Otologic manifestations of malignant osteopetrosis.

OBJECTIVE: To determine the incidence of hearing loss and describe the neurotologic manifestations over time in a large series of patients with malignant osteopetrosis. STUDY DESIGN: Longitudinal study. SETTING: Tertiary care referral center. PATIENTS: Thirty-two patients, including 19 infants (< 1 yr of age at initial visit) and 13 children (aged 1-7.6 yr at initial visit), with autosomal recessive osteopetrosis were followed-up during a 10-year period from 1991 to 2001. The average length of follow-up was 2.5 years (range, 0-9.1 yr). INTERVENTIONS: Patients underwent annual otologic evaluations including clinical examination, audiologic evaluation (auditory brainstem response, pure-tone thresholds, speech discrimination scores, and tympanograms), and high-resolution computed tomography of the temporal bone. MAIN OUTCOME MEASURES: Incidence of hearing loss, otitis media, and facial paralysis. Serial changes in temporal bone anatomy by computed tomographic scan. RESULTS: Twenty-six percent of infants' ears demonstrated hearing loss during the first year of life, and 78% of children's ears demonstrated hearing loss during the study period. Of the children's ears with hearing loss, 100% had a conductive component and 26% had an additional sensorineural component (mixed hearing loss); VIIIth nerve conduction was normal in 100% of infants and 78% of children. Five patients (16%) had unilateral facial nerve palsy. Common temporal bone findings included thickening and sclerosis of the calvarium; poor pneumatization of the mastoid bone; and narrowing of the external auditory canal, eustachian tube, and internal auditory canal. CONCLUSION: Otologic manifestations are common in malignant osteopetrosis secondary to the formation of dense, brittle bone. Frequent findings include external auditory canal stenosis, otitis media, conductive and sensorineural hearing loss, and facial nerve paralysis.

Occurrence of subclinical tumor in excised facial subunits.

OBJECTIVE: To determine the incidence of subclinical tumor in excised facial subunits in patients undergoing reconstruction after Mohs surgery. DESIGN: The study group comprised 45 patients who had their Mohs defects repaired by a facial plastic surgeon at a tertiary care center (university hospital). In the group, there were 74 biopsy-proved cutaneous neoplasms of the face. The median age of the group was 67 years. Nineteen patients (42%) had multiple tumors. There were 63 basal cell carcinomas (85%) and 11 squamous cell carcinomas (15%). Forty-seven tumors (64%) were primary and 27 (36%) were recurrent. Reconstruction of the defects was based on the principle of aesthetic subunits. Excised subunits were examined by the Mohs surgeon. Further excisions were performed, as necessary, if tumor was present in the subunit. RESULTS: Five patients (11%) had subclinical basal cell carcinomas in their excised facial subunits. Four patients underwent further resections. CONCLUSIONS: In patients with severe sun damage, recurrent tumors, and a history of skin cancer, clinically normal tissue excised during the reconstruction of their Mohs defects may contain subclinical tumor. Consequently, when these "high-risk" patients undergo reconstruction, excised facial subunits should be submitted for pathologic examination.

Incidental metastatic papillary thyroid carcinoma in microvascular reconstruction.

OBJECTIVES/HYPOTHESIS: Occult papillary thyroid carcinoma has a reported prevalence of 1% to 35% based on autopsy studies. Cervical lymphatic metastases from papillary thyroid carcinoma have been associated with a higher likelihood of recurrence with a questionable impact on survival. Without clinically evident disease in the thyroid or cervical nodes, management of these patients presents a treatment dilemma. We propose an individualized treatment plan for patients in whom metastatic papillary thyroid carcinoma is incidentally detected during neck exploration for other purposes. STUDY DESIGN: Retrospective review and discussion of the literature. METHODS: The clinical course of two patients with incidentally discovered metastatic papillary thyroid carcinoma to the cervical lymph nodes is described. Both patients had previously received head and neck irradiation in childhood and required free flap reconstruction of extensive skull base defects following extirpation of meningiomas. RESULTS: Neck dissection specimens from levels I and II obtained during exposure of recipient vessels for microvascular tissue transfer revealed papillary thyroid carcinoma in both cases. The patients subsequently underwent total thyroidectomy, neck dissection, and postoperative radioactive iodine ablation of residual thyroid tissue. After 1 year of follow-up, both patients were without evidence of recurrent disease. CONCLUSIONS: An individualized approach is justified to treat metastatic papillary thyroid carcinoma incidentally discovered during other procedures. The case reports underscore the importance of pathological analysis of surgical specimens obtained during head and neck reconstruction.