Effects of acid on the larynx of the maturing rabbit and their possible significance to the sudden infant death syndrome.

Sudden infant death syndrome (SIDS) has been shown to result from a variety of causes. One group of neonates at high risk for SIDS includes those who develop apnea secondary to gastroesophageal reflux (GER). Reflux has been shown to produce apnea in infants, and aggressive treatment results in significant improvement in symptoms. Because it is a site of resistance in the airway, the larynx plays an important role in the development of apnea. Through its sensory innervation, the larynx also serves as the afferent limb for reflexes that regulate respiration. In order to investigate the relationship between obstructive apnea and central apnea induced by the instillation of acid on the larynx, simulating GER, a rabbit model was developed. Maturing rabbits at 15-day intervals up to 60 days of age were studied using saline and acid solutions. Acid solutions produced obstructive apnea in all age groups. With acid solutions, central apnea occurred in all age groups but had a peak incidence at 45 days. Gasping respirations were seen in all groups but were most common at 30 days of age. Although obstructive and central apnea occurred together as mixed apnea, both types of apnea were seen independently of each other. Acid instilled on the larynx of maturing rabbits resulted in significant obstructive, central, and mixed apnea. Gasping respirations and frequent swallowing were frequent associated symptoms. Acid-induced obstructive apnea in rabbits mirrors symptoms seen in human infants with GER. Central apnea in infants with GER is seen less commonly; however, central apnea as the result of laryngeal stimulation has been demonstrated repeatedly in several animal models. Central apnea, culminating in fatal asphyxia, has been described in several animal models. The larynx appears to play a pivotal role in the development of apnea in susceptible infants with GER.

Head and neck manifestations of histiocytosis-X in children.

The clinical presentation and course of 100 children aged 6 months to 22 years who had histiocytosis-X and were treated at the Children's Hospital of Philadelphia from 1974 to 1987 were reviewed. Sixty-three percent of patients presented with complaints referable to the head and neck. Lesions of the skull occurred most frequently, followed by cephalic rash, osseous mandibular destruction, enlarged neck nodes, and gingival disruption. Overall, during the course of the disease, 82% of the children with histiocytosis-X developed head and neck manifestations. There were ten deaths in this series, all associated with diffuse or multifocal disease. Interestingly, four of these patients presented with cephalic rash as a harbinger of diffuse and eventually fatal illness. The method of diagnosis and management of histiocytosis-X is discussed.

Periauricular cysts and sinuses.

Periauricular cysts, sinuses, and fistulas occur commonly in the pediatric population. They arise from developmental defects of the first branchial cleft and first branchial arch. In most instances the diagnosis and management of these conditions are straightforward, but exceptional presentations sometimes occur. Failure to recognize these unusual cases may result in inadequate treatment and subsequent recurrence, and even if the correct diagnosis is made, surgical management of these lesions may be complicated. A series of 15 cases of periauricular congenital lesions is reviewed, of which three cases illustrating a diagnostic or surgical challenge are presented. The embryology, presentation, and management of these anomalies are discussed. This is one of the largest series of first branchial cleft anomalies reported in the literature, and our paper uniquely discusses first branchial cleft anomalies and preauricular sinuses together, with an emphasis on the surgical management of facial nerve, external ear, and middle ear involvement.

Vascular anomalies causing symptomatic tracheobronchial compression.

OBJECTIVES/HYPOTHESIS: To review the clinical presentation and diagnostic evaluation of patients with symptomatic congenital vascular anomalies causing tracheobronchial compression and to establish the short- and long-term results of surgical intervention with respect to postoperative complications, persistent symptoms, and ventilator and tracheostomy dependence. STUDY DESIGN: Retrospective review. METHODS: Chart review and telephone follow-up. RESULTS: Between 1987 and 1996, 35 children underwent surgical intervention to relieve symptomatic tracheobronchial compression resulting from a congenital vascular anomaly. Historically, the onset of symptoms occurs within the first months of life; however, only 12 (34%) of patients were diagnosed by 6 months of age and 13 (37%) were diagnosed at greater than 1 year of age. Excluding anomalous innominate artery, chest radiography or barium swallow was suggestive of a congenital vascular anomaly in 30 (94%) of the patients. Magnetic resonance imaging correctly delineated the anatomy of the vascular anomaly in 29 patients. Bronchoscopy was diagnostic in all three patients with anomalous innominate arteries causing tracheal compression. Postoperative follow-up was obtained in 32 (91%) of patients; 25 (78%) of these were asymptomatic at the time of their most recent examination. The remaining patients had persistent stridor, recurrent respiratory tract infections, and/or chronic cough. In all three patients who underwent postoperative bronchoscopy for persistent symptoms, tracheomalacia was demonstrated in the region of previous compression. CONCLUSIONS: Tracheobronchial compression from congenital vascular anomalies is a rare but treatable cause of respiratory symptoms. Early diagnosis requires a prompt, thorough clinical and radiologic evaluation. Surgery affords excellent long-term resolution of symptoms.

Vocal cord paralysis in children.

Bilateral vocal cord paralysis is a common cause of stridor in infants and children. There are significant differences in this entity between children and adults with regard to etiology, diagnosis, management, and outcome. A review of 10 years' experience at Children's Hospital of Philadelphia identified 51 children seen with the diagnosis of vocal cord paralysis. These cases were evaluated with respect to etiology of paralysis, whether unilateral or bilateral, delay in diagnosis, need for tracheotomy, abnormality of voice, surgical treatment, and outcome. Guidelines for management for a child with vocal cord paralysis are presented with emphasis on flexible endoscopic evaluation and conservative management.

Is outpatient tonsillectomy appropriate for young children?

The current literature suggests that outpatient tonsillectomy is a safe, cost-effective procedure. These reports have based their conclusions on the low rates of postoperative bleeding and dehydration. Generally, they have not examined other factors that may influence the postoperative course or identified groups of patients in whom outpatient management may not be appropriate. The literature regarding tonsillectomy in young children is conflicting. A retrospective analysis of the records of 223 children, 36 months of age and younger who had tonsillectomies, was performed. Postoperative airway complications including oxygen desaturation and airway obstruction developed in 115 patients. Seventeen (7.6%) children required postoperative care in an intensive care unit while an additional 117 (52.5%) patients received more than standard management. Preoperative apnea, an age of less than 12 months, and the presence of accompanying medical conditions were associated with a higher incidence of postoperative airway complications. It is recommended that tonsillectomy in patients under 36 months of age be planned as an inpatient procedure.

Nasopharyngeal malignancies in children.

A retrospective analysis identified 29 children with nasopharyngeal malignancies who were evaluated at the Children's Hospital of Philadelphia from 1970 through 1989. Rhabdomyosarcoma (15) and carcinoma (9) were the most common tumor types, and there were distinct differences in the clinical presentations of these two malignancies. Patients with rhabdomyosarcoma were generally younger than those with carcinoma and enjoyed longer survival. Six (67%) of the children with carcinoma were black; all of the patients with rhabdomyosarcoma were white. Patients with carcinoma were also more likely to present with cervical metastases. The presentation, evaluation, and methods of treatment for pediatric nasopharyngeal malignancies are discussed.

Nodular fasciitis of the head and neck in children. A deceptive lesion.

Nodular fasciitis represents a discrete, benign, presumably reactive proliferation of fibroblasts. However, its rapid rate of growth and a sarcomatous histologic appearance are often deceptive. Multiple pathologic reviews are frequently conducted in an attempt to distinguish nodular fasciitis from other lesions. Such confusion is especially problematic in the pediatric otolaryngic population in which nodular fasciitis is not commonly encountered and mesenchymal malignancies of the head and neck are of fundamental concern. Between 1976 and 1988, 12 cases of nodular fasciitis were diagnosed at the Children's Hospital of Philadelphia (Pa). Six children presented with head and neck lesions and ranged from 6 to 13 years of age. The clinical and histopathologic features of these cases are reviewed.

Acoustical factors of noise created by suctioning middle ear fluid.

OBJECTIVE: To quantify the intensity, spectral content, and duration of exposure for suction noise created during aspiration of middle ear fluid following myringotomy. DESIGN: Case series. SETTING: Tertiary care center. PATIENTS OR OTHER PARTICIPANTS: Convenience sample of 245 myringotomies performed on 124 patients ranging in age from 6 months to 14 years (mean age, 2 years 8 months). MAIN OUTCOME MEASURES: Intensity, spectral content, and duration of exposure associated with suction noise were defined by electroacoustic analysis of high-fidelity tape recordings of the noise created during suctioning middle ear fluid. RESULTS: The intensity of suction noise ranged from 74 to 117 dB; most acoustic energy was concentrated in the frequency range between 1.7 and 6.0 kHz, and the duration of exposure varied from 4 to 23 seconds.

Congenital airway abnormalities in patients requiring hospitalization.

OBJECTIVE: To determine the cause of congenital airway abnormalities in pediatric patients requiring hospitalization for their respiratory status. DESIGN AND SETTING: Case series in a tertiary care center. PATIENTS: A 5-year retrospective chart review was conducted at our institution. A total of 174 patients were identified who required hospitalization for their respiratory status as a result of a congenital airway abnormality. RESULTS: Of the 174 patients, 114 (65.5%) were male and 60 (34.5%) were female. Eighty patients (47%) presented within the first 3 months of life. Forty-six patients (26%) were born prematurely, and 49 patients (28%) were diagnosed as having gastroesophageal reflux. The majority of patients (139 [80%]) had multiple presenting symptoms or signs. Stridor was the most common (129 [74%]), followed by accessory respiratory effort, cyanosis, apnea, and failure to thrive. Diagnosis was made at the time of surgical evaluation in 91% of the patients, with the remaining diagnoses made using radiological findings and/or clinical evaluation. Sixty-five patients (37%) had multiple sites of airway abnormalities; laryngeal abnormalities were noted almost 3 times as often as tracheal abnormalities (161 vs 62, respectively). Of the laryngeal abnormalities, laryngomalacia was the most common, followed by glottic web, subglottic stenosis, vocal-cord paralysis, and subglottic hemangioma. Tracheomalacia was the most common tracheal abnormality, followed by external compression and tracheal stenosis. Thirty-three patients (19%) required tracheotomy for management of recurrent respiratory decompensation. CONCLUSIONS: While congenital airway abnormalities are usually self-limited, those patients requiring hospitalization represent a group with a more severe respiratory status who have a greater chance of requiring tracheotomy. The recognizable percentage of patients with gastroesophageal reflux and prematurity accounts for comorbid factors in the need for hospitalization for respiratory issues related to congenital airway abnormalities.

Vocal cord injection in children with unilateral vocal cord paralysis.

Unilateral vocal cord paralysis (UVCP) in children is uncommon and rarely leads to serious sequelae. However, on rare occasions, it can present with severe aspiration and dysphonia. Several therapeutic techniques have been used in adults with UVCP, but the reported alternatives in children have been much more limited. Observation and speech therapy are the standard treatment. We describe three children with UVCP and severe aspiration who were treated with vocal cord injection. The treatment indications, clinical courses, and outcomes of the three cases are detailed. The injection of vocal cords in children is discussed, with an emphasis on those aspects unique to the management of UVCP in pediatric patients. Alternative surgical treatment modalities are also presented. Vocal cord injection is an effective and viable therapeutic option for the management of UVCP in certain pediatric patients with severe aspiration and dysphonia.

Tracheobronchomalacia in children.

Tracheobronchomalacia (TBM) is an important cause of airway distress during infancy, but it generally resolves as the airway enlarges. To assess the origin and natural history of TBM, a chart review and telephone survey were conducted for 50 patients with TBM at the Children's Hospital of Philadelphia (Pa). This study revealed that TBM is a relatively common airway abnormality and is found on 15% of all diagnostic bronchoscopies. Prematurity, low birth weight, bronchopulmonary dysplasia, and prolonged ventilation predispose patients to the most severe symptoms. In our study, a tracheotomy with continuous positive airway pressure was required by 75% of the premature infants and 25% of the full-term infants with TBM. Seventy-one percent of all patients underwent decannulation without any other surgical intervention and remained nearly asymptomatic. Some patients could not undergo decannulation because of other airway lesions. In most instances, TBM is a self-limited disease that resolves without surgery.

Mandibular fractures in the pediatric patient.

Seventy-three patients were admitted to the Children's Hospital of Philadelphia (Pa) between January 1979 and June 1989 with a diagnosis of mandibular fracture. Data were obtained through a retrospective review of these cases. The cases were divided into three age groups that reflected the developing structure of the mandible and the maturation of the dentition. A trend toward a greater number of fractures and a predominance of males is shown with increasing age. Child abuse is a relatively frequent cause of fractures throughout all groups. Associated injuries are more common in young children, except in cases where abuse has been documented. The high osteogenic potential of the pediatric mandible allowed conservative management to be successful in 25% of younger patients and was responsible for a low complication rate overall.

Ventilation tube removal. Indications for paper patch myringoplasty.

Paper patch myringoplasties are commonly performed in children at the time of ventilation tube removal, yet no series documenting their efficacy appears in the otolaryngology literature. Over an 18-month period at the Children's Hospital of Philadelphia (Pa), 163 ventilation tube removals were performed on 131 children aged 1 to 18 years. In 95 of these ears, a paper patch myringoplasty was performed after tube removal. No significant difference (87% vs 85%) in the overall healing rate at 6 months was shown between these two groups. An analysis of the variables potentially affecting outcome revealed that healing following simple tube removal was influenced by the frequency of tube placement and by tube size. Paper patch myringoplasty significantly improved outcome (93% vs 61%) in ears with more than three previous tube placements.

Endoscopic assessment in children with tracheotomies.

Pediatric tracheotomy is a safe procedure, and the tube can be maintained for a prolonged period. There are, however, few guidelines regarding the endoscopic assessment of the airway following tracheotomy. From January 1988 through December 1989, the Division of Otolaryngology of The Children's Hospital of Philadelphia (Pa) performed tracheotomies on 81 patients. Thirty-two children had direct laryngoscopy and bronchoscopy at the time of the procedure, 1 week later, and at 6-month intervals for a minimum of 18 months. Four children had normal airways while 21 had intraluminal stomal granulomas, 16 had development of subglottic stenosis, nine had collapse of the anterior tracheal wall, and three had development of distal tracheal granulations. Sixteen children had multiple lesions. Anatomic changes occur in the airways of the majority of children with long-term tracheotomies, and endoscopic evaluation is an essential part of their care.

Congenital tracheal stenosis. The otolaryngologist's perspective.

Congenital tracheal stenosis is a rare congenital anomaly, with less than 70 reported cases in the literature. The presenting signs and symptoms of stridor, recurrent pneumonia, and respiratory distress are commonly seen in other conditions. The rarity of congenital tracheal stenosis and the diverse presentations make accurate early diagnosis difficult and frequently lead to inappropriate treatment. We treated three patients with congenital tracheal stenosis who presented with different sites of stenosis. Each patient displayed different symptoms and required individualized management. The treatment of congenital tracheal stenosis depends on identifying the site and extent of the stenosis. We reviewed the embryogenesis and treatment of this abnormality and developed a new classification system that will aid in the management of congenital tracheal stenosis.

Microbiology of the tonsils and adenoids in a pediatric population.

To investigate the microbial flora of the tonsils and adenoids, the core tissue from the tonsils and adenoids of 50 children undergoing tonsillectomy and adenoidectomy for either recurrent infection or airway obstruction was cultured aerobically and anaerobically, and the number of bacterial colonies was quantitated. The most common organisms isolated were alpha-hemolytic streptococci, nonpathogenic Neisseria species, Haemophilus species, Staphylococcus aureus, and Corynebacterium species. No anaerobes were identified. Bacterial isolates from the tonsils and adenoids were similar in number and frequency of occurrence. Potential pathogenic bacteria (Haemophilus species, S aureus, beta-hemolytic streptococci, and Streptococcus pneumoniae) were identified in 40 patients. Seventy-three percent of these patients shared a common pathogen in tonsil and adenoid tissue. Haemophilus species were recovered in 54% of patients and S aureus in 46%. No significant difference exists between the type and number of pathogens in patients undergoing adenotonsillectomy for recurrent infection or obstruction.

The effect of antibiotic therapy on recovery after tonsillectomy in children. A controlled study.

Tonsillectomy continues to be a commonly performed operation in the pediatric age group. The postoperative period is often protracted and characterized by throat and ear pain, intermittent fever, foul odor from the oral cavity, and poor oral intake. Consequently, antibiotics are frequently prescribed in an effort to minimize these symptoms and/or avoid complications such as dehydration or secondary infection of the operative site. However, to our knowledge, no study to date has been performed to demonstrate the efficacy of antibiotic therapy in this setting. At the Children's Hospital of Philadelphia, a prospective, randomized, double-blind study was undertaken in which ampicillin (or placebo) was administered intravenously at the time of surgery and for 12 to 24 hours postoperatively. The children then continued to receive oral amoxicillin therapy (or placebo) for an additional seven days. The patients were then evaluated for the incidence and severity of postoperative symptoms and complications. Intraoperative cultures of the oropharynx and tonsillar tissue, as well as cultures of the tonsillar fossa, were obtained following completion of one week of therapy. Our results indicate that ampicillin sodium/amoxicillin trihydrate therapy is well tolerated and safe in the nonallergic child and is effective in minimizing fever and other troublesome postoperative symptoms, such as pain, lassitude, mouth odor, and poor oral intake after tonsillectomy.

Rhabdomyosarcoma of the head and neck in children.

Rhabdomyosarcoma is the most common soft-tissue sarcoma in infants and children, with the head and neck being the most frequent site of involvement. Treatment for this neoplasm has undergone many changes, with a much improved prognosis using a combination of surgery, radiation therapy, and chemotherapy. This retrospective analysis presents the management and outcome of 60 children (aged 3 months to 18 years) with rhabdomyosarcoma of the head and neck evaluated at the Children's Hospital of Philadelphia (Pa) between 1970 and 1987. The overall death rate for all head and neck sites decreased from 50% in 1970 to 1979 to 23% in 1980 to 1987, reflecting the improved management protocol.

Computed tomography in the evaluation of pediatric neck infections.

In children, infections involving both the superficial and deep neck spaces are common. Children so affected typically present with fever, neck mass, neck stiffness, and, occasionally, airway compromise. Radiologic modalities used in the evaluation of neck infections include plain lateral neck radiography, ultrasound, computed tomography, and magnetic resonance imaging. All these modalities have proved useful in the treatment of such infections, specifically the decision to perform incision and drainage. The charts of 66 patients-33 with superficial and 33 with deep neck infections-were analyzed with respect to symptoms, signs, computed tomography findings, and need for surgical intervention. Computed tomography was not particularly helpful in superficial neck infections with regard to the decision to perform surgical drainage; however, it did localize and demonstrate the extent of infection. In deep neck infections we found a 92% correlation between computed tomographic evidence of an abscess and surgical confirmation of one. Contrast-enhanced computed tomography remains an excellent tool in the treatment of neck infections in children.