| OBJECTIVE: To describe the occurrence of fatal laryngeal edema in patients with hereditary angioedema due to C1 esterase inhibitor deficiency. patients AND methods: We describe 6 patients from various regions of germany who died from laryngeal edema within the last 10 years. Furthermore, we conducted a retrospective survey of 58 patients with hereditary angioedema, originating from 46 affected families. The data were obtained from the attending physicians and from the patients' relatives. RESULTS: Among the 6 reported patients, aged 9 to 78 years, hereditary angioedema had been diagnosed in 3 and was undiagnosed in 3. None of them had an emergency cricothyrotomy or received C1 inhibitor concentrate. The interval between onset of the laryngeal edema and asphyxiation was 20 minutes in a 9-year-old boy, and in the other patients, the interval was 1 to 14 hours (mean for all, 7 hours). The retrospective survey of 58 patients with hereditary angioedema revealed 23 deaths by asphyxiation (40%). The average age of all 29 patients at the time of asphyxiation was 39 years. CONCLUSION: laryngeal edema in hereditary angioedema may be fatal. Most of the patients asphyxiated between their 20th and 50th years of life, but asphyxiation can occur even in children. The possibility that the first episode of laryngeal edema may be fatal must be emphasized to the relatives, and attending physicians must have a high degree of awareness. ( info) |
32/674. Vascular ring formed by right aortic arch with aberrant left subclavian artery and left ligamentum arteriosum: a rare cause of respiratory distress in newborn infants. Vascular ring, in which the trachea and esophagus are completely surrounded by vascular structures, is one of the causes of respiratory distress in children. Right aortic arch with aberrant left subclavian artery is a common aortic arch anomaly; however, respiratory distress due to vascular ring is seldom associated with this anomaly. We report herein a newborn infant treated surgically because of severe respiratory distress caused by vascular ring formed by right aortic arch with aberrant left subclavian artery and left ligamentum arteriosum. As laryngomalacia was first thought to be the reason for respiratory distress, we suggest that patients with respiratory distress diagnosed with laryngomalacia be evaluated for possible vascular ring. ( info) |
| sarcoidosis is a chronic granulomatous disease of unknown pathogenesis. Reports of sarcoidosis are much less common in the pediatric literature than in the adult literature. The disease is usually systemic; rarely, however, single organs are affected. Isolated laryngeal involvement is an unusual presentation. We report a well-documented case of isolated laryngeal sarcoidosis in a 14-year-old white boy who presented to our institution with a 6-month history of dysphonia, dyspnea on exertion, and extremely sonorous snoring at night owing to his supraglottic airway disease. To our knowledge, this is only the second case of isolated laryngeal sarcoidosis reported in the pediatric literature. We review the literature and discuss the differential diagnosis, diagnostic evaluation, and treatment with carbon dioxide laser epiglottectomy and intralesional glucocorticoid deposition, which resulted in marked resolution of our patient's symptoms. ( info) |
34/674. Bilateral laryngoceles in a young trumpet player: case report. We report the case of a 16-year-old trumpet player who was referred for an otolaryngologic consultation after his band leader noticed that a neck mass would protrude while the boy was playing. x-rays revealed the presence of bilateral laryngoceles, and computed tomography demonstrated bilateral, air-filled outpouchings of the laryngeal saccules during forced expiration. There was no evidence of any other intra-laryngeal or cervical pathology. Surgery was deferred while the laryngoceles remained reducible and asymptomatic, and the boy was cleared to continue playing. ( info) |
| nocardia farcinica is an emerging pathogen in immunocompromised hosts, accounting for 20% of nocardia isolates in the USA and 13-44% of isolates in europe. The case of a 72-year-old lymphoma patient with a laryngeal abscess caused by nocardia farcinica is presented. The initial clinical manifestation was unilateral vocal cord paralysis, which improved following surgical drainage of the abscess and therapy with imipenem. The English-language literature on human nocardia farcinica infection is reviewed. ( info) |
| Supraesophageal complications of GERD have become more commonly recognized or suspected by physicians. However, the direct association between these complications and GERD has often been difficult, if not impossible, to establish. Furthermore, the majority of patients with suspected supraesophageal complications of GERD do not have either the characteristic symptoms of heartburn and regurgitation or the definitive findings of esophageal inflammation, which would help reinforce the suspicion of a connection between the supraesophageal complications and GERD. Frequent acid reflux has been shown in patients with various bron-chopulmonary, laryngopharyngeal, or oral cavity disorders. GERD is one of the most common gastrointestinal complaints in the population. It is possible that the supraesophageal problems and acid reflux are mutually independent disorders that occur in the same person. The suspected mechanisms of GERD-related supraesophageal complications appear to be directed through two pathways: by a vagal reflex between the esophagus and tracheobronchial tree triggered by acid reflux or by microaspiration that causes contact damage to mucosal surfaces. The most useful diagnostic modality available to the clinician to aid in the diagnosis of supraesophageal GERD complications is the ambulatory pH recording technique. However, the sensitivity and specificity of this test for recording esophageal or pharyngeal acid reflux events has been critically challenged. Despite the many clinical studies that support the theory that GER has a role in suspected supraesophageal complications, only 1 long-term prospective controlled study of a large group of patients with asthma has shown the positive effects of the elimination of acid reflux. With the focus now on "outcomes medicine," there is a serious need for appropriately designed, controlled studies to answer the many questions surrounding a cause-and-effect association between acid reflux and supraesophageal disorders. Because of the lack of convincing proof between acid reflux and suspected supraesophageal complications, the physician must resort to an intent-to-treat strategy as both a primary therapy and a diagnostic trial. High-dose PPI therapy for prolonged periods is the recognized conservative therapy. Operative therapy (i.e., fundoplication operation) is the procedure of choice when overt regurgitation occurs or when medical therapy, although successful, is not practical for long periods. Controlled, well-designed clinical trials and more sophisticated techniques to measure and quantify acid reflux are crucial in the future to help determine which patients with suspected supraesophageal complications actually have acid reflux as a primary cause. The medical community needs to be alerted to the possibility of an association between GERD and supra-esophageal complications so that patients with a GERD-related complication will be recognized and effectively treated. ( info) |
37/674. lingual nerve injury during suspension microlaryngoscopy. lingual nerve injury is an uncommon complication of laryngoscopy. We report a case of isolated unilateral lingual nerve injury that occurred during suspension microlaryngoscopy. The injury was transient, with complete return of sensation within 3 months after surgery. Several mechanisms have been proposed to explain the occurrence of lingual nerve injury during laryngoscopy, including direct compression of the nerve caused by the laryngoscope, stretching of the nerve caused by cricoid pressure or instrumentation, and compression of the nerve between the medial and lateral pterygoid caused by manipulation of the mandible. The precise mechanism of injury in this case was not obvious, but stretching of the lingual nerve caused by pressure of the suspended laryngoscope on the tongue or retrolingual region was likely. The transient nature of the injury and the rapid return of the nerve to baseline function in this case are consistent with a neurapraxic injury. ( info) |
| The present report is concerned with the observation of histiocytosis X in the larynx in a man aged 57 years. This localisation of the process is quite unusual and we have found only one similar case in the literature. The differential diagnosis and possible etiological factors are briefly discussed. ( info) |
| Primary laryngeal amyloidosis is a rare benign disease of unknown aetiology. It can present with dysphonia or stridor. A woman presenting with airway compromise, who required a tracheostomy, is reported. ( info) |
40/674. Vallecular cyst: report of four cases--one with co-existing laryngomalacia. Congenital vallecular cysts are rare. In this report, four infants having vallecular cysts encountered over a six-year period from 1992 to 1997 were reviewed. All of them presented with upper aerodigestive tract symptoms. Marsupialization was performed in three of them and CO2 laser excision was performed in the fourth patient. There was no recurrence of the cyst in any patient. One of them also had co-existing laryngomalacia. The degree of airway collapse caused by laryngomalacia improved after cyst removal. The laryngomalacia resolved spontaneously. cyst fluid culture was performed in one of the patients and yielded staphylococcus aureus but there was no other definite indicator of infection. staphylococcus aureus could also be isolated in the respiratory tract from two of the other patients. ( info) |