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Biomedical subjects

S W Beasley

Publications and source records attributed to S W Beasley.

At least 19 recordsLinked to original sources

Management of the premature infant with esophageal atresia and hyaline membrane disease.

Prematurity of 34 weeks' gestation or less occurs in 12% of infants born with esophageal atresia and distal tracheoesophageal fistula, and is likely to result in moderate or severe hyaline membrane disease. In the past, these infants have often had their surgical treatment delayed or staged. Based on our experience of 24 such patients since 1977, we propose that optimal treatment involves early division of the distal tracheoesophageal fistula via a thoracotomy and simultaneous repair of the esophagus where the patient's condition permits. This should be in the first 12 hours of life, before the hyaline membrane disease becomes fully established. Gastrostomy is not required and may be contraindicated.

Contraindications

Intussusception: prediction of outcome of gas enema.

Gas has replaced barium as the preferred medium for enema reduction of intussusception in tertiary pediatric institutions. Previously, adverse clinical features that in combination predicted a low likelihood of successful reduction using barium had been identified and used to select patients suitable for attempted enema reduction. This study examines whether these adverse features are equally applicable to gas reduction of intussusception. For any given number of adverse features, gas enema reduction was found more likely to be successful than barium reduction and, in the absence of clinical evidence of peritonitis, multiple adverse features probably do not represent a contraindication to attempted gas enema reduction.

Child, Preschool

Familial occurrence of complete agenesis of the diaphragm.

The tenth occurrence of complete unilateral agenesis of the diaphragm (McKusick 22240) in siblings is reported in this study. It is likely that parents of children with this type of congenital diaphragmatic hernia have a significantly increased recurrence risk for future offspring. Genetic counselling and antenatal ultrasound should be considered for subsequent pregnancies in families with a previous child with complete agenesis of the diaphragm.

Abnormalities, Multiple

Influence of anatomy and physiology on the management of oesophageal atresia.

Anatomical and physiological considerations specific to newborn infants with congenital thoracic abnormalities directly affect the timing and nature of surgical intervention during the neonatal period. This paper uses oesophageal atresia as an example of a common neonatal thoracic condition to highlight the way in which these considerations have influenced the approach to their surgical management. The type of surgical approach employed during thoracotomy determines the likelihood of subsequent chest wall deformity: an intercostal approach is preferable to rib resection. Multiple thoracotomies adversely affect the chest wall appearance and lung function. In premature infants with respiratory distress syndrome early surgical closure of the tracheo-oesophageal fistula is advantageous, and gastrostomy alone often prolongs the ventilatory difficulties. The upper oesophagus can be extensively mobilized with little danger to its blood supply, whereas the lower oesophagus, because it receives a segmental supply, is more vulnerable to ischaemia. The severity and distribution of tracheomalacia is reflected in its symptomatology. Tracheomalacia often coexists with gastro-oesophageal reflux, which should be corrected by a fundoplication if respiratory symptoms persist or an oesophageal stricture develops. The numerous factors producing heat loss in the newborn during thoracotomy are discussed. It is clear that an understanding of the anatomy and physiological changes which occur in the neonate is required if these infants are to be treated effectively and safely.

Esophageal Atresia

Long-term continence in patients with high and intermediate anorectal anomalies treated by sacroperineal (Stephens) rectoplasty.

Thirty-seven patients treated at the Royal Children's Hospital, Melbourne, by Stephens (sacroperineal or sacroabdominoperineal) rectoplasty were reviewed after 5 to 32 years. Continence was assessed using the Kelly, Templeton, Kiesewetter, and Wingspread criteria. Although approximately 20% of the patients were shown to have unsatisfactory control with each clinical scoring system, the remainder have a socially acceptable level of fecal control. Several patient groups had more favorable results than others: (1) females; (2) patients who did not have an abdominal component to their rectoplasty; and (3) patients reviewed more than 10 years after surgery. Twenty-eight patients reached their final level of continence in the first few years and nine improved gradually throughout childhood.

Anal Canal

The leadpoint in intussusception.

Fifty-six patients with a pathological lesion at the leadpoint of their intussusception were encountered at the Royal Children's Hospital, Melbourne, over a 16-year period. The most common lesions were Meckel's diverticula, followed by small bowel polyps, lymphosarcomas, and duplication cysts. Age at presentation was the only factor discriminating these patients from patients with idiopathic intussusception. Unlike previous studies, duration of symptoms was not useful in identifying a pathological leadpoint. Hydrostatic reduction was successful in reducing the leadpoint in three of 21 attempts. Neither morbidity nor mortality was encountered in a policy of initial enema reduction of intussusception, regardless of age.

Adolescent

Progression of intussusception.

The position of the apex in intussusception indirectly reflects the length of the intussusceptum in the majority of cases in which the intussusception begins in the region of the terminal ileum. The relationship of the apex to the duration of symptoms and the ease of enema reduction suggests that progression of intussusception occurs rapidly after the onset of symptoms and is normally complete by the time the patient presents to hospital. Further increase in length of the intussusception is probably limited by progressive edema of the intussusceptum and by the length of small bowel mesentery available to it. The position of the apex per se is not a contraindication to attempted enema reduction, although once the apex has reached the rectosigmoid region successful enema reduction is less likely.

Barium Sulfate

Secondary esophageal surgery following repair of esophageal atresia with distal tracheoesophageal fistula.

During the period 1948 through 1988, 498 patients with esophageal atresia and distal tracheoesophageal fistula were admitted to the Royal Children's Hospital, Melbourne. Fifty patients had a second operative procedure on the esophagus, for anastomotic stricture (30), recurrent fistula (15), both (4), and a postmyotomy diverticulum (1). During the same period, nine patients underwent esophageal replacement and 33 patients a Nissen fundoplication. Improvements in the technique of esophageal anastomosis, and in recent years the use of fundoplication to correct gastroesophageal reflux have led to a marked reduction in the need for secondary surgery to the esophagus after repair of esophageal atresia. Esophageal replacement is rarely required in esophageal atresia and distal tracheoesophageal fistula. One-layer end-to-end esophageal anastomosis using interrupted sutures resulted in the lowest rate of recurrent fistula and anastomotic stricture.

Anastomosis, Surgical

Tracheostomy.

The records of all children who had a tracheostomy performed over a 10 year period from January 1979 were reviewed. Altogether 142 patients aged 1 day to 24.8 years received a tracheostomy, 70 in the first year of life. The conditions necessitating tracheostomy were trauma (n = 21), acquired subglottic stenosis (n = 21), subglottic haemangioma (n = 16), Guillain-Barré syndrome (n = 14), Pierre Robin syndrome (n = 9), craniofacial disorders (n = 9), micrognathia (n = 5), and others in 47. In patients successfully decannulated the median period of tracheostomy was 104 days (range 3 days to 9.0 years) and in 25 patients the tracheostomy is still in situ. Eighty four patients (60%) were discharged from hospital with their tracheostomy in situ and no patient was kept in hospital because of a tracheostomy beyond four weeks. There were two tracheostomy related deaths in hospital. Both patients had severe acquired subglottic stenosis secondary to ventilation for lung disease of prematurity. There were no tracheostomy related deaths at home; complications included tracheal granulomas and polyps (n = 19). After removal of the tracheostomy 13 children had a fistula requiring surgical closure and four required revision of the tracheostomy scar. Tracheostomy is well tolerated in small children, with few complications and can be managed safely in the home by the family.

Adolescent

Growth after gut resection for Crohn's disease.

Seventeen children underwent 19 bowel resections as part of their management of Crohn's disease. Thirteen children had evidence of retardation of linear growth preoperatively, of whom 12 exhibited catch up growth, crossing at least one centile band, after resection. Seven of the 12 showed early signs of puberty at the time of resection and one child was regarded as being fully pubertal. All children were symptomatic before their surgery; at one year 11 were asymptomatic and three others were substantially improved. Surgical resection of localised Crohn's disease, followed by adequate nutritional support has lead to catch up growth and a prolonged symptom free period in most of our patients and this was not limited by their age or stage of puberty.

Adolescent

Developments in the management of oesophageal atresia and tracheo-oesophageal fistulas.

Since 1948, when the first patient with oesophageal atresia and a tracheo-oesophageal fistula was treated successfully at the Royal Children's Hospital, Melbourne, 569 infants with one or both conditions have been managed at that institution. The mortality rate in those in whom surgical repair of the oesophageal atresia and distal tracheo-oesophageal fistula was attempted has declined from 55% in the first 10 years to less than 1% in the last 10 years of the series. Earlier diagnosis and improvements in resuscitation, transport, neonatal intensive care, anaesthesia, the treatment of associated anomalies and surgical technique all are likely to have contributed to the decline in the mortality and morbidity rates. Problems which remain unresolved in part relate to the aetiology and embryogenesis of oesophageal atresia, the management of long-gap atresia, and the treatment of gastro-oesophageal reflux and tracheomalacia. This article outlines the improvements in management which have occurred already and speculates about what the future may hold.

Esophageal Atresia

Pulmonary sequestration and related congenital disorders. A clinico-radiological review of 41 cases.

Forty-one children with pulmonary sequestration complex were reviewed. This disease is a spectrum of disorders involving the bronchopulmonary airway, the arterial supply to the lungs, the lung parenchyma and its venous drainage. The review showed pulmonary sequestration to be ubiquitous in its presentation: "classical" features of sequestration occurred in 20 (49%) children studied. The early diagnosis of children with sequestration outside infancy demands a high index of suspicion by the radiologist. The role of angiography in demonstrating an anomalous systemic arterial supply to the lung in suspected bronchial and pulmonary arterial sequestration is described in relation to alternative imaging techniques available.

Abnormalities, Multiple

Chest wall deformity in patients with repaired esophageal atresia.

Chest wall deformities developed after thoracotomy for esophageal atresia, in 77 of 232 patients (33%) who did not have a congenital vertebral anomaly. Anterior chest wall asymmetry was present in 47, scoliosis in 18 and a combination of both in 12 patients. Scoliosis was convex away from the incision in two thirds of those affected. Anterior chest wall deformity was more common in patients greater than 25 years of age, and scoliosis was more common in patients who had had multiple thoracotomies. Breast surgery to minimize inequality was required in three female patients, and spinal surgery in one patient. Twenty-two of 53 patients with a congenital vertebral anomaly developed scoliosis, eight of whom required surgery. The scoliosis was probably the result of the vertebral anomaly in these patients, who are particularly at risk for progressive deformity.

Adolescent

The relationship between cerebral palsy and cryptorchidism.

This study examined the reported association between cerebral palsy and cryptorchidism. A comparison was made among 25 boys with cerebral palsy under the age of 2 years and 6 months, 25 boys with cerebral palsy aged between 5 and 10 years, and age-matched controls. The testes remained in the same position with age in boys with cerebral palsy, whereas in normal children the testes were slightly lower initially (P less than .005) and became lower still with growth (P less than .001). This result, taken in conjunction with previous studies, casts doubts on the theories of early damage to the hypothalamic-pituitary-gonadal axis as the cause of maldescent in cerebral palsy. It is postulated that any apparent increase in cryptorchidism in older patients with cerebral palsy may be caused by spasticity of the cremaster muscle leading to pathologic retraction of the testis out of the scrotum.

Age Factors