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Antiepiligrin cicatricial pemphigoid of the larynx successfully treated with a combination of tetracycline and niacinamide.

A case of antiepiligrin cicatricial pemphigoid that primarily involved the larynx and required a tracheostomy was studied. The diagnosis was based on the direct immunofluorescence findings of a biopsy specimen from the glottis, immunofluorescence using normal and 1M sodium chloride-split normal human skin as substrates, and immunoprecipitation. A dramatic clinical improvement was observed after the combined administration of tetracycline hydrochloride and niacinamide. The tracheal stoma could be shut after the narrow segment was cut by means of carbon dioxide laser therapy. The patient showed no respiratory difficulty during the 2-year follow-up period. The combined therapy of tetracycline and niacinamide is thus considered to be an effective treatment for various types of cicatricial pemphigoid.

Aged↗

The role of mitomycin in the prevention and treatment of scar formation in the pediatric aerodigestive tract: friend or foe?

OBJECTIVE: To evaluate the role of mitomycin in the prevention and treatment of scar formation in the pediatric aerodigestive tract. DESIGN: Prospective study; institutional review board-approved clinical trial. SETTING: Tertiary care pediatric medical center. PATIENTS: Fifteen patients; choanal atresia in 5 patients, airway stenosis in 8 patients, hypopharyngeal stenosis in 1 patient, and esophageal stenosis in 1 patient. OUTCOME: The efficacy and safety of mitomycin in the prevention of scar formation. INTERVENTION: All patients underwent surgical repair of the stenotic area, followed by topical application of mitomycin (1 mL of 0.4 mg/mL) for 4 minutes. RESULTS: Ten patients (67%) showed major improvement, 4 patients (27%) showed minor improvement, and 1 patient (7%) showed no improvement. CONCLUSION: Topical application of mitomycin can play an effective role in the prevention and treatment of scar formation in the aerodigestive tract.

Administration, Topical↗

Congenital high airway obstruction syndrome and airway reconstruction: an evolving paradigm.

OBJECTIVES: To refine the classic definition of, and provide a working definition for, congenital high airway obstruction syndrome (CHAOS) and to discuss the various aspects of long-term airway reconstruction, including the range of laryngeal anomalies and the various techniques for reconstruction. DESIGN: Retrospective chart review. PATIENTS: Four children (age range, 2-8 years) with CHAOS who presented to a single tertiary care children's hospital for pediatric airway reconstruction between 1995 and 2000. CONCLUSIONS: To date, CHAOS remains poorly described in the otolaryngologic literature. We propose the following working definition for pediatric cases of CHAOS: any neonate who needs a surgical airway within 1 hour of birth owing to high upper airway (ie, glottic, subglottic, or upper tracheal) obstruction and who cannot be tracheally intubated other than through a persistent tracheoesophageal fistula. Therefore, CHAOS has 3 possible presentations: (1) complete laryngeal atresia without an esophageal fistula, (2) complete laryngeal atresia with a tracheoesophageal fistula, and (3) near-complete high upper airway obstruction. Management of the airway, particularly in regard to long-term reconstruction, in children with CHAOS is complex and challenging.

Abnormalities, Multiple↗

Tracheostomy in preterm infants: current trends.

OBJECTIVE: To study the indications for and outcomes of tracheostomy in a population of preterm infants. DESIGN: Retrospective analysis of case records. SETTING: Two university-affiliated tertiary care children's hospitals. Patients We identified premature infants who required tracheostomies from January 1, 1997, through January 31, 2001. Information on weight, gestational age, comorbid conditions, indication for tracheostomy, and outcomes was collected. Infants were divided by birth weight into group 1 (<1000 g; n = 19 [very low birth weight]) and group 2 (> or =1000 g; n = 14). Comorbid conditions were scored and a total score was calculated for each patient. RESULTS: Group 1 had a higher incidence of patent ductus arteriosus, bronchopulmonary dysplasia, intraventricular hemorrhage, and retinopathy of prematurity. The incidence of congenital or genetic defects was equal in groups 1 and 2 (11 infants [58%] and 8 infants [57%], respectively). Group 1 had a higher average number of failed extubations (5.17 vs 3.18) and a higher oxygen requirement (48.7% vs 30.3%) compared with group 2. Weight at tracheostomy was essentially equal in groups 1 and 2 (3.6 vs 3.7 kg). Subglottic stenosis and laryngotracheomalacia were equally common findings in groups 1 and 2. The average comorbidity score for group 1 was higher than that for group 2 (6.7 vs 2.8). The most common indication for tracheostomy was ventilatory dependence (n = 24 [73%]), compared with airway obstruction (n = 6 [18%]) and pulmonary toilet (n = 3 [9%]). Overall, 6 patients (18%) had a complication related to the tracheostomy. CONCLUSIONS: Severity of pulmonary disease was the most significant factor associated with the need for tracheostomy in preterm infants. A tracheostomy can safely be performed in these infants with minimal morbidity.

Birth Weight↗

Controlled risk of stenosis after surgical excision of laryngeal hemangioma.

OBJECTIVE: To evaluate the risk of subglottic stenosis after surgical excision of congenital subglottic hemangioma. DESIGN: Retrospective analysis and case series. SETTING: Tertiary care teaching hospital. PATIENTS: A total of 13 pediatric patients diagnosed with subglottic hemangioma with unilateral, bilateral, or circular lesions and more than 50% airway obstruction between 1992 and 2001. INTERVENTION: Open surgical excision was performed as a single-stage procedure either as primary or secondary intention. The cricoid cartilage was left open at the end of the procedure. Postoperative intubation was carried out in a pediatric intensive care unit. MAIN OUTCOME MEASURE: An adequate airway after surgical excision. RESULTS: All patients were successfully extubated. No recurrence was noted. Three patients developed subglottic stenosis, two grade 1 and one grade 2. All 3 showed a favorable outcome and did not require reintubation. One needed endoscopic management of the stenosis. Of these 3 cases, 2 occurred after carbon dioxide laser treatment (out of 3) and 1 after circumferential dissection (out of 3). CONCLUSIONS: Extubation after surgery was successful in all cases of subglottic hemangioma. Risk of subglottic stenosis was limited and occurred only after circumferential dissection, especially if associated with prior traumatic laser damage of the hemangioma.

Airway Obstruction↗

A tissue-engineering technique for vascularized laryngotracheal reconstruction.

OBJECTIVE: To perform laryngotracheal reconstruction (LTR) using a vascularized neotracheal segment. DESIGN: A neotracheal segment was created within the sternocleidomastoid muscle. An anterior cricoid split procedure was performed using a pedicled, vascularized neotracheal segment. Results were compared with a control group that underwent anterior cricoid split using standard (avascular) autografted cartilage. Cross-sectional area, cartilage viability, extrusion, mucosalization, and wound healing were compared between groups. SUBJECTS: Sixteen female New Zealand white rabbits. INTERVENTIONS: Eight animals underwent placement of a cartilage-wrapped silicone implant into the sternocleidomastoid muscle. After 2 weeks, the silicone implant was removed, leaving a fibrovascular "foreign body" capsule and the interwoven autografted cartilage. The neotracheal segment was trimmed to create an anterior graft for LTR. The remaining animals underwent standard anterior graft LTR using autografted auricular cartilage. The reconstructed segments were harvested for comparison at 2 and 4 weeks. RESULTS: All reconstructed animals survived the postoperative period. No significant differences in stenosis rates or mucosalization were noted between groups. Two animals in the standard LTR group had microabscess formation, and no graft extrusions were encountered. CONCLUSION: A pedicled neotracheal graft can be used for anterior cricoid split procedures in rabbits.

Animals↗

The use of posterior cricoid grafting in managing isolated posterior glottic stenosis in children.

OBJECTIVE: To review our experience using posterior cricoid grafts to manage pediatric patients with isolated posterior glottic stenosis (PGS). DESIGN: Retrospective review over a 12-year period. SETTING: Tertiary care pediatric hospital. PATIENTS: All patients with isolated PGS treated between 1990 and 2002, in whom PGS was the dominant airway lesion and laryngotracheoplasty was required. Patients with concomitant vocal cord paralysis, a history of posterior laryngeal clefting, a Bogdasarian type I stenosis, or subglottic stenosis worse than grade I were excluded. MAIN OUTCOME MEASURES: Cause, operative intervention, decannulation rate, failure rate, and requirement for secondary procedures. RESULTS: A total of 29 patients ranging in age from 2 to 8 years were treated (21 with a history of prolonged intubation and 8 with a history of laryngeal trauma). Twenty patients had tracheotomies in place at the time of airway reconstruction and the remainder had stridor. Costal cartilage was the preferred graft material and was used in 27 patients. Six patients were referred with a diagnosis of bilateral vocal cord paralysis, but on evaluation were found to have PGS and mobile vocal cords. In 12 patients, repair was accomplished in a single-stage procedure; a suprastomal stent was placed in 17 patients. Overall decannulation rate was 97%, though a second procedure was required in 4 patients. One patient remained tracheotomy dependent; 4 had poor voice, including 2 with a history of laryngeal fracture; and 2 had late arytenoid prolapse. CONCLUSIONS: Isolated PGS in children is effectively managed with costal cartilage grafting of the posterior cricoid. This series has seen an evolution in management, with shorter stenting periods, placement of flanged posterior grafts without sutures, and graft placement without complete laryngofissure.

Adolescent↗

Pediatric cricotracheal resection: surgical outcomes and risk factor analysis.

OBJECTIVE: To identify risk factors for operation-specific outcomes of pediatric cricotracheal resection (CTR). DESIGN: We identified the first 100 consecutive children undergoing CTR at our institution from January 1, 1993, to December 31, 2004. Retrospective review of medical records provided data on demographics, operation dates, decannulation dates, and proposed risk factors, including age, stenosis grade, vocal cord function, Down syndrome, history of distal tracheal surgery, history of open laryngotracheal surgery, presence of tracheotomy at the time of operation, use of suprahyoid release, extended CTR, and use of chin-to-chest sutures. Complete data sets were available for 93 patients. We performed multivariable logistic regression analysis to identify significant independent risk factors. SETTING: A tertiary care children's hospital. PATIENTS: All patients younger than 18 years who underwent CTR at our institution. MAIN OUTCOME MEASURES: Operation-specific and overall decannulation rates. RESULTS: Results of the preoperative evaluation showed grade III or IV stenosis in 89 patients (96%). The overall decannulation rate included 87 patients (94%); the operation-specific decannulation rate, 66 patients (71%). The only significant risk factor for failure to decannulate after 1 operation was the presence of unilateral or bilateral vocal cord paralysis (P = .007). CONCLUSIONS: Cricotraceal resection may be safely performed in patients with multiple airway lesions. Patients with a history of vocal cord paralysis who undergo CTR often require more than 1 open airway procedure for decannulation and should be counseled appropriately. This study represents the largest reported series of pediatric CTR.

Adolescent↗

Fibroblast transplantation in the airway: implications for subglottic stenosis.

OBJECTIVE: Because subglottic stenosis (SGS) represents one of the most challenging pathologies confronting the pediatric otolaryngologist, our laboratory is investigating the role fibroblasts play in mucosal scar formation in the course of SGS development. Our objective is to establish cell transplantation into the subglottic mucosal wound bed as a viable tool for examining the cellular processes that underlie the development of SGS. DESIGN: A series of 2 animal experiments, with animals assigned to a control, vehicle-only, or cell-treated group. SETTING: John G. Rangos Sr Research Center, Children's Hospital of Pittsburgh, Pittsburgh, Pa. SUBJECTS: Twenty-six New Zealand white rabbits. This animal model has been well established in the study of SGS formation. INTERVENTIONS: Fluorescently labeled exogenous fibroblasts were transplanted into the injured subglottis of the rabbits. RESULTS: Exogenous fibroblasts derived from fetal and adult dermis and subglottic mucosa were successfully transplanted into the injured subglottic mucosa of adult rabbits. Transplanted fibroblasts survived into the latter stages of wound healing (at 14 and 21 days) and appeared to be associated with a mild inflammatory cell influx and active remodeling of the mucosal wound bed. CONCLUSION: Cell transplantation is a viable tool for the study of fibroblast activity in the mucosal wound bed.

Animals↗

Laryngotracheal consequences of pediatric cardiac surgery.

OBJECTIVE: To determine the incidence and character of clinically significant laryngotracheal anomalies in pediatric patients undergoing surgical repair of congenital cardiac defects at a tertiary care center. DESIGN: Single-center retrospective review. PATIENTS: The charts of pediatric patients who required surgical treatment for congenital heart disease over a 4-year period were reviewed. Forty-eight of 1957 patients were seen in inpatient consultation by the otolaryngology service. The parameters studied included cardiac diagnosis, reason for consultation, findings on examination, and follow-up. RESULTS: There were 16 (33%) cases of subglottic stenosis, which were graded according to the Cotton-Myer classification system as follows: grade 1 (n=8); grade 2 (n=3); and grade 3 (n=5). Three of the 16 patients with subglottic stenosis required tracheotomy and 4 required laryngotracheal reconstruction. Nine (19%) of the 48 patients were diagnosed as having unilateral true vocal cord paralysis and 3 (6%) as having bilateral paralysis. With the exception of 1 patient, all patients with true vocal cord paralysis on the left side had undergone repair of the aortic arch. CONCLUSIONS: Pediatric patients with congenital cardiac disease are predisposed to laryngeal anomalies owing to (1) frequent intubation, (2) prolonged ventilatory support, and (3) recurrent laryngeal nerve injury. In our patients, subglottic stenosis was the most common laryngeal abnormality. When recognized early, in the eschar phase, most of these cases can be managed with sequential endoscopic debridement, which is conceptually similar to debridement that is performed after functional endoscopic sinus surgery. Established stenosis requires more vigorous intervention, the invasive degree of which depends on the length and circumference of the narrowing. Unilateral vocal paralysis tends to be a self-limited problem, while an elegant solution to bilateral paralysis remains elusive.

Child↗

Cricotracheal resection in children weighing less than 10 kg.

OBJECTIVE: To review cricotracheal resection (CTR) in children weighing less than 10 kg. DESIGN AND SETTING: Retrospective study of 17 patients (mean follow-up, 23 months) from 3 ear, nose, and throat pediatric centers. PATIENTS: Seventeen children (10 boys and 7 girls; mean age, 14.6 months; and mean weight, 7.6 kg) undergoing CTR from June 1995 to March 2003. MAIN OUTCOME MEASURES: Decannulation rates and endoscopies. RESULTS: The cause was congenital subglottic stenosis in 2 children (12%) and acquired subglottic stenosis in 15 (88%). All but 1 had grade 3 or 4 stenosis. The mean hospitalization duration was 34 days. Single-stage CTR was performed in 11 children (65%), with peroperative decannulation in 7. Extubation of these patients occurred between days 3 and 9. Decannulation of the other 6 patients was performed after a median of 15 days. Sixteen (94%) of the 17 children were decannulated. Four patients required additional carbon dioxide laser treatment for subsequent glottic or subglottic edema or granulomas, but no reintubation was necessary. One child could not be decannulated because of bronchopulmonary disease, and subglottic stenosis recurred. Long-term tracheotomy was avoided in all other patients. Another child died of cardiac disease. All other patients remained free of significant subglottic stenosis at follow-up. CONCLUSIONS: Cricotracheal resection in small children weighing less than 10 kg was a safe and effective procedure for severe subglottic stenosis. To our knowledge, this is the first reported attempt of CTR in this weight category, providing results comparable to those published in older children.

Body Weight↗

Prevention of anterior glottic stenosis after bilateral vocal fold stripping with mitomycin C.

OBJECTIVE: To examine the effects of immediate application of topical mitomycin C (MMC) on the prevention of anterior glottic stenosis (AGS) after microsurgical stripping of both vocal folds, including the anterior commissure, in a canine model. DESIGN: Prospective randomized experimental study. INTERVENTIONS: Twelve canine larynges were injured by a stripping procedure of both entire membranous vocal folds. The dogs were randomly divided into 2 groups for treatment with 1.0 mg/mL of MMC or with isotonic sodium chloride solution (control) for 5 minutes immediately after surgery. Three and 6 weeks after surgery, the glottic webs were lysed and repeatedly treated with MMC or isotonic sodium chloride solution. The glottic wound healing and AGS formation were examined every week. Ten weeks after the initial surgery, all larynges were collected and examined histologically. RESULTS: The stripping procedure induced AGS, affecting 58% to 86% (mean, 72%) of the length from the anterior commissure to the vocal process in the control group. The application of MMC at the time of initial surgery significantly lowered the incidence and extent of the web formation (P = .004). The AGS lesions were resolved by web lysis and treatment with MMC, without significant local adverse effects. Histological staining for collagen and elastin revealed that MMC treatment did not induce excessive fibrotic or atrophic changes in the lamina propria of the vocal folds. CONCLUSION: Bilateral stripping of the membranous vocal folds induces significant AGS, which can be minimized by use of MMC at initial surgery.

Alkylating Agents↗

Bacterial colonization of airway stents: a promoter of granulation tissue formation following laryngotracheal reconstruction.

OBJECTIVE: To investigate whether airway granulation, a common occurrence during laryngotracheal reconstructive surgery and a common cause of delays in definitive treatment and treatment failure, is associated with a microbial etiology. DESIGN: Prospective case-control study. SETTING: Tertiary referral airway reconstruction unit. PATIENTS: Patients who had an airway stent as part of their treatment for laryngotracheal stenosis. INTERVENTIONS: All airway stents were sent for microbiological analysis. Information about patient demographics, lesion characteristics, and presence of airway granulation tissue at different times during treatment were obtained and correlated against the microbiological findings from airway stents. MAIN OUTCOME MEASURES: A chi2 test was used to correlate airway colonization with specific pathogens and occurrence of airway granulation. Logistic regression analysis was used to identify independent microbiological predictors of airway granulation. RESULTS: Thirty-one airway stents were removed from 26 patients. The mean (SD) age at presentation was 42 (18) years, and postintubation tracheal stenosis was the most common etiology. There were highly significant associations between stent colonization with Staphylococcus aureus and Pseudomonas aeruginosa and the occurrence of airway granulation (P<.02), and these microorganisms were independently associated with the risk of developing airway granulation. Furthermore, S aureus was associated with persistence of airway granulation on average 4 months following removal of the stent. CONCLUSIONS: Airway granulation seems to be associated not with polymicrobial airway colonization but with infection with specific pathogenic microorganisms. All patients undergoing laryngotracheoplasty should receive antibiotic prophylaxis to cover these microorganisms, and the development and use of antibiotic-impregnated airway stents should be explored.

Adult↗

Anterior and posterior cartilage graft dimensions in successful laryngotracheal reconstruction.

OBJECTIVE: To describe the dimensions of cartilage grafts used for successful laryngotracheal reconstruction, with the goal of establishing appropriate sizes for "off-the-shelf" tissue-engineered cartilage grafts. DESIGN: A retrospective review of prospectively maintained operative illustrations of a single surgeon's experience. SETTING: Two tertiary children's hospitals. PATIENTS: A consecutive sample of 54 patients (tracheotomized or intubated) with a diagnosis of subglottic stenosis. INTERVENTIONS: Each patient underwent anterior (n = 30), posterior (n = 3), or anterior and posterior (n = 22) laryngotracheal reconstruction. Rib cartilage was used in 51 patients and thyroid cartilage was used in 3 patients. MAIN OUTCOME MEASURE: Successful or failed extubation. RESULTS: Of the 54 patients, 48 (89%) were successfully decannulated. The mean +/- SEM length of the anterior graft was 20.7 +/- 10.3 mm, and the mean width of the anterior graft was 7.7 +/- 2.5 mm. The mean length of the posterior graft was 13.9 +/- 2.9 mm, and the mean width of the posterior graft was 4.2 +/- 0.9 mm. CONCLUSIONS: With the prospect of tissue-engineered cartilage implants becoming available for laryngotracheal reconstruction, the most appropriate templates for designing these implants should be based on the geometric dimensions of grafts carved from native tissues in cases that have been successfully decannulated. Based on our analysis, the use of 2-mm increments for the posterior grafts suggests a set of molds that are 2, 4, and 6 mm wide and 22 mm long. Using 2 x 2-mm increments for the anterior grafts indicates that 36 mold sizes will be sufficient for 90% of predicted cases.

Cartilage↗

Laryngeal stenosis.

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Constriction, Pathologic↗