Hiatus hernia

Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-in-Chief: Amandeep Singh M.D.[2]

A hiatus hernia is the protrusion (or herniation) of the upper part of the stomach into the thorax through a tear or weakness in the diaphragm. Hiatus hernia may be classified into four subtypes: type I: Sliding hernia and type II, III, IV: paraesophageal hernias (rolling hernias). It is understood that hiatus hernia is the result of either decreased elastin of phrenoesophageal membrane due to aging that increases the risk of developing hiatal hernia. Pressure gradient between intra-abdominal and intra-thoracic pressure leads to the esophagogastric junction being displaced into normal hiatus. A rise in intraabdominal pressure and lower thoracic pressure can cause hernia. Esophageal shortening, pulls the junction into the hiatus. This physiological shortening occurs as a normal response to swallowing. The LES is shorter and weaker in large hiatus hernia and have severe reflux symptoms and trauma. Paraesophageal hernia are less common and have lesser incidence of GERD. Hiatus hernia may be caused by older age, trauma, congenital defects, increase in the abdominal pressure, obesity, and smoking. Hiatus hernia presents as gastroesophageal reflux disease (GERD) with dysphagia and must be differentiated from other causes of dysphagia. Hiatus hernias affect around 1 to 20% of the population. If left untreated, patients with a hiatus hernia may progress to develop strangulation, esophageal adenocarcinoma, and gastric volvulus. Prognosis is generally excellent and recovery after surgery in a large hernia is approximately 90%. The disease is well controlled with medical therapy but not cured. The symptoms include acid reflux, and pain, similar to heartburn, in the chest and upper stomach. In most patients, hiatus hernias cause no symptoms. Sometimes patients experience heartburn and regurgitation, when stomach acid refluxes back into the esophagus. Physical examination of patients with hiatus hernia is usually normal and unhelpful in the diagnosis. On chest radiographs, a paraesophageal hernia may appear as a soft-tissue-opacity lesion posterior to the heart near the esophageal hiatus. CT helps verify migration of the stomach cranially through the hiatus. Sagittal and coronal reformatted images often help demonstrate the hernia and the hiatal defect. Barium swallow may be helpful in the diagnosis of a hiatus hernia. Findings on a barium swallow suggestive hiatus hernia include anatomy and size of a hernia, the orientation of the stomach location of the gastroesophageal junction. In majority of cases, patients experience no discomfort and no treatment is required. However, when a hiatal hernia is large, it is likely to cause esophageal stricture which results in discomfort. Symptomatic patients benefit from not lying down immediately after meals and also benefit by elevating the head of their beds. If stress has been identified as the major risk factor, stress reduction techniques may be practiced, or if overweight, weight loss may be indicated. Certain medications causes lower esophageal sphincter (or LES to relax those medications should be avoided. Anti-acid drugs like proton pump inhibitors and H2 receptor blockers can be used to decrease the acid secretion. Surgery is the mainstay of treatment for patients with a symptoms or complications. Paraesophageal hernias can be repaired transabdominally or transthoracically. Laparoscopic approach is preferred for most patients. A Nissen-fundoplication is usually done with the surgery. A fixation of the stomach to the abdominal wall (anterior gastropexy) can be used to reduce the risk of gastric reherniation into the thoracic cavity. Complications include pneumonia, pulmonary embolism, heart failure, postoperative leak, and recurrence.

In 1846, Henry Ingersoll Bowditch was first to described hiatus hernia following postmortem examination. In the recent treatment strategies laparoscopic fundoplication have indicated very relatively low complication when compare to other techniques, quick recovery, and relatively good long term effects.

Hiatus hernia may be broadly classified into sliding hernia and paraesophageal hernias. Sliding hernia is also called type I hernia and paraesophageal hernia is divided into three subtypes including type II, type III and type IV.

It is understood that hiatus hernia is the result of either decreased elastin of phrenoesophageal membrane due to aging or imbalance in pressure gradient between intra-abdominal pressure and intra-thoracic pressure leading to the esophagogastric junction being displaced into normal hiatus. A rise in intra-abdominal pressure and lower thoracic pressure can cause hernia. Occasionally, esophageal shortening pulls the junction into the hiatus. This physiological shortening may occur as a normal response to swallowing. The LES is shorter and weaker in large hiatus hernia and have severe reflux symptoms and trauma. Paraesophageal hernia are less common and have lower incidence of gastroesophageal reflux disease.

The cause of hiatus hernia has not been identified clearly. Hiatus hernia is defined as the herniation of contents of the abdominal cavity via the esophageal hiatus of the diaphragm due to weakening of the muscles around esophagus. Hiatus hernia may be caused by older age, trauma, congenital defects, increase in the abdominal pressure, obesity, and smoking.

Hiatus hernia presents as gastroesophageal reflux disease (GERD) with dysphagia and must be differentiated from other causes of dysphagia.

Hiatus hernias affect around 1 to 20% of the population. Out of this 9 % are symptomatic, depending on the ability of the lower esophageal sphincter (LES). Approximately 95% of these categorize under “sliding” hiatus hernias, in which the lower esophageal sphincter protrudes above the diaphragm along with the stomach, and only 5% is the “rolling” type (paraesophageal), in which the lower esophageal sphincter (LES) remains stationary but the stomach protrudes above the diaphragm. A hiatus hernia is more common in older people.

Common risk factors in the development of hiatus hernia include aging, obesity, trauma, scoliosis, and congenital defects.

There is insufficient evidence to recommend routine screening for hiatus hernia.

The symptoms of a hiatus hernia usually develop in the first decade of life in children and start with symptoms such as vomiting, heartburn, regurgitation, and dysphagia. If left untreated, patients with a hiatus hernia may progress to develop strangulation, esophageal adenocarcinoma, and gastric volvulus. Prognosis is generally excellent and recovery after surgery in a large hernia is approximately 90%. The disease is well controlled with medical therapy but not cured.

When a patient is suspected for having a sliding hiatus hernia and symptoms of gastroesophageal reflux disease (GERD) which includes regurgitation, heartburn, and dysphagia epigastric pain or fullness, nausea, or vomiting. High resolution manometry with esophageal pressure topography (EPT) is the most sensitive test for the diagnosis of hiatal hernia.

The majority of patients with hiatus hernia are asymptomatic. However, some cases may develop gastroesophageal reflux disease which is associated with heart burn, regurgitation, and dysphagia.

Physical examination of patients with hiatus hernia is usually normal and unhelpful in the diagnosis. In some cases, hiatus hernia may develop gastroesophageal reflux disease (GERD). Patients with GERD usually appear ill due to the pain. Common physical examination include hoarseness of voice, laryngitis, otitis media, and lung wheezes.

There are no ECG findings associated with hiatus hernia.

An x-ray may be helpful in the diagnosis of a hiatus hernia. Findings on an x-ray suggestive of a hiatus hernia include retrocardiac opacity with the air-fluid level.

Ultrasound may be helpful in the diagnosis of a hiatus hernia. Findings on an ultrasound suggestive of a hiatus hernia include intra-abdominal esophagus measurement, gastroesophageal junction location, bowel diameter, and gastroesophageal angle.

Chest CT scan may be helpful in the diagnosis of a hiatus hernia. Findings on CT scan suggestive of a hiatus hernia include retrocardiac air-fluid level and organs within the hernia sac.

MRI may be helpful in the diagnosis of a hiatus hernia. Findings on MRI suggestive of a hiatus hernia include contiguous high-signal lesion extending from retroperitoneum into the thorax.

A hiatal hernia occurs when a part of the stomach protrudes into the thoracic cavity through the esophageal hiatus of the diaphragm. Approximately 99% of hiatal hernia are sliding, and the rest 1% are paraesophageal hernia. Barium swallow may be helpful in the diagnosis of a hiatus hernia. Findings on a barium swallow suggestive hiatus hernia include anatomy and size of a hernia, the orientation of the stomach location of the gastroesophageal junction.

There are no other diagnostic studies associated with a hiatus hernia.

In most cases, patients experience no discomfort and no treatment is required. However, when a hiatal hernia is large, it is likely to cause esophageal stricture which results in discomfort. Symptomatic patients benefit from not lying down immediately after meals and also benefit by elevating the head of their beds. If stress has been idetified as the major riskfactor, stress reduction techniques may be practiced, or if overweight, weight loss may be indicated. Certain medications causes lower esophageal sphincter (or LES to relax those medications should be avoided. Anti-acid drugs like proton pump inhibitors and H2 receptor blockers can be used to decrease the acid secretion.

Surgery is the mainstay of treatment for patients with a symptoms or complications. Paraesophageal hernias can be repaired transabdominally or transthoracically. Laparoscopic approach is preferred for most patients. A Nissen-fundoplication is usually done with the surgery. Overall mortality and morbidity rates associated with laparoscopic paraesophageal hernia repair are low. A fixation of the stomach to the abdominal wall (anterior gastropexy) can be used to reduce the risk of gastric reherniation into the thoracic cavity. Complications include pneumonia, pulmonary embolism, heart failure, postoperative leak, and recurrence.

There are no established measures for the primary prevention of hiatus hernia.

Effective measures for the secondary prevention of a hiatus hernia include preventing and treating gastroesophageal reflux disease(GERD) that includes lifestyle modifications, management of GERD by using proton pump inhibitors and surgery to prevent recurrence.

Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-in-Chief: Vamsikrishna Gunnam M.B.B.S [2]

Hiatus hernia may be broadly classified into sliding hernia and paraesophageal hernias. Sliding hernia is also called type I hernia and paraesophageal hernia is divided into three subtypes including type II, type III and type IV.

• Hiatus hernia may be classified into four subtypes:^[1][2]
  • Type I or sliding hernia: Approximately 95% of all hiatal hernias are type 1 sliding hernias. Type I sliding hernia characterized by the displacement of the gastroesophageal (GE) junction just above the diaphragm into the thoracic cavity.
  • Type II Paraesophageal hernias: A paraesophageal hernia contains a hernia sac hence is a true hernia. Type II hernias are characterized by upward displacement of the gastric fundus through phrenoesophageal membrane, while the gastroesophageal junction remains attached to the preaortic fascia and the median arcuate ligament.
  • Type III Paraesophageal hernia: Type III hernia, is like a combination oftype I and II, having a part of the stomach that has herniated through the hiatus, but also have an abnormal placement of the gastroesophageal junction in the thoracic cavity.
  • Type IV Paraesophageal hernia: Type IV hernia is associated with a large defect in the phrenoesophageal membrane and it may contain stomach along with other contents including colon, spleen, pancreas, or small intestine.

Hiatal hernia

Type I Sliding hernia

Paraesophageal hernias

Type II

Type III

Type IV

Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-in-Chief: Amandeep Singh M.D.[2]

It is understood that hiatus hernia is the result of either decreased elastin of phrenoesophageal membrane due to aging or imbalance in pressure gradient between intra-abdominal pressure and intra-thoracic pressure leading to the esophagogastric junction being displaced into normal hiatus. A rise in intra-abdominal pressure and lower thoracic pressure can cause hernia. Occasionally, esophageal shortening pulls the junction into the hiatus. This physiological shortening may occur as a normal response to swallowing. The LES is shorter and weaker in large hiatus hernia and have severe reflux symptoms and trauma. Paraesophageal hernia are less common and have lower incidence of gastroesophageal reflux disease.

• Lower esophageal sphincter (LES) is the group of smooth muscles in the distal esophagus at the gastroesophageal junction.

• LES prevents the reflux of gastric contents into esophagus.

• The mechanisms or structures preventing the reflux include:
  • Angle of His: Anhle of his is the angle between the cardia of stomach and distal portion of esophagus. It functions as a valve or flap.
  • Diaphragmatic crura
  • High LES pressure as compared to intraabdominal pressure
  • Phrenoesophageal membrane

• Phrenoesophageal ligament or membrane is fibrous elastic connective tissue arising from crura and inserts circumferentially into esophageal muscles.
• It maintains and support LES by anchoring it during peristalsis.

• The parts of diaphragm involved in the pathophysiology of the hiatus hernia include:^[2][3]
  • Crura (crus of diaphragm)
  • Esophageal hiatus (opening in diaphragm for esophagus at T10)

• It is understood that hiatus hernia is the result of either:^[4]
  • Decreased elastin of phrenoesophageal membrane due to aging^[5][6]
  • Imbalance in pressure gradient between intra-abdominal pressure and intra-thoracic pressure leading to the esophagogastric junction being displaced into normal hiatus.A rise in intraabdominal pressure and fall in intra-thoracic pressure may lead to development of hernia.^[7]
    • The causes of increased intra-abdominal pressure that may lead to hiatus hernia include:^[8]
      • Coughing
      • Vomiting
      • Heavy lifting
      • Obesity
      • Pregnancy
      • Physical strain
  • Occasionally, esophageal shortening pulls the junction into the hiatus. This physiological shortening may occur as a normal response to swallowing.^[9]The LES is shorter and weaker in large hiatus hernia and have severe reflux symptoms.^[10]
  • Trauma
• The symptoms in hiatus hernia are the result of reflux esophagitis and the mechanisms involved include:^[4]
  1. Low resting LES pressure
  2. Prolonged time taken to clear acid
  3. Delayed gastric emptying
  4. Transient LES relaxation which occurs more frequently in hiatus hernia
• The pathophysiology of hiatus hernia depends on the histological subtype:^[11]
  • Paraesophageal hernia are less common and have lower incidence of gastroesophageal reflux disease.
  • The initial clinical presentation including dysphagia, postprandial nausea and vomiting are result of impaired gastric emptying due to mechanical obstruction.
  • It enlarges with time and results in symptoms are related to the abnormal intra-thoracic location of the stomach.
  • The gastrosplenic and gastrocolic ligament are laxed.
• The hiatus hernia reduces LES sphincter.^[4]

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• Hiatus hernia is possibly transmitted in autosomal dominant to male to male inheritance pattern.^{[12][13][14][15]}
• Sliding hiatus hernia is usually sporadic but some cases have been seen following familial pattern, as do the paraoesophageal hiatus hernia.^[16]

Conditions associated with hiatus hernia include:

• Gastroesophageal reflux disease^[17]
• Esophagal ring^[18]
• Congenital diaphragmatic hernia^[12][19]
• Epiphrenic diverticula^[20]

• On gross pathology, characteristic findings of hiatus hernia include:^[18]
  • Herniated portion gets dilated and undergo ischemic changes
  • Ischemic necrosis can be seen

• On microscopic histopathological analysis, variable chronic inflammation, ischemia and necrosis may be seen in hiatus hernia.^[18]

Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-in-Chief: Vamsikrishna Gunnam M.B.B.S [2]

The cause of hiatus hernia has not been identified clearly. Hiatus hernia is defined as the herniation of contents of the abdominal cavity via the esophageal hiatus of the diaphragm due to weakening of the muscles around esophagus. Hiatus hernia may be caused by older age, trauma, congenital defects, increase in the abdominal pressure, obesity, and smoking.

Common Causes

Common causes of hiatus hernia include:^[1][2]

• Age: Hiatus hernia is most common in people who are 50 years or more of age.

• Obesity: Obesity cause Increased pressure in the abdomen.
• Gender: Hiatus hernia is more common in women than men.

Less Common Causes

Less common causes of disease name include:

• Trauma: when undergoing surgery
• Frequent coughing
• Straining with constipation
• Frequent bending over or heavy lifting
• Heredity:Being born with an unusually large hiatus
• Smoking
• Stress

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-in-Chief: Amandeep Singh M.D.[2]

Hiatus hernia presents as gastroesophageal reflux disease (GERD) with dysphagia and must be differentiated from other causes of dysphagia.

• Hiatus hernia presents as gastroesophageal reflux disease (GERD) with dysphagia and must be differentiated from other causes of dysphagia:

Disease	Signs and Symptoms								Barium esophagogram	Endoscopy	Other imaging and laboratory findings	Gold Standard
	Onset	Dysphagia			Weight loss	Heartburn	Other findings	Mental status
		Solids	Liquids	Type
GERD /Hiatus hernia	Gradual or sudden onset	+	–	Progressive	±	+	Cough Hoarseness	Normal	Free acid reflux Esophagitis with scarring Strictures	Erythema, erosions and ulceration Barrett’s esophagus Esophageal rings	Esophageal manometry may show decreased tone of LES	24 hour esophageal pH monitoring
Esophageal stricture	Gradual or sudden	+	–	Progressive	±	±	Odynophagia Cough Chest pain	Normal	Sacculations Fixed transverse folds Esophageal intramural pseudodiverticula	Mucosal edema Circumferential thickening in GERD Pale mucosa with white exudate in lymphocytic  esophagitis Swelling and hemorrhagic congestion in  caustic ingestion	Manometry may show dysmotility CT scan for staging malignant strictures	Barium esophagogram
Esophageal web	Gradual	+	+/-	Progressive	–	±	Findings of the underlying cause such as iron deficiency anemia or bullous pemphigoid	Normal	Symmetrical narrowing of the esophagus	Smooth membrane not encircling the whole lumen	Videofluoroscopy shows mucosal and submucosal foldings	Barium esophagogram
Plummer-Vinson syndrome	Gradual	+	–	Non progressive	±	–	Glossitis Koilonychia	Normal	Thin projections on the anterior esophageal wall Multiple upper esophageal constrictions	Direct visualization of esophageal webs Superior to esophagogram	Videofluoroscopy shows mucosal and submucosal foldings	Triad of Iron deficiency anemia Esophageal webs Glossitis
Diffuse esophageal spasm	Sudden	+	+	Non progressive	+	+	Chest pain	Normal	Nonperistaltic and nonpropulsive contractions Corkscrew or rosary bead esophagus	Inconclusive	Manometry shows high-amplitude esophageal contractions CT scan may show hypertrophy of esophageal muscles	Manometry
Achalasia	Gradual	+	+	Non progressive	±	–	Regurgitation of undigested food Chest pain	Normal	“Bird’s beak” or “rat tail” appearance Dilated esophageal body Air fluid level (absent peristalsis) Absence of an intragastric air bubble	Dilated esophagus Residual food fragments Normal mucosa	Residual pressure of LES > 10 mmHg Incomplete relaxation of the LES Increased resting tone of LES Aperistalsis	History of dysphagia with positive endoscopy and manometry
Systemic sclerosis	Gradual	+	+	Progressive	±	+	Muscle and joint pain Raynaud’s phenomenon Skin changes	Normal	Dysmotility Patulous esophagus	Mucosal damage Peptic stricture (advanced cases)	Positive serology for Antinuclear antibodies Rheumatoid factor Creatine kinase ESR	Skin biopsy
Zenker’s diverticulum	Gradual	+	–		±	–	Food regurgitation Halitosis Cough Hoarseness	Normal	Thin projections on esophageal wall over Killian’s triangle	Outpouching of posterior pharyngeal wall Exclude the presence of SCC	CT & MRI shows out-pouching over the posterior esophagus in the Killian’s triangle	Barium esophagography
Esophageal carcinoma	Gradual	+	+	Progressive	+	±	Lymphadenopathy Cachexia	Normal	Irregular stricture Pre-stricture dilatation	Esophageal obstruction Staging of disease	CT and PET scan is an optional test for staging of the disease	Biopsy
Stroke (Cerebral hemorrhage)	Sudden	+	+	Progressive	+	±	Dysarthria Limb weakness Fatigue	Impaired	Pooling of contrast in the pharynx Aspiration of barium contrast into the airway	Reduced opening of upper esophageal sphincter Reduced larynx elevation	CT without contrast shows acute hemorrhage as a hyperattenuating clot	CT without contrast
Motor disorders (Myasthenia gravis)	Gradual	+	+	Progressive	±		Ptosis Diplopia Fatigue	Normal	Stasis in pharynx and pooling in pharyngeal recesses	Velopharyngeal insufficiency Delayed swallowing function	CT may show anterior mediastinal mass (thymoma) Positive tensilon test	Anti–acetylcholine receptor antibody test

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-in-Chief: Vamsikrishna Gunnam M.B.B.S [2]

Hiatus hernias affect around 1 to 20% of the population. Out of this 9 % are symptomatic, depending on the ability of the lower esophageal sphincter (LES). Approximately 95% of these categorize under “sliding” hiatus hernias, in which the lower esophageal sphincter protrudes above the diaphragm along with the stomach, and only 5% is the “rolling” type (paraesophageal), in which the lower esophageal sphincter (LES) remains stationary but the stomach protrudes above the diaphragm. A hiatus hernia is more common in older people.

• It is estimated that greater than 95 percent of hiatus hernias are type I (sliding) hiatus hernia.
• Approximately 5 percent of the hiatus hernias are with type II, III, and IV (paraesophageal) hernias.

• The mortality rate of elective paraesophageal hernia repair is approximately 1.4 percent.^[1]

• Hiatus hernias commonly affects individuals older than 50 years of age.

• There is no racial predilection to hiatus hernia.

• Women are more commonly affected by Hiatus hernias than men.

Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-in-Chief: Amandeep Singh M.D.[2]

Common risk factors in the development of hiatus hernia include aging, obesity, trauma, scoliosis, and congenital defects.

• Common risk factors in the development of hiatus hernia include:^[1][2][3]
  • Aging
  • Obesity^[4]
  • Trauma^[5]
  • Scoliosis^[6]

• Less common risk factors in the development of hiatus hernia include:
  • Congenital defects such as Intestinal malrotation in children^[7]
  • Kyphosis
  • Pectus excavatum
  • Smoking

Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-in-Chief: Amandeep Singh M.D.[2]

The symptoms of a hiatus hernia usually develop in the first decade of life in children and start with symptoms such as vomiting, heartburn, regurgitation, and dysphagia. If left untreated, patients with a hiatus hernia may progress to develop strangulation, esophageal adenocarcinoma, and gastric volvulus. Prognosis is generally excellent and recovery after surgery in a large hernia is approximately 90%. The disease is well controlled with medical therapy but not cured.

• The symptoms of a hiatus hernia usually develop in the first decade of life in children and start with symptoms such as vomiting, heartburn, regurgitation, and dysphagia.^[1]
• If left untreated, patients with a hiatus hernia may progress to develop strangulation, esophageal adenocarcinoma, and gastric volvulus.^[2]

• Common complications of hiatus hernia include:
  • Anemia from bleeding from erosions caused by reflux esophagitis^[3]
  • Esophageal adenocarcinoma^[2]
  • Gastric volvulus and perforation in paraesopahgeal type^[4]
  • Strangulation in paraesophageal hernia

• Prognosis is generally excellent and recovery after surgery in a large hernia is approximately 90%.^[5]
• The disease is well controlled with medical therapy but not cured.