Esophagus, Esophageal Atresia, TEF

Question 1

What is the best surgical approach to repair the most common type of TEF?

A. Left thoracotomy

B. Right thoracotomy

C. Left cervicotomy

D. Right cervicotomy

E. Midline laparotomy

Answer 1

ANSWER: B

Five types of TEFs exist.

Type C, esophageal atresia with a distal TEF, is the most common type accounting for 80%–90% of all TEFs.

Radiographs will show a coiled orogastric tube in the proximal esophagus and air within the stomach.

The best access to the esophagus for repair of this fistula is through a right thoracotomy.

As part of the preoperative workup, an echocardiogram should be obtained to evaluate for congenital cardiac anomalies as well as a right-sided aortic arch.

If identified, repair can be performed through a left-sided thoracotomy.

Type A (5%–10%) is pure atresia without a fistulous connection. Radiographs will also show a coiled orogastric tube in the proximal esophagus, but there is no air within the stomach.

Type E (4%), or H type, is a TEF without atresia. This often presents in the first few days of life with repeated choking with attempted feeds and cyanotic events as the baby aspirates into the lungs. However, this can be missed in the newborn period and may present later in life with recurrent aspirations or pneumonias. These fistulas are located at the thoracic inlet, and a right cervicotomy allows for the best access for fistula ligation.

The final two types of TEF, type B atresia with both a proximal and distal TEF and type D atresia with a proximal TEF, are both extremely rare.

The surgical approach should be through a right thoracotomy.

Question 2

Which of the following is a complication after the repair of a TEF?

A. Anastomotic leak

B. Esophageal stricture

C. Recurrent fistula

D. Gastroesophageal reflux

E. All of the above

Answer 2

ANSWER: E

COMMENTS: All of the options are potential complications following the repair of a TEF.

The early complications include anastomotic leak, esophageal stricture, and recurrent fistula, while late complications include gastroesophageal reflux disease (GERD) and tracheomalacia.

Esophageal stricture is the most common early complication and may occur in up to 80% of cases.

This complication is managed with serial esophageal dilations.

If the stricture fails to respond, resection of the anastomosis with a new anastomosis is warranted.

Anastomotic leak is another early complication, occurring in about 15% of repairs.

The majority of these leaks are not clinically significant and may be managed with continuous drainage and parenteral nutrition until the leak has sealed.

Large leaks within the first few days of repair are generally the result of a technical error or ischemia of the anastomosis; surgical revision is advised to avoid development of a tension pneumothorax and mediastinitis.

GERD is by far the most common late complication and is thought to be related to shortening of the intraabdominal portion of the esophagus.

Question 3

An 8-h-old newborn has mild respiratory distress and excessive drooling. An abdominal radiograph shows a complete lack of air in the GI tract. What is the most likely diagnosis?

A. Bilateral choanal atresia

B. Pyloric atresia

C. Duodenal atresia

D. Esophageal atresia with a distal TEF

E. Esophageal atresia without a TEF

Answer 3

ANSWER:

E

Question 4

How common is esophageal atresia (EA) with or without tracheoesophageal fistula (TEF)?

Answer 4

The worldwide prevalence of EA is estimated at 2–3 per 10,000 births, and 70–90% are associated with TEF.

Spontaneous intrauterine fetal demise occurs in ~3% of cases.

Question 5

A 3000-g infant is born with esophageal atresia and a distal TEF. If the infant does not exhibit respiratory distress and associated anomalies are not present, which of the following is the preferred treatment?

A. Gastrostomy, cervical esophagostomy, and delayed repair

B. Gastrostomy, sump tube drainage of the proximal pouch, and delayed repair

C. Fistula ligation and delayed esophageal repair

D. Division of the fistula with primary esophageal anastomosis

E. Primary repair with colonic interposition

Answer 5

ANSWER:

D

COMMENTS: The timing and type of surgical intervention for esophageal atresia and TEF depend on the maturity of the infant and associated cardiorespiratory problems or other congenital anomalies.

Medically stable infants weighing more than 2500 g are treated by primary repair with fistula division, closure of its tracheal end, and end-to-end anastomosis of the esophageal segments.

Unstable infants with respiratory issues are treated by gastrostomy and sump drainage of the blind proximal pouch until they are ready for repair.

A distal TEF often results in the loss of ventilatory pressure or retrograde aspiration of gastric contents into the lungs, further exacerbating respiratory compromise.

Therefore some infants may benefit from primary fistula ligation without esophageal repair, with or without gastrostomy placement.

The final esophageal repair is performed after cardiorespiratory recovery.

Question 6

The VACTERL association most commonly includes which of the following?

A. Ankylosis

B. Imperforate anus

C. Eye deformities

D. Congenital cystic lung malformation

E. Choanal atresia

Answer 6

ANSWER:

B

COMMENTS: Neonates have a gasless GI tract at birth. They start to swallow soon after birth, and air reaches the colon within 6 to 12 h.

In pure esophageal atresia without an associated fistula, swallowed air has no access to the GI tract, and so abdominal films show a gasless abdomen.

Esophageal atresia is also suggested when an infant drools excessively because of an esophageal obstruction or spits up during attempted feedings.

When an orogastric tube is passed in an infant with esophageal atresia, a chest radiograph shows the tube coiled in a blind pouch in the chest.

About 85%–90% of patients with a tracheoesophageal malformation have a blind proximal pouch with a distal TEF, also known as TEF type C.

Respiratory symptoms are secondary to aspiration from the esophageal pouch or retrograde reflux of gastric contents through the fistula into the lungs.

In esophageal atresia with a TEF, the inspired air reaches the stomach and small bowel through the TEF.

Contrast-enhanced studies and a bronchoscopy may be useful in select cases to confirm the diagnosis and demonstrate the location of the fistula.

Recognition of the anatomy of the anomaly is important for establishing appropriate initial treatment and definitive repair.

Air fills the stomach but fails to pass into the duodenum and small bowel in neonates with pyloric atresia, a rare congenital anomaly.

Radiographic studies show extreme distention of the stomach with air-fluid levels.

Choanal atresia is narrowing or blockage of the posterior nasal airway by soft or boney tissue.

It is a congenital condition that is due to the failure of the recanalization of the nasal airway during embryologic development.

Neonates, being obligatory nasal breathers, have major respiratory problems when born with bilateral choanal atresia but do not have difficulty swallowing air.

The VACTERL association refers to a group of commonly associated congenital defects: vertebral anomalies, imperforate anus, cardiac defects, tracheoesophageal fistula, radial and renal malformations, and limb defects.

Question 7

What is the etiology of EA?

Answer 7

EA is a congenital anomaly that results from abnormal embryonic development of the foregut. The exact mechanisms are unknown, yet the process likely involves a combination of genetic, environmental, and biomechanical factors.

Question 8

Which genetic syndromes and other congenital anomalies are associated with EA/TEF?

Answer 8

The most common congenital anomalies associated with EA/TEF involve the Vertebral, Anorectal, Cardiac, TEF, Renal and Limb abnormality (VACTERL) spectrum of disorders (Table 5.1).

Nearly 70% of infants with EA will have at least one other associated anomaly, most commonly cardiac (~35%), with many patients having multiple.

Associated chromosomal abnormalities include trisomies 18 and 21, as well as CHARGE syndrome.

Question 9

What are the relationships between prematurity, low birth weight, and EA/TEF?

Answer 9

Up to one-third of patients with EA are born prematurely, likely due to associated polyhydramnios.

Low birth weight infants with EA have a higher risk of mortality than their normal weight counterparts, and often surgical repair is delayed until infants reach 1,500–2,000 g to lower the risk of operative morbidity.

Question 10

What is the mortality associated with EA/TEF?

Answer 10

Prior to the first successful EA/TEF repair in 1941, mortality of this disorder was 100%.

Survival has steadily increased over time and is currently>90%.

Mortality is primarily related to the associated comorbidities of congenital heart disease and prematurity.

Question 11

How is EA classified?

Answer 11

The most widely used classification system for EA/TEF is the Gross classification, which includes types A–E.

The most common malformation is Gross Type C, which involves EA associated with a distal TEF.

Gross Type E has no atresia but instead an isolated, or “H-type” TEF.

Question 12

Other congenital anomalies associated with esophageal atresia and their respective incidences?

Answer 12

Associated anomaly and incidence (%):

Cardiac 35%
Renal 23%
Vertebral 22%
Anorectal 20%
Limb 14%
Chromosomal 11%

Question 13

How is EA diagnosed?

Answer 13

Prenatal diagnosis of EA is about 20% in patients with EA/TEF and over 50% in patients with isolated EA.

Prenatal imaging findings that are indicative of EA include polyhydramnios, a small or absent stomach bubble, and a dilated upper esophageal pouch (“pouch sign”).

The sensitivity and specificity of prenatal diagnosis are significantly improved with MRI over ultrasound.

After birth, infants with EA commonly present with early feeding intolerance and increased oral secretions that can lead to respiratory compromise.

The diagnosis of EA is confirmed with failed attempts at orogastric tube placement followed by an abdominal x-ray demonstrating coiling of the enteric tube within the proximal esophageal pouch.

The presence of an associated distal TEF is indicated by the finding of gas within the stomach and/or intestine.

Question 14

What further clinical workup is recommended following the diagnosis of EA/TEF?

Answer 14

Further workup is aimed at determining EA type, evaluating for the presence of a TEF, estimating esophageal gap length (distance between the proximal and distal esophageal pouches), and assessing for other anomalies (VACTERL).

An x-ray of the chest and abdomen can diagnose a TEF if intestinal gas is present, estimate the length of the proximal esophageal pouch by placing gentle downward traction on the oral-esophageal (OE) tube, and identify vertebral anomalies.

Preoperative laryngotracheobronchoscopy is used to assess for coexisting laryngeal clefts and to identify the location of a TEF.

Occasionally pre-operative fluoroscopy is utilized to diagnose EA and determine EA type and gap length.

Echocardiogram is essential prior to EA repair to identify congenital heart disease and to determine the side of the aortic arch.

Approximately 5% of EA/TEF patients will have a right sided aortic arch, and this may alter the decision regarding which side the surgical repair should be performed on.

Lastly, renal ultrasound and anorectal exam are performed to evaluate for renal anomalies and anorectal malformations.

Question 15

How is long gap atresia defined and what is its impact on management?

Answer 15

The term “gap length” refers to the distance between the proximal and distal esophageal pouches, and primary EA repair can be difficult or impossible to achieve if the gap is long.

There is no consensus on the specific definition of long gap EA, however it is often described by the number of vertebral bodies present between the two ends of esophagus (typically 2–5), the gap length in centimeters (typically 2–5 cm), or based on a surgeon’s intraoperative gestalt.

Question 16

How can one assess esophageal gap length preoperatively?

Answer 16

Gap length can be evaluated preoperatively by plain film or fluoroscopy.

The length of the proximal pouch is estimated by placing gentle downward traction on the OE tube while an x-ray is taken.

In patients with Type C or D EA/TEF, the esophageal length can be estimated by using the carina on x-ray as a landmark for the proximal extent of the distal pouch.

Therefore, esophageal gap length can be predicted by the distance between the distal extent of the proximal pouch and the carina on x-ray.

In patients with Type A or B EA that have a gastrostomy tube in place, fluoroscopy can be utilized to determine the length of the distal pouch either via contrast administration alone or by inserting a guidewire, endoscope, or metal probe retrograde through the g-tube site and advancing into the distal pouch.

Question 17

What are the important aspects of preoperative management in the setting of EA/TEF?

Answer 17

Immediately following a diagnosis of EA, the patient should be positioned in reverse-Trendelenburg position and an OE tube placed to continuous suction with its tip located just above the distal end of the proximal esophageal pouch.

Adequate decompression of the proximal pouch is essential to prevent aspiration and further respiratory compromise.

Respiratory status should be carefully monitored as intubation and mechanical ventilation may be necessary.

Non-invasive positive pressure strategies should be avoided as they can result in significant gastric distention when a TEF is present.

If severe gastric distention occurs and impedes ventilation, emergent percutaneous decompression of the stomach using a large-bore needle can be lifesaving.

Other options for management of severe gastric distention include placing a gastrostomy tube, advancing the endotracheal tube past the fistula in intubated patients, and finally ligating and dividing the TEF if all else fails.

Question 18

When should initial TEF ligation be considered prior to definitive EA
repair?

Answer 18

TEF ligation may be necessary prior to definitive EA repair if the fistula is causing physiologic compromise and EA repair is delayed due to patient size, long gap length, or other reasons.

Question 19

How do outcomes of thoracoscopic versus standard thoracotomy approaches compare for EA/TEF repair?

Answer 19

A recent meta-analysis comparing open and thoracoscopic approaches to esophagoesophagostomy for EA/TEF repair found no differences in outcomes including anastomotic leak rate, esophageal stricture rate, pulmonary complications, time to first oral feeding, or blood loss.

Thoracoscopic repair was found to decrease post- operative ventilation time and length of stay, though operative times were significantly longer compared to open repair.

Question 20

What are surgical options for long gap EA if primary esophagoesophagostomy is not possible?

Answer 20

The least invasive option is placement of a gastrostomy tube followed by delayed primary repair, generally after waiting a period of ~12 weeks.

This theoretically allows the esophageal pouches to lengthen from somatic growth as well as a combination of pooled oral secretions (proximal pouch) and gastroesophageal reflux (distal pouch).

Other options for esophageal lengthening include circular or spiral myotomy, the Kimura extrathoracic lengthening procedure, and the Foker staged suture-traction method.

Finally, esophageal replacement with a stomach, colon, or small bowel interposition graft may be required if esophageal preservation is not feasible.

Question 21

What postoperative complications are most common following EA/TEF repair?

Answer 21

Over 60% of patients undergoing EA/TEF repair will have a post-operative complication, with the most common being anastomotic stricture (>40%).

Other common complications include anastomotic leaks (~20%), recurrent fistulas (~5%), and vocal cord paralysis (~5%).

Recent research has found associations between the use of transanastomotic esophageal tubes with increased rates of anastomotic stricture, as well as placement of prosthetic material between the esophageal and tracheal suture lines with increased rates of anastomotic leak.

Question 22

What long-term comorbidities are associated with EA following repair?

Answer 22

Dysphagia, esophageal dysmotility, gastroesophageal reflux (GERD) and respiratory conditions such as wheezing and recurrent infections are common problems that persist following EA/TEF repair and must be managed over a lifetime.

Question 23

Are there guidelines for the management of GERD and subsequent screening for Barrett esophagus and malignancy later in life?

Answer 23

Due to the nearly 4-fold increased risk of Barrett esophagus identified in adults with repaired congenital EA and the relative lack of reported GERD symptoms in this population, current guidelines recommend performing lifelong endoscopic surveillance (with multistaged biopsies) starting before the age of 15 years.

Further, fundoplication should be considered in the setting of recurrent esophageal stricture, respiratory complications, or medically refractory GERD.

Question 24

What is known about the quality of life (QOL) for individuals who were born with EA?

Answer 24

Though there is relatively little data regarding QOL in older patients born with EA, overall adult survivors report their health-related QOL to be equivalent to that of the general population.

Question 25

Regarding achalasia cardia, which one is false?

A. There is failure of relaxation of the lower oesophagus.

B. Histochemistry shows an increase in neuropeptide, VIP and gastrin.

C. Nifedipine and calcium channel antagonists are used as medical treatment.

D. Pneumatic dilatation is one of the treatments.

E. Myotomy over 4–5 cm is the surgical treatment.

Answer 25

B. Histochemistry shows decrease, not increase, in neuropeptide, VIP and gastrin.

Question 26

Among the diagnostic tools of achalasia cardia, which of the following is true?

A. CT scan is the investigation method of choice.

B. No finding appears on plain X-rays.

C. A barium meal shows dilatation of lower oesophagus.

D. Oesophageal manometry shows pressure greater than 30 mmHg.

E. Monitoring of pH over twenty-four hours is diagnostic.

Answer 26

D.

Barium meal shows narrowing of lower oesophagus.

CT is not the investigation of choice, plain x-rays may show air-fluid level in lower oesophagus.

Barium meal shows rate tail appearance due to funneling and narrowing of oesophagus.

24-h pH monitoring is required for diagnosis of gastroesophageal reflux.

Question 27

In surgery for achalasia cardia, which one is false?

A. Preservation of anterior vagus nerve.

B. preservation of posterior vagus nerve.

C. Incision of 4–6 cm long made in Myotomy.

D. At least 50 per cent of the circumference of oesophageal mucosa is separated from constricting muscle.

E. Avoidance of fundoplication.

Answer 27

E. Loose (floppy) fundoplication is part of the procedure.

Question 28

Regarding complications of surgery for achalasia cardia, which one is false?

A. Mediastinitis is due to failure of detection of mucosal perforation.

B. Recurrence of symptoms appear if muscle is not separated at least 50 percent of circumference of oesophagus.

C. Gastroesophageal reflux (GER) occurs due to inadequate fundoplication.

D. Dysphagia for liquid develops due to tight fundoplication.

E. GER is more common than residual or recurrence of achalasia.

Answer 28

E. Gastroesophageal reflux incidence is about 15 percent, while residual or recurrence is 25 percent.

Question 29

What is the traditionally proposed embryology behind Esophageal Atresia with/without TEF?

Answer 29

Theory of invaginating longitudinal tracheoesophageal folds merging to form a septum (caudally to cranially)

4th week AOG:
- Foregut starts to differentiate into a ventral respiratory part, and a dorsal esophageal/digestive tract.
- Laryngotracheal diverticulum invaginates ventrally into the mesenchyme.
- The ventral respiratory system separates from the esophagus by the formation of lateral tracheoesophageal folds that fuse in the midline and create the tracheoesophageal septum.

6th-7th week AOG:
Separation between trachea and esophagus is complete.

Incomplete fusion of the folds results in a defective tracheoesophageal septum and an abnormal connection between the trachea and esophagus.

However, there is little evidence to support this, hence newer theories have also been proposed.

Question 30

What are alternative theories for embryology behind EA/TEF?

Answer 30

1) Imbalance in the growth of cranial and caudal folds

In chick embryo studies, cranial and caudal folds were found in the region of tracheoesophageal separation.

EA/TEF would then be due to an imbalance in the growth of these folds.

2) Foregut occlusion and failure of recanalization. Rat studies suggest that EA/TEF results from disturbances in either epithelial proliferation or apoptosis.

3) EA may be a component of cephalic neurocristopathy.

The observation that there is a clear association of neural crest-implicated cardiovascular anomalies (aortic arch, conotruncal and membranous ventricular septal defects), as well as thymic, thyroid, parathyroid, and facial malformations with EA, as seen with the DiGeorge syndrome, suggests that the pathogenesis of EA may be related to defective pharyngeal arch development.

4) The distal fistula tract and esophagus are of possibly of respiratory origin, and defects in lung morphogenesis account for this aspect of the EA-TEF anomaly.

5) Environmental teratogens have also been implicated.

Question 31

What is the genetic theory behind the embryology of EA/TEF?

Answer 31

More recent studies show that ectopic expression of sonic hedgehog occurs in the tissues between the notochord and the gut.

Knockout mice models have helped elucidate the functions of different genes in the development of foregut aberrations such as EA/TEF.

Also, the relationship between BMP4 (bone morphogenic protein) and Nog, the gene encoding noggin (which is a BMP antagonist), may also have an impact on the development of EA/TEF.

Question 32

What are some environmental teratogens implicated in the pathogenesis of EA+TEF?

Answer 32

EA has occurred in infants born to mothers with:

1) prolonged exposure to contraceptive pills
2) exposure to progesterone and estrogen during a pregnancy
3) hyperthyroidism and diabetes
4) intrauterine exposure to thalidomide and diethylstibestrol
5) use of methimazole in early pregnancy
6) maternal phenylketonuria
7) infant with fetal alcohol syndrome

Question 33

What is the birth incidence and epidemiology of EA/TEF?

Answer 33

1 in 2500-3000 live births
Slight male preponderance

Risk for a 2nd child with EA/TEF among parents of one affected child: 0.5-2%, increasing to 20% when more than one child is affected.

Empirical risk of an affected child born to an affected person: 3-4%

Relative risk for EA/TEF in twins (vs singletons): 2.56 (high risk among twins of same gender)

Lack data but reported:
White ethnicity
First pregnancy
Increasing maternal age (twofold increased risk for women 35-40yo, threefold for >40yo)
Rate of multiple births high for each type of EA-TEF anomaly
In vitro fertilization patients

Chromosomal anomalies in 6-10%

Question 34

Syndromes associated with EA/TEF?

Answer 34

Down syndrome
the DiGeorge sequence
the polysplenia sequence
Holt-Oram syndrome
the Pierre Robin sequence
Feingold syndrome
Fanconi syndrome
Townes-Brock syndrome
Bartsocas-Papas syndrome
McKusick-Kaufman syndrome
theCHARGE association (coloboma, heart defects, atresia choanae, developmental retardation, genital hypoplasia, and ear deformities)
The schisis association (omphalocele, neural tube defects, cleft lip and palate, and genital hypoplasia)
cerebral hypoplasia
Potter syndrome (bilateral renal agenesis)
Trisomy 18

Chromosomal anomalies are found in 6-10% of patients.
Trisomy 18 > 21

Question 35

Genes associated with EA/TEF?

Answer 35

Three separate genes have been associated with EA/TEF:

1) MYCN haploinsufficiency in Feingold syndrome

2) CHD7 in CHARGE syndrome, and

3) SOX2 in the anophthalmia–esophageal–genital (AEG) syndrome

H&A

Question 36

Gross classification of EA/TEF?

Answer 36

Gross type A (7%)
Pure esophageal atresia without TEF
- If no concomitant proximal fistula, upper esophagus ends at the level of the azygos vein.
- Distal esophagus is short and often suspended by a fibrous band.
- Long gap.

Gross type B (2%)
Esophageal atresia with proximal fistula
- Fistula located at thoracic aperture or higher in the neck.
- Gasless abdomen

Gross type C (85%)
Esophageal atresia with distal fistula
- Proximal esophagus usually at 3rd-4th thoracic vertebrae.

Gross type D (<1%)
Esophageal atresia with proximal and distal fistulas

Gross type E (4%)
H-Type Fistula without esophageal atresia
- Fistula is usually at the thoracic aperture or higher in the neck.

Gross type F
Esophageal stenosis

Question 37

Associated anomalies with EA?

Answer 37

The factors responsible for the early disturbance in organogenesis leading to EA may affect other organs or systems as well.

EA can be divided clinically into isolated EA and syndromic EA. Anomalies most frequently encountered in syndromic EA:

Cardiac (13-34%)
Vertebral (6-21%)
Limb (5-19%)
Anorectal (10-16%)
Renal (5-14%)

Nonrandom associations:
VACTERL (20%)
Vertebral
Anorectal
Cardiac
Tracheoesophageal
Renal
Limb
*Hydrocephalus also recently considered

CHARGE
Coloboma
Heart defects
Atresia of the choanae
Developmental retardation
Genital hypoplasia
Ear deformities

Complex cardiac deformities may account for most deaths associated with EA malformations (most common are VSDs and ASDs).

Question 38

How do you diagnose EA/TEF prenatally?

Answer 38

Prenatal UTS (20-40%)
1) Polyhydramnios
2) Absent/small gastric bubble
3) Dilated cervical esophagus (pouch sign)

MRI may be used as adjunct in the diagnosis of EA anomalies suspected on UTS.

Question 39

How do you diagnose EA/TEF postnatally?

Answer 39

Clinical
1) Pooling of saliva in the proximal esophagus and mouth
2) Regurgitation, choking, coughing on feeding
3) Cyanosis, respiratory distress
4) Inability to pass feeding catheter through the mouth into the stomach
5) Distension of abdomen (if with fistula)

Imaging:
1) CXR AP/Lat: Pass OGT, instill air to distend upper esophageal pouch, and do a frontal and lateral chest film with downward pressure on the tube.
- (+) Air in stomach: distal TEF
- (-) Air in stomach: EA w/o distal fistula (or fistula occluded with mucus)
- Tip should curl up in the upper pouch

2) Barium esophagography in the prone position (For TEF without EA)
- Repeated choking during feedings and aspiration infiltrates
- Bronchoscopy with or without esophagoscopy is required to confirm the diagnosis.

Question 40

What workup is needed for EA/TEF preoperatively?

Answer 40

Clinical
1) Examine rib cage for deformity
2) Examine spine for congenital scoliosis
3) Examine upper limbs for radial anomalies
4) Assess anus for patency and position
5) Auscultate heart for any murmurs
6) Palpate abdomen for masses (kidney anomalies)

Imaging
1) Echocardiogram (only requirement preop; others can be done as outpatient)
- Identify cardiac anomalies
- Delineate laterality of aortic arch and descending aorta, to aid in determining surgical approach.
—> Right-sided arch (3%): Left thoracotomy preferred
—> Left- sided arch: Right thoracotomy

2) Renal ultrasound

Other considerations
1) Rigid Bronchoscopy
Screen for:
- laryngotracheoesophageal cleft
- tracheal stenosis
- tracheal bronchus to the right upper lobe
- proximal fistulas
- location of distal fistula (helps predict gap)
—> Trifurcation fistula: long gap (closer to carina, longer gap)
—> Fistula 2cm above carina: short gap
- incidence of simultaneous proximal and distal fistula: <5%
- may prolong procedure, infant may decompensate prior to fistula ligation

2) Geneticist

Question 41

How are EA/TEF patients classified in terms of risk and plan of management?

Answer 41

Waterston Risk Groups
A (100% Survival)
BW >2500g
Otherwise healthy.
—> Immediate primary repair.

B (85% Survival)
BW 2000-2500g, well
Higher weight with moderate associated anomalies (non cardiac plus PDA, VSD, ASD)
—> Delayed repair.

C (65% Survival)
BW < 2000g or
Higher with severe associated cardiac anomalies
—> Staged repair.

—

Spitz (Okamoto modification)
Class I (Low risk, 100% survival)
- No major cardiac anomaly, BW =/> 2000g

Class II (Moderate risk, 81% survival)
- No major cardiac anomaly, BW < 2000g

Class III (Relatively high risk, 72% survival)
- Major cardiac anomaly, BW =/> 2000g

Class IV (High risk, 27% survival)
- Major cardiac anomaly, BW < 2000g

Question 42

What is the preoperative management for an EA/TEF patient?

Answer 42

Patient must be transferred to a level I pediatric surgical center.

1) Insert a F10 tube into the upper esophagus and placed on continuous suction (sump catheter— may use F5 as suction catheter inserted in F10).
2) Position child head-up and on his/her side.
3) If in respiratory distress, consider gentle low-pressure ventilation via endotracheal intubation. Forceful ventilation must be avoided to prevent lung damage, gastric distention, and perforation with insufflation through the distal fistula.

4) Start broad-spectrum antibiotics.
5) Pulmonary physiotherapy.
6) IV therapy with 10% dextrose and hypotonic saline.
7) Administer Vitamin K.

Question 43

How is primary repair via thoracotomy done?

Answer 43

1) Child placed in a left lateral decubitus position. A small axillary roll is placed under the chest to enlarge the right-sided intercostal spaces.

2) Surgeon stands to the right of the patient (infant’s back), with the assistant opposite. (Consider opposite positioning for right-sided aorta)

3) Patient’s arm is positioned over the head. Leave tube in place so it can be advanced during the operation.

4) A slightly curved 4-5cm long incision is made 1cm below the inferior tip of the scapula.

5) Via muscle sparing approach, open the auscultatory triangle and retract the muscles (latissimus dorsi posteriorly and the serratus anterior anteriorly). If the serratus muscle needs to be transected, this should be done as low as possible to preserve the long thoracic nerve. The fourth or fifth intercostal space is then entered.

6) Via extrapleural approach, the pleura is gently pushed away from the endothoracic fascia, first in the middle of the incision so that an infant rib spreader can be inserted and opened.

7) As rib spreader is further opened, gently push away the pleura even more posteriorly until the posterior mediastinum is exposed.

8) The distal fistula may start from the trachea directly underneath the azygos vein, in which case the azygos vein is transected between 3-0 or 4-0 absorbable ligatures or simply cauterized and divided. If the distal fistula originates more cephalad on the trachea, the vein can be left intact. Recently, a relationship between division of the azygos vein and the development of an anastomotic leak has been suggested.

9) The distal esophagus is easily found because it distends with each inspiration and the vagus nerve is intimately attached. Once identified, the distal segment should be followed proximally to locate where the fistula enters the trachea. The fistula should be dissected and mobilized close to the trachea, which will spare as many vagal nerve branches as possible. The fistula can be encircled with a vessel loop or suture to aid in exposure.

10) There are several ways to ligate the fistula on the tracheal side. Ligation without division can result in a higher recanalization rate. We prefer to divide the fistula sharply after placing traction sutures at each end. A series of 5-0 PDS (Ethicon, Inc., Sommerville, NJ) sutures are then used to close the tracheal side, taking care not to compromise the tracheal lumen. Another option is to apply a single 5-mm clip across the fistula where it connects to the membranous trachea. We have found this technique to be simple and efficient and results in a smaller pouch remnant on the posterior tracheal wall. However, there are anecdotal reports of clip migration and recanalization of the fistula using this technique. The tracheal closure can be checked by irrigating with warm water and applying a higher ventilation pressure to assess for an air leak.

11) Identify proximal pouch by asking anesthesiologist to push on the repogle tube. A traction suture is sometimes helpful for traction during blunt dissection.

12) Once mobilized, the tip of the proximal pouch is amputated to expose lumen and mucosa.

13) An end to end anastomosis is done with 5-0 absorbable sutures, starting in the middle of the back wall of each esophageal segment, including both mucosa and muscular wall with each bite. The sutures in the back wall of the anastomosis are tied intraluminally. Then, the front part of the anastomosis is performed with sutures tied on the outside. Before finishing the anastomosis, an 8 French or 10 French tube is passed into the stomach. This helps protect the lumen from inadvertent closure and also allows for gastric decompression. Others feel the placement of an intraluminal tube or stent causes an increased risk of stricture or leak, and choose not to use it.

14) The use of a chest drain is optional. We usually place a drain and leave it until a contrast study is obtained on day 4 or 5. The chest incision is then closed in layers. The ribs should be approximated with one 3-0 absorbable suture. This suture should be tied gently so that the intercostal space is not obliterated. The skin is closed with a 5-0 absorbable subcuticular suture.

Question 43

How is primary repair via thoracotomy done?

Answer 43

1) Child placed in a left lateral decubitus position. A small axilla ray roll is placed under the chest to enlarge the right-sided intercostal spaces.

2) Surgeon stands to the right of the patient (infant’s back), with the assistant opposite. (Consider opposite positioning for right-sided aorta)

3) Patient’s arm is positioned over the head. Leave tube in place so it can be advanced during the operation.

4) A slightly curved 4-5cm long incision is made 1cm below the inferior tip of the scapula.

5) Via muscle sparing approach, open the auscultatory triangle and retract the muscles (latissimus dorsi posteriorly and the serratus anterior anteriorly). If the serratus muscle needs to be transected, this should be done as low as possible to preserve the long thoracic nerve. The fourth or fifth intercostal space is then entered.

6) Via extrapleural approach, the pleura is gently pushed away from the endothoracic fascia, first in the middle of the incision so that an infant rib spreader can be inserted and opened.

7) As rib spreader is further opened, gently push away the pleura even more posteriorly until the posterior mediastinum is exposed.

8) The distal fistula may start from the trachea directly underneath the azygos vein, in which case the azygos vein is transected between 3-0 or 4-0 absorbable ligatures or simply cauterized and divided. If the distal fistula originates more cephalad on the trachea, the vein can be left intact. Recently, a relationship between division of the azygos vein and the development of an anastomotic leak has been suggested.

9) The distal esophagus is easily found because it distends with each inspiration and the vagus nerve is intimately attached. Once identified, the distal segment should be followed proximally to locate where the fistula enters the trachea. The fistula should be dissected and mobilized close to the trachea, which will spare as many vagal nerve branches as possible. The fistula can be encircled with a vessel loop or suture to aid in exposure.

10) There are several ways to ligate the fistula on the tracheal side. Ligation without division can result in a higher recanalization rate. We prefer to divide the fistula sharply after placing traction sutures at each end. A series of 5-0 PDS (Ethicon, Inc., Sommerville, NJ) sutures are then used to close the tracheal side, taking care not to compromise the tracheal lumen. Another option is to apply a single 5-mm clip across the fistula where it connects to the membranous trachea. We have found this technique to be simple and efficient and results in a smaller pouch remnant on the posterior tracheal wall. However, there are anecdotal reports of clip migration and recanalization of the fistula using this technique. The tracheal closure can be checked by irrigating with warm water and applying a higher ventilation pressure to assess for an air leak.

11) Identify proximal pouch by asking anesthesiologist to push on the repogle tube. A traction suture is sometimes helpful for traction during blunt dissection.

12) Once mobilized, the tip of the proximal pouch is amputated to expose lumen and mucosa.

13) An end to end anastomosis is done with 5-0 absorbable sutures, starting in the middle of the back wall of each esophageal segment, including both mucosa and muscular wall with each bite. The sutures in the back wall of the anastomosis are tied intraluminally. Then, the front part of the anastomosis is performed with sutures tied on the outside. Before finishing the anastomosis, an 8 French or 10 French tube is passed into the stomach. This helps protect the lumen from inadvertent closure and also allows for gastric decompression. Others feel the placement of an intraluminal tube or stent causes an increased risk of stricture or leak, and choose not to use it.

14) The use of a chest drain is optional. 103,104 We usually place a drain and leave it until a contrast study is obtained on day 4 or 5. The chest incision is then closed in layers. The ribs should be approximated with one 3-0 absorbable suture. This suture should be tied gently so that the intercostal space is not obliterated. The skin is closed with a 5-0 absorbable subcuticular suture.

Question 44

What is the initial treatment for esophageal atresia without a distal fistula?

Answer 44

A gasless abdomen is the signature of EA without distal fistula.

The standard initial treatment is a gastrostomy with laryngotracheobronchoscopy during the same anesthesia, to exclude a proximal fistula and other associated tracheobronchial anomalies.

In EA without a distal fistula and in the absence of a duodenal obstruction, the stomach is usually small, which can make insertion of the gastrostomy difficult. Once placed, bolus feedings should be instituted to enlarge both the small stomach and the distal pouch.

Question 45

What are the options for lengthening procedures in long gap esophageal atresia?

Answer 45

1) Upper pouch bougienage
- Weighted bougie is passed through the mouth into the upper pouch.
- Forward pressure applied 1-2x/day x 6-12 weeks, before attempting delayed primary repair.

2) Electromagnetic field
- Magnets used to pull together metallic bullets in two ends of the esophagus to shorten the gap.

3) Nylon thread attached to silver olives within each lumen
- Olives were pushed toward each other over time until two ends of the esophagus pressed together and created a fistula.

4) Tacking sutures to approximate ends, then placing a bridging silk suture.

5) Multi- staged, extrathoracic elongation of Kimura
- Upper part of esophagus is mobilized, and initially brought out as an end cervical esophagostomy.
- Every 2-3 weeks, the esophagus and its cutaneous stoma are surgically mobilized and translocated down the anterior chest wall until enough length is achieved.

6) Foker technique
- Traction sutures on both proximal and distal pouches exit through the chest wall and are serially pulled in opposite directions until the pouches approximate.
- Induces esophageal growth and expedite approximation of pouches, allowing for earlier primary repair (10-14 days).

7) Internal traction sutures

8) Intraoperative techniques
- Foker traction done intraop: achieve length after 20-30mins of traction on the esophageal ends.
- Circular myotomy: Upper part of esophagus, to decrease tension. (modification: use balloon catheters or cuffed endotracheal tubes inflated in the upper esophageal pouch) —> complications include leak, impaction of food particles, ballooning, esophageal pseudodiverticulum, stricture
- Distal pouch circular myotomy in addition to proximal myotomy (modifications: spiral upper pouch myotomy with oblique suture closure of the muscular layer to minimize diverticula formation, improve motility)
- Create full thickness anterior flap of upper pouch wall. When folded distally, flap can be rolled into a tube and attached to the lower esophageal segment. (Mod: Create 5mm posterior flap or anterior esophageal flap, plus end to end oblique anastomosis with spatulated distal esophagus.)
- Completely mobilize distal esophagus down to the esophageal hiatus of the diaphragm. Part of fundus may be brought up into the chest to facilitate anastomosis.
- Combined abdominal and thoracic procedure. Ligate and divide left gastric artery, with transverse or diagonal division of the lesser curvature of the stomach, and mobilization of the gastric cardia and upper fundus into the chest. Add partial fundoplication to treat anticipated GER.
- Collis gastroplasty with Nissen fundoplication to lengthen distal end.

Other maneuvers:
- Postoperative head flexion
- Paralysis of striated muscles of the neck
- Mech vent support to minimize esophageal disruption

Question 46

Factors placing EA infants at increased risk for death and long term morbidity?

Answer 46

1) Lower birthweight (<1500g/prematurity)
2) Major CHD
3) Severe associated anomalies and ventilator dependency
4) Long gap length between two ends of esophagus

Question 47

Predictors of complications in EA?

Answer 47

1) Twin birth
2) Preoperative intubation
3) BW <2500g
4) Long gap atresia
5) Anastomotic leak
6) Postoperative intubation > 4 days
7) Inability to feed at the end of 1 month

Question 48

Regarding thoracotomy for esophageal atresia, which of the following is false?

A. Ligation and division of azygos vein.
B. Identification and preservation of recurrent laryngeal nerve.
C. Identification and mobilisation of lower pouch and fistula.
D. Single-layer anastomosis.
E. Chest drain is optional.

Answer 48

B Identify and preserve vagus nerve. Recurrent laryngeal nerve is quite high and does not come across.

Syed/MCQ

Question 49

Indication of cervical esophagostomy includes all except:

A. Short gap oesophageal atresia
B. When attempt of primary anastomosis of esophagus fails.
C. Disruption of primary anastomosis
D. Extensive stricture of esophagus after caustic ingestion
E. Foreign body perforation of esophagus

Answer 49

A

Long gap oesophageal atresia is an indication of cervical oesophagostomy.

Syed/MCQ

Question 50

Regarding procedure of cervical esophagostomy, which one is correct?

A. Right side préfèrred
B. Incision 1cm above and parallel to lateral third of clavicle.
C. Ligation and division of external jugular vein
D. Retract carotid artery, internal jugular vein, and vagus nerve medially.
E. Identify and divide recurrent laryngeal nerve.

Answer 50

C

Procedure includes:

Left-sided approach is preferred.

Incision 1 cm above and parallel to medial third of clavicle.

Ligate and divide external jugular vein.

Retract carotid sheath with its contents (carotid artery, vagus nerve and internal jugular vein) laterally.

Identify and preserve recurrent laryngeal nerve.

Syed/MCQ

Question 51

A full-term newborn patient is noted to have noisy breathing. Suctioning of the oropharynx yields copious amounts of secretions that continue to accumulate. An attempt to feed the infant results in choking. An anterior-posterior chest radiograph demonstrates the catheter curled in the upper esophageal pouch, along with the presence of air in the stomach and bowel. Which of the following is pertinent to involved pathophysiology?
Choices:
1. Diverticular outpouching of the trachea from the midgut
2. Abnormal duplication of the caudal foregut during the fourth and fifth weeks of embryonic development
3. Abnormal posterior positioning of the tracheoesophageal septum
4. The esophagus failed to recanalize during week 8 of embryonic development

Answer 51

Answer: 3 - Abnormal posterior positioning of the tracheoesopha-geal septum
Explanations:
•The infant most likely has a tracheoesophageal fistula (TEF) and esophageal atresia. TEF occurs due to abnormal septation of the caudal foregut during the fourth and fifth weeks of embryonic de-velopment.
• Under normal conditions, the trachea forms as a diverticulum of the foregut and develops a complete septum that separates it from the esophagus.
• Fistula formation in conjunction with esophageal atresia (EA) occurs during an abnormal posterior positioning of the tracheoe-sophageal septum, resulting in a retained connection between the trachea and esophagus.
• Isolated EA without TEF commonly occurs when the esophagus fails to recanalize during week 8 of embryonic development.

StatPearls

Question 52

Gastroesophageal reflux is a complication of oesophageal atresia repair; which of the following procedures do you do for its correction.

A. Oesophageal dilatation.

B. Resection and anastomosis.

C. Oesophageal replacement.

D. Fundoplication.

E. Aortopexy.

Answer 52

D

Fundoplication is the procedure for correction of gastroesophageal reflux if medical treatment fails.

Oesophageal dilatation is for correction of oesophageal stricture. If dilatation fails in stricture may needs resection and anastomosis or oesophageal replacement.

Aortopexy may be required in severe tracheomalacia.

Syed/MCQ

Question 53

For VACTERAL association, all of the following statements are false except:

A. V stands for vaginal atresia.

B. A stands for arterial lesion.

C. C stands for coronary lesion.

D. T stands for thoracic wall anomaly.

E. R stands for renal anomaly.

Answer 53

E

R stands for renal anomaly. VACTERL stands for vertebral defects, anal atresia, cardiac defects, tracheoesophageal fistula, renal anomalies and limb abnormalities.

Syed/MCQ

Question 54

In CHARGE association, all of the following are true except:

A. C stands for coloboma.

B. H stands for heart defect.

C. A stands for atresia choanea.

D. R stands for developmental retardation.

E. E stands for Oesophageal atresia.

Answer 54

E

E stands for ear deformities. CHARGE is an abbreviation for Coloboma, heart defects, atresia choanae (also known as choanal atresia), growth retardation, genital abnormalities and ear abnormalities.

Syed/MCQ

Question 55

Regarding oesophageal atresia, which of the following is false?

A. Foetal ultrasound shows upper oesophageal pouch, filling, and emptying.

B. If foetal ultrasound is unable to detect stomach, this indicates atresia without fistula.

C. Failure to advance nasogastric tube 10 cm beyond mouth or nose.

D. Cyanotic episode, frothing, and respiratory distress are all common symptoms.

E. Choice of operation is oesophagostomy and gastrostomy when patient is fit.

Answer 55

E

Choice of operation is oesophagostomy and gastrostomy when the patient is not fit.

Syed/MCQ

Question 56

Regarding thoracotomy for oesophageal atresia, which of the following is false?

A. Ligation and division of azygos vein.

B. Identification and preservation of recurrent laryngeal nerve.

C. Identification and mobilisation of lower pouch and fistula.

D. Single-layer anastomosis.

E. Chest drain is optional.

Answer 56

B

Identify and preserve vagus nerve. Recurrent laryngeal nerve is quite high and does not come across.

Syed/MCQ

Question 57

Indication of cervical oesophagostomy includes all except:

A. Short gap oesophageal atresia.

B. When attempt of primary anastomosis of oesophagus fails.

C. Disruption of primary anastomosis.

D. Extensive stricture of oesophagus after caustic ingestion.

E. Foreign body perforation of oesophagus.

Answer 57

A

Long gap oesophageal atresia is an indication of cervical oesophagostomy.

Syed/MCQ

Question 58

Regarding procedure of cervical oesophagostomy, which one is correct?

A. Right side preferred.

B. Incision 1 cm above and parallel to lateral third of clavicle.

C. Ligation and division of external jugular vein.

D. Retract carotid artery, internal jugular vein, and vagus nerve medially.

E. Identify and divide recurrent laryngeal nerve.

Answer 58

C

Procedure includes Left-sided approach is preferred.

Incision 1 cm above and parallel to medial third of clavicle.

Ligate and divide external jugular vein.

Retract carotid sheath with its contents (carotid artery, vagus nerve and internal jugular vein) laterally.

Identify and preserve recurrent laryngeal nerve.

Syed/MCQ

Question 59

Regarding H-type congenital tracheoesophageal fistula, which of the following is false?

A. Tube oesophagogram is in supine position.

B. Bronchoscopy and oesophagoscopy can confirm the diagnosis.

C. Cervical approach is more commonly used for ligation and division.

D. Inferior thyroid artery and middle thyroid vein is ligated and divided.

E. Preserve recurrent laryngeal nerve.

Answer 59

A

Tube oesophagogram is done in prone position to visualise H-type fistula.

Syed/MCQ

Question 60

Regarding oesophageal rupture, which of the following is false?

A. Boerhaave syndrome refers to oesophageal rupture secondary to forceful vomiting and retching.

B. In neonates, left-sided pneumothorax is more common.

C. Surgical empyema may be present.

D. Oesophagoscopy offers no diagnostic advantage.

E. Decision depends upon site of perforation.

Answer 60

B

In neonates right-sided, while in adults left-sided pneumothorax occurs because of close adherence of aorta to oesophagus on the left side in infants, which provides additional support.

Oesophagoscopy offers no diagnostic advantage and may actually enlarge the perforation, making subsequent repair more difficult.

Decision of treatment in oesophageal perforation depends on site of perforation, whether perforation is free or contaminated, the interval from injury to diagnosis and systemic response to injury.

Syed/MCQ

Question 61

Regarding complications of oesophageal atresia, the following statements are true except:

A. There is direct relationship between post-operative leakage and the gap between two ends of oesophagus.

B. Leakage is noted in 10–20 percent of cases.

C. All those who develop dysphagia require surgery.

D. Incidence of gastroesophageal reflux is about 50 percent.

E. Incidence of scoliosis increases with second thoracotomy.

Answer 61

C

Incidence of dysphagia is about 50 percent. Only about 10 percent require surgery up to 10 years of age.

Leakage is seen in 10–20 percent of cases.

By one thoracotomy, the incidence of scoliosis increases to 10 percent, and by second thoracotomy it increases to 20 percent.

Syed/MCQ

Question 62

In oesophageal atresia, a tube meets atretic pouch of oesophagus usually at what distance from lip or nostril?

A. 4–6 cm.

B. 8–12 cm.

C. 14–18 cm.

D. 20 cm.

E. More than 20 cm.

Answer 62

B 8–12 cm

Syed/MCQ

Question 63

The most common type of oesophageal atresia is:

A. Oesophageal atresia with proximal tracheoesophageal fistula.

B. Oesophageal atresia with distal tracheoesophageal fistula.

C. Oesophageal atresia with fistula to both pouches.

D. H-type fistula (no atresia).

E. Isolated atresia (no fistula).

Answer 63

B Oesophageal atresia with distal tracheoesophageal fistula.

The incidence is
A ≤ 1 percent,
B 86 percent,
C 1 percent,
D 4 percent, and
E 8 percent.

Syed/MCQ

Question 64

About associated anomalies in oesophageal atresia, all are true except:

A. Overall associated anomalies are 50–70 percent.

B. Cardiovascular anomalies are 29–35 percent.

C. Gastrointestinal anomalies are 10 percent.

D. Genitourinary anomalies are found in 20 percent cases.

E. Neurological defect has been seen in 10 percent cases.

Answer 64

C Gastrointestinal anomalies are found in 24 percent of cases.

Syed/MCQ

Question 65

Incidence of anastomotic leak noted in oesophageal atresia with tracheoesophageal atresia repair is:

A. 5–10 percent of cases.

B. 10–20 percent cases.

C. 20–30 percent cases.

D. 30–40 percent cases.

E. 40–50 percent cases.

Answer 65

B 10–20 percent of cases.

Syed/MCQ

Question 66

Recurrent tracheoesophageal fistula is noted in:

A. less than 1 percent cases.

B. 1–2 percent cases.

C. 2–5 percent cases.

D. 5–8 percent cases.

E. 8–10 percent cases.

Answer 66

C 2–5 percent of cases

Syed/MCQ

Question 67

H-type variety of congenital tracheoesophageal fistula is usually repaired by which of the following incision?

A. Right cervical incision.

B. Right thoracic incision

C. Midline abdominal incision.

D. All are useful.

E. None of the above.

Answer 67

A Right cervical incision.

Syed/MCQ

Question 68

Isolated oesophageal atresia comprises what percentage of oesophageal atresia?

A. 2–4 percent.

B. 6–8 percent.

C. 10–12 percent.

D. 14–16 percent.

E. 18–20 percent.

Answer 68

B 6–8 percent

Syed/MCQ

Question 69

Regarding aortopexy, which of the following is false?

A. Right anterior thoracotomy is preferred.

B. Incision at the level of third rib.

C. Internal memory vessels at the medial end of the incision is divided.

D. Lobe of thymus is divided with care of to preserve phrenic nerve.

E. Adventitia of aorta and pericardium is sutured with the posterior surface of sternum.

Answer 69

A

Left anterior thoracotomy is preferred for aortopexy.

Syed/MCQ

Question 70

About indication of oesophageal replacement with colon, which one of the following is the best answer?

A. Oesophageal atresia with inadequate length for primary anastomosis.

B. Stricture that is too long for resection and anastomosis.

C. Extensive Barrett’s oesophagus in the distal oesophagus.

D. All of the above are correct.

E. None of the above are correct.

Answer 70

D

All A, B and C are indications for oesophageal replacement.

Syed/MCq

Question 71

The best answer among following for oesophageal replacement with colon is:

A. Right colon is used.

B. Transverse colon is used.

C. Left colon is used.

D. Any of above is used.

E. None of the above is used.

Answer 71

D

Right, transverse or left colon may be used.

Syed/MCQ

Question 72

The preferred approach for oesophageal replacement with colon is:

A. Right thoracotomy.

B. Left thoracotomy.

C. Midline sternotomy.

D. All of the above.

E. None of the above.

Answer 72

A

Right thoracotomy is preferred method of oesophageal replacement with colon, as most of thoracic oesophagus is situated more towards right side than left.

Syed/MCQ

Question 73

Which of the following is considered alkali, causing caustic strictures of oesophagus?

A. Hydrochloric acid.

B. Sodium hydro-oxide.

C. Ammonium hydro-oxide.

D. Sodium hypochlorite.

E. Sodium polyphosphate.

Answer 73

B

Sodium hydroxide is an alkali.

Ammonium hydro-oxide comes in the category of ammonia.

Sodium hypochlorite and sodium polyphosphate are detergents.

Hydrochloric acid is an acid.

Other alkalis include potassium hydro-oxide.

All above cause caustic stricture of oesophagus.

Syed/MCQ

Question 74

Regarding Injury after caustic ingestion, all of the following statements are correct except:

A. Acid causes liquefactive necrosis.

B. Oesophagus is primarily damaged at area of holdup.

C. Perforation of oesophagus may occur.

D. Fibrosis and shortening may lead to shortening of oesophagus.

E. Aortoesophageal fistula may develop.

Answer 74

A

Acid ingestion causes coagulative necrosis, while after alkali ingestion liquefactive necrosis occurs.

Syed/MCQ

Question 75

Regarding caustic ingestion and oesophagus, all of the following are true except:

A. Damage is directly related to the concentration of a caustic substance.

B. In case of alkali ingestion, induction of vomiting is contraindicated.

C. Fiberoptic endoscopy is indicated after 48 hours.

D. Contrast oesophagogram is done at 2 weeks.

E. If fever, sepsis and upper abdominal signs are present, perforation may have occurred.

Answer 75

C

Fiberoptic endoscopy is indicated within 24–48 hours.

In case of pharyngeal burn with stridor, early oesophagoscopy is contraindicated because of upper airway obstruction.

In case of alkali ingestion, induction of vomiting is contraindicated because the alkali is mostly neutralised by gastric acid, and consequences of acid regurgitation may induce further injury.

Syed/MCQ

Question 76

Regarding endoscopic grading of oesophageal injury after caustic ingestion, which statement is true?

A. Grade I is blister formation and superficial ulcer.

B. Grade IIa is a deep discrete or circumferential ulcer.

C. Grade IIb is small scattered area of necrosis.

D. Grade IIIa is extensive necrosis.

E. All of the above are false.

Answer 76

E

The grading of oesophageal injury after caustic ingestion is

grade 0 is normal,
grade I is hypermia,
grade IIa is blister and superficial ulcer,
grade IIb is deep discrete or circumferential ulcer,
grade IIIa is small scattered area of necrosis,
grade IIIb is extensive necrosis and perforation.

Syed/MCQ

Question 77

Poor prognostic factor in stricture of oesophagus following caustic ingestion include all except:

A. Delayed presentation.

B. Extensive grade III injury.

C. Delayed fibrotic stricture that cracks on dilatation.

D. Stricture longer than 2 cm.

E. Inadequate lumen patency despite if repeated dilatation over 9–12 months.

Answer 77

D
Stricture longer than 5 cm has poor prognostic sign.

Syed/MCQ

Question 78

Emergency retrieval is indicated for a:

A battery in the oesophagus

B battery in the stomach

C battery in the intestine

D a single magnet in the stomach

E a single magnet in the intestines.

Answer 78

A

objects found within the oesophagus should generally be considered impacted. Because impacted oesophageal foreign bodies may lead to significant morbidity (and even mortality), removal of impacted oesophageal foreign bodies is mandatory.

Batteries located in the oesophagus should be removed urgently because of the risk of oesophageal burns and resultant complications. The procedure of choice is flexible fibreoptic endoscopy.

most swallowed foreign bodies pass harmlessly through the GI tract once they have reached the stomach.

Button (disc) batteries in the stomach or intestines do not need to be removed immediately, as they generally pass through the lower GI tract without difficulty.

Button batteries retained in the stomach or at a fixed spot in the intestines should be removed.

The ingestion of multiple magnets may lead to the development of pressure necrosis and subsequent perforation as the opposite poles of the magnets will become attracted to one another.

This may be particularly true when the magnets are ingested at intervals. However, a single magnet poses no such risk and should spontaneously pass.

SPSE 1

Question 79

Which of the following is true regarding impaction of oesophageal foreign bodies?

A There is an equal distribution of sites of impaction along the entire length of the oesophagus.

B The most common site of impaction is at the lower oesophageal sphincter (LOS) at the gastro-oesophageal junction.

C The most common site of impaction is at the mid-oesophagus, in the region where the aortic arch and carina compress the oesophagus.

D The most common site of impaction is at the anatomical change from oesophageal skeletal muscle to smooth muscle.

E Most oesophageal foreign body impaction occurs in children with a pre-existing oesophageal abnormality.

Answer 79

D

most complications of paediatric foreign body ingestion are due to oesophageal impaction, and this usually occurs at one of three typical locations.

The most common site of oesophageal impaction is at the thoracic inlet.

This is defined as the area between the clavicles on chest radiograph, and the site of anatomical change from the skeletal muscle to the smooth muscle of the oesophagus.

The cricopharyngeus sling at C6 is also at this level and may ‘catch’ a foreign body.

About 70% of blunt foreign bodies that lodge in the oesophagus do so at this location.

Another 15% become lodged at the mid- oesophagus, in the region where the aortic arch and carina overlap the oesophagus on chest radiograph.

The remaining 15% become lodged at the lES at the gastro-oesophageal junction.

Children with pre-existing oesophageal abnormalities (e.g. repair of a tracheo-oesophageal fistula) are likely to have foreign body impaction at the site of the abnormality.

If a child with no known oesophageal pathology has a blunt foreign body lodged at a location other than the three typical locations described above, the possibility of a previously unknown oesophageal abnormality should be considered.

This latter population makes up a small minority of oesophageal ingestions overall.

SPSE 1

Question 80

Two hours ago, during dinner, a healthy 3-year-old child swallowed a coin that is confirmed on X-ray to be located in the mid-oesophagus. He is in no distress and is asymptomatic. Which of the following is not a reasonable treatment plan?

A use of a Foley catheter under fluoroscopy to extract the coin

B use of an oesophageal bougie to advance the coin into the stomach

C emergency flexible endoscopy

D flexible endoscopy in 6 hours

E allow clear fluids and reassess in 12 hours with a repeat X-ray

Answer 80

C

Endoscopy (oesophagoscopy) is by far the most commonly used means of oesophageal foreign body removal and is usually the procedure of choice. most children with oesophageal foreign bodies are stable from a cardiovascular and respiratory standpoint. Endoscopy usually can be delayed until the child’s stomach is emptied and a surgical team is assembled. However, pointed objects and batteries should be removed as rapidly as possible to avoid further injury to the oesophageal mucosa and subsequent mediastinitis. Because endoscopy is relatively invasive and expensive, other methods of oesophageal foreign body removal have been investigated and are probably more cost-effective when used appropriately. These have been performed most commonly on children with oesophageal coins, and should be performed only in previously healthy children whose ingestion of a blunt object was witnessed less than 24 hours prior to the procedure.

Foley catheter method: blunt foreign bodies may be removed by use of a Foley catheter. Typically, the patient is restrained in a head-down position on a fluoroscopy table, and an uninflated catheter is inserted distal to the object. The catheter balloon is then inflated and gently withdrawn, drawing the foreign body with it. on some occasions, the object is dislodged and passed into the stomach. Progress is typically monitored fluoroscopically. This procedure is performed without radiographic monitoring at some centres with extensive experience and does not require general anaesthesia.

Bougienage method: blunt oesophageal foreign bodies may be advanced into the stomach with a bougie. While the child is sitting upright, the lubricated instrument is gently passed down the oesophagus, dislodging the object. The object is then expected to pass through the rest of the GI tract; thus, this procedure should not be performed on children with known lower GI tract abnormalities. A brief observation period and a repeat radiograph should follow any removal procedure to rule out retained foreign bodies and other complications (e.g. pneumomediastinum). In recent studies, the bougienage method has been shown to be far more cost-effective than endoscopy, for properly selected patients.

Spontaneous passage: blunt foreign bodies located at the lES often pass spontaneously within several hours of ingestion. This has been best studied in coin ingestions. Previously healthy children may be given food and drink and have repeat radiographs 24 hours following ingestion. often, the coin passes through the lES, and a removal procedure can be avoided. Although blunt foreign bodies located in other areas of the oesophagus are less likely to spontaneously pass, this strategy may be an appropriate alternative for stable children with normal oesophageal anatomy and a foreign body in the thoracic inlet or the midoesophagus. This may be most successful in asymptomatic children. Follow-up imaging should be arranged to ensure transit, and once the oesophagus is clear, nearly all foreign bodies are evacuated – 97% in one large series.

SPSE 1

Question 81

A 3-year-old presents with acute wheezing after being held down and fed peanuts by her older brother. A normal chest X-ray is obtained in the emergency department. What should the next step be?

A spiral CT of the chest
B bronchodilators
C Heimlich’s manoeuvre
D bronchoscopy
E oesophagoscopy

Answer 81

D

Bronchoscopy should be performed in any patient with a definitive or suspected diagnosis of a tracheobronchial foreign body in order to confirm the diagnosis and to remove the foreign body. most practitioners recommend rigid bronchoscopy, which should be done as soon as the patient is deemed stable.

Spiral CT of the chest would not contribute to the care of this child and would subject her to unnecessary radiation.

Bronchodilators are of benefit in a child with reactive airway disease, but this history indicates a probable ingested foreign body as a cause of the wheezing.

The Heimlich manoeuvre is widely recommended as a first-aid measure but is of dubious efficacy in the stable patient and can sometimes displace an inhaled object into a relatively riskier location. It should be reserved for complete airway obstruction without other available resources.

oesophagoscopy is of no value in this context, as the child’s symptomatology indicates the foreign body is affecting the tracheobronchial tree.

SPSE 1

Question 82

The most common radiographic finding on chest X-ray after aspiration of a foreign body is:

A a normal study
B unilateral hyperlucency
C atelectasis
D identification of the foreign body
E post-obstructive pneumonia.

Answer 82

B

A wide variety of radiological findings are possible after aspiration of a foreign body.

As recently reported by Bittencourt et al. (2006) the most common finding is unilateral hyperlucency distal to the obstructing material (see Table 24.1).

Identification of the foreign body is relatively rare (<25%), and the development of pneumonia is a late finding.

SPSE 1

Question 83

The most common site of an aspirated foreign body is:

A the trachea
B the left mainstem bronchus
C the right mainstem bronchus
D at the vocal cords
E all sites equally common.

Answer 83

C

The most common location of aspirated foreign bodies is the right main bronchus as indicated by several investigators on the subject. The proposed mechanism for this asymmetrical distribution is the more direct course that the right bronchus follows after the tracheal bifurcation when compared with the left bronchus.

A recent review (Saki et al., 2009) found the distribution of aspirated foreign bodies to be as follows:

● right mainstem bronchus in 560 (55.1%)

● left mainstem bronchus in 191 (18.8%)

● trachea in 173 (17.1%)

● vocal cords in 75(7.4%)

● both bronchi in 16 (1.6%).

SPSE 1

Question 84

The most common presenting complaint after aspiration of a foreign body is:

A asymptomatic
B coughing
C wheezing
D dyspnoea
E stridor.

Answer 84

B

Saki et al. (2009) review the presenting symptoms in their series, as well as in the available data reported in literature. In their review, coughing was the most common symptom (73%), and this was seen consistently in the publications on the subject. It is important to note, however, that a significant minority of patients will present late with no appreciable history. These patients may manifest persistent pneumonia or lung abscesses, in which case a high index of suspicion of a foreign body aspiration needs to be maintained.

SPSE 1

Question 85

Which of the following complications can occur following ingestion of multiple magnets?

A intestinal perforation
B malabsorption
C intestinal obstruction
D acute volvulus
E all of the above

Answer 85

E

Numerous reports have recently been published illustrating the many complications associated with the ingestion of multiple magnets.

If more than one magnet is ingested, the magnets may be attracted to one another leading to pressure necrosis between two loops of intestine.

ultimately, this may result in a perforation or an enteric fistula.

Should an enteroenteric fistula occur, malabsorption and diarrhoea may ensue.

Intestinal obstruction has also been reported, both secondary to the magnets themselves, and as a consequence of an acute intestinal volvulus.

SPSE 1

Question 86

A 2-year-old boy presents to the emergency department with stridor and drooling; the parents cannot provide any additional history. An upright chest radiograph confirms that a coin has become impacted in the subglottic area. Which of the following is not appropriate management?

A topical anaesthesia to the respiratory mucosa

B emergency rigid bronchoscopy

C possible tracheotomy

D racemic adrenalin

E corticosteroids

Answer 86

D

long-standing foreign bodies are at risk of embedding themselves within the surrounding tissues, making them particularly challenging to remove by endoscopic means. Rigid bronchoscopy under general anaesthesia is the treatment of choice as it provides optimal conditions for foreign body removal. Sufficient anaesthesia is required for the procedure in order to prevent laryngospasm, for which topical anaesthesia with tetracaine or lidocaine is an important adjunct.

The presence of localised oedema is an indication for systemic corticosteroid therapy, either before or after the procedure.

Tracheostomy is occasionally indicated in foreign body extraction, especially when the foreign object is impacted in the subglottic region, or if the foreign body is large and obstructs the glottis during removal.

Racemic adrenalin is useful in the treatment of laryngotracheitis as it leads to mucosal vasoconstriction and the reduction of oedema via alphaadrenergic receptors located in the airway.

However, it has not been shown to be of benefit in the context of an airway foreign body.

SPSE 1

Question 87

A button battery is noted on abdominal X-ray (AXR) to be in the stomach of an otherwise asymptomatic 2-year-old boy. Treatment options include all except:

A endoscopic removal
B repeat AXR in 6 hours
C administration of ipecac (emetic agent)
D discharge with a repeat AXR in 48 hours
E discharge and have parents strain the stool.

Answer 87

C

Button batteries within the stomach or intestine do not need to be removed immediately, as the vast majority will pass through the lower GI tract without difficulty.

Button batteries retained in the stomach or at a fixed point in the intestine should be removed.

Generally, these patients may be managed on an outpatient basis.

Serial daily radiographs are not required, and as long as the patient remains asymptomatic, weekly radiographs may be obtained if it cannot be recovered after straining the stool.

Intervention is required when symptoms arise (e.g. peritonitis, bowel obstruction), or if the foreign body has been retained more than 2 weeks in the same location.

It is important to realise that it is often difficult to clearly define its location based on plain radiographs.

If the battery is suspected to be in the stomach, it should be removed by endoscopy.

The administration of ipecac or other emetic agent is not indicated and may result in aspiration.

SPSE 1

Question 88

Which patient is at highest risk for a foreign body aspiration?

A an unwitnessed 18-month-old girl
B a witnessed 6-month-old girl
C an unwitnessed 6-year-old boy
D a witnessed 18-month-old boy
E a witnessed 6-year-old girl

Answer 88

D

In the year 2000, in the united States, foreign body aspiration accounted for more than 17 000 emergency department visits and 160 deaths in children aged 14 years or younger.

Airway foreign bodies are the fifth most common cause of death in children younger than 1 year.

The peak ages during which aspiration of foreign bodies occur are the toddler through preschool ages, although foreign bodies have been found in the airways of patients of all ages and sizes.

Foreign body aspiration occurs with a slight male predominance and is typically (although not always) witnessed by a friend or family member.

SPSE 1

Question 89

Regarding the pathophysiology of oesophageal injury in a button battery ingestion, which of the following is not true?

A Liquefaction necrosis typically occurs because sodium hydroxide is generated by the current produced at the anode.

B Injury occurs as a consequence of multiple mechanisms including thermal, caustic and toxic phenomena.

C Maximal effects typically occur in less than 6 hours.

D The alkali of a button battery is effectively neutralised by stomach contents.

E Because lithium batteries contain no alkali, they do not pose the same risk as other button batteries.

Answer 89

E

Disc batteries do not usually cause problems unless they become lodged in the GI tract.

The most common location that disc batteries become lodged, resulting in clinical consequences, is the oesophagus.

Batteries that successfully traverse the oesophagus are unlikely to lodge at any other location, and are effectively neutralised by GI secretions.

They can be managed expectantly. oesophageal damage can occur in a relatively short period of time when a disc battery is lodged in the oesophagus.

liquefaction necrosis may occur as a result of the production of sodium hydroxide when current is produced by the battery (usually at the anode).

However, the necrosis is multifactorial as electrochemical analysis ex vivo suggests that thermal, caustic and toxic phenomena are all contributing factors.

Perforation has occurred as rapidly as 6 hours after ingestion, with maximal effects occurring in as little as 2 hours. lithium batteries make up only 1% of all battery ingestions overall, but although they contain no alkaline material they tend to be larger and have a greater voltage, and have been shown to cause similar mucosal damage as alkaline batteries.

SPSE 1

Question 90

Therapeutic manoeuvres that may aid in tracheobronchial foreign body retrieval include all except:

A laryngoscopic examination of the oropharynx under conscious sedation with possible extraction with Magill forceps

B extraction with a Fogarty balloon catheter via a rigid bronchoscope

C extraction using telescopic forceps with a rigid bronchoscope

D a repeat bronchoscopy to retrieve and to reassess for foreign body fragments

E segmentectomy of the affected lung lobe.

Answer 90

A

General anaesthesia provides optimal conditions for the removal of most airway foreign bodies. In patients with laryngeal foreign bodies (not tracheobronchial), removal without anaesthesia can be attempted.

Rigid bronchoscopy is the primary tool for evaluating and treating aspirated foreign bodies.

Forceps remain the most commonly employed instrument for retrieval, but the Fogarty catheter has been described to aid in extracting material inaccessible to grasping instruments.

In cases that result in fragmentation of the foreign body, a second endoscopy may be required.

Should foreign material completely obstruct the airway of a pulmonary segment on repeat bronchoscopy, the surgeon should be prepared to perform a segmentectomy as required.

SPSE 1

Question 91

Which of the following factors makes an underlying oesophageal pathology more likely?

A impaction at the level of the cricopharyngeus

B the child is older than 5 years

C the child is female

D the foreign body is a tack

E the ingestion was unwitnessed

Answer 91

B

Typical foreign body impaction occurs in the area of the cricopharyngeus muscle, at the level of the aortic arch, or at the loS. Impaction at another site may indicate an underlying anatomical abnormality, but is non-specific.

In one series of patients, an impacted food bolus was associated with an abnormality in 70% of cases; tacks or other solid objects are more commonly seen in children without underlying pathology. Impaction at an older age (>5 years) has been noted to be associated with an increased likelihood of detecting an underlying anatomical abnormality.

A follow-up oesophagram may be indicated if the diagnosis remains a possibility.

The male-to-female ratio for aspirated foreign bodies in young children is 1 : 1, although in adolescents, males are more commonly affected than females.

There is no correlation with an underlying anatomical abnormality based on gender or if the aspiration is witnessed.

SPSE 1

Question 92

You are consulted to evaluate a 33-week premature neonate with slowly worsening abdominal distension, non-bilious emesis and a palpable mass in the epigastrium. He has stable vital signs and had previously been taking breast milk. Abdominal plain film reveals considerable gastric air and an intraluminal soft tissue mass within the stomach air bubble. After an abdominal ultrasound, management should include:

A nil by mouth, parenteral fluids and repeat imaging after several days

B laparotomy, gastrotomy and resection of the mass

C laparotomy, antrectomy and Bilroth I reconstruction

D CT scan of the abdomen

E upper endoscopy.

Answer 92

A

This description is classic for a lactobezoar, which is a compacted mass of undigested milk concretions. Although case series describing this entity are small, prematurity is a consistently described risk factor. It has been reported with virtually every milk product available, including breast milk and soy-based formulae. They frequently present with partial or complete gastric outlet obstruction in neonates or infants.

AXR findings include a soft-tissue mass within a large stomach air bubble. ultrasound and contrast radiographs can further support the diagnosis.

CT scan of the abdomen subjects the child to needless radiation and is not indicated. upper endoscopy is technically demanding in neonates and is not justified given the working diagnosis.

The preferred treatment for a presumed lactobezoar is initiation of intravenous hydration, and the cessation of enteral feedings.

Resolution of symptoms and the radiological findings provide confirmatory evidence of the diagnosis.

While gastric perforation has been reported in the context of a lactobezoar, laparotomy should only be reserved for rare cases of complication.

SPSE 1

Question 93

The commonest type of oesophageal atresia is:

A oesophageal atresia (OA) with distal tracheo-oesophageal fistula (TOF)

B OA without TOF

C TOF without OA

D OA with fistula to both pouches

E OA with a proximal TOF.

Answer 93

A

OA is of five types.

The commonest type is oA with distal ToF, with an incidence of 85.8%. This is also known as type ‘C’ oA.

The next common type is the pure oA with no fistula. The incidence of this anomaly is 7%–8%.

SPSE 1

Question 94

VACTORL association includes the following except:

A vertebral anomalies

B anorectal malformation

C cardiac anomalies

D tracheo-oesophageal anomalies

E liver anomalies.

Answer 94

E

VACToRl association is a spectrum of clinical conditions in the human neonate that includes vertebral (V), anorectal (A), cardiac (C), tracheo-oesophageal (To), renal (R) and limb (l) defects.

This spectrum of anomalies has not been recognised as a specific syndrome in humans but rather represents a non-random association of congenital defects of poorly known aetiology and pathogenesis and its components have been variable.

SPSE 1

Question 95

Which of the following is not true about prenatal diagnosis of oesophageal atresia?

A It is associated with maternal polyhydramnios.

B Ultrasound shows an anechoic area in the middle of the fetal neck.

C Small or absent stomach bubble on ultrasound.

D Predictive value of prenatal ultrasonography is around 40%–50%.

E Rarely diagnosed antenatally.

Answer 95

D

The predictive value of prenatal ultrasonography in the diagnosis of oA is only 20%–40%. Presence of anechoic shadow in the fetal neck with a small or absent stomach bubble on ultrasound associated with the maternal polyhydramnios is suggestive of oA antenatally.

Fetal mRI may be an important adjunct for the prenatal diagnosis of oesophageal anomalies. oA is rarely diagnosed antenatally.

SPSE 1

Question 96

Which one of these statements about oesophageal atresia is true?

A Most cases are diagnosed very late in life.

B It is usually associated with excessive salivation.

C Feeding is followed by regurgitation, choking and coughing.

D A and B are correct.

E B and C are correct.

Answer 96

E

most neonates with oA are diagnosed early in life. The earliest clinical sign is excessive salivation.

Feeding is followed by regurgitation, choking and coughing.

There may be associated cyanosis, respiratory distress, inability to swallow and inability to introduce a nasogastric tube into the stomach.

Some babies with distal fistula may distend their stomach, leading to elevation of the diaphragm and subsequent pulmonary compromise.

SPSE 1

Question 97

Which of the following investigations is essential before taking a neonate with OA to the operating room (OR)?

A renal ultrasound (US)
B MRI of the neck
C ECG
D echocardiogram (ECHO)
E spinal US

Answer 97

D

An ECHo is essential before taking the patient to the oR.

ECHo is useful in diagnosing associated cardiac anomalies that could alter the prognosis in these patients.

It also reveals the site of the aortic arch. Babies with right-sided aortic arch may need a left thoracotomy for ToF repair.

SPSE 1

Question 98

In neonates with OA and TOF, bronchoscopy may be useful for which of the following purposes?

A to locate the site of distal fistula
B to determine the number of fistulas
C for temporary occlusion of fistula
D to inject glue in recurrent TOF
E all of the above

Answer 98

E

In babies with oA and ToF, bronchoscopy is useful. Bronchoscopy is performed using a rigid bronchoscope. It is usually done just before positioning the patient for the oA and ToF repair.

Bronchoscopy has several advantages.

It provides information about the site, size, location and number of fistulas.

It allows placement of guide wire through the fistula allowing for better identification of the fistula during repair.

In babies who have a distal ToF with severe gastric distension and ineffective ventilation, bronchoscopy can be used to block the fistula site temporarily using a Fogarty catheter and save the baby.

In patients with recurrent ToF repair following previous open or thoracoscopic repair of oA and ToF, bronchoscopy can be used for injection of glue at the recurrent fistula site.

SPSE 1

Question 99

Which of the following factors is not implicated in the pathogenesis of oesophageal stricture after OA repair?

A excessive tension on the anastomosis
B two-layered anastomosis
C anastomotic leak
D gastro-oesophageal reflux (GOR)
E transpleural approach

Answer 99

E

oesophageal stricture is a common problem after oA repair. most babies benefit from a few dilatations.

The factors implicated in the pathogenesis of these stricture are tension on the anastomosis, two-layered anastomosis, anastomotic leak, use of non-absorbable silk suture, GoR and ischaemia at the site of repair.

A single-layered interrupted absorbable-suture anastomosis with minimum tension is preferred and helps prevent this complication.

Anastomotic leak after oA repair produces scarring in the area of anastomosis and forms a stricture that may need several dilatations or even resection.

There is a high incidence of GoR in patients with oA and ToF.

Antireflux treatment should be considered in all these patients after ToF repair.

Some of these patients may have recurrent strictures and may need fundoplication with dilatation of the stricture or even resection of the stricture.

There is no evidence that either the transpleural or extrapleural approach increases/decreases incidence of stricture formation.

SPSE 1

Question 100

All of these statements are true about OA except:

A GER disease (GORD) is a common complication

B GORD may be due to shortened intra-abdominal portion of the oesophagus

C short, floppy Nissen’s wrap is considered to be better than traditional Nissen’s wrap

D GORD is contributing factor for recurrent strictures

E most patients with GORD and recurrent strictures can be managed with antireflux medications.

Answer 100

E

There is a high incidence of GoR in patients with oA and ToF. This may be because of the shortened intra-abdominal portion of the oesophagus.

Also, there is altered motility at the site of anastomosis leading to reduced clearance which makes the effects of reflux more permanent.

medical antireflux treatment should be considered in all these patients after ToF repair.

Some of these patients may have recurrent strictures and may need fundoplication with dilatation of the stricture or even resection of the stricture.

A short, floppy Nissen’s wrap has been shown to be better than the traditional Nissen’s wrap. This has shown to produce less dysphagia than the original technique.

SPSE 1

Question 101

Which of the following is true about tracheomalacia in OA?

A It is defined as a weakness in the tracheal wall.

B Symptoms may be difficult to distinguish from recurrent TOF.

C It commonly requires surgery for correction.

D A and B are correct.

E A and C are correct.

Answer 101

D

Tracheomalacia is defined as a weakness in the wall of the trachea. It may be mild, moderate and severe in variety.

most cases are mild to moderate, and usually get better as the child grows.

The severe variety is where there is a complete collapse of the anterior and posterior walls of the trachea at expiration or when the child coughs.

This type may benefit from surgical treatment.

Although almost 75% of patients with oA and ToF have tracheomalacia, only about 20%–25% of patients are symptomatic from it.

Typical presentation is with ‘barking’ cough.

Symptoms may be difficult to distinguish from recurrent ToF, oesophageal stricture or GoR.

In severe cases the child may have acute, life-threatening apnoeic spells and may benefit from aortopexy.

The weak trachea is compressed between dilated oesophagus and aorta, and this pressure can be relieved by doing an aortopexy that fixes the aorta to the sternum and prevents it from compressing trachea.

SPSE 1

Question 102

In OA and distal TOF associated with duodenal atresia, which of the following is true?

A Duodenal atresia should be repaired first.

B Gastrostomy should be performed first.

C Gastric perforation does not occur.

D TOF should be ligated first.

E Upper GI contrast is required for diagnosis.

Answer 102

D

oA and distal ToF associated with duodenal atresia is a difficult problem to manage.

Since there is a communication between the trachea and distal oesophagus the air tends to take the path of least resistance and it escapes from the trachea into the distal oesophagus and then into the GI tract.

If the child is intubated it is difficult to ventilate the child because of the reason mentioned above.

If there is an associated duodenal atresia the problem becomes worse; the stomach and duodenum continue to distend till they burst/perforate making the child very sick.

Therefore oA and distal ToF associated with duodenal atresia is an emergency and needs immediate treatment – emergency thoracotomy and ligation of fistula.

The other option is to do an immediate bronchoscopy and place a Fogarty balloon catheter in the fistula to block it as a temporising manoeuvre.

Performing a gastrostomy would complicate the problem even more and may lead to immediate demise of the child from inability to ventilate.

Diagnosis is mostly clinical and based on plain X-rays. upper GI contrast studies are not required to make a diagnosis.

SPSE 1

Question 103

Regarding H-type TOF, which of the following is false?

A Prone cine oesophagram is diagnostic.

B Bronchoscopy and placement of guide wire through the fistula may be useful to identify the fistula during surgery.

C It usually presents later than the other OA anomalies.

D Most cases can be closed through a neck incision.

E The ansa cervicalis may be damaged.

Answer 103

E

H-type ToF is an anomaly where there is a connection between the trachea and the oesophagus without oesophageal atresia.

Babies with this type of anomaly usually present late with symptoms of choking with feeds, excessive gas in the GI tract with distension, or with recurrent pneumonia.

The overall incidence of this anomaly is about 4.2% of all the oA anomalies.

Diagnosis can be made by cine prone oesophagram or by rigid bronchoscopy.

At the time of bronchoscopy, a guide wire is placed through the fistula to help identify the fistula at the time of surgery.

Surgical repair is usually performed through a cervical approach and the fistula is divided and closed.

During the surgical repair the recurrent laryngeal nerve (contralateral more than ipsilateral) is at risk of injury.

SPSE 1

Question 104

Regarding surgical repair of oesophageal atresia, which of the following is true?

A It is usually accomplished through a left thoracotomy.

B The intrapleural approach prevents empyema.

C Usually a double-layer anastomosis is recommended.

D An upper pouch flap may be required to bridge the gap in some cases.

E All the above are correct.

Answer 104

D

Surgical repair of oA can be accomplished by either the open thoracotomy approach or the by the thoracoscopic approach.

The right side is the usual site of entry. In cases where there is a right-sided aortic arch, a left-sided approach is used.

The most preferred route is the extrapleural approach because leakage from the anastomosis with potential infection would be contained outside the pleura thus preventing an empyema.

The first step is to identify the fistula and divide it. Next the upper pouch is mobilised and an end-to-end interrupted singlelayer absorbable suture anastomosis is performed without tension.

In cases where the gap between the two ends is greater, a flap from the upper pouch may help bridge the gap.

SPSE 1

Question 105

In babies with a large distal TOF the following may be necessary to prevent gastric perforation except:

A endotracheal intubation beyond the fistula

B bronchoscopic occlusion of the fistula

C emergency gastrostomy

D emergency thoracotomy and ligation of fistula

E emergency thoracotomy, ligation of the fistula and repair of OA.

Answer 105

C

In patients with type ‘C’ anomaly with a large ToF the air follows the path of least resistance and bypasses the lung.

This distends the stomach and creates a risk of gastric perforation.

Pushing the endotracheal tube beyond the fistula is an important first step to solve this problem. This may not always be successful since the fistula may be low or at the bifurcation of the trachea.

The other options are to perform either an emergency bronchoscopy with balloon occlusion of the fistula or an emergency thoracotomy and ligation of the fistula.

Performing a gastrostomy would complicate the problem even more and may lead to immediate demise of the child from inability to ventilate.

If the child’s condition permits and the anaesthetist is happy with intraoperative patient stability, both a ligation of fistula and oesophageal anastomosis may be accomplished.

SPSE 1

Question 106

Which of the following is not true about congenital stenosis of the oesophagus?

A It can be of three types.

B The tracheobronchial remnant requires surgical excision.

C In most cases the stenosis is longer than 3 cm.

D A and B are correct.

E B and C are correct.

Answer 106

D

Congenital stenosis of the oesophagus is a rare condition.

There are three types: (1) a membranous web or a diaphragm, (2) fibromuscular thickening and (3) narrowing secondary to tracheobronchial remnants.

The length of the narrow segment is usually less than 3 cm.

Types 1 and 2 are located in the mid-portion of the oesophagus while type 3 is seen in the distal third of the oesophagus.

Type 1 is the rarest and type 2 is the commonest variety of oesophageal stenosis.

The first two conditions may be treated with dilatation but the third type usually needs surgical excision and end-to-end oesophageal anastomosis.

SPSE 1

Question 107

All of the following is true about laryngotracheo- oesphageal cleft (LTOC) except:

A type IV may involve one or both main bronchi

B risk of injury to the recurrent laryngeal nerve is higher with the anterior pharyngotomy approach of repair

C may be associated with OA and TOF

D type II extends beyond the cricoid lamina to the cervical trachea

E type I anomaly may not require surgical correction.

Answer 107

B

LTOC is a rare anomaly. It involves a midline communication between the larynx, trachea and oesophagus.

It is classified into four types:
type I is limited to the larynx;
type II extends beyond the cricoid lamina to the cervical trachea;
type III involves the entire trachea down to the carina;
type IV extends beyond the carina to involve one or both of the main bronchi.

Several other anomalies may be associated with lToC.

oA with ToF may occur in 20%–36% of patients with lToC.

Type I anomaly may not require surgical correction, but types II, III and IV do need surgical correction.

A right thoracotomy and extrapleural approach is used for repair. The approach may be lateral through the tracheo-oesophageal groove or anterior by opening the anterior wall of trachea.

There is a high risk of injury to the recurrent laryngeal nerve, which is greater with the lateral approach than with the anterior approach.

Treatment of this anomaly is complex and needs a multidisciplinary approach.

Survival is poor and ranges from 50% to 75%. It is also associated with a lot of morbidity.

Inability to wean the patient from the ventilator, pharyngo-oesophageal dysfunction and GoR are common postoperative problems.

SPSE 1

Question 108

Which of the following is true regarding caustic ingestion in children?

A It is always accidental.

B Most cases occur in children <3 years.

C It occurs more commonly in girls.

D It occurs more commonly in boys in all age groups.

E It is always associated with oesophageal injuries.

Answer 108

B

Caustic ingestion in children is a major health hazard with around 5000 cases per year in the united States. It most commonly occurs in children aged 1–3 years.

Boys are more frequently involved than girls. In children older than 5 years, nonaccidental injury is very likely.

Among adolescents, females predominate and the cause is usually intentional.

Approximately 20% of caustic ingestions result in some degree of oesophageal injury.

SPSE 1

Question 109

Which of the following is true regarding caustic ingestion?

A Acid and alkali ingestion can cause the same degree of injury.

B Acid ingestion causes more severe injuries than alkali ingestion.

C Ferrous sulphate tablets may induce caustic injury to the oesophagus.

D Acid injuries are more common in children.

E Acid injuries cause most damage in the duodenum and proximal small bowel.

Answer 109

C

The pH and physical form of the substance ingested play an important role in the site and type of injury.

A pH >12 or <1.5 is associated with severe corrosive injuries.

Strong alkalies in both liquid and granular form are the principal cause of severe injuries. These include sodium hydroxide (lye), potassium hydroxide and sodium carbonate. unlike alkalies, which do not have much taste, strong acids are bitter, burn on contact and are usually expectorated.

When they are swallowed, they pass rapidly through the oesophagus and cause most substantial damage in the antrum of the stomach.

This is due to pooling of swallowed acid proximal to the pylorus, which goes into spasm on contact with acid.

The injury tends to be worse when the stomach is empty.

The duodenum and proximal small intestine are relatively protected by pylorospasm.

Ferrous sulphate in both tablet and capsule form may induce caustic injury to the oesophagus and stomach.

SPSE 1

Question 110

Which of the following is not true regarding acid ingestion?

A The duodenum and small bowel are relatively protected.

B A hard eschar is formed.

C It usually results in full-thickness injury.

D Injury is worse when the stomach is empty.

E Most substantial damage occurs in the antrum of the stomach.

Answer 110

C

In cases of acid ingestion, the duodenum and proximal small bowel are relatively protected by pylorospasm.

most injuries occur in the antrum of the stomach and tend to be worse when the stomach is empty.

Acid ingestion causes coagulation necrosis with a hard eschar formation.

The protective coagulum layer at the site limits acid penetration through the mucosa.

SPSE 1

Question 111

The characteristic features of alkali ingestion include all of the following except:

A tissue destruction continues until alkali is neutralised

B destruction extends beyond the muscle layer

C coagulation necrosis occurs

D the common sites of injury are the entrance to oesophagus, mid-oesophagus and proximal to the oesophagogastric junction

E in the solid form, the injuries are usually limited to the oropharyngeal and supraglottic region.

Answer 111

C

Alkalis tend to be tasteless and odourless, so large quantities can be ingested.

The solid form results in more oropharyngeal and supraglottic injuries because of limited quantities of ingestion.

In cases of alkali ingestion, liquefactive necrosis occurs with destruction of the epithelium and submucosa, and may extend through the muscle layer.

A friable discoloured eschar forms, beneath which tissue destruction continues until all the alkali has neutralised.

The principal sites of damage are the areas of hold up such as cricopharyngeal area, the midoesophagus where it is crossed by the aortic arch and left main stem bronchus, and immediately proximal to the gastro-oesophageal junction.

SPSE 1

Question 112

Which of the following is not true regarding the clinical presentation of caustic ingestion?

A Drooling is an indication of pharyngeal injury.

B Oesophageal injury can occur without evidence of oropharyngeal injury in 20% of cases.

C Clinical presentation depends on the concentration and form of the ingested substance.

D Can present with surgical emphysema.

E Most cases remain asymptomatic.

Answer 112

B

most children with caustic ingestion remain asymptomatic and do not develop signs of corrosive injuries.

others present with signs of oral burns, retrosternal pain, tachycardia, dyspnoea and agitation.

The extent and severity of injury depends on the concentration and form of the ingested substance. oesophageal injury without any evidence of oropharyngeal injury can occur in up to 10% of cases.

Drooling and inability to swallow indicate severe posterior pharyngeal or upper oesophageal injury.

Acute respiratory distress may occur as a result of posterior pharyngeal oedema or upper airway burns.

Severe injuries can also result in oesophageal perforation with mediastinitis, shock and surgical emphysema in the neck and chest.

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Question 113

Which of the following is most urgent in the initial management of caustic ingestion in children?

A maintaining airway and oxygenation
B inducing vomiting
C fibre-optic endoscopy
D urgent surgery
E chest radiograph

Answer 113

A

Initial management, irrespective of the type of caustic ingestion, is directed at achieving haemodynamic and respiratory stability.

Attempts to remove the ingested substance by emesis or lavage are strictly contraindicated as they may reintroduce the corrosive material onto the epiglottis, vocal cords and larynx.

No neutralising chemicals should be given as they produce heat when interacting with the ingested substance, which may increase the severity of the injury.

After stabilisation of the patient, chest and abdominal radiographs are obtained as a baseline and to rule out free air in the mediasternum or abdomen.

likewise, contrast oesophagram is not helpful in the early stages of acute injury, unless there is a strong suspicion of perforation, or endoscopy cannot be performed for some reason.

Water-soluble contrast should always be used in case perforation is present.

urgent surgery is reserved for rare cases of severely charred oesophagus when oesophagectomy is performed and cervical oesophagostomy and feeding jejunostomy or gastrostomy is established.

upper gastrointestinal endoscopy is usually performed 24–48 hours after injury.

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Question 114

All of the following are true regarding the use of endoscopy in caustic ingestions except:

A it is best recommended between 24 and 48 hours after injury

B it helps in staging and management of oesophageal injuries

C it is vital to visualise the entire upper gastrointestinal tract

D there is a high risk of perforation associated with this procedure

E it is contraindicated in cases of severe pharyngeal burns

Answer 114

C

The timing of fibre-optic endoscopy is not universally agreed, but it is thought to be safe and more accurate if done within 24–48 hours after injury. This is because it takes 12–24 hours for the injured areas of the oesophagus and stomach to demarcate. The risk of perforation is high after 4–7 days, as there is completion of necrotic tissue sloughing and early granulation.

Endoscopy helps to grade the severity of the injury, plan management and predict long-term outcomes.

Perforation is a severe complication that may be accompanied by mediastinitis and even mortality. In the presence of pharyngeal burns with stridor, early oesophagoscopy is contraindicated because of the risk of aggravating the airway obstruction. Indirect laryngoscopy is useful in these cases.

Although an attempt is made to visualise the entire upper gastrointestinal tract, it is not always necessary and can be potentially dangerous and increases the risk of perforations. This can be avoided by examining the oesophagus only to the level of first significant injury.

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Question 115

Which of the following is true regarding the use of steroids in caustic ingestion?

A Long-term treatment with high dosage of steroids is indicated.

B Steroids definitely help in reduction of stricture formation.

C Steroids should always be used concomitantly with antibiotics in all cases.

D Stricture formation is related to degree of burns irrespective of steroid treatment.

E It reduces incidence of malignancy post caustic injuries of oesophagus.

Answer 115

D

The role of steroids in caustic injuries is still a mater of debate. Animal experiments have suggested that steroids inhibit inflammatory response and decrease the risk of stricture formation and perforation.

However clinical trials and retrospective reviews showed no statistical difference in prevention of strictures. Anderson et al. (1990) showed that with first- and second-degree oesophageal injuries, strictures developed in 5% of the steroid-treated patients and none of the controls, with third-degree injuries strictures occurred in 90% of the steroidtreated children and 100% of the controls.

Stricture formation is thus related to the degree of burn irrespective of steroid treatment.

It has been suggested that antibiotics should be used concomitantly along with steroids. This is based on animal studies where the risk of steroid-induced complications was shown to be higher.

There is no evidence that it decreases incidence of malignancy.

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Question 116

Which of the following is true regarding the use of antibiotics in caustic ingestion?

A They are routinely indicated in caustic injuries.

B They are indicated in cases of oesophageal or gastric perforation.

C They decrease the incidence of oesophageal or gastric perforation.

D They reduce the risk of stricture formation.

E They are indicated only when steroids are used.

Answer 116

B

The routine use of broad-spectrum antibiotics in caustic injuries still remains a matter of debate.

They are definitely indicated where there is clinical evidence of oesophageal or gastric perforation.

There is no evidence to show any decrease in rate of stricture formation or perforation following antibiotic usage.

Some authors advocate use of antibiotics along with steroids to decrease steroid-induced complications.

If considered, their use may more be justified in full-thickness injuries because of the risk of mediastinitis or peritonitis secondary to loss of mucosal and muscular barrier.

However, the role of both steroids and antibiotics in caustic ingestion is still controversial.

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Question 117

Which of the following is true regarding the feeding regimen for caustic ingestion?

A A nasogastric tube is recommended in all cases.

B The patients are kept ne per oris for 7–10 days.

C Oral feeding is commenced as soon as patient can swallow saliva.

D Total parenteral nutrition is used until endoscopic evidence of healing has occurred.

E Nasogastric tubes are contraindicated in caustic ingestion.

Answer 117

C

The routine use of a nasogastric tube is not recommended in all cases of caustic ingestion.

Introduction of a nasogastric tube before endoscopy should be avoided as it may result in a perforation. It should be passed only after the degree and severity of caustic injury has been assessed at endoscopy.

For patients with severe injuries, nasogastric tube may be passed for early feeding. It can also function as an intraluminal stent.

There is no evidence to show that a period of nil by mouth is of any benefit. oral feeding should be commenced as soon as the patient is able to swallow saliva.

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Question 118

Complications of caustic ingestion include which of the following?

A oesophageal strictures

B oesophageal carcinoma

C gastric outlet obstruction

D Barrett’s oesophagus

E all of the above

Answer 118

E

The severity of complications relates to the depth of injury.

Superficial mucosal injuries are painful but resolve without long-term sequelae.

Deep caustic injuries result in healing by fibrosis and subsequent stricture formation.

Full-thickness injuries can result in perforation and posterior fistula formation with either the trachea or aorta.

Gastric outlet obstruction is less common, but can occur specially after strong-acid ingestion resulting in the need for a partial gastrectomy.

The risk of oesophageal cancer after caustic strictures is around 5% with a latent period of 15–40 years.

Hence lifelong surveillance is important in these cases.

Barrett’s oesophagus has also been reported following sodium hydroxide ingestion (lye).

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Question 119

The management of oesophageal strictures includes all of the following except:

A oesophageal dilatation
B resection and anastomosis
C colonic patch procedures
D pyloroplasty
E gastric tube oesophagoplasty.

Answer 119

D

oesophageal dilatations are the mainstay treatment of oesophageal strictures.

These are usually commenced 3–4 weeks after injury.

These should be done at least once a week, commencing with catheters that are at least one or two French sizes smaller than the estimated diameter of the stricture.

If dilatations fail and a dense stricture develops, it requires further management. localised strictures may be resected with end-to-end anastomosis.

local steroid injections have been combined with dilatation with some success.

In those cases that have failed dilatation, various oesophageal bypass/substitution procedures such as colonic interposition, gastric tube oesophagoplasty, jejunal interposition, gastric advancement and colonic patch procedures have been used.

Pyloroplasty is often combined with some of these procedures, but in itself is not a method for treatment of oesophageal strictures.

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Question 120

Poor prognostic factors for oesophageal dilatation in caustic strictures include all of the following except:

A older children
B delay in presentation
C stricture greater than 5 cm
D ongoing oesophageal ulceration
E extensive grade III injury on initial endoscopic assessment.

Answer 120

A

Generally oesophageal dilatation is quite effective in moderate oesophageal injuries.

Poor prognostic factors include delay in presentation, extensive grade III injury, ongoing oesophageal ulceration, a dense fibrotic stricture that cracks on dilatation, a stricture longer than 5 cm, and inadequate lumen patency despite repeated dilatations over a 9–12 month period.

There is no difference in outcome or severity based on age at presentation.

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Question 121

Which of the following is true regarding long-term management of caustic injuries?

A Long-term surveillance with endoscopy is recommended.

B Surgical management of associated gastro-oesophaeal reflux is not recommended.

C Oesophageal stents are indicated in all cases of caustic strictures unresponsive to dilatations.

D Prolonged use of antireflux medications.

E Oesophageal strictures can be avoided if dilatations are commenced immediately after the injury.

Answer 121

A

oesophageal dilatation should not be performed immediately following ingestion, as it has an increased risk of perforation.

It is usually best to wait for 3–4 weeks until the acute lesion has healed and fibrosis has developed.

once commenced, to be effective, routine weekly dilatations are recommended.

A water-soluble contrast study is usually done 7–10 days following injury to assess the presence of a stricture.

The incidence and severity of gastro-oesophageal reflux must be investigated and excluded as a contributing cause of persisting stricture.

Gastro-oesophageal reflux must be managed surgically before definitive procedures are attempted.

There is no need for prolonged antireflux medications once gastro-oesophageal reflux is surgically corrected.

Because of an increased risk of oesophageal cancer following caustic strictures (5%) and a long latent period of 15–40 years, lifelong surveillance is necessary.

oesophageal stents can be used to maintain the patency of the lumen and prevent adhesions of the de-epithelialised area of the oesophagus.

If used, stents should be left in place for at least 6 weeks.

Not all cases of failed dilatation respond to stents and in many cases these are not tolerated well.

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