Familial congenital stridor.

Almost a century has passed since Rilliet in 1851 described a case of stridor occurring in a young infant. This he presumed to be due to compression on the trachea by either a thyroid or thymic swelling. In 1853 Rilliet and Barthez in their classic French textbook on diseases of children were the first to describe as a clinical entity the condition which later came to be known as congenital laryngeal stridor. Thirty years later Lees (1883) in Great Britain gave a description of a case of 'a peculiar form of obstructed inspiration,' in which examination by a laryngoscopic mirror previously had shown the aryteno-epiglottic folds to be close together and almost overlapping. Because the child had died of diphtheria, the larynx was examined post mortem. Lees claimed to be the first to describe the laryngoscopic appearances and post-mortem findings of such a case, and he stated that he had seen clinically a total of four cases with a similar condition. In the following year Gee (1884) described several instances of ' respiratory croaking of babies,' and made the observation that the disorder caused more annoyance to others than to the child. The description of ' infantile respiratory spasm' by John Thomson in 1892 helped to separate out a large group of cases of laryngeal stridor in young infants which ran a benign and self-limiting course. In 1897 Sutherland and Lambert Lack described eighteen cases of ' congenital laryngeal obstruction,' of which six had been examined by a laryngeal mirror. In all of these, they noted the epig!ottis sharply folded on itself, the two lateral folds being in close apposition. The aryteno-epiglottic folds were approximated, and thus the upper aperture of the larynx was reduced to a long narrow slit. The thin folds bounding the aperture 'flapped to and fro' on respiration. They were convinced that there was a valvular action of the upper aperture of the larynx, dependent partly on a peculiar congenital malformation in the anatomy of the larynx and partly on the flaccidity of these parts in infants. This is the view that is now widely held. In collaboration with Logan Turner, Thomson presented a comprehensive description of this group in 1900. They described the classical picture of the child who developed noisy breathing shortly after birth. The stridor comprised a croaking sound which accompanied inspiration. Expiration was accompanied by a short croak when the stridor was loud, but at other times it was noiseless. There was no distress and no cyanosis. There was marked inspiratory indrawing of the thoracic and abdominal walls, except in the mildest cases. During the first few months of life the stridor increased in loudness as the child got stronger, but after remaining about the same for a few more months, it gradually lessened and disappeared during the course of the second year. Structurally there was an exaggeration of the peculiarities of the infantile larynx. They postulated an ill-coordinated, spasmodic action of the muscles of respiration as the primary cause, and the soft collapsible nature of the larynx as playing only a secondary part in the condition. Theyconsidered that the peculiar laryngeal deformity was thus purely an acquired one as the result of a nervous imbalance. While this view has not now many followers, Thomson's (1892) original clinical description of the condition could hardly be improved upon. Reardon (1907) reviewed the already fast growing literature on the subject. Thereafter the increasing use of direct laryngoscopy led to more accurate diagnosis in cases of infantile laryngeal stridor. M'Kenzie (1925) described a case of severe congenital laryngeal stridor with dysphagia, aged three years, on whom a tracheotomy had to be performed, and he gave a critical review of the theories of the etiology of supra-glottic stridor. He reported observations made by Paterson using direct laryngoscopy. When Paterson held one side of the sucked-in margin of the laryngeal orifice with forceps, the noise stopped at once. Although Variot had, a generation previously, suggested cutting away the aryepiglottic folds, Iglauer (1922) was apparently the first to undertake partial epiglottidectomy in a case of congenital laryngeal stridor, and he advocated this procedure in all such cases subject to asphyxial attacks. Schwartz (1944) claimed to have taken the first coloured motion picture of the larynx during respiration in a case of congenital laryngeal stridor, and reproduced in his paper striking photographs from the film. He suggested the adoption of the term ' inspiratory laryngeal collapse,' and stressed the role of micrognathia as an etiological factor in these cases. He showed how the muscular and ligamentous forces concerned in the upper aperture of the larynx were affected by micrognathia. He