Letter: Transtracheal ventilation.
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Humans↗
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Results for “LARYNGOSTENOSIS”
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Progressive laryngotracheal stenosis with short stature and arthropathy.
Laryngotracheal stenosis is rare in adults, especially in the absence of a malignancy. It is most commonly caused by fibrosis following endotracheal intubation or tracheal trauma. Other conditions causing progressive airway narrowing include the mucopolysaccharidoses and autoimmune disorders. With the exception of storage diseases, there are no well-defined genetic disorders with progressive airway narrowing as a common complication. We have evaluated three unrelated individuals with this potentially life-threatening finding, all of whom have a previously unrecognized condition. Each patient had short stature and joint stiffness with no evidence for infectious, inflammatory, or metabolic diseases as a cause of their condition. None of our patients had clinical findings indicative of known skeletal dysplasias or storage diseases. They had minor facial anomalies which included deeply set eyes, bushy eyebrows, and flat midface. Given the unique findings of our patients including adult onset critical tracheal stenosis, short stature, progressive joint limitation, and distinct facial anomalies, we conclude that they have a previously undescribed condition.
Abnormalities, Multiple↗
Idiopathic subglottic stenosis in a nine-year-old boy: diagnosis and management.
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Child↗
Acute leukemia presenting as laryngeal obstruction.
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Aged↗
Endoscopic laser surgery in the treatment of radiation failure of early laryngeal carcinoma.
BACKGROUND: This study was undertaken to analyze the local control rates and survival of patients undergoing endoscopic laser surgery for squamous cell carcinoma of the larynx as a salvage procedure for recurrent tumors after radiotherapy. METHODS: Twenty-four patients underwent endoscopic laser surgery for radiation failure at our hospital from 1988 to 1995. All patients were men, between 45 and 88 years old. The localization of the recurrence was in the supraglottis in 3 cases and in the glottis in 21 cases. The postirradiation recurrences were staged rT1 in 18 patients and rT2 in 6 patients. RESULTS: Local recurrence after laser surgery developed in six patients (25%) who had a total laryngectomy. Voice preservation was thus achieved in 75% of patients. No patient died because of the laryngeal cancer, and the observed 5-year survival rate was 76%. CONCLUSIONS: These results indicate that in selected cases, endoscopic laser surgery may be used successfully for treating radiation failure of early laryngeal carcinomas.
Actuarial Analysis↗
Long-term outcome of surgically treated acquired subglottic stenosis in infancy.
There is little information about the long-term outcome of infants with acquired severe subglottic stenosis (SGS) who require surgical intervention. We retrospectively identified infants with acquired subglottic stenosis who required anterior cricoid split (ACS) or tracheostomy for primary airway management; some of these children later required laryngotracheoplasty (LTP). All children were treated at our hospital from 1989-1997. During follow-up, we assessed patients for persistent symptoms (stridor at rest, exercise limitation, difficulty with respiratory tract infections, recurrent croup, and voice alteration), and we measured lung function when possible. We identified 34 infants with acquired SGS: 13 treated primarily with tracheostomy, and 21 with ACS. Nine patients could not be extubated following ACS and required tracheostomy, while the 12 who were extubated were followed up at a mean time of 76 months postoperatively; 3 had moderate stridor at rest, 1 moderate exercise limitation, and none had recurrent croup. Four of 5 who had lung function measured had moderately severe extrathoracic airflow limitation. From the tracheostomy group (n = 13) and the failed ACS group (n = 9), 2 patients were decannulated without further surgery, 17 underwent LTP, and 2 have LTP planned. Fifteen of the 17 patients who had LTP have been decannulated. Follow-up, at a mean time of 58 months postoperatively, showed none with stridor at rest, 3 with moderate exercise limitation, none with recurrent croup, and 2 with moderate voice alteration. All 5 patients who had lung function measured had airflow limitation, 1 being severe. In conclusion, ACS facilitates extubation in selected patients with severe, acquired SGS of infancy, and the long-term outcome of patients successfully extubated is excellent. Failure of ACS invariably means tracheostomy, and subglottic repair by LTP is associated with a good long-term outcome.
Airway Obstruction↗
Confirmation of existence of a new syndrome: LAPS syndrome.
In l998, Hopkin et al. described a new disorder characterized by progressive laryngotracheal stenosis, short stature, and arthropathy. We provide extensive clinical information on two additional unrelated patients with this distinctive disorder and propose the name LAPS syndrome, to reflect the most consistently recognized features, namely, Laryngotracheal stenosis, Arthropathy, Prognathism, and Short stature.
Abnormalities, Multiple↗
Pachyonychia Congenita (Jadassohn-Lewandowsky syndrome): a seventeen-member, four-generation pedigree with unusual respiratory and dental involvement.
Pachyonychia Congenita (PC) is an autosomal dominant syndrome of thick nails and other epithelial defects. A hospitalization for severe respiratory distress in a 3-year-old boy with PC and an affected father led to the discovery of 17 affected persons in a kindred spanning four generations. Nine relatives had varying degrees of upper respiratory tract obstruction, and eleven had dental aberrations. We review PC and discuss other unusual findings in this kindred, ie, arthritis in four affected relatives and a discrepancy between expected and observed ratios of affected to unaffected offspring of mothers with PC.
Adult↗
Two lethal chondrodysplasias with giant chondrocytes.
Two infants, each with a different form of sporadic lethal chondrodysplasia, had multinucleated giant chondrocytes in resting cartilage. Both patients also had severe laryngeal stenosis and normal chromosomes. The radiographic abnormalities in one patient conformed to the recently delineated entity, atelosteogenesis (spondylohumerofemoral hypoplasia or giant cell chondrodysplasia); the other patient had an unclassified type of spondyloepiphyseal dysplasia. The findings in these two patients and the previously reported observations in spondylohumerofemoral dysplasia confirm that chondrocytic giant cells are a causally nonspecific phenomenon.
Cartilage↗
VATER/VACTERL association: clinical variability and expanding phenotype including laryngeal stenosis.
Vertebral abnormalities and anorectal and tracheoesophageal defects are the main manifestations in the VATER/VACTERL association. Radial defects vary from radial aplasia to thumb duplication. Heart and renal defects are reported with lower frequency. Additional malformations, such as the laryngeal stenosis described in the present patient, may expand the phenotype of the association. The wide spectrum of congenital abnormalities confirms the high clinical variability of VATER/VACTERL association which seems to be due to a disruption of blastogenesis.
Esophageal Atresia↗
Pulmonary hyperplasia in the Fraser cryptophthalmos syndrome.
We report on 2 sibs with the Fraser cryptophthalmos syndrome who had pulmonary hyperplasia and laryngeal stenosis. A third unrelated patient with Fraser syndrome had laryngeal stenosis, renal agenesis, and normal lung development, rather than the expected pulmonary hypoplasia. Three additional cases of pulmonary hyperplasia in the Fraser syndrome were ascertained from a review. In all of these cases the likely mechanism for pulmonary hyperplasia is retention of fetal lung fluid by laryngeal or tracheal obstruction.
Abnormalities, Multiple↗
Clinical features and therapeutic management of subglottic stenosis in patients with Wegener's granulomatosis.
OBJECTIVE: To determine the clinical features and optimal treatment of subglottic stenosis (SGS) in patients with-Wegener's granulomatosis (WG). METHODS: Review of 43 patients with SGS and treatment of 20 patients with intratracheal dilation-glucocorticoid injection therapy. RESULTS: SGS developed in 43 of 189 patients with WG who were followed up at the National Institutes of Health Clinical Center. The diagnosis of SGS occurred in the absence of other features of active. WG in 21 of 43 patients (49%). In 21 patients (49%), SGS began while the patient was receiving systemic immunosuppressive therapy for disease activity involving other sites. Tracheostomy was required in 10 of 18 patients (56%) who were treated with systemic immunosuppressive therapy. In 20 patients treated with intratracheal therapy, none required tracheostomy and 6 with previous tracheostomies were decannulated. CONCLUSION: SGS often occurs independently of other features of active WG and is frequently unresponsive to systemic immunosuppressive therapy. Intratracheal dilation-injection therapy provides a safe and effective treatment for WG-associated SGS and, in the absence of major organ disease activity, should be used without concomitant systemic immunosuppressive agents.
Adult↗
Anatomical aspects of postintubational subglottic stenosis.
The subglottic regions of 54 human adult male and female larynges were studied with regard to anatomical aspects of postintubational stenosis. Fourteen specimens were impregnated with curable polymers and cut into 600-800 microns sections along different planes. Forty formalin-fixed hemilarynges were dissected. Measurements of the upper cricoid lamina and the thickness of the endocricoid soft tissues were taken for statistical analysis. Immediately beneath the glottis, the upper part of the cricoid lamina consists of two lateral plates with an average angle of 110 degrees. Distally, the cricoid adopts a more and more rounded lumen. At the level of the cricothyroid joint, the definite airway lumen is always laterally narrowed by a prominent thickening of the endocricoid soft tissue. Large amounts of loose connective tissue facilitate the development of edema in case of injury in this region. Dorsally, the submucous stratum is smaller and consists mainly of dense connective tissue. The blood vessels are fixed to the cricoid perichondrium by collagenous fibers. Any pressure applied from the airway lumen will force the vessels against the nonresilient cartilage, resulting in occlusion and ischemia. These pathophysiologic mechanisms are important for the development of early laryngeal damage during endotracheal intubation, possibly resulting in posterior stenosis due to scarring later on.
Aged↗
Single-stage surgical repair of benign laryngotracheal stenosis in adults.
BACKGROUND: Benign laryngotracheal stenosis causes considerable morbidity. In a retrospective study, we describe the results of our surgical treatment. METHODS: Between June 1999 and June 2002, 14 adults with laryngotracheal stenosis were referred to our hospital. Stenosis resulted from mechanical ventilation in 11 patients, from Wegener's granulomatosis in 2 patients, and from strangulation in 1 patient. Eleven patients had a tracheotomy. One patient was found unfit for surgery. Nine patients underwent cricotracheal resection (CTR) with end-to-end anastomosis, and four patients underwent single-stage laryngotracheoplasty (SS-LTP) without stenting. RESULTS: There were no perioperative deaths. Patients were extubated after mean of 3 days (range, 0-10 days; CTR 2.3 days vs SS-LTP 3.5 days, p=.45). There were in-hospital complications in five patients. Mean hospital stay was 19 days (range, 8-53 days; after CTR 24 days vs SS-LTP 9 days, p=.015). With regard to airway patency and voice recovery, 10 patients (77%) had good results, including 1 patient with two readmissions, and 3 (23%) had satisfactory results, including 1 patient with 11 additional nonsurgical interventions. CONCLUSIONS: Benign laryngotracheal stenosis in the adult patient can be repaired successfully using a strategy of two single-stage surgical procedures. All patients had good or satisfactory functional results. A multidisciplinary approach was essential to achieve these good results.
Adult↗
Supracricoid laryngectomy with cricohyoidoepiglottopexy for advanced glottic cancer.
BACKGROUND: Supracricoid laryngectomy with cri-cohyoidoepiglottopexy (CHEP) is a conservative surgical procedure indicated in selected cases of advanced glottic carcinoma. METHODS: This study is a review of our experience with 43 patients with T3/T4 glottic squamous cell carcinoma who underwent CHEP in our institution. All but two patients underwent selective neck dissections. All patients were staged on the basis of the 2002 TNM classification. Rates of recurrence and death were estimated by the Kaplan-Meier method. RESULTS: The 5-year disease-specific survival and 5-year relapse-free survival rates were 78% and 83%, respectively. Neck metastases were found in three patients. Cartilage invasion occurred in 11 cases. The average length of hospital stay was 5.7 days. The mean time of enteral feeding tube was 33.8 days, and the mean time for tracheotomy was 29.6 days. Overall, normal swallowing was achieved in 74.4% of patients. Eleven patients had mild and major complications. Laryngeal stenosis emerged as the most frequent major complication. Three patients (6.9%) had local recurrences. Two patients (4.6%) had neck metastases. CONCLUSIONS: On the basis of this study, over a 7-year period with 43 patients with advanced glottic cancer, a successful on-cologic outcome is confirmed.
Aged↗
Laryngotracheal stenosis: a 5-year review.
Twenty-seven children with varying degrees of laryngotracheal stenosis were treated at Texas Children's Hospital (TCH) from January 1984 to January 1989. Nineteen children had a residual airway demonstrable preoperatively, and following laryngotracheoplasty, 18 (95%) were successfully decannulated; all but 1 had a normal voice. Eight children had complete stenosis. Of these children, 6 could be decannulated, but 3 required a second laryngotracheoplasty. Four children now have voice abnormalities.
Child↗
Aneurysmal bone cyst of the cricoid cartilage: an unusual cause of subglottic stenosis.
Aneurysmal bone cysts (ABC) are locally aggressive bony tumors which rarely occur in the head and neck. Only 3 cases have been reported in the larynx previously, 1 involving the cricoid cartilage. A patient is presented who developed subglottic stenosis with airway compromise due to an ABC arising from the cricoid cartilage. The literature on bony tumors of the laryngeal cartilages was reviewed and the clinical and pathologic findings in this case highlighted.
Adult↗
T-tube modification to decrease the risk of plugging.
Laryngotracheal stenosis is a recognized complication of local injuries, such as prolonged intubation. Surgical reconstruction with the use of a tracheal T-tube is widely recommended. We report a serious complication consisting of the sudden obstruction of the T-tube by viscous secretions. In order to prevent this undesirable consequence, a simple modification of the T-tube technique is proposed.
Airway Obstruction↗