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Publications (2)1.62 Total impact

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    ABSTRACT: Craniofacial and skull base rhabdomyosarcomas (RMSs) are rare. Surgeons are involved both in the resection and the reconstruction of these tumors and in the management of late sequelae of earlier treatment. These tumors are highly heterogeneous, and the initial presentation may be insidious. We wished to determine how diagnostic difficulties could be overcome and how management outcomes could be optimized. All cases of RMS in patients who presented to our craniofacial program between 2004 and 2008 were retrospectively reviewed. Eight consecutive patients with RMS were treated. Six patients are Maori. Four patients had surgical resections of their primary tumors including 3 with curative intent. One had a recurrent tumor, and 3 had functional reconstruction after an earlier treatment. Inaccuracies in histologic diagnosis occurred in 2 patients. Two patients developed postoperative cerebrospinal fluid leakage. Two patients had leptomeningeal involvement and died within 10 months. All patients treated with curative intent achieved complete macroscopic margins. Two remained disease-free at 10 and 22 months. Treatment must be in the context of a multidisciplinary team with the appropriate craniofacial, neurosurgical, and reconstructive skills. Expedient and accurate histologic diagnoses by experienced pathologists are critical in optimizing the outcomes. Oncologists must be well resourced to administer chemoradiation in a timely manner. Leptomeningeal invasion is a dire prognostic feature. Cerebrospinal fluid leak after resection must be identified early and repaired. Late consequences of early childhood craniofacial cancer treatment may be ameliorated by tailored reconstructions. The predominance of Maori in our series has not been reported previously.
    The Journal of craniofacial surgery 09/2009; 20(5):1388-93. · 0.81 Impact Factor
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    ABSTRACT: Techniques for sagittal synostosis correction continue to evolve to improve outcomes and minimize morbidity. The techniques now used by our craniofacial service are spring-assisted cranioplasty for younger children (generally up to 9 months of age) and biparietal barrel staving with cross-struts using bioabsorbable plates for older children. We evaluate the evolution of, and rationale for, our current methods of treatment. All patients who underwent surgery for craniosynostosis over the period 1982 to 2007 were retrospectively reviewed. Patients with sagittal synostosis were analyzed according to the type of operative procedure performed. Blood loss, operative time, and hospital stay were compared between techniques using analysis of variance. Seventy-one patients had a cranial vault remodeling procedure for craniosynostosis. Thirty-four patients had sagittal synostosis with scaphocephaly. During the period 1982 to 2000, 13 patients had a strip craniectomy, and 5 patients had a barrel stave and morcellation procedure. Since 2001, the cross-strut technique using bioabsorbable plates has been used in 7 patients. In 2005, the spring cranioplasty technique was introduced and has been used in 9 patients. There were no deaths and no serious complications. One patient treated with springs had a second spring procedure performed to further increase biparietal width. Spring-assisted cranioplasty had a significantly shorter operating time than other techniques (P < 0.01). The availability of both absorbable plates and expansile cranial springs has revolutionized the techniques our unit uses for scaphocephaly correction. Our early experience with these techniques has shown that the techniques are reliable and give good cranial shape and form with minimal treatment morbidity.
    The Journal of craniofacial surgery 01/2009; 20(1):129-33. · 0.81 Impact Factor