Esophageal Motility Disorder: Hypercontractile Esophagus

Here are the study notes for the provided text, organized into sections with detailed bullet points:

• Motility Disorders of the Esophagus*

Hypercontractile (Jackhammer) Esophagus

• Definition: Esophageal motility disorder characterized by increased pressure during peristalsis
• Also known as Nutcracker esophagus
• Clinical presentation: Chest pain similar to diffuse esophageal spasm, dysphagia to both solids and liquids
• Diagnostics: Manometry (definitive) → increased pressure during peristalsis, Upper endoscopy and Esophagram usually normal
• Management: Lower esophageal pressure, reduced esophageal contractility with medications (Dicyclomine, CCBs, nitrates, botulinum toxin injection, TCAs)

Achalasia

• Definition: Loss of peristalsis and LES relaxation failure (degeneration of Auerbach's plexus → ↑ LES pressure)
• Epidemiology: Most common in patients < 50 years old
• Clinical presentation: Dysphagia to both solids and liquids at the same time, regurgitation of undigested food, chest pain, cough, may develop malnutrition, weight loss, and dehydration
• Diagnostics: Barium esophagram → Bird's Beak appearance of LES, Manometry → increased LES pressure + lack of peristalsis, Upper endoscopy usually done to rule out esophageal cancer
• Management: Decrease LES pressure with medications (Botulinum toxin injection, nitrates) or surgery (Esophageal Myomectomy/Heller myotomy)

Neurogenic Dysphagia

• Definition: Faulty transmission of nerve impulses to the pharyngeal muscles
• Etiology: Generally caused by associated neuromuscular disease (MG, ALS, MS, stroke)
• Clinical presentation: Dysphagia to both solids and liquids, windpipe aspiration, and nose regurgitation
• Management: Directed at the underlying condition

Zenker Diverticulum

• Definition: Pharyngoesophageal pouch (false diverticulum) due to weakness at the junction of Killian's triangle
• Clinical presentation: Dysphagia, regurgitation of undigested food, cough, feeling of lump in the neck, and halitosis
• Diagnostics: Barium esophagram with video fluoroscopy (initial test of choice) → collection of dye behind the esophagus at the pharyngeal junction
• Management: Observation if small (< 1 cm) and asymptomatic, surgery (diverticulectomy, cricopharyngeal myotomy) for larger diverticula

Scleroderma Esophagus

• Definition: Scleroderma → group of rare diseases that causes hardening and tightening of the skin
• Clinical presentation: CREST syndrome → multisystem connective tissue disorder
• Management: Aimed at improving symptoms, dysphagia, and underlying complications
• Structural Disorders of the Esophagus*

Mallory-Weiss Tear

• Definition: Tear that occurs in the esophageal mucosa at the junction of the esophagus and stomach
• Pathogenesis: Mucosal lacerations develop secondary to a sudden increase in intra-abdominal pressure (forceful retching or vomiting after EtOH binge)
• Clinical presentation: Upper GI bleeding preceded by retching or vomiting
• Diagnostics: Upper endoscopy (test of choice)
• Management: Assess hemodynamic stability, fluids, and blood products as needed, endoscopic therapy, pharmacologic vasoconstrictors (Octreotide)

Esophageal Varices

• Definition: Dilated gastroesophageal submucosal veins as a complication of portal vein hypertension
• Etiology: Most common = cirrhosis
• Clinical presentation: Upper GI bleed → hematemesis, melena, hematochezia, severe → signs of hypovolemia
• Diagnostics: Upper endoscopy (test of choice) → dx and tx
• Management: Acute variceal bleed → stabilize patient, endoscopic variceal ligation (initial tx of choice), pharmacologic vasoconstrictors (Octreotide), balloon tamponade, surgical decompression (TIPS transjugular intrahepatic portosystemic shunt)
• Liver Function*

Liver Anatomy

• Largest gland in the body and largest single organ (after the skin)
• Accounts for 2.5% of adult body weight
• Located in RUQ of abdomen, protected by rib cage and diaphragm
• Dual blood supply enters through porta hepatis

Liver Functions

• Digestive: bile production and processes nutrients
• Hematologic: removing senescent RBCs and synthesis of plasma proteins, clotting factors, albumin
• Vascular: storage of blood
• Immunologic: produces immune factors, removes bacteria, and produces lymph
• Metabolic functions: removes waste, stores glycogen, minerals, vitamins, blood sugar homeostasis, excretes bilirubin

Liver Enzymes

• ALT and AST: markers of liver injury
• Normal levels: < 80 U/day
• Pattern of drinking, females > males, genetics (FH of alcoholism, genetic abnormalities), diet
• Hepatic Fibrosis*
• Definition: Happens before cirrhosis, due to chronic liver injury, can resolve
• Hepatic lobules collapse → fibrous septa form → hepatocytes regenerate with nodule formation
• Extracellular matrix components accumulate in the liver
• Good to measure to guide treatment, estimate time to cirrhosis, screening for portal hypertension

Cirrhosis

• Definition: Chronic degenerative disease, cells are damaged and replaced by scar tissue → decreased function (life-threatening)
• Causes in the US: Hep C, Alcoholic Liver disease, Non-alcoholic liver disease
• Epidemiology: 9th leading cause of death in the US
• Clinical presentation: Directly correlates to severity of disease, jaundice, weakness, fatigue, anorexia, weight loss
• Diagnostics: Liver biopsy is the gold standard, serology, imaging (Elastography, Transient Elastography, Magnetic Resonance Elastography)

Let me know if you'd like me to add or clarify anything!### Gallstones

• Ursodeoxycholic acid is an oral bile salt used to dissolve cholesterol stones, reserved for non-surgical options and limited to a maximum of 2 years.
• Complications of gallstones include developing cholecystitis, cholangitis, and pancreatitis, and a rare but highly associated risk of GB cancer.

Cholecystitis

• Definition: Acute or chronic inflammation of the gallbladder.
• Etiology: 90% of cases are caused by a gallstone impacted in the cystic duct, while 10% are acalculous, often resulting from recent major surgery/illness, history of vasculitis, AIDS, CMV, cryptosporidiosis, or microsporidiosis.
• Clinical Presentation:
  • Right upper quadrant (RUQ) and/or epigastric pain after fatty foods.
  • Nausea and vomiting (N/V).
  • Fever.
  • Murphy's sign: inhibition of inspiration during palpation of the RUQ, causing the patient to lose their breath.
• Diagnostics:
  • Complete Blood Count (CBC) shows elevated WBC.
  • AST/Alk Phos levels are usually elevated.
  • Amylase/lipase levels indicate pancreatic involvement.
  • Ultrasound (U/S) reveals stones, GB wall thickening, pericholecystic fluid, and a positive Murphy's sign.
  • HIDA (hepatic iminodiacetic acid) scan is a nuclear test that is best for cystic duct obstruction.
  • CT scan is used as a second line to U/S or HIDA to rule out other pathologies.
• Treatments:
  • Analgesics and antiemetics.
  • IV antibiotics (cephalosporin or fluoroquinolone + metronidazole).
  • Surgical resection (cholecystectomy) or stent placement, either urgently or electively.
• Complications:
  • Abscess or gangrene.
  • Choledocholithiasis/cholangitis.
  • Perforation.
  • Fistula to the bowel.
  • Pancreatitis.
  • Cancer.
  • Repeated attacks may lead to cholecystitis, a permanent inflammatory state, resulting in a porcelain gallbladder (calcified, non-functional) or cancer.

Emphysematous Cholecystitis

• Definition: A type of cholecystitis associated with gas-forming bacteria (e.g., Clostridia, E. coli).

Esophagus Anatomy

• Definition: A muscular tube connecting the pharynx to the stomach.
• Upper Esophageal Sphincter (UES) keeps food from entering the trachea.
• Lower Esophageal Sphincter (LES) prevents gastric content reflux into the esophagus.
• The esophagus has four layers: adventitia, muscular, submucosa, and mucosa.
• The lining is stratified squamous epithelium.
• The squamo-columnar junction (Z line) is where the squamous lining of the esophagus meets the columnar lining of the gastric mucosa.

Terms

• Dysphagia: subjective sensation of difficulty or abnormality of swallowing.
• Odynophagia: pain with swallowing.
• Esophagitis: inflammation or irritation of the esophagus.
• Globus sensation: a non-painful sensation of a lump, tightness, food bolus, or retained food in the pharyngeal or cervical area.

Procedures

• Upper Endoscopy (EGD): uses a thin scope with a light and camera to look inside the upper digestive tract.
• Barium Esophagram (Barium Swallow): a non-invasive imaging test that uses barium contrast X-rays to take images of the upper GI tract.
• Esophageal Manometry: a thin, flexible tube with pressure sensors is passed through the nose, down the esophagus, and into the stomach to evaluate motility and muscle contractions.

Dysphagia

• Definition: alarm symptoms that warrant prompt evaluation to define the exact cause and initiate appropriate treatment, due to structural or motility abnormalities in the passage of solids or liquids from the oral cavity to the stomach.
• Characteristics:
  • Inability to initiate a swallow or sensation of hindrance.
  • Not due to normal aging process.
  • Acute or non-acute.
  • Functional dysphagia: meets Rome IV criteria, without evidence of esophageal mucosal or structural abnormality, GERD, or eosinophilic esophagitis.
• Differentiating locations:
  • Oropharyngeal dysphagia:
    • Difficulty initiating a swallow.
    • Points to the cervical region.
    • Accompanied by regurgitation, aspiration, and sensation of residual food.
    • Drooling, coughing, choking, and dysphonia.
  • Esophageal dysphagia:
    • Difficulty swallowing several seconds after initiating a swallow.
    • Sensation of food being obstructed when passing from the upper esophagus to the stomach.
    • Points to the suprasternal notch or area behind the sternum.
    • Arises within the body of the esophagus, lower sphincter, or cardia.

Differentiating Types of Dysphagia

• Solids only + progressive symptoms:
  • Gradually progressive: esophageal stricture (acid reflux, radiation, treatment, or eosinophilic esophagitis).
  • Rapidly progressive: cancer, may have additional symptoms (chest pain, odynophagia, anemia, anorexia, and weight loss).
• Solids only + intermittent symptoms:
  • May be eosinophilic esophagitis, esophageal ring/web, or vascular anomaly.
• Solids +/- liquids:
  • May be esophageal motility disorder, distal esophageal spasm, hypercontractile esophagus, or functional disorder.
• Dysphagia + odynophagia:
  • Common in both infectious and medication-induced esophagitis.
  • Less commonly present in reflux esophagitis and Crohn's disease.

Quiz on esophageal motility disorder, characterized by increased pressure during peristalsis, and its treatment options including Botox, pneumatic dilation, and surgery.