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Objective We have used cardiopulmonary bypass with left pulmonary artery reimplantation for pulmonary artery sling repair since 1985. This review presents our current results with this technique, emphasizing the importance of diagnosis and treatment of frequently associated tracheal stenosis. Methods Since 1985, 34 patients have undergone pulmonary artery sling repair using a median sternotomy and cardiopulmonary bypass. Age ranged from 9 days to 43 years (mean 2.1 ± 7.5 years, median 0.2 years). Twenty-seven patients (79%) had tracheal stenosis secondary to complete cartilage tracheal rings. All patients had preoperative airway imaging with rigid bronchoscopy, and since 2000 all patients have had computed tomography imaging of the chest with 3-dimensional reconstruction (n = 14). Tracheal repair has included pericardial patch tracheoplasty (n = 7), tracheal autograft (n = 10), tracheal resection (n = 4), and slide tracheoplasty (n = 5). All patients had an echocardiogram, and cardiac lesions repaired simultaneously included atrial septal defect (4), tetralogy of Fallot (2), and ventricular septal defect (1). One patient had a severely hypoplastic right lung, and 3 patients had an absent right lung. In these patients, the left pulmonary artery was translocated anterior to the trachea. In all other patients, the left pulmonary artery was reimplanted into the main pulmonary artery. Results There have been no early deaths or complications related to the use of cardiopulmonary bypass. Median hospital stay was 24 days. There have been 4 late deaths. Two late deaths were the result of complications of tracheal surgery (1 pericardial patch [6 months postoperatively] and 1 autograft [1.7 years postoperatively]). One child died of biliary atresia (0.2 years postoperatively), and 1 child died of pneumonia (5.8 years postoperatively). All recent tracheal stenosis repairs have been with slide tracheoplasty. All left pulmonary arteries are patent with a mean percent flow by perfusion scan of 41% ± 13%. Older patients (n = 2) have noted a significant improvement in exercise tolerance. Conclusions Pulmonary artery sling is best repaired with median sternotomy, cardiopulmonary bypass, and left pulmonary artery reimplantation. This resulted in uniformly patent left pulmonary arteries in all patients. Preoperative computed tomography imaging, echocardiography, and bronchoscopy are essential for precise operative planning. The frequently associated tracheal stenosis is best repaired with slide tracheoplasty.

Background Infants with congenital tracheal stenosis may also have unilateral lung agenesis or severe lung hypoplasia. The purpose of this review is to evaluate our results with these patients and compare their presentations and outcomes to those of tracheal stenosis patients with two lungs. Methods Our database was queried for patients undergoing tracheal stenosis repair since 1982. Patients were divided into two groups based on pulmonary anatomy of single lung (SL = unilateral lung agenesis or severe hypoplasia) or two lungs (BL = bilateral lungs) and analyzed to compare presentation and outcomes. Results From 1982 to 2008, 71 patients had tracheal stenosis repair. Bilateral lungs were present in 60 patients; 9 patients had an absent (4) or severely hypoplastic (5) right lung, and 2 patients had an absent left lung (SL = 11). Age at repair was similar between groups; median age 0.42 years in the SL group (mean 0.80 ± 1.0 years) versus 0.37 years in the BL group (mean 0.91 ± 2.1 years, p = not significant [ns]). In the SL group 8 of 11 (73%) were intubated preoperatively versus 15 of 60 (25%) in the BL group ( p = 0.004). In the SL group 4 of 11 (36%) patients had pulmonary artery sling versus 20 of 60 (33%) of BL patients ( p = ns). In the SL group 2 of 11 (18%) versus 14 of 60 (23%) in the BL group had intracardiac anomalies requiring simultaneous repair ( p = ns). Procedures included pericardial tracheoplasty (2 vs 26), tracheal autograft (4 vs 16), slide tracheoplasty (3 vs 8), and tracheal resection (2 vs 10). Overall mortality (operative and late) was 2 of 11 (18%) SL versus 10 of 60 (17%) BL ( p = ns). Median postoperative length of stay was 43 days SL (mean 48.6 ± 40) versus 30 days BL (mean 52.2 ± 65) ( p = ns). The incidence of postoperative tracheostomy (SL group) was 0 of 3 for slide tracheoplasty and 5 of 8 for the other techniques ( p = 0.12). Conclusions Despite the increased severity of pathology and increased critical presentation of tracheal stenosis patients with unilateral lung agenesis or severe hypoplasia, outcome measures of mortality and length of stay were similar to patients with two lungs. The incidence of associated pulmonary artery sling (1 of 3) and intracardiac anomalies (1 of 4) was similar. Unilateral lung agenesis or severe hypoplasia should not preclude operative repair of tracheal stenosis. Slide tracheoplasty is our current procedure of choice for these infants.

Congenital subglottic stenosis is rare and as a consequence may not be considered in children experiencing respiratory difficulty at birth. Diagnosis after a child already is intubated complicates the recognition and blurs the boundary between congenital and acquired lesions. This article discusses the anatomy of the larynx, its common anatomic variations, and its response to trauma, a thorough understanding of which is required for the accurate diagnosis and treatment of this complicated problem. The authors discuss evaluation and assessment options to guide treatment.

Objective The aim of the study is to review the safety and efficacy of partial fistulectomy with 3-layered primary closure without postoperative intubation for persistent tracheocutaneous fistula (TCF). Design This is a retrospective study. Setting The study was conducted in a tertiary care pediatric hospital. Patients Records of 49 children treated for persistent TCF between 1996 and 2005 were reviewed. Patients were studied if the TCF was closed using a 3-layered primary closure, and they had at least 1 year of follow-up. Thirty-nine patients met inclusion criteria. Results All patients were extubated immediately after surgery. Drains were removed, and all patients were discharged on the first postoperative day. The most common indications for tracheostomy were prolonged intubation and subglottic stenosis. There were 2 major and 3 minor complications. One major complication involved subcutaneous emphysema that developed on the seventh postoperative day because of cough. The other involved a poorly controlled diabetic patient who developed a postoperative infection with dehiscence. All fistulas remained closed at follow-up. Conclusion Partial excision and primary closure of persistent TCF is safe and effective. Drain placement and overnight observation are imperative. Careful patient selection is important. Routine postoperative intubation is not necessary.

Summary Objective Recurrent tracheoesophageal fistula (TEF) is a difficult problem in both diagnosis and management. Revision open repair with thoracotomy is challenging and has a significant associated morbidity. Because of the technical difficulty and the substantial morbidity, several authors have suggested and implemented endoscopic management. This paper reviews and describes the endoscopic techniques and management of recurrent TEF. Methods This retrospective study evaluates three patients who underwent endoscopic (bronchoscopic) management of recurrent TEF at Children's Memorial Hospital in Chicago, Illinois. Median follow-up is 48 months. A current literature review is presented with a synthesis of the data on techniques and results. Results Two of the three patients had successful bronchoscopic closure using fibrin adhesive. The mean number of procedures required was two. Including these patients, 62 patients with endoscopic closure of congenital TEF have been reported in the literature. The overall success rate is 60% with a mean of 2.1 procedures per patient. Of the obliterating agents described, tissue adhesive and fibrin adhesive have been employed most frequently with success rates of 48 and 55%, respectively. Conclusion Endoscopic repair of recurrent TEF is an effective and safe alternative to second thoracotomy and open surgical repair. More than one endoscopic procedure is usually necessary for successful closure.

Objectives: We undertook to identify data that facilitate determination of an accurate diagnosis of the cause of stridor in infants and to develop a framework to conceptualize the problem. Methods: We reviewed medical records of patients less than 1 year of age with the presenting symptom of stridor who were initially evaluated in the outpatient setting of a tertiary children's hospital. Infants with obvious congenital syndromes, cerebral palsy, or hypotonia were excluded. All infants underwent history-taking, physical examination, and when symptoms were mild, office flexible laryngoscopy. With moderate or severe stridor, a more complete endoscopic evaluation was undertaken in the operating room. Results: Of 202 patients, 119 (59%) were boys and 83 (41%) were girls. Their ages ranged from 3 days to 11 months; 175 (87%) were 6 months of age or younger. Congenital anomalies were diagnosed as the cause of stridor in 170 (84%). Congenital laryngeal anomalies caused stridor in 157 (78%); congenital tracheal abnormalities were the cause in 13 (6%). The most common congenital laryngeal anomaly was laryngomalacia (94%). Forty-two (21%) of the 202 patients had at least 1 other anomaly that contributed to airway compromise. Half of all patients had laryngopharyngeal reflux, the most common associated condition. Of patients referred with a presumptive diagnosis by non-otolaryngologists, 28 of 94 (30%) were referred with erroneous presumptive diagnoses for which they were being treated, the most common of which was tracheomalacia. Conclusions: A standard, rational approach to the evaluation of stridor in infants facilitates management. A framework for evaluation is presented.

Inflammatory myofibroblastic tumor (IMT), popularly known as inflammatory pseudotumor, is a slow growing quasi-neoplastic lesion with a distinct histologic appearance and benign clinical course. A case of a neck IMT with infiltration into the trachea causing asthmalike symptoms in a 12-year-old girl is described. Both tracheal and neck IMT have been described, but no other case has displayed this infiltration. A review of the pertinent literature and the etiology, diagnosis, treatment, and outcomes of this tumor are discussed. It is important to consider IMT in a differential diagnosis because it can be easily misdiagnosed as a malignancy. A surgeon must not perform radical surgery, radiation, or chemotherapy until a final pathologic diagnosis is made because of the nature of this lesion.

To report the perioperative management and surgical outcomes in a large series of pediatric patients with endoscopically repaired type 1 posterior laryngeal cleft (PLC).Case series with chart review.Urban, tertiary care, free-standing pediatric hospital.Patients who underwent endoscopic carbon dioxide laser-assisted repair of type 1 posterior laryngeal clefts between January 2006 and December 2012. Medical records were reviewed.Fifty-four patients (34 male) underwent repair of type 1 PLC. Median age was 25.5 months (range, 2-120 months). Indications for repair included aspiration (n = 39; 72%), chronic bronchitis (n = 13; 24%), and stridor with feeds (n = 2; 4%). No children remained intubated postoperatively. Thirty-three patients (61%) stayed in overnight observation ("Obs PLC") and 21 patients (39%) stayed in the pediatric intensive care unit ("PICU PLC") postoperatively. Between Obs PLC and PICU PLC groups, there was no significant difference in age (mean 22 vs 30 months, respectively; P = .28). Comorbidities were similar between the groups. Symptoms improved in 41 of the 54 patients (76%). No postoperative complications were noted. Two patients required revision PLC repair. The cost of admitting a patient to a lower acuity location was estimated to be 60% less per day than cost of a PICU admission.The endoscopic surgical repair of a type 1 PLC is successful and has a low morbidity and complication rate. Patients may be safely managed in an observation unit and without postoperative intubation. This approach achieved a marked cost reduction in postoperative care.

Upper airway obstruction in the newborn is frequently due to congenital laryngeal anomalies. One of these, the posterior laryngeal cleft, is often associated with subglottic stenosis and respiratory difficulty. This discussion of the anterior laryngeal cleft reports findings in an infant who required intubation immediately after birth and survived only 3 days. The anterior cricoid cleft was associated with severe congenital anomalies including congenital tracheal stenosis.

Background/Purpose: Since 1996 a new procedure—the free tracheal autograft—has been used to repair long segment congenital tracheal stenosis (LSCTS). The purpose of this report is to examine the intermediate-term results of that technique. Methods: Between January 1996 and July 1999, 10 infants underwent repair of LSCTS using a free tracheal autograft. Age ranged from 10 days to 23 months (mean age, 6.6 months). Six infants had a pulmonary artery (PA) sling; 5 had intracardiac anomalies. On cardiopulmonary bypass (CPB) the trachea was incised anteriorly through the area of stenosis. The midportion of the stenotic trachea was excised, and an end-to-end anastomosis was made posteriorly. The excised tracheal segment was trimmed and sutured in place anteriorly as a free autograft. In 5 patients the autograft was not long enough, and the upper portion of the tracheal opening was patched with pericardium. Results: There was 1 death 26 days postoperatively in a child that had simultaneous repair of tetralogy of Fallot and required extracorporeal membrane oxygenation postoperatively for cardiac failure. The other 9 children are alive and well at 2 to 44 months postoperatively (mean follow-up, 24 months). One child had autograft dehiscence and required replacement of the autograft with an aortic homograft. Two children have tracheostomies at 6 and 36 months postoperatively. All children have had serial postoperative bronchoscopic examinations. Most recent bronchoscopies have shown widely patent tracheal lumina in all survivors. Conclusion: Intermediate-term follow-up of children with a free tracheal autograft continues to support our use of this technique as our procedure of choice for infants with LSCTS. J Pediatr Surg 35:813-819. Copyright © 2000 by W.B. Saunders Company.

Primary tracheal neoplasms in children, especially malignant lesions, are extremely rare. We report a unique case of low-grade mucoepidermoid carcinoma of the trachea in a 4-year old child, presenting as new-onset asthma. Using the ventilating resectoscope, endoscopic excision was accomplished without complications. The case is presented with documentation including airway films, endoscopic photographs, and histopathology. The diagnosis of tracheal masses depends upon maintaining a high index of suspicion, complemented by imaging and timely diagnostic endoscopy. A review of the literature is included.

This paper reports the identification of the fragmented, distorted cricoid cartilage. The laryngeal findings in four patients with this acquired abnormality are presented. The postmortem whole organ serial section of their larynges is described and illustrated with horizontal sections from the Laryngeal Development Laboratory in Chicago. The histopathologic sequence, pathogenesis, and clinical relevance are elucidated.

This study was undertaken in order to establish the incidence of aerodigestive tract foreign body accidents among older children and adolescents, and to investigate the circumstances surrounding these events. A review of patients treated over a 5-year period identified 367 children from whom aerodigestive tract foreign bodies were removed. Seventeen percent of these patients were 5 years of age or older. Among these children, 88% aspirated nonfood items, half of which were school supplies; 78% of the group 5 years old and under aspirated food items. Among older children with esophageal foreign bodies, 31 % had food impactions, compared with 7% in the younger group; 70% of these children had a history of some anatomic abnormality of the esophagus. These data suggest that older children and adolescents represent a distinct group of patients at risk for foreign body accidents. Pediatricians and parents of children in this age group should discourage the practice of using the oral cavity as a repository for school supplies, and should stress the need for adequate preparation and mastication of food, particularly among children with esophageal abnormalities.

Objective: To present the pathologic findings of acquired lesions of the larynx in infants. Setting: The Laryngeal Development Laboratory of Children's Memorial Hospital, Chicago, Ill. Materials: One hundred fifteen larynges received at postmortem examination from 1975 to 1992. Main Outcome Measure: Acquired laryngeal lesions from intubation trauma. Technique: Whole-organ serial section of larynges. Results: Fifty-three of the 115 specimens exhibited acquired laryngeal lesions: 36, submucosal mucous gland hyperplasia; 12, submucosal fibrosis; 10, granulation tissue; eight, ulceration; eight, fragmented or distorted cricoid; four, cricoid ossification; four, ductal cysts; three, healed furrows, and one, anterior glottic synechia. Some specimens exhibited more than one type of acquired abnormality. (Arch Otolaryngol Head Neck Surg. 1995;121:537-543)

Acquired laryngeal stenosis is the most serious long-term complication of endotracheal intubation in children. Employing the whole-organ serial section technique, the sequence of histopathologic changes leading to stenosis was studied. Ulceration occurs when an endotracheal tube causes mechanical abrasion and/or induces pressure necrosis on the laryngeal mucosa. Secondary healing of ulceration produces granulation tissue and subsequent fibrous scar tissue. Most exuberant granulation tissue resolves without sequelae, but some becomes firm, almost avascular fibrous scar tissue. The accumulation of submucosal fibrous tissue may decrease the size of the glottic or subglottic lumen. Contraction of scar tissue causes a distortion of glottic and subglottic laryngeal complex, leaving a reduced and irregularly shaped glottic and subglottic lumen. Submucosal mucous gland hyperplasia directly reduces the inner diameter of the airway. Finally, compromise of the laryngeal lumen may occur when the duct of a mucous gland is obstructed by scarring resulting from intubation: mucus accumulates in the dilated duct, producing a ductal cyst.

Knowledge of laryngeal and tracheobronchial development and anatomy is essential to the pediatric airway endoscopist. Normal and pathologic airway anatomy is discussed in this chapter.

This description of congenital tracheal lesions is presented with a focus on the information derived from the specimens collected for the Laryngeal Development Laboratory at the Children's Memorial Hospital in Chicago from 1975 to 1992. During this period, 115 laryngotracheal specimens were obtained, 22 of which were found to have congenital tracheal anomalies. Six specimens were determined to have tracheomalacia, including one with primary tracheomalacia and five with secondary tracheomalacia: three were associated with tracheoesophageal fistula and two with aberrant innominate artery. Thirteen specimens had a trapped first tracheal arch; one, complete tracheal rings; and two, tracheal cartilaginous sleeve. Three specimens had tracheal agenesis, and three, tracheoesophageal fistula. Some specimens have more than one tracheal anomaly. Sixteen patients also had anomalies of other systems.

Objectives:We review the diagnosis and management of type I posterior laryngeal clefts (PLCs).Methods:We performed a retrospective study at a tertiary-care children's hospital of children who were diagnosed with a PLC between January 2003 and August 2008. We studied concurrent airway anomalies, comorbidities, presenting symptoms, age at the time of aspiration resolution, and rate of aspiration resolution.Results:Sixty-seven children with PLCs were identified (41 boys and 26 girls). Fifty-nine had type I clefts, 6 had type II, and 2 had type III. Of the 59 type I cases, 15 (25.4%) were surgically repaired by endoscopy. Eleven of these 15 children (73.3%) have had symptomatic improvement since the surgery, and 7 of those 11 (63.6%) are tolerating thin liquids by mouth. Two of the 15 (13.3%) displayed no improvement with surgery, and 2 of the 15 (13.3%) were lost to follow-up. Forty-four of the 59 type I clefts (74.6%) were managed nonsurgically. Twenty of these 44 children (45.5%) did not present with aspiration. Twenty-four of the 44 (54.5%) presented with aspiration, and 16 of the 24 (66.7%) are now tolerating thin liquids by mouth. Seven of these 24 patients (29.2%) are still aspirating, and 1 has died. The average time to resolution of aspiration was 7.8 months for the surgical group and 13.6 months for the nonsurgical group (p = 0.19). In the surgical group, the average age at resolution of aspiration for patients who received their diagnosis at 0 to 6 months of age was 21.5 months; that for those with a diagnosis at 6 to 12 months was 27.3 months; and that for those with a diagnosis at older than 12 months was 27.3 months (p = 0.31). In the nonsurgical group, the average age at resolution of aspiration for patients who received their diagnosis at 0 to 12 months of age was 15.8 months; that for those with a diagnosis at 12 to 24 months was 27.3 months; and that for those with a diagnosis at older than 24 months was 77.3 months (p = 0.0015).Conclusions:We found that 1) the reported incidence of type I PLCs is increasing; 2) type I PLCs can often present without clinical aspiration; 3) aspiration caused by type I PLCs can be managed medically or surgically; and 4) operative intervention is advantageous for patients who have severe symptoms or who have persistent aspiration after 2 years of age.

To determine if age and comorbid conditions effect outcomes in children undergoing supraglottoplasty for severe laryngomalacia.Retrospective study.Urban tertiary-care children's hospital.Children undergoing supraglottoplasty for severe laryngomalacia between February 2004 and July 2008. 56 patients were identified.Persistence of upper airway obstruction, revision surgery (supraglottoplasty), and additional surgery (tracheostomy).33/56 (58.9%) patients had no comorbid conditions and 23/56 (41.1%) patients had comorbid conditions. In noncomorbid patients, 36.4% of those less than 2 months of age at the time of surgery required revision supraglottoplasty, compared to 5.3% of patients between 2 and 10 months (p0.05). Compared to the 2-10-month age group, there was a significantly higher percentage of patients with comorbid conditions in the10-month group (32.1% vs. 79%, p0.01). Patients with comorbid conditions were diagnosed at a significantly later age than those without (6 mo vs. 2 mo, respectively), and had significantly higher rates of revision supraglottoplasty (47.8% vs. 18.2%) and tracheostomy (39.1% vs. 0.0%). 70% of children with neurological conditions required revision surgery, with 60% requiring tracheostomy. The revision surgery and tracheostomy rates were significantly higher compared to the noncomorbid group (p0.01 and p0.0001). Children with cardiac conditions had a higher rate of tracheostomy than noncomorbid children (30% vs. 0%, p0.01). 16.7% of children with genetic conditions required supraglottoplasty, and none required tracheostomy.In noncomorbid patients, those undergoing supraglottoplasty less than 2 months of age had a significantly higher rate of revision supraglottoplasty. Patients with neurologic and cardiac comorbidities require tracheostomy at a significantly higher rate than noncomorbid patients.

Objective To quantify the prevalence and the impact of synchronous airway lesions identified by endoscopy in infants undergoing supraglottoplasty for severe laryngomalacia (LM). Design Retrospective study. Setting Tertiary care pediatric hospital. Patients Sixty patients who underwent supraglottoplasty for severe LM from 2002 to 2006. Patients who underwent preoperative tracheotomy, had previous airway surgery, or did not have 6 months of follow-up were excluded. Fifty-two patients met inclusion criteria. Intervention Supraglottoplasty (with carbon dioxide laser). Main Outcome Measures Presence of synchronous airway lesions and their contribution to upper airway obstruction (UAO) and their effect on the postoperative course after supraglottoplasty. Results Fifty-eight percent of patients had synchronous airway lesions (SALs), of whom 77% had subglottic stenosis (SGS) and 47% had tracheomalacia, bronchomalacia, or both. Sixty-three percent of all patients required postoperative nonsurgical airway support. Eight patients had residual UAO requiring additional surgical intervention, with 3 revision supraglottoplasties and 7 tracheotomies performed. Infants with neurological conditions had a high rate of surgical intervention (55%; P = .001). Patients with SGS exceeding 35% but without any neurological condition had a prolonged hospital stay (>3.6 days; P = .02) and an 83% incidence ( P = .04) of postoperative UAO requiring intubation. Infants with LM with laryngeal edema (LE) alone had increased frequency of postoperative nonsurgical airway support ( P = .02) and a prolonged hospital stay of 1 day ( P = .01) compared with infants without edema. Conclusions There is a high incidence of SALs in patients undergoing supraglottoplasty. Neurological conditions, hypoplastic mandible, SGS greater than 35%, and preexisting LE independently adversely affected the postoperative course.

Objectives/Hypothesis: Early mandibular lengthening by distraction osteogenesis provides an alternative to traditional methods of airway management in infants with Pierre Robin sequence (PRS). Little evidence in the medical literature quantitatively demonstrates the changes in skeletal, soft tissue, and hypopharyngeal spaces with mandibular distraction. Study Design: Prospective analysis of a cohort of three patients with PRS. Methods: We reviewed a series of infants with PRS and severe upper airway obstruction who underwent mandibular distraction. The infants underwent mandibular lengthening with the same internal, unidirectional distraction osteogenesis device. Standardized serial computed tomography (CT) scans were obtained according to established protocol. Computed tomography data were extracted and analyzed with medical image analysis software for mandibulo-maxillary arch harmony, symmetry, hypopharyngeal airway volume, geniohyoid distance, distraction osteogenesis bone volume, and mandibular length. Results: Mandibulo-maxillary alveolar ridge distances were corrected to 0.5 mm after distraction. Clinical examination showed good arch harmony without open-bite or cross-bite deformities. Mandibular ramus was lengthened by 19.5%; the body, 43.4%. After distraction, total mandibular length was increased by 26.2%; hypopharyngeal airway volume, 192%; posterior distance from pharyngeal wall to tongue base, 198.9%; and geniohyoid distance, 14.1%. Conclusions: Unidirectional internal microdistractors can achieve good mandibulo-maxillary arch harmony. Hypopharyngeal airway volume increases substantially, with an even greater increase in distance between tongue base and posterior pharyngeal wall. As the distal mandibular segment is distracted, the hyoid moves anteriorly, with minor increase in geniohyoid relationship. Internal mandibular microdistraction devices represent a substantial advance in airway obstruction management in infants with micrognathia. Laryngoscope, 2009

Summary Objective To review the incidence, risk factors, and treatment of aspiration following CO2 laser-assisted supraglottoplasty for severe laryngomalacia (LM). Design IRB approved retrospective study of pediatric patients with severe LM treated with CO2 laser supraglottoplasty over a 5-year period. Setting Tertiary pediatric hospital. Patients Fifty-two patients met inclusion criteria. Indication for supraglottoplasty was respiratory distress in 90% of patients and/or failure to thrive in 19%. Interventions All children underwent bilateral CO2 laser supraglottoplasty and were assessed postoperatively with swallow evaluation by a speech pathologist. Videoflouroscopic evaluation was utilized to confirm aspiration and guide management. Main outcome measures Aspiration, treatment required to manage aspiration, duration of treatment required. Results Thirty-seven percent (20/52) of patients had postoperative aspiration. Aspiration was demonstrated on videoflouroscopic swallow study (VFSS) after supraglottoplasty is 28% (12/43). All patients with newly diagnosed aspiration had treatment with thickened and/or nasogastric feedings with mean resolution time of 6 months. In nine children with preoperative aspiration, eight (89%) had postoperative aspiration and seven required gastrostomy tube placement for feeding management. All individuals requiring gastrostomy for aspiration management had neurological conditions. In the absence of preoperative clinically evident aspiration, children with neurological conditions have an equivalent rate of postoperative aspiration as healthy children. The only risk factor for postoperative aspiration was preoperative aspiration. Conclusion Aspiration is more common after CO2 laser-assisted supraglottoplasty than previously recognized. In otherwise healthy children, postoperative aspiration is of short duration and can be treated with conservative measures. Optimal treatment after supraglottoplasty includes screening for and management of aspiration in conjunction with a speech pathologist.

Objective We sought to review our experience with infants and children with anatomically complete vascular rings (ie, double aortic arch and right aortic arch with left ligamentum) and define perioperative trends in diagnostic imaging, operative techniques, and clinical outcomes. Methods From 1946 through 2003, 209 patients (113 with double aortic arch and 96 with right aortic arch) underwent surgical repair. Mean and median ages at the time of the operation were as follows: double aortic arch, 1.4 ± 2.4 years and 0.75 years, respectively; right aortic arch, 2.7 ± 3.9 years and 0.9 years, respectively. Fourteen (14.6%) patients with right aortic arch had an associated Kommerell diverticulum. Cardiac diagnoses were present in 26 (12.4%) of 209 patients. Results There has been no operative mortality since 1959. In the past 30 years, mean hospital stay decreased from 8 to 3 days. Primary means of diagnosis has shifted from barium swallow and angiography to computed tomographic scanning or magnetic resonance imaging. In the past 10 years, 73% of patients had preoperative or intraoperative bronchoscopy. The technique of operation has shifted to a muscle-sparing left thoracotomy without routine chest drainage. In 7 recent patients with right aortic arch and a Kommerell diverticulum, the diverticulum was resected, and the left subclavian artery was transferred to the left carotid artery as a primary procedure. Conclusions At our institution, computed tomographic scanning has replaced barium swallow as the diagnostic procedure of choice for vascular ring evaluation. We recommend both preoperative bronchoscopy and echocardiography. Use of a muscle-sparing thoracotomy without routine chest drainage has decreased mean hospital stay. For patients with a right aortic arch and associated Kommerell diverticulum, we recommend diverticulum resection with left subclavian artery transfer to the left carotid artery.

Thirty-six patients with persistent tracheocutaneous fistula (TCF) after pediatric tracheotomy were managed at Children's Memorial Hospital in Chicago between June 1987 and July 1992. Persistent TCF was managed with surgical excision and primary closure. The mean patient age was 5 years 7 months, and the mean duration between decannulation and fistula closure was 21 months. There were no major complications and four minor complications. While most surgeons advocate other techniques, we feel that excision with primary closure is the preferred method for persistent TCF. The technique requires an airtight tracheal closure with loose closure of the peristomal soft tissue. Careful preoperative evaluation, postoperative monitoring, and wound drainage are stressed.

Objectives: A Kommerell’s diverticulum in patients with a right aortic arch may become aneurysmal and be an independent cause of tracheoesophageal compression, even after ligation and division of a left ligamentum. We review the indications for and results of Kommerell’s diverticulum resection and left subclavian artery transfer in children with a right aortic arch who previously underwent vascular ring (ligamentum) division. Methods: From 1998 through 2001, eight children have been referred with recurrent respiratory symptoms (n ¼ 8) and/or recurrent dysphagia (n ¼ 4) after vascular ring division. Each child had a right aortic arch with a left ligamentum and had undergone division of the ligamentum elsewhere. All had a Kommerell’s diverticulum that was not addressed at the initial operation. All patients had a repeat left thoracotomy with resection of the diverticulum. Five patients had division and reimplantation of the left subclavian artery into the left carotid artery to relieve the sling-like effect of the retroesophageal left subclavian artery on the right aortic arch. One other patient had primary Kommerell’s diverticulum resection and transfer of the left subclavian artery to the left carotid artery. Results: The mean age at the initial operation was 1.7 ^ 0.9 years, and the mean age at reoperation was 8.0 ^ 3.7 years. In all patients postoperative bronchoscopy confirmed relief of the tracheal compression. There were no complications related to the subclavian artery transfer. Two patients developed postoperative chylothorax, one requiring thoracic duct ligation. The median hospital stay was 5 days. All patients had dramatic resolution of their preoperative symptoms. Conclusions: Kommerell’s diverticulum is an important anatomic structure that can cause recurrent symptoms in patients with a right aortic arch after ligamentum division. In selected patients, reoperation with resection of the Kommerell’s diverticulum and transfer of a retroesophageal left subclavian artery results in relief of symptoms. This technique has become our procedure of choice as a primary operation for children with a right aortic arch and a significant Kommerell’s diverticulum. q 2002 Elsevier Science B.V. All rights reserved.

We have used six different techniques in 61 operations performed at Children's Memorial Hospital (Chicago, IL) between 1982 and 2001 on 54 infants with complete tracheal rings and primary or recurrent tracheal stenosis. Short-term and long-term outcomes are reviewed for all techniques including pericardial tracheoplasty, tracheal autograft, tracheal resection, cartilage tracheoplasty, slide tracheoplasty, and aortic homograft patch tracheoplasty. Patients' ages ranged from 7 days to 72 months; mean age at operation was 6 months. There were 33 boys and 21 girls. Associated pulmonary artery sling was present in 19 of 54 patients (35%). Significant associated intracardiac anomaly was present in 13 of 54 patients (24%). The number of complete tracheal rings involved ranged from two to 18 rings (mean, 14+/-5). All procedures were performed with the use of cardiopulmonary bypass. Simultaneous repair of pulmonary artery sling and cardiac abnormalities was undertaken. There were three early deaths, two after pericardial tracheoplasty and one after tracheal autograft. There were eight late deaths, five after pericardial tracheoplasty, one after tracheal autograft, one after slide tracheoplasty, and one after tracheal resection. Median length of hospital stay was 60 days for pericardial tracheoplasty, 28 days for tracheal autograft, 14 days for tracheal resection, and 18 days for the slide tracheoplasty. Follow-up is complete in all patients. Tracheal autograft is currently our procedure of choice for patients with long-segment tracheal stenosis because of its use of all-autologous material, technical ease of performance, already-present epithelial lining of the autograft, intrinsic maintenance of the cartilage contour, potential for growth, and ready availability. We limit the use of tracheal resections to patients with eight or less rings of tracheal stenosis.

Objective: To correlate clinical and histological findings in patients with laryngomalacia who required surgical intervention. Methods: Retrospective study of all patients undergoing supraglottoplasty by a single surgeon (MEG) for severe laryngomalacia between October, 1999 and November, 2000. Results: Nine patients were identified, of which seven had clinical evidence of GER. Seven patients had co-existing abnormalities or delays of neuromuscular development including seizure disorder, agenesis of the corpus callosum, obstructive sleep apnea, primary aspiration, a history of apparent life-threatening events, and craniosynostosis. Varying degrees of subepithelial edema and significant dilation of the subepithelial lymphatics were noted in all specimens. Submucosal inflammation was minimal to mild, and intraepithelial inflammation was rare to absent in all sections. No submucosal gland hyperplasia was seen in the samples from any patient. Two specimens contained cuneiform cartilage, both of which were histologically characterized as fibrocartilage. Conclusions: In this series, the histopathology of tissue excised during the treatment of severe laryngomalacia was dominated by submucosal edema and lymphatic dilation. Further study is needed to investigate comorbidities that may contribute to the need for intervention in children with laryngomalacia.

Objective: Review the short- and long-term outcomes of a single institution experience in infants with congenital tracheal stenosis, comparing four different operative techniques used from 1982 through 2000. Methods: Hospital and clinic records of 50 infants and children who had surgical repair of congenital tracheal stenosis secondary to complete tracheal rings were reviewed. Age at surgery ranged from 7 days to 72 months (median, 5 months, mean 7.8 ± 12 months). Techniques included pericardial patch tracheoplasty (n = 28), tracheal autograft (n = 12), tracheal resection (n = 8), and slide tracheoplasty (n = 2). All procedures were done through a median sternotomy with cardiopulmonary bypass. Seventeen patients had a pulmonary artery sling (35%), and 11 had an intracardiac anomaly (22%), Results: There were three early deaths (6% early mortality), two after pericardial tracheoplasty and one after autograft. There were six late deaths (12% late mortality), five after pericardial tracheoplasty and one after slide tracheoplasty. Length of stay (median) was 60 days (pericardial tracheoplasty), 28 days (autograft), 14 days (resection), and 18 days (slide). Reoperation and/or stent placement was required in seven patients (25%) after pericardial tracheoplasty, in two patients (17%) after autograft, in no patients after resection, and in one patient (50%) after slide tracheoplasty. Conclusions: Our current procedures of choice for infants with congenital tracheal stenosis are resection with end-to-end anastomosis for short-segment stenoses (up to eight rings) and the autograft technique for long-segment stenoses. Associated pulmonary artery sling and intracardiac anomalies should be repaired simultaneously.

Objective: to report a case of recurrent respiratory papillomatosis with diffuse involvement of the esophagus in a child. Design: retrospective case report and literature review. Setting: Tertiary Children's Hospital. Conclusion: endoscopy is recommended for detection of esophageal papillomas, especially in patients with significant laryngeal lesions or post-cricoid involvement.

The objective of this study was to determine whether specimens from infants with palatal defects (PDs) with or without craniofacial malformations (CFMs) exhibit aberrant laryngeal histologic findings compared with specimens from normal infants. Ten laryngeal specimens from infants with PDs with or without CFMs were histologically compared with 7 laryngeal specimens defined as normal from the same collection. Both groups were similar in terms of demographics and airway manipulation. All infants were prelingual. Comparisons were made at 3 levels: supraglottic, glottic, and subglottic. Histologically, no significant differences in primary laryngeal structures were found between the PD with or without CFM group and the group defined as normal. Acquired and intubation-type injuries, such as inflammation, ulceration, capillary congestion, and scar tissue, were more prevalent and severe in the study group. The primary laryngeal histologic findings of specimens from individuals with PDs with or without CFMs do not differ substantially from those from normal individuals; however, individuals with PDs do appear to be somewhat more susceptible to intubation injury and other acquired laryngeal injury. Meticulous airway management is essential.

Foreign bodies of the upper aerodigestive tract in the pediatric population are a common occurrence. However, despite significant advances in prevention, first aid and endoscopic technology, they remain a diagnostic and therapeutic challenge. Early diagnosis is the key to successful and uncomplicated management of these accidents. An orderly and systematic approach to these patients including a careful history, physical exam and radiographic studies is detailed in this review.

Throughout our daily interactions with medical students and residents, each of us, whether we like it or not, functions as a role model. Since role modeling is primarily a passive function — teaching by example — we may not be acutely aware of this role and its importance. In what respect is the concept of the role model important to otolaryngology—head and neck surgery? In addition to the function of specific training — teaching the trade — most of the literature on role modeling cites 2 major areas of significance: 1) influencing medical students' career choices and 2) facilitating socialization into the world of medicine with the establishment of an appropriate professional identity. This brief article reviews some of the current literature, catalogs those attributes that have been identified as those of excellent role models, and offers some thoughts as to what our specialty might consider in response to the challenges to medical education in the changing health care environment.

A retrospective chart review was performed to quantify the postoperative complications and outcomes of 82 consecutive cases of laryngotracheal reconstruction (LTR) and cricotracheal resection (CTR) performed at a pediatric tertiary care hospital over the last 9 years. Six cases of respiratory syncytial virus (RSV) bronchiolitis and 8 cases of cervical pseudomonal wound abscess (PWA) were identified in a total of 12 patients. All of these infections occurred after single-stage LTR or CTR. Both RSV bronchiolitis and PWA were associated with significantly more unexpected days of intubation and admission to the intensive care unit, as well as higher rates of failure of LTR. Ossification of the cricoid cartilage, grade IV subglottic stenosis, and untreated gastroesophageal reflux disease (GERD) were also associated with restenosis. Trisomy 21 did not significantly influence the success rate of pediatric LTR. Both RSV bronchiolitis and PWA are potentially preventable complications of pediatric LTR and CTR. We propose strategies to prevent these infections. We also advocate the treatment of GERD during the healing phase of LTR.

Background . The classic surgical approach to pulmonary artery (PA) sling has been through a left thoracotomy with division of the left PA and reimplantation into the main PA anterior to the trachea. Another approach is anterior left PA translocation with distal tracheal resection. Since 1985, we have repaired PA sling with a median sternotomy approach, cardiopulmonary bypass, and division and reimplantation of the left PA into the main PA with simultaneous repair of associated tracheal stenosis. The purpose of this review is to determine the outcome of that strategy. Methods. From 1985 to 1998, 16 infants had surgical treatment of PA sling, 14 had left PA division and reimplantation into the MPA, 2 patients had repair using the translocation technique. Mean age at repair was 6.9 months, median age was 4 months. All infants, except 1 with an absent right lung, were operated on at the time of diagnosis. All had rigid bronchoscopy, which revealed associated complete tracheal rings in 12 patients. Seven patients had tracheal repair with pericardial tracheoplasty, 4 had repair using a tracheal autograft technique, and 2 had a distal tracheal resection (one for tracheomalacia). Of the 2 patients having the translocation technique, 1 had a severely hypoplastic right lung and the other had complete absence of the right lung. Results . There has been no operative mortality. Hospital stay ranged from 5 to 188 days (mean 36 ± 42 days). There was 1 late death 7 months postoperatively from respiratory complications of pericardial tracheoplasty. All left pulmonary arteries are patent and blood flow to the left lung by nuclear scan (n = 10) ranges from 24% to 46% (mean 35% ± 9%). Conclusion . The strategy of median sternotomy, cardiopulmonary bypass, and left PA division and reimplantation into the main PA with simultaneous tracheal repair has resulted in a low operative mortality and excellent patency of the left pulmonary artery. Results with repair of the commonly associated complete tracheal rings has recently improved with the use of the free tracheal autograft technique.

A precise delineation of each laryngeal abnormality is critical to planning rational therapy. Since congenital cricoid cartilage anomalies are easily overlooked, a systematic approach to their identification is as important as a clear concept of their classification. The purpose of this communication is to present the pathologic findings and classification of congenital cricoid cartilage deformities and to summarize updated information derived by the whole organ macrosection technique. The classification of congenital subglottic stenosis is based on study of the English-language literature and the collection in the Laryngeal Development Laboratory at The Children's Memorial Hospital in Chicago. Twenty-nine specimens with congenital cricoid abnormalities have been identified. Fourteen have an elliptical cricoid cartilage; 12, a laryngeal cleft; 5, a flattened shape; 1, a large anterior lamina; and 3, generalized thickening. Some specimens have more than 1 anomaly. Eight cases of fragmented or distorted cricoid cartilages are thought to be acquired lesions. An anterior submucous cricoid cleft has been identified and is presented for the first time.

The congenital absence of the epiglottis is a very rare anomaly. We present a patient who has a complete absence of the epiglottis, illustrated with an endoscopic photograph, review the literature and discuss the management of patients with this condition.

Primary tracheal neoplasms are extremely rare lesions in the pediatric age group. This study reviews the English-language literature to better characterize these lesions in children and reports 2 additional patients. Reports of only 36 infants and children through adolescence with primary tracheal neoplasms were discovered after an exhaustive literature review of the last 30 years. The data are analyzed with regard to pathology, demographics, symptomatology, site, and percent luminal obstruction. We report 2 additional patients with photographic documentation, imaging studies, and histopathology. Of the 36 previously reported lesions, 64% were characterized as benign and 36% as malignant. Fifty-six percent of all lesions were initially misdiagnosed as asthma. The most common site was the posterior membranous wall of the cervical trachea. In 14 (39%) of the 36 patients, the lesions obstructed more than 50% of the lumen at the time of diagnosis. The timely diagnosis of tracheal masses depends upon maintaining a high index of suspicion and conducting an efficient workup, including definitive evaluation by bronchoscopy. The evaluation and the differential diagnosis of tracheal neoplasms in the pediatric population is discussed.

This article reviews laryngeal cleft anomalies from the Laryngeal Development Laboratory at Children's Memorial Hospital in Chicago and includes a discussion of the classification of laryngotracheoesophageal clefts based on previous work and the information presented herein. Of the 115 laryngeal specimens obtained between 1975 and 1995, 11 have laryngeal cleft anomalies. Eight have a submucous laryngeal cleft. There is 1 laryngotracheoesophageal cleft, type II (partial cricoid cleft); and there are 2 laryngotracheoesophageal clefts, type III (complete cricoid cleft). The histopathologic findings are presented in detail and the literature is reviewed. Photomicrographs and drawings illustrate the pathology and classification. Clinical presentation, diagnosis, evaluation, and management are discussed, as is the embryology.

Pericardial patch tracheoplasty has been used for surgical correction of long-segment congenital tracheal stenosis caused by complete tracheal rings in infants. The case histories of 2 infants with descriptions of the histopathologic changes in their respective tracheas are presented. Complete reepithelialization of the graft site with ciliated pseudostratified columnar epithelium was found, suggesting the likelihood of normal mucociliary flow in the trachea. The pericardial patches were replaced by mature scar tissue in the graft site, establishing a functional tracheal lumen. Wound healing in the trachea is reviewed. Obstruction by exuberant granulation tissue is an ongoing concern. Pericardium continues to be an important option as graft material for tracheal reconstruction in infants with long-segment congenital tracheal stenosis. Arch Otolaryngol Head Neck Surg. 1997;123:1069-1072

Objectives: To describe a condition identified in the pediatric population that narrows the supraglottic larynx. This condition has been termedsupraglottic stenosis/collapse. To discuss common factors present in these children and to examine potential causes of this specific condition. Design: Retrospective review of patients with supraglottic stenosis/collapse from October 1985 to June 1993. Setting: Academic tertiary care children's hospital. Patients: Seventeen patients aged 7 months to 14 years underwent laryngoscopy and bronchoscopy for evaluation or follow-up of upper airway obstruction or respiratory failure and were found to have supraglottic stenosis/collapse. Main Outcome Measures: Each patient's records were reviewed for the average age at the time of diagnosis, history of prematurity, evidence of gastroesophageal reflux, history of long-term intubation, and any associated medical problems. In addition, patients were compared based on previous airway surgery (tracheostomy, laryngotracheal reconstruction, or anterior cricoid split). Results: Chronic upper airway obstruction was identified in 17 patients caused by supraglottic stenosis/collapse. All patients (100%) had undergone tracheostomy prior to the recognition of supraglottic stenosis/collapse. Fifty-nine percent of the patients had undergone laryngotracheal reconstruction, and 53% had a history of gastroesophageal reflux. Conclusion: Supraglottic stenosis/collapse is a distinct entity that may be related to prior laryngeal or tracheal surgery. This diagnosis should be considered when evaluating and treating patients with chronic upper airway obstruction following laryngeal and/or tracheal surgery. Arch Otolaryngol Head Neck Surg. 1997;123:337-341

We have managed 23 infants and children with severe tracheal stenosis due to congenital complete tracheal rings producing a long-segment stenosis of the trachea. Nineteen (83%) have survived this life-threatening cause of airway obstruction, 7 of whom also had pulmonary artery slings. Pericardial patch tracheoplasty facilitated by partial cardiopulmonary bypass is currently our preferred technique for surgical repair. Eighteen patients (78%) underwent operative intervention, 3 of whom (17%) have died since surgery. The mean follow-up is 4.5 years. Bronchoscopy is essential for preoperative diagnosis and accurate intraoperative incision of the trachea, and is critical for long-term postoperative airway management. The more distal lesions are associated with increased complications and a higher mortality rate.

This description of congenital laryngeal lesions is presented with a focus on the information derived from the specimens collected for the Laryngeal Development Laboratory at the Children's Memorial Hospital in Chicago from 1975 to 1992. During this period, 115 laryngeal specimens were obtained, 33 of which were found to have congenital laryngeal anomalies. Two specimens are thought to have laryngomalacia; 4, thyroid cartilage anomalies; 1, absence of the epiglottis; and 29, cricoid cartilage deformities. Of the cricoid cartilage deformities, 1 had large anterior lamina; 1, generalized thickening; 8, elliptical shape; 4, flattened shape; 8, occult cleft larynx; 1, partial cricoid cleft; 3, complete cricoid cleft; and 8, fragmented or distorted shape. Some specimens have more than one type of anomaly.

Congenital anomalies of the tongue are uncommon. Since de Jussieu first described lingual hypoplasia in 1719, fewer than 50 cases have been described. These are usually in association with the hypoglossia-hypodactylia syndrome. We present a case of a newborn with absent anterior tongue, a rudimentary posterior remnant and associated micrognathia. Embryology and theories of pathogenesis are discussed.

Between 1982 and 1990, 15 children have undergone pericardial patch tracheoplasty, 13 for complete tracheal rings and 2 for acquired tracheal stenosis. Eleven had complete tracheal rings from the thoracic inlet or the cricoid to the carina. Diagnosis was by bronchoscopy (15), computed tomography (10), and magnetic resenance imaging (3). As first described by Idriss in 1984, the surgical technique uses a median sternotomy approach, extracorporeal circulation, bronchoscopic guidance, and the use of pericardium as a tracheal patch. There was one perioperative death of mediastinitis (operative survival, 93%). There were two late deaths caused by complications of tracheostomy (1) and direct laryngoscopy and bronchoscopy (DLB) (1). Follow-up is complete in 12 intermediate-term survivors and ranges from 0.4 to 8.2 years (mean, 4.2 ± 2.9 years). Five children required multiple DLBs with dilation and excision of granulation tissue; one of these patients underwent tracheoplasty revision 5 months after the initial procedure for residual tracheal rings, and four of these children have had tracheostomies, two temporary and two currently (4 months, 7 years). Seven children had widely patent tracheas observed at follow-up DLB with little or no residual stenosis after tracheoplasty. All survivors are currently asymptomatic, except for the two children with tracheostomies. Pericardial patch tracheoplasty offers effective therapy with relatively low operative mortality for infants and children with severe long segment tracheal stenosis. On intermediate-term follow-up, nearly all survivors are essentially free of symptoms of residual tracheal stenosis.

To assess the ability of simultaneous cisplatin, 5-Fluorouracil, and radiation to substitute for surgery and radiation in advanced head and neck cancer, we have retrospectively selected from our phase II study a subgroup of 29 patients having primary disease requiring either more than a hemiglossec-tomy or a laryngectomy for control. Patients included 22 with stage IV and 7 with stage III disease, 12 tongue, 10 hypopharynx, and 7 larynx primaries. The treatment consisted of concurrent cisplatin, 5-Fluo-rouracil, and split-course radiation every other week for a total of 7 cycles within 13 weeks. With a median follow-up of 5 years, 86% of patients had preservation of speech and/or swallowing function. Median survival was 45 months, with 14 (48%) patients currently alive and disease free, 11 (38%) dead from their cancer, and 4 (14%) dead of other causes. The overall failure rate was 38%. Advanced-stage presence of N3 nodal disease and fewer than 7 cycles of chemotherapy received were significantly associated with increasing failure rates. This program of concomitant cisplatin, 5-Fluorouracil, and radiation produced control rates quite competitive with surgery and radiation and is appropriate for definitive testing in a randomized trial.

The aspiration or ingestion of a foreign body into the upper aerodigestive tract is a common accident that befalls children and adults alike. Sharp and pointed objects pose special problems. The basic principles of their extraction were meticulously developed by Chevalier Jackson, whose concepts of the mechanical problems encountered and demonstrations of their solutions are responsible for keeping morbidity and mortality figures at a minimum. The principles of evaluation and management of sharp and penetrating foreign bodies of the upper digestive tract are described.

A 14-year-old girl developed progressive hoarseness and breathing difficulty due to a mass in the subglottic larynx. A biopsy specimen obtained at direct laryngoscopy snowed localized aggregates of amyloid. The lesion was excised completely with a carbon dioxide laser. This is the second case of laryngeal amyloidosis in a child reported in the English-language literature.