Congenital Disease of Esophagus Flashcards
(29 cards)
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
