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American Association of Plastic Surgeons
89th Annual Meeting Abstracts

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Craniofacial Neurofibroma
MCKAY MCKINNON, MD.
CHILDREN'S MEMORIAL HOSPITAL, CHICAGO, IL, USA.

CRANIOFACIAL NEUROFIBROMA
Purpose: The resection and reconstruction for craniofacial neurofibroma remains a surgical failure in far too many patients. A recent case which culminated in facial allo-transplantation emphatically illustrates this failure of standard treatment, and not only of adequate resection, but also of satisfactory reconstruction. The vascular component of craniofacial NF is also poorly understood and poses a large surgical risk.
This study attempts to systematically address the reasons for poor outcomes and to suggest a radical reassessment of treatment strategy based on a large surgical experience with plexiform craniofacial NF.
Methods: A consecutive group of 53 patients with plexiform craniofacial neurofibromas who underwent surgical treatment by the author over an eight year period were studied by retrospective clinical, radiographic and chart review. No patients were excluded from review of at least six months follow-up.
Results: The patients ranged in age from 15 months to 72 years. Nine patients required staged procedures, including one patient who had a neurofibrosarcoma within a benign neufibroma. There were no deaths. One patient suffered a cerebrovascular hemorrhage after tumor resection and cranial reconstruction in a non-adjacent area of the brain. Two patients suffered skin necrosis following subcutaneous tumor resection. Two patients revealed reappearance of tumor adjacent to the cavernous sinus 15 years following prior excisions. One patient suffered failure of frontal branch nerve reconstruction following successful tumor resection and secondary nerve grafting. No patients demonstrated tumor recurrence within at least two years of surgery by clinical and MRI exams. Vascular embolisation was performed pre-operatively in one patient who had a high flow lesion with bruit. Communication of intra-lesional vascular lakes with the jugular venous system was demonstrated in three patients.
Conclusion: The major errors of craniofacial neurofibroma treatment are: (1) Delayed diagnosis; (2) Treatment delay; (3) Inadequate primary and secondary surgery, and (4) Failure to perform fundamental maxillofacial and craniofacial reconstructive procedures following resection.
Treatment of craniofacial neurofibroma should begin in the early stage of the manifest disease, before functional impairment. Surgery should be aggressive if functional compromise is imminent, even in the very young. Autogenous reconstruction should be intuitive in young patients who often require re-operative care. Craniofacial principles should be an integral part of the reconstruction strategy. Surgeons should be co-responsible for surveillance of this highly complex patient group. The vascular manifestations of NF are uniquely complex and are previously unreported. These conclusions and mainly positive results are at variance to many previous reports and deserve additional studies for validation and elucidation of tumor dormancy.


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