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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

are distinct, recognizable, but highly variable skeletal manifestations of achondroplasia, including cervicomedullary compression. 1 Because of premature closure of cranial base synchondroses, the foramen magnum is smaller in both the transverse and sagittal dimensions in individuals with achondroplasia compared to those with average stature. 4 Stenosis of the foramen magnum and subsequent compression of neurological tissues requiring cervicomedullary decompression (CMD) is estimated to occur in 5%–41% of children with achondroplasia. 5–8 The wide variability

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

of symptomatic cervicomedullary compression over an 11-year period (1993–2003) in pediatric patients with achondroplasia. We identified 43 patients from our database who were treated during this period, and our clinical experience with these individuals is presented here. Clinical Material and Methods The records of all pediatric patients with heterozygous achondroplasia who underwent neurosurgical treatment at our institution between 1993 and 2003 were reviewed retrospectively. Forty-three patients who underwent cervicomedullary decompression during that 11

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Kevin M. Stanko, Young M. Lee, Jennifer Rios, Adela Wu, Giovanna W. Sobrinho, Jon D. Weingart, Eric M. Jackson, Edward S. Ahn, Kaisorn L. Chaichana, and George I. Jallo

from the patients’ electronic medical charts, and data integrity was verified by random spot-checking and double data collection by 2 independent observers. The follow-up duration for each patient was up to the last Chiari-related note entered into the patient’s medical chart. Tonsillar Cauterization Procedure Pediatric neurosurgeons performed all cervicomedullary decompressions and tonsillar cauterizations carried out in this study. The decision to cauterize the tonsils was based on the surgeon’s determination of whether the flow of CSF posterior to the

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

central sleep apnea and sudden infant death. 7 , 10 , 18 , 19 , 26 , 30 To this point, numerous single-center case reports and case series have described the role and safety profile of cervicomedullary decompression for foramen magnum stenosis in children with achondroplasia. 2 , 3 , 11 , 14 , 16 , 23–25 , 32 , 33 It is now widely considered the standard of care for children exhibiting signs and symptoms of foramen magnum compression. 28 However, given the infrequency with which any one center performs this procedure, it has been difficult to ascertain how many

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The transoral approach to the superior cervical spine

A review of 53 cases of extradural cervicomedullary compression

Mark N. Hadley, Robert F. Spetzler, and Volker K. H. Sonntag

✓ The transoral-transclival surgical approach is the most direct operative approach to pathology ventral to the brain stem and superior spinal cord. In selected patients, this approach is efficacious in the treatment of extradural compressive lesions from the cervicomedullary junction to the C-4 vertebra.

The authors have used the transoral surgical approach in treating 53 patients with lesions compressing the ventral extradural brain stem or the cervical cord. The evaluation, management, and long-term outcome of these patients are described (median follow-up time 24 months). The operative morbidity rate in this series was 6%, and the operative mortality rate was zero. The authors review specific features of the transoral procedure, including methods of retraction, microsurgical techniques, and adjunctive measures to avoid cerebrospinal fluid fistulae, that contributed to these good results.

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Frank J. Attenello, Matthew J. McGirt, April Atiba, Muraya Gathinji, Ghazala Datoo, Jon Weingart, Benjamin Carson, and George I. Jallo

deformity after cervicomedullary decompression. We set out to determine the time frame of curvature progression and assess which patient subgroups are at greatest risk for progression of spinal deformity postoperatively. Clinical Materials and Methods In 21 consecutive patients undergoing posterior fossa decompression for CM-I–associated scoliosis at The Johns Hopkins Hospital between 1995 and 2005, we reviewed presenting symptoms, neurological deficits, demographic data, comorbidities, pre- and postoperative radiological studies, operative records, and follow

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Emil Pásztor, János Vajda, Pál Piffkó, Miklós Horváth, and Ildikó Gádor

✓ Although the number of reports concerning the transoral approach to anteriorly placed lesions of the craniocervical junction are increasing, the development of this technique is still in its early stages. The indications and surgical methods vary widely, and there is much room for discussion of the technical details. Eight cases operated on via transoral surgery during the last 4 years are presented in support of the transoral approach to tumors in the craniocervical region.

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Nathan Moskowitz, Benjamin Carson, Steven Kopits, Roy Levitt, and Graeme Hart

to manage the patient's hydrocephalus markedly decreased the number of apneic events and led to improvement in oxygenation. Only by the application of a special halo device and constant oxygen saturation monitoring could we safely undertake cervicomedullary decompression surgery. This procedure did not lead to an immediate improvement of neurological or respiratory symptoms; however, because of the extreme nature of the compression and the presence of all other simultaneous factors interfering with respiration, it is possible that improvement may take place over a

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Craniovertebral junction malformations

Clinicoradiological findings, long-term results, and surgical indications in 63 cases

Nicola Di Lorenzo, Aldo Fortuna, and Beniamino Guidetti

10–19 11 20–29 15 30–39 14 40–49 11 50–59 8 60–69 1 Operative Technique The posterior approach involved cervicomedullary decompression by means of suboccipital craniectomy and upper cervical laminectomy. The dura mater was always left open or a dural homograft applied. 5 Our policy in the event of cerebellar herniation was, as a rule, to let well enough alone, removing the extremely redundant nervous tissue in only a few instances and excising the arachnoidal adhesions, usually until the inferior part

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David M. Benglis and David I. Sandberg

option in such patients. References 1 Aryanpur J , Hurko O , Francomano C , Wang H , Carson B : Craniocervical decompression for cervicomedullary compression in pediatric patients with achondroplasia . J Neurosurg 73 : 375 – 382 , 1990 2 Bagley CA , Pindrik JA , Bookland MJ , Camara-Quintana JQ , Carson BS : Cervicomedullary decompression for foramen magnum stenosis in achondroplasia . J Neurosurg 104 : 3 Suppl 166 – 172 , 2006 3 Carson B , Winfield J , Wang H , Reid C , McPherson R , Kopits S