Congenital Disease of Esophagus Flashcards
Development of foregut begins during which week of gestation
4th week
Foregut derivatives include:
- Pharynx and respiratory system (single entity that later develops into unique structures due to lateral septation)
- Esophagus
- Stomach
- Duodenum
Describe process of embryologic esophagal development
- Esophagus elongates
- Lumen occluded by proliferating endothelium
- Lumen recanalizes as a series of separte vacuoles that coalesce
Caused by failure of lateral septation of embryronic foregut
Tracheoesophageal fistura (TEF) / Esophageal atresia (EA)
Fistual tract presumed to arise from a defect in which structure with TEF/EA
Branching lung bud
MC type of TEF/EA
EA with distal TEF (Type C, 84%)
- 2nd MC: isolated EA (Type A, 8%)
Determines mode of presentation of TEF/EA
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
Associated anomalies with TEF/EA
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
First diagnostic test for TEF/EA
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
TOC for TEF/EA
- 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
Surgical approach to TEF/EA repair
- 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
Specific compications associated with TEF/EA repair
- PNA
- Anastomotic leak
- Esoophageal stricture
- GERD
Second MC benign esophageal lesion
Esophageal cysts/duplication
- Rare
- Spectrum of pathology
Spectrum of pathology for Esophageal Cysts/Duplication includes:
- True esophageal duplications cysts
- Bronchogenic cysts
- Enteric cysts
Presentation of esophageal cysts
- Assymptomatic (MC)
- Progressive enlargement:
- obstruction
- ulceration (may contain ectopic gastric mucosa)
- hemorrhage
- infection
Indication for surgical resection of esophageal cysts
Incidental discovery
3 defining characteristics of esophageal duplication cysts
- Present within esophageal wall
- Covered by two muscle layers
- Contain embryonic lining similar to that of esophagus
Common characteristics of esophageal duplication cysts
- 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
MC location of esophageal duplication cysts
Right and Posterior to esophagus
T/F
Suspected esophageal cysts should be biopsied with EUS to confirm diagnosis
False:
- DO NOT biopsy due to risk of infection and hemorrhage
Surgical approach to resection of esophageal cysts
- Thoracic exposure (thoracotomy)
- Dissection between muscular planes of esophagus
- Resection of cysts (avoid mucosal injury)
- Buttressed repair
- Drainage and decompression
Postoperative care for esophageal cyst resection
- NGT decompression
- Postoperative UGI contrast imaging to demonstrate mucosal integrity
MC age of presentation of congenital esophageal stenosis/web
Adulthood
MC location of esophageal stenosis/web
Distal esophagus
3 histologic subtypes of esophageal stenosis/web
- Tracheobronchial rests (cartilage and respiratory glands)
- Membranous diaphragm
- Fibromuscular stenosis
Diagnostic evaluation and w/u of suspected esophageal stenosis/web
- Endoscopy
- Biopsy
- pH monitoring (exclude GERD as cause of stricture)
TOC for esophageal stricture/web
Pneumatic dilation with fluoroscopy
- May not be sufficient if cartilaginous rings present in tracheobronchial rests
- Good outcomes for membranous and fibromuscular types
Components of the DeMeester Score
- Total % time pH < 4.0
- % time pH < 4.0 in upright position
- % time pH < 4.0 in recumbent position
- Total reflux episodes
- Total reflux episodes longer than 5min
- Duration of the longest reflux episode
what type of testing shoudl be performed if the patient cannot be off PPI
Esophageal impedance testing
Can detect the type of reflux regardless of pH
