Special Surgery 5. Benign Diseases of the Esophagus: Diagnostics and Treatment

I. Achalasia

Definition and Pathogenesis

• Achalasia: Motility disorder of the esophagus
• Main problem: impaired LES relaxation + absent/ineffective peristalsis
• Atrophy of inhibitory neurons in Auerbach myenteric plexus → LES cannot relax → functional obstruction at EGJ → food retention → proximal esophageal dilatation

Etiology

• Primary/idiopathic: most common
• Secondary achalasia / pseudoachalasia:

• Chagas disease: Trypanosoma cruzi infection
• Gastric cardia/EGJ cancer, esophageal cancer, malignant infiltration

• Suspicion of pseudoachalasia: older patient, short history, marked weight loss, difficult endoscope passage through EGJ

Clinical Features

• Progressive dysphagia to solids and liquids
• Food stuck in the esophagus
• Regurgitation of undigested food, vomiting
• Nocturnal cough, aspiration, aspiration pneumonia
• Weight loss
• Retrosternal/chest pain

Diagnosis

• Barium swallow: bird-beak narrowing at EGJ + dilated esophagus proximal to LES
• Upper endoscopy: retained food/saliva, tight EGJ; exclude tumor/pseudoachalasia
• Esophageal manometry: gold standard

• Shows absent peristalsis + incomplete LES relaxation

Treatment

1. Definitive treatment
2. Conservative / high-risk patient treatment

1. Definitive Treatment

• Goal: reduce LES obstruction; peristalsis usually does not recover
• Pneumatic dilation: endoscopic balloon disruption of LES; risk = perforation
• Laparoscopic Heller myotomy: longitudinal incision of LES muscle

• Usually combined with partial fundoplication → prevents reflux

• POEM: peroral endoscopic myotomy; useful minimally invasive option, especially spastic/type III achalasia

2. Conservative / High-Risk Patient Treatment

• Botulinum toxin injection into LES: elderly/high surgical-risk patients; temporary effect
• Nitrates, calcium channel blockers: decrease LES tone, weak/short effect

II. Esophageal Diverticulum

Definition

• Esophageal diverticulum: Abnormal outpouching from the esophageal wall
• Food retention → regurgitation, halitosis, aspiration

Classification

1. According to Location

• Upper esophageal diverticulum: Zenker diverticulum, hypopharyngeal/pharyngoesophageal
• Middle esophageal diverticulum: Rokitansky diverticulum, near tracheal bifurcation
• Lower esophageal diverticulum: Epiphrenic diverticulum, just above diaphragm

2. According to Histology

• True diverticulum: all wall layers protrude
• False diverticulum: only mucosa and submucosa protrude

3. According to Pathomechanism

• Pulsion diverticulum: inadequate sphincter relaxation/motility disorder → increased intraluminal pressure

• Usually false diverticulum
• Common sites: Zenker and epiphrenic diverticula

• Traction diverticulum: mediastinal inflammation/scarring → retraction of esophageal wall

• Usually true diverticulum
• Common site: middle esophagus

Main Types

• Zenker diverticulum: most common, pulsion false diverticulum through Killian triangle
• Rokitansky / mid-esophageal diverticulum: classically traction type; may follow mediastinal inflammation/lymphadenitis
• Epiphrenic diverticulum: distal pulsion diverticulum; often associated with achalasia or other motility disorder

Clinical Features

• Dysphagia
• Regurgitation of undigested food
• Chronic cough
• Aspiration, aspiration pneumonia
• Halitosis, neck gurgling/swelling in Zenker diverticulum

Diagnosis

• Barium swallow: best initial test; shows pouch, neck and retention
• Endoscopy: use cautiously in large Zenker diverticulum because of perforation risk
• Esophageal manometry: important before epiphrenic diverticulum surgery to detect motility disorder

Treatment

• Small asymptomatic diverticulum → observation
• Symptomatic diverticulum → treat pouch + underlying sphincter/motility disorder
• Zenker diverticulum:

• Endoscopic stapling/septotomy
• Open diverticulectomy or diverticulopexy + cricopharyngeal myotomy

• Epiphrenic diverticulum: diverticulectomy + esophageal myotomy ± partial fundoplication
• Mid-esophageal diverticulum: treat underlying inflammatory/motility cause; surgery if large, symptomatic or complicated

III. Hiatal Hernia

Definition and Etiology

• Hiatal hernia: proximal stomach and/or EGJ passes through esophageal hiatus into mediastinum
• Risk factors: advanced age, smoking, obesity, pregnancy, ascites, chronic cough/COPD, connective tissue weakness

Types

1. Sliding type, type I
2. Rolling / paraesophageal type, type II
3. Mixed type, type III
4. Upside-down stomach / giant paraesophageal hernia, type IV

1. Sliding Type: Type I

• Most common, about 90-95%
• EGJ and gastric cardia slide above diaphragm
• Usually causes GERD symptoms

2. Paraesophageal Type: Type II-IV

• Type II: EGJ remains below diaphragm; fundus herniates beside esophagus
• Type III: EGJ and stomach both herniate
• Type IV: large hernia with stomach ± other organs in thorax
• Main danger: incarceration, obstruction, volvulus, strangulation, bleeding

Clinical Features

• Often asymptomatic
• Sliding hernia: heartburn, epigastric/retrosternal pain, acid regurgitation, dysphagia, cough, hoarseness

• Worse supine, after meals, at night
• Chronic reflux → esophagitis → stricture / Barrett esophagus → adenocarcinoma risk

• Paraesophageal hernia: postprandial fullness, vomiting, epigastric/substernal pain, dysphagia/odynophagia, dyspnea
• Complications: upper GI bleeding, Cameron ulcers, gastric obstruction, volvulus, ischemia, perforation

Diagnosis

• Barium swallow: defines anatomy, type, size, obstruction/volvulus
• Upper endoscopy: esophagitis, Barrett, ulcer, stricture, bleeding; identifies Z-line/EGJ and diaphragmatic pinch
• CT: large paraesophageal hernia, volvulus or acute complication
• Esophageal manometry: preoperative planning and exclusion of major motility disorder

Treatment

1. Sliding hernia
2. Paraesophageal hernia

1. Sliding Hernia

• Treat as GERD
• Lifestyle: weight reduction, diet change, smoking cessation, avoid late meals, elevate head of bed
• Medical: PPI
• Surgery only for refractory GERD, complications or large symptomatic hernia after objective workup

2. Paraesophageal Hernia: Type II-IV

• Symptomatic type II-IV → surgical repair if medically fit
• Emergency operation: volvulus, strangulation, perforation, obstruction, uncontrolled bleeding
• Usual operation: laparoscopic transabdominal repair

• Reduction of herniated stomach/organs
• Dissection/reduction of hernia sac
• Esophageal mobilization
• Closure of hiatal defect / cruroplasty
• Fundoplication: reinforces anti-reflux barrier
• Gastropexy: selected high-risk or recurrent cases

• Fundoplication types: Nissen 360 degrees, Toupet posterior partial, Dor anterior partial

IV. Gastroesophageal Reflux Disease (GERD)

Definition and Etiology

• Gastroesophageal reflux: Regurgitation of stomach contents into the esophagus
• GERD: Reflux causing troublesome symptoms and/or esophageal injury/complications
• Main mechanism: incompetent anti-reflux barrier at EGJ/LES
• Reduced LES tone: alcohol, calcium channel blockers, nitrates, anticholinergics, previous surgery
• Increased intra-abdominal/intragastric pressure: obesity, pregnancy, chronic cough, ascites, delayed gastric emptying
• Hiatal hernia

Clinical Features

• Heartburn / retrosternal burning pain
• Acid regurgitation
• Worse after meals, supine position, bending, at night
• Epigastric pain, dyspepsia/indigestion
• Dysphagia, non-cardiac chest pain
• Chronic cough, hoarseness, reflux laryngitis, asthma-like symptoms
• Alarm symptoms: dysphagia/odynophagia, weight loss, bleeding/anemia, persistent vomiting

Complications

• Reflux esophagitis, ulcer
• Peptic stricture
• Barrett esophagus → adenocarcinoma risk
• Reflux laryngitis
• Aspiration pneumonia

Diagnosis

• Typical heartburn/regurgitation without alarm signs → clinical diagnosis ± empirical PPI trial
• Upper endoscopy:

• Indications: alarm symptoms, refractory symptoms, suspected complication, preoperative evaluation
• Findings: erythema, erosions, ulcers, peptic stricture, Barrett mucosa

• 24-hour pH monitoring / pH-impedance: gold standard for unclear, atypical or refractory cases
• Esophageal manometry: not diagnostic for GERD; needed before fundoplication to exclude achalasia/major motility disorder
• Barium swallow: hiatal hernia, stricture, short esophagus or preoperative anatomy

Treatment

1. Conservative therapy
2. Medical therapy
3. Surgical therapy

1. Conservative Therapy

• Weight reduction if overweight/obese
• Avoid trigger foods/drinks: fatty meals, alcohol, soda, caffeine, spicy food
• Do not eat 2-3 h before sleeping
• Smoking cessation
• Elevation of head of bed for nocturnal symptoms
• Avoid drugs decreasing LES tone if possible: CCB, nitrates, anticholinergics

2. Medical Therapy

• PPI: mainstay
• H2-receptor antagonist: mild/nocturnal symptoms or add-on in selected cases
• Antacids/alginates: intermittent symptom relief

3. Surgical Therapy

• Indications: unsuccessful optimized medical treatment, medication intolerance, objective GERD with complications, large hiatal hernia/paraesophageal component
• Laparoscopic fundoplication:

• Nissen fundoplication: 360-degree wrap of gastric fundus around distal esophagus/LES
• Partial fundoplication: Toupet/Dor if dysphagia risk or motility disorder
• Hiatal hernia repair/cruroplasty if present