traheoezofag fistula

7/27/2019 Traheoezofag Fistula

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Tracheo-oesophagealfistulaHighlights

Summary

Overview

Basics

Definition

EpidemiologyAetiology

Pathophysiology

Classification

Diagnosis

History & examination

TestsDifferential

Step-by-step

Criteria

Guidelines

Case history

TreatmentDetails

Step-by-step

Guidelines

Follow Up
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History & exam

Key factors presence of risk factors maternal hx of polyhydramniosis inability to swallow secretions inability to pass nasogastric tube

Other diagnostic factors laboured respiration coughing
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type C

stabilization and surgical correction

type E nothing by mouth and surgical division of fistula

Treatment details

Summary Occurs in 1 of every 3000 live births. There are no known pre-disposing factors;

however, the condition is associated with VATERsyndrome.

Surgical intervention is required as a newborn. Patients may have long-term complications, such

as eating difficulties, GORD, and tracheomalacia.

Definition

Tracheo-oesophageal fistula is a malformation that arises from defectiveseparation of the oesophagus and trachea. This most commonly results in ablind-ending upper oesophageal pouch with a fistulous connection betweenthe distal oesophageal segment and the trachea.

EpidemiologyOne in every 3,000 births are affected, with a slight female preponderance.The majority of infants have Gross type C, comprising 85% to 90% of all

cases. Diagnosis typically occurs at or shortly following birth due to the blindproximal pouch. The second most common type is a pure atresia (Gross A),which comprises 4% to 7% of all tracheo-oesophageal fistulas. Gross type Doccurs 3% of the time. A much rarer form, the H-type fistula (Gross E), occursin approximately 2% to 3% of cases. This type of fistula may be undiagnoseduntil later in life. Type B occurs only 1% of the time.[2] [3] [4]
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AetiologyThe trachea and oesophagus arise from the common foregut, initially startingas a common tube, then separating into 2 distinct tracheal tubes. The tracheahas primitive lung buds at the caudal end of the primitive trachea. The

separation starts during the 4th week of gestation. A failure of normal divisioncan result in various defects including atresias and fistula formation. Thisfailure in organogenesis may be secondary to a failure of gene expression.Suspected genes include Sonic hedgehog homologue (Shh), a keydevelopmental gene or possibly other genes involved in development of therespiratory tract.[5] Other genes include forkhead box (FOX) gene family,Sry-related HMG box (SOX) gene family, and zinc finger protein (GLI2).

Pathophysiology

When oesophageal atresia is present, the infant is unable to swallow anyliquid, including his or her own secretions. The baby cannot drink until eitherthe atresia is repaired or the stomach is accessed via the anterior abdominalwall, or if a fistula is present, a connection between the airway and thealimentary tract. In type C, this connection can cause gaseous distension toform between the stomach and small bowel on x-ray. In rare cases, there isan associated intestinal atresia, which can lead to over-expansion andrupture of the stomach. Gastric contents can also reflux back through thefistula and cause aspiration, resulting in a chemical and bacterialpneumonitis.[6]

Motility of the oesophagus is always affected, with the distal segment havingthe most marked disordered peristalsis.

There is also a lower resting pressure of the lower oesophageal sphincterresulting in a higher incidence of GORD.

ClassificationGross classification: the surgery of infancy and childhood, 1954[1]Type A

Pure atresia (4% to 7%)
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Type B

Proximal fistula with distal atresia (1%)

Type C

Proximal atresia with distal fistula (85% to 90%)

Type D

Proximal and distal fistula (3%)

Type E

H-type fistula (2% to 3%)

Tracheo-oesophageal fistula withoutoesophageal atresia

MonitoringThe patients should be monitored for GORD, stricture and possible recurrentfistula. A post-operative upper GI is usually obtained at 3 to 6 months toevaluate the anastomosis, as well as the extent of reflux. Further studies areobtained depending on the patients' progress and overall condition. Antireflux

medications are usually continued until there is no clinical or radiographicevidence of GORD. Tracheomalacia may be a long-term issue. Cyanoticspells or episodes of less severe respiratory decompensation may warrantmore aggressive treatment. If the baby is having trouble with feeds orsecretions, or seems to have increased breathing effort, a barium swallowstudy should be obtained.


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Patient InstructionsParents should observe the baby for evidence of GORD such as frequentvomiting, persistent cough, gas, or colic (frequent crying or increasedagitation). Also, parents should look for aspiration or feeding intolerance.Parents should be instructed on resuscitation techniques before the infant isdischarged from the hospital. If the baby is having trouble with feeds orsecretions, or seems to have increased breathing effort, medical care shouldbe sought. Because of the oesophageal dysmotility, children may have poorfeeding.

Complications

Complicationhide all

failure to thrive

see our comprehensive coverage of Failure to thriveMay result from feeding difficulties secondary to poooesophageal stricture or severe gastro-oesophagea

GORD

see our comprehensive coverage of Disorders of infThis occurs due to poor oesophageal motility and a The initial method of therapy is medical treatment, banti-reflux procedure.[15] [16] [17] [7] [18]

chest wall malformation and scoliosisArises secondary to a former thoracotomy as a newby using a thoracoscopic approach to the surgical re

oesophageal stricture
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Typically, patients present with chocking, gagging andue to GORD and tension at the anatomosis site. Inblockers, and approximately 10% to 20% of patients

oesophageal dilations.[20] [18]

tracheomalacia

Often the cartilaginous wall of an infant with tracheowall may collapse during inspiration causing near comay result in symptoms which range from noisy brea

exertion to near death or cyanotic spells. If these symbe referred for bronchoscopy. If the patient has signworsen symptoms. Symptoms usually resolve as thecartilages become stiffer; however, severe cases maaortopexy or even tracheostomy.

PrognosisPatients with isolated tracheo-osophageal fistula without commonlyassociated congenital abnormalities (i.e., cardiac and chromosomal) have agood prognosis and survive to live healthy lives. Motility disorders andrespiratory complications may occur. In one study, 58% of adults with infantilerepair had GORD.[14]

Differential diagnosis

Condition

Differentiating

signs/symptoms Differentiating tests

Laryngeal cleft These

patients

can present

with

symptoms

similar to

This is best differentiated from a tracheo-oesophageal fistula b

oesophagoscopy.
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an H-type

fistula with

cyanotic

spells,

feeding

difficulties,

and

recurrent

chest

infections.

Case historyA newborn presents at birth with mild respiratory distress and increased oral

secretions. An attempt to pass a suction catheter meets with resistance. Anattempt to pass a replogle tube also meets with resistance.

Other presentationsMany patients are now diagnosed prenatally. Fetal ultrasound showspolyhydramnios and often no stomach bubble.

If the baby presents with a pure atresia (no fistula), the initial findings atdelivery are similar. The nasogastric tube coils in the upper oesophagealpouch; however, there is no air in the bowel as there is no connection to theairway. Associated anomalies may be present, such as cardiac, renal, limb,and spinal abnormalities.

History & examinationKey diagnostic factorshide allpresence of risk factors (common)

Key risk factors include trisomy 18 and 21 and afamily history, which may include Feingold'ssyndrome (autosomal dominant).


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maternal hx of polyhydramniosis (common) Polyhydramnios can be an indicator of some sort

of intestinal obstruction. An inability of the fetus to

swallow amniotic fluid results in polyhydramnios.In this scenario, the perinatologist assesses forevidence of a stomach bubble on prenatalultrasound. An inability to detect the stomach ishighly suggestive of an oesophageal atresia.

inability to swallow secretions (common) If an infant cannot tolerate his or her own

secretions or feed, this suggests a significantobstruction of the oesophagus. In these cases,an attempt should be made to pass a catheterinto the stomach. An inability to do so is highlysuggestive of oesophageal atresia.

inability to pass nasogastric tube (common) A nasogastric tube coiled in upper oesophageal

pouch may occur. If the diagnosis is unclear, 1mL of barium may be passed gently underfluorescent control to confirm proximal atresia.

Other diagnostic factorshide alllaboured respiration (common)

Mild or moderate respiratory distress is a non-specific finding occurring with many conditions inthe newborn. Tracheo-oesophageal fistula is oneof the more rare causes. Respiratory distress


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should lead to a chest x-ray to aid a properdiagnosis.

coughing (common)

Suggests difficulty swallowing or toleratingsecretions. Like laboured breathing, this finding isnon-specific.

choking (common) Choking suggests difficulty swallowing or

tolerating secretions, but is an extremely non-specific finding.

VACTERL syndrome (common) Can form part of the more extensive VACTERL

syndrome: vertebral defects, imperforate anus,cardiac defects, tracheo-oesophageal fistula,radial and renal dysplasia, limb anomalies.

cyanosis (uncommon) This finding is relatively rare, but increasing

respiratory distress can present with time as thestomach becomes more distended with air and/orif gastric contents reflux back through the fistula.

Risk factorshide all

Weaktrisomy 18 and 21

Present in up to 6% of patients who haveassociated malformations in other organ systems.


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Familial or syndromic cases of oesophagealatresia account for less then 1% of the total.[7]

family history

Feingold's syndrome is an associated diseasewith a rare autosomal dominant inheritancepattern.[2] [8]

Diagnostic tests1st tests to orderhide all

Test

prenatal ultrasound

A part of routine prenatal screening or in cases of sufamilial syndrome. If evidence of polyhydramnios, ul

x-ray chest and abdomen

Evidence of respiratory distress or poor handling of snasogastric tube warrants an x-ray. An x-ray of the cobtained immediately after birth in patients who are fistula on prenatal ultrasound

Tests to considerhide all

Test

bronchoscopy
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Suspicion of an H-type fistula due to recurrent aspiraalso be performed prior to repair of classic type C fis

Also helpful to identify tracheo-oesophageal fistula, adebate as to whether this procedure is really necessstress prior to surgical repair.

oesophagoscopy

Performed with suspicion of H-type tracheo-oesopha

barium swallow

Performed if a suspicion of H-type fistula. Patients poften difficult to visualise the fistula.

Step-by-step diagnostic approachThe diagnosis is based on clinical suspicion. In general, newborns presentwith feeding difficulties or respiratory distress. Inability to pass a nasogastrictube is often the first clue of an oesophageal atresia. As prenatal ultrasoundhas become more common and sensitive, increased numbers of theselesions are being detected in utero.

Clinical evaluationInfants who are born without a prenatal diagnosis may be born completelyasymptomatic or with varying degrees of respiratory distress, such aslaboured breathing, coughing, choking, and cyanosis. The first clue that anoesophageal atresia exists may be an inability to tolerate feedings. The infantis excessively mucusy and requires frequent suctioning. Patients with the


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more rare form of H-type fistula may go undetected until later in life whenthey present with recurrent episodes of aspiration pneumonia, feedingdifficulties or chronic cough. Additionally, a maternal history ofpolyhydramniosis may indicate the fetus' inability to swallow amniotic fluid,suggesting a blockage.

The most insidious type is E, which can be very difficult to diagnose. Thesepatients often present with repeated episodes of aspiration with no clearaetiology. This condition is difficult to diagnose and may be missed onrepeated exams.[4] [9] [10]

It may form part of the more extensive VACTERL syndrome: vertebraldefects, imperforate anus, cardiac defects, tracheo-oesophageal fistula,radial and renal dysplasia, limb anomalies.

Prenatal ultrasoundA small or absent stomach bubble and polyhydramnios has a sensitivity of56% in predicting oesophageal atresia. All infants born to a mother withpolyhydramnios should have a nasogastric tube passed at birth.

Investigations at birth

An x-ray of the chest and abdomen should always be obtained immediatelyafter birth in patients who are suspected to have a tracheo-oesophagealfistula on prenatal ultrasound. An x-ray is also warranted if there is evidenceof respiratory distress or poor handling of secretions, or an inability to passnasogastric tube. The x-ray should be completed with a nasogastric tube inplace. The nasogastric tube should be stiff and wide bore. The x-ray will showthe nasogastric tube coiled in the upper oesophageal pouch with or withoutgas in the stomach and bowel.

A barium swallow is only necessary if an H-type fistula is suspected or in acase of oesophageal atresia, where a fistula may occur between the upperpouch and trachea. This is extremely rare and as a result, this study is oftenunnecessary.
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Some surgeons perform a bronchoscopy at the time of surgical repair to lookfor an upper pouch fistula. As this is extremely rare, this test is generallyunwarranted.

Bronchoscopy and oesophagoscopy are performed in cases of suspected H-type fistulas, as these lesions are hard to diagnose and may be missed on anupper GI series.

Diagnostic criteria

Prenatal ultrasound

Polyhydramnios and a small or no stomach bubble may be seen as a part ofroutine prenatal screening, or in cases of suspected chromosomal anomalyor familial syndrome.

Treatment Options


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Patient

group

Treatmentline Treatmenthide all

type A 1st stabilization and gastrostomy

Surgical repair of theoesophagus of type Apure atresia at birth isoften challenging.

However, as there is noconnection to the trachea,these patients are lesssusceptible to acuterespiratory distress.

First, type A can be

managed with anasogastric tube in theupper oesophagealpouch. Within the first fewdays of life, a gastrostomytube is placed to allow for

enteral feeds and thestomach contrast study toidentify the length of thelower pouch.

If the length between theproximal and distal


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Treatment approachThe majority of patients require surgery to ligate the fistula and establishoesophageal continuity. The condition is not compatible with life as thepatient cannot eat and is at significant risk for aspiration. Patients with pure

atresia and no fistula also require surgery but can be managed either bydirect gastric feeds or IV hyperalimentation with a delayed repair of theoesophagus.

Gross classification: the surgery of infancy andchildhood, 1954

Type A: pure atresia (4% to 7%)

Type B: proximal fistula with distal atresia (1%)

Type C: proximal atresia with distal fistula (85%to 90%)

Type D: proximal and distal fistula (3%)

Type E: H-type fistula (2% to 3%);tracheoesophageal fistula without esophagealatresia.[1]

Type ASurgical oesophageal repair at birth is often challenging. However, without aconnection to the trachea, these patients are less susceptible to acuterespiratory distress.

First-line patients may be managed with a nasogastric tube in the upperoesophageal pouch, providing continuous suction. Within the first few days of
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life, a gastrostomy tube is placed to allow for enteral feeds and the stomachcontrast study to identify the length of the lower pouch. Bronchoscopy ismandatory in cases of isolated atresia (gross A) to rule out an upper pouchfistula (10% -15% of cases). If the length between the proximal and distaloesophageal segments is 4 vertebral bodies or longer, then the patient is

allowed to grow prior to attempting a repair. Growth is monitored by usingcontrast radiographs.

If the gap between the proximal and distal segments of the oesophagus isless than 2 vertebral bodies, primary oesophageal repair can be performedimmediately or around 2 to 3 months of age. Standard therapy includes aright thoracotomy, although this procedure is now performedthoracoscopically. Thoracoscopic treatment is available in limited centres andonly for experienced endoscopists.

In cases of a very long gap, various techniques have been designed tostretch or increase the oesophageal length.

In some cases of a failed oesophageal repair or an extremely long gap,oesophageal replacement surgery is planned.

Type BThese patients cannot eat and are at risk for repeated aspiration of oralsecretions due to a proximal fistula. The patients can be managed initially bya suction catheter in the upper oesophageal pouch which should limitsecretions going into the trachea. Surgery should take place in the first 24 to48 hours of life to ligate and divide the fistula and establish oesophagealcontinuity.

Type CThe first-line treatment is with surgical correction, aimed at dividing thetracheo-oesophageal fistula to prevent lung aspiration. Then, anastomosis ofthe 2 oesophageal ends is completed, in order to establish continuity of theoesophagus. Classically, this procedure has been performed via a rightthoracotomy incision but is now performed in many centres using athoracoscopic approach, which avoids the inherent morbidity of a


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thoracotomy. This operation should be performed within the first 24 hours oflife to prevent complications of aspiration and abdominal distension. Beforesurgery, the patient is stabilized by placement of a nasogastric tube todecompress the upper blind pouch.

Type DThis type is extremely rare and involves not only oesophageal atresia butfistulas in the proximal and distal pouches. A suction catheter should beplaced prior to surgery to decompress the upper pouch and limit thesecretions entering the trachea. Surgical management includes division of thefistulas and an anastomosis of the proximal and distal oesophageal pouches.

Type EThese patients often present in late childhood or in early adulthood withevidence of choking, gagging or recurrent aspiration. Once diagnosed, thepatient should be kept nothing by mouth until the fistula is divided. Through aright neck incision at the level of the thoracic inlet, fistula repair is performed.The fistula can also be reached thoracoscopically through the right chest.

1

traheoezofag fistula

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