Prevention of Fixed, Angular Kyphosis in Achondroplasia

Pauli, Richard M.; Breed, Alan L.; Horton, V. Kim; Glinski, L P; Reiser, Catherine A.

doi:10.1097/01241398-199711000-00006

Cited by 62 publications

(52 citation statements)

References 15 publications

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“…More severe kyphosis is associated with unsupported sitting before there is adequate trunk muscle strength. 37,38 Parents should be counseled to avoid unsupported sitting and to avoid devices that cause curved sitting or "C sitting," such as "umbrella-style" strollers and soft canvas seats during the first year of life. Use of feeder seats for upright positioning should be recommended.…”

Section: The Prenatal Visitmentioning

confidence: 99%

Health Supervision for Children With Achondroplasia

Trotter¹,

Hall²

2005

Pediatrics

190

137

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ABSTRACT. Achondroplasia is the most common condition associated with disproportionate short stature. Substantial information is available concerning the natural history and anticipatory health supervision needs in children with this dwarfing disorder. Most children with achondroplasia have delayed motor milestones, problems with persistent or recurrent middle-ear dysfunction, and bowing of the lower legs. Less often, infants and children may have serious health consequences related to hydrocephalus, craniocervical junction compression, upper-airway obstruction, or thoracolumbar kyphosis. Anticipatory care should be directed at identifying children who are at high risk and intervening to prevent serious sequelae. This report is designed to help the pediatrician care for children with achondroplasia and their families. Pediatrics 2005;116:771-783; achondroplasia, short stature, children, health supervision.

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Section: The Prenatal Visitmentioning

confidence: 99%

Health Supervision for Children With Achondroplasia

Trotter¹,

Hall²

2005

Pediatrics

190

137

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“…It is the most prevalent form of dwarfism, with an incidence of approximately 1 in 25,000 (Pauli et al 1997). Owing to the endochondral ossification defect, limb and spine growth disturbances occur in achondroplasia (Thomeer and van Dijk 2002).…”

Section: Discussionmentioning

confidence: 99%

Surgical treatment of achondroplasia with thoracolumbar kyphosis and spinal stenosis—a case report

et al. 2006

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“…Prevalence is estimated around 94% in children younger than 1 year of age. The treatment in early childhood is to prevent the development of a fixed deformity by sitting modification, physiotherapy and bracing [2]. Without early conservative management, approximately 11% of patients developed important and progressive deformity with a significant risk of spinal cord compression with neurological impairment during growth [3].…”

Section: Historical Reviewmentioning

confidence: 99%

Surgical treatment of a 180° thoracolumbar fixed kyphosis in a young achondroplastic patient: a one-stage “in situ” combined fusion and spinal cord translocation

et al. 2010

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An achondroplastic patient with a thoracolumbar kyphosis was first seen at the age of 16 at our institution. His only concern at that time was the aesthetic implication of his deformity. His physical examination was normal except for loss of the neurologic reflexes in the lower limbs. The radiographs showed a fixed 180°thoracolumbar kyphosis with correct frontal and sagittal balances. No spinal cord anomaly was found on MRI. Two years later, he developed a progressive neurogenic claudication of the lower limbs.He was still neurologically intact at rest. The MRI showed an abnormal central spinal cord signal in front of the apex of the kyphosis associated with the narrow congenital spinal canal. In regards to this progressive neurological worsening, a surgical treatment was decided. We decided to perform a front and back arthrodesis combined with a spinal cord decompression without reduction of the deformity. A five-level hemilaminotomy was performed with a posterior approach at the kyphosis deformity. The spinal cord was individualised onto 10 cm and the left nerve roots were isolated. A decancellation osteotomy of the three apex vertebrae and a disc excision were performed. The posterior aspect of the vertebral body was then translated forward 2 cm and in association with the spinal cord. Two nerve roots were severed laterally to approach the anterior part of the kyphosis and a peroneal strut graft was inlayed anterolaterally. A complementary anterior and a right posterolateral fusion was made with cancellous bone. The patient was immobilised in a cast for 3 months relayed by a thoracolumbosacral orthosis for 6 months. At 3 years follow-up, the neurogenic claudication had disappeared. No worsening of the kyphosis was observed. His only complaint is violent electric shock in the lower limbs with any external sudden pressure on the spinal cord in the area uncovered by bone.

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Prevention of Fixed, Angular Kyphosis in Achondroplasia

Cited by 62 publications

References 15 publications

Health Supervision for Children With Achondroplasia

Health Supervision for Children With Achondroplasia

Surgical treatment of achondroplasia with thoracolumbar kyphosis and spinal stenosis—a case report

Surgical treatment of a 180° thoracolumbar fixed kyphosis in a young achondroplastic patient: a one-stage “in situ” combined fusion and spinal cord translocation

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