RESUMO
BACKGROUND: Complex spinal deformities necessitate surgical interventions that may intervene with intrathecal injections in patients with spinal muscular atrophy (SMA). This study aimed to determine the effect of spinal deformity correction surgery on nusinersen administration. METHODS: Pediatric patients with SMA, operated by a single surgeon, either via magnetically controlled growing rod (MCGR) or definitive fusion (DF) with skip instrumentation, were evaluated retrospectively in terms of safety and feasibility of intrathecal injections. Patients' and their parents' perspectives were evaluated through a questionnaire regarding any shift in the setting of injections. RESULTS: Fourteen patients with 15 spinal surgeries (10 MCGR and 5 DF) were included. Eleven patients received intrathecal treatment both before and after the surgery. Preoperative (n=3) and postoperative (n=9) fluoroscopic guidance was required leading to a shift in the application settings in 6 patients. Of 106 preoperative injections, 15% required fluoroscopy and 2% required anesthesia. Postoperatively, of 88 injections, 73% required fluoroscopy and 26% required anesthesia. No patients discontinued intrathecal injections due to technical difficulties associated with the spinal surgery. CONCLUSIONS: This study demonstrates that spinal surgery does not prevent safe and successful intrathecal nusinersen injections. In the DF group, the skip instrumentation technique provided access to interlaminal space for intrathecal injections. In either surgical group, no further auxillary approach was required. Modifications in the injection technique require an institutional approach, and concerns of patients and their families should be addressed accordingly. LEVEL OF EVIDENCE: IV.
Assuntos
Injeções Espinhais , Oligonucleotídeos , Fusão Vertebral , Humanos , Oligonucleotídeos/administração & dosagem , Estudos Retrospectivos , Masculino , Feminino , Pré-Escolar , Criança , Fusão Vertebral/métodos , Lactente , Fluoroscopia , Atrofias Musculares Espinais da Infância/tratamento farmacológico , Atrofias Musculares Espinais da Infância/cirurgia , Resultado do Tratamento , Atrofia Muscular Espinal/tratamento farmacológico , Atrofia Muscular Espinal/cirurgia , Estudos de ViabilidadeRESUMO
OBJECTIVES: To investigate the clinical outcomes of anterior cervical decompression and fusion (ACDF) surgery for the treatment of Hirayama disease (HD). METHODS: In this study, 15 patients with HD who underwent ACDF operation between March 2022 and March 2023 with complete data were retrospectively analyzed. Following the diagnosis, conservative treatment was ineffective, and thus, disease progression severely affected the quality of life (QOL) of patients. ACDF was performed in the China-Japan Friendship Hospital, and patients were regularly followed up postoperatively. The cervical range of motion (ROM), the anteroposterior and transverse diameter of the spinal cord, and their ratio was measured before and after the operation. The neurologic function of patients before and after the last follow-up was evaluated using the selected brief-Michigan Hand Questionnaire (sb-MHQ), whilst the overall therapeutic effect after the operation was evaluated using Odom's criteria. RESULTS: All patients were followed up for an average of 12 ± 4.5 (6-18) months. Dynamic X-ray displayed that the ROM of cervical vertebrae decreased from 72.73 ± 12.72° (53-97°) to 33.53° ± 10.34° (15-54°) (P < 0.001). Moreover, flexion cervical magnetic resonance imaging (MRI) performed after the operation revealed that spinal cord compression was markedly relieved, and the ratio of the anteroposterior diameter of the spinal cord to the transverse diameter increased from 0.27 ± 0.09 to 0.43 ± 0.03 (P < 0.001). At the last follow-up visit, finger extension tremor symptoms were alleviated, although they did not completely disappear. Contrastingly, muscle atrophy showed no significant improvement. Finally, the sb-MHQ score significantly increased from 17.33±1.76 preoperatively to 24.80±1.78 at the last follow-up (P<0.001). CONCLUSIONS: Our results collectively highlighted the efficacy of ACDF for the treatment of HD. This procedure can limit excessive cervical flexion and repeated compression of the spinal cord during cervical movement and considerably improve upper limb functions.
Assuntos
Vértebras Cervicais , Descompressão Cirúrgica , Fusão Vertebral , Atrofias Musculares Espinais da Infância , Humanos , Atrofias Musculares Espinais da Infância/cirurgia , Masculino , Fusão Vertebral/métodos , Descompressão Cirúrgica/métodos , Vértebras Cervicais/cirurgia , Resultado do Tratamento , Estudos Retrospectivos , Adulto , Adulto Jovem , Feminino , Adolescente , Amplitude de Movimento Articular , Qualidade de Vida , SeguimentosRESUMO
PURPOSE: Hirayama disease, a rare cervical myelopathy in children and young adults, leads to progressive upper limb weakness and muscle loss. Non-invasive external cervical orthosis has been shown to prevent further neurologic decline; however, this treatment modality has not been successful at restoring neurologic and motor function, especially in long standing cases with significant weakness. The pathophysiology remains not entirely understood, complicating standardized operative guidelines; however, some studies report favorable outcomes with internal fixation. We report a successful surgically treated case of pediatric Hirayama disease, supplemented by a systematic review and collation of reported cases in the literature. METHODS: A review of the literature was performed by searching PubMed, Embase, and Web of Science. Full-length articles were included if they reported clinical data regarding the treatment of at least one patient with Hirayama disease and the neurologic outcome of that treatment. Articles were excluded if they did not provide information on treatment outcomes, were abstract-only publications, or were published in languages other than English. RESULTS: Of the fifteen articles reviewed, 63 patients were described, with 59 undergoing surgery. This encompassed both anterior and posterior spinal procedures and 1 hand tendon transfer. Fifty-five patients, including one from our institution, showed improvement post-treatment. Eleven of these patients were under 18 years old. CONCLUSION: Hirayama disease is an infrequent yet impactful cervical myelopathy with limited high-quality evidence available for optimal treatment. The current literature supports surgical decompression and stabilization as promising interventions. However, comprehensive research is crucial for evolving diagnosis and treatment paradigms.
Assuntos
Vértebras Cervicais , Discotomia , Fusão Vertebral , Atrofias Musculares Espinais da Infância , Humanos , Atrofias Musculares Espinais da Infância/cirurgia , Fusão Vertebral/métodos , Vértebras Cervicais/cirurgia , Discotomia/métodos , Masculino , Adolescente , Criança , Resultado do TratamentoRESUMO
BACKGROUND: Hirayama disease (HD) is a characterized by progressive amyotrophy of the upper limbs due to a forward displacement of the cervical dura during neck flexion. METHODS: Unlike other treatment options aiming at preventing cervical flexion (e.g., collar or arthrodesis), laminoplasty with tented duraplasty addresses dural dysplasia. Technically, the procedure consists in enlarging the dural sac by performing an expansile duraplasty that is secured to the yellow ligaments, in association with an open-book laminoplasty. CONCLUSION: Laminoplasty with tented duraplasty is a surgical option addressing the cause of HD to prevent further neurological deterioration while preserving cervical motion.
Assuntos
Laminoplastia , Atrofias Musculares Espinais da Infância , Humanos , Atrofias Musculares Espinais da Infância/cirurgia , Pescoço , Extremidade Superior , Vértebras Cervicais/cirurgiaRESUMO
BACKGROUND: Hirayama disease (HD) is a cervical compressive myelopathy. Anterior cervical discectomy and fusion (ACDF) is identified as the best surgical approach. We evaluated surgical outcomes and factors influencing ACDF in HD. METHODS: Between 2015 and 2019, 126 patients with HD underwent ACDF. Contrast magnetic resonance imaging of the cervical spine in full flexion was performed. Clinical examination and preoperative/postoperative assessment of hand function using Fugl-Meyer assessment, Jebsen-Taylor hand function test, and handheld dynamometry were performed at 3-monthly intervals for 1 year. Surgical outcomes were assessed as per the Odom criteria and Hirayama outcome questionnaire. RESULTS: Age at onset and duration of illness were 12-31 years (mean, 18 ± 2.7) and 1-96 months (32.7 ± 24.4), respectively. All patients had progressive weakness and wasting of the affected limb. Cord atrophy was seen in 97.1%, with epidural detachment and engorgement of the posterior epidural venous plexus in all. All patients underwent ACDF. Of these patients, 54% had an excellent/good outcome and 39% had a satisfactory outcome as per the Odom scale at last follow-up (mean, 44.9 ± 16.5 months) after surgery. Handheld dynamometry showed improvement from preoperative values to 1 year follow-up. Duration of illness and age at onset had a negative correlation and the preoperative Fugl-Meyer score had a positive correlation with improvement. CONCLUSIONS: ACDF resulted in remarkable improvement or stabilization in neurologic deficits in many patients with HD. Because motor disability ensues over time, early surgical intervention during the progressive phase is advocated.
Assuntos
Pessoas com Deficiência , Transtornos Motores , Atrofias Musculares Espinais da Infância , Humanos , Atrofias Musculares Espinais da Infância/cirurgia , Atrofias Musculares Espinais da Infância/diagnóstico , Vértebras Cervicais/diagnóstico por imagem , Vértebras Cervicais/cirurgia , Vértebras Cervicais/patologia , Resultado do TratamentoRESUMO
STUDY DESIGN: A retrospective chart review. OBJECTIVE: The aims of this study were to review pathophysiology, workup, and treatment for Hirayama disease (HD); and to assess outcomes from a single institution. SUMMARY OF BACKGROUND DATA: HD is a rare, painless, cervical myelopathy with distal upper extremity weakness, muscle wasting, and spinal cord atrophy. Disease progression-a consequence of repeat flexion injury-occurs up to 5 years from the initial diagnosis. METHODS: Single-institution review of pediatric HD patients from 2010 to 2020. RESULTS: Patients (n=10 male, n=2 female) presented in the second decade (14-20 y) with painless progressive distal upper extremity weakness and atrophy without sensory loss. Electromyography (n=12) demonstrated denervation in C7-T1 myotomes and flexion/extension magnetic resonance imaging showed focal cord atrophy and anterior displacement of the posterior dura with epidural enhancement in flexion. Treatment included observation and external orthoses (n=9) and anterior cervical discectomy with fusion (n=3). One of the 9 patients managed conservatively experienced further deterioration; no patient who underwent anterior cervical discectomy with fusion progressed. CONCLUSIONS: Patients with HD require a multidisciplinary approach to diagnosis and treatment to preserve function. Treatment is preventive and aims to minimize flexion injury by inhibiting motion across involved joints. First-line management is avoidance of neck flexion and use of rigid orthosis; in cases of failed conservative management and/or rapid clinical deterioration, surgical fixation can be offered.
Assuntos
Atrofias Musculares Espinais da Infância , Humanos , Masculino , Feminino , Criança , Estudos Retrospectivos , Atrofias Musculares Espinais da Infância/cirurgia , Atrofias Musculares Espinais da Infância/diagnóstico , Atrofia Muscular , Imageamento por Ressonância Magnética , América do Norte , Vértebras Cervicais/diagnóstico por imagem , Vértebras Cervicais/cirurgia , Vértebras Cervicais/patologiaRESUMO
Spinal muscular atrophy (SMA) is an autosomal recessive neuromuscular disease characterized by the degeneration of alpha motor neurons in the spinal cord, resulting in progressive proximal muscle weakness and paralysis. SMA is classified into types I-IV based on the age at symptom onset or maximum motor function achieved, and its clinical manifestations vary. SMA affects maxillofacial growth because of muscle dysfunction and results in abnormal maxillofacial morphology. In addition, definitive diagnosis is not often made because of the older onset age and symptoms are rarely severe. Therefore, the possibility of undiagnosed SMA in craniofacial surgeries must be considered. This report described a case of an SMA type III recognized after delayed recovery from the neuromuscular blockade in an orthognathic surgery under general anesthesia.
Assuntos
Anestésicos , Atrofia Muscular Espinal , Bloqueio Neuromuscular , Cirurgia Ortognática , Atrofias Musculares Espinais da Infância , Humanos , Atrofias Musculares Espinais da Infância/diagnóstico , Atrofias Musculares Espinais da Infância/cirurgia , Atrofia Muscular Espinal/diagnóstico , Neurônios MotoresRESUMO
INTRODUCTION: Spinal muscular atrophy (SMA) is a severe, inherited neuromuscular disorder characterized by progressive muscle weakness and atrophy. Cardiac pathology co-existence is reported more frequently in the severely affected patient groups. Structural heart anomalies, mainly septal, and outflow tract defects are commonly observed pathologies. CASE PRESENTATION: We herein report the case of a 23 days-old female patient with the diagnosis of spinal muscular atrophy type 2 complicated with structural heart defects. Successful pulmonary banding, and at the age of 17 months, subsequent surgical atrial and ventricular septal defect closure were performed on our patient who was under treatment of Nusinersen Sodium. Post-operative recovery was uncomplicated. Cardiac assessments were normal, and the patient was neurologically improving in her recent follow-up. CONCLUSION: In the literature, there are no reported cases of successful surgical repair of heart defects in spinal muscular atrophy patients. These patients can be perceived as risky surgical candidates with suboptimal postoperative recovery given the unfavorable disease prognosis of SMA in untreated patients. We report our promising experience with a SMA type 2 patient undergoing a disease-modifying medical treatment. The SMA patients under treatment may be potential candidates for successful surgical cardiac correction given their overall improved prognosis.
Assuntos
Comunicação Interventricular , Atrofia Muscular Espinal , Atrofias Musculares Espinais da Infância , Humanos , Criança , Feminino , Lactente , Atrofias Musculares Espinais da Infância/complicações , Atrofias Musculares Espinais da Infância/tratamento farmacológico , Atrofias Musculares Espinais da Infância/cirurgia , Comunicação Interventricular/cirurgia , SódioRESUMO
Hirayama disease is a motor neuron disease predominantly affecting adolescent males. The identifying feature of Hirayama disease is unilateral forearm and intrinsic muscle weakness that spares the brachioradialis, termed "oblique atrophy." Hirayama disease progresses slowly over several years, followed by an abrupt arrest. The pathognomonic finding is the anterior displacement of the cervical spinal cord with the detachment of the posterior dura. Systematic clinical evaluation and appropriate diagnostic studies are crucial to rule out a variety of compressive, immune-mediated, and genetic disorders. We present a patient with Hirayama disease whose hand function was improved dramatically by a tendon transfer after nearly 3 years without a definitive diagnosis and call attention to the hand surgeon's role in identifying this rare disease to enable timely functional restoration.
Assuntos
Atrofias Musculares Espinais da Infância , Transferência Tendinosa , Masculino , Adolescente , Humanos , Atrofias Musculares Espinais da Infância/cirurgia , Atrofias Musculares Espinais da Infância/diagnóstico , Músculo Esquelético , Atrofia Muscular/cirurgia , Imageamento por Ressonância MagnéticaRESUMO
INTRODUCTION: Finger trembling is a characteristic physical finding in Hirayama disease. Although conservative treatment is recommended to stop disease progression, surgery is optional in some cases. However, the postoperative recovery of finger trembling is scarcely reported. CASE PRESENTATION: A 26-year-old Japanese female patient whose chief complaint was left finger trembling with active finger extension presented at our hospital. Hand weakness without muscle atrophy of the left arm was observed. MRI showed left-side oriented intramedullary signal change with concomitant cord atrophy at C4-5 and C5-6. The CT myelogram (CTM) on flexion showed anterior cord compression and anterior shift of posterior dura matter from C4 to C6. And CTM on extension showed the resolution of both findings. Electrophysiological studies showed active and chronic neuronal damage and preserved motor neuron pool of hand muscle. Since she had exhibited a gradual aggravation of symptoms over a period of 5 years, she underwent anterior cervical discectomy and fusion after careful assessment of both conservative and surgical treatment. Finger trembling recovered soon after surgery. DISCUSSION: Finger trembling is an unfamiliar physical finding in terms of postoperative recovery prediction. Anterior horn cell impairment is postulated as a cause of finger trembling. Postural restoration of spinal cord shape and cerebrospinal fluid around the cord with preserved neural function could facilitate functional recovery.
Assuntos
Compressão da Medula Espinal , Atrofias Musculares Espinais da Infância , Adulto , Vértebras Cervicais/cirurgia , Feminino , Humanos , Compressão da Medula Espinal/etiologia , Atrofias Musculares Espinais da Infância/complicações , Atrofias Musculares Espinais da Infância/diagnóstico , Atrofias Musculares Espinais da Infância/cirurgia , Extremidade SuperiorRESUMO
INTRODUCTION: The purpose of this report is to present a rare case of Hirayama disease (HD) in a patient with a history of late-onset symptomatic vein of Galen aneurysmal malformation (VGAM). This report may provide new insights into the pathophysiology of HD, a rare disorder consisting of insidious onset of unilateral weakness and atrophy of the forearm and intrinsic hand muscles. These symptoms are believed to result from cervical myelopathy affecting the anterior horn cells due to abnormal compressive forces on the spinal cord from adjacent anatomical structures (i.e., dura and/or epidural veins), but this has not been proven. VGAM is a rare congenital cerebral vascular malformation, consisting of high-flow arteriovenous shunting between a persistent median prosencephalic vein and arterial feeders, which most frequently presents in the early neonatal period with congestive heart failure. CASE PRESENTATION: We report the case of an otherwise healthy boy who presented with heart failure due to VGAM at 7 years and subsequently presented at 14 years with left-sided HD. His cervical MRI with neck flexion revealed enlarged epidural veins at the C5-C6 spinal level with anterior, leftward displacement of the posterior dura and spinal cord as well as left hemicord flattening and/or atrophy at this level. He underwent successful surgical treatment by C5-C6-C7 osteoplastic laminotomies and tenting and expansile autologous duraplasty, during which enlarged, engorged epidural veins were confirmed and coagulated. This halted the progression of his left hand weakness and atrophy and allowed significant functional improvement. Postoperative catheter angiogram showed no anatomical connection between the persistent VGAM and the engorged epidural veins, and genetic testing revealed no genetic predisposition of vascular malformations. DISCUSSION/CONCLUSION: In this patient, a combined surgical technique was performed that included epidural venous plexus coagulation with posterior autologous duraplasty and dural fixation using tenting sutures. This combined approach led to a favorable clinical and radiographic outcome with no complications or limitations and has not been previously proposed in the literature, to our knowledge. Although not completely ruled out, we found no angiographic connection or genetic predisposition to suggest there is a pathophysiological link between HD and VGAM.
Assuntos
Veias Cerebrais , Atrofias Musculares Espinais da Infância , Malformações da Veia de Galeno , Humanos , Imageamento por Ressonância Magnética , Masculino , Atrofias Musculares Espinais da Infância/complicações , Atrofias Musculares Espinais da Infância/diagnóstico , Atrofias Musculares Espinais da Infância/cirurgia , Malformações da Veia de Galeno/complicações , Malformações da Veia de Galeno/diagnóstico por imagem , Malformações da Veia de Galeno/cirurgiaRESUMO
Hirayama disease (HD) is a relatively uncommon cause of lower cervical myelopathy. A number of surgical approaches have been described in patients with HD in literature. We reviewed the literature and did a systematic review and meta-analysis of the studies which presented the clinical outcome following surgical intervention in HD. A systematic search of literature was performed with the keywords "Surgical treatment in Hirayama Disease", "Surgical approach in Hirayama Disease" and "Hirayama disease surgery". Data related to clinical outcome following surgery was pooled to calculate the pooled proportion of clinical improvement following anterior and posterior surgical approach. Thirty-four articles met the inclusion criteria and were included in the final review. Altogether, there were 10 types of surgical procedures performed for Hirayama disease. The most commonly described surgical technique was anterior cervical discectomy and fusion with cervical plating. The pooled proportion of patients experiencing clinical improvement following all cervical approaches was 80% (95% confidence interval 76 to 84%). Pooled proportion was maximum for anterior cervical plating (96% (95% confidence interval 62 to 100%)) and minimum for ACDF without plating (57% (95% confidence interval 20 to 88%)). Subgroup analysis based on different surgical approaches was not significant (p value = 0.61). The pooled proportion of patients experiencing clinical improvement following anterior and posterior cervical approach was 80% (95% confidence interval 76 to 84%) and 81% (95% confidence interval 66 to 91%). The indications of surgical treatment in patients with HD include poor patient compliance for neck collar or rapidly progressing severe disease. Good results with more than 80% chances of clinical improvement have been reported following various anterior and posterior surgical approaches. However, there was no significant difference in the pooled outcome of different surgical approaches. Most common technique used in literature is anterior cervical discectomy and fusion with plating.
Assuntos
Doenças da Medula Espinal , Fusão Vertebral , Atrofias Musculares Espinais da Infância , Vértebras Cervicais/cirurgia , Discotomia , Humanos , Doenças da Medula Espinal/cirurgia , Atrofias Musculares Espinais da Infância/cirurgia , Resultado do TratamentoRESUMO
OBJECTIVE: Surgical treatment is widely used to treat patients with Hirayama disease (HD). However, postoperative follow-up with abundant samples is still scarce. This study investigated short-term to midterm clinical outcomes after anterior cervical discectomy and fusion (ACDF) among patients with HD. METHODS: We enrolled 115 patients with HD who had undergone ACDF. Radiographic parameters included cervical lordosis (CL), sagittal vertical axis, segment lordosis (SL), T1 slope (T1S), T1S minus CL, range of motion (ROM), upper/lower adjacent segment ROM, and upper adjacent SL. Electrophysiologic parameters included the maximal compound muscle action potentials (CMAPs) of abductor digit minimi and abductor pollicis brevis, the latency of the ulnar nerve F reaction, and abnormal spontaneous action potentials. Clinical assessment included the selected brief-Michigan Hand Questionnaire and Odom scale. RESULTS: The average age was 19.5 ± 4.5 years. The mean follow-up time was 16.35 ± 9.21 months. CL, SL, and T1S increased, whereas sagittal vertical axis and ROM decreased at the final follow-up (P < 0.001). Upper adjacent SL, upper adjacent ROM, and lower adjacent ROM were stable after ACDF (P > 0.05). The maximal CMAPs of abductor digit minimi and the latency of the ulnar nerve F reaction improved bilaterally (P < 0.05), whereas there was no significance in the maximal CMAPs of abductor pollicis brevis (P > 0.05). Abnormal spontaneous action potentials reduced remarkably. The selected brief-Michigan Hand Questionnaire score increased after surgery (P < 0.001). The Odom scale showed a ratio of 79.1% (excellent and good ratio). CONCLUSIONS: This study showed favorable radiologic, electrophysiologic, and clinical outcomes after ACDF among patients with HD.
Assuntos
Discotomia/métodos , Fusão Vertebral/métodos , Atrofias Musculares Espinais da Infância/cirurgia , Adolescente , Adulto , Vértebras Cervicais/cirurgia , Seguimentos , Humanos , Masculino , Resultado do Tratamento , Adulto JovemRESUMO
BACKGROUND: Children with spinal muscular atrophy (SMA) sustain a progressive reduction in pulmonary function (PF) related to both muscular weakness and the concomitant effects of spinal deformity on the thorax. Growth-friendly instrumentation is commonly utilized for younger patients with scoliosis and SMA to halt the progression of spinal curvature, but its effect on PF in these patients has not previously been investigated. Using the change in Early Onset Scoliosis 24-Item Questionnaire (EOSQ-24) PF subdomain scores, the authors will investigate whether PF improves in patients with SMA after a growth-friendly intervention. METHODS: This was a multicenter retrospective cohort study from 2 international registries of patients with SMA undergoing spinal deformity surgery from 2005 to 2015. Data collected were age, sex, degree of major coronal curve, type of growth-friendly construct, forced vital capacity (FVC), and EOSQ-24 scores at the patient's preoperative, 1-year postoperative, and 2-year postoperative visits. Differences in EOSQ-24 PF scores and FVC between baseline and postoperative assessment were examined by paired tests. RESULTS: A total of 74 patients were identified (mean age, 7.6±2.3 y, major curve 68.1±22.4 degrees, 51.4% female individuals). The mean EOSQ-24 PF scores improved significantly from 70.6 preoperatively to 83.6 at 1 year (P=0.092) and 86.5 at 2 years postoperatively (P=0.020). The scores in patients with rib-based constructs showed steeper increases at 1-year assessments than those in patients with spine-based constructs. The mean paired FVC value decreased from 63.9% predicted preoperatively, to 57.6% predicted at 1 year postoperatively (P=0.035), and 61.9% predicted preoperatively, to 56.3% predicted at 2 years postoperatively (P=0.178). CONCLUSIONS: Patients with SMA who received growth-friendly instrumentation did experience improvements in PF as measured by EOSQ-24 assessing the caregivers' perception. Given the uncertain reliability of PFTs in this young population, EOSQ-24 is an important tool for measuring improvements in health-related quality of life. LEVEL OF EVIDENCE: Level III-retrospective study.
Assuntos
Atrofia Muscular Espinal/complicações , Aparelhos Ortopédicos , Qualidade de Vida , Escoliose , Atrofias Musculares Espinais da Infância , Criança , Desenvolvimento Infantil , Feminino , Humanos , Masculino , Medidas de Resultados Relatados pelo Paciente , Período Pós-Operatório , Reprodutibilidade dos Testes , Testes de Função Respiratória/métodos , Escoliose/etiologia , Escoliose/fisiopatologia , Escoliose/psicologia , Escoliose/cirurgia , Atrofias Musculares Espinais da Infância/fisiopatologia , Atrofias Musculares Espinais da Infância/psicologia , Atrofias Musculares Espinais da Infância/cirurgia , Resultado do TratamentoRESUMO
The objective of this study was to identify the influence of the measurements of "loss of attachment" on the surgical outcome in Hirayama disease (HD). Forty-two patients with HD underwent neutral and cervical-flexion magnetic resonance imaging (MRI) before surgery, and the cervical-flexion MRI was repeated at the 3-month postoperative visit. The longitudinal separation range (LSR) of loss of attachment, the maximum forward-shifting (MFS) degree in the cervical cord, and the relative morphological changes of the cervical cord were measured on MRI. Additionally, all patients underwent handgrip strength (HGS) testing, the Disabilities of the Arm, Shoulder and Hand (DASH) questionnaire, and Medical Research Council scales at the 1-year postoperative visit. Postoperatively, the cervical-flexion X/Y and the LSR decreased significantly at a mean of 94.17±8.65 days (range, 75-110 days) (P<.01), while the cervical-flexion A/B increased (P<.01). Loss of attachment was resolved in fused segments in all 42 patients, but there were 7 instances of residual loss of attachment at adjacent segments. Twenty (47.6%) of the 42 patients' DASH scores decreased at the 1-year postoperative visit. According to the logistic regression analysis, both LSR and MFS were related to the surgical outcomes. Receiver operating characteristic curve analysis found that area under the curve and cutoff values were 0.959 and 4.5, respectively (P<.05) for LSR and 0.782 and 0.215, respectively (P<.05) for MFS. Anterior cervical fusion procedures can effectively improve the abnormal loss of attachment and prevent further progression of HD. The LSR is an important risk factor for the prognosis, and longer fused segments may be required when the LSR is 5 segments or more. [Orthopedics. 2021;44(1):30-37.].
Assuntos
Vértebras Cervicais/diagnóstico por imagem , Vértebras Cervicais/cirurgia , Medula Espinal/diagnóstico por imagem , Fusão Vertebral , Atrofias Musculares Espinais da Infância/diagnóstico por imagem , Atrofias Musculares Espinais da Infância/cirurgia , Adolescente , Progressão da Doença , Feminino , Força da Mão , Humanos , Imageamento por Ressonância Magnética , Masculino , Prognóstico , Curva ROC , Amplitude de Movimento Articular , Fusão Vertebral/métodos , Atrofias Musculares Espinais da Infância/fisiopatologia , Adulto JovemRESUMO
Constant neck flexion has been considered crucial to reducing anastomotic tension after tracheal resection. However, in rare cases, anteflexion can cause cervical cord damage, leading to acute neurological disorders such as tetraplegia. Here, we report a case of 5-year-old boy presenting with acute neurological disorder triggered by a chin-to-chest position over 4 days of deep sedation after cricotracheal resection. The radiological findings would suggest a mechanism similar to Hirayama disease, in which a shift of the dura leads to chronic muscular weakness and atrophy in young populations.
Assuntos
Medula Cervical , Compressão da Medula Espinal , Atrofias Musculares Espinais da Infância , Vértebras Cervicais/diagnóstico por imagem , Vértebras Cervicais/cirurgia , Pré-Escolar , Humanos , Imageamento por Ressonância Magnética , Masculino , Compressão da Medula Espinal/diagnóstico por imagem , Compressão da Medula Espinal/etiologia , Compressão da Medula Espinal/cirurgia , Atrofias Musculares Espinais da Infância/diagnóstico , Atrofias Musculares Espinais da Infância/cirurgiaRESUMO
BACKGROUND: Progressive C-shaped scoliosis with marked pelvic obliquity is common to spinal muscular atrophy (SMA). Reducing the number of procedures with effective deformity control is critical to minimize the risk of pulmonary complications. This study reports the preliminary results of magnetically controlled growing rods (MCGR) in SMA-related collapsing spine deformity. METHODS: Inclusion criteria for this retrospective review were: (1) SMA type 2 patients, (2) early onset scoliosis (below 10 y), (3) collapsing spine deformity with pelvic obliquity, (4) growth-friendly scoliosis treatment with MCGR, (5) in between 2014 and 2017. Extracted data included demographic and clinical information, radiologic parameters, surgical details, and final status of the patients. RESULTS: A total of 11 patients (7 boys, 4 girls) were included. The average age at index surgery was 8.2 (6 to 10) years. Dual MCGR was implanted in 8 patients. In 3 patients, because of curve rigidity and inability of apex to be brought into the stable zone, apical fusion with gliding connectors (convexity) and a single MCGR (concavity) was preferred. Instrumentation included the pelvis in 9 and stopped at the lumbar spine (L3) in 2 patients at the index procedure. Average preoperative deformity of 81.8 degrees (66 to 115) decreased to 29 degrees (11 to 57) postoperatively and was 26 degrees at average 35 months (16 to 59). Pelvic obliquity of 20.9 degrees (11 to 30) decreased to 4.9 degrees (2 to 8) after index surgery and was 6.5 degrees (2 to 16) at the last follow-up. T1-S1 height of 329 mm (280 to 376) after index surgery increased to 356 mm (312 to 390) after 9.2 (4 to 20) outpatient lengthening. No neurologic, infectious, or implant-related complication was recorded. Distal adding-on deformity occurred in 2 patients without initial pelvic fixation.One patient deceased secondary to pneumonia at 16 months after surgery. CONCLUSIONS: Short-term results indicate that MCGR may be a good option in SMA-associated collapsing spine deformity to reduce the burden of repetitive lengthening procedures. The authors recommend apical deformity control in the convex side in case of curve rigidity. In addition, including the pelvis in the instrumentation at index surgery is critical to prevent distal adding-on. LEVEL OF EVIDENCE: Level IV-retrospective case series.
Assuntos
Próteses e Implantes , Escoliose/cirurgia , Atrofias Musculares Espinais da Infância/cirurgia , Algoritmos , Criança , Feminino , Humanos , Vértebras Lombares/cirurgia , Imãs , Masculino , Pelve/cirurgia , Estudos Retrospectivos , Resultado do TratamentoRESUMO
OBJECTIVE: We present the case of a 19-year-old boy who had the classic radiologic and clinical presentations of Hirayama disease treated with anterior cervical diskectomy and fusion (ACDF). We also propose ACDF as promising surgery for the treatment of Hirayama disease. Hirayama disease is an initially progressive disease caused by cervical neck flexion compressing the anterior horns of the lower cervical spinal cord. CASE DESCRIPTION: Our patient presented with an insidious, progressive weakness in his right hand, which had been ongoing for 1 year. Physical examination revealed various degrees of right forearm and hand muscle wasting, and decreased right hand extend power with motor grade Ш. Cervical flexed magnetic resonance imaging showed a spinal cord was being compressed-most noticeably at the level of the fifth cervical vertebral body-and that the dorsal epidural space was abnormally expanding. The patient underwent ACDF at the C4-6 level. The pain and paresthesia improved immediately after the surgery. His motor grade improved immediately after the operation, and there were improvements of a modest reversal of muscle wasting at 1 year postoperatively. CONCLUSIONS: ACDF could be considered as an effective treatment option for the treatment of Hirayama disease. Our patient's finger function improved. Therefore we believe that anterior fusion might be the best choice of treatment.
Assuntos
Discotomia/métodos , Fusão Vertebral/métodos , Atrofias Musculares Espinais da Infância/cirurgia , Adolescente , Vértebras Cervicais/cirurgia , Descompressão Cirúrgica/métodos , Humanos , Masculino , Compressão da Medula Espinal/etiologia , Compressão da Medula Espinal/cirurgia , Atrofias Musculares Espinais da Infância/complicaçõesRESUMO
BACKGROUND: Weakness affects motor performance and causes skeletal deformities in spinal muscular atrophy (SMA). Scoliosis surgery decision-making is based on curve progression, pulmonary function, and skeletal maturity. Benefits include quality of life, sitting balance, and endurance. Post-operative functional decline has not been formally assessed. OBJECTIVE: To assess the impact of scoliosis surgery on motor function in SMA types 2 and 3. METHODS: Prospective data were acquired during a multicenter natural history study. Seventeen participants (12 type 2, 5 type 3 with 4 of the 5 having lost the ability to ambulate) had motor function assessed using the Hammersmith Functional Motor Scale Expanded (HFMSE) performed pre-operatively and at least 3 months post-operatively. Independent t-tests determined group differences based on post-operative HFMSE changes, age, and baseline HFMSE scores. RESULTS: Three participants had minimal HFMSE changes (±2 points) representing stability (mean changeâ=â-0.7). Fourteen participants lost >3 points, representing a clinically meaningful progressive change (mean changeâ=â-12.1, SDâ=â8.9). No participant improved >2 points. There were no age differences between stable and progressive groups (pâ=â0.278), but there were significant differences between baseline HFMSE (pâ=â0.006) and change scores (pâ=â0.001). Post-operative changes were permanent over time. CONCLUSIONS: Scoliosis surgery has an immediate impact on function. Baseline HFMSE scores anticipate post-operative loss as higher motor function scores were associated with worse decline. Instrumentation that includes fixation to the pelvis reduces flexibility, limiting the ability for compensatory maneuvers. These observations provide information to alert clinicians regarding surgical risk and to counsel families.
Assuntos
Transtornos dos Movimentos/fisiopatologia , Procedimentos Ortopédicos , Avaliação de Processos e Resultados em Cuidados de Saúde , Complicações Pós-Operatórias , Escoliose/cirurgia , Atrofias Musculares Espinais da Infância/fisiopatologia , Atrofias Musculares Espinais da Infância/cirurgia , Adolescente , Criança , Pré-Escolar , Feminino , Seguimentos , Humanos , Masculino , Transtornos dos Movimentos/etiologia , Procedimentos Ortopédicos/efeitos adversos , Escoliose/etiologia , Atrofias Musculares Espinais da Infância/complicaçõesRESUMO
BACKGROUND: The aim of this study was to determine the effects of magnetically controlled growing rods surgery (MCGRS) of the scoliosis on pulmonary function in children with neuromuscular scoliosis. METHODS: Seven patients, 85.7% female (mean±SD age: 6.7±1.2 years), with neuromuscular scoliosis (4 SMA II, 2 congenital myopathies and 1 VACTER syndrome), received MCGRS of the spine in the thoraco-lumbar area. The outcome measures were clinical features and pulmonary function (forced vital capacity [FVC] and forced expiratory volume in 1st second [FEV1], were collected. All measures were collected at pre-, post-intervention and follow-up (short-term [0-6 months], mid-term [7-12 months], and long-term [13-24 months]). RESULTS: MCGRS reduced Cobb angle after intervention in 100% in subjects and this result was maintained at 24-month follow-up (all, P=0.001). There was no significant difference in FVC or FEV1 between preoperative and each postoperative period, (P>0.05). Analyses of the correlation coefficients indicated no significant associations between changes in pulmonary function and scoliosis. CONCLUSIONS: The current study found that MCGRS addressed to the scoliosis maintained pulmonary function during long-term follow-up; However, pulmonary function was not associated with scoiosis in children with neuromuscular scoliosis.