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This study reviews our experience from 1977 to 1991 with 32 children born with esophageal atresia and tracheoesophageal fistula (EA-TEF) who later underwent surgery for severe tracheomalacia. Indications for surgery included dying spells (21), inability to extubate the airway (nasotracheal tube 5, tracheotomy 3), and recurrent pneumonia (3). Diagnosis was confirmed by chest x-ray, esophagogram, and bronchoscopy. Aortopexy was performed in 31 of 32 children; a splint without aortopexy was used in one older girl. Splinting was also necessary in two of the 31 at the initial operation, when aortopexy failed to prevent tracheal collapse. There were four initial failures. Currently, 29 children are well (median follow-up, 6.6 years). Two have a tracheostomy in place, and one, who had a vascular ring divided after aortopexy, died at home of unknown cause 1 month later. These findings indicate that aortopexy provides long-term relief of severe symptoms of tracheomalacia associated with EA-TEF in almost all affected children. When aortopexy fails, the insertion of an airway splint may succeed; otherwise, tracheostomy is necessary.

An enteric valve to prevent colonic content reflux and to prolong small bowel transit time was created by passing small bowel through a submucosal tunnel in the colon. Barium enema, Gl tract series, and iso- and antiperistaltic pressure measurements showed the valve to be of competence equal to the ileo-cecal valve. Late pathological examination revealed a patent valve with minimal scar formation. The optimal submucosal tunnel length was between 4 and 6 cm. This valve can, theoretically, increase the absorptive capacity in short bowel syndrome and may serve as a substitute for the critically important ileo-cecal valve.

The long-term functional results of splinting a collapsing major airway with a silastic Marlex mesh prosthesis were assessed. Six patients in whom follow-up has been longer than 4 years (mean 5.3 years) were studied. The prosthetic semirigid splints had been implanted in five children with tracheomalacia and one with bronchomalacia. Mean age at the time of airway splinting was 4 years (range 6 months to 8 years). At their last clinical evaluation, all six children were leading normal active lives. Three had mild respiratory symptoms not related to the splinting. The only long-term complication was a serous effusion that developed around the splint and compressed the trachea in one child 2 years postoperatively. Tracheal fluoroscopy, barium swallow, and computed tomography scans of the trachea in five patients demonstrated satisfactory tracheal caliber without airway collapse during expiration and coughing. Pulmonary function testing showed a mild increase in airway resistance in one child who had had a tracheostomy. These results demonstrate that the application of composite synthetic graft to a segment of a malacic airway in young patients can provide long-term relief from airway collapse without compromising airway growth.

A newborn baby suffering from massive hemorrhage due to erosive gastritis, was treated successfully by devascularization of the stomach which is a rather simple procedure. Experience with adults as well as with this patient justifies the use of this operation when other measures fail to stop massive bleeding in hemorrhagic gastritis.

Familial dysautonomia (FD) is a rare incurable genetic disorder with multisystem involvement. Most of its clinical manifestations are related to disorders of the autonomic nervous system. The disease is associated with specific disturbances of the upper gastrointestinal tract: pharyngoesophageal dyskinesia, gastroesophageal reflux, and prolonged gastric emptying. About 40% of the dysautonomic children manifest repeat vomiting crises. In view of the extensive gastrointestinal symptomatology, children with FD are prone to repeated aspiration pneumonia and chronic respiratory failure, while inadequate calory and fluid intake may lead to a chronic state of hypovolemia and severe failure to thrive. Control of vomiting, prevention of aspiration due to abnormal swallowing, and the assurance of adequate calory intake are three major objectives in the treatment of the dysautonomic child. Medical treatment of the gastrointestinal disorders using different drugs has had limited success. This study reviews the surgical experience in ten children with FD. The type of the procedure used was determined by the severity of the upper GI disturbances. Nine children underwent gastroesophageal Nissen fundoplication and gastrostomy. In seven of them, a pyloroplasty was added. Gastrostomy alone was done in one patient only. Postoperative complications included transient dysphagia in four patients, gastric dilatation in four patients, and dumping syndrome in one. There has been no incidence of immediate postoperative death. One child died 6 months after operation from severe and irreversible respiratory failure. Following operation, the patients still suffered from dysautonomic crises but these were not associated with vomiting.(ABSTRACT TRUNCATED AT 250 WORDS)

An intraluminal tracheal stent (ITS) was used experimentally in rabbits and piglets, as well as clinically in infants with tracheal stenosis, to facilitate airway reconstruction. The ITSs were constructed of stainless-steel springs covered with silicone rubber. They were implanted in seven piglets (6 to 8 kg), five rabbits (3 to 5 kg) and three infants. No animals developed severe respiratory distress and all appeared to tolerate the ITS. Postmortem examinations 1 to 8 weeks after surgery showed (1) loss of stent fixation (one pig), (2) increased tracheal secretions, (3) pneumonia (one pig, two rabbits), and (4) focal squamous metaplasia of tracheal mucosa. Stents used to treat three infants (2 to 5 months of age) with complex tracheobronchial stenosis were placed at the time of periosteal tracheoplasty in two. Recurrent stenosis necessitated a second tracheoplasty and stenting in one, and a long tracheostomy tube and balloon dilatations in the other. The third child had endoscopic stent insertion to alleviate severe airway collapse after esophageal tracheoplasty. The child died from progressive respiratory failure after stent dislodgment. Although the stents were well tolerated in animals and they enhanced critical ventilation of all pulmonary lobes in infants after tracheal reconstruction, certain modifications such as alternative methods of fixation, accommodation for tracheal growth, and reduction in tissue reactivity are necessary before further use of the ITS can be advocated.

Twenty-two infants with congenital tracheal stenosis (CTS) were treated in our hospital between 1965 and 1987. Diagnosis was confirmed by endoscopic and radiographic methods. Patients had a spectrum of tracheobronchial lesions from localized stenosis to more complex deformities involving the carina and bronchi. Other anomalies were found in all patients with the occurrence of vascular slings or rings in 11 patients (50%). Six infants treated nonoperatively died from severe CTS and other lethal anomalies. Five of 16 patients (31%) treated surgically survived. Localized CTS in four cases was treated by dilatation, tracheostomy, or tracheal resection with primary anastomosis (two survivors, 2 non-survivors). Funnel-shaped deformities and extensive tracheobroncial stenosis were treated by tracheal reconstruction using a variety of autogenous tissue and prosthetic grafts (three survivors, nine non-survivors). The overall mortality was 77%. A new intratracheal stent was used in two patients. The stent was a flexible steel spring covered with a silicone rubber sheath. In one patient, it was placed within the trachea at the time of repair and removed later with the bronchoscope. A stent was used in a second patient with intermittent airway obstruction following an esophageal tracheoplasty. In this case, the device failed to alleviate the obstruction, and the infant expired from progressive respiratory failure. Issues of importance in the management of infants with CTS are: (1) adequate evaluation of the tracheobronchial tree, (2) awareness that tracheobronchography may precipitate further respiratory decompensation, (3) assessment of vascular and other anomalies requiring surgical correction, and (4) selection of an appropriate therapeutic approach.

A 5-mo-old male had disabling diarrhea and malabsorption following massive small bowel resection. His transit time was 10 min. After 9 mo of conservative treatment, a 24-cm isoperistaltic segment of colon was interposed 6.5 cm from the ligament of Treitz. Transit time was increased to 105 min, and he was eventually able to be maintained on an enteric diet without significant diarrhea. Colon interposition requires little manipulation of the remaining small intestine and does not rely on active intestinal obstruction to effect an increased transit time.