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General Thoracic Surgery Notecards > Congenital Disease of Esophagus > Flashcards

Congenital Disease of Esophagus Flashcards

1
Q

Development of foregut begins during which week of gestation

A

4th week

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2
Q

Foregut derivatives include:

A
  • Pharynx and respiratory system (single entity that later develops into unique structures due to lateral septation)
  • Esophagus
  • Stomach
  • Duodenum
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3
Q

Describe process of embryologic esophagal development

A
  • Esophagus elongates
  • Lumen occluded by proliferating endothelium
  • Lumen recanalizes as a series of separte vacuoles that coalesce
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4
Q

Caused by failure of lateral septation of embryronic foregut

A

Tracheoesophageal fistura (TEF) / Esophageal atresia (EA)

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5
Q

Fistual tract presumed to arise from a defect in which structure with TEF/EA

A

Branching lung bud

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6
Q

MC type of TEF/EA

A

EA with distal TEF (Type C, 84%)

  • 2nd MC: isolated EA (Type A, 8%)
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7
Q

Determines mode of presentation of TEF/EA

A

Presence or absence of EA

  • EA in utero: polyhydramnios
  • EA not diganosed in utero: excessive secreations and inability to feed
  • Presenece of distal TEF:
    • gastric distention (from respiratory tract)
    • reflux of gastric content into trachea
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8
Q

Associated anomalies with TEF/EA

A

Occur in up to 50% of TEF/EA infants

  • VATR:
    • Vertebral defects
    • Anal atresia
    • TEF
    • Radial and renal dysplasia
  • CHARGE:
    • Coloboma
    • Heart defects
    • Atresia of nasal choanae
    • Retardation of growth
    • Genital anomalies
    • Ear anomalies
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9
Q

First diagnostic test for TEF/EA

A

NGT

  • Unable to pass into stomach (coiled in proximal esophagus on lateral CXR)
  • Careful administration of a small amount of contrast can characterize presence of fistula
  • Other diagnostic modalities:
    • CT with reconstruction or MRI
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10
Q

TOC for TEF/EA

A
  • Proper resuscuitation
  • NGT (semi-sitting position)
  • Abx
  • Avoid positive pressure ventilation
    • If required, care must be take to watch for abdominal distention (distal TEF)
  • Surgical correction and restoration of GI continuity
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11
Q

Surgical approach to TEF/EA repair

A
  • Right thoracotomy
  • Division of azygous
  • Localization of fistula
  • Ligation and division of fistula
  • Primary reconstruction of esophagus
    • Esophageal lengthening: myotomies
    • Staged interposition of conduits (stomach or colon)
  • Gastrostomy tube placement
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12
Q

Specific compications associated with TEF/EA repair

A
  • PNA
  • Anastomotic leak
  • Esoophageal stricture
  • GERD
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13
Q

Second MC benign esophageal lesion

A

Esophageal cysts/duplication

  • Rare
  • Spectrum of pathology
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14
Q

Spectrum of pathology for Esophageal Cysts/Duplication includes:

A
  • True esophageal duplications cysts
  • Bronchogenic cysts
  • Enteric cysts
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15
Q

Presentation of esophageal cysts

A
  • Assymptomatic (MC)
  • Progressive enlargement:
    • obstruction
    • ulceration (may contain ectopic gastric mucosa)
    • hemorrhage
    • infection
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16
Q

Indication for surgical resection of esophageal cysts

A

Incidental discovery

17
Q

3 defining characteristics of esophageal duplication cysts

A

The criteria for classifying an esophageal cyst include the following:

  1. The cyst is within or attached to the esophageal wall.
  2. Two muscle layers cover the cyst.
  3. The lining is squamous, columnar, cuboidal, pseudostratified, or ciliated epithelium.
  • Ectopic gastric mucosa is seen in approximately 20% to 30% of the cases and is common in esophageal and midgut duplication cysts.
    • Surgical removal is the treatment of choice in most symptomatic cases. Simple cysts show enucleation, whereas duplications get excised.
18
Q

Common characteristics of esophageal duplication cysts

A
  • Do not communicate with esophageal lumen
  • Demonstrated on barium swallow
  • May be seen as extrinic mass on endoscopy or US
  • MC location: right and posterior to esophagus
  • Endoscopic ultrasound (EUS) is the modality of choice to evaluate esophageal cysts.
    • EUS can further characterize the echogenicity of the lesion.
    • It is a good tool to distinguish them from bronchogenic cysts by the absence of cartilage, proximity to the esophagus, and double muscle layer around them.
19
Q

MC location of esophageal duplication cysts

A

Right and Posterior to esophagus

20
Q

T/F

Suspected esophageal cysts should be biopsied with EUS to confirm diagnosis

A

False:

  • DO NOT biopsy due to risk of infection and hemorrhage
21
Q

Surgical approach to resection of esophageal cysts

A
  • Thoracic exposure (thoracotomy)
  • Dissection between muscular planes of esophagus
  • Resection of cysts (avoid mucosal injury)
  • Buttressed repair
  • Drainage and decompression
22
Q

Postoperative care for esophageal cyst resection

A
  • NGT decompression
  • Postoperative UGI contrast imaging to demonstrate mucosal integrity
23
Q

MC age of presentation of congenital esophageal stenosis/web

A

Adulthood

24
Q

MC location of esophageal stenosis/web

A

Distal esophagus

25
Q

3 histologic subtypes of esophageal stenosis/web

A
  1. Tracheobronchial rests (cartilage and respiratory glands)
  2. Membranous diaphragm
  3. Fibromuscular stenosis
26
Q

Diagnostic evaluation and w/u of suspected esophageal stenosis/web

A
  • Endoscopy
  • Biopsy
  • pH monitoring (exclude GERD as cause of stricture)
27
Q

TOC for esophageal stricture/web

A

Pneumatic dilation with fluoroscopy

  • May not be sufficient if cartilaginous rings present in tracheobronchial rests
  • Good outcomes for membranous and fibromuscular types

General Thoracic Surgery Notecards (38 decks)

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