Edward M. Kiely

Esophageal atresia: Five year experience with 148 cases

One hundred forty-eight infants with abnormalities of the esophagus treated over a 5-year period were reviewed: 87% comprised esophageal atresia with distal tracheoesophageal fistula. The survival rate for infants in risk group A was 100%, for risk group B, 86%, and for risk group C, 73%. There were six infants with associated anomalies incompatible with survival. Anastomotic leaks occurred in 21% of cases, strictures in 18% and recurrent tracheoesophageal fistula in 12%. The use of braided silk for the repair was associated with a high complication rate. There was a distinct advantage in delaying surgery pending improvement in aspiration pneumonia. Low birth weight was not considered a contraindication for primary repair. Emergency ligation of the fistula was considered a more appropriate procedure than gastrostomy for the infant with associated severe respiratory distress syndrome in whom mechanical ventilation was providing difficult. Congenital cardiac anomalies were the single most common cause of mortality and correction of these anomalies should be pursued aggressively. There were definite advantages of preoperative endoscopy, particularly in identifying proximal fistulae. Elective postoperative ventilatory support for infants with a very tense anastomosis was successful in preventing leaks in five infants. There were no advantages to routine gastrostomy. Severe gastroesophageal reflux and tracheomalacia demand aggressive management. Mortality was directly related to the severity of associated congenital anomalies.

Oesophageal atresia: At-risk groups for the 1990s

The authors analyzed the outcome for 357 infants with oesophageal atresia and 15 with H-type tracheoesophageal fistula treated from 1980 through 1992. Survival according to Waterston risk categories was 99% for group A, 95% for group B, and 71% for group C. Presently, with optimal management, virtually all infants in groups A and B should survive. When examining the risk factors in the infants who died, two criteria were found to be important predictors of outcome: birth weight of less than 1,500 g and the presence of major congenital cardiac disease. A new classification for predicting outcome in oesophageal atresia is proposed: group I: birth weight > or = 1,500 g, without major cardiac disease, survival 97% (283 of 293); group II: birth weight < 1,500 g, or major cardiac disease, survival 59% (41 of 70); and group III: birth weight < 1,500 g, and major cardiac disease, survival 22% (2 of 9).

The clinical application of the malone antegrade colonic enema

Continent, catheterizable enterostomies (Malone stomas) have been formed in 25 patients. A reversed appendicocecostomy was used for the majority, although experience has led to modifications of this procedure. Antegrade enemas are regularly administered via these stomas in the treatment of fecal incontinence and intractable constipation. There have been only two important surgical complications, and the technique has proved to be highly successful. Follow-up of the patients has shown that individual requirements can be met with variations in the administration of the enemas.

Peritoneal drainage or laparotomy for neonatal bowel perforation? A randomized controlled trial.

Objective:To determine whether primary peritoneal drainage improves survival and outcome of extremely low birth weight (ELBW) infants with intestinal perforation. Summary Background Data:Optimal surgical management of ELBW infants with intestinal perforation is unknown. Methods:An international multicenter randomized controlled trial was performed between 2002 and 2006. Inclusion criteria were birthweight ≤1000 g and pneumoperitoneum on x-ray (necrotizing enterocolitis or isolated perforation). Patients were randomized to peritoneal drain or laparotomy, minimizing differences in weight, gestation, ventilation, inotropes, platelets, country, and on-site surgical facilities. Patients randomized to drain were allowed to have a delayed laparotomy after at least 12 hours of no clinical improvement. Results:Sixty-nine patients were randomized (35 drain, 34 laparotomy); 1 subsequently withdrew consent. Six-month survival was 18/35 (51.4%) with a drain and 21/33 (63.6%) with laparotomy (P = 0.3; difference 12% 95% CI, −11, 34%). Cox regression analysis showed no significant difference between groups (hazard ratio for primary drain 1.6; P = 0.3; 95% CI, 0.7–3.4). Delayed laparotomy was performed in 26/35 (74%) patients after a median of 2.5 days (range, 0.4–21) and did not improve 6-month survival compared with primary laparotomy (relative risk of mortality 1.4; P = 0.4; 95% CI, 0.6–3.4). Drain was effective as a definitive treatment in only 4/35 (11%) surviving neonates, the rest either had a delayed laparotomy or died. Conclusions:Seventy-four percent of neonates treated with primary peritoneal drainage required delayed laparotomy. There were no significant differences in outcomes between the 2 randomization groups. Primary peritoneal drainage is ineffective as either a temporising measure or definitive treatment. If a drain is inserted, a timely “rescue” laparotomy should be considered. Trial registration number ISRCTN18282954; http://isrctn.org/

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.

The failure rate of surgery for gastro-oesophageal reflux

PURPOSE The aim of this study was to document the presenting symptomatology and radiological findings of failed fundoplication, to determine the risk factors involved in recurrent gastro-oesophageal reflux, and to assess the results of revisional surgery. METHODS Sixty-six patients requiring redo fundoplication during the past 15 years were studied. Sixty children had their initial fundoplication performed at our institution. RESULTS The median time from initial fundoplication to the diagnosis of failure was 1.5 years. The main presenting symptoms were severe retching (n = 33), recurrent vomiting and aspiration (n = 26) and intolerable gas bloat (n = 7). Severe dysphagia occurred in two children with tight Nissen fundoplications. Contrast radiographic studies demonstrated fundoplication failure and correlated with the operative findings in 85% of cases. Risk factors for failure comprised hypertonic cerebral palsy, severe learning difficulties, oesophageal atresia, CHARGE syndrome, and tracheomalacia. The cause for the fundoplication failure included herniation of the fundoplication into the posterior mediastinum (failed crural repair, n = 30), disruption of the wrap (n = 10), combination of herniation and disruption (n = 22), and a tight wrap (n = 4). After revisional surgery, 13 children had persisting symptoms, five of whom underwent a third antireflux procedure. CONCLUSIONS The predominant cause of fundoplication failure is herniation into the posterior mediastinum, which occurred most frequently in children with hypertonic cerebral palsy. This may be prevented by meticulous attention to accurate apposition of the crura at initial procedure. Redo fundoplication failed to resolve the preoperative symptoms in 20%, with five children requiring a third fundoplication.

Hypercapnia and Acidosis During Open and Thoracoscopic Repair of Congenital Diaphragmatic Hernia and Esophageal Atresia Results of a Pilot Randomized Controlled Trial

Objective: We aimed to evaluate the effect of thoracoscopy in neonates on intraoperative arterial blood gases, compared with open surgery. Background: Congenital diaphragmatic hernia (CDH) and esophageal atresia with tracheoesophageal fistula (EA/TEF) can be repaired thoracoscopically, but this may cause hypercapnia and acidosis, which are potentially harmful. Methods: This was a pilot randomized controlled trial. The target number of 20 neonates (weight > 1.6 kg) were randomized to either open (5 CDH, 5 EA/TEF) or thoracoscopic (5 CDH, 5 EA/TEF) repair. Arterial blood gases were measured every 30 minutes intraoperatively, and compared by multilevel modeling, presented as mean and difference (95% confidence interval) from these predictions. Results: Overall, the intraoperative PaCO2 was 61 mm Hg in open and 83 mm Hg [difference 22 mm Hg (2 to 42); P = 0.036] in thoracoscopy and the pH was 7.24 in open and 7.13 [difference −0.11 (−0.20 to −0.01); P = 0.025] in thoracoscopy. The duration of hypercapnia and acidosis was longer in thoracoscopy compared with that in open. For patients with CDH, thoracoscopy was associated with a significant increase in intraoperative hypercapnia [open 68 mm Hg; thoracoscopy 96 mm Hg; difference 28 mm Hg (8 to 48); P = 0.008] and severe acidosis [open 7.21; thoracoscopy 7.08; difference −0.13 (−0.24 to −0.02); P = 0.018]. No significant difference in PaCO2, pH, or PaO2 was observed in patients undergoing thoracoscopic repair of EA/TEF. Conclusions: This pilot randomized controlled trial shows that thoracoscopic repair of CDH is associated with prolonged and severe intraoperative hypercapnia and acidosis, compared with open surgery. These findings do not support the use of thoracoscopy with CO2 insufflation and conventional ventilation for the repair of CDH, calling into question the safety of this practice. The effect of thoracoscopy on blood gases during repair of EA/TEF in neonates requires further evaluation. (ClinicalTrials.gov Identifier: NCT01467245)

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.

Neonatal gastrointestinal perforation

During the 7-year period from 1980 to 1986, 56 neonates with gastrointestinal (GI) perforation were treated in the Hospital for Sick Children, London. The overall mortality rate was 30%. The highest mortality rate (60%) was associated with gastric perforation, which in this series occurred only in premature neonates. Colorectal perforation carried the lowest risk of mortality (17%). The mortality rate from small bowel perforation was 35%. Prematurity and low birth weight were significant factors increasing the mortality rate from neonatal intestinal perforation. The predominant cause of perforation in the small and large intestine was necrotizing enterocolitis and the most common site of perforation was the terminal ileum. Mechanical ventilation was thought to be the cause of the perforation in four of the five neonates with gastric perforation. Primary anastomosis is a definite option in neonates who are stable intraoperatively. The mortality rate and in-patient stay for neonates who had primary anastomosis were less than for those who had stomas.

Necrotizing enterocolitis: Extent of disease and surgical treatment

PURPOSE The aim of this study was to evaluate the results of surgical treatment of necrotizing enterocolitis (NEC) according to the extent of disease and to establish if resection of the ileocecal valve represents a poor prognostic factor. METHODS The authors reviewed all cases of NEC (n = 161) treated in our hospital during the last 11 years; of these, 83 required surgical intervention. Definitions used by the authors include isolated, disease in a single intestinal segment; multifocal, disease in two or more intestinal segments; and pan-intestinal, majority of small and large bowel involved. RESULTS Twenty-five neonates had isolated NEC, 46 neonates had multifocal NEC, and 12 had pan-intestinal involvement. Survival rate was affected by the extent of intestinal involvement, the lowest survival rate (33%) being noticed in neonates with pan-intestinal involvement. In patients with isolated NEC, postoperative complications and survival rate were not affected by the modality of operative treatment. Conversely, in patients with multifocal NEC, survival rate was higher (85%) after resection and primary anastomosis compared with enterostomy (50%; P =.03). Resection of the ileocecal valve was not associated with increased morbidity and mortality. CONCLUSIONS (1) Resection and primary anastomosis is a valid treatment option in both isolated and multifocal NEC. (2) Neonates with NEC adapt rapidly to the loss of the ileocecal valve.