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Biomedical subjects

E M Kiely

Publications and source records attributed to E M Kiely.

At least 19 recordsLinked to original sources

Delayed surgery for congenital diaphragmatic hernia.

Between January 1987 and December 1990, 67 neonates were treated for congenital diaphragmatic hernia, symptomatic within 6 h of birth. The mortality rate was 33 per cent. Preoperative stabilization was achieved in 47 patients, all of whom survived initial treatment, although two died later. Stabilization could not be achieved in 20 neonates, all of whom died within 3 days of birth, 18 without undergoing operation and two after early repair. Intensive resuscitation with controlled, delayed operation for congenital diaphragmatic hernia gives long-term results similar to those of urgent operative repair. This approach avoids operation in the majority of those who subsequently die.

Female

Abdominal inflammatory myofibroblastic tumours in children.

Inflammatory myofibroblastic tumours (inflammatory pseudotumours) occurring at intra-abdominal sites in children have rarely been described. This paper reports three patients with this tumour, two of whom presented with fever, anaemia and an abdominal mass, the third with chronic duodenal obstruction. All had experienced significant weight loss. At operation, each had a large fibrous tumour (7-18 cm in diameter) originating from the transverse mesocolon, small bowel mesentery and duodenum respectively. Intraoperative frozen section histological examination in one patient was misinterpreted as a sarcoma. All the lesions were judged to have been completely excised, but one was ruptured during operation and the patient subsequently developed recurrent tumour nodules. Abdominal inflammatory myofibroblastic tumours are rare. They may be suspected before operation but their clinical, radiological and pathological features may be confused with those of malignancy. Complete excision is necessary to avoid local recurrence.

Child

Anastomotic leakage following surgery for esophageal atresia.

Of 199 neonates undergoing primary or delayed primary repair of esophageal atresia, 34 (17%) developed anastomotic leakage, 7 of which (3.5%) were major anastomotic disruptions. Infants with major leaks developed signs within 5 days and all required early reoperation, necessitating abandonment of the esophagus in 6. The remaining 27 were minor leaks demonstrated by water-soluble contrast studies and were successfully treated nonoperatively. Gastroesophageal reflux was unassociated with this complication but the use of braided silk sutures was associated with a significantly increased risk of anastomotic leakage when compared with polyglycolic acid (relative risk, 3.2) or polypropylene (relative risk, 2.6) sutures. Following anastomotic leakage there was a significantly increased risk (relative risk, 2.04) of subsequent esophageal stricture formation.

Anastomosis, Surgical

Congenital esophageal stenosis due to tracheobronchial remnants: a rare but important association with esophageal atresia.

Congenital esophageal stenosis caused by tracheobronchial remnants occurred in eight children, six of whom had associated esophageal atresia and/or tracheoesophageal fistula. Symptoms usually began in early infancy but delayed diagnosis was a common feature. The mean lag period between presentation and definitive operation was 4.6 years (range, 1 month to 16 years). Errors in diagnosis were common. Six were initially diagnosed as having inflammatory strictures secondary to reflux esophagitis. Seven children were subjected to repeated esophagoscopy and bouginage of the "stricture" (mean no. = 3.4), with invariable failure to ameliorate dysphagia. Antireflux procedures were performed in three patients. In all children, symptoms were dramatically relieved following resection of the stenotic segment or esophageal replacement. Although a rare entity, congenital esophageal stenosis due to tracheobronchial remnants should be considered a possibility in patients with esophageal stricture, presumed to be inflammatory in nature, which fails to respond to standard therapy.

Adolescent

CHARGE and esophageal atresia.

CHARGE association was diagnosed in 61 infants, 20 of whom died, mainly during the first 2 years of life. Esophageal atresia and/or tracheoesophageal fistula were present in 10 neonates. Axial skeletal anomalies occurred in 7 of the 10, but none had preaxial limb defects typical of the VATER association. All had major cardiac anomalies, predominantly tetralogy of Fallot. The majority of patients had primary repair of the esophagus. The postoperative course was stormy in all patients, with a high incidence of complications and 70% died. The recognition of features of the CHARGE association is important because it has major prognostic and therapeutic implications.

Abnormalities, Multiple

Morbidity and mortality in 46 patients with the VACTERL association.

During a 10-year period, 313 infants were treated at the Hospital for Sick Children for esophageal atresia, of whom 46 had features of the VACTERL association. These 46 patients had a mortality rate of 24%, most deaths being caused by cardiovascular abnormalities. Infants weighing less than 2,050 g had a mortality rate of 26%, double that of heavier infants. During the first year of life there was considerable morbidity resulting from the tracheoesophageal abnormalities. Whenever possible primary repair of the esophagus should be performed; however, many patients with VACTERL association had an unusually long gap between the two ends of the esophagus, and repair under tension was associated with a high incidence of complications. Of the 35 survivors, 25 are in a satisfactory, stable condition, but 10 still require treatment necessitating repeated admission to hospital. Anorectal, renal or skeletal anomalies rarely caused death, but, when severe, were associated with a poor quality of life.

Abnormalities, Multiple

Primary anastomosis in apple-peel bowel syndrome.

During a 15-year period, 12 infants with apple-peel bowel were treated; the first 4 by initial enterostomy with delayed anastomosis and the remaining 8 by primary anastomosis. All four infants in the enterostomy group required parenteral nutrition, compared with only six of the eight who had a primary anastomosis. Seven of the eight infants treated by primary anastomosis were in hospital for less than 50 days, (however, the remaining child was an in-patient for over 2 years, but did suffer from short-bowel syndrome with only 17 cm of small intestine). In comparison, all four of the enterostomy group were hospitalised for between 70 and 175 days. All twelve patients survived.

Anastomosis, Surgical

Xiphoomphaloischiopagus tripus conjoined twins: successful separation following abdominal wall expansion.

A case of xiphoomphaloischiopagus tripus conjoined twins is reported. Particular emphasis was paid to the large abdominal wall defect that would be produced by separation, and the complications resulting from the use of intraperitoneal Silastic tissue expanders are described. As a result of adequate tissue expansion and the use of a vascularized pedicle graft from the fused limb, primary abdominal wall and pelvic closure was possible without complications of wound healing. The consistent finding of a shared lower urinary tract with one ureter from each infant entering each bladder should be anticipated in this type of twinning.

Abdominal Muscles

Gastric retention of swallowed coins after pyloromyotomy.

Swallowed coins are a frequent problem in paediatric practice. The majority that reach the stomach will pass unimpeded through the gastrointestinal tract. Prolonged gastric retention of a swallowed penny is reported in three children who had previously undergone pyloromyotomy for infantile hypertrophic pyloric stenosis. The coins were removed after laparotomy in two patients and endoscopically in the third. This abnormal retention may reflect a long-term disturbance of pyloric function after pyloromyotomy.

Child, Preschool

Preliminary report: the antegrade continence enema.

The principles of antegrade colonic washout and the Mitrofanoff non-refluxing catheterisable channel were combined to produce a continent catheterisable colonic stoma. The intention was that antegrade washouts delivered by this route would produce complete colonic emptying and thereby prevent soiling. The procedure has been successfully carried out in five patients with intractable faecal incontinence.

Adolescent

The functional sequelae of sacrococcygeal teratoma.

Twenty-seven patients with sacrococcygeal teratoma were followed for a mean period of 5 years (range, 2 to 12 years). Eleven (41%) had some form of functional impairment. Fecal and/or urinary incontinence was present in nine children and two had weakness of the lower limbs secondary to sciatic nerve palsy. Tumors with large intrapelvic extensions requiring an abdominoperineal approach for resection were associated with a higher incidence of functional sequelae (67%). It is recommended that patients with sacrococcygeal teratoma (particularly those with a presacral extension) be closely monitored for functional deficits so that early treatment can be instituted.

Fecal Incontinence

Anastomotic stricture following repair of esophageal atresia.

Anastomotic strictures developed in 74 (37.2%) of 199 patients undergoing primary or delayed primary repair of esophageal atresia with or without tracheoesophageal fistula. Significant predisposing factors included the use of braided silk sutures (relative risk 1.72 and 1.49, compared with polyglycolic acid and polypropylene sutures), the presence of gastroesophageal reflux and leakage of the anastomosis (relative risk 2.29 and 2.04, respectively). Tracheomalacia, personnel factors, and recurrent fistula did not affect the rate of stricture formation. Seventy-one patients responded to dilatation alone, whereas three required stricture resection or esophageal substitution. All three patients requiring surgical intervention and 14 requiring five or more dilatations developed symptoms within the first 6 months after esophageal anastomosis. Antireflux surgery was carried out in 19 (25.7%) of the 74 patients.

Anastomosis, Surgical

Recurrent tracheo-oesophageal fistula: experience with 24 patients.

Over an 11 year period, 275 infants with oesophageal atresia and tracheo-oesophageal fistula were treated. Of these, 22 (8%) developed a recurrent fistula. An additional two patients with an established recurrent fistula were referred for secondary surgery. Infants who develop respiratory symptoms associated with feeding following repair of an oesophageal atresia should be investigated urgently with cine-radiography and/or bronchoscopy. Once the diagnosis of a recurrent fistula has been firmly established, operative repair should be undertaken. In our series of 24 patients, there were three deaths (12.5%), four patients developed a second recurrence (16.6%), while a secondary oesphageal replacement was carried out on five patients.

Esophageal Atresia

Tracheo-oesophageal fistula and pre-operative mechanical ventilation.

Twelve patients with tracheo-oesophageal fistula (TOF) and restrictive lung disease necessitating pre-operative ventilation are reported. Eight patients had respiratory distress syndrome, four had aspiration pneumonia, and 11 had associated oesophageal atresia. Two patients in whom a preliminary gastrostomy was performed died. Emergency ligation of the fistula was performed in 10 patients, nine of whom survived. Following division of the fistula, respiratory function improved dramatically in three patients and primary oesophageal repair was performed. Three patients underwent delayed primary repair and the oesophagus was sacrificed, with a view to replacement at a later date, in the remaining three patients. We believe that the presence of a TOF in a neonate with poorly compliant lungs requiring mechanical ventilation represents a serious surgical challenge. Gastrostomy alone should never be performed. Ligation of the fistula with either immediate or delayed primary repair of the oesophagus are the treatments of choice.

Esophagus