Spinal stenosis surgery in pediatric patients with achondroplasia

Sciubba, Daniel M.; Noggle, Joseph C.; Marupudi, Neena I.; Bagley, Carlos A.; Bookland, Markus J.; Carson, Benjamin S.; Ain, Michael C.; Jallo, George I.

doi:10.3171/ped.2007.106.5.372

Cited by 37 publications

(56 citation statements)

References 33 publications

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“…Also consistent with previous findings of safety and efficacy of decompression in the child with achondroplasia, 10,11 our data showed significant benefit in terms of reduced postoperative symptomatology (up to 57.5% overall) and limited adverse postoperative outcomes in terms of reduced likelihood of revision surgery (odds ratio = 0.08) with fewer incidences of new-onset neurogenic findings (Tables 1-3). Although new-onset findings J Pediatr Orthop Volume 30, Number 5, July/August 2010 Achondroplasia: Lumbar Stenosis/Surgical Decompression affected 2 patients who eventually underwent revision surgery, 5 patients who did not undergo revision surgery also developed new-onset findings.…”

Section: Discussionsupporting

confidence: 92%

Surgical Decompression for Lumbar Stenosis in Pediatric Achondroplasia

Baca

Abdullah

Ting

et al. 2010

Journal of Pediatric Orthopaedics

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Section: Discussionsupporting

confidence: 92%

Surgical Decompression for Lumbar Stenosis in Pediatric Achondroplasia

Baca

Abdullah

Ting

et al. 2010

Journal of Pediatric Orthopaedics

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“…Only 1 shunt has been inserted in a child with achondroplasia in the past 20 years at The HSC. The practice of decompressive surgery for cervicomedullary compression, however, appears to be increasing with the recognition of the incidence of sudden death in children with achondroplasia, 33,40 a greater ability to document central apnea on sleep studies, and good-quality MR imaging of this region on which stenosis and spinal cord signal change can be identified. There are many unique features to consider when contemplating surgery in the child with achondroplasia, and these need to be assessed to minimize unnecessary procedures and operative morbidity.…”

Section: Discussionmentioning

confidence: 99%

“…The cohort of children with achondroplasia seen in this quaternary referral center is a superselected group, and is unlikely to represent the general pediatric population of patients with achondroplasia. 33 Symptomatic spinal canal stenosis in our pediatric series was only identified in 3 patients. One patient underwent surgery prior to 1988 and was not followed in our clinic, so for the period in which we have an accurate total number of children with achondroplasia in whom followup was ongoing, 2 (1.2%) of 162 underwent decompressive surgery.…”

Section: Discussionmentioning

confidence: 99%

“…33 Immediate postoperative morbidity is reported to relate primarily to durotomy and subsequent CSF leakage. Delayed instability is also a major consideration, with some authors advocating fusion in pediatric patients following decompression 33 if there is evidence of a thoracolumbar kyphosis at the surgical level(s). Delayed instability was evident in 1 of our 3 patients who underwent decompression.…”

Section: Discussionmentioning

confidence: 99%

“…2,21,28,29,35,41 In a recently reported pediatric spine surgery series from The JHU, 20% of the 44 patients had undergone shunt insertion. 33 We provide the example of a 6-year-old child with achondroplasia who had moderate ventriculomegaly and a head circumference on the 75th percentile for individuals with the disease, who was not treated with shunt placement, and who remains well and has had a normal developmental outcome to this point (Fig. 6).…”

Section: Discussionmentioning

confidence: 99%

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Neurosurgical implications of achondroplasia

King¹,

Vachhrajani²,

Drake³

et al. 2009

PED

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Object Achondroplasia is the most common form of human short-limbed dwarfism. The pediatric neurosurgeon is frequently required to treat children with achondroplasia who have hydrocephalus, cervicomedullary compression (CMD), and spinal canal stenosis. Accordingly, the authors have reviewed the experience of neurosurgery in children with achondroplasia at The Hospital for Sick Children. Methods The medical records and neurosurgery database at The Hospital for Sick Children were searched to identify all children with achondroplasia who underwent at least 1 neurosurgical procedure between 1956 and the present. Results Twenty-nine children with achondroplasia underwent 85 surgical procedures: 52 for CSF diversion in 12 patients, 20 for CMD in 18 patients, 8 for spinal disorders in 4 patients, and 5 for miscellaneous purposes in 4 patients. The CSF shunts were placed almost exclusively before 1990 and were associated with a significant number of complications. Patients undergoing CMD did very well, with only 1 patient failing to improve clinically. Conclusions This review provides a historical perspective on the evolution of treatment of pediatric patients with achondroplasia. The use of CSF diversion procedures, formerly fraught with complications, is now rare following the realization of the natural history of CSF space enlargement in these patients. Cervicomedullary compression is more commonly recognized due to better imaging. Central apnea is now better detected by routine sleep studies. Spine surgery, although rare, requires evaluation of both spinal stenosis and instability. These patients are best evaluated by a multidisciplinary team.

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Natural history of achondroplasia: A retrospective review of longitudinal clinical data

Okenfuss

Moghaddam

Avins

2020

American J of Med Genetics Pt A

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There are limited data on the longitudinal frequency and severity of the symptoms and complications of achondroplasia. We undertook a retrospective electronic chart review of 114 patients to develop a more thorough understanding of the lifetime impact of achondroplasia. Craniocervical stenosis (involving the foramen magnum with or without cervical vertebrae C1 and/or C2) was noted in nearly 50% of patients with craniovertebral junction imaging; however, corrective decompression surgery was only needed in 6% of patients. No children in our cohort died at 4 years of age or under. Kyphosis was present in most patients but usually resolved in early childhood. Cervical and lumbar stenosis were diagnosed in children and adults while, genu varum, elbow contractures, and radial head dislocations were identified during childhood. Central sleep apnea and obstructive sleep apnea were present in children, while the diagnosis of obstructive sleep apnea was shown to recur in adulthood. Cardiovascular risk factors were present in only 7% of patients. A range of mental health disorders were identified, with most diagnoses being made before 18 years of age. Our data show that achondroplasia has a significant impact on patients' physical health, and complications continue to be reported and require intervention throughout patients' lifetimes. This highlights the need for continuous support beyond pediatric care, by adult care clinicians experienced with managing the long-term complications of achondroplasia.

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Spinal stenosis surgery in pediatric patients with achondroplasia

Cited by 37 publications

References 33 publications

Surgical Decompression for Lumbar Stenosis in Pediatric Achondroplasia

Surgical Decompression for Lumbar Stenosis in Pediatric Achondroplasia

Neurosurgical implications of achondroplasia

Natural history of achondroplasia: A retrospective review of longitudinal clinical data

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