11/45. Cicatricial pemphigoid in the upper aerodigestive tract: diagnosis and management in severe laryngeal stenosis.Pemphigoid is a group of rare, acquired, autoimmune subepithelial blistering diseases. The condition has been subclassified into bullous pemphigoid and cicatricial pemphigoid (CP). diagnosis is based on clinical presentation, evidence of subepithelialvesicles or bullae on routine histologic analysis, and direct and indirect immunofluorescence studies. Cicatricial pemphigoid is characterized by linear deposition of immunoreactants, principally IgG and complement factor 3, along epithelial basement membranes. Cicatricial pemphigoid usually leads to mucosal scarring. We present a case of severe CP that led to laryngeal and subglottic stenosis and involvement of both eyes and the oral, nasal, and nasopharyngeal mucosae. Treatment with dapsone, corticosteroids, azathioprine sodium, cyclosporine A, cyclophosphamide, methotrexate sodium, and mycophenolate mofetil between 1997 and 2001 only resulted in temporary disease control. The patient has been treated with leflunomide for the past 8 months, and there have been no relapses. Treatment of CP with leflunomide has not been described in the literature until now.- - - - - - - - - - ranking = 1keywords = upper (Clic here for more details about this article) |
12/45. Double trouble: prolapsing epiglottis and unexpected dual pathology in an infant.A 3-week-old full-term female neonate was admitted with a 4-day history of episodic stridor, desaturations and difficult feeding. Initial assessment using fluoroscopy suggested distal tracheomalacia. Inhalational induction for examination under anaesthesia of the upper airway at 4 weeks of age caused almost complete airway obstruction due to severe anterior, or epiglottic, laryngomalacia. This airway obstruction was unresponsive to continuous positive airway pressure, the use of an oropharyngeal airway and hand ventilation and required urgent tracheal intubation using suxamethonium. Epiglottopexy, a relatively unknown procedure, was performed uneventfully 2 days later, with complete relief of the respiratory compromise. However, the infant remained desaturated postoperatively. A ventilation perfusion scan subsequently revealed multiple pulmonary arteriovenous malformations, unsuitable for embolization and requiring nocturnal home oxygen therapy. review at 3 months of age found a thriving infant with no airway obstruction and good epiglottic positioning on examination under anaesthesia. Although the patient's oxygen requirements had diminished, the long-term outcome remains uncertain.- - - - - - - - - - ranking = 0.25keywords = upper (Clic here for more details about this article) |
13/45. The perinatal management of severe laryngeal stenosis.BACKGROUND: Conditions that completely occlude the fetal larynx are rare. We describe the perinatal course of an infant with severe congenital subglottic stenosis, for whom the prenatal recognition of airway occlusion led to enhanced neonatal management. CASE: Serial ultrasound examinations from 23-37 weeks' gestation led to the diagnosis of upper airway obstruction in the fetus of a 29-year-old woman. Significant findings included fetal ascites, echogenic enlarged lungs, and a dilated fluid-filled trachea. The infant was delivered in a level III hospital with a neonatologist and anesthesiologist in attendance. The cord was not clamped until a tracheostomy was secured. CONCLUSION: Upper-airway obstruction can be diagnosed prenatally by the presence of secondary manifestations. infant survival may depend on foreknowledge of the pathology and prompt, directed efforts at bypassing the airway obstruction.- - - - - - - - - - ranking = 0.25keywords = upper (Clic here for more details about this article) |
14/45. Tracheal sounds in upper airway obstruction.A boy with subglottic narrowing secondary to laryngotracheitis presented with noisy breathing. Acoustic measurements of tracheal sounds at standardized air flows correlated well with the clinical course and with spirometric assessments. This indicates the potential value of respiratory sound characterization in patients with upper airway obstruction.- - - - - - - - - - ranking = 3.919998077419keywords = breathing, upper (Clic here for more details about this article) |
15/45. Congenital subglottic stenosis in two unrelated pairs of siblings.Subglottic stenosis in infants is mostly acquired (secondary) and caused by granulation tissue or submucosal mucous gland hyperplasia after prolonged endotracheal intubation. Subglottic stenosis may also be congenital (primary), and it usually occurs sporadically. There are some reports of its association with inherited anomalies, but there are no previous reports of its familial occurrence in otherwise healthy children. This report describes two pairs of siblings referred for acute inspiratory stridor in whom subglottic stenosis was diagnosed by endoscopy. They were all born at term, and their parents were unrelated. One child had an anteriorly located anus but no other abnormalities. CONCLUSION: Without any surgical intervention all had normal breathing at rest, but inspiratory stridor during respiratory infections and upon physical exercise at follow-up 4-9 y later.- - - - - - - - - - ranking = 2.669998077419keywords = breathing (Clic here for more details about this article) |
16/45. Vascular-pedicled costal cartilage graft for the treatment of subglottic and upper tracheal stenosis.BACKGROUND/PURPOSE: Free costal cartilage graft for the treatment of subglottic and tracheal stenosis is widely used, but postoperative granulation formation is a problem. To reduce the risk of granulation formation after free costal graft, a new operation of costal cartilage graft with vascular pedicle was introduced. methods: A vascular pedicled fifth costal cartilage graft is prepared using internal thoracic artery and vein and intercostal artery and vein as a vascular pedicle. The prepared graft is brought to the upper trachea. The anterior wall of cricoid is split, and the costal cartilage graft is implanted to the split part and tracheostomy. Extubation on the next day is possible if the general condition of the patient permits. RESULTS: In 3 cases of subglottic or upper tracheal stenosis, this operation was performed. All the patients had tracheostomy made during early infancy. The postoperative course was uneventful, and all the patients were extubated soon after the operation. No granulation tissue was observed by postoperative bronchoscopic examinations. CONCLUSIONS: Costal cartilage graft with vascular pedicle is a safe and useful new operation for the treatment of subglottic and upper tracheal stenosis. There also is a possibility of using this procedure for the treatment of long segment tracheal stenosis.- - - - - - - - - - ranking = 1.75keywords = upper (Clic here for more details about this article) |
17/45. Fetal laryngeal stenosis/atresia and congenital high airway obstructive syndrome (CHAOS): a case report.Congenital high airway obstructive syndrome (CHAOS) is a rare but fatal disease with predictably characteristic features including stenotic or atretic upper airway, hyperplastic lungs, elevated diaphragm, massive fetal ascites and fetal hydrops. diagnosis of CHAOS by ultrasound scan is possible and clinically important since advanced intrauterine surgery to correct the defect is possible. We report a case of fetus of CHAOS with massive ascites, pulmonary hyperplasia and laryngeal stenosis/atresia. We feel that it is important to recognize the entity both by ultrasound scan and by the pathologist so that some cases can be corrected by intrauterine fetal surgery.- - - - - - - - - - ranking = 0.25keywords = upper (Clic here for more details about this article) |
18/45. Postmortem diagnostics using MSCT and MRI of a lethal streptococcus group A infection at infancy: a case report.Postmortem cross-sectional imaging using multislice computed tomography (MSCT) and magnetic resonance imaging (MRI) was considered as a base for a minimal invasive postmortem investigation in forensic medicine such as within the Virtopsy approach. We present the case of a 3-year-old girl with a lethal streptococcus group A infection and the findings of postmortem imaging in this kind of natural death. Postmortem MSCT and MRI revealed an edematous occlusion of the larynx at the level of the vocal cords, severe pneumonia with atelectatic parts of both upper lobes and complete atelectasis of both lower lobes, purulent fluid-filled right main bronchus, enlargement of cervical lymph nodes and pharyngeal tonsils, and additionally, a remaining glossopharyngeal cyst as well as an ureter fissus of the right kidney. All relevant autopsy findings could be obtained and visualized by postmortem imaging and confirmed by histological and microbiological investigations supporting the idea of a minimal invasive autopsy technique.- - - - - - - - - - ranking = 0.25keywords = upper (Clic here for more details about this article) |
19/45. Use of a ProSeal laryngeal mask airway and a Ravussin cricothyroidotomy needle in the management of laryngeal and subglottic stenosis causing upper airway obstruction.We report the successful use of a ProSeal laryngeal mask airway (PLMA) as a dedicated airway to allow fibre-optic inspection and passage through a tightly stenosed glottic and subglottic lesion, before fibre-optic-guided transtracheal placement of a Ravussin needle and jet ventilation. The described technique avoided both tracheostomy and the potential of 'seeding' the tumour by passage of the needle through the mass. The PLMA may be a useful 'dedicated airway' and has several advantages over the classic LMA(double dagger) and intubating LMA when used for this purpose. These include improved airway seal and reduced risk of aspiration. Four other cases of use of the PLMA as a dedicated airway during management of difficult airways are discussed. double daggerLMA is the property of Intavent Ltd.- - - - - - - - - - ranking = 1keywords = upper (Clic here for more details about this article) |
20/45. Successful treatment of laryngeal stenosis in laryngo-onycho-cutaneous syndrome with topical mitomycin C.Laryngo-onycho-cutaneous syndrome is a very rare entity found in Punjabi families. It affects the skin, nails, and larynx. Laryngeal involvement may cause lethal airway obstruction, and has in the past proved very difficult to treat. mitomycin C is an antibiotic that acts as an alkylating agent, inhibiting dna synthesis. It reduces fibroblast proliferation, and has previously been used to treat choanal atresia and laryngeal stenosis. We report an 18-year-old man with complete transglottic laryngeal stenosis secondary to laryngo-onycho-cutaneous syndrome. An airway was established by dissection with a bougie and sickle knife, and was initially maintained by the upper limb of a Montgomery T-tube. Laryngeal granulation tissue present on removal of the T-tube was treated with topical mitomycin C (2 mg/mL) applied for 4 minutes on two occasions with an interval of 1 month. A year later, the airway remained patent, with no granulation tissue.- - - - - - - - - - ranking = 0.25keywords = upper (Clic here for more details about this article) |
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