Oesophageal atresia is correctable and survivable in infants less than 1 kg

Pediatr Surg Int (2016) 32:571–576 DOI 10.1007/s00383-015-3851-4 ORIGINAL ARTICLE Oesophageal atresia is correctable and survivable in infants less than 1 kg Edward J. Hannon1,2 • Jennifer Billington1 • Edward M. Kiely1 • Agostino Pierro1 • Lewis Spitz1,2 • Kate Cross1 • Joseph I. Curry1 • Paolo De Coppi1,2 Accepted: 24 November 2015 / Published online: 18 April 2016 Ó The Author(s) 2016. This article is published with open access at Springerlink.com Abstract Introduction Management of oesophageal atresia (OA) and trachea-oesophageal fistula (TOF) in babies of low birth weight is challenging especially when associated with other anomalies. Birth weight of \1500 g has previously formed part of a classification system designed to predict outcome, alongside the cardiac status of the patient. Improvements in neonatal care have led to increasing numbers of premature low birth weight infants surviving. The aim of this study was to look at the experience of our institution in the extremely low birth weight (ELBW) patients. Methods A retrospective review of our institutions OA database was performed from 1993 to June 2015. Patients of birth weight less than 1000 g were included. A review of our OA/TOF clinical database and notes review established the following; gestation, birth weight, associated anomalies, operative procedures, morbidity and mortality. Results Of 349 patients with OA across the 22-year period, 9 ELBW patients were identified (\1000 g). Six males and three females. Gestational age ranged from 23 to 34 weeks and median birth weight was 815 g ranging from 630 to 950 g. Overall survival was 56 % (5/9). There were double the numbers of ELBW OA/TOF patients seen in the second half of the study period & Paolo De Coppi 1 Department of Paediatric and Neonatal Surgery, Great Ormond Street Hospital, London, UK 2 Stem Cells and Regenerative Medicine Section, Developmental Biology and Cancer Programme, UCL Institute of Child Health, 30 Guilford Street, London WC1N 1EH, UK presumably the result of improving neonatal care. Seven patients had type C OA with TOF and underwent emergency TOF ligation, two had concomitant oesophageal repair. One of these patients died from NEC; the other survived. Of the five who had isolated TOF ligation three died—two from cardiac disease and one from prematurity. Both type A patients survived and after initial gastrostomy placement one had a primary delayed repair, the other a gastric transposition. All three babies under 800 g died—one from cardiac disease the others from conditions indicative of their prematurity—necrotising enterocolitis and intraventricular haemorrhage. Conclusions 50 % survival is achievable in OA/TOF under 1 kg and the Spitz classification is still applicable in this group as a whole. However, none of the current classification systems are applicable in infants \800 g who in our study all had poor outcomes. We suggest these should be considered as separate group when predicting outcomes. Keywords Oesophageal atresia
Trachea-oesophageal fistula
Extremely low birth weight Introduction Oesophageal atresia (OA) with or without tracheoesophageal fistula (TOF) occurs in 1 in 3000–4500 live births and survival of these patients has improved over the last 30 years [1]. The principle of surgery for these cases remains the early repair of any TOF and primary or delayed repair of the oesophagus. The only significant change to this surgical management over the last 10 years has been the advent of the minimally invasive approach. However, improvements in pre and postoperative neonatal care and anaesthetic care, especially of low birth weight and 123 572 premature babies have led to improved outcomes in all neonates including those with OA/TOF [2–4]. In attempts to predict survival, Spitz classified OA/TOF patients into three groups according to birth weight ([1500 g) and the presence of major cardiac defects [5]. This classification has been broadly adopted when reporting OA/TOF outcomes and has changed practice in some centres which advocate early TOF ligation alone in babies less than 1500 g with delayed OA repair in attempts to minimise early morbidity and mortality [6]. More recent attempts to reclassify outcome in light of improving neonatal care have suggested different weight limits than Spitz [7] or scoring systems which take into account other associated anomalies [8]. However, none of these studies have specifically focussed on the very low birth weight population. With the improvement in neonatal care we have seen increasing numbers of very low (VLBW) and extremely low birth weight (ELBW) babies in our centre. We therefore wanted to review our experience of managing this ‘high-risk’ group of patients, focussing on operative strategies, co-morbidities, classification and survival. Methods A retrospective review of our departmental OA/TOF patient database (1993–June 2015) was performed in order to identify all ELBW patients (defined as birth weight \1000 g). This database was created from hospital diagnostic coding data, cross checked with neonatal discharge summaries and surgeon log books to minimise error. This would therefore include any cases which died before surgical intervention. Each of the cases identified were then reviewed using the database, electronic records and clinical notes review. The following outcomes were identified: birth weight and gestation, presence of a cardiac defect, other associated abnormalities, operative approach, morbidity and mortality including cause of death. A major cardiac defect was defined as per Spitz [5] as cyanotic disease or non-cyanotic disease requiring surgery or medical treatment for heart failure. A minor cardiac defect was defined as any other cardiac disease, including patient ductus arteriosus (PDA) and atrial septal defect (ASD). In addition, the presence of VACTERL, chromosomal abnormalities and other congenital anomalies were noted. Survival was defined as survival to discharge from hospital. Patients identified were subsequently classified using the Spitz classification for comparison of survival rates. Statistical analysis was not performed on the results given the low number of cases. 123 Pediatr Surg Int (2016) 32:571–576 Results In total 349 cases of OA/TOF were found across the 22-year study period, 9 (six males) were identified as being ELBW babies. In these nine patients gestational age ranged from 23 to 34 weeks and median birth weight was 815 g ranging from 630 to 950 g (Table 1). There were two cases from twin pregnancies. The incidence of cases across the time period is shown in Table 2. This demonstrates the increasing numbers of ELBW babies seen as a percentage of all OA/TOF babies in the second half of the study period with double the number of cases and double the proportion of all OA/TOF patients under 1000 g. Overall survival was 56 % (5/9) and was associated to the type of OA. There were no survivors in patients weighing \800 (...truncated)