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CONGENITAL MALFORMATION OF THE ESOPHAGUS

Owoupele . A . G Ml 610 Moscow 2013

DEFINITION
It is the failure of the esophagus to form a continuous passage from pharynx to stomach during embryonic development resulting in obstruction in infants normal swallowing routes.
INCIDENCE

Approximately in infants of 3,000-3,500 live births. Occur Both males and females, frequently associated with prematurity. It is the 25th most common birth defect.

ETIOLOGY
Unknown Chromosomal anomalies (trisomy 18, trisomy 21, and trisomy 13) Failure of embryonic development Digestive tract problems(diaphragmatic hernia, intestinal atresia or imperforated anus.) Congenital heart diseases(ventricular septal defect,tetralogy of fallot or patent ductus arteriosus.) Renal and urinary tract problems(horseshoe or polycystic kidney,absent kidney or hypospadias)

"LONG GAP", PURE OR ISOLATED ESOPHAGEAL ATRESIA ESOPH AGEAL ATRESIA WITH PROXIMAL TEF (TRACHEOESOPHAGEAL FISTULA) ESOPHAGEAL ATRESIA WITH DISTAL TEF (TRACHEOESOPHAGEAL FISTULA) ESOPHAGEAL ATRESIA WITH BOTH PROXIMAL AND DISTAL TEFS (TWO TRACHEOESOPHAGEAL FISTULAS) TEF (TRACHEOESOPHAGEAL FISTULA) ONLY WITH NO ESOPHAGEAL ATRESIA ESOPHAGEAL STENOSIS

Pathophysiology
Esophagus developed from first segment of embryonic gut.

During the

4th and 5th week of gestation ,forgut normally

lengthens and separate longitudinally and longitudinal portion fuse to form parallel channels. Anomalies involving trachea and

esophagus are caused by defective incomplete fusion of the


tracheal folds following separation or altered cellular growth during embryonic development.

CLINICAL MANIFESTATION
Excessive Salivation & Drooling Frothy white bubbles in babys mouth 3CS of TEF Chocking (when the baby is feeding) Coughing (when the baby is feeding) Cyanosis (when the baby is feeding) Vomiting Breathing Difficulty Abdominal Distension( very round full abdomen)

Apnea Increased respiratory distress Pneumonitis Regurgitation or Gagging Signs of gastro esophageal reflux Chronic respiratory problems

DIAGNOSTIC EVALUATION
History collection Physical Examination ECG Bronchoscopy Radiographic Studies (X-Ray, Ultrasound, CT scan, MRI)

PLAIN X-RAY WITH CONTRAST IN THE UPPER ESOPHAGUS SHOWING ATRESIA

Genetic testing. Molecular genetic testing Chromosome analysis EA/TEF may be suspected Prenatally by: Ultrasound examination Fetal MRI

EA may be detected Postnatally by: Failure to pass a nasogastric (NG) tube and radiographs that demonstrate coiling of the NG tube the pouch. Tracheal compression and deviation on plain chest radiographs. Absence of a gastric bubble on plain radiographs, which may suggest EA without a TEF or EA with a proximal TEF. Three-dimensional CT scanning.

MANAGEMENT MEDICAL MANAGEMENT


Treatment include:Maintanance of patient airway. Prevention of pneumonia. Gastric pouch decompression. Surgical repair of anomalies. Supportive therapy. Stop oral intake, start IV fluids.

SURGICAL MANAGEMENT transoesophageal fistula and esophageal atresia repair


Normal anatomy

Procedure, part 1

Part 2

Cervical Esophagostomy Bauginage Esophageal replacement procedure 1. Colon Interposition . 2. Gastric tube interposition. ET Tube intubation

Complication Of Surgery
Reaction to medicines. Breathing problems. Bleeding. Infection. Collapsed lung(pneumothorax). Food leakage from the area that is repair. Low body temperature(hypothermia). Narrowing of the repaired organ. Re-opening of the fistula.

Esophageal strictures / Esophageal stenosis


An esophageal stricture which is also known as esophageal stenosis is were the esophagus gradually narrows. Which can lead to problems with swallowing. Etiology The strictures are caused by inflammation in the esophagus which leads to scar tissue building up causing it to slowly over time narrow. It can be caused by or associated with gastroesophageal reflux or esophagitis Disfunctional lower esophageal sphincter Disordered motiity Hiatal hernia Post esophageal treatment i.e lasre therapy or photodynamic therapy. Pathology The esophagus in very server cases may be reduced to an opening the size of a pencil or smaller which then causes food and fluids to be delayed and to move across the opening into the stomach slowly.

Symptoms
A feeling that food is getting stuck in the esophagus difficulty in swallowing (this may gradually occur over time) Swallowing may be uncomfortable, (not necessarily painful) Bitter taste in the mouth Choking Coughing Shortness of breath Patient may be present with aspiration or recurrent pneumonia Weight loss

Diagnosis
Anamnesis ; history of dysphagia and regurgitations X-ray with barium meal CT scan Endoscopy

Treatment
Endoscopic dilation of the stenosis Dilation, which may be done at the same time as the upper endoscopy examination and diagnosis. Techniques Bougie - soft rubber or plastic dilators are moved across the stricture, gently opening it., and the surgeon with increase the size of the dilator accordingly. Guided wire - A thin wire, placed across the stricture, is used to guide increasingly wider dilators over it. Balloons - balloons shaped like sausages can be placed across the stricture. Then the balloon is sharply inflated to open the narrowed area. If gastroesphageal reflux has been the cause of the stricture it needs to be treated with proton pump inhibitor or H2 antagonist

Balloon and Bougie soft rubber or plastic dilators