Giant cell angiofibroma of the nasolacrimal duct.

PURPOSE: To describe clinical and histologic features of the first case, to our knowledge, of giant cell angiofibroma located in the nasolacrimal duct region in a 28-year-old woman. METHODS: Interventional case report. A left nasolacrimal duct tumor was excised en bloc by lateral rhinotomy. Histopathologic examination was performed with the use of light microscopy. Immunohistochemical staining included S-100 protein, muscle-specific actin, desmin, myoglobin, vimentin, and CD34. RESULTS: The lesion was characterized by haphazardly arranged oval to spindled cells, a myxoid and collagenous stroma, multinucleated giant cells, prominent blood vessels, and pseudovascular spaces. Tumor cells were strongly positive for vimentin and CD34 and were negative for other antigens. After excision, there has been no recurrence over 4 years of follow-up. CONCLUSIONS: Originally described as an orbital tumor, giant cell angiofibroma also may occur in the nasolacrimal duct and lacrimal sac region. This mesenchymal neoplasm should be included in the differential diagnosis of lacrimal drainage system tumors.

Familial occurrence of acinic cell carcinoma of the parotid gland.

We report the familial occurrence of acinic cell carcinoma involving the parotid gland, the first such report of which we are aware. The familial occurrence of any salivary gland neoplasm is rare. Several reports are present in the literature, including pleomorphic adenoma, Warthin tumor, carcinoma of the submandibular gland, and malignant lymphoepithelial lesion. We report the case of a 35-year-old man who underwent excision of a left parotid gland acinic cell carcinoma. Eight years later, his daughter presented at the age of 16 years with a nontender parotid gland mass that was excised and found also to be acinic cell carcinoma. The histologic features of both neoplasms were typical of acinic cell carcinoma. While this may represent a coincidental event, the possibility that this familial occurrence is a manifestation of common genetic or environmental risk cannot be excluded.

Central deafness in a young child with Moyamoya disease: paternal linkage in a Caucasian family: two case reports and a review of the literature.

A case of 'central deafness' is presented in a 3-year-old male Caucasian child with Moyamoya disease (MMD); a rare, progressive and occlusive cerebrovascular disorder predominantly affecting the carotid artery system. Documentation of normal peripheral auditory function and brainstem pathway integrity is provided by acoustic admittance, otoacoustic emission and brainstem auditory evoked potential measurements. The lack of behavioral response to sound, and absent middle and long latency auditory evoked potentials suggest thalamo-cortical dysfunction. Magnetic resonance imaging showed diffuse ischemic damage in subcortical white matter including areas of the temporal lobes. In addition, there were multiple and focal cortical infarctions in both cerebral hemispheres, focused primarily in the frontal, parietal and temporal areas. Taken together, these structural and functional abnormalities in addition to severely delayed speech and language development are consistent with the diagnosis of central deafness and suggest a disconnection between higher brainstem and cortical auditory areas. The child's father also has MMD, but was diagnosed only recently. The presence of paternal linkage is informative since it rules out x-linked recessive and maternal inheritance. To our knowledge, this represents the first documented case of paternal linkage in MMD with central deafness in a Caucasian child with no apparent Japanese ancestry. Herein, we focus on central auditory dysfunction and consider how lesion-induced changes have contributed to a deficit in basic auditory responsiveness, including a severe disturbance in receptive and expressive auditory-based speech and language skills.

Temporal-order discrimination for selected auditory and visual stimulus dimensions.

Thresholds for the discrimination of temporal order were determined for selected auditory and visual stimulus dimensions in 10 normal-adult volunteers. Auditory stimuli consisted of binary pure tones varying in frequency or sound pressure level, and visual stimuli consisted of binary geometric forms varying in size, orientation, or color. We determined the effect of psychophysical method and the reliability of performance across stimulus dimensions. Using a single-track adaptive procedure, Experiment 1 showed that temporal-order thresholds (TOTs) varied with stimulus dimension, being lowest for auditory frequency, intermediate for size, orientation, and auditory level, and longest for color. Test performance improved over sessions and the profile of thresholds across stimulus dimensions had a modest reliability. Experiment 2 used a double-interleaved adaptive procedure and TOTs were similarly ordered as in Experiment 1. However, TOTs were significantly lower for initially ascending versus descending tracks. With this method, the reliability of the profile across stimulus dimensions and tracks was relatively low. In Experiment 3, psychometric functions were obtained for each of the stimulus dimensions and thresholds were defined as the interpolated 70.7% correct point. The relative ordering of TOTs was similar to those obtained in the first two experiments. Non-monotonicities were found in some of the psychometric functions, with the most prominent being for the color dimension. A cross-experiment comparison of results demonstrates that TOTs and their reliability are significantly influenced by the psychophysical method. Taken together, these results support the notion that the temporal resolution of ordered stimuli involves perceptual mechanisms specific to a given sensory modality or submodality.

Tissue response to suture materials implanted subcutaneously in a rabbit model.

We compared the tissue response to a nonabsorbable monofilamented suture made of expanded polytetrafluoroethylene (ePTFE), which has recently been introduced for use in plastic surgery, with the response to 10 other commercially available absorbable sutures and nonabsorbable monofilamented and multifilamented sutures. The sutures were used to secure a patch of ePTFE implanted in the dorsum of adult New Zealand White rabbits. At 30, 60, and 120 days after implantation, the tissue response to the sutures was assessed with respect to the number of foreign-body giant cells present, the thickness of the fibrous capsule that developed, and the general inflammatory response (n = 4 for each suture for each time period). Analysis of variance revealed that specific suture type was significantly associated with foreign-body giant cell count and fibrous capsule thickness. Tevdek had a significantly higher value for mean number of foreign-body giant cells. Silk and Tevdek had significantly thicker fibrous capsules, and ePTFE suture had a significantly thinner capsule. Absorbable sutures and nonabsorbable multifilamented sutures evoked a more extensive tissue response than monofilamented sutures; the differences between nonabsorbable monofilamented and nonabsorbable multifilamented sutures were significant for capsule thickness. In general, suture made of ePTFE produced a minimal tissue response. It should be a good choice for use in facial plastic surgery, in which excellent functional and aesthetic results are critical.

p53 protein expression in benign lesions of the upper respiratory tract.

OBJECTIVE: p53 is a tumor suppressor gene that is lost or mutated in most forms of human malignancy. There are, however, very few studies evaluating p53 expression in normal epithelium or benign lesions. DESIGN: We screened for p53 protein expression in a variety of benign epithelial lesions of upper respiratory tract using monoclonal antibody DO-1 on paraffin-embedded material. SUBJECTS: We studied a total of 109 cases: 16 cases of juvenile and 36 cases of adult laryngeal papillomatosis, 10 cases each of laryngeal nodules and laryngeal polyps, 17 cases of inverted papilloma, and 20 cases of nasal polyps. RESULTS: Nuclear immunoreactivity for p53 protein was demonstrated in 14 (88%) of 16 cases of juvenile laryngeal papillomatosis, 33 (92%) of 36 cases of adult laryngeal papillomatosis, 4 (40%) of 10 cases of laryngeal nodules, 8 (80%) of 10 cases of laryngeal polyps, 7 (41%) of 17 cases of inverted papilloma, and 2 (10%) of 20 cases of nasal polyps. These results pertained only to the basal epithelial layer in all cases of laryngeal nodules, laryngeal polyps, and nasal polyps. Intermediate layer cells were also positive for p53 in the majority of the cases of both juvenile (69%) and adult (75%) laryngeal papillomatosis and in a minority of the cases of inverted papilloma (18%). CONCLUSIONS: Overexpression of p53 protein is commonly demonstrable in benign epithelial lesions of the upper respiratory tract. This observation suggests that p53 protein accumulation may occur in the absence of mutation of the p53 gene and may correlate with epithelial proliferative activity.

Modified Bernard-Burow cheek advancement and cross-lip flap for total lip reconstruction.

Subtotal lip reconstruction can be performed using local flaps such as the cross-lip flap or fan flap. Total lip reconstructive efforts usually are suboptimal in providing an adequate oral sphincter, an acceptable aesthetic result, or both. For total upper, lower, or extensive combined soft-tissue defects that include both lips, traditional methods of reconstruction include the use of regional flaps. More contemporary reconstructive efforts emphasize staged reconstruction with local tissue flaps using "like" tissue in a sequential fashion to achieve a successful outcome. We describe the reconstruction of total upper, lower, or extensive combined defects and report on 7 cases using a 2-staged method of reconstruction based on the modifications of the Bernard-Burow and Abbe flaps. The principle of esthetic units an intact modiolus and oral sphincter are emphasized to ensure excellent aesthetic and functional results.

Acute sensorineural hearing loss following intravenous ketoralac administration.

Ketoralac is an injectable nonsteroidal antiinflammatory drug (NSAID) widely used in both out-patient and in-patient settings. Side effects such as acute renal failure, hyperkalemia, gastritis, gastrointestinal bleeding, and asthmatic exacerbation, although rare, have been previously reported. We report the case of a 20-year-old female with polyarteritis nodosa (PAN) who developed bilateral sensorineural hearing loss 25 minutes after receiving 30 mg of intravenous ketoralac. The patient denied any previous medication sensitivities, and was taking oral methotrexate and prednisone at the time of emergency department admission. Both PAN and methotrexate have been independently demonstrated to cause sensorineural hearing loss. We postulate that the patient's hearing loss was the result of ketoralac's direct and indirect ototoxic effects that were potentiated as a result of her underlying illness and medications. We recommend the cautious use of ketorolac in patients with underlying illnesses where NSAID-induced ototoxicity could result in adverse otologic consequences.

Gastric mucormycosis.

A case of gastric mucormycosis proven on culture and histologic examination and successfully treated with resectional surgery and Amphotericin B is described.