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Objectives: Studies of medical error demonstrate that errors and adverse events (AEs) are common in hospitals. There are little data of errors on pediatric surgical services. Methods: We retrospectively reviewed 50 randomly selected inpatient admissions to the otolaryngology service at a tertiary care children's hospital. We used a “zero-defect” paradigm, recording any error or adverse event—from minor errors such as illegible notes to more significant errors such as mismanagement resulting in a bleeding emergency. Results: A total of 553 errors/AEs were identified in 50 admissions. Most (449) were charting or record-keeping deficiencies. Minor AEs (n = 26) and moderate AEs (n = 8) were present in 38% of admissions; there were no major AEs or permanent morbidity. Medication-related errors occurred in 22% of admissions, but only two resulted in minor AEs. There was a positive correlation between minor errors and AEs; however, this was not statistically significant. Conclusions: Multiple errors occurred in every inpatient pediatric otolaryngology admission; however, only 26 minor and eight moderate AEs were identified. The rate of errors per 1,000 hospital days (6,356 per 1,000 days) is higher than previously reported in voluntary reporting studies, possibly due to our methodology of physician review with a “zero-defect” standard. Trends in the data suggest that the presence of small errors may be associated with the risk of adverse events. Although labor-intensive, physician chart review is a valuable tool for identifying areas for improvement. Although small errors were common, there were few harms and no major morbidity. Laryngoscope, 2009

Objectives: The objectives of this study were to describe otolaryngologists' emotional reactions to errors and adverse events, their efforts to take responsibility, and their attempts to implement improvements. Study Design and Methods: A retrospective, anonymous survey of 2,500 U.S. otolaryngologists who were members of the American Academy of Otolaryngology–Head and Neck Surgery about errors in their practice was conducted. Respondents were asked whether an error had occurred in their practice in the past 6 months and, if so, to describe the error, its consequences, and any corrective actions taken. Two aspects of these reports stood out, which were beyond the scope of the original study: the respondents' emotional responses and their corrective actions. Results: The response rate was 18.6%. Two hundred ten (45%) respondents reported a total of 212 analyzable error reports and 230 corrective actions. Corrective actions included disclosure to the patient (20 [9%]), ameliorating the consequences of the event to the patient (107 [50%]), personal practice changes (14 [7%]), improvements in the respondent's practice or department (60 [28%]), and hospitalwide or broader corrective actions (19 [9%]). Emotional reactions to errors and adverse events were reported by 22 (10%) otolaryngologists, including regret, embarrassment, guilt, anxiety, loss of temper, and irritation. Legal action was mentioned by five physicians (2%). Conclusions: Otolaryngologists took actions not only to treat their patients, but also to improve patient care in their practice, department, hospital, or community. Emotional reactions to errors and adverse events are common and need to be addressed in medical training and practice.

Vascular endothelial growth factor–A (VEGF-A) is known to play an important role in the angiogenic response essential for tumor growth in a variety of human and experimental tumors. This study was designed to investigate whether VEGF-A may play a role in the pathogenesis of recurrent respiratory papillomatosis (RRP). A retrospective study with institutional review board approval was performed at a tertiary care medical center on 12 patients with a history of laryngeal RRP. Their ages at the time of initial diagnosis ranged from 19 to 96 months (mean, 56 months). All patients had involvement of right and left true vocal cords. All patients required multiple endoscopic procedures (range, 4 to 66; mean, 12). Normal pediatric larynx samples from 5 autopsy patients were used as controls. Formalin-fixed, paraffin-embedded sections of laryngeal squamous papillomas from the 12 patients with a diagnosis of RRP and the 5 control patients were examined by in situ hybridization for the presence of messenger RNA (mRNA) for VEGF-A and vascular endothelial growth factor receptor 1 (VEGFR-1) and vascular endothelial growth factor receptor 2 (VEGFR-2). The biopsy specimens were from the true vocal cord (N = 10) or subglottis (N = 2) in the patients with RRP and consisted of large sections of larynx including the true vocal cord in the control patients (N = 5). Strong expression of VEGF-A mRNA was noted in the squamous epithelium of papillomas of all 12 patients. Strong expression of VEGFR-1 and VEGFR-2 was noted in the endothelial cells of the underlying vessels in all 12 patients. Neither strong labeling of VEGF-A mRNA nor labeling of its receptors was noted in the control patients. We conclude that the angiogenic growth factor VEGF-A is strongly expressed in the epithelium of squamous papillomas in RRP. Also, VEGFR-1 and VEGFR-2 mRNAs are strongly expressed by underlying vascular endothelial cells, suggesting an important role in the pathogenesis of RRP.

Objective: Simulation is a tool that has been used successfully in many high performance fields to permit training in rare and hazardous events. Our goal was to develop and evaluate a program to teach airway crisis management to otolaryngology trainees using medical simulation. Methods: A full-day curriculum in the management of airway emergencies was developed. The program consists of three airway emergency scenarios, developed in collaboration between attending otolaryngologists and faculty from the Center for Medical Simulation. Following each scenario, the participants are led in a structured, video-assisted debriefing by a trained debriefer. Didactic material on team leadership and crisis management is built into the debriefings. Pediatric otolaryngology fellows, residents, and medical students have participated in the four courses that have been held to date. Participants evaluated the program on a five-point Likert scale. Results: A total of 17 trainees participated in four pilot training courses. The survey data are as follows: overall program, 5.0 (SD, 0.00); course goals, 4.79 (SD, 0.43); realism, 4.36 (SD, 0.63); value of lecture, 4.71 (SD, 0.47); and quality of debriefings, 4.92 (SD, 0.28). Sample comments include: “This is a valuable tool for students and residents since true emergencies in ORL are often life-threatening and infrequent,” and “This is a great course—really all physicians should experience it.” Overall evaluation was extremely positive and both residents and fellows described the course as filling an important void in their education. Conclusion: Medical simulation can be an extremely effective method for teaching airway crisis management and teamwork skills to otolaryngology trainees at all levels.

Objectives were 1) to review the presentation, natural history, and management of subglottic hemangioma; 2) to assess the affect of five variables (age, gender, degree of subglottic narrowing, location and extent of subglottic hemangioma, and lack or presence of other hemangioma) and the outcome of six different treatment modalities (conservative monitoring, corticosteroid, laser surgery, tracheotomy, laryngotracheoplasty, and interferon) in the management of subglottic hemangioma; and 3) to present specific guidelines to help determine the best possible treatment modality at the time of initial presentation.Retrospective review in the setting of three tertiary care pediatric medical centers.Methods included 1) extensive review of the literature; 2) a systematic review with respect to age, gender, presentation, associated medical problems, location and degree of subglottic narrowing, initial treatment, need for subsequent treatments, outcome, complications, and prognosis; and 3) statistical analysis to determine the effect of five variables (age, gender, degree of subglottic narrowing, location and extent of subglottic hemangioma, and lack or presence of other hemangioma) and the outcome of six different treatment modalities (conservative monitoring, corticosteroid, laser surgery, tracheotomy, laryngotracheoplasty, and interferon).In all, 116 patients with a mean age of 4.7 months were treated. The most common location of subglottic hemangioma was the left side. The range of subglottic narrowing was 10% to 99% (mean percentage, 65%). Twenty-six patients (22%) were managed with a single treatment modality, which included conservative monitoring (n = 13), corticosteroid (n = 11), and tracheotomy (n = 2). Ninety patients (78%) required multimodality treatments. Overall, the treatments included conservative monitoring (n = 13), corticosteroid (n = 100), tracheotomy (n = 32), CO2 laser (n = 66), interferon (n = 5), and laryngotracheoplasty (n = 25). Complication rates included the following: conservative monitoring (none), corticosteroid (18%), tracheotomy (none), CO2 laser (12%), interferon (20%), and laryngotracheoplasty (20%). The following variables showed statistical significance in the outcome of different treatment modality: 1) degree of subglottic narrowing (P.001), 2) location of subglottic hemangioma (P.01), and 3) presence of hemangioma in other areas (P.005). Gender (P.05) and age at the time of presentation (P.06) did not show any statistical significance on the outcome of the treatments.Each patient should be assessed comprehensively, and treatment should be individualized based on symptoms, clinical findings, and experience of the surgeon. The authors presented treatment guidelines in an attempt to rationalize the management of subglottic hemangioma and to help determine the best possible treatment modality at the time of initial presentation.

Almost all modern medical care is delivered in the setting of many overlapping systems. Each system may consist of multiple providers and in most cases electronic and mechanical components. Even “simple” outpatient care is delivered by teams of providers, administrators, and devices. Critically ill inpatients are cared for in extraordinarily complex systems with hundreds of human and non-human elements. The science of complex systems has exploded in recent decades, and there is a large body of knowledge about how such systems function effectively or ineffectively. Many principles of systems science are simple to understand and apply, but few Otolaryngologists are well educated about them. A basic knowledge of systems science will greatly improve the Otolaryngologist’s ability to function in complex health care systems and to provide the best care for his or her patients. Laryngoscope, 114:1810–1814, 2004

Objective To further evaluate the role of chromosomal translocation (15;19) in the presentation of the carcinoma (CA) of the upper aerodigestive tract. Study design and setting A retrospective study at a tertiary care pediatric medical center. Results Seven patients with a mean age of 12 years presented with CA of nasopharynx (N = 2), sinonasal region (N = I), parotid gland (N = 2), or larynx (N = 2). Treatments included combinations of surgery (N = 5), chemotherapy (N = 5), and radiation therapy (N = 4). One patient with sinonasal CA and one patient with laryngeal CA had chromosomal translocation (15;19); these patients both died of their disease with a mean survival of 6 months. The 5 patients without translocation (15;19) responded well to treatment and are disease-free with a mean follow-up of 47 months. Conclusion The preliminary results appear to indicate poor prognosis associated with the presentation of chromosomal translocation (15;19) despite aggressive multi-modality treatment. Further investigation is needed to better understand the cause and relationship of the translocation (15;19) and aggressive behavior of these tumors.

Objective: To review the biology of nasal glioma and encephalocele and to present an algorithm for preoperative evaluation and surgical management. Design: Retrospective review and analysis. Setting: Tertiary care medical center: 1970 to 2002. Patient: Sixteen patients with glioma (n = 10) and encephalocele (n = 6). Outcome: Age at the time of presentation, sex, signs and symptoms, imaging findings, surgical approach, pathology, complications, rate of recurrence, and follow-up were recorded. Results: Ten patients presented with nasal glioma with a mean age of 9 months. All patients underwent surgical excision. No complication was encountered with a mean follow-up of 3.5 years. Six patients presented with encephaloceles with a mean age of 15.5 months. All patients underwent surgical excision. Complications included cerebrospinal fluid leak (n = 1) and epiphora (n = 1). Follow-up was 1 to 14 years (mean, 4 years). Conclusion: Nasal glioma and encephalocele are rare, benign, congenital lesions with a potential for intracranial extension. Evaluation should include a complete rhinologic and neurologic examination. Preoperative imaging with a thin-cut axial and coronal computed tomography scan andlor multiplanar magnetic resonance imaging is essential. Surgical intervention should be performed soon after diagnosis to alleviate the increased risk of meningitis. A frontal craniotomy approach is recommended if intracranial extension is identified based on preoperative evaluation, followed by an extracranial resection. If there is no evidence of intracranial extension, a conservative extracranial approach is recommended.

Lymphatic malformations (LMs) are uncommon congenital lesions that may occur throughout the body, although the head and neck region is the most common site. Most LMs are seen at birth. However, they may present in adolescence or adulthood, mainly as a result of trauma or infection. We report the case of a 7-year-old boy who presented with an LM of the cervicofacial region causing airway compression. We discuss the causes of delayed presentation of these congenital lesions. An overview of the causation, natural history, diagnosis, and treatment options is presented.

A retrospective chart review of children who had airway foreign body removed via direct laryngoscopy and bronchoscopy (DLB) from 1987-1997 was conducted in Children's Hospital, Boston. Patient characteristics noted included age, sex, and clinical presentation. Pre-operative radiographic findings, reason for delay in evaluation, DLB findings, length of procedure, reason for repeat DLB, and types of foreign body etc. were recorded. Serious complications from aspirated foreign bodies such as severe airway obstruction and death tend to occur in infants and younger children because of their small airway size. A history compatible with foreign body aspiration dictates diagnostic endoscopy with or without radiologic confirmation. Chest and airway radiographs supplemented by fluoroscopy can increase the ratio of correct and early diagnosis. Fluoroscopy should be universally accepted as an initial diagnostic technique in airway foreign body evaluation. Fluoroscopy is not a worthwhile investigation if a preceeding chest radiograph suggests the presence of a foreign body. Long-standing airway foreign bodies are associated with considerable morbidity, and early diagnosis remains the key to successful and uncomplicated management of foreign body aspiration. Education aimed at increasing diagnostic acumen of the physicians and heightening of public awareness are the most important steps needed to reduce the morbidity and mortality. Parents should be instructed to abstain from feeding nuts and seeds to young children and to keep small, potentially ingestible objects out of their reach.

The multi-cytokine inducer OK-432 is a pulverized preparation of the low-virulence SU strain of Streptococcus pyogenes of human origin. A reduction of the tumour mass in the OK-432-injected areas was observed in 11 out of 13 patients with metastatic and/or recurrent head and neck cancer. Complete response (CR), partial response (PR) and minor response (MR) were noted in six, three and two cases respectively. OK-432 local administration therapy could create a new strategy for cancer therapy.

Objective: To ascertain changes in laryngeal anatomy and function associated with poor voice outcome after laryngotracheal reconstruction (LTR) and to specifically describe dysphonia in pediatric patients treated for subglottic stenosis. Design: A survey was undertaken of patients having undergone LTR for subglottic stenosis. Twelve patients were recruited during the year 1990-1991. Nine patients were able to complete the endoscopic portion of the protocol that included fiberoptic and direct laryngoscopy. Speech samples were qualitatively evaluable in only six of nine patients (vocal quality, loudness, and intelligibility) and objectively in five of nine patients (fundamental frequency and pitch perturbation). Patients: All patients in this consecutive sample had undergone LTR for congenital or acquired subglottic stenosis. The average age was 6 years. Each patient had previously undergone an average of two prior open laryngeal procedures and five endoscopic procedures. Outcome Measures: Correlation between anatomic and functional changes in the post-LTR larynx and voice outcome. Results: Endoscopic evaluation of the post-LTR larynx showed a 78% incidence of altered anatomy and 44% incidence of altered function. Speech sample analysis showed the following: 100% decreased vocal quality, 50% decreased intelligibility, 100% decreased volume, and 80% low fundamental frequency and increased jitter. Conclusions: Children with high-grade subglottic stenosis and multiple prior surgeries are at high risk for poor voice outcome after LTR. Future prospective studies will allow substantiation of this observation and clarification of which changes in laryngeal structure and function arise from surgery per se. (Arch Otolaryngol Head Neck Surg. 1994;120:641-647)

The management of nasal deformities in children often presents the clinician with a challenging dilemma. In the younger child, emphasis should be placed on correction of the nasal airway whereas, in older children, external cosmetic deformity can be considered with safety. Appropriate surgical techniques will ensure an appropriate outcome. Careful planning, as well as sensitivity to growth and development of the nasal structures should be considered when such a procedure is to be undertaken. Older children and adolescents present no unique difficulties in septorhinoplasty and the usual principles commonly applied to adults may be followed. A safe and effective technique can be designed for each child and adolescent afflicted with both internal and external nasal deformities.

Hemangioma is a well-recognized cause of airway obstruction in the infant with stridor. Corticosteroid and laser therapy are effective in stabilizing the airway in most cases. There are, however, some extensive airway lesions that are not adequately managed by these modalities. This report describes the use of recombinant interferon alfa-2a in 15 patients with life-threatening airway hemangiomas. All patients had failed corticosteroid and/or laser therapy. Multiple upper airway sites were involved, including the base of the tongue, supraglottis, subglottis, trachea, and mediastinum. Eleven patients have completed therapy and are doing well. Four patients have resolving lesions on the drug regimen. Life-threatening airway lesions unresponsive to conventional treatment should be considered for a trial of interferon alfa-2a.

Dr John J. Conley was an integral part of the house of surgery in the latter part of the 20th century. Conley placed the ethical values of practice at the forefront of his teachings and transcribed many valuable lessons in his writings. In 1993, he wrote his version of the Hippocratic Oath outlining 12 important principles. Those principles are revisited here as a way to celebrate the joy of the calling that is medical practice. In addition, 7 new elements are added as a way to enhance that joy in the light of 21st-century medicine. The uplifting experience found in a career filled with ethical conduct is the legacy we should all strive to achieve.

Objective To identify and quantify errors and adverse events on an inpatient academic tertiary-care pediatric otolaryngology service, a trigger tool was developed and validated as part of a quality improvement initiative. Study Design Retrospective record review. Setting Children's Hospital Boston quality improvement initiative. Subjects and Methods Fifty inpatient admissions were reviewed. The gold standard for errors and adverse events identification was a detailed chart review by two board-certified otolaryngologists blinded to trigger tool findings. Results Trigger tool interrater reliability ranged from poor to high for admission triggers (kappa = 0.35, 95% confidence interval [95% CI] −0.07 to 0.76), discharge triggers (kappa = 0.63, 95% CI 0.27-0.99), medical records triggers (kappa = 0.61, 95% CI 0.11-1.00), and medication triggers (kappa = 0.90, 95% CI 0.71-1.00). Errors and adverse events were found in all admissions: three percent were potentially harmful, and 93 percent were documentation-related. Conclusion The trigger tool was successful in identifying clerical and administrative errors and adverse events but failed to identify complex errors and adverse events. A hybrid approach for chart review may be cost-effective in pediatric otolaryngology.

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.

The consultants agree that an open biopsy is generally necessary to establish the histology of a pediatric head and neck neoplasm. Although a frozen section may be useful to ascertain whether tumor tissue has been sampled, definitive therapy should be based only on the histopathologic interpretation of the permanent specimen. The consultants also agree that certain studies should be obtained prior to a biopsy. Dr. Grundfast recommends a chest x-ray, liver function tests, complete blood count, and an magnetic resonance scan. Dr. Healy would obtain additional CT cuts and an MRI scan. Dr. Richardson prefers coronal cuts on the CT scan and an MRI. The skull base is regarded as the area that might pose the greatest difficulty in obtaining a surgical margin. In addition, Dr. Healy states that eustachian tube involvement would compromise the resection. Although all consultants agree that the facial nerve should be sacrificed, they disagree as to how to reconstruct this defect. Dr. Grundfast would restore the mandibular profile with a prosthesis and reconstruct the soft tissue defect with a myocutaneous flap. Dr. Healy would use an iliac bone graft for the skull base defect and would replace soft tissue with a rectus free flap or a myocutaneous flap. Dr. Richardson favors a latissimus dorsi free flap. Because the survival rates for poorly differentiated fibrosarcomas of the head and neck are so low, the experts recommend adjunctive chemotherapy. Drs. Grundfast and Richardson would also advise radiotherapy. Dr. Healy feels that the morbidity of radiotherapy is too high and would use it only in cases of positive margins, parameningeal involvement, or perineural invasion.

Objective:To provide an up to date review of the literature on aneurysmal bone cysts, including their diagnosis, pathology, pathophysiology, radiology and management.Method:Retrospective review of six cases over a 15-year period.Results:Six patients (age range, eight months to 17 years; mean, 9.6 years) presented with an aneurysmal bone cyst in the mandible (n = 3), maxilla (n = 2) or occipital soft tissue (n = 1). Each patient underwent primary excision, with one subsequent recurrence.Conclusion:Aneurysmal bone cysts are benign but locally destructive entities which may occasionally present to otolaryngologists, since they can involve the head and neck region, in particular the mandible.

Objectives/Hypothesis: To study the presentation, management, and long-time outcome of children presenting with lingual thymid. Study design: Institutional review board approved, retrospective study (1993-2004) Methods: The study was conducted at a tertiary care pediatric medical center. The main outcomes measured were initial presentation, radiographic finding, endocrine evaluation, surgical outcome, pathologic features, complications, need for hormonal replacement. Resuts: Four patients presented to the Department of Otolaryngology and Communications Enhance. ment. Children's Hospital Boston with liognal thyroid between 1993 and 2004. All patients were female, with an age range of 2 to 12 years (x=6). All patients presented with a mass (1.4-3.5 cm) and most with respiratory or feeding difficulty. Magnetic resonance imaging was obtained in three patients and revealed a mass unsistant with lingual thydoir. Thyroid seen confirmed the lingual thyroid as the only fonctioning thyroid in all four patients. None of the patients responded to hormonal replacemement, and all underwent surgical excision of the mass, Surgical approach included midline glossotomy (n = 2) and CO 2 laser excision (n = 3). Pathologie evaluation confirmed lingual thyroid in all four patients. No evidence of machignancy was sen in any patient. All four patients require lifelong hormonal replacement. Conclusions: Lingual thyroid is a race condition. with an incidence of 1.100.000. This infrequent congenital anormally is often asymptomatic until a pathologic stress such as systemic disease or physiologie stress such as puberty causes enlargement of the ecloptic tissue, leading to dysphagia, dysphonia, and dyspnea. The work-up should include routine blood wr*ork including thyroid function tests thyrotropin, thyroxine, and thyroid hormone binding ratio; iodine thyroid scintigraphy; and computerized tomography of magnetic resonance imaging. The majority of patients require surgical excision of the symptomatic mass and, in case of absence of arthotopic thyroid atic mass and, in case of absence of arthotopic thyroid tissue, long-term thyroid hormone replacement.

Objectives: Vascular endothelial growth factor A (VEGF-A) is important in the angiogenic response for wound healing. This study investigated whether VEGF-A may play a role in the pathogenesis of acquired airway stenosis. Methods: Eight lesions from 5 pediatric patients with subglottic stenosis after airway reconstruction (N = 4) or prolonged intubation (N = 1) and normal laryngeal tissue from 5 autopsy patients were included. Formalin-fixed sections of subglottic tissue from each patient were examined by in situ hybridization for the presence of messenger RNA (mRNA) for VEGF-A, vascular endothelial growth factor receptor 1 (VEGFR-1), and vascular endothelial growth factor receptor 2 (VEGFR-2). Results: Strong expression of VEGF-A mRNA was noted in hyperplastic squamous epithelium overlying granulation tissue. Strong expression of VEGFR-1 and VEGFR-2 was noted in the endothelial cells within granulation tissue. No strong labeling of VEGF-A mRNA or its receptors was noted in 2 specimens with mature scar tissue or in the control specimens. Conclusions: The angiogenic growth factor VEGF-A is strongly expressed in hyperplastic epithelium overlying granulation tissue in airway stenosis. Also, VEGFR-1 and VEGFR-2 mRNAs are strongly expressed in the endothelial cells of granulation tissue. This finding suggests an important role of VEGF-A in the pathogenesis of airway scar formation and stenosis.

Summary Objective To evaluate the presentation, imaging characteristics and treatment outcome of pleomorphic adenoma of the parotid in the pediatric population. Design Retrospective study with institutional review board approval. Setting Tertiary care pediatric medical center. Methods An extensive review of medical records with regard to presentation, imaging, histopathology, complication, recurrence and prognosis on patients 18 years or younger presenting from 1983 to 2005. Results Eleven patients (six females, five males) were identified. The most common presentation was an asymptomatic mass. Preoperative imaging was done on nine patients: MRI ( N = 6), CT ( N = 3), ultrasound ( N = 2), and sialogram ( N = 1). Initial treatments included: superficial parotidectomy ( N = 5), total parotidectomy ( N = 3), excisional biopsy followed by superficial parotidectomy ( N = 2), and excisional biopsy ( N = 1). There were two recurrences (18%); one presenting 7 months following excisional biopsy who underwent superficial parotidectomy and one occurred 3 years following total parotidectomy requiring revision parotidectomy and radiation. Other complications included: transient facial nerve paresis ( N = 5; 45%) and permanent weakness ( N = 1; 9%). The patients were followed an average of 18 months. Conclusions Pleomorphic adenoma is one of the most common tumors of the parotid in children. The most common presentation is an asymptomatic mass. A preoperative evaluation with MRI or CT scan can be helpful in determining the extent of the lesion and surgical planning. Complete excision via superficial or total parotidectomy with preservation of facial nerve is the treatment of choice. Long-term follow up is recommended, though was difficult in a tertiary care center.

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.

Objective To review the presentation and associated congenital abnormalities of laryngeal cleft and present guidelines for its evaluation and management. Design A 10-year retrospective study (1994-2004) with institutional review board approval. Setting Two pediatric tertiary care medical centers. Patients Twenty-two pediatric patients (mean age, 21 months) with laryngeal cleft. Intervention Surgical repair of laryngeal cleft. Main Outcome Measures Sex, age, symptoms, other associated abnormalities, method of evaluation, type of laryngeal cleft, method of surgical repair, treatment outcome, complications, and long-term follow-up. Results All 22 patients underwent surgical repair for laryngeal cleft. Airway endoscopy confirmed the following types of laryngeal clefts: type 1 (n = 3), type 2 (n = 10), and type 3 (n = 9). Surgical repair techniques included an open approach with or without interposition graft (n = 16) and an endoscopic approach (n = 6). Conclusions Early diagnosis and proper repair of laryngeal cleft are essential to prevent pulmonary damage and associated morbidity. Each patient should be assessed properly, and the surgical approach should be individualized based on the symptoms, other associated findings on airway endoscopy, and type of cleft.

(2004). Cancer of the Larynx in Children and Adolescents: A Neoplastic Lesion with a Different Etiology. Acta Oto-Laryngologica: Vol. 124, No. 9, pp. 992-994.

To develop a preliminary classification system for errors in otolaryngology.A retrospective, anonymous survey was distributed to 2,500 members of the American Academy of Otolaryngology-Head and Neck Surgery (AAO-HNS). Respondents were asked whether an error had occurred in their practice in the last 6 months, and if so, to describe the error, its consequences, and any corrective action taken.There were 466 (18.6%) responses. Two hundred ten (45% of respondents) otolaryngologists reported 216 errors. A classification system for errors in otolaryngology was developed. Errors were classified as related to history and physical (1.4%), differential or final diagnosis (1.4%), testing (10.4%), surgical planning (9.9%), wrong-site surgery (6.1%), anesthesia-related (3.3%), wrong drug/dilution on the surgical field (3.8%), technical (19.3%), retained foreign body (0.9%), equipment-related (9.4%), postoperative care (8.5%), medical management (13.7%), nursing/ancillary (0.5%), administrative (6.6%), communication (3.8%), and miscellaneous (0.9%). There were 78 cases of major morbidity and 9 deaths. If these data are representative, there may be more than 2,600 episodes of major morbidity and more than 165 deaths related to medical error in otolaryngology patients annually.Human error in otolaryngology occurs in all practice components, including diagnostic, treatment, surgical, communication, and administrative. Types of errors reported by otolaryngologists differ from those reported by other specialists. Error classification systems may need to reflect each specialty's realm of practice. Errors in otolaryngology cause appreciable morbidity and mortality. Quantitative study of errors and the development of targeted prevention and amelioration strategies should be a high priority.

Embryologic remnants of thyroid tissue often line the thyroglossal duct tract and may commonly become cystic. Calcification in such a cyst is thought to be a specific marker for malignancy, which may develop in 1% of thyroglossal duct cysts. We describe a 3-year-old boy with a midline neck mass that showed radiologic calcification. Pathologic evaluation revealed psammomatous calcification in association with a benign thyroglossal duct cyst. These findings, not previously reported (to our knowledge), expand the radiologic differential diagnosis of calcified neck masses and broaden the spectrum of pathologic findings seen in association with benign thyroglossal duct cysts.

A new procedure for aspiration which closes the larynx at the level of the false cords is reported. This method is not harmful to the vocal folds and maintains arytenoid movement, thus preserving phonatory function. With the advance of the operative technique for aspiration and swallowing rehabilitation, patients who have recovered from aspiration can be helped. Our procedure can be recommended for such cases.

Objectives To evaluate the clinical, audiologic, and temporal bone computed tomograpic findings in patients with hemifacial microsomia and to use the OMENS (each letter of the acronym indicates 1 of the following 5 dysmorphic manifestations: O, orbital asymmetry; M, mandibular hypoplasia; E, auricular deformity; N, nerve involvement; and S, soft tissue deficiency) grading system to assess possible correlations between the severity of dysmorphic features with the type of abnormalities in the temporal bone and with degree of hearing deficit. Design Retrospective study. Setting Tertiary care children's hospital. Patient Forty patients with hemifacial microsomia. Result Mandibular hypoplasia and auricular abnormalities were the most common clinical manifestations, present in 39 patients (97%) and 38 patients (95%), respectively. Conductive hearing loss was noted in 35 patients (86%) and sensorineural hearing loss in 4 patients (10%). Facial nerve weakness was present in 20 patients (50%). Twenty patients had unilateral aural atresia, 12 patients had unilateral aural stenosis, and 7 patients had bilateral anomalies. Moderate hypoplasia or atresia of the middle ear was noted in 36 patients (90%) and ossicles were malformed in 30 patients (75%). Hypoplasia of the oval window was the most common inner ear abnormality. Conclusions Severity of craniofacial features (total OMENS score) significantly correlated with the degree of temporal bone abnormality, but no correlation was noted with the degree or type of hearing loss. We recommend the following: (1) use of the OMENS classification system for documentation and analysis of dysmorphic finding in hemifacial microsomia; (2) complete audiologic evaluation in all patients with hemifacial microsomia regardless of the type of craniofacial abnormalities; and (3) temporal bone computed tomography for further evaluation of hearing deficit.

The management of laryngeal and tracheal stenosis continues to challenge us, despite advances in surgical techniques and better understanding of the wound healing process. Injury to the airway mucosa is the inciting event, regardless of the cause of the stenosis. Mitomycin-C is an antineoplastic antibiotic that acts as an alkylating agent by inhibiting DNA and protein synthesis. It can inhibit cell division, protein synthesis, and fibroblast proliferation. Topical application of mitomycin-C (0.4 mg/mL) was used as an adjuvant treatment in the endoscopic laser management of laryngeal and tracheal stenosis in 15 patients. Fourteen patients (93%) have shown improvement of their airway and resolution of their preoperative symptoms. After a mean follow-up of 18 months, no complication was noted with regard to the application of mitomycin-C. This study gives promising findings on the efficacy and safety of mitomycin-C as an adjuvant treatment in the management of selected cases of laryngeal and tracheal stenosis.

• Lesions of the base of the tongue are rare in the pediatric population. However, when present these lesions may lead to airway obstruction and feeding difficulties. Two patients with lesions of the base of the tongue are described to demonstrate the diagnosis and management of this difficult clinical problem. The use of a transoral median glossotomy for total excision of these lesions is advised in this article. This approach affords excellent surgical exposure, early return to a normal diet, and lack of facial scars. ( Arch Otolaryngol Head Neck Surg. 1992;118:1350-1352)

Purpose of review The study of errors in medicine has proliferated in the past few years. This spark was ignited by the Institute of Medicine's report in 2000, titled 'To Err is Human'. Studies of errors have been performed in both medical and surgical specialties. Surgical skill and technology has proliferated over the last century to such an extent that outcomes will probably not improve from focusing finite resources on attempts to enhance surgical skill or developing new instrumentation. Advancing medicine in the 21 Century will probably depend on our ability to address and ameliorate errors and adverse events in healthcare. Directing attention to this previously little studied realm of medicine has the ability to dramatically reduce morbidity and mortality. Small changes have the ability to result in outcomes that are far beyond what is predicted by incremental measures. Recent findings There have been studies identifying and classifying errors in otolaryngology in out-patient and in-hospital settings, addressing allergy sera misadministration, providing quality assurance for pathology reports in head and neck malignancies, implementing computerized patient records, and detailing problems that occur when patients have a test ordered and the results are not followed up. Summary The study of errors in medicine is going to dramatically increase in the next decade. The practicing otolaryngologist must be vigilant for errors, use surgical time-outs, should have a system in place to track tests ordered and results received, and most importantly have the capability to identify and ameliorate errors as they occur on an individual and institutional level.

Objectives: To characterize otologic management of two patient groups, those with the CHARGE association and those not strictly labeled as CHARGE but with several features of the disorder (CHARGE-like), in order to determine: (1) the clinical validity and utility of managing CHARGE-like children in a similar manner to patients with the strictly defined CHARGE association, (2) the progression and prognosis of hearing loss and (3) the identification of factors that may predict the degree of hearing loss. Design: Case series. Setting: Tertiary care urban children's hospital. Patients: 37 children, 22 in the CHARGE group and 15 in the CHARGE-like group. Interventions: Otorhinolaryngologic and audiologic management. Main outcome measures : Otorhinolaryngologic and audiologic evaluation. Results: All patients required otologic and/or audiologic care. Bilateral hearing loss was found in 32 patients (86%) and unilateral hearing loss in five patients (14%) when hearing was assessed in the absence of otitis media. Among the 32 patients with bilateral hearing loss, 31 (97%) were able to be fit with useful hearing aids. External ear anomalies were present in 25/37 (68%) patients, and middle ear and ossicular anomalies were identified in four cases (4/37, 11%). 36/37(97%) patients required surgical management of otitis media. Three patients (3/37, 8%) exhibited radiographic evidence of inner ear deformity. Facial nerve dysfunction was noted in the records of 14/37 (38%) patients. No statistically significant difference was found when CHARGE and CHARGE-like patients were compared for degree of hearing loss ( P =0.5964), type of hearing loss ( P =0.2657), worsening of hearing level ( P =0.7908), or anomalies of the external ear ( P =0.6921), ossicles ( P =0.7908), inner ear ( P =0.7908) or facial nerve ( P =0.6409). Patients with external ear anomalies did not exhibit statistically different degrees ( P =0.3125) or types ( P =0.1515) of hearing loss from patients without auricular anomalies. The presence of facial nerve anomaly correlated significantly ( P =0.0021) with profound hearing loss. Conclusions: Children who are CHARGE-like may be may be considered equivalent in terms of otologic and audiologic management to children strictly defined as CHARGE patients. These children all require otologic care due to the high prevalence of middle ear disease and the underlying permanent hearing loss that is both stable and aidable. The degree of hearing loss cannot be predicted by external ear morphology, but may be predicted by facial nerve palsy.

Objective: To examine the use of hyoid as a readily available autologous grafting material for the anterior cricoid split (ACS) procedure. Design: Prospective analysis of 20 patients undergoing ACS with hyoid interposition grafting for subglottic stenosis over a 3-year period. The patients received at least 1 year of follow-up after surgery. Setting: Tertiary care children's hospital. Patients: Twenty infants (age range, 2-9 months) with endoscopically confirmed acquired and congenital subglottic stenosis. Presenting symptoms included stridor, failure to extubate, and recurrent atypical croup. All 20 children underwent ACS with hyoid interposition grafting. Results: All 20 patients exhibited improvement in their symptoms of airway obstruction. All 12 patients in whom extubation had previously failed subsequently underwent successful extubation. The 8 patients with symptoms of stridor and atypical croup showed marked improvement in their symptoms. Serial bronchoscopy revealed mucosal healing and incorporation of the hyoid grafts. Conclusion: Hyoid provides a readily available and reliable grafting material for interposition grafting in the ACS procedure for neonates and infants. Arch Otolaryngol Head Neck Surg. 1997;123:1277-1280

Malignant laryngeal neoplasms in children are uncommon and present diagnostic and therapeutic challenges. A 15-year retrospective review of laryngeal tumors at our institution identified four patients with malignant neoplasms. These children, 7 months to 16 years of age, were treated for squamous cell carcinoma, choriocarcinoma, rhabdomyosarcoma, and primitive neuroectodermal tumor. This report details these cases, emphasizing diagnostic approaches, treatment decisions (medical oncologic management, surgical resection), and rehabilitation measures for postlaryngectomy communication. A multi-institutional pediatric head and neck tumor registry would be a useful reference to assist with individual treatment decisions.

Innominate artery compression of the trachea in infants can cause severe biphasic stridor, cyanosis, and respiratory arrest. These episodes are sometimes referred to as “dying spells.” In the past, aortopexy has been used for the treatment of this problem, but since many have questioned its success, its use has not been popularized or generally accepted. Over the past 4 years, 12 children have been successfully treated for innominate artery compression of the trachea with aortopexy. These children all initially presented with significant respiratory and/or feeding difficulties. Preoperative and postoperative videos were obtained to document the degree of tracheal compression and/or lumen size before and after surgery. Since treatment, all patients have been without further feeding problems or cyanosis. When performed in selected patients, aortopexy is successful in relieving tracheal obstruction from innominate artery compression. This article discusses proper patient selection, operative technique, and follow-up care. The management of compression in patients with less severe symptoms will also be addressed.

Patients with subglottic hemangioma present with biphasic stridor during infancy. The natural history of this lesion is characterized by progressive airway obstruction during the proliferative phase of the lesion, followed by resolution of symptoms during the subsequent involutive phase. Although this is a benign neoplasm, it can be associated with a fatal outcome. Treatment options have been directed at maintaining airway patency during the proliferative phase. The senior author (G.B.H.) originally described the role of the carbon dioxide laser in management of these patients in 1980. Thirty-one patients were treated for subglottic hemangioma at The Children's Hospital, Boston, between 1980 and 1990. The 10-year experience of management of subglottic hemangioma with endoscopic vaporization using the carbon dioxide laser is reviewed to assess the efficacy of this technique.

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.

Cordyceps ophioglossoides is one of the Japanese old ’Kanpoh‘ drugs used for metrorrhagia as a decoction. We found that CO-N, a galactosaminoglycan from Cordyceps ophioglossoides, reacted with sera from patients with some collagen diseases. By using CO-N, we made a new serological activity index (CO—N reac tion). In the present study, we investigated CO-N reaction on patients with Wegener's granulomatosis (WG). The aggregation titres of CO-N (CO-N numbers) displayed a possible correlation with their clinical activity. CO-N reaction might also serve an important role in supporting the diagnosis of active WG and in helping to assess the degree of disease activity. The purpose of this report is to introduce this new serological activity index for Wegener's granulomatosis.

Most patients have no response to injectable collagen or silicone, but some cases may have positive or ‘undersea’(= clinically negative but immunologically positive) response to collagen. From the results of the Macrophage migration inhibition test, the relative immunogenicity was augmented most when we used implants with the following combination. The firstimmunization was collagen and the second one was collagen with silicone.The augmented antigenicity might be enough to cause an allergic reaction to the patients who had no response to each implant alone. The purpose of this article is to warn of the potential hazard of injection.

Fifteen Japanese patients with Wegener's granulomatosis (WG) were evaluated according to their lymphocyte subset abnormalities. Two colour immunofluorescent flow cytometry was used to distinguish the lymphocyte subset alterations. WG group showed a decrease in the percentage of CD4+ cells and the increase of CD8+cells. Within the NK cell family, the functionally unidentified CD8+57+cells were markedly elevated. The disproportion of thelymphocyte subsets (CD4+↓ CD8+57+↑) were similar to those of Acquired Immunodeficiency Syndrome (AIDS) and AIDS relating complexes (ARC). To assesssilent infection of Human immunodeficiency virus (HIV), the polymerase chain reaction (PCR) wasdone for all patients to selectively amplify specific HIV proviral DNA sequences in peripheral blood mononuclear cells, HIV-1 proviral DNA was not found in any patients but these changes of WG might suggest a possible adaptive response to unknown viral infection.