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  • By Author: Jallo, George I. x
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Daniel M. Sciubba, Joseph C. Noggle, Neena I. Marupudi, Carlos A. Bagley, Markus J. Bookland, Benjamin S. Carson Sr., Michael C. Ain and George I. Jallo

Object

Achondroplasia is a hereditary form of dwarfism caused by a defect in endochondral bone formation, resulting in skeletal abnormalities including short stature, shortened limb bones, macrocephaly, and small vertebral bodies. In the pediatric population, symptomatic spinal stenosis occurs at all spinal levels due to the abnormally narrow bone canal. In this study, clinical outcomes were assessed in children with achondroplasia after spinal canal decompression.

Methods

A retrospective review was conducted involving pediatric patients with heterozygous achondroplasia and symptomatic stenosis after decompressive procedures at the authors' institution within a 9-year period. Measured outcomes included resolution of symptoms, need for repeated surgery, presence of fusion, development of deformity, and complications.

Forty-four pediatric patients underwent a total of 60 decompressive procedures. The average patient age at surgery was 12.7 years (range 5–21 years). Forty-nine operations were performed for initial treatment of stenosis, and 11 were performed as revision surgeries on previously operated levels. A large proportion of patients (> 60%) required additional cervicomedullary decompressions, most often preceding the symptoms of spinal stenosis. Of the initial procedures, decompression locations included 32 thoracolumbar (65%), 10 lumbar (20%), four cervical (8%), two cervicothoracic (4%), and one thoracic (2%). Forty-three of the decompressive procedures (72%) included spinal fusion procedures. Of the 11 revisions, five were fusion procedures for progressive deformity at levels previously decompressed but not fused (all thoracolumbar), five were for decompressions of symptomatic junctional stenosis with extension of fusion, and one was for repeated decompression at the same level due to recurrence of symptomatic stenosis.

Conclusions

Decompression of the spinal canal in pediatric patients with achondroplasia can be accomplished safely with significant clinical benefit. Patients with a history of cervicomedullary compression may be at an increased risk of developing symptomatic stenosis prior to adolescence. Fusion procedures are recommended in patients with a large decompression overlying a thoracolumbar kyphosis to avoid progressive postoperative deformity.

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Carlos A. Bagley, Karl F. Kothbauer, Sean Wilson, Markus J. Bookland, Fred J. Epstein and George I. Jallo

Object

Currently, the optimal treatment of children harboring myxopapillary ependymomas of the spinal cord remains somewhat debatable. The authors present a retrospective study in which they evaluated the records of patients in whom resection of these lesions had been performed.

Methods

Fourteen pediatric patients who had undergone resection of a spinal cord tumor between September 1982 and July 2004 were identified from the database as having histologically classified myxopapillary ependymomas. There were 10 boys and four girls ranging in age from 7 to 18 years (mean age 12.6 years); 71% of the patients were boys. The clinical presentation of the tumor's course was slow and indolent, and the patients had a mean symptom duration of 19.6 months. Twelve patients, who underwent a total of 16 operations, were available for long-term follow-up review. Thirteen gross-total resections and three subtotal resections were performed. There were no deaths due to surgery. Postoperatively, patients initially remained at their preoperative level of function or improved. Patients who had undergone previous surgery and radiotherapy were treated more conservatively than patients who were undergoing surgery for the first time. Four children experienced significant complications following treatment.

Conclusions

As the authors demonstrate in this study, excellent outcomes may be obtained with the use of aggressive surgical techniques with the goal being that of gross-total resection. Despite the best of resections, however, the risk of recurrence remains. Therefore, periodic neuroimaging surveillance of the neuraxis and close clinical follow up are warranted throughout the patient's life. The role for adjunctive chemo- and radiotherapy remains to be defined in the management of myxopapillary ependymomas.