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[Double aortic arch: clinical aspects, diagnosis and therapy in children and adults].

Double aortic arch is a rare vascular anomaly which usually causes tracheal and esophageal compression in the first few months of life. During the last 30 years, 7 children, 2 to 24 months old, and one 29-year-old woman with double aortic arches have been treatedatour institution. Symptoms, diagnosis and treatment of these patients were evaluated. Dyspnoe, stridor, recurrent pulmonary infections, feeding problems and failure to thrive were the leading symptoms. Despite typical symptoms from early childhood, the diagnosis was missed in our adult patient. Typical compression of the esophageus and the trachea was visualized by esophagography by 7 and bronchoscopy/-graphy by 6 patients. Angiography was performed in all children, whereas magnetic resonance angiography and computed tomography were done in the adult patient. Resection of the smaller aortic arch, left in 3 and right in 5, through a right or a left posterolateral thoracotomy was uncomplicated and fully resolved the symptoms in all patients. Typical symptoms in early childhood should lead to prompt diagnosis and surgical treatment of double aortic arch. Surgical resection of the smaller aortic arch should also be performed in oligosymptomatic patients to prevent complications later. Preoperative angiography can be replaced by the less invasive magnetic resonance imaging and computed tomography.

Adult↗

Type-2 submucosal posterior laryngeal cleft diagnosed on CT scan.

Posterior laryngeal cleft (PLC) is a rare developmental abnormality, which requires early diagnostic laryngoscopy and management if considerable morbidity and mortality are to be avoided. We report the case of a 25-year-old man with a life-long history of a weak, breathy voice, in whom the diagnosis of PLC was made by CT scanning. This is the first reported case of a PLC being diagnosed by CT scanning. Whilst direct laryngoscopy should remain the investigation of choice in congenital stridor, CT scanning may occasionally be useful.

Adult↗

Pediatric dyspnea caused by supraglottic stenosis: a rare complication of alkali corrosive injury.

Acquired supraglottic stenosis is a rare, but potential late sequela of corrosive injury, usually occurring 1 to 2 months after the initial trauma. The presentation may be delayed in children and is potentially lethal. We describe two pediatric patients, aged 31 and 16 months, who developed progressive inspiratory stridor and hoarseness about 2 months after accidental alkali ingestion. Fiberoptic laryngoscopy showed supraglottic stenosis. Intubation was achieved with a very small endotracheal tube under fiberoptic laryngobronchoscopic guidance prior to general anesthesia. Both patients successfully underwent CO(2) laser treatment under direct laryngoscopic guidance. There has been no case of recurrence reported after 1 year of follow-up other than a thin membranous scar in one case at the 3rd month of follow-up, which was easily dealt with by bronchoscopy dilatation. For such patients, monthly laryngoscopic examinations are advisable after alkaline corrosive injury for a minimum of 6 months. Prompt diagnosis and early airway intervention can reduce the need for tracheostomy and decrease the mortality rate.

Alkalies↗

Idiopathic laryngeal spasm: management and long-term outcome.

Idiopathic laryngeal spasm (ILS) is an uncommon disorder characterised by brief episodes of stridor, occurring at any time. Subsequent outpatient ENT examination is normal. These episodes cause considerable anxiety for both patient and physician. Little is known about the initiating event(s) in this condition or the long-term outcome. Using a combination of telephone and postal questionnaires with case note review, we have reviewed a cohort of 21 patients with this diagnosis managed by the senior author over the last 15 years. None of the 19 patients who responded were worse; 13 (68%) described improvement or complete resolution of symptoms. ILS is difficult to classify in the spectrum of vocal cord disorders, but appears distinct to those previously described. The condition responds well to a conservative management approach of reassurance and counselling.

Adolescent↗

Polypoid type of laryngeal sarcoidosis--case report and review of the literature.

A 45-year-old mentally retarded woman was discovered to produce a stridor upon inhalation. Fiberoptic laryngoscopic study revealed a smooth-surfaced, wide, and pedunculated tumor on the right arytenoid and the right aryepiglottic fold. Histopathological study revealed that the tumor was characterized by granulomatous inflammation. The condition was diagnosed as laryngeal sarcoidosis. The tumor was resected surgically. Surgical resection of the tumor and injection of corticosteroid should be performed for polypoid-type laryngeal sarcoidosis. The case is discussed in detail, along with a review of the literature pertaining to sarcoidosis of the larynx.

Female↗

Laryngotracheal stenosis in children.

The diagnosis of laryngotracheal stenosis should be suspected in children with stridor, feeding difficulties, or atypical croup. Only half of the children with congenital laryngotracheal stenosis require tracheotomy, and many of these children can be decannulated following uncomplicated surgical therapy. In contrast, tracheotomy-dependent patients with acquired laryngotracheal stenosis require more extensive surgical intervention, which should be carried out as early as possible to provide the best opportunity for developing normal oral communication.

Child↗

Leeches in the larynx.

This is a report of four patients who inhaled leeches. They presented with severe attacks of inspiratory stridor, difficulty in breathing and spitting blood. Indirect laryngoscopy revealed a dark greenish living foreign body in the larynx in each case. Laryngoscopy was performed with local anaesthesia in two of the cases and general anaesthesia was used in the other two. The leeches were removed with forceps.

Adult↗

Ligneous conjunctivitis, hydrocephalus, hydrocele, and pulmonary involvement in a child with homozygous type I plasminogen deficiency.

UNLABELLED: Ligneous conjunctivitis is a rare and unusual form of chronic pseudomembranous conjunctivitis which usually starts in early infancy. Plasminogen deficiency has recently been associated with ligneous conjunctivitis. The disease may be associated with pseudomembranous lesions of other mucous membranes in the mouth, nasopharynx, trachea, and female genital tract and also with congenital hydrocephalus. In this report, a 1-month-old Turkish boy who had pseudomembranous conjunctivitis, occlusive hydrocephalus, and hydrocele is presented. After surgery for ventriculo-peritoneal shunt establishment, he developed inspiratory stridor, respiratory distress, and pulmonary atelectasis. Tracheal pseudomembranes were also demonstrated by bronchoscopy. Plasminogen antigen level and plasminogen activity were very low. Genomic DNA from the patient was screened for mutations in the plasminogen gene and a homozygous L650fsX652 mutation (deletion of 2081C) was detected. Both of his parents were heterozygous for this mutation. He died due to respiratory failure during follow-up. CONCLUSION: Ligneous conjunctivitis related to type I plasminogen deficiency is relatively common in the Turkish population, however, mutations are heterogeneous and a common founder is unlikely.

Chromosome Deletion↗

Biotinidase deficiency: the importance of adequate follow-up for an inconclusive newborn screening result.

UNLABELLED: Biotinidase deficiency is an inherited metabolic disorder characterized by inability to recycle protein-bound biotin. It usually presents with ataxia and seizures, though atypical presentations have also been described. We report a 15-month-old boy with profound biotinidase deficiency who presented with laryngeal stridor and subsequently developed severe ataxia and lactic acidosis. Subsequently, it was discovered that the patient's newborn screening test for biotinidase activity had been inconclusive, but confirmatory testing had not been done. Brain magnetic resonance imaging showed multiple white matter non-enhancing T2 hyperintensities, which largely resolved following 6 months of biotin therapy; however, there was residual deafness and mental retardation. CONCLUSION: An argument is made for universal newborn screening in biotinidase deficiency and improved mechanisms for follow-up of positive screens, because delay in diagnosis results in irreversible morbidity, newborn screening is cost effective, and early therapy prevents the neurologic sequelae.

Acidosis, Lactic↗

Prolonged croup due to herpes simplex virus infection.

UNLABELLED: Herpes simplex virus (HSV) is an uncommon cause of acute laryngitis in immunocompetent patients since it mostly occurs in immunocompromised subjects. We present two previously healthy children with prolonged gingivostomatitis and stridor (lasting 3 and 4 weeks) in whom HSV-1 was isolated from subglottal ulcers. CONCLUSION: HSV should be considered a possible pathogen in cases of prolonged or atypical croup not only in immunocompromised or elderly patients but also in otherwise healthy children.

Croup↗

Postextubation airway management with nasal continuous positive airway pressure in a child with Down syndrome.

We describe our experience with use of variable-flow nasal continuous positive airway pressure (NCPAP) to manage postextubation stridor in a 31-month-old child with Down syndrome (DS). Although it has been recognized that children with DS tend to develop obstruction of the upper airway postoperatively, little is known concerning appropriate management of this situation. Although there are surprisingly few reports of use of variable-flow NCPAP for children older than preterm infants, we successfully treated postextubation ventilatory complications by providing variable-flow NCPAP without complications such as pneumothorax.

Anesthesia, Inhalation↗

Pediatric otolaryngologic manifestations of gastroesophageal reflux disease.

In adults, an estimated 4% to 10% of chronic, nonspecific laryngeal disease seen in otolaryngologic clinics is associated with gastroesophageal reflux disease (GERD). Although no such estimates exist in children, many investigators have reported extraesophageal manifestations of GERD, of which the most common is the association of GERD with asthma and chronic cough. A variety of signs and symptoms of otolaryngologic disease also have been attributed to GERD, including hoarseness, laryngitis, chronic rhinitis, sinusitis, globus pharyngeus, recurrent croup, laryngomalacia, stridor, subglottic stenosis, otalgia, vocal cord granulomas, and oropharyngeal dysphagia. However, proof of the association between these manifestations of otolaryngologic disease and GERD is sparse. Furthermore, the manifestations of otolaryngologic disease often occur in the absence of such classic systems of GERD as heartburn or chest pain. This review explores the role of GERD in otolaryngologic disease in children.

Age Factors↗

Common aortic arch anomalies: diagnosis and management.

Vascular rings are a rare form of congenital malformation that completely encircle the trachea and esophagus with vascular structures. The two most common forms are double aortic arch and right aortic arch with an aberrant left subclavian artery and a left-sided ligamentum arteriosum. Patients may present with symptoms of airway or esophageal impingement, including "noisy breathing," stridor, respiratory distress, difficulty feeding, or dysphagia. Typically, the more severe the airway or esophageal compression, the earlier the age at presentation. Diagnosis was traditionally made by posterior compression of the esophagus demonstrated by barium swallow; in the current era, CT angiography or magnetic resonance angiography is used. Although helpful to delineate intracardiac anatomy and associated structural heart defects, echocardiography is less useful for imaging vascular structures when atretic segments comprise part of the vascular ring. Management includes surgical division of the structures contributing to the vascular ring. Some centers also perform surgical remodeling of Kommerell's diverticulum because this structure may contribute to continuing tracheal or esophageal compression even after the ring has been released. Patients with associated tracheomalacia may continue to have symptoms that can last for months, but ultimately resolve with time. Long-term results are generally excellent with minimal morbidity and mortality.

Journal Article↗

Successful reconstruction of extensive laryngotracheal strictures after inhalation burn injury: report of a case.

We report a rare case of long segmental laryngotracheal stenosis following inhalation burn injury. The patient presented 2 months after his injury with progressive stridor and dyspnea necessitating tracheostomy. A computed tomographic scan of the neck revealed stenosis extending from the vocal cords to the top of the sternum. Repair was successfully carried out with multiple surgical procedures employing hinge-flap closure tented with autogenous tissue.

Adult↗

Pulmonary Kaposi's sarcoma in the acquired immune deficiency syndrome. Clinical, radiographic, and pathologic manifestations.

Pulmonary Kaposi's sarcoma related to the acquired immune deficiency syndrome (AIDS) has not been well characterized. To define the clinical, radiographic, and pathologic features of this entity, 11 autopsy-proved cases of pulmonary Kaposi's sarcoma were reviewed. The most common clinical symptoms were dyspnea and cough, but hemoptysis and stridor were also found. Nodular infiltrates and pleural effusions were the most commonly found radiographic abnormalities. Pulmonary function tests were sensitive in detecting the pulmonary abnormalities due to Kaposi's sarcoma. A low diffusion capacity, lack of arterial desaturation with exercise, and obstruction to airflow were suggestive of pulmonary involvement with this malignancy. Although endobronchial Kaposi's sarcoma was visualized at bronchoscopy as cherry-red, slightly raised lesions, bronchial biopsy specimens always showed no abnormalities. Transbronchial brushings and biopsy specimens and analysis of pleural fluid were also not helpful in establishing a diagnosis. In the seven subjects with extensive parenchymal Kaposi's sarcoma at autopsy, the pleura was always involved. Eight subjects had involvement of the tracheobronchial tree. In all of the subjects, pulmonary Kaposi's sarcoma was a significant cause of morbidity, and in three of 11 subjects (27 percent) it was the direct cause of death.

Acquired Immunodeficiency Syndrome↗

Bronchoscopic phototherapy at comparable dose rates: early results.

Photodynamic therapy is a recently introduced treatment for surface malignancies. Since January 1987, 10 patients with endobronchial neoplasms have had bronchoscopic photodynamic therapy at similar dose rates (400 mW/cm) for total atelectasis (2), carinal narrowing with respiratory insufficiency (2), or partial obstruction without collapse (4). Two patients underwent photodynamic therapy as a preliminary to immunotherapy. Histologies included endobronchial metastases (colon, ovary, melanoma, and sarcoma, 1 each; and renal cell, 3) and primary lung cancer (3). The 2 patients with total atelectasis had complete reexpansion after photodynamic therapy, which permitted eventual sleeve lobectomy in 1. Carinal narrowing was ameliorated in the 2 patients seen with inspiratory stridor, thereby permitting hospital discharge. Endoscopically resected fragments after photodynamic therapy exhibited avascular necrosis. These data support further controlled studies of photodynamic therapy by thoracic surgical oncologists to define its limitations as well as to improve and expand its efficacy as a palliative or surgical adjuvant.

Adult↗

Main bronchial sleeve resection with pulmonary conservation.

Twenty-seven main bronchial resections (19 left, 8 right) were performed without pulmonary resection between 1975 and 1991. The patients were 17 men and 9 women with an average age of 35 years (range, 20 to 65 years). Tumors comprised 55% of the lesions, including 9 carcinoid tumors (33%), 2 mucoepidermoid tumors, 2 fibrous histiocytomas, 1 hemangiopericytoma, and 1 large cell carcinoma. Scarring and stenosis secondary to multiple causes occurred in 10 patients (37%). Two patients had miscellaneous lesions. Presenting symptoms included dyspnea (52%), wheezing or stridor (44%), cough (41%), hemoptysis (37%), and pneumonia (18%). Preoperative chest roentgenogram was abnormal in 60% of patients, whereas tomograms delineated the lesion in 94%. All patients had bronchoscopy for lesion evaluation. Anesthesia was accomplished through a long single-lumen endotracheal tube in 19 cases and a double-lumen tube in 8 cases. Mobilization and exposure techniques to create a tension-free anastomosis were critical for left main bronchial resections and included pretracheal mobilization (100%), neck flexion (100%), tracheal and main bronchial retraction (85%), aortic and pulmonary artery retraction (44%), and intrapericardial hilar release (33%). All resections were for cure; there was no operative mortality. Morbidity in 4 patients (15%) included an anastomotic stenosis (successfully reresected), prolonged air leak and pneumonia, transient recurrent nerve palsy, and atelectasis. Median 5-year follow-up revealed 92% of patients alive, with only one of two late deaths being disease-related. Main bronchial resection is an ideal technique for selected benign and malignant lesions, allowing complete pulmonary parenchymal preservation.

Adult↗

Airway complications in relapsing polychondritis.

To analyze the importance of airway involvement in relapsing polychondritis, an illustrative case report is presented and 62 patients reported in the literature with serious airway complications are reviewed. There were 47 female and 17 male patients, with an average age of 40.3 years (range, 2 to 73 years). Patients were seen with hoarseness, breathlessness, cough, stridor, wheezes, and tenderness over laryngotracheal cartilages. Respiratory tract involvement was confirmed by conventional radiography, tomography, computed tomography, dynamic pulmonary function tests, and bronchoscopy. Corticosteroids and antiinflammatory and immunosuppressive agents were used in these patients. Tracheostomy was performed in 18 patients. Death occurred in 13 patients despite tracheostomy or corticosteroid therapy, or both. A detailed analysis of the clinical, radiological, and pulmonary function studies is presented, with emphasis on upper airway mechanics. The medical and surgical management options are reviewed, including the use of endotracheal prosthesis and extraluminal splinting in dynamic airway collapse.

Adolescent↗