Dr. Pranoti Arjunrao Pol    
M.S. General Surgeon, World Laparoscopy Hospital 2019 Batch, India

Achalasia Cardia (Cardiospasm)

• Achalasia is a rare primary motility disorder of the esophagus that affects one person in 100,000 per year and is characterized by the absence of esophageal peristalsis and incomplete relaxation of a frequently hypertensive lower esophageal sphincter (LES) in response to swallowing.
• Achalasia cardia is a precancerous condition 7 times the chances of Squamous cell carcinoma (8% after 15 years).
• Etiology: Autoimmune-mediated destruction of inhibitory neurons in response to an unknown insult in genetically susceptible individuals. Stress, Vit B1 deficiency, Chaga’s disease caused by Trypanosoma cruzi common in South America called Sleeping sickness. Diffuse oesophageal spasm Corkscrew esophagus.

        

• Pathological changes in Achalasia: Auerbach’s Myenteric plexus inflammation with injury and loss of ganglion cells and fibrosis of myenteric nerves. There is a significant reduction in the synthesis of nitric oxide and vasoactive intestinal polypeptide along the entire length of esophagus more in LOS. There is a pencil-shaped narrowing of the cardia(O-G junction) with enormous dilatation of proximal esophagus which contains foul-smelling fluid and is more prone n for aspiration pneumonia.

 

• Symptom

Dysphasia with solids and liquids                   
Regurgitation of undigested food                                   TRIAD
 Weight loss.
Heartburn.
Chest pain,  nocturnal cough
 
 
STAGING / GRADING :

1. Proximal dilatation , 4cm,
2. Dilatation between 4-6cm,
3. Dilatation .6cm,
4. Sigmoid dilatation.

 
 
CLINICAL SCORING OF ACHALASIA CARDIA:

1. No weight loss or dysphagia or retrosternal pain or regurgitation.
2. Weight loss, 5kg occasional dysphagia retrosternal pain, regurgitation.
3. Weight loss5-10kg daily dysphagia retrosternal pain regurgitation.
4. Weight loss.10kg dysphagia and regurgitation during each meal retrosternal pain several times a day.

 
CLINICAL PRESENTATION AND DIAGNOSIS:

• Achalasia cardia is more common in females age group: 20 to 40 years.
• Barium esophagogram: There is a smooth tapering of the lower esophagus leading to the closed LES, resembling a “bird’s beak.”  Dilatation of proximal esophagus- cucumber esophagus,  Absence of fundic gas bubble, sigmoid esophagus or megaoesophagus.
• Esophageal manometry establishes the diagnosis showing esophageal peristalsis and insufficient LES relaxation with swallowing. All patients should undergo an upper endoscopy to exclude pseudoachalasia arising from a tumor at the gastroesophageal junction.

 Chest X-ray:   patches of pneumonia. The double meditational strip of the dilated esophagus and on lateral view air-fluid level in the posterior mediastinum.

• Oesophagoscopy: confirm the diagnosis, rule out carcinoma. On oesophagoscopy there will be closed LES rosette-like) with atonic, dilated proximal esophagus. Oesophagoscopy with biopsy is preferred.

 
Treatment:

Conservative &  Surgical.

Conservative management: All available treatment options are directed at the palliation of symptoms only.
 1. Pharmacotherapy Smooth muscle relaxants - calcium channel blockers and long-acting nitrates sublingually. Botulinum toxin injections- associated with a high recurrence rate.
2. Dilatation: Plummers pneumotic dilatation using  30-40 mm diameters, but the risk of perforation 5 %, Negus hydrostatic balloon dilatation- 30mm diameter balloon is inflated for 3 minutes.
3.Esophageal stents,
4. POEM Per Oral Endoscopic Myotomy.
5. Surgical Treatment of Achalasia: The goal of surgery is to alleviate the distal esophageal obstruction by a division of the circular muscle fibers comprising the LES.

Per Oral Endoscopic Myotomy
 

    
APPROACHES:   Five different technical approaches:

• Open transabdominal.
• Open transthoracic:  Resection of OG junction / transhiatal total oesophagectomy in severe cases- megaoesophagus/ metaplasia.
• Thoracoscopic.
•  Laparoscopic Heller Myotomy and Dor Fundoplication.
• Robotic approach.

 
 
TASK ANALYSIS:

1. Procedural steps


 General anesthesia followed by Patient positioning,
 Clean  and sterile draping
 Pneumoperitoneum is created with  CO ₂  Insufflation  via veress needle at 5cm superior to the umbilicus 
 Insertion of ports
 Liver retraction by Nathanson's retractor and identification of pars flacida
 Exposing the right and left crura of the diaphragm
 Mobilization of esophagus intra-abdominally
 Approximating the gap between both crura through sutures
 Mobilization and preparation of stomach for wrapping by dividing short gastric  vessels
 Plication of the fundus of the stomach around the mobilized esophagus
 Haemostasis achieved 
 Removal of ports under the vision 
 Skin closure.

Anesthesia:   General anesthesia

1. Patient Position:

The patient is placed in supine atop a beanbag to create a saddle under the perineum to avoid sliding when in steep reverse Trendelenburg. Pneumatic compression stockings are used for prophylaxis of deep vein thrombosis.
General endotracheal anesthesia is given and an oro-gastric tube is placed to keep the stomach decompressed and tube is removed before starting the myotomy.
 
The legs are placed in stirrups with knees flexed 20–30°.
The surgeon stands between the patient’s legs, with an assistant on the patient’s left and one on the patient’s right side

Fig: Position of the patient and the surgical team in the operating room.
 
Port placement according to SAGES guideline
 
2. Quadro manometric parameters:

1) Preset pressure:  15 mm of Hg.
2) Cautery: monopolar and patient return plate, Harmonic device.
3) A light source and coaxial alignment with the surgeon  are checked.
4) Co2 cylinder checked for availability of sufficient Co2 for insufflation.
5) Working status of all the lap instruments along with insulation is checked properly.

3. Operative steps:
    
Access and Insufflation

1. Veress needle is checked for patency and spring action.
2. A small stab incision using number 11 blade at  5cm superiorly to  the umbilicus is made
3. Lift the lower abdominal wall between your palm and four fingers 
4. Measure the thickness of the abdominal wall 
5. Hold the Veress needle like a dart exposing only 4cm + the measured abdominal wall thickness, and direct it at 45 degrees to the patient abdominal axis yet 90 degrees to the abdominal wall aiming towards the anus 
6.  Feel two give away clicks.  
7. Veress needle correct placement inside the abdominal cavity using the following tests :
i) Irrigation and suction test: free flow of 5cc saline with no resistance, then on suction, only air must come back 
ii) Hanging drop test : put one drop of saline at the opening of the veress needle then lift the abdominal wall, the drop must go through the needle 
iii) Connect to the insufflator : intra-abdominal pressure must be zero
8. Start insufflation with Co2 gas at flow rate of 1L/min and quadromanometric readings on the insufflator  is checked to make sure that the rise in the intraabdominal pressure is proportional to the total amount of gas pumped 
i) When reaching 12 mmHg pressure  incision is enlarge to 1.1 cm (smiling incision) then insert a size 10mm port at 90 degrees using screw movement to the right and left 
9. Remove  the trocar and connect the gas tube to the port
10. Connect the 30° telescope to the camera head and white balance setting is achieved.
11. Insert the telescope into the abdominal cavity above the umbilicusdiagnostic laparoscopy is performed to look for any intrabdominal pathology.
Liver retraction: Through the epigastric 5mm port a Nathanson’s self-retaining retractor is  introduced and liver is lifted and retracted exposing the gastro-esophageal junction and pars flacida ligament
 
4. Port placement: All ports are made according to “ baseball diamond concept”
Five 10 mm trocars are used for the operation
A)  First trocar (A) in the midline, 14 cm distal to the xiphoid process, and it is used for the 30° scope. This trocar can also be placed slightly to the left of the midline. This port must be placed with caution since the insertion site is just above the aorta. Initially inflate the abdomen to a pressure of 18 mm Hg to increase the distance between the abdominal wall and the aorta. Once this port is placed, the intraperitoneal pressure is reduced to 15 mm Hg and the other trocars are placed under direct vision.
B) Second trocar (B) in the right midclavicular line at the same level of the previous trocar, and from where the insertion of Nathanson Liver retractor is done to lift the left lateral segment of the liver to expose the oesophagogastric junction. Hold the retractor in place by a self-retaining system fixed to the operating table.


Port placement

C).  Third trocar (C) in the left midclavicular line at the same level as the other 2 trocars and the Babcock clamp is inserted or it is used for instruments used to divide the short gastric vessels.
D). Fourth (D) and Fifth (E) trocars under the right and left costal margins are placed so that their axis forms an angle of about 120° with the camera. These ports are used for the dissecting and suturing instruments

5. Dissection:

Identification of Pars flacida: 
After liver is retracted using Nathanson Karl Storz self-retaining retractor, pars flacida is identified as it is the thinnest (transparent) layer of peritoneum devoid of any fat. 
Dissection is started by dividing this layer and exposing the caudate lobe of the liver. 
Just medial to caudate lobe lies the inferior vena cava, care is taken not to do overshooting of the instruments. 
 
 
Division of the Gastro hepatic Ligament; Identification of the Right Crus of the Diaphragm; and the Posterior Vagus Nerve:  
Dissection starts above the caudate lobe of the liver & continued toward the diaphragm until the right crus is identified. Then, by blunt dissection, the crus is separate from the right side of the esophagus, and the posterior vagus nerve identify. Since monopolar current tends to spread laterally and the posterior vagus nerve may sustain damage even without direct contact, bipolar instrument is safe- Harmonic.
                                   
Division of Gastrohepatic ligament                                          Retro oesophageal window
 
Division of Peritoneum and Phreno-esophageal membrane above the Oesophagus:  Identification of the Left Crus of the Diaphragm and Anterior Vagus Nerve

Transaction of  the peritoneum is done and phrenoesophageal membrane above the esophagus. Anterior Vagus Nerve is embedded into the oesophageal wall. By blunt dissection separation of  the left crus from the esophagus is done . Continue the dissection into the mediastinum, lateral, and anterior to the esophagus, to expose 6–7 cm of the esophagus but not deep into mediastinum as it may cause pneumothorax. Dissection of Left Crus
 
Division of the Short Gastric Vessels: Division of short brevis artery by putting traction on stomach anteromedially.
 
 Dissection of brevis vessel.

 
MYOTOMY:  
                        
Fig: The myotomy includes cutting of the muscular layer of the oesophagus & the upper part of the stomach.
The esophagus is mobilized several centimeters into the mediastinum until there is enough room for the Myotomy. The fat pad is removed to expose the gastroesophageal junction in order to expose the right side of the esophagus. Use a Babcock clamp to pull the stomach downward and to the left. The surgeon and assistant each grasp one side of the esophagus and retract in opposite directions to provide better exposure for myotomy. The esophageal muscle fibers are split and dissected laterally and the longitudinal fibers and the circular fibers are cut 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. Care should be taken to avoid perforation of the esophageal mucosa.
The completeness of the myotomy is checked at the end of the procedure which is done with an endoscope where the lower esophagus is inspected or with the use of intraoperative manometry.
In patients who have had previous treatment with botulinum toxin injection, fibrosis can occur at the level of the gastroesophageal junction leading to loss of the normal anatomic planes. In these cases, there is an increased risk of mucosal perforation which is close with fine absorbable material (5–0), and test the repair with saline or methylene blue. Bleeding may also occur during the myotomy,  from submucosal veins at the level of the gastroesophageal junction applying pressure with a sponge helps to control it.
 

Extension of Myotomy.

Dor Fundoplication: GERD  occurs in about 50% of patients if a myotomy alone is performed. A 360° fundoplication, is avoided due to postoperative dysphagia.
A partial fundoplication anterior Dor fundoplication is the procedure of choice. The Dor fundoplication is constructed using two rows of sutures. The first row of sutures is on the left side of the esophagus and has three stitches. The uppermost stitch incorporates the gastric fundus, the muscle layers of the left side of the esophagus, and the left pillar of the crus. The second and the third stitch incorporate the muscle layers of the left side of the esophagus and the gastric wall only. The fundus is then folded over the exposed mucosa so that the greater curvature of the stomach lies next to the right pillar of the crus.
 

Laparoscopic view: DOR FUNDOPLICATION
 
 
 
The second row of sutures also consists of three stitches. The uppermost stitch incorporates the gastric fundus, the right side of the cut edge of the muscle layers, and the right pillar of the crus. The second and third stitches are placed between the greater curvature of the stomach and the right pillar of the crus  Finally, two additional stitches are placed between the anterior rim of the oesophageal hiatus and the superior aspect of the fundoplication (without incorporating the esophageal wall) to decrease the tension on the right row of sutures.
 
COMPLICATIONS: 

• Oesophageal leak,
• Pneumothorax,
• Dysphagia,
• Abnormal gastroesophageal reflux :  in 6–33%

POSTOPERATIVE CARE: 

Postoperative day 1  Soft meal diet is advised and instructed to avoid meat and bread for 2 weeks.  Patients are discharged within 23 h and 90% of patients are discharged within 48 h.
Most patients are able to resume regular activities in 7–14 day.
 
REFERENCES:
1.Burpee SE, Mamazza J, Schlachta CM, et al. Objective analysis of gastroesophageal reflux after laparoscopic Heller myotomy: an anti-reflux procedure is required. Surg Endosc. 2005;19:9–14.
2. Chen Z, Bessell JR, Chew A, Watson DI. Laparoscopic cardiomyotomy for achalasia: clinical outcomes beyond 5 years. J Gastrointest Surg. 2010;14:594–600.
3. Patti MG, Fisichella PM. Laparoscopic Heller myotomy and Dor fundoplication for esophageal achalasia. How I do it. J Gastrointest Surg. 2008;12:764–6.
4. Portale G, Costantini M, Rizzetto C, et al. Long-term outcome of laparoscopic Heller-Dor surgery for esophageal achalasia. Possible detrimental role of previous endoscopic treatment. J Gastrointest Surg. 2005;9:1332–9.
 
.