Recurrence of spondylothoracic dysplasia (Jarcho-Levin syndrome) in a family.

We describe a rare occurrence of two consecutive cases of spondylothoracic dysplasia (Jarcho-Levin syndrome) in a nonconsanguineous Hispanic family. Serial measurements of pulmonary function and energy expenditure were useful in one of these infants for assessment of the evolution and severity of restriction of pulmonary function and the determination of timely therapeutic intervention.

Mycobacterial cervical adenitis in children: medical and surgical management.

This is a review of the treatment results of cervicofacial mycobacterial adenitis in 85 children and adolescents. Twenty-three patients were treated with only anti-tuberculous medications (Group A). Thirteen underwent surgical procedures at the time of presentation for drainage of abscess or diagnostic biopsy, followed by treatment with anti-tuberculous medications (Group B). Forty-nine were initially treated with anti-tuberculous medications, with or without needle aspirations, and subsequently required surgery for one of the following reasons: 1) drainage and/or excision of abscess (Group C); 2) diagnostic biopsy (Group D); and 3) excision of persistent, enlarging, or recurrent nodes, or of draining fistulas (Group E). Four patients who were initially treated with surgery required a second procedure for persistent or recurrent disease. The cure rate for patients treated only with medications (Group A) was 95%. The cure rate for patients in the surgical groups after the initial procedures were 50% for Group B, and 100% for Groups C, D, and E. The overall cure rate was 92% for surgical treatment groups B-E and 93% for all 5 treatment groups. The clinical features of the disease, treatment outcome, and guidelines for management are discussed.

Tonsil and adenoid surgery for airway obstruction: perioperative respiratory morbidity.

A series of 160 consecutive patients undergoing tonsil and adenoid surgery for upper airway obstruction is reported. The ages ranged from 8 months to 13 years. Sixty-seven percent were 2, 3, or 4 years of age. All were routinely admitted overnight postoperatively. Forty-five (28%) remained in the hospital longer than one night (2 to 20 days). Postoperative respiratory problems were the reason for prolonged hospital stay in 30 of these 45 patients. Preoperative "danger-signals" of potential postoperative respiratory problems were: a history of severe obstructive symptoms with apnea and moderate or strongly positive sleep study, daytime somnolence, need for urgent T&A, and cardiomegaly. Risk factors present in a smaller number of patients were obesity, congenital stenosis of airways, and bronchopulmonary dysplasia. We suggest that children with these danger signals not be considered as candidates for outpatient T&A surgery.

Familial vocal cord dysfunction associated with digital anomalies.

Familial vocal cord dysfunction is a rare condition that has been reported in only a few instances. This is a report of identical male twins, both of whom had congenital bilateral abductor vocal cord paresis associated with finger deformities. The vocal cord paresis progressed to paralysis that required tracheotomy, then returned to a slowly resolving paresis during which the vocal cords had uncoordinated motion generally known as synkinesis. Another male sibling and the mother had a history of stridor during infancy and finger deformities. Several other relatives had digital abnormalities, and an infant first cousin with finger abnormalities required a tracheotomy for vocal cord paralysis.

Pediatric hypertrophic gastropathy.

Four previously healthy children presented in a 6-week period with marked hypoproteinemia without liver disease, malnutrition, or significant proteinuria. They all had strikingly similar radiographic findings consisting of enlarged folds confined to the fundus and body of the stomach. Three of the children had prodromal symptoms suggesting a viral illness. Cytomegalovirus was cultured from the urine in all cases and from the gastric biopsy specimens in three patients. Two of these patients also showed intranuclear inclusions in their biopsy specimens compatible with cytomegalovirus. It is not certain if cytomegalovirus was the cause of the illness.

Tracheotomy in infants and young children: the changing perspective 1970-1985.

One hundred fifty-three children 3 years of age or younger who had tracheotomies performed during the past 15 years are reviewed. During this time, short-term endotracheal intubation for airway obstruction from acute infections and long-term intubation for patients on ventilators have replaced early tracheotomy for these conditions. The number of tracheotomies decreased during each of three 5-year periods, from 73 to 55 to 25, respectively. Improvements in medical management resulted in prolonged survival of children with multiple abnormalities and resulted in more prolonged tracheotomies. Early complications occurred in 12% of patients and late complications occurred in 26%. In spite of changes in the indications, basic fundamentals of pediatric tracheotomy management remain unchanged.

Corticosteroid management of airway hemangiomas: long-term follow-up.

Nine children with airway obstructing hemangiomas were managed with corticosteroid therapy for durations of 6 weeks to 17 months. One patient required a tracheotomy. Steroid therapy improved the airways of the other 8 patients. Therapy was continued for 8 months or longer in 6 patients, none of whom developed life-threatening infections. Examinations 1 1/2 to 7 1/2 years after therapy revealed all of the patients to be within normal range for height and weight, but 3 of the 6 patients treated for 8 months or longer were in the lower range of normal. Steroid therapy is an effective method of protecting the airway in some patients with hemangiomas. Its disadvantage is that therapy may be necessary for several months. The risk of infections and growth suppression is minimized by using small maintenance dosage and alternate day therapy.