Neurofibromatosis

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Also known as Von Recklinghausen's disease, neurofibromatosis is an autosomal-dominant hereditary disorder that causes CNS tumors and cutaneous neurofibromata. The most common manifestation of this disease that is encountered in the practice of otolaryngology is the acoustic neuroma. Occasionally, a lesion involves the larynx (13). Figure 6 depicts a very large lesion in the posterior glottis of an infant. Treatment is difficult because complete removal of such a lesion in the larynx is usually not possible without total laryngectomy, and recurrence is common with partial removal.

FIGURE 6 Large neurofibroma of the posterior glottis in an infant.

REFERENCES

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8. Whiteside OJ, Martinez Devesa P, Ali I, et al. Mucous membrane pemphigoid: nasal and laryngeal manifestations. J Laryngol Otol 2003; 117(11):885-888.

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10. Neel HB III, McDonald TJ. Laryngeal sarcoidosis: report of 13 patients. Ann Otol Rhinol Laryngol 1982; 91(4 Pt 1):359-362.

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12. Wu CL, Linne OC, Chiang CW. Herpes zoster laryngis with prelaryngeal skin erythema. Ann Otol Rhinol Laryngol 2004; 113(2):113-114.

13. Holt GR. E.N.T. manifestations of Von Recklinghausen's disease. Laryngoscope 1978; 88(10): 1617-1632.

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