Faculty Bibliography

Respiratory distress in premature newborns is associated with deficiency of surfactant in the bronchoalveolar lining fluid; this may be influenced by a local deficiency of antioxidants. Severe L-buthionine-S,R-sulfoximine-induced depletion of glutathione (GSH, a major antioxidant) in rodents is associated with lung type 2 cell lamellar body damage and decreased concentrations in lung and bronchoalveolar lavage fluid (BALF) of phosphatidyl choline (a major component of surfactant). At birth, prematurely born newborns (30-34 weeks) had lower peripheral venous plasma GSH concentrations than term (> 36 weeks) babies; these levels decreased further with increasing prematurity (< 27 weeks, with respiratory distress). On day 2, the peripheral venous plasma GSH concentrations reached a nadir, and the lowest levels were found in the most premature newborns. Lymphocyte GSH concentrations were lowest on day 2 and day 7, and in prematures (< 27 weeks, with respiratory distress) remained below adult lymphocyte GSH levels for at least 4 weeks. At birth, prematures (< 27 weeks, with respiratory distress) had a central plasma arterio-venous (A-V) GSH gradient across the lung (an estimate of lung uptake of GSH) of 0.72 +/- 0.15 (mean +/- SD) mumol/L; on day 2, the A-V gradient did not change significantly (0.49 +/- 0.09 mumol/L). At birth, these prematures had markedly decreased BALF GSH concentrations (compared with adult levels), and they were not significantly changed during the first 4 weeks of life. These results suggest that GSH deficiency is present in prematures and that it increases with the degree of prematurity. At birth, GSH deficiency will compromise the lungs' defense against oxidative stress injury. Oxidative stress is likely to increase if hyperoxic treatment is given for respiratory distress in these infants

A retrospective analysis was performed on 103 pediatric patients, less than 5 years of age, undergoing tracheotomy at New York Hospital between 1980 and 1990. Charts were reviewed with respect to primary diagnosis, indication for tracheotomy, duration of the tracheotomy, complication rate and mortality rate. Approximately 62% of the tracheotomies were performed in patients less than 12 months of age, with the most common indication being an acquired or congenital airway abnormality. The number of patients receiving tracheotomies for neurological disorders, however, increased more than threefold over the course of this review. Approximately one-third of the patients experienced immediate, early or delayed complications. There was a significant correlation between the complication rate and weight at the time of the tracheotomy as well as the degree of prematurity of the child. Over one half of the infants under 2000 g suffered complications. A mortality rate of 2.9% was noted in our study with mucous plugging of the tracheotomy being the most common etiology of death

Complete nasal aplasia is an extremely rare clinical entity and most infants are stillborn when this is associated with holoprosencephaly. A viable 3-year-old infant born with frontonasal arrest without holoprosencephaly is presented. The child's main complaint was lack of a nasal airway, which made eating extremely difficult. A method for craniofacial reconstruction of the nasopharynx is presented

Since the advent of antituberculous therapy, tuberculosis of the ear has decreased in incidence; but of late, cases of both pulmonary and otologic tuberculosis are on the rise. In addition, the treatment of aural tuberculosis is now more difficult due to resistance to one or more of the routinely used antituberculous pharmacotherapeutic agents. Urban areas and selected populations have been particularly endangered by the re-emergence of this disease. In light of this developing situation, three cases of aural tuberculous infections are presented. Typical and atypical presentations of the disease, including history, signs, symptoms, and radiographic findings are discussed, as are treatment options. The importance of aural tuberculosis as part of the general increase in incidence and resistance of the disease is examined

Epidermolysis bullosa (EB) is a group of rare inherited disorders in which minor trauma causes blister formation in the skin and mucosa, including the esophagus. Morbidity varies with the type of disease and ranges from occasional trivial skin blisters to death in infancy. Laryngeal involvement presenting as hoarseness and respiratory distress has been reported in nine patients, five of whom had junctional EB. We present the sixth case of junctional EB with laryngeal involvement, and offer guidelines for otolaryngologists and anesthesiologists caring for these fragile patients

Hiccups (singultus) usually present as a common annoyance lasting for short periods. Rarely, they may be the harbinger of a serious disease. We present the case of a 19-year-old man in which intractable hiccups was the first and most prominent symptom of a serious underlying neurologic disorder. The patient had been examined by his pediatrician, and despite multiple medical regiments and physical maneuvers, his symptoms persisted. A thorough head and neck examination revealed a right-sided vocal cord paralysis. This finding prompted obtaining a magnetic resonance imaging scan, which demonstrated a type I Arnold-Chiari malformation associated with a large cervicothoracic syringomyelia. The patient was referred to the neurosurgical service and subsequently underwent a ventriculoperitoneal shunt placement. There was considerable initial improvement in his neurologic status and cessation of the hiccups. However, the symptoms recurred within 1 month. The case report as well as a brief review of the relevant pathophysiologic and etiologic considerations and several treatment modalities for hiccups is presented

Congenital nasal pyriform aperture stenosis has recently been described as a cause of nasal airway obstruction in the newborn. As some investigators have observed, the nasal pyriform aperture is narrowed owing to bony overgrowth of the nasal process of the maxilla. This overgrowth is confirmed by computed tomography. In six previously reported cases, follow-up as long as 16 months revealed normal facial growth. We describe six patients with congenital nasal pyriform aperture stenosis. On eruption of deciduous dentition, four of the six patients were found to have a single prominent central maxillary incisor, ie, 'megaincisor.' Computed tomographic scans have confirmed this dental anomaly as well as maxillary bony overgrowth. A single maxillary incisor has been described as an autosomal dominant microform of holoprosencephaly, a developmental field defect. We propose that in some patients congenital nasal pyriform aperture stenosis may represent more than an isolated congenital abnormality of the airway and may, in fact, be a midfacial dysostosis with associated endocrine and central nervous system abnormalities

Lower tracheal and endobronchial lesions represent a difficult management problem. While there has been some success in the treatment of these lesions with the carbon dioxide laser, the limitations of its delivery system have restricted its use in infants and neonates. The potassium titanyl phosphate (KTP) laser, transmitted via a flexible quartz fiber, can be precisely manipulated even through rigid pediatric bronchoscopes as small as 3.0 mm. In addition, the Hopkins telescopic lens may be used to improve visualization and control. We report our experience using the KTP laser to treat 15 pediatric patients with tracheal and endobronchial lesions. The nature of the lesions, the details of the technique, and the results are presented

Teratomas of the head and neck are a particular type of developmental malformation or tumor that are composed of cells with a full range of histogenic potential. They occur almost exclusively in the newborn and infancy periods. Treatment is directed at complete surgical removal with preservation of normal anatomic structures