Surgical advances in the treatment of lesions of the pediatric airway: the role of the carbon dioxide laser.

Transoral microsurgery of the larynx has now made it possible to treat multiple lesions heretofore only treatable by external approach. The introduction of the surgical carbon dioxide laser has been the single greatest advance in transoral surgery of the airway in the past 15 years. This modality of therapy offers many features not found in any other known form of surgery. It is especially useful in the pediatric airway where the avoidance of edema and the need for minimal scarring is most critical. Its unique aspects make this form of treatment most desirable in the pediatric laryngotracheal region.

Ataxia and hearing loss secondary to perilymphatic fistula.

Ataxia is rarely attributed to lesions of the peripheral vestibular system. In 1973, the first case of ataxia and hearing loss secondary to a labyrinthine fistula was reported. Until now, this syndrome has not been reported in patients under the age of 10 years. A case is presented of a 5-year-old boy with symptoms of ataxia and hearing loss as well as vertigo and tinnitus after head trauma. Three physical findings appear to be most characteristic of patients with perilymphatic fistulas: a positive fistula response, positive positional testing with the involved ear down, and evidence of vestibular ataxia when testing station and gait. The absolute diagnosis of perilymphatic fistula can only be established by exploration of the middle ear space. If a fistula is found, it may be sealed with soft tissue and, if this fails, actual stapedectomy may be required.

The fourth branchial cleft anomaly.

The fourth branchial cleft anomaly has long been postulated. A case in which a lower neck sinus tract extended under the clavicle near the subclavian vessels is presented. The distinguishing features of bronchogenic and branchogenic anomalies are discussed. It appears that unless a complication (e.g., abscess, inflammatory process) of the fourth branchial cleft sinus tract occurs within the mediastinum, mandating full exploration, the complete form of the anomaly may continue to remain undocumented.

Rhabdomyosarcoma of the head and neck: diagnosis and management.

Rhabdomyosarcoma represents the most common soft-tissue neoplasm of the head and neck found in children. The tumor is most commonly seen in Caucasian children under the age of 12, usually presenting as a painless mass. Distant metastases are frequently present, especially in regional lymph nodes, lung, and bone marrow. All patients suspected of having this disorder should have a thorough examination of the head and neck, as well as a complete radiographic evaluation of the primary region. Histologic disagnosis should be made as rapidly as possible. Once this has been accomplished, a thorough search for metastatic disease can be undertaken. Therapy must be individualized, and a team approach is advocated. The roles of surgery, radiation therapy, and chemotherapy are discussed. A staging system and a treatment-therapy plan are outlined.

Carcinoma of the palatine arch.

1. Carcinoma of the palatine arch is part of a regional diathesis of carcinoma of the mucosa of the upper aerodigestive tract and this concept has a definite influence on the choice of treatment. 2. Surgery has an important role in the management of both the primary tumors and the regional metastatic nodes. 3. Radiation therapy is indispensible in the management of carcinoma of the tonsillar crypts, stage IV tumors of the palatine arch, and primary tumors of the palatine arch with certain specific extensions. 4. Comprehensive management of carcinoma of the palatine arch requires careful evaluation of each patient and his disease, vigorous application of the chosen method of treatment by either the surgeon or the radiotherapist, and continuous follow-up study and supportive treatment for the rest of the patient's life.

A randomized trial of preoperative radiotherapy in cancer of the oropharynx and hypopharynx.

One hundred patients with stage II and stage III cancer of the oropharynx and hypopharynx were treated under a protocol in which they were randomly selected for treatment by surgery alone or by combined preoperative radiotherapy and surgery. The schedule of preoperative radiation therapy chosen was 2,000 rads from a cobalt 60 machine delivered in five days. Eighty-six of the patients were evaluable at three years; there was no difference in the outcome of the treatment of the two groups. A similar study is urgently needed to determine the value of postoperative radiotherapy in the management of similar cancers.

An approach to the nasal septum in children.

Surgery of the nasal septum has long been discouraged in the pediatric population. Concerns about growth and development of the nose have led surgeons to adopt an extremely cautious attitude toward the correction of nasal septal deformities in childhood. More recently, studies have shown that with proper preservation of septal cartilage, surgery can be safely undertaken in this area. Frequently, however, exposure to the nasal septum is limited in small children due to the size of the nasal vestibule. Sublabial septoplasty has been safely carried out now in ten patients ranging in age from 4 to 9 years. This approach allows for complete access to the nasal septum while avoiding any external cosmetic deformity. The nasal septum has been preserved in all cases through morsalization and repositioning. Growth and development of the nose has been followed for up to 60 months without evidence of deformity or alteration. Sublabial septoplasty would, therefore, appear to be a safe and cosmetically acceptable approach to the correction of severe nasal septal deformities of childhood.

An experimental model for the endoscopic correction of subglottic stenosis with clinical applications.

The use of long-term endotracheal intubation in the treatment of respiratory distress syndrome in the newborn has seen an increase in the number of cases of acquired subglottic stenosis. Multiple methods of correction have been advocated in conjunction with the use of antibiotics, corticosteroids, and indwelling stents. An animal experiment utilizing 64 dogs was undertaken in an effort to devise an endoscopic procedure using the CO2 laser which might be successfully employed in the human patient. An attempt was also made to study the efficacy of antibiotics, corticosteroids, soft stents and rigid stents. Animals were divided into multiple groups, and their larynges were studied grossly and histologically some weeks after therapy was completed. Analysis of the results was undertaken, and the method with the best outcome was then employed in the human portion of the study. Twenty-four human patients, 12 with severe stenosis and 12 with partial stenosis were evaluated, and their outcome is reported.

Sleep apnea syndrome associated with upper airway obstruction.

A working protocol is presented which has developed to help the clinician in the diagnosis and management of patients suffering from the sleep apnea syndrome associated with various types of upper airway obstruction.

The microbiology of chronic middle ear effusions in children.

Serous otitis media is the most common cause of hearing loss in childhood. Traditionally, this entity has been felt to be a sterile process. This study was undertaken to determine the incidence of infected middle ear fluid in patients with asymptomatic middle ear effusions. The external auditory canal, nasopharynx and middle ear fluids of 57 patients undergoing myringotomy were cultured. Of 57 patients, 26 had positive cultures; 67% of patients less than 36 months of age had positive culture, with a bacterial flora closely resembling that of acute otitis media. Further investigation is required to determine the significance of such infection together with the optimum therapy for chronic effusions of the middle ear in young children.

Fulminant aspergillosis of the nose and paranasal sinuses: a new clinical entity.

Fulminant aspergillosis of the nose and paranasal sinuses represents a new clinical entity occurring in individuals with depressed immunological responses. It is marked by a rapid malignant course, requiring early recognition, aggressive surgery and chemotherapy. Clinical manifestations include a rapidly progressive gangrenous mucoperiostitis advancing relentlessly to destruction of the nasal cavity and the paranasal sinuses within a few days. The recent emergence of this form of aspergillosis appears to be directly related to the increased intensity of chemotherapy and immunosuppression in the treatment of previously fatal neoplastic diseases. Control of this disease process requires aggressive therapy. This may include radical sinus ablation, debridement of nasal structures, chemotherapy and possible correction of immunological deficits, i.e., bone marrow transplantation. Four cases are discussed in detail to present the clinical spectrum of this new disease entity.

Congenital cholesteatoma of the middle ear in children: a clinical and histopathological report.

Forty-one children with congenital cholesteatoma of the middle ear seen from 1978 through 1989 are reviewed. The most common presentation was that of an asymptomatic white mass behind a normal intact tympanic membrane. Computed tomography (CT) scan was useful in documenting extension beyond the mesotympanum. Surgical removal was performed using an extended tympanotomy for lesions in the middle ear and tympanomastoidectomy for those that had extended into attic and mastoid air cells. Observation over an average 3.1-year period indicated that 80% of children were free of disease after initial surgery. Residual disease that required further surgery was present in 20%. The importance of early diagnosis of congenital cholesteatoma is strongly advocated. The prognosis is better when the cholesteatoma is confined to the anterosuperior quadrant of the middle ear. Seventeen patients in this study had such a lesion, and extended tympanotomy allowed removal of an encapsulated closed cholesteatoma with normal postoperative hearing and no residual cholesteatoma. The average age was 2.3 years. Temporal bone histopathological studies of three cases of congenital cholesteatoma demonstrate two distinct pathological types of congenital cholesteatoma. A "closed" keratotic cyst in the anterior mesotympanum, which is easily removed, and an "open" infiltrative type in which there is no containment of the keratotic debris and the cholesteatoma matrix is in direct continuity with middle ear mucosa. Surgical extirpation of the "open" type is difficult and more likely to be associated with residual disease.

Penetrating trauma of the oropharynx in children.

Penetrating trauma of the oropharynx is not an uncommon problem in the pediatric population. Innocuous injuries with minor soft-tissue trauma have been associated with severe neurologic sequelae. A review of 77 oral trauma cases that occurred from 1981 to 1990 at Boston Children's Hospital was undertaken to see if constant factors could be identified so that a treatment protocol might be outlined for these patients. Twenty-three (30%) patients sustained injury to the soft palate and peritonsillar area. Fifty percent of these cases required surgical debridement and repair. None of the 23 patients developed neurological sequelae. Physical examination may not correlate with the development of symptoms nor the mechanism of injury. Care should be taken during the examination to identify any neurological problems that may not relate to the degree of injury. Conservative management can be entertained if no abnormal neurologic findings are noted.

Cerebrospinal fistulas in children.

Cerebrospinal fluid leaks can be difficult management problems. Little information has been published about handling these cases in the pediatric population. A review of cases over the past 10 years at Boston Children's Hospital was undertaken to identify any prognostic factors and therapeutic recommendations. Twenty-seven patients were identified. While most patients responded to conservative therapy, those with leaks that persisted longer than 8 days required some type of operative closure. Severity of injury had little correlation with the occurrence of a leak. The findings in this study indicate that most pediatric cases can be managed on a conservative basis.

Bifid epiglottis: a rare laryngeal anomaly.

Two cases of bifid epiglottis are presented: one with an associated laryngeal cyst and another with an associated cricoid stenosis. The occurrence of multiple laryngeal anomalies in association with bifid epiglottis has not previously been described. The occurrence of an extra digit is noted to be statistically significant both in the current series and in a review of the literature. A brief review of the embryologic classification and staging by the Carnegie System, and the correlation of the time sequence of development of the epiglottis is presented. No correlation is made as to the mechanism of the origin of this laryngeal anomaly, as adequate embryologic knowledge of the development of the pharynx is not available at this time.

Treatment of subglottic hemangioma with the carbon dioxide laser.

Congenital subglottic hemangioma is a potentially life-threatening lesion usually presenting in the first three months of life. Over the years, multiple treatment modalities have been advocated. However, none has been uniformly successful, and morbidity and mortality remain high. With the advent of the carbon dioxide surgical laser, it soon became apparent that this was an ideal tool for the pediatric larynx. The already documented properties of the laser have led to the application of this form of tissue destruction in 11 patients with subglotic hemangioma. This modality has proven to be a safe, effective and rapid means of treating this troublesome lesion.

Hemophilus influenzae type B soft tissue infections of the head and neck.

Hemophilus influenzae soft tissue infections produce a variety of specific and dangerous pathologic manifestations in the head and neck. Problems include periorbital cellulitis and abscess, sinusitis, supraglottitis and otitis media. Complications include meningitis, airway obstruction and death. Pediatric patients are especially susceptible to these infections and complications. Effective management includes correct diagnosis, prevention of complications, and high doses of appropriate antibiotics. Increasing ampicillin resistance necessitates alternative antibiotic regimens. Specific physical findings, diagnostic studies and appropriate current treatments are discussed with illustrative cases from Children's Hospital Medical Center, Boston, and reference to pertinent recent literature.

Central nervous system complications associated with acute otitis media in children.

The purpose of this article is to review a subset of pediatric patients with otitis media occurring simultaneously with meningitis and other central nervous system diseases. Of the 92 cases reviewed, 61.3% involved patients under 12 months of age. Meningitis associated with otitis media involved 91% of the patients; the remainder had nonmeningitic central involvement. The most striking finding was the high incidence of Haemophilus influenza type-B, a relatively uncommon pathogen of the middle ear. This finding highlights the need to select antibiotic coverage to adequately treat Haemophilus influenza type-B.

Bacterial tracheitis--two-year experience.

Inflammatory illnesses of the pediatric airway cause significant morbidity and mortality. Bacterial tracheitis is a distinct entity with features common to both croup and epiglottitis. Ten patients between the ages of 3 months and 12 years were treated at Children's Hospital, Boston, MA., for bacterial tracheitis. The clinical presentation and medical management is discussed. Seven of the patients required both direct laryngoscopy and bronchoscopy; one patient required urgent intubation; and one patient required indirect laryngoscopy. In one patient the diagnosis was based on clinical and radiographic findings in conjunction with tracheal aspirates. Seven of the 10 patients did well with aggressive medical management. Three patients required endotracheal intubation. No patient required tracheotomy, and there were no cardiopulmonary arrests. It is of particular interest that although the patients in this series presented in a manner similar to that of patients in other published series, the management is significantly different and the overall outcome is significantly better.

Computed tomography in the evaluation of choanal atresia in infants and children.

While traditional confirmatory clinical tests are helpful in establishing the diagnosis of choanal atresia, they give little useful information concerning the composition, position, and thickness of the atresia plate. Surgeons experienced in the repair of choanal atresia have recognized that detailed anatomic assessment is valuable in the preoperative selection of the method of repair and design of the repair once undertaken. The utilization of high-resolution computerized tomography to provide such information in a group of infants and children with choanal atresia forms the basis for this report. The application of computed tomography in the evaluation of choanal atresia and preoperative surgical planning is discussed. Based on this experience, we feel that the introduction of high-resolution computerized tomography is a significant advance in the diagnostic and therapeutic approach to choanal atresia and should be the single radiographic procedure of choice.