malformations of oesophagus

8/12/2019 Malformations of Oesophagus

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DEFINITION

It is the failure of the esophagus to form a continuous passagefrom pharynx to stomach during embryonic development resultingin obstruction in infants normal swallowing routes.

INCIDENCE

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


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ETIOLOGYUnknownChromosomal anomalies (trisomy 18, trisomy 21, and trisomy 13)Failure of embryonic developmentDigestive tract problems(diaphragmatic hernia, intestinal atresia or

imperforated anus.)Congenital heart diseases(ventricular septal defect,tetralogy offallot or patent ductus arteriosus.)Renal and urinary tract problems(horseshoe or polycystickidney,absent kidney or hypospadias)


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"LONG GAP", PURE OR ISOLATED ESOPHAGEAL ATRESIA ESOPH AGEAL ATRESIA WITH PROXIMAL TEF

(TRACHEOESOPHAGEAL FISTULA) ESOPHAGEAL ATRESIA WITH DISTAL TEF (TRACHEOESOPHAGEA

FISTULA) ESOPHAGEAL ATRESIA WITH BOTH PROXIMAL AND DISTAL TEF (TWO TRACHEOESOPHAGEAL FISTULAS) TEF (TRACHEOESOPHAGEAL FISTULA) ONLY WITH NO

ESOPHAGEAL ATRESIA ESOPHAGEAL STENOSIS


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PathophysiologyEsophagus developed from first segment of embryonic gut.During the 4 th and 5 th 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.


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CLINIC L M NIFEST TION

Excessive Salivation & DroolingFrothy 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)

VomitingBreathing Difficulty

Abdominal Distension( very round full abdomen)


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ApneaIncreased respiratory distressPneumonitisRegurgitation or Gagging

Signs of gastro esophageal refluxChronic respiratory problems


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DI GNOSTIC EV LU TION

History collectionPhysical ExaminationECGBronchoscopy

Radiographic Studies (X-Ray, Ultrasound, CT scan, MRI)


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PLAIN X-RAY WITH CONTRAST IN THEUPPER ESOPHAGUS SHOWING ATRESIA


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Genetic testing. Molecular genetic testing Chromosome analysis

EA/TEF may be suspected Prenatally by:-

Ultrasound examination Fetal MRI


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EA may be detected Postnatally by:Failure to pass a nasogastric (NG) tube and

radiographs that demonstrate coiling of the NG tubethe pouch.

Tracheal compression and deviation on plain chestradiographs.

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.


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MEDIC L M N GEMENT

Treatment include:-Maintanance of patient airway.Prevention of pneumonia.Gastric pouch decompression.

Surgical repair of anomalies.Supportive therapy.Stop oral intake, start IV fluids.

SURGIC L M N GEMENT


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SURGIC L M N GEMENTtransoesophageal fistula and esophagealatresia repair

Normal anatomy


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Procedure, part 1 Part 2


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Cervical EsophagostomyBauginageEsophageal replacement procedure

1. Colon Interposition .

2. Gastric tube interposition. ET Tube intubation


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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.


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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 withswallowing.

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 tomove across the opening into the stomach slowly.


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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


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Diagnosis

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


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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 itneeds to be treated with proton pump inhibitor or H2 antagonist


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Balloon and Bougie soft rubber or plastic dilators

malformations of oesophagus

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