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Войти в исследования Child's nervous system : ChNS : official journal of the International Society for Pediatric Neurosurgery · 2025
Progressive scoliosis in a pediatric patient with cerebellar ataxia: surgical challenges and literature review.
СтатьяКлинический случайСистематический обзорНазвание и аннотация: Перевод в очереди
Обработка статьи · этапы, ошибки и управление
Загрузка состояния…
Ошибки и ограничения
История · было → стало
Состояние обновляется каждые 5 секунд, пока блок открыт. Процент показывается только там, где известен объём работы. Пауза останавливает продолжение этапа; текущий запрос к источнику может завершиться.
Asunis Emanuela, Vitulli Francesca, Nicotra Roberto, Rubino Alfonso, Cicala Domenico, Cinalli Giuseppe, Colella Gianluca
Аннотация
BACKGROUND: Spinocerebellar ataxia (SCA) and Friedreich's ataxia (FA) frequently give rise to progressive scoliosis, which impairs respiratory and postural function. Although spinal fusion is the most reliable method for correcting deformities, perioperative neurological vulnerability and monitoring failures complicate decision-making. This study combines a systematic review with a detailed pediatric case to clarify current evidence on surgical management, complications, and outcomes.
METHODS: We present a 12-year-old girl who underwent posterior fusion (T4-L4) for presumed adolescent idiopathic scoliosis. Early proximal junctional kyphosis (PJK) prompted planned revision, which was terminated after the intraoperative loss of somatosensory evoked potentials (SSEPs). Subsequent imaging revealed cerebellar atrophy consistent with SCA. Definitive surgery extended the construct to T2 and incorporated a T4 pedicle subtraction osteotomy. Persistent gait and balance deficits were managed with intensive rehabilitation. In parallel, a PRISMA-compliant systematic search of PubMed and Embase (2000-May 2025) identified pediatric studies reporting scoliosis surgery in SCA or FA. Four studies (105 patients) met the inclusion criteria and were analyzed for surgical strategy, neuromonitoring performance, and complications.
RESULTS: In the index case, the unreliability of the SSEP necessitated staged correction; transient unilateral lower limb weakness resolved partially over 6 months. A literature synthesis revealed PJK in 23-25% of patients and high SSEP failure rates (up to 91%); nevertheless, no permanent neurological injuries were reported. Infection (≤ 4%) and pseudarthrosis were infrequent, whereas one cardiopulmonary death occurred. Motor evoked potential (MEP) is seldom employed but may offer greater sensitivity. Disease severity, rather than surgical variables alone, influences long-term functional outcomes.
CONCLUSIONS: Spinal fusion can be performed safely in pediatric SCA/FA patients when meticulous multidisciplinary planning, individualized neuromonitoring strategies, and aggressive postoperative rehabilitation are adopted. Early recognition of underlying ataxia is critical for risk stratification and the timing of intervention. Prospective, longitudinal studies are warranted to refine guidelines and optimize neurological and orthopedic results in this complex population.
METHODS: We present a 12-year-old girl who underwent posterior fusion (T4-L4) for presumed adolescent idiopathic scoliosis. Early proximal junctional kyphosis (PJK) prompted planned revision, which was terminated after the intraoperative loss of somatosensory evoked potentials (SSEPs). Subsequent imaging revealed cerebellar atrophy consistent with SCA. Definitive surgery extended the construct to T2 and incorporated a T4 pedicle subtraction osteotomy. Persistent gait and balance deficits were managed with intensive rehabilitation. In parallel, a PRISMA-compliant systematic search of PubMed and Embase (2000-May 2025) identified pediatric studies reporting scoliosis surgery in SCA or FA. Four studies (105 patients) met the inclusion criteria and were analyzed for surgical strategy, neuromonitoring performance, and complications.
RESULTS: In the index case, the unreliability of the SSEP necessitated staged correction; transient unilateral lower limb weakness resolved partially over 6 months. A literature synthesis revealed PJK in 23-25% of patients and high SSEP failure rates (up to 91%); nevertheless, no permanent neurological injuries were reported. Infection (≤ 4%) and pseudarthrosis were infrequent, whereas one cardiopulmonary death occurred. Motor evoked potential (MEP) is seldom employed but may offer greater sensitivity. Disease severity, rather than surgical variables alone, influences long-term functional outcomes.
CONCLUSIONS: Spinal fusion can be performed safely in pediatric SCA/FA patients when meticulous multidisciplinary planning, individualized neuromonitoring strategies, and aggressive postoperative rehabilitation are adopted. Early recognition of underlying ataxia is critical for risk stratification and the timing of intervention. Prospective, longitudinal studies are warranted to refine guidelines and optimize neurological and orthopedic results in this complex population.