David P. Drake

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).

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.

Effectiveness of fundoplication in early infancy

PURPOSE The aim of this study was to evaluate the results of fundoplication performed in the first 4 months of life. METHODS Case-notes of 66 patients less than 4 months old who underwent fundoplication between January 1986 and July 1997 were reviewed. The mean length of follow-up was 24 months (range, 3 to 115 months). Diagnosis of gastrooesophageal reflux (GOR) was based on a barium meal in 55 patients or 24-hour pH monitoring in 23 patients. RESULTS Fundoplication was performed at a median age of 9 weeks (range, 3 to 15 weeks). Associated anomalies were identified in 56 infants and included oesophageal atresia or tracheooesophageal fistula (n = 19), lung abnormalities (n = 7), congenital diaphragmatic hernia (n = 4), and various other complex syndromes including neurological disorders (n = 26). Isolated GOR occurred in 10. Ninety percent of infants with isolated GOR improved clinically after the fundoplication compared with 64% of those with associated anomalies. Only 58% of infants in whom GOR developed after oesophageal atresia repair improved after fundoplication. Vomiting was reduced after fundoplication in 76% of infants, apnoea and/or respiratory symptoms improved in 66%, whereas failure to thrive incidence improved in only 38% of infants. Revision of the fundoplication was required in 16 (24%) patients. There were eight deaths, all of which occurred at least 6 months after the fundoplication. CONCLUSIONS Fundoplication in early infancy is unsuccessful in a high proportion of patients. The highest incidence of failure and redo fundoplication occurred in infants with associated anomalies. The best results were achieved in infants with emesis and respiratory symptoms.

Surgical outcomes of esophageal atresia without fistula for 24 years at a single institution

PURPOSE The aim of the study was to evaluate the surgical outcome of esophageal atresia (EA) without fistula for 24 years at a single tertiary center for pediatric surgery. METHODS The study used a retrospective chart review of infants diagnosed with EA without fistula between 1981 and 2005. RESULTS Of 33 patients with EA without fistula, 31 charts were available. Mean birth weight was 2327 g (range, 905-3390 g), and 71% were male. Most common associated anomalies were cardiac (n = 6; 19%) and renal (n = 5; 16%), followed by vertebral (n = 4; 13%) and anorectal (n = 2; 7%). The median initial esophageal gap was 5 vertebral bodies. Six had a primary repair, and 25 patients had esophageal replacement at a median age of 7 months. This involved gastric transposition in 20 (1 followed failed jejunal interposition), colonic interposition in 5, jejunal interposition in 1 (after a failed colonic), and repair at another center in 1. With a median review of 9 years, 21 patients had long-term sequelae with the need for multiple further surgical procedures including an antireflux procedure in 5. One patient died. CONCLUSIONS Management of EA without fistula remains challenging. Most patients required staged treatment that included esophageal replacement. The frequency of late complications indicates the need for programmed long-term review.

Decreased cerebral oxygen saturation during thoracoscopic repair of congenital diaphragmatic hernia and esophageal atresia in infants

BACKGROUND/PURPOSE Congenital diaphragmatic hernia (CDH) and esophageal atresia with tracheoesophageal fistula (EA/TOF) can be repaired thoracoscopically, but this may cause hypercapnia, acidosis, and reduced cerebral oxygenation. We evaluated the effect of thoracoscopy in infants on cerebral oxygen saturation (cSO(2)), arterial blood gases, and carbon dioxide (CO(2)) absorption. METHODS Eight infants underwent thoracoscopy (6 CDH and 2 EA/TOF). Serial arterial blood gases were taken. Regional cSO(2) was measured using near-infrared spectroscopy. Absorption of insufflated CO(2) was calculated from exhaled (13)CO(2)/(12)CO(2) ratio measured by mass spectrometry. RESULTS CO(2) absorption increased during thoracoscopy with a maximum 29% ± 6% of exhaled CO(2) originating from the pneumothorax. Paco(2) increased from 9.4 ± 1.3 kPa at the start to 12.4 ± 1.0 intraoperatively and then decreased to 7.6 ± 1.2 kPa at end of operation. Arterial pH decreased from 7.19 ± 0.04 at the start to 7.05 ± 0.04 intraoperatively and then recovered to 7.28 ± 0.06 at end of operation. Cerebral hemoglobin oxygen saturation decreased from 87% ± 4% at the start to 75% ± 5% at end of operation. This had not recovered by 12 (74% ± 4%) or 24 hours (73% ± 3%) postoperatively. CONCLUSIONS This preliminary study suggests that thoracoscopic repair of CDH and EA/TOF may be associated with acidosis and decreased cSO(2). The effects of these phenomena on future brain development are unknown.

Thoracoscopic repair of congenital diaphragmatic hernia: intraoperative ventilation and recurrence

INTRODUCTION Thoracoscopic repair of congenital diaphragmatic hernia (CDH) has been described, but its efficacy and safety have not been validated. The aim was to compare our experience of thoracoscopy with laparotomy repair. METHODS After ethics approval, we reviewed the notes of neonates with CDH operated in our institution between 2003 and 2008. Two historical groups were compared: infants who underwent laparotomy (2003-2008) or thoracoscopy (2007-2008). Data were compared by t test or Mann-Whitney tests. RESULTS Thirty-five children had open repair of CDH, and 13 had thoracoscopic repair. Groups were homogeneous for age and weight. Five (38%) neonates who had thoracoscopy were converted to open for surgical difficulties (n = 4) and O(2) desaturation (n = 1). Patch repair was used in 12 (34%) open and 6 (46%) thoracoscopic repairs. End-tidal CO(2) was significantly elevated during thoracoscopy, but this was not reflected in arterial CO(2) or pH. There were 3 (8%) recurrences after open repair and 2 (25%) after thoracoscopy (P = .19). CONCLUSION Thoracoscopic repair of CDH is feasible. Arterial blood gases should be closely monitored. Despite higher EtCO(2), conversion to open was mainly because of difficult repair. A randomized trial is necessary to assess the effect of thoracoscopy on ventilation and recurrences.

Advanced necrotizing enterocolitis part 1: mortality.

AIM OF THE STUDY The aim of this study was to investigate the factors associated with mortality in infants referred for the surgical treatment of advanced necrotizing enterocolitis (NEC). METHODS Retrospective review of all infants with confirmed (Bell stage II or III) NEC treated in our unit during the past 8 years (January 2002 to December 2010). Data for survivors and nonsurvivors were compared using Mann-Whitney test and Fishers exact test and are reported as median (range). RESULTS Of the 205 infants with NEC, 35 (17%) were medically managed; 170 (83%) had surgery; 66 (32%) infants died; all had received surgery. Survivors and nonsurvivors were comparable for gestational age, birth weight, and gender distribution. Overall mortality was 32%, the highest mortality was in infants with pan-intestinal disease (86%) but remained significant in those with less severe disease (multifocal 39%; focal disease 21%). The commonest cause of mortality was multiple organ dysfunction syndrome and nearly half of the nonsurvivors had care withdrawn. CONCLUSION Despite improvement in neonatal care, overall mortality (32%) for advanced NEC has not changed in 10 years. Mortality is significant even with minimal bowel involvement.

Resection and primary anastomosis in necrotizing enterocolitis.

The standard technique for dealing with surgical necrotising enterocolitis (NEC), namely resection of gangrenous bowel with exteriorisation of the ends, was used in seven infants with disappointing results. Only one tolerated full oral feeds by 1 month. A change to a primary anastomosis after resection was made and performed on ten infants. Seven tolerated full oral feeds within 2 weeks. These children had less morbidity and a shorter hospital stay. This technique has definite short and long term advantages and is recommended as the definitive procedure for most surgical NEC cases.

The Management of Oesophageal Atresia in Neonates With Right-Sided Aortic Arch

BACKGROUND/PURPOSE The management of oesophageal atresia (OA) in association with a right-sided aortic arch (RAA) is challenging. It is controversial whether right or left thoracotomy is the most appropriate surgical approach. The aim of this study was to determine the prevalence of RAA in patients with OA and the most appropriate surgical approach. METHODS The case records of all the neonates with OA treated over an 18-year period (1980 through 1997) were reviewed. Patients with RAA were analysed with particular reference to the preoperative investigations, operative approach and findings, and postoperative complications. RESULTS Of the 476 case notes reviewed, 12 (2.5%) had a RAA, and 5 of these infants had major cardiac anomalies. The diagnosis was not suspected on preoperative chest x-ray in any of these infants. In only 1 of the 5 neonates who had a preoperative echocardiogram was RAA suspected. A right thoracic approach was performed in the 11 infants in whom a RAA was not suspected. Immediate conversion to a left thoracotomy with primary anastomosis was carried out in 2 infants. In 2 further infants, fistula ligation alone was performed because of a long gap atresia. Anastomosis via the right thoracotomy was performed in 7 infants. There were 3 anastomotic leaks, 1 intractable stricture, and 1 recurrent fistula. Three patients (25%) had a double aortic arch, and, as a result, a left thoracotomy had to be abandoned in 2. There were 3 deaths, 2 as a result of major cardiac anomalies and 1 related to prematurity and sepsis. CONCLUSIONS RAA occurs in 2.5% of infants with OA. Preoperative chest x-ray and echocardiogram are unreliable as diagnostic modalities. Anastomosis via a right thoracotomy is associated with a 42% leak rate. The presence of a double aortic arch can make the oesophageal anastomosis via a left thoracotomy as difficult as via a right thoracotomy.

Recurrent Necrotizing Enterocolitis

In the decade 1981 to 1991, 16 infants developed recurrent necrotizing enterocolitis (NEC). They comprised 12 (6%) of 196 neonates referred for further management of NEC and four others referred with major congenital anomalies. Their median gestational age was 32 weeks (range, 27 to 40), median birth weight was 1,260 g (range, 790 to 3,230), and the sex distribution was equal. Recurrent NEC occurred after a median interval of 37 days (range, 11 to 163) from the onset of the initial episode. All but one of the 16 infants were either premature (n = 10) or mature with major congenital anomalies (n = 5). Nine patients had previously undergone surgery for NEC. Medical treatment was successful in 11 patients with recurrent NEC. Two infants died, both of liver failure related to parenteral nutrition; one of whom had suffered four separate episodes of NEC and was found to have a superior mesenteric artery occlusion. There was no consistent association between recurrent NEC and the type or timing of enteral feeds or the anatomical site or method of management of the original attack. The mortality of 12.5% was similar to that for primary NEC.