Search

The frequency of spinal and costal anomalies in infants with congenital esophageal atresia is demonstrated in 20 cases. Abnormal numbers of vertebral bodies in the thoracic and lumbar spine were found in 50% of the patients. The maximum number of thoraco-lumbar vertebrae was 19. Malformations of the vertebral bodies were frequent (35%). 13 pairs of ribs were found in 25% of the cases. Because these anomalies are easily recognized in radiographs they can provide additional hints in suspected esophageal atresia.

Summary Twelve patients with esophageal atresia have been managed by planned delay (4–13 wk) followed by direct esophageal anastomosis. During the period of delay, the infants were maintained on continuous proximal esophageal suction and gastrostomy feeding. The proximal esophageal pouch was elongated by daily bougienage in all infants and in one instance the distal pouch was also elongated with a bougie. Five infants had atresia without fistula (Type A), four with proximal fistula only (Type B), and three with an isolated distal fistula (Type C). All of these patients, including those with Type C atresia, had a wide gap between the esophageal segments. After the definitive surgery, eight patients developed anastomotic leaks sufficient to cause symptoms. These leaks were tolerated by all infants and eventually closed spontaneously. Nine patients developed anastomotic stenosis requiring dilatations. The anastomotic stricture was later resected in two of these patients, in each, 3 yr after the primary esophageal anastomosis. Presently, all 12 patients are living at home, able to eat and have normal activities. Five patients have no symptoms, four complain of occasional mild dysphagia with solid food when they eat too fast or take large bites of food and in three patients periodic esophageal dilatations are necessary to relieve dysphagia for solids. In a patient with esophageal atresia and a large gap between the esophageal segments, esophageal pouch lengthening during a period of planned delay makes anatomic reconstruction of the esophagus possible and esophageal replacement unnecessary. Our experience with this technique has been gratigying. The prerequisite for this elongation regimen is presence of the lower esophageal pouch, albeit short and small.

A technique is described whereby a fistula is created between the upper and lower esophageal segments by means of a silk suture.

Features of the syndromes of oculoauriculovertebral dysplasia (Goldenhar), congenital heart disease and abnormality of the upper limb (Holt-Oram), and congenital heart disease and facial palsy (Cayler) have jointly been present in a girl.The association of these syndromes in one patient and the common features that are present in various case reports in the literature suggest that these are not separate syndromes, but parts of one syndrome.

There has been a progressive improvement in the survival rate of underweight babies treated by a one-stage primary esophagoesophagostomy due to improved surgical technique, better understanding of the physiology of the small baby; better preoperative preparation, anesthesia, and postoperative, and nursing care; and the establishment of intensive care units. Only the most desperately ill babies and the very small babies with severe respiratory problems and/or a number of gross associated anomalies are managed in stages. The fact that the baby is small is not in itself sufficient reason to deprive him of the benefits of a one-stage primary repair of the anomaly.

Twelve subtotal replacements of the oesophagus by the colon in infants with oesophageal atresia were performed. The right colon was used and it was led through a retrosternal tunnel. This procedure was carried out when the child was at least 8 kg. in weight and was thriving and in good health. The literature on the subject is reviewed and the technique and results in this small series are presented. Although this is an extensive operation and complications are not uncommon, colonic substitution of the oesophagus can confidently be recommended in infancy.

N DECEMBER 1970, the Liverpool Regional Hospital Board made money available for the purchase of a pediatric gastroscope. This report is based on the clinical experience gained using the Olympus (BF 5B) fiberoptic bronchoscope as a pediatric gastroscope on 41 patients. At present there is no instrument specifically designed as a pediatric gastroscope. Various manufacturers who were approached stated that such an instrument might become available when the backlog of orders for instruments for the various endoscopic units had been completed. The need for pediatric gastroscopy and endoscopy is obvious. The indications for endoscopic examination are different in children than in adults. In most cases, the need is for examination only and biopsy is seldom necessary. Photography, both still and cine, is a desirable extra. The age range of children requiring investigation starts from the neonatal period. The size of the esophagus in a full-term neonate is 9-10 cm in length and 4-6 mm in diameter. The growth in size of the trachea in infants is well known and is related to the age rather than the weight of the child. To determine the size of the required endotracheal tube, anesthetists use a guide based on the age of the child. 1 No such formula is available for judging the size of instrument to be passed clown an infant's esophagus. A 10-mm rigid esophagoscope can be passed easily into the esophagus of a neonate. An adult 13-mm gastroscope can be passed easily in a 7-yr-old patient. Various instruments were inspected and the 5-mm bronchoscope (Olympus BF 5B) was selected with the specifications shown in Table 1. Three deficiencies soon became apparent: the inadequate working length of the instrument, especially in older children; the short depth of focus, preventing a sharp image of objects further than 45 mm away from the tip; and poor-quality photographs of certain areas. Cine photography was unsuccessful with the standard compact light source (CLS).

The purpose of this communication is to present two cases of congenital tracheo-esophageal fistula without esophageal atresia which have not been previously reported, and to discuss some roentgenologic considerations of this rare and interesting anomaly. The fistulous tract in each case was visualized roentgenoscopically and recorded on roentgenograms. In each instance the tract was repaired. In most reported cases, the fistulous tract has arisen from the anterior wall of the esophagus and extended to the posterior wall of the trachea; in one case, the communication was with the left main-stem bronchus. The esophageal and tracheal openings of the fistulous tracts have varied from the size of a pinhead to several millimeters in diameter. The incidence of this lesion among all types of tracheo-esophageal anomalies is reported as 3 to 10 per cent, but judging from the few articles dealing with fistula without atresia, would seem much less. The diagnosis is made more often at postmortem examination than durin...

The mortality of primary repair of esophageal atresia in infants without associated major anomalies who are mature by weight, has declined to acceptable levels. The same cannot be said of similar infants who are premature. The high mortality following primary repair in this group has led to the clinical trial of temporizing procedures designed to delay esophageal anastomosis, such as fistula ligation, fistula division and gastric division. A limited experience with these procedures has not produced results which suggest that they will necessarily represent an improvement over primary anastomosis. The extrapleural approach to esophageal anastomosis was associated with a higher survival rate than the transpleural approach in the group of premature infants under study. This difference was more impressive among infants weighing less than 2,000 gm. In this group almost all survivors were treated by an anastomosis performed extrapleurally. The results in a series of premature infants treated by the extrapleural approach would appear to be the logical standard against which the described innovations in management can be measured.

In a series of 40 patients with esophageal atresia and tracheoesophageal fistula, the incidence of musculoskeletal anomalies was 45% (18 patients). An extra mesodermal segment was found in 15 patients (13 paired thoracic vertebrae and 13 paired ribs in nine patients; six lumbar vertebrae in 6 patients). The mortality in grade A infants with an extra mesodermal segment was significantly higher than in those infants with a normal spine ( p > 0.01). It is suggested that segmentation or abnormal segmentation of the embryo may be important as a cause of esophageal atresia.

There appears to be no question about the advantages of endotracheal intubation in the administration of anaesthesia for the repair of oesophageal atresia and tracheo-oesophageal fistula. Controlled respirations provide ideal working conditions when every advantage is needed for working in a small space. Ventilation is maintained even though there may be severe pulmonary disease. Minimal anaesthesia is required. Laryngeal oedema cannot be held as a deterrent with all the advantages to be gained. Finally, the anaesthesiologist is in a position to aid in the early diagnosis.

Perforation of the posterior pharyngeal wall during or immediately after delivery produced symptoms of esophageal atresia and traumatic diverticulums of the pharynx in two newborn infants. Nasogastric tubes could not be passed probably because of cricopharyngeal spasm. Although traumatic pseudodiverticulums of the pharynx in newborn infants have not previously been described in the American literature, it is likely that the condition is not rare.

A survey of radiographs of 44 infants with tracheoesophageal anomalies revealed the presence of extra vertebrae in 75 per cent of the cases. Vertebral hypersegmentation was present in infants with esophageal atresia alone, esophageal atresia with tracheoesophageal fistula, and tracheoesophageal fistula without esophageal atresia. The presence of additional vertebrae indicates that an excessive number of somites developed during embryonic life. This finding lends support to the theories that tracheoesophageal anomalies result from a growth disturbance in early embryonic life, that different types of tracheoesophageal anomalies have a common pathogenetic mechanism, and that the causative insult predates actual separation of the trachea and esophagus. Vertebral hypersegmentation should serve as an important adjunct in the diagnosis of tracheoesophageal anomalies, particularly in those lesions that are difficult to demonstrate radiologically.

Summary The elemental diet Codelid 72-H has been used in 12 newborn surgical patients over the past 2 yr. The diet is easily prepared and administered and, when given in sufficient concentration, provides total caloric requirements. It allows early feeding in patients with short gut, minimizes gastroesophageal reflux, and permits feeding in the face of partial intestinal obstruction. The limitations of the diet include a low caloric value at low concentration and a high osmolality in concentrations isocaloric to milk. Prolonged feeding may result in deficiency states.

THE causative agents of atresia of the esophagus must be primarily genetic or else act during prenatal life, for the defect is present at birth. The evidence bearing on the subject has been brought together here for analysis, and is presented as a stimulus to further research on the problem of etiology. This report is based on a study of 107 infants with esophageal atresia (102 with associated tracheoesophageal fistula) seen at the Children's Hospital from January, 1936, through 1948 or born at the Boston Lying-in Hospital (6 cases) between 1934 and 1945, inclusive; 90 died during infancy and the . . .

Inability of a newborn infant to swallow secretions is usually due to esophageal atresia, but there are other, less common causes. Our patient suffered from a congenital diverticulum of the pharynx, demonstrated by radiography and at operation, and had an associated intestinal malrotation. She was successfully managed by a two-stage procedure with preliminary excision of the lesion from the chest and neck and later correction of the intestinal obstruction.

Summary Three cases of trisomy 17–18 associated with oesophageal atresia and tracheo-oesophageal fistula are described, and the clinical features of two other cases of trisomy 17–18 are summarised. It is concluded that the association between the two conditions is not so rare as previous reports suggest.

66 _ W HAT IS THE BEST way to do the anastomosis?” When this question is cast among surgeons who treat esophageal atresia, brisk dialogue always follows. The question is important because esophageal anastomotic complications-leak and stricture-are technical failures, possibly avoidable, that cause death or disability in a significant number of cases. Today the infant born with esophageal atresia and tracheoesophageal fistula should have at least a 60 percent chance to live a normal life. Associated anomalies cause death in about 25 percent of the patients.2 Respiratory problems, especially in premature infants, also contribute to mortality. When the anastomosis leaks, the death and morbidity rates are high. Strictures cripple, may require many dilatations or a secondary anastomosis, and carry a significant mortality. Usually the gap between the esophageal segments is short enough to permit a comfortable primary anastomosis. When this is possible, the surgeon’s method of anastomosis, accuracy of suturing, and gentleness with tissues determine how well the reconstructed esophagus will work. Imperfect technic increases the chance of anastomotic complication. There are three types of anastomoses used in esophageal atresia. In a survey of the members of the Surgical Section of the American Academy of Pediatrics,’ the single layer end-to-end anastomosis was most popular. It also had the highest incidence of leaks, the highest mortality from leaks and a substantial incidence of strictures. Unquestionably it is the easiest anastomosis to perform. The Haight telescopic anastomosisl was used almost as often as the single layer end-to-end. It had the lowest incidence of leaks, the lowest mortality from leaks, but the highest stricture rate. It is more difficult to perform. The two layer end-to-end anastomosis was least popular in the survey. It ranked between the single layer end-to-end and the Haight anastomoses in number of leaks and survival from leaks, and was comparable to the single lnver in the number of strictures. It is the most difficult to perform. Tables 1 and 2 are taken from the survey.” Potts suggested refinements of the two layer end-to-end anastomosis.” His technic was applied to 102 patients who had esophageal atresia with distal tracheoesophageal fistula, 75 from the Children’s Memorial Hospital, Chicago4 and 27 personallv treated bv the author. Four percent leaked; significant i stricture formation was 8 percent. A significant stricture is one that is symptomatic alld requires more dilatation than the routine passage of soft catheters performed in most cases.

Proximal pouch tracheoesophageal fistulas were discovered in 13 of 170 patients (7.7%) who had esophageal atresia and/or tracheoesophageal fistula. Approximately one-third of these were discovered preoperatively, one-third intraoperatively, and the remainder during the first 10 mo postoperatively. We feel that the incidence of this anomaly will be seen to be much higher than previously reported if surgeons specifically look for it.

Prolonged intubation of the trachea has a useful place in the intensive care of infants and children, complementary to the use of tracheostomy, but it carries some mortality and morbidity. During a two-year period there were 5 deaths directly associated with anaesthesia in 134 patients, compared with 7 deaths associated with tracheostomy in over 500 patients over a nine-year period at the same hospital. If tubes are left in situ for long periods subglottic oedema and stenosis may present an extremely serious problem. This may be reduced by careful selection of tube size, by the use of polyvinyl-chloride tubes instead of red rubber ones, and by the avoidance of trauma during intubation. The problem may be more common in cases of laryngotracheobronchitis. Tube obstruction has proved fatal when using small tubes and can occur at any time after intubation. It can only be avoided by meticulous nursing care and attention to humidity. It is not possible to state a maximum time during which tubes may be safely left in the trachea. Tracheostomy is preferred in this hospital if an artificial airway is required for longer than forty-eight to seventy-two hours.