*2.1.4 Surgical management*

Ideally, EA/TEF is corrected in a single procedure. Staged procedure, beginning with decompressive gastrostomy and fistula takedown, followed by esophageal

reconstruction at a later date, is reserved for those too unstable to tolerate general anesthesia due to respiratory or cardiac defects. Infants with long gap atresia also undergo delayed repair to allow elongation of the proximal and distal esophageal ends.

In current practice, the minimally invasive approach using video assisted thoracoscopy is preferred to open thoracotomy. If the open approach is employed, a right posterolateral thoracotomy incision is made at the fourth intercostal space, sparing the serratus anterior and latissimus dorsi muscles. Extrapleural dissection is carried until the azygous vein is encountered, which is then divided. In the case of type A, the lower esophageal pouch and its associated fistula are identified. The fistula is resected. The proximal esophageal pouch is then mobilized to establish tension free continuity between the two ends. If a proximal fistula is present, this is ligated prior to mobilization. The esophagus is reconstructed via a single layer end-to-end anastomosis. A chest tube is placed and remains until post-operative esophogram confirms patency of the anastomosis. Anastomotic leaks tend to heal without intervention and are managed by continuation of chest tube and antibiotics.

#### *2.1.5 Outcomes*

Thoracoscopic approach has led to improved outcomes and most infants grow to lead fairly normal lives, given the lack of concurrent anomalies such as cardiac defects. Most commonly, gastroesophageal reflux (GER) and esophageal strictures are lifelong issues endured by the patient. GER may be asymptomatic or lead to persistent cough, respiratory problems or esophageal stricturing. Primary management is medical with anti-reflux medications and prokinetics. Surgical correction of GER with fundoplication is last resort. Esophageal strictures may form many years after repair and are best managed by endoscopic dilation. Recurrent or refractory esophageal strictures require surgical resection and re-anastomosis.

#### **2.2 Duodenal atresia**

#### *2.2.1 Embyrology*

It results due to the failure of duodenal recanalization and most commonly occurs in the second portion of the duodenum distal to ampulla of Vater but any segment can be affected.

#### *2.2.2 Clinical presentation*

Emesis and feeding intolerance occurs in the first 24–48 h of life. The type of emesis—bilious versus non—depends on the location of atresia relative to the major duodenal papilla. If obstruction is distal to it, infant will exhibit bilious emesis. Obstruction proximal to the ampulla causes non-bilious emesis. Abdomen will not be distended due to proximal nature of obstruction. A palpable mass in the epigastrium may be appreciated on physical exam.

#### *2.2.3 Diagnosis*

The "double bubble" on abdominal x-ray indicates air in stomach and duodenum but not in distal small bowel and colon. An UGI series must be obtained to rule out malrotation, which can also present with bilious emesis early in life and is a surgical emergency. UGI may reveal a duodenal web, which is an intraluminal diverticulum that appears as an elongated, conical silhouette resembling a "windsock". Echocardiogram and renal ultrasound are performed to rule out any other defects as there is an association with trisomy 21 and its related complications.

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*Congenital Anomalies of the Gastrointestinal Tract DOI: http://dx.doi.org/10.5772/intechopen.92588*

"The diamond D", Diamond Duodenoduodenostomy—A transverse incision is made in the proximal widened duodenum and a longitudinal incision in the distal tapered portion of the duodenum (**Figure 2**). The anastomosis is created in a diamond shape to facilitate mucosal abutment between the two incongruent duodenal diameters. During repair, evaluation for duodenal web must be performed because they are not always identified on pre-operative UGI and can cause persistent obstruction if not corrected. If present, a longitudinal duodenotomy is performed over the area of the web and it is excised. Careful attention must be paid to its location relative to the major duodenal papilla so as to not disrupt the integrity of the ampulla of Vater. The duodenotomy is closed in a transverse fashion to avoid

There tend to be few, if any, long term complications following correction of duodenal atresia. Persistent obstruction may indicate missed duodenal web and requires re-operation. Delayed gastric emptying may occur in the early postoperative period and does not warrant any intervention; most cases resolve with time and

The exact etiology is unknown. Exposure to erythromycin has been implicated

It is characterized with feeding intolerance and non-bilious emesis that becomes projectile over time; usually presenting around 2–4 weeks of life, however, may not present up until 6–12 weeks. Emesis is non-bilious because the site of obstruction, the pylorus, is proximal to the ampulla of Vater. It tends to occur in first born

On physical exam, may be able to palpate an "olive like" firm, mobile mass in the right upper quadrant or epigastrium, however this is often difficult to appreciate on a restless infant. Abdomen is otherwise soft and non-distended. Ultrasound is diagnostic and demonstrates a pyloric channel length ≥ 16 mm, wall ≥4 mm in

Repeated vomiting of gastric acid (HCl) leads to hypochloremia, alkalosis and dehydration. Hypovolemia stimulates aldosterone secretion with resultant sodium resorption and potassium secretion. Thus, the infant's laboratory panel will reveal hypochloremic, hypokalemic metabolic alkalosis. Hydrogen is shifted extracellularly in exchange for potassium to correct the acid–base imbalance, exacerbating hypokalemia. Eventually, worsening hypokalemia stimulates the renal hydrogenpotassium pump to resorb potassium and secrete hydrogen, resulting in acidic urine. This is termed "paradoxical aciduria" because bicarbonate secretion should take precedence in an alkalotic state, but the nephrons prioritize correction of

potassium at the expense of hydrogen loss instead.

enteral feedings can be advanced in small volumes as tolerated.

*2.2.4 Surgical management*

narrowing of the lumen.

*2.2.5 Outcomes*

**2.3 Pyloric stenosis**

*2.3.1 Embryology*

as a risk factor [1].

Caucasian males.

*2.3.3 Diagnosis*

thickness.

*2.3.2 Clinical presentation*

## *2.2.4 Surgical management*

*Congenital Anomalies in Newborn Infants - Clinical and Etiopathological Perspectives*

reconstruction at a later date, is reserved for those too unstable to tolerate general anesthesia due to respiratory or cardiac defects. Infants with long gap atresia also undergo delayed repair to allow elongation of the proximal and distal esophageal ends. In current practice, the minimally invasive approach using video assisted thoracoscopy is preferred to open thoracotomy. If the open approach is employed, a right posterolateral thoracotomy incision is made at the fourth intercostal space, sparing the serratus anterior and latissimus dorsi muscles. Extrapleural dissection is carried until the azygous vein is encountered, which is then divided. In the case of type A, the lower esophageal pouch and its associated fistula are identified. The fistula is resected. The proximal esophageal pouch is then mobilized to establish tension free continuity between the two ends. If a proximal fistula is present, this is ligated prior to mobilization. The esophagus is reconstructed via a single layer end-to-end anastomosis. A chest tube is placed and remains until post-operative esophogram confirms patency of the anastomosis. Anastomotic leaks tend to heal without intervention and are managed by continuation of chest tube and antibiotics.

Thoracoscopic approach has led to improved outcomes and most infants grow to lead fairly normal lives, given the lack of concurrent anomalies such as cardiac defects. Most commonly, gastroesophageal reflux (GER) and esophageal strictures are lifelong issues endured by the patient. GER may be asymptomatic or lead to persistent cough, respiratory problems or esophageal stricturing. Primary management is medical with anti-reflux medications and prokinetics. Surgical correction of GER with fundoplication is last resort. Esophageal strictures may form many years after repair and are best managed by endoscopic dilation. Recurrent or refractory

It results due to the failure of duodenal recanalization and most commonly occurs in the second portion of the duodenum distal to ampulla of Vater but any

Emesis and feeding intolerance occurs in the first 24–48 h of life. The type of emesis—bilious versus non—depends on the location of atresia relative to the major duodenal papilla. If obstruction is distal to it, infant will exhibit bilious emesis. Obstruction proximal to the ampulla causes non-bilious emesis. Abdomen will not be distended due to proximal nature of obstruction. A palpable mass in the epigas-

The "double bubble" on abdominal x-ray indicates air in stomach and duodenum but not in distal small bowel and colon. An UGI series must be obtained to rule out malrotation, which can also present with bilious emesis early in life and is a surgical emergency. UGI may reveal a duodenal web, which is an intraluminal diverticulum that appears as an elongated, conical silhouette resembling a "windsock". Echocardiogram and renal ultrasound are performed to rule out any other defects as

there is an association with trisomy 21 and its related complications.

esophageal strictures require surgical resection and re-anastomosis.

**88**

*2.1.5 Outcomes*

**2.2 Duodenal atresia**

segment can be affected.

*2.2.2 Clinical presentation*

trium may be appreciated on physical exam.

*2.2.1 Embyrology*

*2.2.3 Diagnosis*

"The diamond D", Diamond Duodenoduodenostomy—A transverse incision is made in the proximal widened duodenum and a longitudinal incision in the distal tapered portion of the duodenum (**Figure 2**). The anastomosis is created in a diamond shape to facilitate mucosal abutment between the two incongruent duodenal diameters. During repair, evaluation for duodenal web must be performed because they are not always identified on pre-operative UGI and can cause persistent obstruction if not corrected. If present, a longitudinal duodenotomy is performed over the area of the web and it is excised. Careful attention must be paid to its location relative to the major duodenal papilla so as to not disrupt the integrity of the ampulla of Vater. The duodenotomy is closed in a transverse fashion to avoid narrowing of the lumen.
