*2.8.2.1 History*

*Pediatric Surgery, Flowcharts and Clinical Algorithms*

*Single gene mutations associated with oesophageal atresia.*

Postnatally, the neonate is not able to swallow saliva and food. This leads to accumulation of saliva and food in the upper oesophageal segment (pouch). The accumulated saliva and food then spill over into the lungs through the larynx and trachea. Patients are therefore prone to the development of aspiration pneumonia. Patients with distal tracheo-oesophageal fistula also tend to aspirate gastric secre-

The distal tracheo-oesophageal fistula also allows air to escape from the trachea to the stomach and hence the intestines. Consequently, patients can develop gross distention of the stomach and intestine, especially if patient is resuscitated using an Ambu bag. The distended stomach can then splint the diaphragm, impairing diaphragmatic excursion and thereby causing respiratory distress. The distended

In patients with delayed diagnosis, the constant regurgitation of swallowed food leads to malnutrition. Such patients tend to have poor surgical outcome and overall

Associated congenital anomalies, especially severe cardiac and renal anomalies, also affect management and survival of patients. Low birth weight that may result from premature delivery owing to polyhydramnios also tends to affect management

Prenatal diagnosis helps in planning of delivery and possible prenatal referral to

Oesophageal atresia should be suspected prenatally in patients with evidence of polyhydramnios. Polyhydramnios will present with symphysio-fundal height

tions into the lungs, especially if lying in a head-down position.

Oesophageal atresia may be detected prenatally or postnatally.

stomach can also rupture leading to chemical peritonitis.

**54**

**Table 4.**

**Table 3.**

survival.

and survival.

**2.8 Clinical presentation**

*2.8.1 Prenatal presentation and diagnosis*

centres that have the facilities to manage such patients.

*Common associated anomalies in non-syndromic oesophageal atresia.*

Postnatally, patients with oesophageal atresia present with drooling of saliva (excessive salivation). A history of antenatal polyhydramnios gives more credence to the diagnosis of oesophageal atresia. Delayed diagnosis leads to aspiration of saliva from the upper oesophageal pouch, causing aspiration pneumonia with cough, cyanosis and fever. Feeding leads to worsening of the aspiration pneumonia. Hence, early detection and avoidance of oral feeds helps to promote good outcome of patients.

Patients with distal tracheo-oesophageal fistula are also at risk of aspiration of gastric secretions into the lungs. In addition, swallowed air passes through the distal tracheo-oesophageal fistula into the stomach. This can lead to gross gastric distention, especially following Ambu bagging, resulting in diaphragmatic splinting and sometimes gastric rupture. They may thus present with respiratory distress, gross abdominal distention and sometimes peritonitis.

Patients with tracheo-oesophageal fistula without oesophageal atresia are usually diagnosed late. They present with recurrent cough associated with feeding and recurrent episodes of pneumonia.

Other symptoms would depend on associated congenital anomalies. Patients with cardiac anomalies may present with cyanosis. Patients with renal agenesis will have a history of anuria.

During history taking, one should seek for a maternal history of uncontrolled diabetes mellitus and the use of drugs such as oral contraceptives, antithyroid drugs (carbimazole and methimazole) and thalidomide. These are risk factors for oesophageal atresia.

The history of the maturity and weight of the baby at birth are also important considerations.

#### *2.8.2.2 Physical examination*

The principles of physical examination of a patient with suspected oesophageal atresia are to confirm the diagnosis, assess for aspiration pneumonia and evaluate for associated congenital anomalies.

To confirm the diagnosis, a stiff radiopaque 10-gauge French catheter is passed through the mouth into the oesophagus. The tube is not passed through the nose because it may traumatize the nasal passages. The diagnosis of oesophageal atresia is heightened if the tube fails to reach the stomach by getting arrested in the proximal oesophageal pouch at about 10 cm from the alveolar margin. A soft and smaller tube may coil in the upper oesophageal pouch, giving an impression of reaching the stomach. The secretions sucked through the tube can be tested with litmus paper to differentiate between saliva (basic) in the upper oesophageal pouch and gastric secretions (acidic).

The presence of fever, respiratory distress, reduced air entry in the lungs (especially on the right side) and crepitations in the lungs are an indication of aspiration pneumonia. However, patients in heart failure will have similar chest findings, and

#### *Pediatric Surgery, Flowcharts and Clinical Algorithms*

these are usually bilateral. Gastric distention and gastric rupture will be associated with respiratory distress, abdominal distention and abdominal signs of peritonitis.

Meticulous examination for associated anomalies is done. The presence of cyanosis may be due to cyanotic heart disease. The chest is examined for heart murmurs and crepitations. The abdomen is examined for any palpable flank mass. The perineum is examined for ARM. The spine and the limbs are also examined for any anomaly. Indeed, the VACTERL association could be used as a guide to take history, examine and investigate for any associated congenital anomaly.

#### *2.8.2.3 Investigations*

Investigations are also done to confirm the diagnosis, to assess for complications (aspiration pneumonitis and gastric rupture) and to evaluate for associated anomalies.

Oesophageal atresia is confirmed by taking a plain X-ray of the neck, chest, and abdomen with a radiopaque tube passed through the mouth in situ. In the presence of oesophageal atresia, the X-ray will show the tube arrested in the upper oesophageal pouch or coiled in the upper pouch. The same X-ray is also evaluated for evidence of associated aspiration pneumonia, cardiac enlargement, dextrocardia and vertebral anomalies.

The abdominal portion will help to determine the presence of air in the stomach and intestine. The presence of air in the stomach and intestine indicates the presence of a distal tracheo-oesophageal fistula. Pneumoperitoneum indicates gastric rupture. A gasless abdomen implies the absence of a distal tracheo-oesophageal fistula. Such a patient may either have no tracheo-oesophageal fistula or a proximal fistula. The proximal fistula can be identified with the aid of bronchoscopy [32]. **Table 5** summarises the important diagnostic features of OA.

Abdominal ultrasound to assess the kidneys, ureters and the urinary bladder is also done. Patients with bilateral renal agenesis or severe multicystic dysplastic kidneys will need no further management since such patients will not survive. Renal scintigraphy will confirm non-functioning kidneys.

Ultrasonography (USG) of the spine is used to assess the spinal cord for anomalies, including tethered cord. The USG of the spinal cord should be done before 3 months of age since the bones become ossified after this age, rendering the use of USG impossible. Magnetic resonance imaging (MRI) may be used to evaluate the spinal cord. It is, however, very expensive and implies the need for general anaesthesia in a new born with a relatively high anaesthetic risk. It is not readily available at most centres, especially in the developing countries.


**57**

**Table 6.**

classification [38].

*Oesophageal Atresia: Drowning a Child in His/Her Own Saliva*

Echocardiogram is used to evaluate the heart for a congenital heart disease and the direction of the arch of the aorta. The direction of the arch of the aorta (normally to the left) is utilised in deciding the side of the thoracotomy incision (usually on the right side since the arch is normally to the left). At our centre, echocardiogram is not routinely done for every patient since it is expensive and not readily available. Hence, we selectively use it for only patients with clinical evidence

Patients with suspected chromosomal and genetic anomalies are evaluated to confirm the anomaly. However, this is not available at most centres in developing centres, including our centre. Patients with confirmed trisomy 18 (Edwards syndrome) should have any planned surgical intervention abandoned since prognosis

Patients with Down syndrome should be evaluated for the possibility of associ-

Other investigations that are supportive are full blood count (FBC) and blood urea, electrolytes, and creatinine (BUE&Cr). Blood gases may be analysed in

Prognostic classification is done based on risk factors that affect survival of infants with OA. It is used to guide operative treatment and to compare case outcomes over time and between centres. The first prognostic risk stratification of patients with OA was developed in 1962 (Waterston classification) [4]. This is based on birth weight, the presence of pneumonia and associated congenital anomalies (**Table 6**). It has provided important contribution to the care of infants with OA. Patients in group A (good-risk category) are offered immediate primary repair of the defect. Those in group B (moderate-risk category) are treated with delayed primary repair, while

Several new classification schemes have been developed because of improvement in neonatal intensive care and availability of more treatment options for multiple congenital anomalies. These new schemes include refinement of the Waterston classification by Randolf and colleagues in 1989 [33]. This is based on the overall physiologic status of the patient. Poenaru et al. [34] also developed a new prognostic classification based on severe pulmonary dysfunction with preoperative mechanical ventilation and severe associated anomalies. Spitz et al. [35] developed a new risk classification based on birth weight and major cardiac anomaly (**Table 7**). Currently, the Spitz classification is the most commonly used system [36, 37]. Another new prognostic classification system has been developed by adding preoperative respiratory distress syndrome and pneumonia to the Spitz

ated duodenal atresia, congenital heart disease and Hirschsprung's disease.

those in group C (high-risk category) are managed by staged repair.

*DOI: http://dx.doi.org/10.5772/intechopen.84525*

of congenital heart disease.

for these patients is very poor.

patients who are critically ill.

**2.9 Clinical prognostic classification of patients**

*Waterston prognostic classification of oesophageal atresia infants.*

**Table 5.** *Important diagnostic features of oesophageal atresia.*

*Pediatric Surgery, Flowcharts and Clinical Algorithms*

*2.8.2.3 Investigations*

and vertebral anomalies.

these are usually bilateral. Gastric distention and gastric rupture will be associated with respiratory distress, abdominal distention and abdominal signs of peritonitis. Meticulous examination for associated anomalies is done. The presence of cyanosis may be due to cyanotic heart disease. The chest is examined for heart murmurs and crepitations. The abdomen is examined for any palpable flank mass. The perineum is examined for ARM. The spine and the limbs are also examined for any anomaly. Indeed, the VACTERL association could be used as a guide to take history,

Investigations are also done to confirm the diagnosis, to assess for complications (aspiration pneumonitis and gastric rupture) and to evaluate for associated anomalies. Oesophageal atresia is confirmed by taking a plain X-ray of the neck, chest, and abdomen with a radiopaque tube passed through the mouth in situ. In the presence of oesophageal atresia, the X-ray will show the tube arrested in the upper oesophageal pouch or coiled in the upper pouch. The same X-ray is also evaluated for evidence of associated aspiration pneumonia, cardiac enlargement, dextrocardia

The abdominal portion will help to determine the presence of air in the stomach and intestine. The presence of air in the stomach and intestine indicates the presence of a distal tracheo-oesophageal fistula. Pneumoperitoneum indicates gastric rupture. A gasless abdomen implies the absence of a distal tracheo-oesophageal fistula. Such a patient may either have no tracheo-oesophageal fistula or a proximal fistula. The proximal fistula can be identified with the aid of bronchoscopy [32].

Abdominal ultrasound to assess the kidneys, ureters and the urinary bladder is also done. Patients with bilateral renal agenesis or severe multicystic dysplastic kidneys will need no further management since such patients will not survive.

Ultrasonography (USG) of the spine is used to assess the spinal cord for anomalies, including tethered cord. The USG of the spinal cord should be done before 3 months of age since the bones become ossified after this age, rendering the use of USG impossible. Magnetic resonance imaging (MRI) may be used to evaluate the spinal cord. It is, however, very expensive and implies the need for general anaesthesia in a new born with a relatively high anaesthetic risk. It is not readily available

examine and investigate for any associated congenital anomaly.

**Table 5** summarises the important diagnostic features of OA.

Renal scintigraphy will confirm non-functioning kidneys.

at most centres, especially in the developing countries.

**56**

**Table 5.**

*Important diagnostic features of oesophageal atresia.*

Echocardiogram is used to evaluate the heart for a congenital heart disease and the direction of the arch of the aorta. The direction of the arch of the aorta (normally to the left) is utilised in deciding the side of the thoracotomy incision (usually on the right side since the arch is normally to the left). At our centre, echocardiogram is not routinely done for every patient since it is expensive and not readily available. Hence, we selectively use it for only patients with clinical evidence of congenital heart disease.

Patients with suspected chromosomal and genetic anomalies are evaluated to confirm the anomaly. However, this is not available at most centres in developing centres, including our centre. Patients with confirmed trisomy 18 (Edwards syndrome) should have any planned surgical intervention abandoned since prognosis for these patients is very poor.

Patients with Down syndrome should be evaluated for the possibility of associated duodenal atresia, congenital heart disease and Hirschsprung's disease.

Other investigations that are supportive are full blood count (FBC) and blood urea, electrolytes, and creatinine (BUE&Cr). Blood gases may be analysed in patients who are critically ill.
