**2.9 Clinical prognostic classification of patients**

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

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 classification [38].


#### **Table 6.** *Waterston prognostic classification of oesophageal atresia infants.*


#### **Table 7.**

*Spitz prognostic classification of infants with oesophageal atresia.*

#### **2.10 Management of patients**

#### *2.10.1 Pre-operative management*

The aim of preoperative management is to make patient stable before surgical management of the oesophageal atresia. This involves prevention of complications, treatment of life-threatening complications, management of life-threatening associated congenital anomalies and general supportive measures.

Complications to be prevented are mainly aspiration and diaphragmatic splinting by gaseous distention of the stomach and intestines. Maintaining the child in a partly upright position and by repeated or continuous suctioning of the upper oesophageal pouch prevents aspiration. These measures keep the proximal oesophagus empty and reduce the likelihood of overflow of saliva into the lungs. The repeated suctioning of the upper oesophageal pouch should be done every 10 min or more often if the child appears to have excessive mucus or air bubbles. The best tube for suctioning, especially for continuous suctioning, is the Replogle tube. The partial upright position prevents aspiration of stomach secretions. Infants with OA should not be fed to prevent aspiration. The child should have minimal handling to prevent excessive crying and consequent filling of stomach with air. In addition, vigorous resuscitation by Ambu bagging should be avoided to prevent gastric distention.

The main life-threatening complication is respiratory distress. Respiratory distress may be due to prematurity, other congenital abnormalities, aspiration pneumonia, or diaphragmatic splinting. Diaphragmatic splinting in patients with OA results from excessive escape of air through the distal fistula into the stomach, causing distention of the stomach and intestines and, in some cases, causing gastric rupture. Thorough evaluation of the infant to determine the underlying cause and prompt management is paramount. Gross gastric distension and perforation, causing pneumoperitoneum and elevation of the diaphragm, can cause major morbidity by worsening ventilation. Prompt needle decompression of the abdomen should be offered. This is followed by urgent laparotomy to control the air leakage. This involves insertion of a Foley catheter through the gastric perforation into the lower oesophagus, thereby occluding the distal TOF and allowing thoracotomy to proceed, at which time the distal TOF is divided. Blood gases should be monitored, but if monitoring facilities are not available, the infant must be kept pink at all times, and pulse oximetry for monitoring is considered standard.

Patients with duct-dependent congenital heart disease should be stabilised, by keeping the duct (patent ductus arteriosus) patent with prostaglandin E1 infusion, before surgery is done.

General supportive measures include keeping the child warm, vitamin K administration, provision of IV fluids/parenteral nutrition and administration of broad-spectrum antibiotics.

**59**

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

The operative management may be immediate primary repair, delayed primary repair or staged repair; and this depends on the prognostic category of the patient and the gap between the upper and the lower oesophageal segments. Patients with pneumonia and duct-dependent congenital heart disease need to be stabilised and planned for delayed primary repair. Patients with long-gap oesophageal atresia could also be managed with delayed primary repair. Further growth of the upper oesophageal pouch tends to occur if primary repair is delayed for about 3 months. Hence, the role of total parenteral nutrition (TPN) becomes significantly important. No consensus has been reached for the definition of long-gap OA. However, long gap is

Primary repair is achieved mostly through a right thoracotomy (readers should read operative textbooks for details of the surgical procedure). However, in patients with right-sided arch of aorta, left thoracotomy is done. The patient is placed at the right side uppermost and with a towel folded underneath the left chest to give lateral flexion. The right arm is extended above the head and the head slightly flexed. A transverse incision is centred on the inferior angle of the scapula, and the chest is entered through the fourth intercostal space. Approach to the oesophagus may be extrapleural or transpleural. The extrapleural approach is favoured because of the less likelihood of developing empyema following an anastomotic leak. The pleura is swept off the chest wall to identify the structures of the posterior mediastinum. The

Any distal TOF is identified and divided close to the trachea. The fistula is then closed with interrupted absorbable sutures such as 4/0 Vicryl, PDS, or Monocryl. Care should be taken to avoid damage to the vagal fibres and blood supply to the

The upper oesophagus is identified with the aid of a tube passed through the mouth or nose by the anaesthetist. It is then mobilised enough to allow an end-toend, one-layer, interrupted oesophageal anastomosis, ensuring that the mucosa and the submucosa are included. The upper oesophageal segment can be extensively mobilized along its full length without risk of significant ischemia. If extensive mobilization of the proximal oesophagus fails to provide adequate length, the lower oesophagus may be mobilized to prevent undue tension on the anastomosis. Mobilising the lower oesophagus without complete disruption of its segmental vascular supply is possible. However, care should be taken to avoid excessive or rough handling of the oesophagus. If, despite extensive mobilisation of the two oesophageal segments, an anastomosis cannot be performed without excessive tension, an oesophageal myotomy (Levaditis procedure) can be performed. Myotomy (circular or spiral myotomy) is usually done using the upper pouch. It can cause significant damage to the nerve and blood supply distal to the myotomy and predisposes to

Patients with TOF without atresia have an oblique fistula running downward from the trachea to the oesophagus, usually at the level of T1–T3. This level is somewhat higher than what is seen in most patients with oesophageal atresia. In these patients, a cervical approach provides the best surgical access as most fistulas are in the root of the neck (at about the level of the second thoracic vertebra). Care should be taken to avoid damage to the recurrent laryngeal nerves, which lie in the grooves between the oesophagus and the trachea and closely related to the fistula,

Staged repair of OA involves division of TOF, placement of feeding gastrostomy and cervical oesophagostomy. Disconnection of distal TOF is done at most centres through thoracotomy. At our centre, however, this is done by dividing the abdominal

defined by some authorities as a gap of ≥3 vertebral bodies or ≥5 cm [39].

*2.10.2 Operative management of oesophageal atresia*

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

azygous vein is then ligated and divided.

diverticulum formation and strictures.

during operative dissection.

distal oesophageal segment.
