Role models in surgery.

Our present training models date back almost 100 years. It is very apparent that trying to reshape an ageing system to meet the demands of today's patients and their physicians is just not going to be effective or efficient. In the past educators cared little about the working conditions for trainees such as the living and learning environment, social support and compensation models. You were just fortunate to be "chosen". Surgical educators in the 21st Century must reexamine their roles and consider what message they are sending to future generations on these and other critical issues that impact on safe patient care.

Role of vascular endothelial growth factor A in children with acquired airway stenosis.

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.

Pleomorphic adenoma of the parotid gland in children.

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.

Otolaryngologists' responses to errors and adverse events.

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.

The presentation and management of laryngeal cleft: a 10-year experience.

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.

Mucoepidermoid carcinoma of the parotid gland in children: A 10-year experience.

OBJECTIVE: To determine the presentation, pathologic features, treatment outcome, and prognosis of mucoepidermoid carcinoma of the parotid gland in children. DESIGN: Retrospective clinical and histopathologic study with institutional review board approval. SETTING: Tertiary pediatric medical center. PATIENTS: Seven children (4 girls and 3 boys) presented with mucoepidermoid carcinoma of the parotid gland between 1994 and 2004. MAIN OUTCOME MEASURES: Clinical presentation, pathologic features, treatment outcome, complications, local recurrence, distant metastasis, and overall survival. RESULTS: All patients presented with an asymptomatic parotid mass. Initial treatment in 7 patients included total parotidectomy (n = 3), superficial parotidectomy (n = 3), transoral enucleation (n = 1), and supraomohyoid neck dissection (n = 1). Four patients required additional surgical procedures because of a close and/or positive margin, including revision parotidectomy (n = 2), total parotidectomy (n = 1), superficial parotidectomy (n = 1), and supraomohyoid neck dissection (n = 1). One patient required postoperative radiation therapy. No evidence of local recurrence or distant metastasis was noted with a mean follow-up of 3.4 years. CONCLUSIONS: Mucoepidermoid carcinoma of the parotid gland is very rare in children. Clinical stage and histologic grade are the main prognostic factors. Complete excision (superficial or total parotidectomy) with preservation of facial nerve is the treatment of choice. Neck dissection should be considered when there is clinical evidence of regional metastasis, high TNM stage, high histologic grade, and involvement of regional nodes. Because of the possibility of long-term adverse effects in pediatric patients, radiotherapy should be used only in selected cases. Long-term follow-up is essential to rule out late recurrence.

Role of vascular endothelial growth factor-A in recurrent respiratory papillomatosis.

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 wasnoted 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.

Quality and safety in a complex world: why systems science matters to otolaryngologists.

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.

Classification and consequences of errors in otolaryngology.

OBJECTIVE: To develop a preliminary classification system for errors in otolaryngology. METHODS: 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. RESULTS: 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. CONCLUSIONS: 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.

The biology and management of subglottic hemangioma: past, present, future.

OBJECTIVES/HYPOTHESIS: 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. STUDY DESIGN: Retrospective review in the setting of three tertiary care pediatric medical centers. METHODS: 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). RESULTS: 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. CONCLUSION: 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.

Nasal glioma and encephalocele: diagnosis and management.

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 and/or 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.

Psammomatous calcification in association with a benign thyroglossal duct cyst.

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.

The biology and management of laryngeal neurofibroma.

OBJECTIVES: To review the presentation of laryngeal neurofibroma, including its association with neurofibromatosis types 1 and 2, and present guidelines for its management. DESIGN: Retrospective study. PATIENTS: Five pediatric patients with laryngeal neurofibroma, 4 girls (80%) and 1 boy (20%), were treated at a tertiary pediatric medical center from 1973 through 2003. Recorded data included age at initial presentation, sex, symptoms, significant medical and family history, preoperative evaluation, location of the tumor, surgical procedure, complications, outcome, and recurrence. RESULTS: The 5 patients presented with stridor and cafe-au-lait spots at or shortly after birth. All patients were diagnosed as having neurofibromatosis type 1 by the established criteria. Studies evaluating the disease processes included plain radiography, computerized tomography, magnetic resonance imaging, barium swallow, and laryngoscopy and bronchoscopy under anesthesia. Pathologic examination of biopsy specimens from all patients showed neurofibromas with plexiform and/or diffuse features. Treatments included tracheotomy (n = 4), carbon dioxide laser excision (n = 4), modified neck dissection (n = 3), partial pharyngectomy (n = 1), supraglottic laryngectomy (n = 1), and endoscopic hemilaryngectomy (n = 1). Three patients were successfully decannulated. Follow-up ranged from 1 to 15 years. One patient was lost to follow-up. No evidence of malignant degeneration was noted. CONCLUSIONS: Neurofibroma of the larynx is a rare condition that should be considered in the differential diagnosis of children with a submucosal laryngeal mass. In our series, all patients had associated neurofibromatosis type 1. Complete surgical excision is the treatment of choice in cases of localized small lesions. To prevent debilitating outcomes due to aggressive surgery, minimally invasive procedures (partial excision via endoscopic approach) may be preferable for larger lesions that infiltrate the surrounding vital structures. Long-term follow up of these patients is essential owing to the possibility of malignant transformation.

The role of mitomycin in the prevention and treatment of scar formation in the pediatric aerodigestive tract: friend or foe?

OBJECTIVE: To evaluate the role of mitomycin in the prevention and treatment of scar formation in the pediatric aerodigestive tract. DESIGN: Prospective study; institutional review board-approved clinical trial. SETTING: Tertiary care pediatric medical center. PATIENTS: Fifteen patients; choanal atresia in 5 patients, airway stenosis in 8 patients, hypopharyngeal stenosis in 1 patient, and esophageal stenosis in 1 patient. OUTCOME: The efficacy and safety of mitomycin in the prevention of scar formation. INTERVENTION: All patients underwent surgical repair of the stenotic area, followed by topical application of mitomycin (1 mL of 0.4 mg/mL) for 4 minutes. RESULTS: Ten patients (67%) showed major improvement, 4 patients (27%) showed minor improvement, and 1 patient (7%) showed no improvement. CONCLUSION: Topical application of mitomycin can play an effective role in the prevention and treatment of scar formation in the aerodigestive tract.

The presentation and management of nasal dermoid: a 30-year experience.

OBJECTIVE: To review the presentation of nasal dermoid in children and present guidelines for its management. DESIGN: Retrospective study (January 1, 1970, through December 31, 2000). SETTING: Tertiary-care pediatric medical center. PATIENTS: Number of patients: 42 (28 boys and 14 girls). Intervention Extensive review of the initial presentation, significant family and medical history, workup, surgical approach, complication, and rate of recurrence. RESULTS: Mean age of presentation was 32 months. The most common presentation was a nasoglabellar mass, in 13 patients (31%). Five patients presented with an associated craniofacial abnormality. Thirty-nine patients (93%) underwent a preoperative imaging workup. Thirty-one (74%) did not show any clinical and/or radiographic indication of intracranial extension. Thirty-four (81%) underwent extracranial excision, and 8 (19%) underwent combined intracranial-extracranial excision. Five patients (12%) presented with recurrence, extracranially in 4 and intracranially in 1. No other complication was noted, with a mean follow-up of 7 years. CONCLUSIONS: Nasal dermoid is a rare congenital anomaly. Preoperative evaluation is essential to rule out intracranial extension. Surgical strategy depends on the location and extent of the lesion, ranging from local excision to a combined intracranial-extracranial approach. Recurrence is uncommon and often easily managed.

The role of chromosomal translocation (15;19) in the carcinoma of the upper aerodigestive tract in children.

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.

Delayed presentation of lymphatic malformation of the cervicofacial region: role of trauma.

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.