Esophageal disorders Flashcards
Common site of esophageal atresia
At or near the tracheal bifurcation
Common association with esophageal atresia
Tracheoesophageal fistula
Genetic defect associated with esophageal atresia
Altering of SHH signaling
What germ layer is tracheoesophageal septum derived from?
Mesoderm
Genetic syndromes associated with tracheoesophageal fistula
Trisomy 21, 18, and partial 13
VACTERAL syndrome
Vertebral defects
Anal atresia
Cardiac defects
Tracheoesophageal fistula
Renal dysplasia
Limb abnormalities
Genetic defect associated with radiologic finding of distal esophagus and stomach filled with air
Tracheoesophageal fistula
Possible esophageal causes of dysphagia
Stenosis
Webs
Rings
Diverticulum
Motility disorders
Achalasia
Ectopic tissue
Causes of acquired esophageal stenosis
Inflammatory scarring caused by GERD, irradiation, systemic sclerosis, or caustic injury
Ledge-like/semi-circumferential protrusions of mucosa seen in the upper esophagus, typically in women over 40 yo
Esophageal webs
Composition of esophageal webs
Fibrovascular CT and overlying epithelium
Triad of Plummer-Vinson syndrome
Cervical esophageal web
Mucosal lesions of mouth and pharynx
Iron deficiency anemia
Complication of Plummer-Vinson syndrome
Carcinoma of oropharynx and upper esophagus
How to diagnose esophageal web?
EGD
Barium esophagogram
2 circumferential rings of distal esophagus
Muscular A ring
Schatzki or mucosal B ring
Band of hypertrophied muscle at the proximal border of LES. Lined by squamous epithelium.
Muscular A ring
Lower esophageal narrowing, usually seen at GE junction. Composed mainly of mucosa and submucosa.
Schatzki ring (B ring)
Epithelium of upper surface of Schatzki ring
Stratified squamous epithelium
Epithelium of lower surface of Schatzki ring
Columnar epithelium
Symptoms of Schatzki ring
Usually asymptomatic, may have intermittent dysphagia
Weak area between cricopharyngeus and thyropharyngeus
Killian’s triangle
Caused by increased pressure within the distal pharynx
Zenker’s diverticulum
Complication of middle esophageal diverticulum
Adhesions to mediastinal lymph nodes
Association of middle esophageal diverticulum
Tuberculosis
Clinical features of large Zenker’s diverticulum
Halitosis
Dysphagia
Regurgitation of food from several hours before
Abnormal, simultaneous and repetitive contraction of the distal esophageal smooth muscle with normal LES tone
Diffuse/distal esophageal spasm
High amplitude contractions of the distal esophagus due to loss of the normal coordination of the inner circular and outer longitudinal layer
Nutcracker esophagus
Triad of achalasia cardia
Incomplete LES relaxation
Increase LES tone
Aperistalsis of the esophagus
Due to degeneration of the NO producing neurons that normally induce LES relaxation
Primary achalasia cardia
Causal organism of Chagas disease
Trypanosoma cruzi
Secondary causes of achalasia cardia
Chagas disease
Diabetic autonomic neuropathy
Malignancy
Amyloidosis
Sarcoidosis
Causes destruction of myenteric plexus leading to failure of peristalsis and esophageal dilation. May also effect duodenal, colonic, and ureteric myenteric plexuses.
Chagas disease
Pt presents with progressive dysphagia, inability to burp, regurgitation, and chest pain. Bird beak deformity on barium swallow.
Achalasia cardia
Possible complication of achalasia cardia
Esophageal cancer
Microscopy of distal esophagus shows inflammation surrounding ganglion cells or absent ganglion with hypertrophic nerve bundle.
Achalasia cardia
Longitudinal mucosal tears near GE junction, mostly associated with severe vomiting/retching
Mallory-Weiss tears
Transmural perforation of the esophagus extending into the mediastinum.
Boerhaave syndrome
Mackler triad in Boerhaave syndrome
Vomiting
Chest pain
Subcutaneous emphysema
LES findings in reflux esophagitis
Decreased LES tone
Incompetent LES
Extraesophageal manifestations of reflux esophagitis
Asthma
Posterior laryngitis
Chronic cough
Recurrent pneumonitis
Dental erosions
Disordered sleep
Complications of reflux esophagitis
Hemorrhage
Barrett esophagus
Strictures
Ulcerations
Associations of eosinophilic esophagitis
Atopic dermatitis
Allergic rhinitis
Asthma
Modest peripheral eosinophilia
Endoscopy of esophagus shows stacked circular rings with strictures and linear furrows
Eosinophilic esophagitis
Microscopy of EGD biopsy shows intraepithelial eosinophils that form clusters and sheets
Eosinophilic esophagitis
Is eosinophilic esophagitis associated with an increased risk of Barrett esophagus?
No
Complication of chronic GERD associated with increased risk of esophageal adenocarcinoma
Barrett esophagus
Intestinal metaplasia within the esophageal squamous mucosa
Barrett esophagus
EGD shows tongue of red, velvety, metaplasia mucosa extending form the GE junction
Barrett esophagus
Diagnostic finding on microscopy for Barrett esophagus
Goblet cells
Signs of dysplasia in Barrett esophagus microscopy
Atypical mitoses
Nuclear hyperchromasia
Irregularly clumped chromatin
Increased N:C ratio
Failure to epithelial cells to mature as they migrate to surface
Features of dysplastic glands on microscopy in Barrett esophagus
Display budding
Irregular shapes
Cellular crowning
Common cancer of upper and mid-esophagus
SCC
Common cancer of lower esophagus
Adenocarcinoma
Most common benign tumor of the esophagus
Leiomyomas
Genetic mutations in early stage esophageal adenocarcinoma
TP53
CDKN2A (p16 and p19-ARF)
Oncogenes associated with progression of esophageal adenocarcnoma
EGFR
ERBB2
MET
Cyclin D1
Cyclin E
Microscopy of distal esophageal biopsy shows mucin, atypical glands with high N:C ratio, abnormal chromatin pattern, and intestinal type morphology. Sometimes with diffusely infiltrative signet-ring cells.
Esophageal adenocarcinoma
Poor prognosis finding of esophageal adenocarcinoma
Spread to submucosal lymphatic vessels
Mutations associated of SCC
SOX2 amplification
Cyclin D1 overexpression
TP53
CDH1
NOTCH1
Gross appearance of early lesions of esophageal SCC
Small, gray-white, plaque-like thickenings
Associated with poor prognosis in esophageal SCC
Lymph mode metastases