Heterotopic neuroglial tissue causing airway obstruction in the newborn.

BACKGROUND: Heterotopic neuroglial (brain) tissue is a rare cause of airway obstruction in newborns. Fewer than 30 cases have been reported in the English literature. Brain heterotopias can mimic more common congenital anomalies of the head and neck. OBJECTIVE: To review our experience in the diagnosis and treatment of children with heterotopic pharyngeal neuroglial tissue. DESIGN: Case series. SETTING: Tertiary care children's hospital. PATIENTS: Four newborns with airway obstruction caused by heterotopic neuroglial tissue. RESULTS: All patients were infants (3 full-term girls and a 32 weeks' gestation boy) who had airway obstruction in the newborn period. All patients underwent preoperative computed tomography and magnetic resonance imaging, which revealed a heterogeneous mass involving the pharynx, neck, and parapharyngeal space. Bony deformities of the skull base and mandible were present in all patients, although intracranial connection was absent. Multiple surgical procedures were performed in all 4 patients. Tracheotomy was performed in 2 patients, gastrostomy tube placement was required in 3, and a nasopharyngeal tube was used in 1. Combined cervicofacial and transoral approaches were used for resection, preserving vital structures. Histopathologic evaluation revealed mature glial tissue and choroid plexus-like structures. CONCLUSIONS: Heterotopic neuroglial tissue must be considered in the differential diagnosis of airway obstruction in the newborn. Management is surgical resection, with attention to vital structures and function-analogous to surgery for lymphangioma. Multiple surgical procedures might be necessary in the treatment of these patients.

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.

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.

Gastroesophageal reflux in patients with subglottic stenosis.

OBJECTIVES: To determine the incidence of gastroesophageal reflux in patients with subglottic stenosis (SGS) and to determine if upper esophageal reflux occurs in addition to lower esophageal reflux in these patients. DESIGN: Esophageal pH probe studies were reviewed in patients diagnosed as having SGS. SETTING: A tertiary care pediatric medical center. PATIENTS: All patients diagnosed as having SGS between January 1990 and July 1996 who had undergone monitoring with an overnight esophageal pH probe. Seventy-four patients qualified for the study. All 74 patients underwent lower probe testing, and 55 of the 74 underwent dual (upper and lower) probe testing. MAIN OUTCOME MEASURES: The percent of time a pH measurement of less than 4.0 was recorded in the upper and lower esophagus. A lower probe pH measurement of less than 4.0 more than 10% of the study time was considered high risk for developing reflux-associated pathologic symptoms. A lower probe pH measurement of less than 4.0 for 5% to 10% of the study time was considered a marginal risk for developing reflux-associated pathologic symptoms. Upper probe criteria for reflux-associated symptoms have not been established. Therefore, patients were grouped as having a pH of less than 4.0 in the upper esophagus for 0%, 0.1% to 0.9%, 1.0% to 1.9%, 2.0% to 3.0%, or more than 3% of the study time. RESULTS: Thirty-seven of the 74 patients who underwent lower probe testing had a pH of less than 4.0 more than 5% of the study time, and 24 had a pH of less than 4.0 more than 10% of the study time. Twelve of the 55 patients who underwent upper probe testing had no measurable reflux; 27 of the 55 had a pH of less than 4.0 more than 1% of the study time; 14 had a pH of less than 4.0 more than 2% of the study time, and 11 had a pH of less than 4.0 more than 3% of the study time. CONCLUSIONS: Gastroesophageal reflux is frequently present in patients with SGS. Gastric contents frequently reach the upper and lower esophagus in these patients. In addition, the high incidence of gastroesophageal reflux in these patients suggests that it may play a role in the development of SGS. The possible effect of gastroesophageal reflux on the surgical repair of SGS requires further study.

Partial cricotracheal resection with primary anastomosis in the pediatric age group.

The traditional approach to severe subglottic stenosis (SGS) in the pediatric age group is laryngotracheal reconstruction (LTR). This approach may be complex and multistaged, with variable and unpredictable success rates in the individual patient. Excellent results have been reported in adults who had severe SGS and underwent partial resection of the cricoid and primary thyrotracheal anastomosis. This procedure has not been widely reported in infants and children. We report our experience with this procedure in 16 pediatric patients with grade III or IV SGS. Eleven patients had multiple previous LTR operations. The preoperative evaluation, surgical techniques, postoperative care, complications, and final results are described and discussed. Fourteen patients were decannulated after the procedure, 1 patient needed a second open procedure prior to decannulation, and 1 patient with concomitant bronchopulmonary dysplasia remains cannulated, for an overall 94% decannulation rate. Fourteen patients have no limitation of respiration, and 1 patient has moderate exercise intolerance. The results of this series suggest that partial cricotracheal resection with primary anastomosis is a relatively safe and effective procedure for pediatric patients with severe SGS.

Vascular compression of the airway: indications for and results of surgical management.

Vascular compression of the airway is a significant cause of respiratory compromise in children. While the indications for surgical repair are sometimes life threatening, they can also be subtle. This retrospective study examines 45 surgical cases of tracheobronchial compromise secondary to vascular compression at a large children's hospital between July 1983 and February 1996. A total of 34 were diagnosed with innominate artery compression, ten with a double aortic arch and one with an anomalous right subclavian artery. The 45 patients, 25 male and 20 female, ranged in age from 12 days to 11 years at surgery (average 13 months). A total of 21 (47%) presented with proven or suspected episodes of cyanosis or apnea. All 45 patients had evidence of vascular compression during microlaryngoscopy and bronchoscopy. The diagnosis was confirmed by magnetic resonance imaging (MRI) in 23/45 (51%), barium swallow in 22/45 (49%) and aortogram in 3/45 (7%). There was one death. One patient had a tracheotomy before surgery and continues to require it after surgery. Complete resolution of symptoms was achieved in 39/45 (87%) with five requiring more than one operation before their symptoms resolved completely. A total of four patients experienced a recurrence of symptoms within a variable length of time after surgery. Surgical indications and treatment alternatives will be discussed.

Cervicomediastinal thymic cysts.

Cervical thymic cysts are among the rarest congenital neck masses. They are probably more frequent than the number of cases reported as many of these lesions are asymptomatic and only discovered incidentally. The initial embryologic development of the thymus begins in the neck, followed by migration into the superior mediastinum. For this reason, extension of cervical thymic anomalies into the mediastinum is possible. Although it is the least common lateral cystic neck mass, it must be differentiated from other pediatric cystic neck masses, the majority of which are anomalies of the branchial system. Due to the possibility of mediastinal extension, the management of these lesions is different than other congenital neck masses. We report two cases of thymic anomalies with mediastinal extension and review the embryology, diagnosis, and management of cervicomediastinal thymic cysts.

Blunt laryngotracheal trauma in children.

OBJECTIVES: To demonstrate the diverse causes and manifestations of blunt laryngotracheal trauma in children, and to recommend an appropriate treatment protocol for these patients. DESIGN: A retrospective review of the medical records of patients treated at a tertiary care children's hospital for blunt laryngotracheal trauma during the 12 years before March 1, 1995 was performed. Clinical signs and symptoms, mechanisms of injury, and the results of laryngoscopy were included. PATIENTS: The study included 23 patients ranging from 2 1/2 to 18 1/2 years of age. The medical records of patients who had sustained an injury as a result of penetrating trauma, intubation, or foreign body were excluded. RESULTS: Four patients urgently required tracheotomies; 2 of these patients required subsequent reconstructive airway procedures. One child required a microlaryngoscopy with relocation of the arytenoid cartilage. The remaining 18 patients were treated conservatively with continuous pulse oximetry, cool mist room air, and serial flexible fiberoptic laryngoscopy. The 18 patients were discharged from the hospital after 24 to 48 hours of observation without sequelae. CONCLUSIONS: The signs and symptoms of blunt laryngotracheal trauma in children are not always specific to the extent or type of injury. A prompt diagnosis and treatment plan are needed to prevent potentially catastrophic complications. Patients with obvious airway compromise require immediate intervention. Those without acute airway symptoms often can be treated conservatively, provided that flexible fiberoptic laryngoscopy confirms a safe airway.

Chronic aspiration in children. When are bilateral submandibular gland excision and parotid duct ligation indicated?

OBJECTIVE: To assess the efficacy of bilateral submandibular gland excision and bilateral parotid duct ligation in treatment of chronic aspiration in neurologically impaired children. DESIGN: Retrospective chart review and telephone follow-up interview. SETTING: Tertiary care center. PATIENTS: All patients from 1986 through 1994 who underwent bilateral submandibular gland excision and bilateral parotid duct ligation and had evidence of at least 1 episode of aspiration pneumonia in the year prior to undergoing surgery. MAIN OUTCOME MEASURE: Two main outcomes measures were (1) the change in number of hospitalizations for pneumonia and total number of lower respiratory tract infections between 1 year before and 1 year after surgical intervention and (2) telephone assessment of patient outcome with respect to parental satisfaction, effect on quality of life, care requirements, amount of suctioning, and use of voice. RESULTS: Sixteen patients aged 16 months to 18 years were included. After surgical intervention, there was a significant decrease in the mean (+/-SD) number of pneumonias (2.3 +/- 1.44 before surgery, 0.9 +/- 1.2 after surgery; P < .001) and hospitalizations (1.2 +/- 0.8 before surgery, 0.4 +/- 0.8 after surgery; P < .005). Six patients had a tracheostomy at the time of surgery, and 1 required a tracheostomy 2 years after surgery. No individual required laryngotracheal separation. Eleven families were able to be contacted by telephone. Caretakers reported that in 8 of 11 patients, quality of life was improved and care requirements decreased. Seven patients used voice for at least some degree of communication. Three patients had postoperative complications involving the parotid glands; all resolved after further therapy. CONCLUSION: Bilateral submandibular gland excision and bilateral parotid duct ligation reduce the incidence of aspiration pneumonias and hospitalization, and decrease overall care requirements in a select group of neurologically impaired children. Because they are voice sparing, are efficacious, and have a low morbidity, they should be considered before laryngotracheal separation or tracheoesophageal diversion.

Selected risk factors in pediatric adenotonsillectomy.

OBJECTIVE: To evaluate the ability of a set of cost-effective criteria to identify before surgery the pediatric patients in whom perioperative respiratory compromise is most likely to develop after adenotonsillectomy. SETTING: A children's hospital medical center. DESIGN: Prospective study using preoperative parental questionnaires and perioperative respiratory status documentation. PATIENTS: All patients scheduled at the outpatient clinic were eligible. MAIN OUTCOME MEASURE: The development of respiratory compromise as defined by at least 1 of the following occurring more than 2 hours after surgery: an oxygen desaturation level of less than 90%, an obstructive breathing pattern, or respiratory distress requiring intervention. RESULTS: The risk of respiratory compromise was significantly increased in patients who were younger than 3 years (P < .001) and in those who had neuromuscular disorders (P < .05), chromosomal abnormalities (P < .005), difficulty in breathing during sleep (P < .005), restless sleep (P < .01), loud snoring with apnea (P < .05), or an upper respiratory tract infection within 4 weeks of surgery (P = .005). Respiratory compromise did not develop in any patients who did not snore (P < .05). CONCLUSIONS: A complete history that includes symptoms suggestive of sleep apnea will assist in the preoperative identification of pediatric patients most at risk for perioperative respiratory compromise after undergoing adenotonsillectomy. Such patients might benefit from overnight observation in a hospital setting. However, when snoring is absent, outpatient surgery is appropriate, as the risk of respiratory compromise is minimal.

Histoplasmosis: the otolaryngologist's perspective.

Within the upper aerodigestive tract, histoplasmosis often mimics carcinoma, making prompt and accurate diagnosis imperative. More severe and potentially lethal infections with Histoplasma capsulatum are now being seen as the numbers of patients at the extremes of age, as well as those with compromised immune systems, increase. We reviewed the cases of 115 hospitalized patients with disseminated histoplasmosis. Of these, 9 patients were identified with otolaryngologic manifestations: 4 were infected with human immunodeficiency virus (HIV), 1 was diabetic, and 3 were renal transplant patients. Sites of involvement included the larynx (in 2 cases) and the oral cavity and oral pharynx (in 7 cases). Eight of the 9 patients had a positive biopsy result; the other, a positive culture. Treatment with amphotericin B was generally effective, while the use of newer azole anti-fungal agents were less effective. As the number of immunocompromised patients continues to increase in modern clinical practice, histoplasmosis will undoubtedly be encountered more frequently in the head and neck area.