We report a case of a skeletally immature achondroplastic adolescent with significant thoracolumbar lordosis who presented with neurogenic claudication and urinary incontinence progressing over a 1-year period. She underwent decompressive lumbar laminectomy from T12 to L5 with preservation of the facet joints. Over the ensuing 4 years of follow-up, she developed a progressive thoracolumbar kyphosis that progressed to 105 degrees. She remained neurologically intact and was fully ambulatory, but had severe back pain in the area of the deformity. Because of the severity and progression of her deformity, a combined anterior and posterior fusion and stabilization was required. We present this case and a review of the literature on spinal stenosis in achondroplasia and the complication of post-laminectomy kyphosis.
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http://dx.doi.org/10.3928/01477447-20080201-01 | DOI Listing |
J Pediatr Orthop
January 2025
Specialist Centre for Paediatric Orthopaedics, neuroorthopaedics and Deformity Reconstruction.
Background: Achondroplasia is the most common form of rhizomelic dwarfism. Aside from disproportionally short extremities, frontal knee malalignments are common. We assessed the effectiveness of guided growth via tension band plates in children with achondroplasia in comparison to patients with idiopathic knee deformities using radiography.
View Article and Find Full Text PDFNeurosurgery
December 2024
Department of Neurosurgery, Leiden University Medical Centre, Leiden , the Netherlands.
Background And Objectives: Adults with achondroplasia are more vulnerable to suffer from neurogenic claudication because of a congenital narrow spinal canal, which makes them susceptible to lumbar spinal stenosis (LSS). The study aims to investigate the correlations between sagittal alignment parameters and the degree of LSS in patients with achondroplasia with LSS.
Methods: The radiological data of adult achondroplasts presented to the neurosurgical clinic of our medical center from 2019 to 2022 were collected.
Pediatr Neurosurg
August 2023
Department of Neurosurgery, Loma Linda University Health, Loma Linda, California, USA.
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.
View Article and Find Full Text PDFSpine Deform
November 2022
Spinal Unit, Pediatric Orthopaedic Surgery and Traumatology Department, Hospital Infantil Universitario Niño Jesús, Av. de Menéndez Pelayo, 65, 28009 JCR, Madrid, Spain.
Purpose: Limb-lengthening surgery to treat short stature has undergone great development in recent years with the use of intramedullary telescopic nails (TIMNs). A limited number of studies have explored the impact of lower limb lengthening on the spine, though their conclusions are not consistent. The aim of this research is to analyze changes in spinopelvic sagittal alignment and balance after lower limb lengthening in achondroplastic patients.
View Article and Find Full Text PDFJ Pediatr Orthop
July 2022
Department of Orthopaedic Surgery and Traumatology.
Background: Patients with achondroplasia may benefit from limb-lengthening surgery with telescopic intramedullary nails (TIMNs). However, the 5-cm maximum length of the nails used in these patients in their original design may be insufficient. The aim of this research is to analyze the outcomes and complications after reusing the same TIMN for a second consecutive 5-cm lengthening in patients with achondroplasia.
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