SAGES Guidelines - Achalasia, Hiatal Hernia, GERD

Question 1

Patients with suspected achalasia should undergo what workup?

Answer 1

barium esophagram, an upper endoscopy, and esophageal manometry to confirm the diagnosis (+++, strong)

Question 2

What does barium esophagram show for achalasia?

Answer 2

smooth tapering of the lower esophagus leading to the closed LES, resembling a “bird’s beak.”

Question 3

Esophageal manometry establishes the diagnosis of achalasia showing what?

Answer 3

esophageal aperistalsis and insufficient LES relaxation with swallowing

Question 4

In the workup of achalasia, what is the upper endoscopy meant to exclude?

Answer 4

pseudoachalasia arising from a tumor at the gastroesophageal junction

Question 5

The pathological changes seen in achalasia?

Answer 5

myenteric inflammation with injury to and subsequent loss of ganglion cells and fibrosis of myenteric nerves; also a significant reduction in the synthesis of nitric oxide and vasoactive intestinal polypeptide. Possibly 2/2 autoimmune-mediated destruction of inhibitory neurons in response to an unknown insult in genetically susceptible individuals; however, a definite trigger has not been identified.

Question 6

Role of pharmacotherapy in achalasia?

Answer 6

Pharmacotherapy plays a very limited role in the treatment of achalastic patients and should be used in very early stages of the disease, temporarily prior to more definitive treatments, or for patients who fail or are not candidates for other treatment modalities (++++, strong).

Smooth muscle relaxants such as calcium channel blockers and long-acting nitrates are effective in reducing LES pressure and temporally relieving dysphagia but do not improve LES relaxation or improve peristalsis. Since the prolonged esophageal transit and delayed esophageal emptying that characterize achalasia make the absorption kinetics and effectiveness of orally administered medications unpredictable, these agents are used sublingually. These drugs decrease LES pressure by approximately 50% with the long-acting nitrates having a shorter time to maximum effect (3-27 min) and symptom improvement in 53-87% of achalasia patients compared with sublingual nifedipine (30-120 min and 0-75% symptom improvement, respectively).

The main limitations of these agents are their short duration of action, the incomplete symptom relief, and decreased efficacy during long-term use. In addition, side-effects such as peripheral edema, headache, and hypotension occur in up to 30% of patients and further limit their use. The use of the available pharmacologic agents is, therefore, limited to symptomatic relief of patients with very early disease with a nondilated esophagus, or as a temporary measure for patients who are awaiting a more definite treatment option, or are high risk for or refuse more invasive options. In addition, the use of some medications may be useful in the case of severe achalasia-related chest pain.

Question 7

What is the role for botox injections in achalasia?

Answer 7

Botulinum toxin injection can be administered safely, but its effectiveness is limited especially in the long term. It should be reserved for patients who are poor candidates for other more effective treatment options such as surgery or dilation (++++, strong).

Question 8

Who should be offered endoscopic dilation for achalasia?

Answer 8

Among nonoperative treatment techniques endoscopic dilation is the most effective for dysphagia relief in patients with achalasia but is also associated with the highest risk of complications. It should be considered in selected patients who refuse surgery or are poor operative candidates (++++, strong).

POEM is a better alternative if can be done.

Question 9

What is the role for esophageal stents in achalasia?

Answer 9

The use of esophageal stents cannot be recommended for the treatment of achalasia (++, strong).
Their use is clearly associated with high complication rates and even mortalities

Question 10

How do you manage a hiatal hernia during a Heller myotomy for achalasia?

Answer 10

When there is a hiatus hernia, adequate mobilization of the esophagus to restore a normal intra-abdominal length is required, and the crura should be closed behind the esophagus making sure not to restrict the esophagus. Crural closure is typically performed after completion of the myotomy.

Question 11

How do you manage the anterior vagus during a Heller myotomy for achalasia?

Answer 11

The epiphrenic fat pad is excised from the anterior LES starting to the left of the anterior vagus nerve to create adequate room to perform the myotomy on the stomach. The anterior vagus nerve is dissected off the distal esophagus so that the myotomy can be taken high up the esophagus beneath the nerve.

Question 12

How do you manage the anterior vagus during a Heller myotomy for achalasia?

Answer 12

The epiphrenic fat pad is excised from the anterior LES starting to the left of the anterior vagus nerve to create adequate room to perform the myotomy on the stomach. The anterior vagus nerve is dissected off the distal esophagus so that the myotomy can be taken high up the esophagus beneath the nerve.

Question 13

During a Heller myotomy for achalasia, describe the myotomy portion.

Answer 13

The surgeon and assistant each grasp one side of the esophagus and retract in opposite directions to provide better exposure and facilitate the myotomy. The esophageal muscle fibers are split and dissected laterally starting with the longitudinal fibers and entering the circular fibers until a small pocket is made between the circular fibers and the mucosa. The myotomy is continued up the esophagus for at least 4 cm and taken onto the stomach for approximately 2 cm. This dissection is tedious and should be done with care to avoid perforation of the esophageal mucosa. The change from esophageal to gastric muscle fibers can be seen as they change from a horizontal circular orientation to an oblique one and are more adhered to the mucosa. There is also bulging of the mucosa at the LES area. Injection of dilute epinephrine into the muscle before myotomy may be useful, as it minimizes bleeding and allows for better visualization of the mucosa.

Question 14

During a Heller myotomy for achalasia, you are checking the mucosa and notice bubbles when insufflating the NGT/EGD. What do you do?

Answer 14

When bubbles are seen, the area from where they emanate should be oversewn with 4-0 Vicryl, and the subsequent fundoplication should be used to cover the area. In the context of perforation, consideration should also be given to drain placement.

Question 15

What fundoplications are typically used during Heller myotomy? Why are they needed?

Answer 15

They prevent reflux and buttress the mucosa.

The most commonly used options for fundoplication after myotomy include an anterior Dor fundoplication or a posterior Toupet fundoplication. For the Dor fundoplication, the greater curvature of the stomach is pulled over the esophagus making sure it is redundant so as not to restrict the LES and is sutured to the crura where they meet anteriorly. Some surgeons also attach it to the edges of the myotomy to hold it open, and some attach the edges of the myotomy to the crura as well. When a Toupet fundoplication is used, the fundus is pulled behind the esophagus and attached to the left and right cut edges of the myotomy to keep it open.

Question 16

What is the effect of prior endoscopic interventions for achalasia patients undergoing Heller myotomy?

Answer 16

Prior endoscopic treatment for achalasia may be associated with higher myotomy morbidity, but the literature is inconclusive.

Question 17

Compare Heller myotomy to pneumatic dilation, botox, and VATS myotomy for achalasia.

Answer 17

Laparoscopic myotomy with partial fundoplication provides superior and longer-lasting symptom relief with low morbidity for patients with achalasia compared with EGD dilation, botox, and VATS achalasia and should be considered the procedure of choice to treat achalasia. (++++, strong).

Question 18

What is the recommended length of the myotomy in lap Heller for achalasia?

Answer 18

The length of the esophageal myotomy should be at least 4 cm on the esophagus and 1-2 cm on the stomach (+, weak).

Question 19

How do you manage epiphrenic diverticula associated with achalasia?

Answer 19

Epiphrenic diverticula should be treated surgically when symptomatic. Given their frequent association with achalasia, esophageal manometry should be pursued to confirm the diagnosis of achalasia when they are identified. A myotomy at the opposite side of the diverticulum that goes beyond the distal extent of the diverticulum should be performed when achalasia is present. In this situation, concomitant diverticulectomy may be indicated based on the size of the diverticulum. When diverticula are not resected, endoscopic surveillance is advised. The optimal approach for their treatment needs further study, and surgeons should be aware of the relatively high incidence of postoperative leaks (+, weak).

Question 20

What are treatment options after a failed myotomy?

Answer 20

Endoscopic Botulinum toxin treatment can be applied safely and with equal effectiveness before or after myotomy (++, weak), but endoscopic balloon dilation after myotomy is currently considered hazardous by most experts (++, weak). Repeat myotomy may be superior to endoscopic treatment and should be undertaken by experienced surgeons (++, strong). Esophagectomy should be considered in appropriately selected patients after myotomy failure (+, weak).

Question 21

Should Peroral Endoscopic Myotomy (POEM) vs. Heller myotomy (HM) be used for achalasia in adults and children?

Answer 21

The Guideline panel suggests that adult and pediatric patients with type I and II achalasia may be treated with either POEM or laparoscopic Heller myotomy based on surgeon and patient’s shared decision-making (conditional recommendation, very low certainty evidence).

Based on their collective experience, the panel suggests POEM over laparoscopic Heller myotomy for type III adult or pediatric achalasia (expert opinion).

Question 22

Should Peroral Endoscopic Myotomy (POEM) vs. Pneumatic Dilatation (PD) be used for achalasia in adults and children?

Answer 22

The Guideline panel recommends peroral endoscopic myotomy over pneumatic dilatation in patients with achalasia (strong recommendation, moderate certainty evidence).
For the subgroup of patients who are particularly concerned about the continued use of PPI post-operatively , the panel suggests that either POEM or pneumatic dilatation can be used based on joint patient and surgeon decision-making (conditional recommendation, very low certainty evidence).

Question 23

Types of achalasia?

Answer 23

Type I (classical achalasia; no evidence of pressurization) achalasia is associated with absent peristalsis and minimal esophageal body pressurization.
Type II achalasia is associated with intermittent periods of pan-esophageal pressurization related to a compression effect.
Type III achalasia has evidence of abnormal contractility (spastic) with premature or spastic distal esophageal contractions.

Question 24

Surgical (fundoplication) versus medical (PPI) management in adult and pediatric patients with chronic or refractory GERD?

Answer 24

The panel suggests managing adult patients with confirmed chronic or chronic refractory gastroesophageal reflux with surgical fundoplication rather than continued medical treatment (conditional recommendation based on very low certainty in the evidence of effects).

No recommendation was made with regard to pediatric patients.

Question 25

Robotic versus laparoscopic fundoplication in adult and pediatric patients with GERD requiring surgery?

Answer 25

The panel suggests that adult patients with gastroesophageal reflux who are candidates for surgery be treated with either robotic or laparoscopic fundoplication based on surgeon and patient’s shared decision-making (conditional recommendation based on low certainty in the evidence of effects).

• For patients who are particularly concerned about long-term PPI use, robotic surgery may be favored over laparoscopic fundoplication when expertise and resources are available.
• Patients who are more concerned about short-term symptom control and the need for re-operation, laparoscopic surgery may be favored over robotic fundoplication
• The efficacy and safety of both were deemed similar.

Question 26

Complete versus partial fundoplication in adult and pediatric patients with GERD who are candidates for surgery?

Answer 26

The panel suggests that adult patients with GERD who are candidates for surgery be treated with either partial or complete fundoplication based on patients values (conditional recommendations based on low certainty in the evidence of effects).

For patients who value improvement in reflux symptoms over the risk of dysphagia, complete fundoplication may be the preferred option.
For patients who value the minimization of dysphagia highly, partial fundoplication may be offered preferentially.

For pediatric patients without large hiatal hernia, the panel suggests either partial or complete fundoplication approaches guided by shared surgeon-patient decision-making (conditional recommendations based on low certainty in the evidence of effects).

Question 27

Division of short gastric vessels or no division in adult patients with GERD undergoing fundoplication?

Answer 27

For patients who value reflux symptom relief more than the long-term risk of gas bloat or small risk of more procedural complications, division of short gastric vessels may be the preferred option.

For patients who value long-term gas bloat, procedural complications, or both more than the improvement in their reflux symptoms, no division may be offered preferentially.

Question 28

Minimal versus maximal dissection in pediatric patients with GERD undergoing fundoplication?

Answer 28

In the pediatric GERD population without large hiatal hernias undergoing fundoplication, the panel suggests minimal rather than maximal dissection during fundoplication (conditional recommendations based on moderate certainty in the evidence of effects).

Question 29

The current anatomic classification has evolved to include a categorization of hiatal hernias into Types I – IV. What are they?

Answer 29

Type I hernias are sliding hiatal hernias, where the gastroesophageal junction migrates above the diaphragm6. The stomach remains in its usual longitudinal alignment7 and the fundus remains below the gastroesophageal junction.
Type II hernias are pure paraesophageal hernias (PEH); the gastroesophageal junction remains in its normal anatomic position but a portion of the fundus herniates through the diaphragmatic hiatus adjacent to the esophagus.
Type III hernias are a combination of Types I and II, with both the gastroesophageal junction and the fundus herniating through the hiatus. The fundus lies above the gastroesophageal junction.
Type IV hiatal hernias are characterized by the presence of a structure other than stomach, such as the omentum, colon or small bowel within the hernia sac.

Question 30

What is the general principle of workup for hiatal hernia?

Answer 30

Hiatal hernia can be diagnosed by various modalities. Only investigations which will alter the clinical management of the patient should be performed.

Question 31

Why do you order contrast studies in patients with hiatal hernia?

Answer 31

helpful to gauge the size and reducibility of the hiatal hernia and to localize precisely the gastroesophageal junction in relation to the esophageal hiatus, may add to suspicion of existing short esophagus

Question 32

In hiatal hernia patients who are receiving oral contrast, which contrast do you choose?

Answer 32

Usually barium.
Given the increased aspiration risk of patients with paraesophageal hernias presenting with acute gastric outlet obstruction, ionic water-soluble contrast should be generally avoided due to the risk of aspiration pneumonitis.

Question 33

You are doing an EGD in a patient with known paraesophageal hernia who presented to ED with dysphagia and pain. Inability or difficulty reaching the duodenum and see a large hiatal hernia. What is the diagnosis?

Answer 33

This presentation is diagnostic of a volvulized paraesophageal hernia.

Question 34

Repair of a type I hernia in the absence of reflux?

Answer 34

not necessary; this is why pH testing is important in these patients

Question 35

How do you manage symptomatic paraesophageal hernias?

Answer 35

All symptomatic paraesophageal hiatal hernias should be repaired (++++, strong), particularly those with acute obstructive symptoms or which have undergone volvulus.

Question 36

elective repair of completely asymptomatic paraesophageal hernias?

Answer 36

Routine elective repair of completely asymptomatic paraesophageal hernias may not always be indicated. Consideration for surgery should include the patient’s age and co-morbidities. (+++, weak)

Question 37

Manage gastric volvulus herniated in the hiatus

Answer 37

Acute gastric volvulus requires reduction of the stomach with limited resection if needed. (++++, strong)

Question 38

Repair of hiatal hernia during bariatric operations?

Answer 38

During operations for Roux-en-Y gastric bypass, sleeve gastrectomy and the placement of adjustable gastric bands, all detected hiatal hernias should be repaired (+++, weak)

Question 39

In the early postoperative period after hiatal hernia repair, what symptom should be managed?

Answer 39

Postoperative nausea and vomiting should be treated aggressively to minimize poor outcomes (++, strong)

Question 40

Thoracic vs abdominal approach for hiatal hernia?

Open vs MIS repair of hiatal hernia?

Answer 40

Hiatal hernias can effectively be repaired by a transabdominal or transthoracic approach (++++, strong). The morbidity of a laparoscopic approach is markedly less than that of an open approach (++, strong).

Laparoscopic hiatal hernia repair is as effective as open transabdominal repair, with a reduced rate of perioperative morbidity and with shorter hospital stays. It is the preferred approach for the majority of hiatal hernias (++++, strong).

Question 41

How do you manage the hernia sac in hiatal hernias?

Answer 41

During paraesophageal hiatal hernia repair the hernia sac should be dissected away from mediastinal structures (++, strong), and then preferably excised (++, weak).

Question 42

What is the benefit of mesh in repair of hiatal hernias?

Answer 42

The use of mesh for reinforcement of large hiatal hernia repairs leads to decreased short term recurrence rates (+++, strong).

Question 43

Are fundoplications needed during hiatal hernia repairs?

Answer 43

A fundoplication must be performed during repair of a sliding type hiatal hernia to address reflux. A fundoplication is also important during paraesophageal hernia repair. (++, weak).

Question 44

What is the management of the GEJ in hiatal hernia repair?

How do you achieve this goal?

Answer 44

A necessary step of hiatal hernia repair is to return the gastroesophageal junction to an infradiaphragmatic position (+++, strong).
At the completion of the hiatal repair, the intra-abdominal esophagus should measure at least 2 – 3cm in length to decrease the chance of recurrence (++, weak). This length can be achieved by combinations of mediastinal dissection of the esophagus and/ or gastroplasty (++++, strong).

Question 45

What options are there for a hiatal hernia patient that has a difficult dissection and an esophagus on tension?

Answer 45

Gastropexy may safely be used in addition to hiatal repair (++++, strong)

Gastrostomy tube insertion may facilitate postoperative care in selected patients (++, strong)

Hernia reduction with gastropexy alone and no hiatal repair may be a safe alternative in high-risk patients but may be associated with high recurrence rates (++, weak). Formal repair is preferred (++++, strong).

Question 46

Does every hiatal hernia repair require a contrast swallow study postoperatively?

Answer 46

Routine postoperative contrast studies are not necessary in asymptomatic patients (+++, strong).

Question 47

In hiatal hernia postop patients, why should particular attention be paid to adequate caloric and nutritional intake (+, strong).

Answer 47

Early postoperative dysphagia is common.

Early postoperative dysphagia rates are up to 50% and the general recommendation is for slow advancement of diet from liquids to solids. Attention should be paid to adequate caloric and nutritional intake in the postoperative period. Expert opinion suggests that most patients will lose 10-15 pounds (4.5 – 7 kg) with laparoscopic fundoplication and hernia repair followed by a graduated diet from liquids to soft solids. If dysphagia persists or weight loss occurs of 20 or more pounds (9 kg) evaluation and intervention for the dysphagia should be considered.

Question 48

Should pediatric patients with hiatal hernias be repaired if symptomatic?

Answer 48

Symptomatic hiatal hernias in children should be surgically repaired (++, weak)

Question 49

Pediatric hiatal hernia surgery guidelines?

Answer 49

A laparoscopic approach in children is feasible. Age or size of the hernia should not be an upfront contraindication to laparoscopy (++, weak)
Gastroesophageal reflux in pediatric patients with a hiatal hernia should be addressed by a concomitant anti-reflux procedure (++, weak)
The current standard of care in children is either excision of the hernia sac or disconnection of the sac from the crura (+++, weak)
To lower the risk of postoperative paraesophageal hernia after fundoplication in the pediatric population, minimal hiatal dissection should be performed (++, weak)
Plication of the esophagus to the crura may decrease recurrence in children (+, weak)

Question 50

A hiatal hernia repair fails after laparoscopic attempt. Should another MIS attempt be made?

Answer 50

Revisional surgery can safely be undertaken laparoscopically by experienced surgeons (+++, strong)