Grading system for the selection of patients with congenital aural atresia.

It is generally recognized that surgery for congenital aural atresia is difficult. In an effort to select those patients who have the greatest chance of success, we have developed a grading scheme based on the preoperative temporal bone CT scan and the appearance of the external ear. Patients are graded on a possible best score of 10. The stapes is assigned the highest rating (2 points), while all other entrees on the scale are 1 point. The grade assigned preoperatively has been shown to correlate well with the patient's chance of success, herein defined as a postoperative speech reception threshold of 15 to 25 dB. A patient with a preoperative grade of 8/10 would, therefore, have a 80 percent chance of achieving this threshold. Patients with scores of 5/10, or less, are not considered surgical candidates, because the risk of the operation would outweigh the potential benefits. We have found that the grading system allows us to avoid impossible surgical cases while allowing for a reasonable prediction of the hearing outcome.

The risk of cholesteatoma in congenital aural stenosis.

Congenital aural stenosis, as compared to congenital aural atresia, carries a much greater risk of cholesteatoma. In a review of over 600 patients with major congenital ear malformations, 50 patients (54 ears) were found to have congenital aural stenosis. Ninety-one percent of the ears in patients 12 years and older with a stenosis of 2 mm or less presented with cholesteatoma. Based on this review, the following opinions are given: 1. A bony ear canal opening of 2 mm or less puts the patient at risk of cholesteatoma formation; 2. the cholesteatoma is slow-growing; and 3. undiagnosed cholesteatoma will cause extensive damage to the ear by early adulthood. We recommend surgery for patients with stenosis of the external ear canal measuring 2 mm or less. The appropriate time for surgery is late childhood or early adolescence, before irreversible damage has occurred.

Laryngeal findings in short rib polydactyly syndrome: case report and embryological correlations.

Short Rib Polydactyly Syndrome (SRPS) is a group of rare congenital disorders characterized by polydactyly, short limbs, and short ribs. Infants with type I SRPS (Majewski syndrome) may exhibit dysgenesis of the larynx, which is manifested by epiglottic hypoplasia. Photographic documentation of laryngeal findings obtained at autopsy in an affected infant is provided. To our knowledge, such photographic documentation has not been previously published. Normal laryngeal development is reviewed, based on the Carnegie system of staging. The developmental errors leading to SRPS are thought to occur at 33 to 48 days of fetal life (Carnegie stages 15 through 19).

Congenital aural atresia.

Close cooperation between the plastic surgeon and the otologist, with each understanding and respecting the importance of the other's role, will provide the atresia/microtia patient with the best possible chance of a successful outcome. The purpose of the atresia repair is to improve hearing. Our goal is to attain, through surgery, hearing thresholds that will enable the patient to discard his hearing aid.

Preventing postoperative complications in the adult cystic fibrosis patient.

Herein we report on a 27-year-old cystic fibrosis (CF) patient who developed bronchospasm, secretory plugging of the trachea, and pneumothorax following general endotracheal anesthesia for intranasal polypectomy and bilateral Caldwell-Luc procedures at an adult facility. Intranasal polypectomy and paranasal sinus procedures are the most common surgical procedures performed on cystic fibrosis patients, making the otolaryngologist a frequent member of the cystic fibrosis team [8]. As survival improves, the pediatric otolaryngologist will find himself following CF patients into their 20's and 30's. Adult care facilities may not be as familiar with these patients as the surgeon would like. The otolaryngologist's familiarity with the unique perioperative requirements of the CF patient can prove invaluable in such a setting. Preoperative assessment should identify any acute pulmonary changes, assess nutritional status, assure good control of blood glucose levels, and rule out clotting abnormalities. Good perioperative hydration and meticulous attention to pulmonary toilet are of foremost importance in the surgical care of the cystic fibrosis patient. Chest physiotherapy and suctioning of the tracheobronchial tree should precede arousal from anesthesia and extubation. By insisting on appropriate anesthetic and perioperative care, the knowledgeable otolaryngologist may circumvent potential postoperative complications in the cystic fibrosis patient.

Pediatric maxillofacial trauma. Age-related variations in injury.

Seventy-two children were treated for maxillofacial injuries by the otolaryngology service between January 1984 and June 1988. Patients were divided into three age groups on the basis of the development of paranasal sinuses and dentition. Differences in fracture characteristics, associated injuries, and treatment modalities were correlated to the maturational changes in the pediatric facial skeleton. The fracture site tended to shift from the upper to the lower aspect of the face with increasing age of the patient. Associated injuries were frequent, especially cranial injuries, and temporal bone fractures were notably more common in the youngest age group. Conservative treatment was found to be successful, particularly in the youngest age group, because of the unique remodeling potential of the pediatric facial skeleton.

Treacher Collins syndrome: an otologic challenge.

Patients with Treacher Collins syndrome have severe middle ear malformations that render operation difficult. We have evaluated 43 patients with Treacher Collins syndrome, on whom only 11 were operated. Computed tomography, the single most important study done preoperatively, routinely showed an underdeveloped temporal bone with islands of bone marrow and absent mastoid pneumatization. The middle ear space was often underdeveloped. Ossicular dysjunction was often noted in which the fused malleus/incus remnant was found 3 to 4 mm distant to the stapes. A common finding was severe dysplasia of the stapes-facial nerve complex that often made the middle ear malformation uncorrectable. Hearing results were much less predictable than in patients with isolated atresia/stenosis of the ear.

Tracheotomy in children with recurrent respiratory papillomatosis.

Fifty-eight patients with laryngeal papillomatosis were managed at Children's Hospital Medical Center, Cincinnati, OH, between January 1978 and December 1987. Twelve of these patients (21%) had tracheotomies. A retrospective review of these 12 cases was undertaken to determine the incidence, pattern, timing, and clinical risk factors for tracheal spread after tracheotomy. Six of 12 patients (50%) developed tracheal papillomas after tracheotomy. Peristomal mucosa was consistently the site of initial involvement followed by progressive distal spread along the length of the tracheotomy tube. Stomal involvement followed tracheotomy by an average of 14 weeks, occurring as early as 7 weeks postoperatively. Mid-tracheal spread followed stomal involvement by an average of 10 weeks. Risk factors for tracheal spread included the presence of subglottic disease at the time of tracheotomy and prolonged cannulation. Whenever possible, tracheotomy should be avoided in patients with recurrent respiratory papillomatosis. When unavoidable, every effort should be made to keep the duration of cannulation as short as possible.

Congenital absence of the nose: a case report.

Congenital absence of the nose (arhinia, congenital nasal atresia) is a rare anomaly which is infrequently described in the literature. Herein we present a case of congenital absence of the nose recently evaluated and treated at Children's Hospital Medical Center (CHMC), Cincinnati, OH. CT and MRI studies were obtained. To our knowledge, no such radiographic evaluations have been described in patients with congenital absence of the nose. In addition to complete absence of the anterior soft tissues of the nose, thin anterior and thick posterior atretic plates were present. MRI was useful in defining the nature of the soft tissue mass which filled the single hypoplastic nasal cavity. The lip, alveolus, and palate were remarkably well developed. A thorough investigation revealed no other congenital defects. At 15 days of life, because of the airway support required and the associated feeding difficulties, a nasal airway was created using a combination of sublabial, transpalatal and percutaneous approaches. Frequent home dilation of the surgically created opening has been successful in maintaining nasal patency thus allowing the patient to go without continuous stenting.

Sudden hearing loss: an update.

Etiologic theories of idiopathic SHL include viral infection, autoimmune disease, vascular insult, and labyrinthine membrane rupture. A review of recent literature on sudden hearing loss indicates that scientific support for viral and autoimmune etiologic mechanisms is increasing. Recently studied treatment protocols include carbogen inhalation and steroid therapy. In patients with moderate hearing losses, oral steroids may be beneficial.

Cricothyroidotomy versus tracheotomy: an otolaryngologist's perspective.

A series of 655 elective cricothyroidotomies presented by Brantigan and Grow in 1975 has fueled a recent explosion in the number of cricothyroidotomies being performed for elective tracheal access. This article reviews Jackson's classic 1921 paper on cricothyroidotomy, Brantigan and Grow's series, seven recent clinical series of elective cricothyroidotomies, and our experience. Voice change is the most frequent complication of cricothyroidotomy occurring in up to 50% of cases. Chronic subglottic stenosis occurs in approximately 2% of cases. Contraindications to cricothyroidotomy include prolonged intubation, airway obstruction following extubation, and laryngeal pathology of any kind.

A predictive model for wound sepsis in oncologic surgery of the head and neck.

A prospective analysis of patients undergoing surgical resection of squamous cell carcinoma of the upper aerodigestive tract was performed in order to identify the patients at risk of postoperative wound infection and to develop a model predictive of wound infection. Fifty-nine patients who underwent extirpative clean-contaminated procedures--all of whom received cefazolin as the sole chemoprophylactic agent, were studied over a 1-year period. Twenty-three variables were recorded for each patient in the study. The overall rate of wound infection was 25.4%. Univariate analysis indicated that three variables were significantly related to the likelihood of postoperative wound infection. These included tumor stage (P = 0.0180), nodal stage (P = 0.0062), and duration of surgery (P = 0.0151). The Biomedical Computer Program (BMDP), a logistic regression program specifically designed for a binary dependent variable (infection vs. no infection) based on independent variables that may be continuous or categorical, was used in development of a model predictive of wound infection. T-stage, N-stage, and the presence of concomitant disease made up the combination of factors found to be most predictive of infection in our study population. Considering "success" to be the development of infection if the probability was 75% or higher, and the absence of infection if the probability was less than 25%, the multiple regression analysis model demonstrated a predictive success rate of 74.6%. Our results indicate that the risk of infection in patients undergoing clean-contaminated oncologic surgery of the head and neck is greatest for patients who have advanced disease that requires prolonged surgery in the presence of concomitant diseases.(ABSTRACT TRUNCATED AT 250 WORDS)

A placebo-controlled, dose-ranging study comparing 0.5 mg, 1 mg and 2 mg flunitrazepam in out-patients.

A placebo-controlled, dose-ranging study was carried out in 18 patients, aged under 65 years, with sleep disturbance to compare the hypnotic effects of 0.5 mg, 1 mg and 2 mg flunitrazepam and placebo. Patients received each treatment in random order for 7 days. Assessments by both doctor and patients were made on entry and at the end of each treatment period. The results showed that the 1 mg flunitrazepam dose was optimal with respect to speed of action, duration of action, quality of sleep and lack of residual clinical sequelae.