bringing excellence to life oesophageal atresia dr. sam chippington martin churchill-coleman

Bringing excellence to life

OESOPHAGEAL ATRESIA

Dr. Sam ChippingtonMartin Churchill-Coleman

Atresia - is the absence of a usual opening in a tubular structure

Embryology – the exact cause of oesophageal atresia is not known but both the oesophagus and trachea originate from the primitive foregut and this is thought to be a separation defect.

Occurrence – Approximately 1:4000 live births

PRENATAL DIAGNOSIS

Ultrasound diagnosis is indicative rather than absolute

Obstetric Ultrasound may show unexplained polyhydramnios

Absent stomach or small stomach

Early diagnosis allows time and place of delivery to be planned so that early paediatric surgical correction and neonatal care is available. This also gives time for preparation and education of the parents.

Normal foetal stomach

Umbilical cord

Gastric bubble

Cross sectional view of fetal abdomen with vertebral column to left of the image, the gastric bubble above and the umbilical cord to the right.

Absence of foetal stomach bubble

Umbilical cord

Absence of gastric bubble

Small foetal stomach bubble

Umbilical vein

small gastric bubble

“collapsed”Spine

FOETAL MRI

Some centres carry out 3rd trimester MRI – using single shot rapid acquisition T2 weighted images – the diagnosis is considered positive if the upper oesophagus is dilated and the lower oesophagus is not demonstrated

CLINICAL DIAGNOSIS

After birth these babies often appear to blow bubbles when attempting to swallow mucous and saliva

If these signs go unnoticed the first feed is almost always accompanied by coughing, choking and cyanosis.

In these cases a replogle tube is passed – typically this will reach 10-12cm before reaching the blind end of the upper pouch.

TYPES OF OESOPAGEAL ATRESIA, TRACHEAL FISTULA OR COMBINATION OF THE TWO

5 TYPES OF MALFORMATION

87% have oesophageal atresia with distal tracheo-oesophageal fistula

8% Isolated atresia with no fistula

4% Isolated tracheo-oesophageal a ‘H’ Type fistula with no atresia

1% Atresia with upper pouch fistula

1% Atresia with upper and lower pouch fistula

NEONATAL ABDOMEN CHEST RADIOGRAPHS

CXR – will normally demonstrate a dilated upper pouch containing a replogle tube

ABDOMEN – a normal bowel gas pattern will be visible where there is connection via fistula to the lower oesophagus

Therefore there are only two types of malformation where the abdomen will be gasless – the isolated atresia without fistula and the fistula to the upper pouch of the oesophagus

Demonstrating an H type Fistula

HEAD FEET

DELAYED DIAGNOSIS

Diagnosis of four of the types is easily made after attempting to feed a new baby

However the ‘H’ Type Fistula because there is no atresia may not be identified for many years

Often ‘H’ Type fistula present with recurrent chest infections or more dramatically when diving into a swimming pool

‘H’ Type fistulae are demonstrated by oesophagogram – taking a series of images with the patient prone and injecting water soluble contrast through a naso-gastric tube which is withdrawn from the stomach to the mouth so that the whole length of the oesophagus is interrogated.

HISTORY OF TREATMENT

Oesophageal atresia is not compatible with life unless it is surgically repaired

Before 1939 when the first successful repair took place this diagnosis was fatal

Since that time surgical techniques and anaesthetics have improved and the great majority of these patients survive to lead a normal life

Most patients nowadays undergo a primary anastomosis in the first few days of life.

Large gaps between the upper and lower pouches are problematic and used to be bridged by colon transplants and J tubes fashioned from the lesser curvature of the stomach.

ASSOCIATED DEFECTS

Associated congenital abnormalities are discovered in approximately one half of patients with oesophageal atresia

The acronym VACTERL has been used to describe the condition of multiple anomalies in these infants

VERTEBRAL – Hemivertebrae and scoliosis

ANORECTAL MALFORMATION

CARDIAC DEFECTS – VSD, Patent Ductus Arteriosus and Tretralogy of Fallot

TRACHEO

ESOPHAGEAL (American esophageal)

RENAL TRACT – Ectopic kidneys, horseshoe, duplex systems, renal agenesis, urethral malformations and hypospadias

LIMB defects – radial agenesis most common

In addition to the vacterl anomolies there is increased incidence of Duodenal atresia, malrotation, intestinal malformations, Meckel’s diverticulum and annular pancreas.

Air filled upper oesophagus

Replogle tube

Hemivertebra

Oesophageal Atresia First described 1703

1936 – First surgical repair 1939 – First successful surgical

repair Gastrostomy inserted, delayed

surgical repair

1936 – First surgical repair 1939 – First successful surgical

repair Gastrostomy inserted, delayed

surgical repair

1941 – First primary oesophageal anastomosis and ligation of tracheo-oesophageal fistula

1945-1965 Focus on successful repair in

otherwise healthy neonates (and birth weight > 2.5kg)

By 1965 Success rate 80-90%

1965- 1990 Refinement of the procedure

Low birth weight Co-existing morbidity

Surgical Anastomosis

Newest approach

Fewer musculoskeletal sequelae Winging of the scapula Asymmetrical chest wall Thoracic scoliosis Breast maldevelopment

Primary oesophageal anastomosis Within 48 hours of birth

Delayed primary oesophageal anastomosis Unfit for surgery Other associated anomalies

e.g.cardiac

Staged repair Long gap oesophageal atresia

‘Stretch’ upper pouch Mechanical Electromagnetic

Wait for growth Colonic Interposition

Acute -Anastomotic leak

Pathogenesis Use silk sutures at the anastomosis Excessive anastomotic tension Excessive distal oesophageal

mobilisation Colonic interposition – graft necrosis

Missed TOF Present with recurrent chest infection

Recurrent TOF Erosion through site of previous

repair Anastomotic suture line leak

Anastomotic Stricture -40% of repairs

Pathogenesis Anastomotic leak Two layer anastomosis Anastomosis under tension Silk sutures Gap length at presentation Associated gastro-oesophageal reflux

Anastomotic stricture

Long Term Complications

Oesophageal function Disordered oesophageal motility

Barium swallow Oesophageal function tests

Manometry pH studies

Oesophageal Function

Abnormal oesophageal motor function

No progressive peristalsis Lack of co-ordinated peristaltic

stripping wave Oesophageal contractions

simultaneous

Can involve whole oesophagus or distal two thirds

Moderate- severe gastro-oesophageal reflux

Incompetent gastro-oesophageal sphincter

? Iatrogenic hiatus hernia

Oesophagitis Symptoms persist into adult life

Abnormal oesophageal motor function No progressive peristalsis

Moderate- severe gastro-oesophageal reflux

Reflux and disordered motility Oesophagitis Pulmonary symptoms

Long Term Complications

Respiratory

Recurrent bronchitis Persistent cough - ‘TOF’ cough Pneumonia

Commonest first 8 years of life

Tracheomalacia (reported in up to 10%)

Respiratory Symptoms

Recurrent inhalation of food Consequence of dysmotility and

reflux Fat seen in tracheal secretion

aspirates

Association between oesophageal stricture and recurrent bronchitis

In Summary

Survival for patients with OA and TOF good

Symptoms relating to dysmotility and reflux have a significant impact Frequently persist into adulthood

bringing excellence to life oesophageal atresia dr. sam chippington martin churchill-coleman

life slide

life atresia

oesophagus slide

life oesophageal atresia

lower pouch fistula

upper pouch fistula

isolated atresia

life clinical diagnosis

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