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Airway compromise and delayed death following attempted central vein injection of propylhexedrine.

Propylhexedrine is a potent alpha-adrenergic drug available as a nasal decongestant, which drug abusers sometimes extract and inject into a central vein. A 25-year-old white male presented to a local emergency department 32 h after attempting to inject his right internal jugular vein with "home-made crank." Following injection, he noted right neck pain, followed by fever and chills. On emergency department admission, he had inspiratory stridor and respiratory distress. Massive edema of his right neck extended from his anterior chest to the right parotid. Neck radiographs showed extensive paracervical swelling with displacement of the trachea. The patient was taken to surgery for nasotracheal intubation with fiberoptic guidance and surgical exploration. The neck contained extensive necrotic tissue that was surgically debrided. In spite of treatment with antibiotics, he developed progressive renal failure and hypotension unresponsive to fluid therapy, followed by cardiopulmonary arrest and death.

Adult↗

Adult retropharyngeal abscess: a case report and review of the literature.

The case of an 18-yr-old otherwise healthy adult with retropharyngeal abscess (RPA) presenting with sore throat and syncope is reported. The case illustrates that sore throat with symptoms out of proportion to oropharyngeal findings should prompt a search for pathology other than simple pharyngitis. A literature search for case reports of RPA in adults was done to derive characterizations about this disease. Adult RPA patients present with sore throat, dysphagia, neck pain and, less commonly, stridor. Adult RPA occurs as a complication of procedures or blunt trauma to the neck, or spread of infection from an adjacent focus. The incidence of underlying disease causing immunosuppression is high. RPA in adults without history of preceding trauma or coexistent illness is unusual.

Adolescent↗

Anesthetic management of children with neuroblastoma.

STUDY OBJECTIVE: To review the 11-year anesthetic experience with neuroblastoma at Children's Hospital Medical Center, Boston, MA. DESIGN: Retrospective study. SETTING: Children's Hospital, Boston, MA. PATIENTS: 59 consecutive children with neuroblastomas who underwent surgical procedures between 1977 and 1989. INTERVENTIONS: Symptoms, physical findings, laboratory data, and results of echocardiographic examination were recorded. Type of chemotherapy and urine catecholamine levels were noted. Intraoperative information was extracted about the surgical procedure, anesthetic technique, blood loss, and intraoperative vital sign changes. Postoperative data were reviewed for complications. MEASUREMENTS AND MAIN RESULTS: 18 patients presented with a posterior mediastinal tumor. Five children presented with stridor, wheezing, pneumonia, and pleural effusion. Two children presented with hypertension. Left ventricular ejection fraction was within normal limits preoperatively in all patients evaluated by echocardiography. Intraoperative hypertension and tachycardia occurred in 3.5% of the children during tumor manipulation. No hypotension was noted following tumor removal. Of the 18 patients presenting with a posterior mediastinal mass, 3 had tracheal deviation caused by the tumor. The intraoperative course and extubation were uneventful in 2 of the patients, and the other patient remained intubated postoperatively. No adverse effect was identified for any particular anesthetic drug or technique used. CONCLUSIONS: Although neuroblastomas may be associated with hypertension upon presentation, intraoperative hypertension is rare. Tracheal compression and deviation were noted in 5.2% of patients with posterior mediastinal masses; however, airway complications did not occur. No specific optimal anesthetic regimen can be recommended.

Abdominal Neoplasms↗

Comparison of intravenous ketorolac and alfentanil as supplements to propofol anesthesia for diagnostic panendoscopy.

STUDY OBJECTIVE: To determine if ketorolac tromethamine is an acceptable alternative to alfentanil as a supplement to propofol for diagnostic panendoscopy. DESIGN: Randomized, double-blind study. SETTING: University medical center. PATIENTS: 40 patients scheduled for panendoscopy and laryngeal tissue biopsy. INTERVENTIONS: Patients were randomly assigned to receive either alfentanil 14.5 micrograms/kg or ketorolac 1.0 mg/kg in a double-blind fashion, 5 to 10 minutes before induction of general anesthesia. MEASUREMENTS AND MAIN RESULTS: Heart rate (HR) and noninvasive blood pressure (BP) were measured and recorded before and immediately after injection of the study drug, after laryngoscopy for the endotracheal tube placement, and after initiation of diagnostic panendoscopy. Bleeding in the operative field was rated by the endoscopist. Observation from discontinuation of the propofol infusion and nitrous oxide inhalation to eye opening, head lifting, and orientation to time and place was observed and recorded. The presence of stridor after extubation, and pulse oximeter-determined arterial blood oxyhemoglobin saturation immediately after extubation and 5 minutes later, were noted. In the recovery room, the ability to tolerate oral fluids, sit, stand, and walk were recorded. Supplementation with ketorolac provides faster recovery from anesthesia as evidenced by shorter time to eye opening, head lifting, and orientation to time and place. However, no intergroup differences were found in measured intraoperative variables (BP and HR following laryngoscopy, tracheal intubation, diagnostic panendoscopy, and tissue biopsy). Operative site bleeding was comparable in both groups. The variables reflecting street readiness and the incidence of nausea and vomiting were statistically comparable. CONCLUSION: Supplementation of propofol anesthesia with ketorolac is an efficacious alternative to supplementation with alfentanil. The faster recovery in the ketorolac group is explained by the mostly peripheral effect of this drug, whereas the slow decline in the alfentanil concentration at the effective site may be responsible for slower emergence from anesthesia.

Alfentanil↗

Brief upper airway dysfunction.

We have analysed the clinical manifestations of nine patients with brief upper airway dysfunction (BUAD) who attended the thoracic department of a major teaching hospital between 1987 and 1991. Episodes of BUAD developed within 1-4 months of presentation in three patients but were undiagnosed for 2.5-12.5 years in six. The mean age at onset was 51 years ranging from 37 to 66 years. The episodes occurred at irregular intervals. They lasted approximately 1-5 min, were frightening and consisted of an initial phase of obstructive apneoa lasting a few seconds to 2 min and a second phase of respiratory distress with inspiratory stridor lasting 1-4 min. Daytime episodes occurred in all and at night in five, waking three of the patients from sleep. In most instances, throat irritability triggered the episodes which were often preceded by cough. Potential causes of throat irritability included respiratory tract infection, allergy, oesophageal reflux and obstructive sleep apnoea. After treatment of throat irritability BUAD has ceased for at least a year in six of the eight with adequate follow-up. In conclusion, BUAD has characteristics clinical features which should enable it to be recognized more frequently, ensuring successful management.

Adult↗

Successful surgical treatment of tracheal compression following right pneumonectomy in infancy. Long-term follow-up.

Two patients (aged 10 and 16 months) underwent right pneumonectomy: one for an oesophageal bronchus, the other for a hypoplastic right lung with sequestration. Both patients subsequently developed severe stridor. The heart rotated to the right and the aortic arch compressed the trachea. This unusual vascular compression was treated by a bypass graft between the ascending and descending aorta and division of the aortic arch between the left carotid and left subclavian artery at the age of 5 years and 4 months, and 20 months respectively. The postoperative recovery was uneventful in the first patient. The second patient required 7 weeks of ventilatory support. Both patients are well (14 and 7 years after surgery). Cardiac catheterisation and angiography in the first patient and Doppler echocardiography in the second demonstrated unrestricted flow through the graft into the descending aorta. Pulmonary function studies showed a restrictive defect with some obstruction in both children. Despite this finding, both patients enjoy a normal life with some sporting activities. The long-term results suggest the effectiveness of this procedure in the treatment of this rare but severe complication of pneumonectomy in infancy.

Aorta↗

Aberrant right subclavian artery associated with common carotid trunk. A rare cause of vascular ring.

We have reviewed four cases of incomplete vascular rings caused by the presence of a common carotid trunk from which arose both carotid arteries associated with an aberrant right subclavian artery. The patients were aged between 3 and 9 months. All patients presented with recurrent respiratory tract infections. Three patients showed signs of malnutrition and failure to thrive caused by episodes of bronchial aspiration from extrinsic compression of the oesophagus. One patient presented with dyspnoea induced by feeding and another had stridor. A common carotid trunk associated with an aberrant subclavian artery was confirmed in all cases. No other associated anomaly was observed in any patient. Ligature and section of the anomalous right subclavian artery was performed in all patients. The low incidence of this type of vascular ring, its physiopathological mechanism and surgical management are discussed.

Carotid Arteries↗

Self-expanding metal stent for tracheobronchial strictures.

To determine the role of expandable metal stent (Wallstent) in treating tracheobronchial strictures, 12 patients with recurrent symptoms of airway obstruction due to either benign or malignant strictures were studied. The seven benign strictures were anastomotic stricture following sleeve resection (2) and single lung transplant (1), tracheal amyloidosis (1), idiopathic chondritis (2), and post-tracheostomy stricture (1). The five malignant strictures were due to recurrent adenoid cystic carcinoma of trachea (1), large-cell carcinoma of lung (1), recurrent laryngeal squamous carcinoma (1), squamous carcinoma of the trachea (1), and malignant melanoma (1). The placement of stents was performed under rigid bronchoscopic guidance with no complications. All patients with benign strictures derived subjective and functional improvement with stenting. No evidence of restenosis within the stented segment in six of the seven benign strictures was found within up to 24 months. Repeated diathermy resection was required in the patient with recurrent amyloidosis through the distal end of the stent. Among the malignant strictures, symptomatic relief was achieved in four of the five patients. One metal stent migrated proximally and was replaced by a Montgomery T tube. One patient with relief of stridor died at 4 months due to carcinomatosis. Tumour ingrowth through the metal stent remains problematic in two patients. However, the incidence of palliative interventions required has markedly reduced after stenting.

Adult↗

Report of an obstructive goiter and its surgical treatment during delivery.

We report a case of a morbidly obese young woman in her third trimester of pregnancy presenting with a history of goiter and respiratory disease. The recent history of this patient was significant for worsening respiratory symptoms over a period of 2 weeks, and, on presentation at 36 weeks gestation, she was stridorous, dyspneic at rest, and had a hoarse voice. Evaluation revealed a morbidly obese individual with a large goiter. She was biochemically euthyroid. Fiberoptic laryngoscopy revealed a left true vocal cord paresis, and ultrasound evaluation was significant for diffuse multinodular enlargement, with each lobe measuring greater than 10 cm and the isthmus measuring 5. Pulmonary function testing revealed a significant degree of upper airway obstruction without significant lower airway disease. Given the patient's clinical signs and symptoms, her tenuous airway, poor candidacy for urgent tracheotomy, and her proximity to delivery, it was agreed that the patient should undergo elective cesarean section and at its completion undergo subtotal thyroidectomy for the obstructive goiter.

Adult↗

Flexible fiberoptic bronchoscopy through the laryngeal mask airway in a small, premature neonate.

The laryngeal mask airway (LMA) was introduced as a supraglottic device in anesthesia for routine use in the normal adult and pediatric population. Because the distal end of properly placed LMA faces the laryngeal inlet, this device can be used as a guide to flexible fiberoptic bronchoscopy (FFB) performance. In this clinical case, we present a small premature neonate with severe congenital biphasic stridor. FFB was performed successfully through the LMA while maintaining a patent airway during general anesthesia and permitting spontaneous respiration, as well as allowing assisted ventilation when necessary. An immediately subglottic intramural mass was revealed and tracheostomy was performed.

Anesthesia, General↗

Squamous cell carcinoma of the subglottis.

STUDY DESIGN: A retrospective study over a 25-year period of patients with laryngeal carcinoma treated by the Department of Otolaryngology at Wake Forest University. METHODS: The boundaries of the subglottis were defined as 5 mm below the free edge of the true vocal folds extending to the inferior border of the cricoid cartilage. All were staged according to American Joint Committee on Cancer: stages I and II were considered early and stages III and IV as late. Patients were grouped by treatment modality of surgery alone, surgery and radiotherapy, radiotherapy alone, and radiotherapy with surgical salvage. RESULTS: Fifteen patients represented 1.4% (15/1098) of laryngeal cancers diagnosed within that period. All patients had squamous cell carcinoma of the subglottis of which 20% (3/15) had early-stage disease (T1-T2) and 80% (12/15) had late-stage disease (T3-T4). Overall 3-year survival was low (25%) and is attributed to a high incidence of advanced-stage disease with a high rate of extralaryngeal spread and/or metastasis, especially to the lungs and paratracheal nodes. CONCLUSIONS: To improve earlier detection by primary care physicians and otolaryngologists and to improve treatment outcome, awareness of subglottic carcinoma and its appropriate evaluation is critical in the patient presenting with hoarseness and/or stridor. Radiation therapy treatment to include the low and upper mediastinal compartments to cover local/regional extralaryngeal involvement is advocated as well as paratracheal lymphatic neck dissection and thyroidectomy in surgically treated lesions.

Adult↗

Tracheomalacia secondary to obstructive sleep apnea.

Tracheomalacia (TM) is abnormal flaccidity of the trachea caused by inadequate cartilaginous support by the C-shaped tracheal rings. This defect generally results in various degrees of dynamic narrowing of the airway lumen. TM usually presents with expiratory stridor, wheeze, and respiratory obstruction. TM is usually associated with tracheoesophageal fistula and esophageal atresia or with other thoracic lesions of vascular rings and tumors. We report a well-documented case of a 20-month-old boy who presented to our institution with a severe history of obstructive sleep apnea and adenotonsillar hypertrophy. On direct laryngoscopy/bronchoscopy, severe TM was noted. Tonsillectomy and adenoidectomy was performed, and by the 37th postoperative day, the TM had completely resolved. We review the literature, discuss the differential diagnosis and clinical evaluation, and propose a new pathophysiological mechanism by which obstructive sleep apnea causes TM.

Adenoidectomy↗

Blunt laryngeal trauma resulting in arytenoid dislocation and dysphonia.

We present a case of left arytenoid dislocation due to blunt laryngeal trauma causing a subsequent large granuloma formation resulting in dysphonia and stridor. The patient underwent emergency excision of the obstructive granuloma and speech therapy was started post-operatively. A few weeks after surgery, the granuloma started to recur and laryngeal manipulation by a specialist osteopath was performed. A few weeks after the conservative management, the recurred granuloma resolved completely and patient's voice improved remarkably. Dislocation of the arytenoid cartilage due to blunt trauma is relatively rare and a consequent spontaneous granuloma formation has not been reported so far in the literature. This is also the first report about efficacy of speech therapy combined with laryngeal manipulation in the management of the arytenoid dislocation and the subsequent laryngeal granuloma.

Accidental Falls↗

Endoscopic ultrasound-guided fine needle aspiration of mediastinal lymph node in patients with suspected lung cancer after positron emission tomography and computed tomography scans.

BACKGROUND: The treatment of patients with non-small cell lung cancer (NSCLC) depends on the stage. Positron emission and computed tomography (CT) scans can identify suspicious lymph nodes that require biopsy. We prospectively evaluated the yield and accuracy of endoscopic ultrasound-guided fine needle aspiration (EUS-FNA) in sampling mediastinal lymph nodes and compared its accuracy to that of 18F-fluorodeoxyglucose positron emission tomography (FDG-PET) and CT in staging NSCLC. METHODS: A consecutive series of patients with suspicious nodes on PET or CT scan in the posterior mediastinal lymph node stations (#5, 7, 8, or 9) were prospectively evaluated by EUS-FNA. The reference standard included thoracotomy with complete lymphadenectomy in patients with lung cancer or if EUS-FNA was benign, repeat clinical imaging, or long-term follow-up. RESULTS: There were 104 patients (63 men) with 125 lesions (117 lymph nodes, 8 left adrenal glands) who underwent EUS-FNA. The sensitivity, specificity, positive predictive value, negative predictive value, and accuracy of EUS-FNA were 92.5%, 100%, 100%, 94%, and 97%, respectively. EUS-FNA was more accurate and had a higher positive predictive value than the PET or CT (p < 0.001) scan in confirming cancer in the posterior mediastinal lymph nodes. EUS-FNA documented metastatic cancer to the left adrenal in all 4 patients with advanced disease. No deaths resulted from EUS-FNA. One patient experienced self-limited stridor. CONCLUSIONS: EUS-FNA is a safe, accurate, and minimally invasive technique that improves the staging of patients with NSCLC. It is more accurate and has a higher predictive value than either the PET scan or CT scan for posterior mediastinal lymph nodes.

Aged↗

Brown-Vialetto-Van Laere syndrome; variability in age at onset and disease progression highlighting the phenotypic overlap with Fazio-Londe disease.

We report four siblings showing features of a pontobulbar palsy, a mixed spinal and upper motor neuropathy and variable deafness. The observation of affected males and females born to consanguineous first cousin parents suggests autosomal recessive inheritance. Two children presented in the first 16 months of life with stridor and died of respiratory failure by the age of 2 years. Hearing loss was not apparent in these infants. In contrast, 2 further siblings developed a bulbar palsy in their sixth year followed by the onset of deafness and features of an anterior horn neuropathy with corticospinal tract involvement. They exhibited a relatively slow but relentless decline over a period of several years. These cases highlight the phenotypic overlap of Brown-Vialetto-Van Laere syndrome with Fazio-Londe disease. Rather than representing two separate disorders, our findings suggest the possibility of a single disease entity which may usefully be considered a form of juvenile amyotrophic lateral sclerosis.

Adolescent↗

Type 2 Gaucher disease: 15 new cases and review of the literature.

OBJECTIVES: To provide a description of type 2 Gaucher disease. To attempt to define type 2 Gaucher disease within the spectrum of early-onset neuronopathic Gaucher disease. BACKGROUND: Type 2 Gaucher disease is a rare disorder due to glucocerebrosidase deficiency that comprises a rapidly progressing neurological degeneration associated with visceral signs. Most data collected rely on the description of single cases or siblings. Cases of perinatal-lethal Gaucher disease are frequently considered as type 2 Gaucher patients, though the clinical presentation is different. METHODS: We retrospectively studied the clinical history of 15 original acute Gaucher disease patients and reviewed the available data of 104 published cases of early-onset neuronopathic Gaucher disease, including 61 patients with the acute type and 43 cases of the perinatal-lethal form. RESULTS: The neurological presentation of type 2 Gaucher disease is homogeneous and characterized by precocious, severe, and rapidly progressive brainstem degeneration in the foreground. The most frequent initial signs are hyperextension of the neck, swallowing impairment, and strabismus. Provoked asphyxic episodes generally appear in a second time. They are followed by prolonged spontaneous apneas that seem to be the main pejorative feature. Other neurological signs may be observed, but epilepsy, myoclonic epilepsy/myoclonus, trismus, stridor, and progressive microcephaly are less characteristic. Psychomotor regression may occur, but is not a typical feature of the disease onset. Chronic or subacute pulmonary disease predominates in the visceral involvement. Hepatosplenomegaly, failure to thrive, thrombocytopenia, and anemia are the other remarkable, albeit non-specific, features. The inflammatory component of Gaucher disease is underlined by the addition of unexplained fever to this systemic clinical picture. The natural history and particular signs of perinatal-lethal Gaucher disease do not belong to the type 2 Gaucher disease phenotype. CONCLUSION: Type 2 Gaucher disease is a clinically homogeneous entity. The specificity of the neurological involvement is sufficient to suspect the diagnosis at the onset of the disease. Type 2 and perinatal-lethal Gaucher diseases are easily distinguishable in most cases.

Adolescent↗

Lower brainstem dysfunction in an infant with persistent primitive trigeminal artery.

A 6-month-old boy with persistent primitive trigeminal artery (PPTA) presented with stridor, dysphagia, delayed motor development and postural neck and shoulder dystonia. Magnetic resonance imaging/angiography and ultrasonography revealed PPTA, with flow from the dilated basilar artery to the right internal carotid artery, lower brainstem compression by the dilated basilar artery, and cerebellar vermis hypoplasia. Evoked potentials showed lower pons and medulla oblongata functional disruption. These lesions may be related to vascular etiology in the lower brainstem or to congenital malformation syndrome involving infratentorial structures. The relationship of this condition to Möbius syndrome is discussed.

Abnormalities, Multiple↗