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Von Recklinghausen's neurofibromatosis
Otolaryngologic Clinics of North America
|February 1, 1987
Summary
Neurofibromatosis, also known as von Recklinghausen's disease, presents diverse head and neck pathologies. Current management focuses on surgical intervention and multidisciplinary care for optimal patient outcomes.
Area of Science:
- Otolaryngology
- Medical Genetics
Background:
- Neurofibromatosis (von Recklinghausen's disease) is a genetic disorder with progressive manifestations.
- While no cure exists, understanding its varied symptoms is crucial for patient care.
Purpose of the Study:
- To outline the otolaryngic manifestations of neurofibromatosis.
- To emphasize the importance of a coordinated, multidisciplinary approach in managing these patients.
Main Methods:
- Review of clinical presentations of neurofibromatosis in the head and neck region.
- Compilation of otolaryngic pathologies associated with the disease (referenced in Table 1).
Main Results:
- Neurofibromatosis affects multiple head and neck structures, leading to a wide spectrum of otolaryngic pathology.
- Surgical management is often a key component of treatment.
Conclusions:
- A comprehensive management plan requires surgical intervention and collaboration among specialists.
- A team-based approach is essential for improving patient function and cosmetic appearance.