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Biomedical subjects

J W Thompson

Publications and source records attributed to J W Thompson.

169 records · Page 10Linked to original sources

Variants of Möbius' syndrome and central neurologic impairment. Lindeman procedure in children.

Möbius' syndrome is a complex neurologic disorder; its etiology and characterization have eluded clinicians for over a century. The pure Möbius' syndrome is rare, and the involvement of the sixth and seventh nerves has been found to have a host of associated defects. These include limb and shoulder abnormalities; multiple cranial nerve defects; occasional mental retardation; external, middle, and inner ear abnormalities; laryngeal neurologic defects that cause stridor, obstruction, and aspiration; and severe dysphagia and associated aspiration with life-threatening pneumonias. The children with severe neurologic dysfunction from central nuclear deficiencies were in many respects so similar in their clinical course that they are included in this paper. All of the patients required a tracheotomy to support the airway and to permit tracheobronchial toilet. A feeding gastrostomy was necessary in all but one patient. To counter life-threatening aspiration in the patients with multiple CNS defects, the Lindeman laryngeal diversion was considered the procedure of choice and proved very effective. The details and problems of this procedure are discussed. The aural defects and laryngeal findings in this series of patients are described in detail.

Abducens Nerve↗

Non-Hodgkin's lymphoma of the larynx in children.

Non-Hodgkin's lymphomas (lymphosarcomas) of the larynx are rare occurrences in children. This paper reports a child 9 years 3 months of age with isolated non-Hodgkin's lymphoma of the larynx, and gives a 15-year follow-up report of a previously published case of a child 4 years 7 months of age. Both children presented with the chief complaint of progressive airway obstruction, and both cases were misdiagnosed, as chronic allergic epiglottitis in the first patient, and nonresponsive croup in the second patient. Once we established a histologic diagnosis and excluded disseminated disease, we instituted treatment with local irradiation and systemic multiagent chemotherapy. Complete remission was achieved, and the two patients have been free of disease since their treatments were started 2 years and 9 years ago, respectively.

Antineoplastic Combined Chemotherapy Protocols↗

Use of botulinum toxin to lateralize true vocal cords: a biochemical method to relieve bilateral abductor vocal cord paralysis.

Using the mongrel dog as an animal model, we studied the effectiveness of botulinum toxin (Oculinum) to lateralize the true vocal cord. This experiment was undertaken in order to determine whether the toxin can improve the airway in subjects with bilateral abductor vocal cord paralysis. The toxin was injected into the cricothyroid muscle to block neuromuscular transmission at the motor end-plate. Paralysis of the cricothyroid muscle was achieved and documented by electromyography and videotaped endoscopy. Paralysis of the cricothyroid muscle decreases the tension of the true vocal cord and allows the cord to take a more lateral position. In this preliminary report, the literature is reviewed, the effect of and action of the toxin are discussed, and the results of the experimental protocol, establishment of dose-response curves, and techniques of injection are presented. Preliminary data suggest that there is an increase in the airway by lateralizing the true vocal cord with this biologic substance, and that this method may have many applications in clinical medicine for the otolaryngologist-head and neck surgeon.

Animals↗

Congenital stenosis of the lower esophagus associated with leiomyoma and leiomyosarcoma of the gastrointestinal tract.

The purpose of this paper is to report our analysis of four generations of a family with congenital strictures of the lower esophagus associated with leiomyoma of the gastrointestinal tract. Two members of the family died of sarcomatous degeneration of the leiomyoma and one is still alive 2 years after resection of the malignancy. Three family members had surgical repair of the congenital stricture. Surgical repair was unnecessary in one, for whom repeated dilatations alleviated the symptoms. Since there were no direct female descendants in this family, it is unknown whether this hereditary disease is carried by a sex-linked dominant gene. There are no reports in the literature of a familial tendency to congenital strictures of the esophagus, nor are there any reports of strictures in association with gastrointestinal leiomyoma and the later development of sarcomatous degeneration. The literature is reviewed, syndromes of leiomyoma of the esophagus and gastrointestinal tract are detailed, the particulars of the family tree and the patients are described, and the study is summarized.

Dilatation↗

Association of Down syndrome and segmental tracheal stenosis with ring tracheal cartilages: a review of nine cases.

Four patients with Down syndrome and midtracheal stenosis, three with proven absence of the midtracheal pars membranacea ("hourglass trachea"), are reported. Five previously reported patients who had Down syndrome and tracheal stenosis of this type are summarized. Respiratory difficulty and stridor were the reported clinical features of all but one of the patients whose clinical story is available. That approximately half the patients with tracheal stenosis with hourglass trachea and midtracheal absence of the tracheal pars membranacea reported had Down syndrome suggests that the association of this pattern of congenital tracheal stenosis with Down syndrome is, although infrequent, significant.

Abnormalities, Multiple↗