Spinal canal stenosis in children with achondroplasia: the role of augmentation laminoplasty—a 15-year single institution experience

Afshari, Fardad T.; Slator, Naomi; Fayeye, Oluwafikayo; Ramakrishnan, Piravin K.; Solanki, Guirish A.

doi:10.1007/s00381-022-05566-9

Cited by 3 publications

(3 citation statements)

References 28 publications

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“…Prior reports in both pediatric and adult populations of achondroplasia and skeletal dysplasias, in general, have reported high rates of complications and revisions. 7,8 This study focuses specifically on the causes of reoperations and tools to potentially mitigate these risks in patients with achondroplasia.…”

Section: Level Of Evidencementioning

confidence: 99%

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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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Section: Level Of Evidencementioning

confidence: 99%

“…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).…”

mentioning

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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“…2). In our series, we have noted a significant reduction in the need to perform an additional spinal fusion procedure over a 15-year follow-up period [15,16].…”

Section: Managementmentioning

confidence: 99%

Myasthenia gravis complicating the surgical management of achondroplasia: a case-based update

et al. 2022

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Background Achondroplasia is the commonest skeletal dysplasia of autosomal dominant inheritance caused by “gain of function” mutations in the fibroblast growth factor receptor 3 (FGFR3) gene. Foramen magnum compression due to accelerated ossification and spinal canal stenosis secondary to reduced interpedicular distance is a hallmark of achondroplasia, driven by G380R nucleotide pair substitution. In severe cases, limb weakness and neurogenic claudication will require surgical decompression. Rarely, a neurological condition may mimic the compressive spinal dysfunction and therefore, non-surgical causes must also be considered in cases of acute neurological deterioration in children with achondroplasia. Myasthenia gravis (MG) is an autoimmune condition resulting in fatigable muscle weakness. There are no reported cases of myasthenia gravis in achondroplasia in the literature. Results We report a child with achondroplasia scheduled for decompressive surgery for severe lumbar canal stenosis presenting with neurological claudication and knee weakness. While waiting for surgery during the COVID-19 pandemic, she developed generalized fatigability and severe weakness raising concerns of acute worsening of cord compression. Urgent investigations ruled out spinal cord compression but identified an unexpected concurrent myasthenia gravis with positive antibodies to acetylcholine receptors. The surgical intervention was postponed averting the potential risk of life-threatening anaesthetic complications. She was successfully managed with a combination of pyridostigmine, steroids, azathioprine, and plasma exchange. Conclusion We report the first case of myasthenia gravis in achondroplasia and review implications in the management.

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Recommendations for neuroradiological examinations in children living with achondroplasia: a European Society of Pediatric Radiology and European Society of Neuroradiology opinion paper

Wright,

Cheung,

Siddiqui

et al. 2023

Pediatr Radiol

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Spinal canal stenosis in children with achondroplasia: the role of augmentation laminoplasty—a 15-year single institution experience

Cited by 3 publications

References 28 publications

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

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

Myasthenia gravis complicating the surgical management of achondroplasia: a case-based update

Recommendations for neuroradiological examinations in children living with achondroplasia: a European Society of Pediatric Radiology and European Society of Neuroradiology opinion paper

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