Clinical and radiologic features, lung function and therapeutic results in pulmonary histiocytosis X.

Clinical appearance, radiologic findings, lung function and results of corticosteroid therapy were analyzed in 42 adult cases of biopsy-proven pulmonary histiocytosis X. Symptoms were present in only 64%. Using the ILO classification 1980, the evaluation of chest radiographs revealed all categories of profusion, size and shape of nodules. The characteristic 'ring figures', i.e. thin-walled cysts, in the parenchyma were detected in 78% of conventional tomographies (n = 27) and in all patients examined by CT (n = 5). In patients with early disease, lung function tests (n = 26) including body plethysmography, pulmonary diffusing capacity and ergospirometry revealed that parameters of gas exchange are most sensitive (TL,CO 84%, KCO 72%). Bronchial reactivity to carbachol was significantly higher than in controls (n = 12). Lung perfusion scintigram showed an abnormal, but uncharacteristic pattern in 81% (n = 26). During corticosteroid therapy, no progression was observed (n = 36). 85% of patients with radiographic evidence of progressive disease improved after administration of corticosteroids (n = 14).

Cholesteatoma in the pediatric age group.

The diagnosis and management of cholesteatoma in children remains controversial. In the past 15 years, the senior author (S.C.P.) has treated 320 patients with cholesteatoma. Patients 18 years and younger composed 40% (125) of the overall group and are the basis for this report. The patient data were compiled using the history, physical examination, audiograms, radiographs, patient questionnaires, surgical findings, and postoperative observations. The surgical treatment selected was determined by the extent of disease, the configuration of the mastoid, and a clinical assessment of eustachian tube function. A middle ear tympanotomy approach was used in 17% of the patients, a canal wall up procedure in 31%, and a canal wall down procedure in 52.3%. The average clinical follow-up was 3.9 years, with the range being from 3 months to 13.5 years. Hearing was maintained or slightly improved in a majority of cases. Residual disease occurred in 8% of patients, and recurrent disease in only 3%.

A review of twenty congenital cholesteatomas of the middle ear in children.

Cholesteatomas, arising within the middle ear space behind an intact tympanic membrane, have been detected more frequently in recent years. This article reviews 19 children (with a mean age of 4.3 years) who underwent surgery over a 7-year period for removal of cholesteatomas from behind intact tympanic membranes. Most of these children were referred by pediatricians who had detected an asymptomatic whitish middle ear mass. Many had histories of treatment for otitis media. Patients with similar histories were excluded from the series if there had been a prior perforation, myringotomy, or otologic surgery. In the series, one fourth of the children had associated congenital malformations, and there were 3 times as many boys as girls. In two thirds of the cases, the cholesteatoma was manifested as an anterior-superior middle ear mass that seemed to arise from the processus cochleariformis, undersurface of malleus area. Many could be removed via an extended anterior tympanotomy operative approach. The results of surgery are reviewed. The possible origins of these localized cholesteatomas are considered. Are they congenital defects that arise from misplaced keratinizing epithelium? Do they arise from mesenchymal cells whose differentiation is stimulated by inflammatory changes within the middle ear?