Bruce Benjamin

Minor Congenital Laryngeal Clefts: Diagnosis and Classification

Cleft larynx is a rare congenital anomaly. Detection of an unsuspected minor cleft may be difficult, but the pediatric laryngologist should suspect the possibility of cleft larynx from the clinical features. Four minor clefts are reported, three cases of supraglottic interarytenoid cleft and one of partial cricoid cleft. The technique for endoscopic diagnosis and the distinctive features are described and a classification into four types is proposed.

Congenital Tracheal Stenosis

Twenty-one cases of congenital tracheal stenosis seen at the Royal Alexandra Hospital for Children 1971 through 1980 were reviewed with regard to the clinical features, associated anomalies, endoscopic findings and radiological evaluation. Congenital tracheal stenosis was usually a serious problem often associated with other major anomalies of the respiratory tract, esophagus, or skeleton. There was no set pattern of presentation. The presenting features included: persistent wheeze or stridor, atypical “respiratory distress,” and atypical “croup” or “bronchiolitis.” Documentation of the nature and extent of the stenosis by endoscopy and, if necessary, tracheobronchogram was important for prognosis and to direct treatment. Congenital tracheal stenosis must be considered in infants who present with atypical respiratory tract obstruction, especially where there is an H-type tracheoesophageal fistula, pulmonary hypoplasia or skeletal abnormalities such as hemivertebrae or a thumb abnormality.

Tracheomalacia in Infants and Children

The clinical features of tracheomalacia depend on the location, length, and severity of the weakness of the tracheal wall. There is firm evidence that tracheomalacia in association with tracheoesophageal fistula is due to malformation and deficiency in the tracheal wall, but in other types of tracheomalacia the evidence is less conclusive. A classification is proposed, based on the known histopathologic and endoscopic changes. Endoscopy is considered the most reliable diagnostic examination.

Ten-Year Review of Pediatric Tracheotomy

One hundred sixty-four consecutive tracheotomies are reviewed over the 10-year period 1972–1981. Early in the series acute inflammatory airway obstruction was the major indication for tracheotomy, being 60% of cases in the first 3 years. In the last 3 years this fell to approximately 15%. After 1975 nasotracheal intubation replaced tracheotomy for acute epiglottitis. More recently it has become the treatment of choice for acute laryngotracheobronchitis. Tracheotomy prior to reconstructive surgery for major craniofacial abnormalities is becoming more frequent. Acquired subglottic stenosis is not a problem in our hospital despite the use of long-term nasotracheal intubation in premature infants, and no tracheotomies were performed for this indication. There were few major complications. Decannulation difficulties were due to obstruction by stomal granulation tissue or displaced flap of anterior tracheal wall. There was no case of hemorrhage, no posttracheotomy stenosis, and no death was attributable to tracheotomy. These results demonstrate that in a major pediatric hospital tracheotomy is a relatively safe and effective procedure with minimal morbidity.

Endoscopy in esophageal atresia and tracheoesophageal fistula.

Between 1970 and 1979 152 infants born with the anomaly of esophageal atresia with or without tracheoesophageal fistula or of congenital tracheoesophageal fistula without atresia were treated at the Royal Alexandra Hospital for Children, Sydney. Recent developments in endoscopic equipment and new techniques of anesthesia allow detailed examination of the respiratory tract and esophagus with minimum trauma and maximum safety. Symptomatology relating to the airway and to the esophagus after surgical repair often occurs in patients who may have tracheomalacia, esophageal anastomotic stricture, esophageal reflux and sometimes recurrent or residual fistula. A definite diagnosis of tracheomalacia can be made by finding the typical triad of anteroposterior narrowing of the tracheal lumen, weakening of the semicircular-shaped cartilages and forward ballooning of the widened posterior membranous tracheal wall. Careful examination of the trachea and esophagus allows identification of an elusive recurrent fistula or an H-type fistula. As the primary results of surgery for esophageal atresia and tracheoesophageal fistula improve, long-term problems are becoming increasingly important. The role of the pediatric endoscopist is vital in the care of these patients.

Recurrent respiratory papillomatosis: a 10 year study.

Sixty cases of recurrent respiratory papillomatosis in both children and adults treated over a 10 year period were reviewed. An unexpected finding was that nine of the 60 patients (15 per cent) had symptoms by two months of age; an earlier onset than previously described. The control rate was 66 per cent for paediatric onset patients and 44 per cent with adult onset; the former required more operations. No anaesthetic deaths or complications were encountered. Glottic webs were the only surgical complication. The findings confirm that there is no relationship between puberty and the rate of control or recurrence. The necessity for precise and comprehensive endoscopic examination of the upper aerodigestive tract using a range of endoscopes and rigid telescopes under general anaesthetic is emphasized.

Idiopathic Subglottic Stenosis: Diagnosis and Endoscopic Laser Treatment

The records of 15 patients with idiopathic subglottic stenosis treated at Royal North Shore Hospital, Sydney, between 1980 and 1994 were reviewed. All were female and had similar characteristic clinical and histopathologic features. Endoscopic laser vaporization was the primary treatment in 12 patients and was successful in maintaining the airway of 8 of these 12; this outcome indicates that the disease can be managed, at least initially, by endoscopic laser treatment in most cases.

Endoscopy in congenital tracheal anomalies

Advances in anesthesia and instrumentation miniaturization of endoscopes and high quality fibreoptic systems now allow safe, detailed and precise endoscopic examination of the upper and lower respiratory tract in neonates as well as older children. When the clinical features and radiographic changes suggest a congenital abnormality of the tracheobronchial tree endoscopic examination can give useful information. We believe congenital anomalies of the trachea, particularly areas of congenital stenosis, are not as uncommon as has been supposed. It is now a practical reality to carefully evalute and photographically document tracheal conditions such as vascular or other extrinsic compression, congenital stenosis or web, tracheoesophageal fistula and its sequelae, tracheomalacia and others.

Pathology of Tangier disease

Two cases of Tangier disease are described in children from families unrelated to each other. Necropsy in one case, the first to be reported in this condition, showed large collections of cholesterol-laden macrophages in tonsils, thymus, lymph nodes, and colon, and moderate numbers in pyelonephritic scars and ureter. As the storage cells may be scanty in marrow, jejunum, and liver, the rectum is suggested as the site of choice for biopsy. The diagnosis was confirmed by demonstrating the absence of α-lipoproteins from the plasma of the living child, and by finding low plasma levels in both parents of both cases. The disease can be distinguished from other lipidoses by differences in the predominant sites of storage, staining reactions, and serum lipid studies.

Anesthesia for laryngoscopy.

The requirements of anesthesia for laryngoscopy and microlaryngeal surgery must be compatible with maximum safety and minimum patient discomfort. Some techniques require the use of an endotracheal tube while some do not. Others use a modified tube or a jetting system. In general, for pediatric endoscopy we prefer spontaneous respiration with inhalational anesthesia supplemented by topical lignocaine (lidocaine), and in adults, a relaxant technique with controlled jet ventilation supplemented by topical lignocaine. A new pediatric microlaryngoscope and a new tube for jet ventilation in older children and adults are described.