hiatus hernia

24/2/2016 Hiatus hernia

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Official reprint from UpToDate www.uptodate.com ©2016 UpToDate

AuthorPeter J Kahrilas, MD

Section EditorNicholas J Talley, MD, PhD

Deputy EditorShilpa Grover, MD, MPH

Hiatus hernia

All topics are updated as new evidence becomes available and our peer review process is complete.Literature review current through: Jan 2016. | This topic last updated: Mar 14, 2014.

INTRODUCTION — Hiatus hernia is a frequent finding by both radiologists (image 1) and gastroenterologists.However, estimates of the prevalence of hiatus hernia vary widely due to inconsistency in the definition. There isalso confusion regarding the normal function of the gastroesophageal junction and the clinical implications of ahiatus hernia.

This topic will review the pathophysiology, classification, clinical manifestations, diagnosis, and management of ahiatus hernia. The surgical management of paraesophageal hernia and the management of gastroesophageal refluxdisease are discussed separately. (See "Paraesophageal hernia: Clinical features and surgical repair" and "Medicalmanagement of gastroesophageal reflux disease in adults" and "Approach to refractory gastroesophageal refluxdisease in adults".)

ANATOMY AND PHYSIOLOGY OF THE GASTROESOPHAGEAL JUNCTION — The distal end of theesophagus is anchored to the diaphragm by the phrenoesophageal membrane, formed by the fused endothoracicand endoabdominal fascia. This elastic membrane inserts circumferentially into the esophageal musculature, veryclose to the squamocolumnar junction, which resides within the diaphragmatic hiatus.

This configuration is altered during swallow-initiated peristalsis, a sequenced contraction of both the longitudinaland circular muscle responsible for bolus propulsion through the esophagus [1]. With contraction of the esophageallongitudinal muscle, the esophagus shortens and the phrenoesophageal membrane is stretched; its elastic recoil isthen responsible for pulling the squamocolumnar junction back to its normal position following each swallow. Thisis, in effect, "physiologic herniation," since the gastric cardia tents through the diaphragmatic hiatus with eachswallow (figure 1) [2].

The globular structure seen radiographically that forms above the diaphragm and beneath the tubular esophagusduring deglutition is termed the phrenic ampulla; it is bounded from above by the distal esophagus and from belowby the crural diaphragm (figure 2) [3]. Physiologically, the phrenic ampulla is the relaxed, effaced, and elongatedlower esophageal sphincter (LES) [4]. Emptying of the ampulla occurs between inspirations in conjunction withrelengthening of the esophagus and contraction of the LES [4,5].

The repetitive stress of swallowing, as well as that associated with abdominal straining and episodes of vomiting,subject the phrenoesophageal membrane to substantial wear and tear, making it a plausible target of age-relateddegeneration. Another potential source of stress on the phrenoesophageal membrane is tonic contraction of theesophageal longitudinal muscle induced by gastroesophageal (GE) reflux and mucosal acidification [6].

Aside from its antegrade propulsive function, the GE junction also serves to minimize GE reflux. This isaccomplished by a complex valvular mechanism, the function of which is partly attributable to the esophagus,partly to the stomach, and partly to the crural diaphragm. The esophageal element has been extensively analyzedand consists of the LES, a 2-cm segment of tonically contracted smooth muscle.

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The proximal margin of the LES extends up to and a short distance proximal to the squamocolumnarjunction.

The distal margin of the LES is more difficult to define, but careful anatomic studies suggest that it iscomposed of elements of the gastric musculature, the opposing clasp, and sling fibers of the gastric cardia(figure 3) [7].

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Physiologic studies have demonstrated that diaphragmatic contraction augments GE junction pressure, in essenceserving as an external sphincter [9]. Furthermore, if the esophagogastric junction is defined as either the end of theLES or the point at which the tubular esophagus joins the saccular stomach, there are normally about 2 cm oftubular esophagus within the abdomen [2].

DEFINITION — Hiatus hernia refers to herniation of elements of the abdominal cavity through the esophagealhiatus of the diaphragm.

CLASSIFICATION — Hiatus hernias are broadly divided into sliding and paraesophageal hernias (figure 4 andimage 2). The most comprehensive classification scheme recognizes four types of hiatus hernia.

Type I: Sliding hernia — A type I or sliding hiatus hernia is characterized by the displacement of thegastroesophageal (GE) junction above the diaphragm. The stomach remains in its usual longitudinal alignment andthe fundus remains below the GE junction.

Type II, III, IV: Paraesophageal hernias — A paraesophageal hernia is a true hernia with a hernia sac and ischaracterized by an upward dislocation of the gastric fundus through a defect in the phrenoesophageal membrane[10,11].

EPIDEMIOLOGY — It is estimated that greater than 95 percent of hiatus hernias are type I (sliding), with type II,III, and IV (paraesophageal) hernias accounting for approximately 5 percent [3,12]. Of the paraesophageal hernias,it is estimated that more than 90 percent are type III and the least prevalent are type II hernias. Estimates ofprevalence of a type I hiatus hernia in the adult population in North America vary from 10 to 80 percent [8].

ETIOLOGY — Although the etiology of most hiatus hernias is speculative, trauma, congenital malformation, andiatrogenic factors have been implicated in some patients with a type I (sliding) hiatus hernias. Type II, III, and IV(paraesophageal) hernias are a recognized complication of surgical dissection of the hiatus as occurs duringantireflux procedures, esophagomyotomy, or partial gastrectomy.

PATHOPHYSIOLOGY

Type I: Sliding hernia — Type I hiatus hernia results from progressive disruption of the gastroesophageal (GE)junction (figure 5) [13,14]. Widening of the muscular hiatal tunnel and circumferential laxity of thephrenoesophageal membrane allows a portion of the gastric cardia to herniate upward. A sliding hernia does nothave a hernia sac and slides into the chest since the GE junction is not fixed inside the abdomen. Thephrenoesophageal membrane remains intact and the hernia is contained within the posterior mediastinum (figure 4and image 2) [8].

Mechanism of gastroesophageal reflux in type I hiatus hernia — Endoscopic and radiographic studiessuggest that 50 to 94 percent of patients with GE reflux disease (GERD) have a type I hiatus hernia as comparedwith 13 to 59 percent of normals [12,15]. The likelihood of symptomatic GE reflux increases with the size of thehiatal hernia. Type I hiatus hernia impacts on reflux both by affecting the competence of the GE junction in

Surrounding the LES at the level of the squamocolumnar junction is the crural diaphragm, composed mainlyof the right diaphragmatic crus [8].

Type II hernia results from a localized defect in the phrenoesophageal membrane where the gastric fundusserves as a lead point of herniation, while the GE junction remains fixed to the preaortic fascia and themedian arcuate ligament (figure 4) [8].

Type III hernias have elements of both types I and II hernias and are characterized by both the GE junctionand the fundus herniating through the hiatus. The fundus lies above the GE junction (image 3).

Type IV hiatus hernia is associated with a large defect in the phrenoesophageal membrane and ischaracterized by the presence of organs other than the stomach in the hernia sac (eg, colon, spleen,pancreas, or small intestine) (image 4).





















preventing reflux and in compromising the process of esophageal acid clearance once reflux has occurred. (See"Pathophysiology of reflux esophagitis".)

Type II, III, and IV: Paraesophageal hernias — While it is unclear if this is either a cause or effect,paraesophageal hernias are associated with abnormal laxity of the gastrosplenic and gastrocolic ligaments, whichnormally prevent displacement of the stomach. As the hernia enlarges, the greater curvature of the stomach rollsup into the thorax. Because the stomach is fixed at the GE junction, the herniated stomach tends to rotate aroundits longitudinal axis, resulting in an organoaxial volvulus (figure 8) [29]. Infrequently, rotation occurs around thetransverse axis resulting in a mesenteroaxial volvulus [29]. Over time, the entire stomach eventually herniates,with the pylorus juxtaposed to the gastric cardia, forming an upside-down, intrathoracic stomach [8].

CLINICAL FEATURES

Clinical manifestations — Most small type I (sliding) hiatal hernias are asymptomatic. Patients with large type Ihernias may have symptoms of gastroesophageal reflux disease (GERD), the most common of which areheartburn, regurgitation, and dysphagia. (See "Clinical manifestations and diagnosis of gastroesophageal reflux inadults", section on 'Clinical manifestations'.)

Complications are rare in patients with type I hiatal hernia and are usually related to reflux. (See "Complications ofgastroesophageal reflux in adults".)

Many patients with type II, III, and IV (paraesophageal) hernias are either asymptomatic or have only vague,intermittent symptoms [10]. The most common symptoms are epigastric or substernal pain or postprandialfullness, nausea, and retching. GERD symptoms are less prevalent as compared with patients with a type Ihernia.

GE junction competence – The "two sphincter" hypothesis of GE junction competence suggests that boththe lower esophageal sphincter (LES) and the crural diaphragm encircling the LES serve a sphinctericfunction [9,16-19]. In particular, the diaphragm augments the LES by a "pinchcock" effect during transientperiods of increased intra-abdominal pressure as occur during inspiration, coughing, or abdominal straining.Experiments that quantified and statistically modeled the susceptibility to reflux during abdominal strainingconcluded that susceptibility to this mode of reflux is proportional to the size of the type I hernia (figure 6)[20]. Thus, although neither hiatus hernia nor a hypotensive LES alone results in severe GE junctionincompetence, the two conditions interact with each other. This conclusion is consistent with the clinicalexperience that exercise, tight-fitting garments, and activities involving bending at the waist exacerbateheartburn, especially after having consumed meals that reduce LES pressure.

Studies suggest that type I hiatus hernia itself may diminish LES pressure [21,22]. In addition, thecompliance or distensibility of the GE junction during sphincter relaxation, an important factor in regulatingboth the volume and the air/liquid content of gastric reflux, is increased in patients with a hiatus hernia andGERD as compared with those with GERD alone and normal controls [23]. A hiatus hernia is alsoassociated with a reduced threshold for eliciting transient lower esophageal sphincter relaxations (tLESRs) inresponse to gastric distension [24]. Furthermore, it is associated with malfunction of the GE barrier duringperiods of low LES pressure, during normal swallow-associated LES relaxation, and during deep inspirationor straining [25]. (See "Pathophysiology of reflux esophagitis".)

Compromise of esophageal emptying – Patients with type I hiatus hernia have prolongation in acidclearance especially while recumbent [26]. The hiatus hernia compromises fluid emptying from the distalesophagus by "re-reflux" from the hernia sac during swallowing (figure 7) [27,28]. Re-reflux occurspredominantly during inspiration and can be attributed to loss of the normal one-way valve function of thecrural diaphragm. By pinching off the distal esophagus, the crural diaphragm prevents backward flow fromthe stomach during each inspiration when it would be favored by a positive abdominal-thoracic pressuregradient. This one-way valve function of the crural diaphragm is grossly impaired with large type I herniasbecause a gastric pouch persists above the diaphragm [27].

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Most complications of a paraesophageal hernia are due to mechanical problems caused by the hernia and includethe following:

Radiographic findings — In patients with paraesophageal hernias, an upright radiograph, computed tomography(CT) scan, or magnetic resonance imaging (MRI) of the chest may reveal a retrocardiac air-fluid level within aparaesophageal hernia or intrathoracic stomach (image 1 and image 5). In type IV paraesophageal hernia, otherorgans within the hernia sac can be identified on CT or MRI of the chest (image 4).

DIAGNOSIS — A type I (sliding) hiatus hernia is suspected in patients with symptoms of gastroesophageal refluxdisease (GERD) including heartburn, regurgitation, and dysphagia. A type II, III and IV (paraesophageal) hernia issuspected in patients with a history of surgical dissection of the hiatus (eg, antireflux procedures,esophagomyotomy, or partial gastrectomy) and epigastric or substernal pain or fullness, nausea, or vomiting.However, hiatus hernia is not a diagnosis that is pursued in and of itself and is usually diagnosed incidentally onupper endoscopy, manometry, or imaging performed to exclude other diagnoses or as part of a preoperative work-up in patients with GERD. (See 'Radiographic findings' above and "Paraesophageal hernia: Clinical features andsurgical repair", section on 'Diagnosis' and "Clinical manifestations and diagnosis of gastroesophageal reflux inadults", section on 'Differential diagnosis' and "Overview of dysphagia in adults", section on 'Differential diagnosisof esophageal dysphagia'.)

Barium swallow — Barium swallow can determine the anatomy and size of the hernia, orientation of thestomach, and location of the GE junction (image 2). A sliding hiatus hernia is characterized by a greater than 2-cmseparation between the mucosal B ring at the site of the squamocolumnar junction and the diaphragmatic hiatus(figure 2). If a B ring is not evident on barium swallow, the demonstration of at least three rugal folds traversing thediaphragm is diagnostic of a sliding hiatus hernia (image 6).

Visualization of a portion of the gastric fundus herniating along the distal esophagus on barium swallow isdiagnostic of a paraesophageal hernia (image 7).

Upper endoscopy — On upper endoscopy, a sliding hiatus hernia is defined as a greater than 2-cm separationbetween the squamocolumnar junction and the diaphragmatic impression using the hash marks on the endoscoperelative to the incisors (picture 1).

Gastric volvulus can cause dysphagia, while postprandial pain is usually related to gastric distension. (See"Gastric outlet obstruction in adults", section on 'Gastric volvulus'.)

Bleeding, although infrequent, occurs from gastric ulceration, gastritis, or erosions (Cameron lesions) withinthe incarcerated hernia pouch [8]. (See "Portal hypertensive gastropathy".)

Respiratory complications can result from mechanical compression of the lung by a large hernia or otherorgans herniating through the hiatus.

Paraesophageal hernias may be diagnosed on an upper endoscopy, but barium swallow is the most sensitivediagnostic test.

Sliding hiatal hernias that are larger than 2 cm in axial span can be diagnosed by barium swallow,endoscopy, or esophageal manometry. In contrast, small sliding hiatus hernias can only be diagnosed withcertainty during surgery [30].

Upper endoscopy and barium swallow are unreliable for defining smaller sliding hiatus hernias as the GEjunction is highly mobile and because of the lack of standardization as to when the size of hiatus herniashould be measured with respect to deglutitive esophageal shortening and the extent of gastric distention.The magnitude of the size estimate therefore has an inherent 2-cm error. Only when a sliding hiatal herniaenlarges further, such that >2 cm of gastric pouch is herniated upward, is its presence obvious becausegastric folds are evident traversing the diaphragm both during swallow-induced shortening and at rest (image6).




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In patients with a paraesophageal hernia, retroflexed view on upper endoscopy shows a portion of the stomachherniating upward through the diaphragm adjacent to the endoscope.

High resolution manometry — On high-resolution manometry (HRM) with esophageal pressure topography(EPT), a hiatus hernia is characterized by the separation of the crural diaphragm from the lower esophagealsphincter (LES) by a pressure trough. Unlike conventional manometry, HRM with EPT can reliably identify asliding hiatus hernia as it permits real-time localization of the esophagogastric junction components withoutswallow or distention-related artifact [31]. It also allows for prolonged observation that enables the identification ofintermittent herniation (figure 9). However, small sliding hiatus hernias (<2 cm) often reduce spontaneously duringprolonged manometric recordings and can only be diagnosed with certainty during surgery. (See "High resolutionmanometry", section on 'Anatomic sphincters'.)

DIFFERENTIAL DIAGNOSIS — The differential diagnosis of hiatus hernia includes other etiologies of epigastricor substernal pain, dysphagia, heartburn or regurgitation, and refractory gastroesophageal reflux disease (GERD).This includes esophagitis, an esophageal motility disorder, functional dyspepsia, and coronary artery disease.While an evaluation to exclude these diagnoses is not required to diagnose a hiatus hernia, it may be necessary inpatients with refractory symptoms and is discussed in detail, separately. (See "Clinical manifestations anddiagnosis of gastroesophageal reflux in adults", section on 'Additional evaluation'.)

MANAGEMENT

Sliding hiatus hernia — Surgical repair of an isolated, asymptomatic type I hiatal hernia is not indicated.Management of patients with a symptomatic sliding hiatus hernia consists of management of gastroesophagealreflux disease (GERD). Medical management of GERD and the role of surgery in the management of GERD arediscussed separately. (See "Medical management of gastroesophageal reflux disease in adults" and "Surgicalmanagement of gastroesophageal reflux in adults".)

Paraesophageal hernia — The optimal management of asymptomatic patients with paraesophageal hernias iscontroversial [32]. While a few experts recommend prophylactic surgical treatment even in the absence ofsymptoms, most experts advocate against it as the annual risk of developing acute symptoms requiring emergentsurgery is less than 2 percent, the risk decreases exponentially after 65 years, and the mortality rate from electiveparaesophageal hernia repair is approximately 1.4 percent [33-37].

Surgical repair is indicated in patients with a symptomatic paraesophageal hernia [37]. Emergent repair is requiredin patients with a gastric volvulus, uncontrolled bleeding, obstruction, strangulation, perforation, and respiratorycompromise secondary to a paraesophageal hernia [10,11,32,33,38,39]. The indications for surgical repair,preoperative evaluation, and the technical aspects of surgical repair of paraesophageal hernias are discussed indetail, separately. (See "Medical management of gastroesophageal reflux disease in adults", section on 'Initialtherapy' and "Paraesophageal hernia: Clinical features and surgical repair", section on 'Indications for surgicalrepair'.)

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SUMMARY AND RECOMMENDATIONS

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Topic 2259 Version 11.0

Hiatus hernia refers to herniation of elements of the abdominal cavity through the esophageal hiatus of thediaphragm. (See 'Definition' above.)

Hiatus hernias are broadly divided into sliding and paraesophageal hernias (figure 4 and image 2). A type I orsliding hiatus hernia is characterized by the displacement of the gastroesophageal (GE) junction above thediaphragm. The stomach remains in its usual longitudinal alignment and the fundus remains below the GEjunction. Type II, III, and IV hiatus hernias or paraesophageal hernias are characterized by an upwarddislocation of the gastric fundus. Approximately 95 percent of all hiatus hernias are sliding and 5 percent areparaesophageal. (See 'Classification' above and 'Epidemiology' above.)

Although the etiology of most hiatus hernias is speculative, trauma, congenital malformation, and iatrogenicfactors have been implicated in some patients with sliding hiatus hernias. Paraesophageal hernias are arecognized complication of surgical dissection of the hiatus. (See 'Etiology' above.)

A sliding hiatus hernia results from progressive disruption of the GE junction that allows a portion of thegastric cardia to herniate upward (figure 5). In contrast, paraesophageal hernias are associated with abnormallaxity of the gastrosplenic and gastrocolic ligaments which allows the greater curvature of the stomach to rollup into the thorax. (See 'Pathophysiology' above.)

Most small sliding hiatus hernias are asymptomatic. Patients with large sliding hiatus hernias may havesymptoms of GE reflux disease (GERD). Many patients with paraesophageal hernias are eitherasymptomatic or have only vague, intermittent symptoms of epigastric or substernal pain or postprandialfullness, nausea, and retching. In patients with paraesophageal hernias, an upright radiograph, computedtomography (CT) scan, or magnetic resonance imaging (MRI) of the chest may reveal a retrocardiac air-fluidlevel within a paraesophageal hernia or intrathoracic stomach (image 1 and image 5 and image 4). (See'Clinical features' above.)

Hiatus hernia is not a diagnosis that is pursued in and of itself and is usually discovered incidentally on upperendoscopy, manometry, or imaging performed to exclude other diagnoses or as part of a preoperative work-up in patients with GERD. Paraesophageal hernias may be diagnosed on an upper endoscopy, but bariumswallow is the most sensitive diagnostic test. Sliding hiatal hernias that are larger than 2 cm in axial spancan be diagnosed by barium swallow, endoscopy, or esophageal manometry. In contrast, small sliding hiatushernias that are less than 2 cm in axial span can only be diagnosed accurately during surgery. (See'Diagnosis' above.)

Repair of an isolated, asymptomatic sliding hiatus hernia is not indicated. Management of patients with asymptomatic sliding hiatus hernia consists of management of GERD. (See 'Management' above and"Medical management of gastroesophageal reflux disease in adults" and "Approach to refractorygastroesophageal reflux disease in adults" and "Surgical management of gastroesophageal reflux in adults".)

Surgical repair for paraesophageal hernias is reserved for symptomatic patients and for management ofcomplications (eg, gastric volvulus, bleeding, obstruction, strangulation, perforation, and respiratorycompromise secondary to a paraesophageal hernia). (See 'Clinical manifestations' above and 'Management'above and "Paraesophageal hernia: Clinical features and surgical repair", section on 'Indications for surgicalrepair'.)

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GRAPHICS

Incidental finding of a hiatus hernia on chest xrayand CT scan

The AP chest xray (A) shows a hiatus hernia (asterisk) with an airfluid level (arrows). A lateral chest xray (B) confirms a hiatus hernia(asterisk) and an air fluid level in the hernia (arrows). A CT scan inthe axial plane (C) shows contrast in the hiatus hernia (asterisk).

AP: anteroposterior; CT: computed tomography.

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Esophageal shortening during swallow

(A) Before swallow Clip B marks the position of the squamocolumnarjunction (SCJ), 35 mm distal to the anchor point on the vertebralbody; clip A is affixed to the esophageal mucosa 31 mm proximally.Clip movements are referenced to point V on the vertebral column.(B) At the time of maximal esophageal shortening during swallow, clipB is 20 mm distal to point V and the distance between clips A and B isreduced to 22 mm, indicative of 29 percent shortening.(C) As elongation proceeds, first both clips descend, after which clip Bdescends, stretching the AB segment back to its initial length.(D) After swallow recovery to normal.

With permission from: Kahrilas PJ, Wu S, Lin S, et al. Attenuation ofesophageal shortening during peristalsis with hiatus hernia. Gastroenterology1995; 109:1818.




Anatomic features of sliding hiatus hernia

Representation of the anatomic features of a sliding hiatus herniaviewed radiographically during swallowing. The "A" ring is a muscularring visible during swallowing which demarcates the superior margin ofthe lower esophageal sphincter. The "B" ring at the squamocolumnarjunction is present in only about 15 percent of individuals; it permitsaccurate division of the phrenic ampulla into the esophageal vestibule(A ring to B ring) and the sliding hiatus hernia (B ring to thesubdiaphragmatic stomach). Rugal folds traversing the hiatus supportthe conviction that a portion of the stomach is supradiaphragmatic.

Reproduced with permission from: Kahrilas PJ. Hiatus hernia causes reflux:Fact or fiction? Gullet 1993; 3(Suppl):21.



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Gastric component of the LES

The proximal margin of the lower esophageal sphincter (LES) extendsup to and a short distance proximal to the squamocolumnar junction(not shown). The distal margin of the LES is more difficult to definebut careful anatomic studies suggest that it is composed of elementsof the gastric musculature, the opposing clasp and sling fibers of thegastric cardia.

Adapted from: LiebermannMeffert D, Allgöwer M, Schmid P, Blum AL. Muscularequivalent of the lower esophageal sphincter. Gastroenterology 1979; 76:31.




Sliding versus paraesophageal hiatus hernia

Distinction between a sliding hiatus hernia (type I) and paraesophageal hernia(type II). With type I hernia, the leading edge is the gastric cardia while withtype II it is the gastric fundus. The squamocolumnar junction maintains its nativeposition in the paraesophageal hernia while it is displaced upward with thesliding hernia.

SC: squamocolumnar.

Kahrilas PJ, Kim HC, Pandolfino JE. Approaches to the diagnosis and grading of hiatalhernia. Best Pract Res Clin Gastroenterol 2008; 22:601.



Barium swallow in a patient with a hiatus hernia

(A) Normal barium swallow showing the gastroesophageal junction(arrow) is at the level of the diaphragm (arrowhead) and the gastricfolds (dashed arrow) are at the same level as the diaphragm. (B) Barium swallow showing a sliding hiatus hernia (asterisk). Thegastroesophageal junction (arrow) is above the diaphragm(arrowhead) and the gastric folds (dashed arrow) are above thediaphragm. (C) Barium swallow showing a paraesophageal hernia (asterisk). Thegastroesophageal junction (arrow) is below the diaphragm(arrowhead).




Barium swallow in a patient with a type III paraesophageal hernia

(A) A chest xray prior to contrast administration shows the nasogastric tube coiled in thechest (arrows). (B) Following barium administration through the nasogastric tube, the gastroesophagealjunction and the entire stomach are noted within the chest.




Barium swallow and computed tomography (CT) scan in a patientwith a type IV paraesophageal hiatus hernia

(A) A barium swallow showing the entire stomach within the chest (asterisk). Thegastroesophageal junction (arrow) is above the diaphragm (arrowhead) and the first part ofthe duodenum (dashed arrow) is at the level of the diaphragm. (B) A CT scan through the lower chest showing the herniated stomach (asterisk) andportions of the colon within the hernial sac in the chest (arrow).




Progressive anatomic disruption of thegastroesophageal junction

Endoscopic appearance and corresponding threedimensionalrepresentation of the progressive anatomic disruption of thegastroesophageal junction as occurs with development of a type Ihiatus hernia. In the grade I configuration, a ridge of muscular tissueis closely approximated to the shaft of the retroflexed endoscope.With a grade II configuration the ridge of tissue is slightly less welldefined and there has been slight orad displacement of thesquamocolumnar junction along with widening of the angle of His. Inthe grade III appearance the ridge of tissue at the gastric entryway isbarely present and there is often incomplete luminal closure aroundthe endoscope. Note, however, that this is not a hiatal hernia because



the squamocolumnar junction is not displaced axially in theendoscopic photograph. With grade IV deformity, no muscular ridge ispresent at the gastric entry. The gastroesophageal area stays open allthe time, and squamous epithelium of the distal oesophagus can beseen from the retroflexed endoscopic view. A hiatus hernia is alwayspresent with grade IV deformity.

Reproduced from: Bredenoord AJ, Pandolfino JE, Smout AJ. Gastrooesophageal reflux disease. Lancet 2013; 381:1933. Illustration used withthe permission of Elsevier Inc. All rights reserved.




Interaction of hiatus hernia size and LES pressure onreflux

Model showing the relationship between hiatus hernia size, lower esophagealsphincter (LES) pressure, and reflux score. The reflux score increased withincreasing hernia size and lower LES pressure, both of which were required formarked reflux.

Reproduced with permission from: Sloan S, Rademaker AW, Kahrilas PJ. Determinants ofgastroesophageal junction incompetence: hiatal hernia, lower esophageal sphincter, orboth? Ann Intern Med 1992; 117:977.




Rereflux with hiatus hernia

Depiction of a radionuclide acid clearance study in a subject with ahiatus hernia. Fifteen seconds after the injection of a 15 mL bolus of0.1 N HCl labeled with 200 microcuries of 99mTcsulfur colloid,subjects swallowed every 30 seconds. The vertical axis represents theregion from the sternal notch to the stomach. The horizontal axis isthe time scale. The radioactivity is represented by the black area andno radioactivity is represented by the absence of black color. Soonafter injection, the radioactivity appears in the stomach. However,there is reflux of isotope into the esophagus followed by clearance ofthe isotope during each of the first three swallows.

Reprinted with permission from: Mittal RK, Lange RC, McCallum RW.Identification and mechanism of delayed esophageal acid clearance in subjectswith hiatus hernia. Gastroenterology 1987; 92:130. Copyright 1987 byAmerican Gastroenterological Association. This material may not bereproduced or distributed in any form or by any means without the properwritten permission of the publisher.




Volvulus in hiatus hernia

Paraesophageal hernias are associated with abnormal laxity ofstructures normally preventing displacement of the stomach, thegastrosplenic and gastrocolic ligaments. As the hernia enlarges, thegreater curvature of the stomach rolls up into the thorax. Because thestomach is fixed at the gastroesophageal junction, the herniatedstomach tends to rotate around its longitudinal axis, resulting in anorganoaxial volvulus (top panels); infrequently, rotation occursaround the transverse axis resulting in a mesenteroaxial volvulus(bottom panels).

Adapted from: Peridikis G, Hinder RA. Paraesophageal hiatal hernia. In: Hernia,Nyhus LM, Condon RE (Eds), JB Lippincott, Philadelphia 1995. p.544.




Barium swallow and computed tomography (CT) scan in a patientwith a paraesophageal hernia

(A) A barium swallow showing a paraesophageal hernia (asterisk). The gastroesophagealjunction (arrow) is at the level of the diaphragm (arrowhead). (B) An axial CT scan through the lower chest showing the region of the gastroesophagealjunction (arrow) at the level of the diaphragm (arrowhead) with a paraesophageal hernia(asterisk).




Barium swallow in a patient with a sliding hiatushernia

A barium swallow shows a moderate sized sliding hiatus hernia(asterisk) above the diaphragm (arrowhead), with welldefined gastricfolds (arrow).




Barium swallow of a large paraesophageal hernia

Barium swallow of a paraesophageal hernia. Note that as the herniated stomachenlarges, it inverts and twists causing a volvulus. In the extreme, this results in anupsidedown stomach. The esophagus is not seen in this image, but if thegastroesophageal junction is at the level of the diaphragm, this would be a type II(paraesophageal) hernia. More commonly, it is above the diaphragm, which makesit a type III (paraesophageal) hernia.




Endoscopic image of a type I sliding hiatus hernia

Retroflexed (A) and forwardlooking (B) endoscopic views of a sliding (type I)hiatus hernia. The squamocolumnar junction (SCJ) marks the distal limit of theesophageal epithelium. Distal to the SCJ is the constriction of thediaphragmatic hiatus.




Subtypes of esophagogastric junction pressure morphology on esophageal pressure topography

EGJ morphology subtypes. For each panel, the instantaneous spatial pressure variation plot corresponding to the red line on the pressure topography plotis illustrated by the black line to the right. The spatial pressure variation plot illustrates the pressure profile from top to bottom, interpolatingintermediate values between the measured values that occur at 1 cm intervals. The two main EGJ components are the LES and CD, which cannot beindependently quantified when they are superimposed as with a type I EGJ (panel A). The RIP, shown by the white horizontal dashed line, lies near theproximal margin of the EGJ. During inspiration (I) EGJ pressure increases, whereas it decreases during expiration (E).Type II EGJ pressure morphology is illustrated in panel B. Note the two peaks on the instantaneous spatial pressure variation plot; the nadir pressurebetween the peaks is greater than the intragastric pressure. The RIP is at the level of the CD.Panels C and D correspond to type III EGJ pressure morphology, defined as the presence of two peaks of the instantaneous spatial pressure variationplot with the nadir pressure between the peaks equal to or less than intragastric pressure. The RIP is at the CD with type IIIa (panel C), whereas it is atthe level of the LES in type IIIb (panel D).

EGJ: esophagogastric junction; s: seconds; LES: lower esophageal sphincter; CD: crural diaphragm; E: expiration; I: inspiration; RIP: respiratory inversion point.

From: Kahrilas PJ, Bredenoord AJ, Fox M, et al, and The International High Resolution Manometry Working Group. The Chicago Classification of esophageal motility



disorders, v3.0. Neurogastroenterol Motil 2015; 27:160. http://onlinelibrary.wiley.com/doi/10.1111/nmo.12477/abstractof John Wiley & Sons Inc. This image has been provided by or is owned by Wiley. Further permission is needed before it can be downloaded to PowerPoint, printed, sharedor emailed. Please contact Wiley's permissions department either via email: [email protected] or use the RightsLink service by clicking on the 'Request Permission'link accompanying this article on Wiley Online Library (http://onlinelibrary.wiley.com).


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