status after scoliosis surgery.
Conclusions:
Our data suggest that prophylactic spinal cord untethering in children with spinal dysraphism undergoing thoracolumbar fusion for scoliosis may not be necessary in patients with moderate curvatures. Our conclusions are limited by the small sample size. A larger review of registry data may yield more powerful conclusions on the necessity of prophylactic spinal cord untethering in this patient population.
Numerous articles have reported the presence of intraspinal anomalies in children with early onset scoliosis (EOS) and have found varying effect of neurosurgical intervention on the risk of spinal deformity progression. However, no study to date has performed a detailed analysis of the implications of intraspinal anomalies on scoliotic development and the implications of neurosurgical intervention on curve progression in children with EOS. This article reviewed the relevant information as it pertains to children < 10 years of age with scoliosis associated with tethered cord, syringomyelia, and Arnold-Chiari (Chiari) malformations. The influence of aspect of the spinal anomaly of spinal deformity is reviewed as well as the identification of risk factors for curve progression following neurosurgical intervention.
»: Globally, the prevalence of myelomeningocele, the most common subtype of spina bifida, is 0.2 to 6.5 per 1,000 live births. In the U.S., adults account for >67% of the overall population with spina bifida.
»: With an estimated prevalence of up to 50%, scoliosis is one of the most common and severe orthopaedic conditions in patients with myelomeningocele.
»: The variable effects that scoliosis can have on an individual, the comorbidities associated with progressive scoliosis, and the risks associated with spine surgery call for a strong partnership and care coordination between medical and surgical teams to deliver a patient-centered approach.
»: A coordinated, structured, planned, and incremental team approach can help individuals achieve the overall goals of functionality and independence, as well as successful transition to adulthood.
»: Teams should consider a patient's social determinants of health (e.g., poverty or language barriers) and the effect of scoliosis on quality of life before proceeding with spinal deformity correction.
Myelomeningocele is a congenital anomaly characterized by a segmental defect in the posterior vertebral elements, an exposed malformed spinal cord, and a related level of paralysis leading to developmental deformities. Additional lesions in the brain or spinal cord occur with variable frequency and severity; they represent other causes for neurologic deterioration and orthopedic deformities. Early onset scoliosis, common in children with myelomeningocele, may be classified as either congenital (due to vertebral malformations) or developmental (due to one or more of the associated neurological lesions). Causes of preventable progressive spinal deformity must be diagnosed and treated promptly. Orthotic treatment may be used selectively to improve seating balance and, in some cases, slow deformity progression. Surgical treatment—challenged by the numerous comorbidities of myelomeningocele—is reserved for scoliosis that causes, or is predicted to cause, disability. Due to numerous comorbidities, an extensive preoperative evaluation is required and the technique must be individualized to the child’s function and highly variable anatomic characteristics. Low profile pedicle screws permit fixation in the dysraphic portion of the spine. Experience with growth-friendly surgery is limited; the results are moderate deformity corrections, limited growth, and high complication rates. Selective use is advised.KeywordsMyelomeningoceleCongenital scoliosisHydrocephalusChiari type II malformationTethered spinal cordHydromyeliaEarly-onset scoliosisGrowth-friendly spinal surgerySpinal orthosis
OBJECTIVE
The aim of this study was to compare the radiological and clinical outcomes of early-onset scoliosis (EOS) patients with or without intraspinal anomalies (IAs) managed with growing rods (GRs), and to evaluate the safety of the GR technique in EOS patients with untreated IAs.
METHODS
EOS patients undergoing GR placement between August 2008 and July 2017 were retrospectively reviewed. Patients with untreated IAs were classified into the EOS+IA group, and those without IAs into the EOS−IA group. The radiographic parameters including Cobb angle of the major curve, T1–S1 height, and apical vertebral translation were measured, and a detailed assessment of the neurological status was performed at each visit.
RESULTS
Seventy-six patients with EOS (32 boys, 44 girls) with an average age of 6.5 ± 2.3 years at initial surgery satisfied the inclusion and exclusion criteria, including 28 patients in the EOS+IA group and 48 patients in the EOS−IA group. The radiographic measurements were comparable between groups preoperatively, postoperatively, and at the latest follow-up. One patient in the EOS+IA group experienced sensory deficit in a unilateral lower extremity after initial surgery, and an intraoperative neurophysiological monitoring event was observed in a patient in the EOS−IA group. No permanent neurological deficit was observed in either group.
CONCLUSIONS
EOS patients with and those without IAs had comparable clinical and radiological outcomes of the GR technique. Repeated lengthening procedures may be safe for EOS patients with untreated IAs.
