|Year : 2018 | Volume
| Issue : 3 | Page : 186-188
Survival in esophageal atresia diagnosed on 19th day of life: Lessons learned
Rahul Gupta1, Dinesh Kumar Barolia1, Neelam Dogra2, Aditya Pratap Singh1, Ramesh Tanger1, Arun Kumar Gupta1
1 Department of Pediatric Surgery, SMS Medical College, Jaipur, Rajasthan, India
2 Department of Anesthesia, SMS Medical College, Jaipur, Rajasthan, India
|Date of Web Publication||6-Feb-2019|
Dr. Rahul Gupta
Department of Paediatric Surgery, SMS Medical College, Jaipur, Rajasthan
Source of Support: None, Conflict of Interest: None
Delayed diagnosis of esophageal atresia (EA) is associated with bad prognosis. Only five cases of survival with delayed presentation in the 3rd week of life have been previously reported. We present a rare case of survival in a neonate, who presented on the 19th day of life with regurgitation of feed and respiratory distress. After preoperative optimization, primary repair was performed the next day. The use of number 10 sterile, blunt-tipped, soft-red rubber catheter to rule out EA shortly after the birth in the delivery (resuscitation) room by attending doctor or trained paramedical personnel is recommended.
Keywords: Delayed diagnosis, esophageal atresia, survival, tracheoesophageal fistula
|How to cite this article:|
Gupta R, Barolia DK, Dogra N, Singh AP, Tanger R, Gupta AK. Survival in esophageal atresia diagnosed on 19th day of life: Lessons learned. Saudi J Health Sci 2018;7:186-8
|How to cite this URL:|
Gupta R, Barolia DK, Dogra N, Singh AP, Tanger R, Gupta AK. Survival in esophageal atresia diagnosed on 19th day of life: Lessons learned. Saudi J Health Sci [serial online] 2018 [cited 2019 Jun 26];7:186-8. Available from: http://www.saudijhealthsci.org/text.asp?2018/7/3/186/251596
| Introduction|| |
Delayed diagnosis of esophageal atresia (EA) with tracheoesophageal fistula (TEF) is a common problem in our country and is associated with bad prognosis. There are isolated reports of neonates with EA with TEF who presented late in 3rd week of life and survived after surgery.,,,, We present a rare case of survival in EA with TEF diagnosed on 19th day and operated on 20th day of life, respectively. We briefly discuss the pertinent literature along with measures to prevent the delayed diagnosis of EA.
| Case Report|| |
A 19-day-old full-term male baby weighing 2000 g presented with a history of regurgitation of feed and respiratory distress since birth. He was born with normal delivery at home and had feeding difficulty and respiratory distress from the 1st day of life. On the 10th day of life, he was admitted to a nearby hospital for the treatment of pneumonia. The patient was referred to a higher center after 4 days of failed medical management; 5 days later, he was referred to our institute with suspicion of EA. At 19 days of age, a red rubber catheter confirmed the diagnosis of EA with TEF [Figure 1]. On examination, the baby was active, had mild respiratory distress and weighed 2000 g. The chest radiograph showed the right lower zone pneumonitis. Preoperative optimization was done with O2 therapy, upper pouch suctioning, chest physiotherapy, nebulization, hydration, broad-spectrum antibiotics, and correction of dyselectrolytemia. The patient received parenteral nutritional therapy for the prevention of ketosis from the day of admission. Next day, the neonate underwent right extrapleural posterolateral thoracotomy with primary repair [Figure 1]. The gap between the upper pouch and lower esophageal fistula was 1.5 cm. A dye study (esophagogram) was performed to evaluate the esophageal continuity. Breastfeeding was started, and the baby was discharged on the 5th postoperative day. The baby is doing well after 1-month of follow-up.
|Figure 1: Preoperative chest radiographs (anteroposterior and lateral views; left) showing red rubber catheter in the esophagus arrested at the T4 vertebral level with the presence of gas in the abdomen; operative photograph (right) showing end-to-end esophageal anastomosis|
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| Discussion|| |
EA with TEF is a familiar congenital malformation with an incidence of one in 2500 live births. The reported incidence of EA with TEF in India is 18,000 cases per year. Although many of the cases may be unreported. In the developed nations, most of the cases of EA are diagnosed on antenatal sonography, while in Indian subcontinent, the diagnosis is mostly postnatal and often late due to conglomeration of factors.,,,,, In one study, nearly 8% of patients were diagnosed after 1st week. In other high-volume tertiary care institute, 3.72% (16/430) of patients in the year 2016 were diagnosed or after 7 days of life.
A high index of suspicion is important (especially in geographical areas with high incidence) for the diagnosis of EA, when dealing with (a) pregnancies having polyhydramnios, (b) newborn with drooling of saliva from the mouth (excessive frothing), choking or transient cyanosis, regurgitation after attempted feeds, and (c) neonates with respiratory distress., The diagnosis of EA is fairly simple. The author recommends the routine use of number 10 sterile, blunt-tipped, soft-red rubber catheter to rule out EA shortly after the birth in the delivery (resuscitation) room in all newborn babies, especially in geographical areas with high incidence of EA. This would prevent EA being missed, and thereby preventing delayed diagnosis and high mortality. Ideally, attending doctor/receiving doctor/pediatrician should perform this procedure or trained paramedical personnel under the supervision of doctor.
The factors leading to high mortality in cases with delayed diagnosis are undernourishment and pneumonitis (both chemical and aspiration). At home, feeding may be attempted which results in the development of aspiration pneumonitis. All these factors consequently lead to higher predisposition to sepsis. Measures to improve the diagnosis of EA (and other neonatal surgical conditions), particularly in Indian subcontinents: (i) anomaly scan of all pregnancies, especially those with polyhydramnios, (ii) timely referral of mother to a well-equipped center, (iii) well-supervised neonatal transport facilities, (iv) improvement in infrastructure and institutional deliveries, and (v) awareness programs (CME programs) and proper training among medical practitioners.,,,,,
Only five cases of survival in EA, Type C with delayed presentation in the 3rd week of life has been previously reported in the literature [Table 1].,,,, In the present case, the patient was diagnosed as late as 19th day of life, with only one previous case with delayed diagnosis beyond this duration (20th day of life). All the cases including the present case were admitted between 15 and 25 days of life. Most were born with home delivery and previously seen by medical personnel. In all these cases, despite delayed presentation, preoperative resuscitation, and optimization were properly performed along with postoperative care.,,,, Our patient was also treated in an advanced neonatal intensive care unit. Some of the challenges for a critical care specialist which may be encountered, while managing EA cases with delayed presentation in 3rd week of life are undernourishment, dehydration, pneumonitis, sepsis, dyselectrolytemia, ketosis, and other metabolic complications. The survival of all these neonates is clear evidence that advancement in newborn care and anesthesia can save such sick neonates with unfortunate delayed presentation. Follow-up as large as 16 years is present in one of the reports.
|Table 1: Esophageal atresia with tracheoesophageal fistula survivors with delayed presentation during 3rd week of life|
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It has been observed that planned delayed primary repair of EA with TEF has the merit of achieving primary esophageal anastomosis in patients with a long gap, due to spontaneous growth of upper pouch. Furthermore, staged repair is useful in patients with severe respiratory distress, as pulmonary condition can be optimized for best outcomes. Delayed management with preoperative optimization of EA has a better outcome as compared to hastily surgery in unstable patient.
In conclusion, delayed diagnosis of EA is associated with bad prognosis. Preoperative optimization along with postoperative care in neonatal intensive care unit is paramount for survival in neonates, who have delayed presentation as late as in 3rd week of life. The use of number 10 sterile, blunt-tipped, soft red rubber catheter to rule out EA shortly after the birth in the delivery (resuscitation) room by attending doctor (pediatrician) or trained paramedical personnel is recommended to prevent delayed and missed diagnosis. The chances of survival in neonates with delayed diagnosis of EA would increase if the neonate is managed preoperatively (preoperative optimization) in the advanced neonatal intensive care unit.
Declaration of patient consent
The authors certify that they have obtained all appropriate patient consent forms. In the form the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.
I am sincerely thankful to faculty and residents of the Department of Anaesthesia and Department of Paediatric Surgery, SMS Medical College, Jaipur, Rajasthan, India, for helping in our endeavor.
Financial support and sponsorship
Conflicts of interest
There are no conflicts of interest.
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