Sjögren's syndrome: severe upper airways obstruction due to primary malignant tracheal lymphoma developing during successful treatment of lymphocytic interstitial pneumonitis.

A wide spectrum of respiratory tract involvement may be seen in Sjögren's syndrome, however, multiple manifestations in one patient are unusual. A 65-year-old woman with a 5-year history of Sjögren's syndrome presented with hoarseness and pulmonary infiltrates. Lymphocytic interstitial pneumonitis, tracheobronchitis with subglottic edema and obstructive small airways disease were initially documented. Dramatic radiographic improvement of the lymphocytic interstitial pneumonitis occurred during 14 months of corticosteroid therapy. However, a rare primary malignant lymphoma of the trachea developed and presented with steroid refractory, upper airways obstruction. Subsequent patient management and the dichotomous behavior of the lymphoid infiltrates are discussed.

Adenoid hypoplasia in the velo-cardio-facial syndrome.

The velo-cardio-facial syndrome is one of the most comprehensively studied congenital malformation syndromes of clefting. More than 20 features have been described to date. The purpose of this investigation is to report evidence for congenital hypoplasia of the adenoids in over 80% of the cases studied. Radiographic and nasopharyngoscopic studies of 30 children with velo-cardio-facial syndrome were reviewed along with 30 age- and sex-matched children with nonsyndromic cleft palate to determine the size of their adenoid masses. Eighty-three percent of the subjects with velo-cardio-facial syndrome were found to have small or absent adenoids, whereas only 20% of the controls were found to have small adenoids. None of the control sample had total adenoid absence. These findings may have significant implications for the hypernasal speech patterns often found in individuals with velo-cardio-facial syndrome and may also be related to histories of frequent upper respiratory illnesses in this group.

Patterns of velopharyngeal valving in normal and cleft palate subjects: a multi-view videofluoroscopic and nasendoscopic study.

The mechanism of velopharyngeal valving in normal subjects and patients with velopharyngeal insufficiency (VPI) has been the subject of keen debate. The authors have studied 80 normal subjects and 500 patients at the Center for Craniofacial Disorders using direct nasopharyngoscopy and multi-view videofluoroscopy to ascertain the patterns and mechanism of velopharyngeal valving. Multiple patterns of valving were found in both populations and were characterized as "coronal," "sagittal," "circular," and "circular with Passavant's ridge." The incidence of the differing patterns of closure were surprisingly similar in frequency in both normal and pathologic populations. The importance of these diagnostic methods is discussed in relation to treatment of patients with VPI, particularly in designing the type and level of pharyngoplasty.

Velopharyngeal insufficiency in the facio-auriculo-vertebral malformation complex.

Twenty-two patients with the Facio-Auriculo-Vertebral Malformation Complex (hemifacial microsomia) were examined with multi-view videofluoroscopy and nasopharyngoscopy at rest and during speech. Fifty-five per cent of the subjects had velopharyngeal insufficiency (VPI) and hypernasal speech. Only two of these 12 subjects had cleft palate. VPI was related to structural and physiological asymmetry of the pharynx. No relationship was found between the severity of facial malformation and the presence of VPI.

Pharyngeal hypoplasia in Treacher Collins syndrome.

Examination of 11 patients with Treacher Collins syndrome (TCS), with the use of multiple-view videofluoroscopy and nasopharyngoscopy of the pharynx, disclosed marked narrowing of the airway. In several patients, the pharynx was less than 1 cm in width at its most narrow point. It is thought that reduced airway in TCS may help to explain the frequent reports of neonatal death associated with the syndrome. Pharyngeal narrowing was found throghout the entire vertical height of the pharynx in all 11 patients. Pharyngeal hypoplasia is probably responsible for reported difficulties in intubating patients with TCS for endotracheal anesthesia and for respiratory complications after palatoplasty and pharyngoplasty. Pharyngeal hypoplasia is considered to be a primary feature of the syndrome and may aid in its diagnosis.

Fibro-optic study of pharyngeal airway during sleep in patients with hypersomnia obstructive sleep-apnea syndrome.

Pharyngeal airway during sleep was observed with help of Fibro-optic flexible bronchoscope and cineradiography in 10 adult patients with Hypersomnia Sleep-Apnea (HSA) syndrome. The results of the study suggest that the structures involved in production of airway obstruction in the patients with HSA syndrome are the muscles of velopharyngeal sphincter and tongue. The laryngeal airway was not obstructed during the episodes of apnea.

Patterns of velopharyngeal closure in subjects with repaired cleft palate and normal speech: a multi-view videofluoroscopic analysis.

Thirty subjects with repaired palatal clefts and normal speech ranging in age from 2 to 12 years were examined in multiple videofluoroscopic projections in order to assess velopharyngeal closure in three dimensions. The following points became evident from the results of this study: 1. All 30 subjects exhibited contact between the superior border of the velum and the adenoid mass in the nasopharynx. Often, a sharp levator eminance was not formed due to the small space between the velum at rest and the adenoids. Thus, there was little room for the velum to elevate. 2. All 30 subjects showed good localized medial movement of the LAPW at the approximate plane of the hard palate. 3. 10 out of 30 subjects, 33%, had a Passavant's Ridge during speech. All 10 of these subjects utilized the ridge as a point of closure, as well as the adenoids. 4. The observed patterns of closure were consistent across varied consonant utterances. 5. The mechanism of velopharyngel closure in this group of subjects is essentially the same as for normal adult speakers and differs only anatomically due to a lack of verticle head growth in children. Thus, in spite of the presence of adenoids and the lack of verticle head development, the basic sphincteric mechanism of velopharyngeal colsure is utilized in these subjects.