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

Abstract

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 repair^{1 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

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 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.

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).

Figure 2.

As a neonate in the incubator postoperatively. TAT, transanastomotic tube.

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.

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).

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 poor survival under 800 g. Their only neonate below 800 g with primary repair of OA at 660 g of weight died of necrotising enterocolitis. A recent large series has described increased mortality and risk of fistula recanalisation in the ELBW neonate.¹⁰

We recommend that management of ELBW or Very Low Birth Weight (VLBW) infants with OA should be on a case by case basis. Through our case report, we want to highlight that a primary oesophageal repair should be attempted where baby is stable. Our neonate was ventilated due to prematurity and had a very low birth weight. In a ventilated baby, ligation of the TOF becomes a priority as air can preferentially go down the fistula instead of ventilating the lungs also distending the stomach and making it further difficult to ventilate. Hence, a gastrostomy only may not suffice and ligation of the fistula takes priority. In our case, after ligating the fistula, the anaesthetists did not report any physiological instability and tissues were amenable to a primary anastomosis. Hence, we proceeded to the full surgical repair.

On reviewing the literature, we believe that this is a new record for the lowest birthweight neonate with OA repaired soon after birth with survival of over 5 years. A 5-year survival is usually considered a good measure of success. The longer the postoperative follow-up, the better and clearer an outcome is assessed. The survival and functional outcome in our case, as assessed at 6 years of age shows the irrefutable success of the primary operation in this patient. Hence, we are publishing this now, rather than at the very early postoperative period. We have also had an emotional letter of gratitude from the mother saying how worthwhile it was, prompting the write up of this 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-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.