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Jeffrey L. Nadel, D. Andrew Wilkinson, Hugh J. L. Garton, Karin M. Muraszko, and Cormac O. Maher

A chondroplasia is the most common form of infantile skeletal dysplasia, with a reported prevalence rate ranging from 0.36 to 1.5 per 10,000 individuals. 17 , 29 Neurological sequelae may present in as many as 47% of patients with achondroplasia 22 as a result of congenital spinal stenosis, foramen magnum stenosis, and macrocrania with or without hydrocephalus. 5 , 6 , 8 , 9 , 13 , 22 , 31 Among these abnormalities, foramen magnum and high cervical spinal stenosis are the most concerning, given that the resultant brainstem compression has been implicated in

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Ruchira M. Jha, Paul Klimo Jr., and Edward R. Smith

A chondroplasia , an autosomal dominant dwarfism syndrome, has a reported incidence of 1 in 26,000, making it the most common of all hereditary skeletal dysplasias. 4 Neurological manifestations of this disorder, which occur in 35–47% of patients, 12 include hypotonia, macrocrania with or without hydrocephalus, psychomotor delay, apnea, sleep disorders, multisegmented compressive spinal syndromes, and foramen magnum stenosis. 2 The latter manifestation is frequently noted in pediatric patients. 2 , 6 The ensuing cervicomedullary compression is

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Carlos A. Bagley, Jonathan A. Pindrik, Markus J. Bookland, Joaquin Q. Camara-Quintana, and Benjamin S. Carson

to make the diagnosis of cervicomedullary compression included the following: 1) the presence of signs or symptoms of chronic brainstem compression (such as apnea, lower cranial nerve dysfunction, swallowing difficulties, hyperreflexia or hypertonia, paresis, or clonus); and 2) neuroimaging evidence of foramen magnum stenosis with or without neurological manifestations. Surgical Procedure All patients with a diagnosis of clinically significant cervicomedullary compression underwent a foramen magnum decompression and upper cervical laminectomy, with or without

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Masaki Matsushita, Kenichi Mishima, Ryusaku Esaki, Naoki Ishiguro, Kinji Ohno, and Hiroshi Kitoh

A chondroplasia (ACH) is one of the most common skeletal dysplasias with disproportionate short stature caused by activating mutations in FGFR3 encoding the fibroblast growth factor receptor 3. 10 , 11 In addition to rhizomelic shortening of the extremities, foramen magnum stenosis (FMS) is one of the most serious neurological complications in patients with ACH, which sometimes results in sudden unexpected death in infants. 9 Cervicomedullary decompression surgery has been indicated for infants who exhibited lower-limb hyperreflexia or clonus on

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Janet M. Legare, Chengxin Liu, Richard M. Pauli, Adekemi Yewande Alade, S. Shahrukh Hashmi, Jeffrey W. Campbell, Cory J. Smid, Peggy Modaff, Mary Ellen Little, David F. Rodriguez-Buritica, Maria Elena Serna, Jaqueline T. Hecht, Julie E. Hoover-Fong, and Michael B. Bober

reflects the various methods of ascertainment used to obtain this information. Signs and symptoms of compression include abnormal neurological examination and long-track signs (e.g., clonus and hyperreflexia), gross motor developmental delay, central apnea, signal changes in the spinal cord as seen on MRI, and even sudden infant death. 5 Life-threatening respiratory difficulties and myelopathy can be cured by CMD. 6 , 9–12 Increased awareness and improved management of critical foramen magnum stenosis began in the early 1980s. 11 , 12 Untreated foramen magnum stenosis

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Moise Danielpour, William R. Wilcox, Yasemin Alanay, Barry D. Pressman, and David L. Rimoin

have undergone surgical decompression and have gained normal neurological function may have a persistence of cord narrowing. The existing literature has focused on the surgical treatment of static myelopathy attributed to fixed cervicomedullary compression due to foramen magnum stenosis. 1 , 2 , 5 , 6 , 9 , 10 The present study calls attention to a related but previously unrecognized dynamic phenomenon. We identified four infants and young children with achondroplasia who developed symptoms of headache and/or nausea and vomiting or severe central sleep apnea after

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Timothy C. Ryken and Arnold H. Menezes

basiocciput, exoccipital bone, and craniovertebral junction can result in foramen magnum stenosis and cervicomedullary compression. 11, 12 These patients may present with severe respiratory compromise and myelopathy. Discussion has focused on the role of surgical decompression of the posterior fossa, particularly in the more severely affected homozygous phenotype. 10, 20, 25, 34 To evaluate the characteristic features of cervicomedullary compression in achondroplasia and the results of surgical decompression, we reviewed six patients with heterozygous achondroplasia and

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Dale Swift, Laszlo Nagy, and Brian Robertson

decompression for foramen magnum stenosis had been performed in 7 of the 8 patients who subsequently had surgery for hydrocephalus. Thus, 31% of patients who underwent suboccipital decompression also underwent surgery for hydrocephalus. The average interval from suboccipital decompression to hydrocephalus surgery was 4.97 months. We identified 3 patients with achondroplasia and hydrocephalus who underwent ETV. These patients were considered for ETV due to MR imaging morphology demonstrating triventricular hydrocephalus with apparent fourth ventricle CSF outflow obstruction

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Hiroshi Yamada, Shigetoshi Nakamura, Masataka Tajima, and Naoki Kageyama

✓ The neurological and neuroradiological manifestations of pediatric achondroplasia are analyzed on the basis of 10 cases. In addition to the classical symptomatology of an enlarging head, with or without increased intracranial pressure, several patients presented symptoms related to a small foramen magnum. Respiratory problems and quadriparesis were also observed in these patients. Six patients who were treated by foramen magnum decompression showed remarkable improvement. Neuroradiological evaluation revealed a significant number of patients with dilated cortical sulci and basal cisterns, and mild dilatation of the ventricles on computerized tomography (CT) and/or ventriculography. Two patients showed signs of obstruction at the fourth ventricle outlets. Some exhibited anomalous dilatation of cerebral veins and dural sinuses, narrowing of the sinuses at the jugular foramen, and enlarged emissary veins. Ventriculoperitoneal or ventriculoatrial shunts were placed in three patients. In the other children with a large head and dilated ventricles, head growth curves paralleled the normal slope, and there was no significant clinical evidence of increased intracranial pressure or enlarging ventricles on follow-up CT scan.

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only delay the ultimate outcome in children with homozygous achondroplasia. References 1. Hecht JT , Horton WA , Butler IJ , et al : Foramen magnum stenosis in homozygous achondroplasia. Eur J Pediatr 145 : 545 – 547 , 1986 Hecht JT, Horton WA, Butler IJ, et al: Foramen magnum stenosis in homozygous achondroplasia. Eur J Pediatr 145: 545–547, 1986 2. Hecht JT , Nelson FW , Butler IJ , et al : Computerized tomography of the foramen magnum: achondroplastic values compared to