Pediatric tracheostomy: a changing procedure?

In 1982, the experience with tracheostomy at The Children's Hospital of Philadelphia was reported for 1971 through 1980. We have now reviewed 450 cases for the period from 1981 through 1992, and compared the characteristics of these cases with those in the previous review. Long-term follow-up was available on 83% of cases, and the median follow-up was 2.96 years. Patients received a tracheostomy for airway obstruction (38%), chronic ventilation (53%), or multiple indications (9%). The mean duration of tracheotomy (adjusted for death and loss to follow-up) was 2.13 years. The tracheostomy-related mortality was 0.5%, and the nontracheostomy-related mortality was 22%. Nineteen percent of patients had complications in the first postoperative week, and 58% had 1 or more late complications. In comparison with the previous study from our institution, there was a great increase in long-term tracheostomy and a continuing trend away from tracheostomy for short-term airway management. Better monitoring and improvements in parental teaching may have contributed to a decrease in tracheostomy-related mortality.

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.

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.

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.

Comparison of endoscopy and radiographic fluoroscopy in the evaluation of pediatric congenital airway abnormalities.

In order to assess the accuracy of conventional dynamic radiographic studies compared to endoscopy in the diagnosis of congenital airway abnormalities, we performed a retrospective chart review for the period between July 1991 to June 1996. A total of 186 patients were identified who required hospitalization for their respiratory status as a result of a congenital airway abnormality. Of these, 19% had both endoscopy and conventional dynamic radiographic evaluation (airway fluoroscopy, barium esophagography, or both). Eleven percent had fluoroscopy only, 62% had endoscopy only, and 9% had neither. In those patients who underwent both endoscopic and conventional dynamic radiographic evaluation, endoscopy was considered to be the definitive procedure for diagnosis. Laryngomalacia was present in 94% of these patients. Thirteen patients had multiple sites of airway abnormalities on endoscopy, and a total of 51 abnormalities were identified. Dynamic radiographic evaluation was correct in four, was suggestive of the abnormality in 12, did not recognize an abnormality in 33, and suggested a different diagnosis (not corresponding to endoscopy) in two. Thus, airway fluoroscopy and/or barium esophagography were correct or suggestive in 16 of 51 abnormalities (31%). We conclude that endoscopy is required in the majority of hospitalized patients for the precise diagnosis of a congenital airway abnormality. Conventional dynamic radiographic studies are helpful to confirm a suspected diagnosis in patients with a strong clinical history and physical examination. When the diagnosis is not clear based on the patient presentation, endoscopy is more definitive than conventional dynamic radiography in identifying pediatric congenital airway abnormalities.

Laryngomalacia: a proposed classification form.

Laryngomalacia is a common cause of stridor in children. The disorder has a heterogenous presentation, from the mildest form, which resolves with maturation, to the most severe form, requiring tracheotomy. While there is a vast literature on the subject, there is neither stratification nor correlation of clinical presentation, endoscopic appearance, treatment and outcome. In order to statistically evaluate the choice of treatment based upon presentation, patients must first be classified by relevant predictors of disease severity. A form is proposed to classify the clinical presentation of laryngomalacia by recording relevant historical and anatomic factors. Historical factors are classified by (1) severity of stridor; (2) weight gain; (3) age at presentation; and (4) neurologic status, forming the mnemonic SWAN. The principal anatomic site of collapse is recorded as: (1) postero-lateral; (2) posterior; or (3) anterior. Endoscopic findings consistent with gastroesophageal reflux disease (GERD) or gross aspiration are noted. Photographic and/or video documentation is performed when possible. A pilot study was undertaken to determine the ease of use of this instrument. Ten children, four boys and six girls, were classified. Ages ranged from 1-day-old to 19 months, with a mean of 9 months. Five children were examined in the clinic and five in the operating room. The form was readily and easily applied, and allowed the heterogeneity of the disorder to be organized. Wider application of this form across institutions, with classification of patients with laryngomalacia by historical and anatomic factors, should allow the accumulation of sufficient numbers of patients to allow statistical analyses of treatment and outcome as they relate to the initial presentation of this disorder of airway dynamics.

Congenital airway abnormalities requiring tracheotomy: a profile of 56 patients and their diagnoses over a 9 year period.

We reviewed the 9 year experience at the Children's Hospital of Philadelphia with patients requiring tracheotomy for a diagnosis of congenital airway abnormalities. Of the 56 patients, 28 (50%) had cardiovascular, or chromosomal abnormalities, neurologic conditions, or congenital syndromes, 24 (43%) were born prematurely, and 13 (23%) were found to have gastroesophageal reflux. Only 18 (32%) went on to eventual decannulation of their tracheotomy with a mean tracheotomy duration of 1.75 years. The majority of patients (75%) had multiple presenting signs. Stridor was the most common (54%), followed by accessory respiratory effort (39%), cyanosis (30%), apnea (29%), and failure to thrive (23%). Twenty eight patients (50%) had multiple airway abnormalities contributing to their need of a tracheotomy for airway protection orr ventilator dependence. Laryngeal abnormalities were found in 71% of patients, tracheal abnormalities in 48% of patients, bronchial abnormalities in 11%, and upper airway obstruction in 14%. Of the laryngeal abnormalities, laryngomalacia was the most common, followed by subglottic stenosis, glottic web, and vocal cord paralysis. Tracheomalacia was the most common tracheal abnormality. The relatively large percentage of patients with cardiovascular or other major malformations, and prematurity, accounts for comorbid factors in the need for prolonged tracheotomy (and low early decannulation rate). Although gastroesophageal reflux was found in a recognizable portion off the patients, it is unclear whether this represents a comorbid condition.

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.

Nontuberculous mycobacterial cervical adenitis.

Granulomatous inflammation is a common finding in pathologic evaluation of surgically excised chronic lymphadenopathy in children. Confusion exists regarding diagnosis and management of these lesions. Over a 10-year period at The Children's Hospital of Philadelphia, a total of 81 children were identified with biopsy-confirmed granulomatous lesions of the head and neck, with nontuberculous mycobacteria (NTM) accounting for 67 of the cases. The typical presentation was that of a nontender mass in the cervicofacial area present for weeks to months, unresponsive to antimicrobials. All underwent surgical excision, which was curative in 54 patients; 13 children required additional procedures. This paper reviews NTM, its typical clinical presentation, difficulty in diagnosis, and the methods of treatment.

The effects of gelatin film stents in the middle meatus.

Controversy exists regarding the management of the middle meatus after pediatric functional endoscopic sinus surgery (FESS). To prevent adhesions following pediatric FESS, gelatin film stenting of the middle meatus has been recommended. The effects of stenting, however, have not been established. Fifty-one children with similar degrees of bilateral sinus disease had a gelatin film stent placed in one middle meatus on completion of FESS, while the opposite meatus was not stented. Two to three weeks later at the time of a second, staged procedure, the sides were compared for the presence of the stent, adhesions, granulaion tissue, and patency of the maxillary sinus ostia. In 11 children the postoperative findings were more severe in the side without the stent, whereas in 29 children they were more severe in the stented side. There was no difference between the sides in 11 children. Although gelatin film stenting benefits some children, it should not be used routinely following pediatric FESS but should be reserved for children who are predisposed to develop adhesions or have poor prognostic factors, such as immunodeficiency and ciliary dyskinesia.

A case of familial sternocleidomastoid tumor of infancy.

Sternocleidomastoid tumor of infancy (STOI) is a benign, firm, fibrous swelling predominantly involving the middle or inferior third of the sternocleidomastoid muscle. Patients may present simultaneously with, or progress to the development of, congenital, muscular torticollis (CMT) during childhood. This pseudotumor affects infants in their first few weeks of life with the vast majority showing complete regression over the ensuing few months. Multiple postulates have been presented as to the exact etiology of STOI; however, the cause is still unknown. Two siblings, both requiring vacuum extraction during delivery, presented at 4 weeks of age to the Children's Hospital of Philadelphia (CHOP) with STOI. The possible genetic predisposition of these siblings to develop STOI is hypothesized. Familial cases of CMT have been reported along with the possible genetic or peripartum factors that could play a role in its development. We reviewed these same mechanisms that could also predispose to familial STOI. Currently, magnetic resonance imaging is the diagnostic modality of choice. Fortunately, the majority of STOI responds to conservative measures with aggressive physical therapy, thus avoiding the need for operative intervention.

Endoscopic repair of traumatic CSF rhinorrhea in a pediatric patient.

Cerebrospinal fluid (CSF) rhinorrhea typically results from trauma to the skull base, producing leaks through either the cribiform plate region or the sphenoid bone. Traditional approaches to the repair of such leaks include a frontal craniotomy or external ethmoidectomy. An endoscopic approach through the nose has also proven to be successful. A 7-year-old male suffered traumatic CSF rhinorrhea and development of a meningocele in the region of the cribiform plate. After demonstrating the site of the leak with fluorescein dye, the defect was repaired via a transnasal endoscopic approach. The evaluation of the child with CSF rhinorrhea, including the presenting symptoms and signs and the radiographic assessment, is presented. The variety of approaches, types of repair and post-operative care are also discussed.

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.

Otologic manifestations of neurofibromatosis.

A chart review of 440 patients with neurofibromatosis seen at the Children's Hospital of Philadelphia Neurofibromatosis Clinic between 1980 and 1991 was performed to look for otologic manifestations. Neurofibromatosis 1 was present in 434 patients, and neurofibromatosis 2 was present in 6. A total of 31 otologic abnormalities related to neurofibromatosis were found in 28 patients. Neurofibromas of the external ear, including the pinna and the external auditory canal, were the most common finding. Middle ear neurofibromas were found in two patients. Acoustic neuromas were seen exclusively in patients believed to have neurofibromatosis 2. One cerebellopontine angle neurofibrosarcoma was found in a patient with neurofibromatosis 1.

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.

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.