Resection of Kommerell's diverticulum and left subclavian artery transfer for recurrent symptoms after vascular ring division.

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=8) and/or recurrent dysphagia (n=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.

Histological insight into the pathogenesis of severe laryngomalacia.

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.

Healing of a free tracheal autograft is enhanced by topical vascular endothelial growth factor in an experimental rabbit model.

OBJECTIVE: In 1996, we introduced the free tracheal autograft technique for repair of congenital tracheal stenosis from complete tracheal rings in infants and children. Sources of possible concern with this procedure include the potential for autograft ischemia, patch dehiscence, and recurrent stenosis. Vascular endothelial growth factor is a potent angiogenic inducer (particularly in the setting of ischemia, hypoxia, or both) and is postulated to promote tissue healing. The purpose of this study was to test the hypothesis that pretreatment of tracheal autografts with topical vascular endothelial growth factor would enhance tracheal healing. METHODS: In a rabbit model of tracheal reconstruction (n = 32), an elliptically shaped portion of the anterior tracheal wall was excised. The excised portion of trachea was one third of the tracheal circumference and 2 cm in length (6 tracheal rings). This portion of trachea (the autograft) was soaked in either vascular endothelial growth factor (5 microg/mL, n = 16) or normal saline solution (n = 16) for 15 minutes before being reimplanted in the resultant tracheal opening. Animals were killed and autografts were examined at 2 weeks, 1 month, and 2 months postoperatively for gross and microscopic characteristics. RESULTS: By 2 weeks, and progressing through 1 and 2 months, autografts treated with vascular endothelial growth factor, as compared with control autografts, had reduced luminal stenosis, submucosal fibrosis, and inflammatory infiltrate (P <.05). The autografts tended to become malaligned in control animals, whereas the tracheal architecture was preserved in rabbits treated with vascular endothelial growth factor. Microvascular vessel density was significantly greater in all vascular endothelial growth factor groups (P <.05) at all time intervals. CONCLUSIONS: Topical treatment of free tracheal autografts with vascular endothelial growth factor in a rabbit tracheal reconstruction model enhanced healing, as evidenced by accelerated autograft revascularization, reduced submucosal fibrosis and inflammation, and preservation of the normal tracheal architecture. Topical vascular endothelial growth factor may improve future results of tracheal reconstruction.

Tracheal surgery in children: an 18-year review of four techniques.

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.

Recurrent respiratory papillomatosis with esophageal involvement.

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.

Etiology and management of pediatric hemoptysis.

OBJECTIVE: To review the diagnostic and treatment strategies of hemoptysis in children. DESIGN: Retrospective analysis of patients evaluated between January 1, 1995, and August 31, 1999. SETTING: Tertiary pediatric referral center. PATIENTS: Nineteen consecutive children presenting with hemoptysis to the otolaryngology service. RESULTS: Chest radiography and bronchoscopy established the correct etiology in 15 patients. Infection and tracheostomy-related complications were the most common underlying problems. Other causes included congenital heart disease, pulmonary hemosiderosis, inflammatory bronchial mass, cystic fibrosis, factitious hemoptysis, and esophagitis. Appropriate management, ranging from antibiotics to emergency embolization, resulted in control of hemoptysis in all patients. CONCLUSIONS: Hemoptysis is a rare but potentially life-threatening symptom of underlying respiratory tract abnormality in children. An efficient systematic evaluation is imperative to identifying the underlying etiology; aggressive management is important because of the potential severity of the problem. The otolaryngologist plays a pivotal role in the diagnosis and management, by flexible endoscopy of the nose, nasopharynx, and larynx, and through the use of rigid bronchoscopy, especially in cases of massive hemoptysis.

Laryngeal histologic findings in infants with palatal defects with or without craniofacial malformations.

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.

Anterior submucous laryngeal cleft.

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.

Complications of pediatric laryngotracheal reconstruction: prevention strategies.

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.

The otolaryngologist as a role model.

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.

Management of congenital subglottic hemangioma.

OBJECTIVE: This study was conducted to assess the safety and efficacy of individualized management of congenital subglottic hemangioma (CSH) at the Children's Memorial Hospital in Chicago; describe treatment modalities, including endoscopic excision with the carbon dioxide (CO2) laser, systemic corticosteroids, and intralesional corticosteroid injection with short-term intubation; and determine the success of these various strategies in avoiding tracheotomy. METHODS: During the 10-year period between January 1, 1988 and December 31, 1997, 28 infants were diagnosed with CSH. A retrospective review of medical records was undertaken to determine demographics, presenting symptoms, location of the lesion, therapeutic modality, and complications. All patients were contacted at the time of writing. RESULTS: Twenty-eight patients met the criteria for this study. Gender distribution was 1.8:1 female to male. Age at diagnosis ranged from 4 weeks to 8 months, with a mean of 78.8 days and a median of 60 days. The location of CSH was most often posterior and on the left. Associated hemangiomas were found in 14 (50%) cases. The most common symptoms were stridor and cough. Management included 1 to 13 operative direct laryngoscopies and bronchoscopies, endoscopic excision with the (CO2) laser, and the use of systemic and intralesional corticosteroids. No patient required tracheotomy. CONCLUSION: Morbidity and the need for tracheotomy in CSH patients can be minimized using a combination of therapeutic modalities. Each infant's treatment is individualized based on the severity of the patient's symptoms and the morphology of the lesion.

Pulmonary artery sling: results with median sternotomy, cardiopulmonary bypass, and reimplantation.

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.

Histopathology of congenital subglottic stenosis.

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 use of balloon-expandable metallic stents in the treatment of pediatric tracheomalacia and bronchomalacia.

OBJECTIVE: To evaluate the use of balloon-expandable metallic stents in the treatment of children with tracheomalacia and bronchomalacia in whom conventional therapy has failed. DESIGN: Retrospective case series. SETTING: Tertiary pediatric otolaryngology and cardiothoracic surgery referral center. PATIENTS: Six patients were identified as having undergone bronchoscopic placement of metallic balloon-expandable stents between 1994 and 1997. The age at stent placement, prior surgical interventions, and indications for and sites of stent placement were noted. Also, the complications related to stent placement and the current airway status of the patients were reviewed. INTERVENTIONS: Twelve balloon-expandable metallic angioplasty stents (Palmaz; Johnson & Johnson Interventional Systems Co, Warren, NJ) were placed bronchoscopically in 6 patients. Six stents were placed in the lower trachea, and 6 were placed in the main bronchi. The stents were balloon expanded under fluoroscopic guidance. MAIN OUTCOME MEASURE: Discontinuation of mechanical ventilation. RESULTS: The age at stent placement ranged from 1.5 to 38 months (mean age at placement, 10 months). The indications for stent placement were (1) tracheomalacia or bronchomalacia, (2) pericardial patch or slide tracheoplasty failure, and (3) bronchomalacia caused by tetralogy of Fallot and large pulmonary arteries. The primary complication of stent placement was postoperative granulation tissue formation. One patient required the removal of 2 tracheal stents because of granulation tissue formation. There were 2 deaths in the series, 1 possibly related to stent placement. Four of the 6 patients were weaned from mechanical ventilation, and 3 experienced prolonged relief of airway obstruction. CONCLUSIONS: Metallic balloon-expandable stents are effective in relieving lower tracheomalacia and bronchomalacia in select patients. Only patients in whom conventional therapy has failed should be considered for stent placement.

Complete repair of Tetralogy of Fallot with absent pulmonary valve including the role of airway stenting.

Tetralogy of Fallot (TOF) with absent pulmonary valve (APV) represents an extreme form of tetralogy where pulmonary insufficiency and mild annular stenosis often results in massive pulmonary arterial (PA) dilatation. The aneurysmal left and right PAs often compress the adjacent trachea and bronchi, leading to airway obstruction and respiratory failure in infancy. Between 1991 and 1997, 11 patients underwent a single stage repair of TOF and APV using a valved (10 patients) or nonvalved (1 patient) homograft conduit and PA reduction arterioplasty. There was one (1/11 [9.1%) perioperative and one (9.1%) late death. Both deaths were related to airway complications. Morbidity associated with postoperative respiratory complications and ventilator-dependency due to underlying tracheobronchomalacia is an important problem. Intermediate follow-up shows a high incidence of reintervention for conduit stenosis and/or insufficiency and tracheobronchial compression. These infants also required multiple hospitalizations for recurrent respiratory infections secondary to their tracheobronchomalacia. Stenting of the right and left main bronchi with balloon expandable metallic stents is a new experimental therapy that has been useful in two recent patients with respiratory failure despite satisfactory intracardiac repair. It may provide an attractive alternative therapy to prolonged mechanical ventilation with positive end expiratory pressure in patients with severe tracheobronchomalacia. Complete repair with a valved homograft conduit and reduction pulmonary arterioplasty in infancy at the time of diagnosis is the procedure of choice for infants with TOF with APV. With this approach the patient outcome is essentially determined by their airway status and airway management.

Congenital absence of the epiglottis.

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.

Mucoepidermoid carcinoma of the trachea in a child.

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.

Tracheal neoplasms in children.

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.