Ch. 319 - Congenital Anomalies (Esophagus Only)

Question 1

MC congenital anomaly of the esophagus

Answer 1

Esophageal atresia (EA)

Question 2

T/F Majority of EA have an associated TEF

Answer 2

T

Question 3

MC form of EA

Answer 3

Type A: Proximal EA with distal TEF

Question 4

Types of EA in order of frequency

Answer 4

A: Proximal pouch with distal TEF, B: Proximal and distal pouch with no TEF, C: TEF (H-type) D: Proximal TEF with distal pouch, E: Proximal and distal TEF

Question 5

Associated features of EA

Answer 5

1) Advanced maternal age 2) European ethnicity 3) Obesity 4) Low socioeconomic status 5) Tobacco smoking

Question 6

Highest risks for mortality in neonates with TEF

Answer 6

1) Less than 1500g at birth 2) Severe cardiac anomalies

Question 7

___% of patients with EA are nonsyndromic

Answer 7

50%

Question 8

MC anomaly associated with EA

Answer 8

VATER or VACTERL syndrome

Question 9

T/F Aspiration of gastric contents via a distal fistula causes more damaging pneumonitis than aspiration of pharyngeal secretions from the blind upper pouch

Answer 9

T

Question 10

Type of TEF that might come into medical attention later in life with chronic respiratory problems, including refractory bronchospasm and recurrent pneumonia

Answer 10

Type C or H-type fistula

Question 11

Perinatal radiographic findings that might alert the physician to EA

Answer 11

Absence of the infant stomach bubble and maternal polyhydramnios

Question 12

EA that can manifest as an air-distended stomach

Answer 12

Distal TEF

Question 13

EA that can manifest as an airless scaphoid abdomen

Answer 13

Pure EA

Question 14

Imaging modalities that can demonstrate an isolated TEF (H-type)

Answer 14

1) Esophagogram with contrast medium injected under pressure 2) Bronchoscopy 3) Methylene blue dye injected into the ET tube during endoscopy is observed in the esophagus during forced inspiration

Question 15

Positioning that minimizes movement of gastric secretions into a distal fistula

Answer 15

Prone

Question 16

T/F ET intubation with mech ventilation is to be avoided in patients with EA

Answer 16

T, it can worsen distention of abdominal viscera

Question 17

Current standard surgical approach to an EA with TEF

Answer 17

Surgical ligation of TEF and primary end-to-end anastomosis of the esophagus via a right-sided thoracotomy

Question 18

Temporizing surgical intervention for EA in patients who are premature or otherwise complicated

Answer 18

Fistula ligation and gastrostomy tube placement

Question 19

Primary repair cannot be done if the gap between the atretic ends of the esophagus is

Answer 19

> 3-4cm

Question 20

Options for repair of widely gapped EA

Answer 20

Using gastric, jejunal, or colonic segments interposed as a neoesophagus

Question 21

___ contributes significantly to the respiratory disease (reactive airway disease) that often accompanies EA and TEF and also worsens the frequent anastomotic strictures after repair of EA

Answer 21

GERD

Question 22

Pulmonary condition associated with EA with TEF that improves as the child grows

Answer 22

Tracheomalacia