Sliding Hernia (Paraesophageal)


Article Author:
Thomas Watson


Article Editor:
Troy Moritz


Editors In Chief:
Sebastiano Cassaro
Joseph Lee
Tanya Egodage


Managing Editors:
Orawan Chaigasame
Carrie Smith
Abdul Waheed
Frank Smeeks
Kristina Soman-Faulkner
Benjamin Eovaldi
Radia Jamil
Sobhan Daneshfar
Saad Nazir
William Gossman
Pritesh Sheth
Hassam Zulfiqar
Navid Mahabadi
Steve Bhimji
John Shell
Matthew Varacallo
Ahmad Malik
Mark Pellegrini
James Hughes
Beata Beatty
Hajira Basit
Phillip Hynes


Updated:
5/5/2019 10:18:07 PM

Introduction

There are four types of hiatal hernias. However, the sliding hiatal hernia (type 1) is the most common and accounts for up to 95% of all hiatal hernias. Type 1 hiatal hernias solely involve "sliding" of the gastroesophageal (GE) junction into the thoracic cavity. Type 2 to 4 hiatal hernias are true paraesophageal hernias (PEHs) and are classified based upon on location of the GE junction as well as what has herniated into the thoracic cavity. A type 2 hiatal hernia has a GE junction in the normal anatomic position, but a portion of the stomach, most often the fundus, has herniated through the hiatus. Type 3, like type 2, have a portion of the stomach that has herniated through the hiatus, but also have an abnormal position of the GE junction in the thoracic cavity. Type 4 has an abnormal GE junction position like type 1 and 3. However, another organ, most often a portion of the colon, has herniated into the thoracic cavity.[1][2]

Etiology

In sliding hiatal hernias, the GE junction migrates above the diaphragm secondary to increased laxity of the phrenoesophageal ligament. The ligament remains intact, and the GE junction migrates into the posterior mediastinum. With less amount of esophagus intra-abdominally, this predisposes the GE junction to malfunction leading to reflux. Regarding type 2 to 4, there is not one predominant theory to explain them all, and thus most PEHs are multifactorial in etiology. PEHs most often are associated with, but not limited to the widening of the esophageal hiatus or congenital versus acquired, such as prior history of surgical dissection, trauma, persistent increases in intra-abdominal pressure, or esophageal shortening due to fibrosis or scarring from repeated exposure to noxious content.[3][4][5]

Epidemiology

The prevalence of sliding hiatal hernias ranges from 10% to 60%. Sliding hiatal hernias represent 95% of all hiatal hernias with paraesophageal hernias making the remaining. Older individuals are affected more commonly than those younger than the age of 50. There is an increased preponderance among obese patients and those who suffer from conditions that increase intra-abdominal pressure, for example, chronic constipation or excessive vomiting. Of the paraesophageal hernias, the most and least frequent are type 3 and 2, respectively.  The term "giant hiatal hernia" refers to a hiatal hernia that has 30% or more of the stomach superior to the hiatus and in the thoracic cavity.

Pathophysiology

Sliding hernias are thought to develop secondary to progressive disruption of the GE junction, which leads to a widening and circumferential laxity of the phrenoesophageal membrane. Herniation results in an alteration of the physiologic anatomy of the GE junction by decreasing the amount of intra-abdominal esophagus thus allowing reflux of gastric contents.  It has been shown the susceptibility of the esophagus to reflux during abdominal straining was directly proportional to the size of a sliding hernia. Sliding hernias predispose to reflux by altering both the competence of the GE junction as well as decreasing the clearance of contents in the distal esophagus. Sliding hiatal hernias impair clearance in the distal esophagus by a phenomenon called "re-reflux." It occurs predominantly during inspiration and is most pronounced in the recumbent position. The crural diaphragm is thought to pinch off the distal esophagus and act as a one-way valve. In the setting of type 1 sliding hiatal hernia, the GE junction is no longer intra-abdominal and has subsequently migrated superiorly through the hiatus and above the crural diaphragm.  As mentioned earlier, type 2 to 4 hiatal hernias are not clearly understood and thought to be from multiple contributing risk factors. Most often the combination of an increased diameter of the hiatus in the setting of persistent increased intra-abdominal pressure set the stage to develop a paraesophageal hernia.

Histopathology

Patients can develop esophageal mucosal ulcerations called Cameron ulcers. These most often are seen in type 1 hiatal hernias. Otherwise, there are no other histopathological changes associated with hiatal hernias.

History and Physical

Most patients with sliding hernias (type 1) are generally asymptomatic. However if symptoms are present, they are usually nonspecific and consist of epigastric abdominal pain, fullness, and usually, have a history of GERD. As mentioned earlier, this is a consequence of the inadequate length of the intraabdominal esophagus leading to reflux of gastric contents. Patients with PEHs can develop symptoms of obstruction. If the obstruction occurs at the level of the GE junction, then dysphagia is the most common symptom. If any contents remain in the distal esophagus, then regurgitation will occur. However, if the distal stomach becomes obstructed, the resulting proximal gastric distention will lead to nausea and vomiting and in some cases chest pain that radiates to the back. Patients that develop Cameron ulcers can show signs and symptoms of anemia such as pallor and fatigue if there is ongoing occult blood loss.

Evaluation

Diagnosis is based primarily on radiographic findings, upper endoscopy, and esophageal manometry. An upper gastrointestinal (GI) series remains a mainstay in the diagnoses of hiatal hernias. The study can show the location of the GE junction, size of the hernia defect, and the orientation of the stomach. Upper endoscopy can evaluate for the presence of Cameron ulcers as well as directly visualize the amount of stomach that has herniated into the chest. High-resolution manometry (HRM) and esophageal pressure topography (EPT) has been shown to reliably detect hiatal hernias, which is characterized by a pressure trough located between the crural hiatus and GE junction. This modality provides information on intraluminal pressure, lower esophageal sphincter function, and coordinated contractile movements of the esophagus. This is important in surgical planning in determining which antireflux procedure would be needed if the repair was indicated.[5][6][7]

Treatment / Management

Patients with a type 1, sliding hernia, do not require surgical management. Medical management of reflux disease with proton-pump inhibitors and H-2 blockers is the mainstay of therapy. Surgical management of asymptomatic patients with paraesophageal hernias also remains controversial. Most experts do not recommend surgical management in asymptomatic patients because the annual risk of developing symptoms in which emergent surgical intervention is necessary is around 2%, yet the mortality rate for elective repair is approximately 1.5%. While consensus is not clear on asymptomatic patients, there is unanimous agreement symptomatic patients benefit from elective surgical repair. The standard of care is laparoscopic repair consisting of four key elements: excision of the hernia sac, maintaining the adequate length of the intra-abdominal esophagus, repair of the hiatal defect, and gastric fixation with the antireflux procedure.[8][9][10][11]

Enhancing Healthcare Team Outcomes

Paraesophageal hernias are rare but when they present are associated with high morbidity and mortality. The condition is best managed by a multidisciplinary team that includes a surgeon, gastroenterologist, internist, radiologist and ICU nurses. The current evidence reveals that asymptomatic patients should be monitored and symptomatic patients need surgery.

The outcomes after surgery are poor and associated with severe complications that include gastric perforation, bleeding, aspiration, wound breakdown and adverse cardiac events. The goal should be to optimize the patient prior to surgery and ensure that the patient is fit for surgery.[12][13]


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Sliding Hernia (Paraesophageal) - Questions

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What is the major difference between a paraesophageal hernia and sliding hernia?



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Which of the following is false about paraesophageal hernias?



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A patient has been diagnosed with a paraesophageal hernia, which of the following is a false statement about the disorder?



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Which of the following statements regarding the repair of a paraesophageal hernia is incorrect?



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A patient presents with a transient episode of retching and inability to vomit. His chest x-ray shows a retrocardiac mass filled with air. He claims to be feeling fine and wants medical treatment for his upset stomach. Which of the following is most appropriate for the management of this patient?



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A 65 year old female has esophagogastroduodenoscopy for upper abdominal pain. The gastroesophageal junction 3 cm proximal to the diaphragmatic esophageal hiatus. Biopsy below the junction shows normal gastric mucosa. What is the diagnosis?



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Which is correct regarding paraesophageal hernias?



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Which of the following statements about the repair of type III paraesophageal hernias (PEH) is true?



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A 74-year-old male with iron deficiency anemia and severe gastroesophageal reflux disease is found to have a type III paraesophageal hernia. He subsequently undergoes upper endoscopy and Cameron ulcers are appreciated. Which of the following is the best next step?



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A 43-year-old female undergoes upper endoscopy for persistent symptoms of reflux disease and is found to have a type I hiatal hernia. Which of the following about type I sliding hiatal hernias is true?



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Which of the following is not true about type I hiatal hernias?



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A 72-year-old female undergoes esophagogastroduodenoscopy for epigastric abdominal pain. She is found to have linear ulcerations in the mucosa consistent with Cameron ulcers. Which of the following is not true regarding hiatal hernias?



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A 46-year-old male presents to the emergency department with sudden-onset chest pain and is diaphoretic. EKG and cardiac enzymes are within normal limits. A chest x-ray obtained showed pneumomediastinum with a silhouette posterior to the cardiac structures. He undergoes emergent exploration via thoracotomy and is found to have a perforation in the body of the stomach with a large hiatal hernia. Which of the following is not true regarding hiatal hernias?



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Sliding Hernia (Paraesophageal) - References

References

Armijo PR,Pokala B,Misfeldt M,Pagkratis S,Oleynikov D, Predictors of Hiatal Hernia Recurrence After Laparoscopic Anti-reflux Surgery with Hiatal Hernia Repair: a Prospective Database Analysis. Journal of gastrointestinal surgery : official journal of the Society for Surgery of the Alimentary Tract. 2019 Apr;     [PubMed]
Arcerito M,Changchien E,Falcon M,Parga MA,Bernal O,Moon JT, Robotic Fundoplication for Gastroesophageal Reflux Disease and Hiatal Hernia: Initial Experience and Outcome. The American surgeon. 2018 Dec 1;     [PubMed]
Patoulias D,Kalogirou M,Feidantsis T,Kallergis I,Patoulias I, Paraesophageal Hernia as a Cause of Chronic Asymptomatic Anemia in a 6 Years Old Boy; Case Report and Review of the Literature. Acta medica (Hradec Kralove). 2017;     [PubMed]
Selvakumar D,Sian K,Iyengar AJ,Mejia R, An unusually large paraesophageal hernia mimicking a Bochdalek hernia. Journal of thoracic disease. 2017 Aug;     [PubMed]
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Nishizawa T,Suzuki H, Hiatal hernia. Nihon rinsho. Japanese journal of clinical medicine. 2016 Aug;     [PubMed]
Namgoong JM,Kim DY,Kim SC,Hwang JH, Hiatal hernia in pediatric patients: laparoscopic versus open approaches. Annals of surgical treatment and research. 2014 May;     [PubMed]
Targarona EM,Grisales S,Uyanik O,Balague C,Pernas JC,Trias M, Long-term outcome and quality of life after laparoscopic treatment of large paraesophageal hernia. World journal of surgery. 2013 Aug;     [PubMed]
Coughlin M,Fanous M,Velanovich V, Herniated pancreatic body within a paraesophageal hernia. World journal of gastrointestinal surgery. 2011 Feb 27;     [PubMed]
Schieman C,Grondin SC, Paraesophageal hernia: clinical presentation, evaluation, and management controversies. Thoracic surgery clinics. 2009 Nov;     [PubMed]
Boushey RP,Moloo H,Burpee S,Schlachta CM,Poulin EC,Haggar F,Trottier DC,Mamazza J, Laparoscopic repair of paraesophageal hernias: a Canadian experience. Canadian journal of surgery. Journal canadien de chirurgie. 2008 Oct;     [PubMed]
Low DE,Unger T, Open repair of paraesophageal hernia: reassessment of subjective and objective outcomes. The Annals of thoracic surgery. 2005 Jul;     [PubMed]

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