Successful primary repair of oesophageal atresia and distal tracheo-oesophageal fistula in a 525 g neonate, the smallest reported in literature.

Case report Successful primary repair of oesophageal atresia and distal tracheo-oesophageal fistula in a 525 g neonate, the smallest reported in literature Rashmi Roshan Singh ‍ ‍,1 Michael Hird,2 Ashwini Joshi1 1 Deaprtment of Paediatric Suregry, Barts Health NHS Trust, London, UK 2 Deaprtment of Neonatology, Barts Health NHS Trust, London, UK Correspondence to Mrs Ashwini Joshi; Accepted 23 February 2021 SUMMARY Advances in neonatal intensive care have meant improved survival of the extremely low birth weight neonate. We report the successful primary repair of a preterm neonate born with an oesophageal atresia and tracheo-oesophageal fistula with a birth weight of just 525 g, on day 1 of life, the smallest survivor so far reported in the literature, now followed up for 5 years. BACKGROUND Series of extremely low birthweight (ELBW) babies with oesophageal atresia and tracheo-oesophageal fistula (OA TOF)1–3 report varying success and survival rates. There has always been controversy about the surgical approach for these extremely fragile premature neonates. The favoured management has been ligation of the fistula and delayed OA repair1 4 5 as opposed to primary repair; the reasons being extreme prematurity, difficult anaesthesia, intraoperative physiological instability and friable tissues.2 3 6 The smallest survivor with a primary repair reported in the literature weighed 740 g, born at 31 weeks gestation.6 Other series have reported their experience with smaller neonates of 630 g1 and 471 g.7 Neither of these had a primary repair nor did they survive. According to the widely used Spitz classification8 the survival rate in neonates with birth weight less than 1500 g in the absence of major cardiac disease is 59% falling in the presence of cardiac disease to 22%. The ELBW neonate needs meticulous surgical and neonatal care to survive this congenital anomaly and accompanying respiratory insufficiency with other associated comorbidities. CASE PRESENTATION © BMJ Publishing Group Limited 2021. No commercial re-use. See rights and permissions. Published by BMJ. To cite: Singh RR, Hird M, Joshi A. BMJ Case Rep 2021;14:e239696. doi:10.1136/bcr-2020239696 We present an infant who was delivered by emergency Caesarean section once she had reached an estimated fetal weight of 500 g at 30 weeks of gestation due to severe intrauterine growth restriction (IUGR) with reversed end diastolic flow. The ante-natal scans had shown persistent IUGR, and an absent stomach bubble. She developed respiratory distress at birth and was intubated and ventilated. She was transferred to our centre shortly after birth, for surgical review of suspected OA TOF. The diagnosis was confirmed at our centre (figure 1) . She had type C OA (Gross classification). She also had a small muscular ventricular septal defect (VSD). She underwent ligation of the TOF and OA repair on day 1 Figure 1 Preoperative (left) and postoperative (right) X-rays demonstrating oesophageal atresia with tracheo-oesophageal fistula (left) and naso-gastric tube postoperatively through the anastomosis into the stomach (right). of life through an extra pleural approach via a right thoracotomy. The TOF was ligated first. This brought about immediate improvement in the ventilation. In agreement with the anaesthetist, as she remained stable, we proceeded to mobilise and anastomose the oesophagus. The tissues were mature and robust enough to perform a good tension free anastomosis using 6/0 PDS sutures over a transanastomotic tube (TAT) (figure 2) . She was extubated 3 days later. She initially received parenteral nutrition. Enteral feeds were gradually introduced via the TAT on day 19 of life. Feeds were briefly stopped due to an episode of line sepsis, during which she required re-intubation. She was then extubated onto biphasic Continuous Positive Airway Pressure (CPAP) on day 40 of life and reached full enteral feeds at day 47 of life, mainly oral and the remainder via a naso-gastric tube. She was discharged to her local neonatal unit at day 54 of life. There were no acute postoperative complications such as anastomotic leak or long-term complication from the anastomosis such as stricture or recurrence of the fistula. Chromosomal analysis did not reveal any abnormality. An endoscopy done for insertion of gastrostomy at 2 years of age showed no evidence of anastomotic stricture. The gastrostomy was to supplement her oral intake of food. OUTCOME AND FOLLOW-UP Five years on, she has mild learning difficulty and a gastrostomy for top up feeds. She has been recently diagnosed with portal hypertension due to portal cavernomas and is under review for renal calculi and unilateral hearing loss. Her VSD (muscular wall defect) closed off spontaneously. Singh RR, et al. BMJ Case Rep 2021;14:e239696. doi:10.1136/bcr-2020-239696 1 Case report Patient’s perspective Thank you again for all that you have done for our daughter over the past 6 years. We will be forever grateful for the lifesaving operation, as without it our daughter would not be with us today. Yes, she has her challenges but wakes up every morning with a smile on her face and the determination and strength of an ox. Learning points ►► Primary repair of oesophageal atresia and tracheo- Figure 2 As a neonate in the incubator postoperatively. TAT, transanastomotic tube. She has not required any oesophageal dilatation until now. She is now 6 years old. She is growing well and following centiles for height and weight. Her weight is between the 9th and 25th centile and her height is between 25th and 50th centile (UK-WHO growth charts).9 She has intact motor skills. She is outgoing, sociable and happy but does have intellectual disability and learning difficulties. She has oral food aversion, which is improving. No unifying genetic diagnosis has been made. She has defied all odds and is enjoying a happy childhood (see the Parent’s perspective section). oesophageal fistula can be attempted in the stable neonate, irrespective of size on a case-by-case basis. ►► Expert surgical technique with precision and delicate tissue handling along with anaesthetic and neonatal expertise at specialised neonatal surgical centres is key to the successful outcome. ►► In our opinion besides birth weight, gestational age should also be taken into consideration as this confers tissue maturity. ►► Chromosome analysis may reveal associated defects. also had an emotional letter of gratitude from the mother saying how worthwhile it was, prompting the write up of this report. Contributors RRS and AJ have been involved in planning, conduct and reporting of the work. MH has critically reviewed the work. DISCUSSION Results in the published literature of OA TOF in babies under 1000 g show survival rate of 55% compared with 95% in babies >1500 g, confirming the difficulty in managing small babies with OA. Results also reflect current neonatal care.The most significant finding from Hannon et al1 is the p (...truncated)