Surgical treatment of spinal stenosis in achondroplasia: Literature review comparing results in adults and paediatrics

Al-Rub, Z. Abu; Lineham, Beth; Hashim, Zaid; Stephenson, John; Arnold, Lydia; Campbell, Jennifer; Loughenbury, Peter; Khan, Almas

doi:10.1016/j.jcot.2021.101672

Cited by 6 publications

(4 citation statements)

References 30 publications

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“…A retrospective review by Baca et al [[14]] of achondroplasia patients undergoing lumbar laminectomy alone versus laminectomy and fusion demonstrated unfused patients are 3.5 times more likely to require revision surgery within 2 years of their initial surgery, with 78% of these patients requiring instrumentation during their revision. This larger proportion of pediatric patients developing post-laminectomy kyphosis as compared to their adult counterparts can be attributed to the constant growth of their skeleton leading to dynamic changes [[7]]. Consequently, surgical techniques that preserve spinal anatomy may improve long-term outcomes.…”

Section: Discussionmentioning

confidence: 99%

“…Achondroplasia, an autosomal dominant disorder caused by a gain of function in the fibroblast growth factor receptor 3 (FGR3), is the most common form of short-limb dwarfism in humans, with an incidence of 1 in 25,000-40,000 live births [1][2][3][4][5][6][7]. Primary and secondary skeletal complications relevant to the neurosurgeon that arise secondary to the abnormal linear bone growth in patients with achondroplasia include foramen magnum stenosis causing cervicomedullary compression, hydrocephalus secondary to impaired venous return in the setting of narrowed jugular foramen, thoracolumbar gibbus deformity, and subaxial spinal canal stenosis related to congenitally short pedicles.…”

Section: Introductionmentioning

confidence: 99%

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Midline-Sparing Interapophysealaminar Decompression Technique for Management of Lumbar Stenosis in Pediatric Achondroplasia

Podkovik¹,

Martins²,

Ghanchi³

et al. 2023

Pediatr Neurosurg

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Introduction: Achondroplasia is the most common form of short-limb dwarfism in humans, with an incidence of 1 in 25,000–40,000 live births. About one-third of achondroplasia patients will require operative intervention for lumbar spinal stenosis, generally presenting with progressive neurogenic claudication. The anatomy of the achondroplastic lumbar spine, with shortened pedicles, hypertrophic zygapophyseal joints, and thickened laminae frequently results in the development of multilevel interapophyseolaminar stenosis, while stenosis is usually absent at the mid-laminar levels secondary to pseudo-scalloping of the vertebral bodies. Treatment remains controversial, as disrupting the posterior tension band with complete laminectomies in the pediatric population puts patients at risk of developing post-laminectomy kyphosis. Case Presentation: A 15-year-old girl with achondroplasia presented to clinic with debilitating neurogenic claudication in the setting of multilevel lumbar interapophyseolaminar stenosis. We present a technical case report of her successful surgical treatment using a midline posterior tension band sparing modification to the interapophyseolaminar decompression technique proposed by Thomeer et al. [J Neurosurg. 2002;96(3 Suppl l):292–7]. Conclusion: We demonstrate that an adequate interapophyseolaminar decompression can be achieved through the performance of bilateral laminotomies, bilateral medial facetectomies, and undercutting of the ventral spinous process while preserving supraspinous and interspinous ligament attachments. Given the generally multilevel nature of lumbar stenosis and longer life expectancies of pediatric achondroplasia patients, decompressive surgical interventions must aspire to minimize disruption of spine biomechanics if fusion surgery is to be avoided.

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Section: Discussionmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

Midline-Sparing Interapophysealaminar Decompression Technique for Management of Lumbar Stenosis in Pediatric Achondroplasia

Podkovik¹,

Martins²,

Ghanchi³

et al. 2023

Pediatr Neurosurg

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“…Timely diagnosis and treatment are important in achieving favorable outcomes for individuals with achondroplasia and symptomatic spinal stenosis. 6 Conservative measures through physical therapy, activity modifications, and epidural injections are often the first line of management. 7 When these measures fail, surgical management, such as decompression and fusion, is the mainstay in achieving optimal outcomes.…”

Section: Level Of Evidencementioning

confidence: 99%

Surgery for Spinal Stenosis in Achondroplasia: Causes of Reoperation and Reduction of Risks

Hariharan,

Nugraha,

Huser

et al. 2024

Journal of Pediatric Orthopaedics

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Background: Individuals with achondroplasia are prone to symptomatic spinal stenosis requiring surgery. Revision rates are thought to be high; however, the precise causes and rates of reoperation are unknown. The primary aim of this study is to investigate the causes of reoperation after initial surgical intervention in individuals with achondroplasia and spinal stenosis. In addition, we report on surgical techniques aimed at reducing the risks of these reoperations. Methods: A retrospective review was conducted over an 8-year period of all patients with achondroplasia at a single institution that serves as a large referral center for patients with skeletal dysplasias. Patients with achondroplasia who underwent spinal surgery for stenosis were identified and the need for revision surgery was studied. Data collected included demographic, surgical, and revision details. Fisher exact test was used to determine if an association existed between construct type and the need for revisions. Results: Thirty-three of the 130 (22%) patients with achondroplasia required spinal stenosis surgery. Twenty-four individuals who met the criteria were selected for analysis. The initial spine surgery was at an average age of 18.7 years (SD: 10.1 y). Nine patients (38%) required revision surgeries, and 3 required multiple revisions. Five of 9 (56%) of the revisions had primary surgery at an outside institution. Revision surgeries were due to caudal pseudarthrosis (the distal instrumented segment) (8), proximal junctional kyphosis (PJK) (7), and new neurological symptoms (7). There was a significant association found between construct type and the need for revision (P=0.0111). The pairwise comparison found that short fusions were significantly associated with the need for revision compared with the interbody group (P=0.0180). PJK was associated with short fusions when compared with the long fusion group (P=0.0294) and the interbody group (P=0.0300). Caudal pseudarthrosis was associated with short fusions when compared with the interbody group (P=0.0015). Multivariate logistic regression found long fusion with an interbody was predictive of and protective against the need for revision surgery (P=0.0246). To date, none of the initial cases that had long fusions with caudal interbody required a revision for distal pseudarthrosis. Conclusions: In patients with achondroplasia, the rate of surgery for spinal stenosis is 22% and the risk of revision is 38% and is primarily due to pseudarthrosis, PJK, and recurrent neurological symptoms. Surgeons should consider discussing spinal surgery as part of the patient’s life plan and should consider wide decompression of the stenotic levels and long fusion with the use of an interbody cage at the caudal level in all patients to reduce risks of revision. Level of Evidence: Level IV—Retrospective case series.

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“…The incidence of achondroplasia is one of 25,000–30,000 live births, which makes it a rare disease [ 1 , 2 , 5 – 7 ]. From a neurosurgical viewpoint, achondroplasia patients possess several risks for neurological deterioration [ 5 , 8 – 10 ]. One major problem is spinal cord injury caused by the narrow skull base and foramen magnum.…”

Section: Introductionmentioning

confidence: 99%

Growth patterns of young achondroplasia patients in Korea and predictability of neurosurgical procedures

Lee,

Shim,

Cho

et al. 2023

Orphanet J Rare Dis

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Background Achondroplasia is an autosomal dominant disorder mainly affecting bony growth, typically resulting in markedly short stature. From a neurosurgical viewpoint, patients sometimes develop spinal cord compression at the narrowed foramen magnum and hydrocephalus. This study aims to construct growth references for height, weight, and head circumference (HC) of young achondroplasia patients in Korea and to evaluate the predictability of the necessity and timing of neurosurgical procedures through growth patterns. Methods Growth data were collected from achondroplasia patients who visited our institution between January 2002 and August 2022. First, we constructed percentile growth curves of height, weight, and HC for the patients under 3 years of age with the generalized additive model for location, scale, and shape (GAMLSS). Second, the growth patterns of the patients with hydrocephalus who underwent neurosurgical procedures such as foramen magnum decompression (FMD) and ventriculoperitoneal (VP) shunt were analyzed. Results There were 125 achondroplasia patients, including 67 males and 58 females. Among 125 patients, 46 underwent FMD, and 5 underwent VP shunt. As short stature and macrocephaly were typical characteristics of achondroplasia, the height of achondroplasia was lower than that of the general population, and HC in achondroplasia showed accelerated growth postnatally. There were no significant changes in HC in hydrocephalus patients before they underwent neurosurgical procedures. The influence of hydrocephalus on the growth patterns of HC in achondroplasia seemed insignificant. Conclusion Growth references for height, weight, and HC in young achondroplasia patients were constructed. It is the first report of growth patterns of achondroplasia in Korea. Unlike other pediatric patients, the diagnosis of hydrocephalus and the necessity of neurosurgical procedures are hard to be predicted with HC in achondroplasia. Neuroimaging should be considered for achondroplasia patients with neurological symptoms.

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Surgical treatment of spinal stenosis in achondroplasia: Literature review comparing results in adults and paediatrics

Cited by 6 publications

References 30 publications

Midline-Sparing Interapophysealaminar Decompression Technique for Management of Lumbar Stenosis in Pediatric Achondroplasia

Midline-Sparing Interapophysealaminar Decompression Technique for Management of Lumbar Stenosis in Pediatric Achondroplasia

Surgery for Spinal Stenosis in Achondroplasia: Causes of Reoperation and Reduction of Risks

Growth patterns of young achondroplasia patients in Korea and predictability of neurosurgical procedures

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