Tethered Cord Syndrome in Low Motor Level Children with Myelomeningocele

Sarwark, John F.; Weber, Daniel T.; Gabrieli, Ana Paula T.; McLone, David G.; Dias, Luciano

doi:10.1007/978-4-431-68373-5_27

Cited by 16 publications

(24 citation statements)

References 9 publications

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“…The respective authors of three series evaluating the prevalence of scoliosis and TCS in Data presented as the number of patients exhibiting the symptom, were extracted from Herman et al [7] J Child Orthop (2013) 7:513-519 517 myelomeningocele noted that the prevalence of scoliosis was greater [19][20][21]. Sarwark et al [21] found an incidence of new or progressive scoliosis of 60 % in patients with low level (L3 and below) MMC. In their study of children with lumbar level MMC, McLone et al reported on 43 children (47 %) who had scoliosis as one of their signs of deterioration.…”

Section: Discussionmentioning

confidence: 99%

The spectrum of musculoskeletal problems in lipomyelomeningocele

Segal

Czoch

Hennrikus

et al. 2013

Journal of Children's Orthopaedics

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Purpose Patients with lipomyelomeningocele (LMMC) represent a unique population within the spectrum of spinal dysraphism. The natural history of LMMC remains poorly defined. The description and prevalence of the presenting orthopaedic clinical signs and symptoms for LMMC have been infrequent and often documented only in general terms. The goal of this study is to define the patterns and prevalence of presenting clinical musculoskeletal signs and symptoms in LMMC patients. Methods This study was a retrospective review of charts of all patients identified as having LMMC in our spina bifida clinic. Patient charts with incomplete data or diagnoses other than LMMC were excluded from the analysis. Data collected included age at initial tethered cord release (TCR); repeat TCR; limb length discrepancy; foot deformities; asymmetry of motor and sensory deficits; presence of scoliosis; orthotic needs; assistive devices; functional status. Results We identified 32 patients with LMMC (21 female and 11 male patients). The majority of patients had their primary TCR by B1 year of age (59 %), with 22 and 19 % having primary TCR at ages 1-15 and [15 years, respectively. Fifteen patients had at least one repeat TCR, with ten of these having more than one repeat TCR. A significant relationship was noted between low back/ radicular pain and repeat TCR (p \ 0.001). Ten patients (31%) had a limb length discrepancy of[2.5 cm, and 53 % of patients had asymmetric involvement. Nine patients (28 %) had scoliosis of whom only one required operative treatment. Fifteen patients had foot deformities. Thirteen patients (41 %) had two or more orthopaedic procedures in addition to other neurologic or urologic procedures. Conclusion The presenting musculoskeletal clinical signs and symptoms in patients with LMMC are uniquely different in terms of both pattern and frequency compared to myelomeningocele and other forms of spinal dysraphism. We noted a high prevalence of asymmetrical involvement, a high operative burden, and a high rate of repeat symptomatic tethered cord syndrome requiring TCR. As previously noted by others, TCR in LMMC does not prevent long-term functional deterioration. These findings may be important to our colleagues providing counsel to their patients with LMMC and to their families.

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Section: Discussionmentioning

confidence: 99%

The spectrum of musculoskeletal problems in lipomyelomeningocele

Segal

Czoch

Hennrikus

et al. 2013

Journal of Children's Orthopaedics

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“…The natural course of valgus unstable feet in children with myelomeningocele with respect to foot deformity, function or patient satisfaction has not been reported previously and is not further investigated in this study. The evaluation of corrective foot surgery in myelomeningocele children is further complicated by the neurological deterioration commonly occurring in these patients, often due to tethered cord and subsequent releases [25][26][27][28]. Patient selection and the indications for surgery in this study were based on the clinical judgement of valgus instability of the feet combined with an evaluation of the overall situation of the child, such as walking ability, and not solely on radiographic measurements or any other objective parameter.…”

Section: Discussionmentioning

confidence: 99%

Grice arthrodesis in the treatment of valgus feet in children with myelomeningocele: A 12.8-year follow-up study

Høiness¹,

Kirkhus²

2009

Journal of Children's Orthopaedics

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Purpose Neurological deficit resulting in the lack of motor control in children with myelomeningocele often leads to a valgus position of the feet and ankles, usually in combination with planovalgus and pronation of the forefoot. The purpose of the study was to evaluate long-term patient satisfaction and clinical effects in ambulating children with lumbosacral myelomeningocele after having performed a Grice arthrodesis of a valgus unstable foot. The clinically most relevant radiographic measurements, such as the frontal and lateral talo-calcaneal angles, were used to evaluate the anatomical effects of the surgery, whereas the main research question was to reveal the patient satisfaction and usefulness of the procedure. Methods The modified Grice-Green extraarticular subtalar arthrodesis was performed by the same surgeon on one standing and 22 walking patients (12 female) with lumbosacral myelomeningocele and valgus instability during the period 1985-1999. Twelve patients had bilateral surgery, giving a total of 35 operated feet. The patients attended a thorough check-up at a mean of 12.8 years (standard deviation [SD] ± 3.2, range 7.7-20.2 years) after surgery. The mean age at surgery was 6.6 years (SD ± 1.8) and at follow-up 19.4 years (SD ± 3.8). Functional parameters, such as walking ability, pain and skin problems, and the need for braces and supportive orthopaedic shoes were noted at the follow-up interview. The parents were interviewed along with the patients in order to obtain all of the necessary information. Loaded radiographs in the lateral and frontal planes were taken of both feet and ankles pre-operatively and at follow-up, except for pre-operative radiographs in six patients that were not loaded and, thus, not included, except for the assessment of ankle valgus. Ankle valgus was assessed from lateral and frontal views of the ankle on a scale from grade 0 to grade 3 according to Malhotra. Frontal and lateral talo-calcaneal angles were measured for the assessment of subtalar varus or valgus. Lateral talo-first-metatarsal (Meary's) angles were measured to investigate the longitudinal arches of the feet. Results The mean lateral talo-calcaneal angle was reduced significantly (P \ 001) from 55.1°(SD ± 8.9) to 38.8°(SD ± 8.1). The mean frontal talo-calcaneal angle was reduced from 24.7°(SD ± 9.7) pre-operatively to 16.6°(SD ± 6.3) at follow-up (P \ 0.001). The mean lateral talo-first-metatarsal angle improved significantly from -16.1°(SD ± 24.7) pre-operatively to 0.9°(SD ± 15.1) at follow-up (P = 0.0015). The calcaneal pitch did not change significantly. In general, ankle valgus worsened during follow-up time, but not significantly (P = 0.113). The visual analogue scale (VAS) score of patient satisfaction improved significantly from 3.7 (SD ± 1.7) prior to surgery to 7.2 (SD ± 1.5) at follow-up (P \ 0.005). Nineteen patients (83%) were satisfied with the surgery and would thus recommend the procedure. Conclusions Based on the radiological findings and patient satisfaction, the patients participa...

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“…6,13 Theoretically, the spinal column lengthening that occurs with scoliosis correction can result in neurological deterioration. Little has been published on whether patients should undergo untethering prior to correction of their scoliosis.…”

Section: Discussionmentioning

confidence: 99%

“…3,9,16 Risk factors for the development of scoliosis in these patients include motor level, ambulatory status, and last intact laminar arch, with scoliosis developing in almost 100% of patients with a thoracic motor level. 9,16 In addition, virtually all patients with MM radiographically demonstrate a tethered spinal cord, 6,13 although only 10-30% exhibit symptoms. 1,2,15 This occurs due to a difficulty in closing the dura mater around the neural placode to keep it bathed in CSF and prevent adherence to the dura.…”

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confidence: 99%

A patient with myelomeningocele: is untethering necessary prior to scoliosis correction?

Samdani¹,

Fine²,

Sagoo³

et al. 2010

FOC

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Object Tethering of the spinal cord is thought to increase the chance of neurological injury when scoliosis correction is undertaken. All patients with myelomeningocele (MM) are radiographically tethered, and untethering procedures carry significant morbidity risks including worsening neurological function and wound complications. No guidelines exist as regards untethering in patients with MM prior to scoliosis correction surgery. The authors' aim in this study was to evaluate their experience in patients with MM who were not untethered before scoliosis correction. Methods Seventeen patients with MM were retrospectively identified and 1) had no evidence of a clinically symptomatic tethered cord, 2) had undergone spinal fusion for scoliosis correction, and 3) had not been untethered for at least 1 year prior to surgery. The minimum follow-up after fusion was 2 years. Charts and radiographs were reviewed for neurological or shunt complications in the perioperative period. Results The average age of the patients was 12.4 years, and the following neurological levels were affected: T-12 and above, 7 patients; L-1/L-2, 6 patients; L-3, 2 patients; and L-4, 2 patients. All were radiographically tethered as confirmed on MR imaging. Fourteen of the patients (82%) had a ventriculoperitoneal shunt. The mean Cobb angle was corrected from 82° to 35°, for a 57% correction. All patients underwent neuromonitoring of their upper extremities, and some underwent lower extremity monitoring as well. Postoperatively, no patient experienced a new cranial nerve palsy, shunt malfunction, change in urological function, or upper extremity weakness/sensory loss. One patient had transient lower extremity weakness, which returned to baseline within 1 month of surgery. Conclusions The study results suggested that spinal cord untethering may be unnecessary in patients with MM who are undergoing scoliosis corrective surgery and do not present with clinical symptoms of a tethered cord, even though tethering is radiographically demonstrated.

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Tethered Cord Syndrome in Low Motor Level Children with Myelomeningocele

Cited by 16 publications

References 9 publications

The spectrum of musculoskeletal problems in lipomyelomeningocele

The spectrum of musculoskeletal problems in lipomyelomeningocele

Grice arthrodesis in the treatment of valgus feet in children with myelomeningocele: A 12.8-year follow-up study

A patient with myelomeningocele: is untethering necessary prior to scoliosis correction?

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