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Neurofibroma of the pinna.

Abstract

Neurofibromas are relatively common lesions of the nervous system, but only a few cases involving the pinna have been reported. The emphasis in most of these reports was on the cosmetic deformity; functional impairment in the form of hearing loss has been mentioned in only a few reports. We report a case of pinnal neurofibroma in which the primary complaint was otitis externa; hearing loss and cosmetic deformity were also present. Surgical excision resulted in an excellent functional and cosmetic outcome. This case demonstrates that neurofibroma of the pinna in the area of the external meatus can produce functional as well as cosmetic impairment, and surgery can produce a very satisfactory result.

Introduction

Neurofibromas are relatively common lesions of the nervous system. They may develop anywhere in the body, including cranial and peripheral nerves. The type of impairment associated with neurofibromas depends largely on the site of the lesion. Cutaneous lesions, especially in the head and neck, generally cause deformity, whereas lesions that affect deeper systems (e.g., the auditory and ocular systems) are more likely to cause functional impairment.

While neurofibromas of the head and neck are not uncommon, they rarely affect the pinna, and only a few such published reports are available. The authors of most of these reports emphasized the disfigurement aspect of the case, (1) but Stevenson et al (2) also reported functional impairment in the form of hearing loss. They noted that neurofibroma-induced hearing loss can be caused by obstruction of the external auditory canal or invasion of the middle and inner ear.

We present a case of neurofibroma of the pinna in which the patient's chief complaint was otitis externa. She also reported cosmetic deformity and hearing loss. All of her complaints responded to surgical treatment.

Case report

A 42-year-old woman presented to our Department of Otolaryngology-Head and Neck Surgery complaining of persistent left otitis externa, swelling around the left pinna, and hearing loss and a sensation of blockage in the left ear. The most troublesome condition was the otitis externa. She reported that the swelling had been present for many years and that it was slowly progressive. She was known to have von Recklinghausen's disease with the typical cafe au lait spots. She had twice previously undergone excision of a neurofibroma, including one that had been located above the left pinna.

Examination revealed a firm, nontender lesion behind the left pinna and a large lesion in the conchal bowl that completely occluded the external auditory meatus (figure 1, A). The ear canal was acutely inflamed and filled with keratinous debris, findings that are consistent with otitis externa. Following aural toilet, pure-tone audiometry revealed a conductive heating loss of 30 dB in the left ear (figure 2, A).

[FIGURES 1-2 OMITTED]

The lesion in the conchal area was excised along with conchal and external meatal skin; the conchal cartilage and perichondrium were preserved. A split-skin graft from the left arm was used to cover the defect. Histologic examination of the specimen identified it as a neurofibroma. The lesion behind the pinna was not removed because it was not causing a problem.

The patient made an uneventful recovery, and a good cosmetic result was achieved (figure 1, B). The conductive hearing loss in the left ear resolved (figure 2, B), and the patient remained free of otitis externa at the 2-year follow-up.

Discussion

Neurofibromas are circumscribed but nonencapsulated neoplasms of the nervous system. They can arise in all peripheral nerve elements, including Schwann's cells, neurons, fibroblasts, and perineural cells. (3) They may occur in isolation or as part of von Recklinghausen's syndrome in combination with cafe au lait skin patches, Lisch's nodules, axillary freckling, and fibroma molluscum. (4) The disease may be inherited as an autosomal-dominant trait with variable penetrance in 50% of patients, or it may occur as a result of a spontaneous mutation. (5) Neurofibromas are usually benign, but some cause local destruction secondary to pressure effects. Malignant transformation has been reported to occur in 2 to 16% of cases. (2)

It has been often reported that hot and humid weather predisposes individuals to otitis externa by causing maceration of the ear canal skin. (6) It is also widely accepted that otitis exterua can be caused by occlusion of the ear canal by, for example, a hearing aid or large osteoma. (7) The exact mechanism of occlusion-induced otitis externa is not well defined, but it is possible that occlusion of the ear canal inhibits skin migration. Another possibility is that occlusion increases the humidity in the ear canal and leads to (7.) Karlowsky JA, Thornsberry C, Jones ME, et al. Factors associated with relative rates of antimicrobial resistance among Streptococcus pneumoniae in the United States: Results from the TRUST Surveillance Program (1998-2002). Clin Infect Dis 2003;36:963-70.

(8.) Pelton SI. Acute otitis media in an era of increasing antimicrobial resistance and universal administration of pneumococcal conjugate vaccine. Pediatr Infect Dis J 2002;21:599-604.

(9.) Arason VA, Kristinsson KG, Sigurdsson JA, et al. Do antimicrobials increase the carriage rate of penicillin resistant pneumococci in children? Cross sectional prevalence study. BMJ 1996;313: 387-91.

(10.) Dagan R, Fraser D. Conjugate pneumococcalvaccine and antibiotic-resistant Streptococcus pneumoniae: Herd immunity and reduction of otitis morbidity. Pediatr Infect Dis J 2000;19(5 suppl):S79-S87; discussion S88.

(11.) Dagan R, Leibovitz E, Leiberman A, Yagupsky E Clinical significance of antibiotic resistance in acute otitis media and implication of antibiotic treatment on carriage and spread of resistant organisms. Pediatr Infect Dis J 2000; 19(5 suppl):S57-S65.

(12.) Eldan M, Leibovitz E, Piglansky L, et al. Predictive value of pneumococcal nasopharyngeal cultures for the assessment of nonresponsive acute otitis media in children. Pediatr Infect Dis J 2000;19:298-303.

(13.) Gray BM, Converse GM III, Dillon HC Jr. Epidemiologic studies of Streptococcus pneumoniae in infants: Acquisition, carriage, and infection during the first 24 months of life. J Infect Dis 1980;142: 923-33.

(14.) Reichler MR, Allphin AA, Breiman RF, et al. The spread of multiply resistant Streptococcus pneumoniae at a day care center in Ohio. J Infect Dis 1992;166:1346-53.

(15.) Yagupsky P. Porat N, Fraser D, et al. Acquisition, carriage, and transmission of pneumococci with decreased antibiotic susceptibility in young children attending a day care facility in southern Israel. J Infect Dis 1998;177:1003-12.

(16.) Henderson FW, Gilligan PH, Wait K, Goff DA. Nasopharyngeal carriage of antibiotic-resistant pneumococci by children in group day care. J Infect Dis 1988;157:256-63.

(17.) Huebner RE, Dagan R, Porath N, et al. Lack of utility of serotyping multiple colonies for detection of simultaneous nasopharyngeal carriage of different pneumococcal serotypes. Pediatr Infect Dis J 2000;19:1017-20.

(18.) Boken DJ, Chartrand SA, Goering RV, et al. Colonization with penicillin-resistant Streptococcus pneumoniae in a child-care center. Pediatr Infect Dis J 1995;14:879-84.

(19.) Black S, Shinefield H, Fireman B, et al. Efficacy, safety and immunogenicity of heptavalent pneumococcal conjugate vaccine in children. Northern California Kaiser Permanente Vaccine Study Center Group. Pediatr Infect Dis J 2000;19:187-95.

(20.) Butler JC, Breiman RF, Lipman HB, et al. Serotype distribution of Streptococcus pneumoniae infections among preschool children in the United States, 1978-1994: Implications for development of a conjugate vaccine. J Infect Dis 1995;171:885-9.

(21.) Dagan R. Clinical significance of resistantorganisms in otitis media. Pediatr Infect Dis J 2000;19:378-82.

(22.) Capeding MR, Nohynek H, Sombrero LT, et al. Evaluation of sampling sites for detection of upper respiratory tract carriage of Streptococcus pneumoniae and Haemophilus influenzae among healthy Filipino infants. J Clin Microbiol 1995;33:3077-9.

(23.) Lloyd-Evans N, O'Dempsey TJ, Baldeh I, et al. Nasopharyngeal carriage of pneumococci in Gambian children and in their families. Pediatr Infect Dis J 1996;15:866-71.

== Cont. 37

skin maceration and subsequent infection. However, our search of the MEDLINE database failed to produce any direct evidence to confirm either of these theories.

It has been suggested (5,8) that subtotal resection of neurofibromas that are tender or that cause deformity or a loss of function is preferable to a complete excision; the latter procedure may result in a loss of normal tissue and still not prevent a recurrence. Indeed, Trevisani et al (1) wrote that "complete surgical excision of these lesions is virtually impossible, if not contraindicated," and Crikelair and Cosman (9) emphasized that amelioration rather than complete eradication should be the therapeutic goal. Large lesions may require reconstruction and elevation of the pinna with dermal strips (4) or Marlex polypropylene mesh. (1) We were able to completely excise the lesion in our patient without causing any substantial cosmetic and functional deficit.

References

(1.) Trevisani TP, Pohl AL, Matloub HS. Neurofibroma of the ear: Function and aesthetics. Plast Reconstr Surg 1982;70:217-19.

(2.) Stevenson TR, Zavell JF, Anderson RD. Neurofibroma of the ear. Ann Plast Surg 1986;17:151-4.

(3.) House WF, Luetje CM, Doyle KJ. Acoustic Tumors: Diagnosis and Management. 2nd ed. San Diego: Singular Publishing; 1997.

(4.) Grabb WC, Dingman RO, Oneal RM, Dempsey PD. Facial hamartomas in children: Neurofibroma, lymphangioma, and hemangioma. Plast Reconstr Surg 1980;66:509-27.

(5.) Griffith BH, McKinney P, Monroe CW, Howell A. Von Recklinghausen's disease in children. Plast Reconstruct Surg 1972;49:647-53.

(6.) Bruner RC, House LR. Thermodynamics of the external auditory canal. Ann Otol Rhinol Laryngol 1967;76:409-13.

(7.) Whitaker SR, Cordier A, Kosjakov S, Charbonneau R. Treatment of external auditory canal exostoses. Laryngoscope 1998;108:195-9.

(8.) Davis WB, Edgerton MT, Hoffmeister SE Neurofibromatosis of the head and neck. Plast Reconstr Surg 1954;14:186-99.

(9.) Crikelair GF, Cosman B. Histologically benign, clinically malignant lesions of the head and neck. Plast Reconstr Surg 1968;42:343-53.

Azhar M. Shaida, MA, FRCS; Matthew W. Yung, PhD, FRCS

From the Department of Otolaryngology-Head and Neck Surgery, The Ipswich Hospital NHS Trust, Ipswich, U.K.

Reprint requests: Mr. Azhar M. Shaida, Consultant ENT Surgeon, The Royal National Throat, Nose and Ear Hospital, 330 Grays Inn Rd., London WC1X 8DA, UK. Phone: 44-207-915-1672; fax: 44-208924-7286; e-mail: doctor@doctor.co.uk
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Author:Yung, Matthew W.
Publication:Ear, Nose and Throat Journal
Date:Jan 1, 2007
Words:1668
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