Achalasia

Achalasia is a motor disorder usually affecting the entire length of the esophagus. Although the cause of achalasia is unclear, approximately 10% of cases occur in children. Even though this disease is relatively uncommon in children, it seems to be increasing in frequency. Two thirds of affected children are boys. Whether the condition is being diagnosed at an earlier age or there is a true increase in its incidence is unclear. This disorder is caused by failure of the LES to relax and failure of the esophagus to advance food into the stomach.

Figure 1: Chest x-ray shows an air-fluid level in the middle of the chest (arrow) in a patient with achalasia.

Most children with achalasia present with symptoms of several months’ to several years’ duration consisting typically of vomiting of undigested food, severe bad breath, and significant dysphagia (difficulty swallowing). These children also may have prolonged coughing spells after meals. Weight loss is common because the nutritional status is affected. Children often complain of pain behind their breastbone or burning secondary to esophageal irritation from food that is stuck. Usually there is a delay in establishing a diagnosis. A chest x-ray may show a widening in the middle of the chest and a fluid level in the esophagus when the child is sitting or standing. (Figure 1A).

The diagnosis usually is suspected after an upper GI series shows narrowing at the LES with a large esophagus above it (bird beak deformity) (Figure 1B)

In addition, there is no relaxation of the LES on UGI study. The diagnosis is confirmed by manometric (pressure) evaluation revealing an elevated resting pressure in the esophagus without evidence of relaxation of the LES (Figure 2). There also is absence of coordinated contractions in the esophagus.

Figure 2: Manometric study shows a markedly elevated resting pressure in the esophagus without evidence of relaxation at the LES in a patient with achalasia.

The ideal treatment of patients with achalasia is controversial. In 1971, Van Trapp introduced the concept of balloon dilation of the narrowed LES for adult patients. Balloon dilation is used in children but seems to be less successful in relieving symptoms than in adults. The incidence of post-dilation GER may be higher in children, and the procedure is technically more difficult in younger patients. Other approaches for relief of symptoms include injection of botulinum toxin into the LES. Although this technique has gained popularity in more recent years, its effectiveness usually lasts only a few months. Medical management is also not successful. Because non-operative therapy is rarely successful, especially for a prolonged period, most children with achalasia require an operation for long-term relief.

In 1913, Heller described an operation for relief of symptoms of achalasia. With advancements in minimally invasive surgery, this procedure has become an attractive approach for surgical management. Although esophagomyotomy (Heller’s procedure) has been reported using either the laparoscopic (through the abdomen) or the thorascopic (through the chest) route, the laparoscopic route is now favored. There are many reports in adults describing the effectiveness of a laparoscopic Heller myotomy for relief of symptoms resulting from achalasia, but the experience in the pediatric literature is not nearly as large.

The most controversial aspect of performing a laparoscopic esophagomyotomy in a child is whether or not an anti-reflux procedure should be added. This controversy also exists in the adult literature. Most adult surgeons favor the performance of an anti-reflux operation because it is relatively easy to accomplish and prevents the need for a second procedure if GER develops. In most children a fundoplication is not performed.

Figure 3: Intraoperative photograph depicts the completed esophagomyotomy to a length of 8cm. The muscle fibers are widely separated (open arrows), and the anterior vagus nerve crosses the submucosa (solid arrow).

Esophagomyotomy
For the laparoscopic esophageal myotomy, the placement of the incisions is similar to that used for fundoplication (Figure 1). Identical instruments are employed for a laparoscopic esophageal myotomy as for a laparoscopic fundoplication.

With an operative plan of myotomy and anterior fundoplication, the initial step is to divide the blood vessels connecting the stomach and spleen in order to free the upper stomach. Next, the anesthesiologist is asked to introduce an appropriate-sized dilator through the mouth and into the stomach to open up the lower esophagus. Some surgeons also use esophagoscopy to do this.

The first step in performing the myotomy is careful separation of the esophageal muscle fibers 1 to 2 cm above the LES (Figure 8). The muscle fibers are gently hooked and pulled away from the inner esophageal linings (Figure 12). The myotomy usually is extended upward to above the opening in the diaphragm where the esophagus enters the abdomen. With a myotomy to this level, the length is 6 to 8 cm (Figure 13). If a longer esophageal myotomy is desired, pulling the esophagus downward may help. A Penrose drain or umbilical tape can be placed around the esophagus and the esophagus retracted downward for this purpose. The myotomy is also continued for 1-2 cm onto the stomach. If there is concern about the completeness of the myotomy, the dilator can be removed and direct endoscopic visualization performed to confirm that an adequate myotomy has been accomplished. If required, the esophagomyotomy can be extended further onto the stomach. If performed, a fundoplication is created.

The postoperative management varies from surgeon to surgeon. Some proceed with a liquid diet the evening of the operation with discharge the following morning. Others prefer to decompress the stomach overnight with a tube placed through the nose and into the stomach. Some may prefer to perform an UGI contrast study the following morning to ensure there is no evidence of perforation (a hole) followed by a liquid diet if no perforation is noted on the contrast study. A mechanical soft diet usually is employed for several days, after which a regular diet may be initiated. There is usually no need for additional x-rays unless symptoms recur.

Diffuse Esophageal Spasm
Diffuse esophageal spasm is even less common than achalasia. Symptoms of this disorder are similar to symptoms seen with achalasia. Patients present with a long-standing history of swallowing problems, regurgitation of undigested food, bad breath, and weight loss. Children also may complain of chest pain under the breast bone of a pressure or burning nature. Lung symptoms, such as pneumonia or asthma, also may be present. Cold beverages may make these symptoms worse. The diagnosis usually is made with pressure measurements (manometry) and an upper GI study. On UGI study, areas of contraction of the esophagus are seen, giving the appearance of a distorted corkscrew or rosary beads. On manometry, there are repeated prolonged high pressure areas in the lower one half to two thirds of the esophagus with relaxation of the LES.

Little is written on the management of children with this disorder. In adults, there have been reports of successful treatment with injection of botulinum toxin in the LES, psychosomatic treatment with antidepressants, and ingestion of peppermint oil along with nitroglycerin. Balloon dilation and surgical myotomy also have been employed with intermittent success. Neither of these invasive approaches is totally satisfactory.

SUGGESTED READINGS

Hurwitz M, Bahar RJ, Ament ME, et al: Evaluation of the use of botulinum toxin in children with achalasia. J Pediatr Gastroenterol Nutr 30:509-514, 2000.
Botulinum toxin was used in 23 children. Because many of the children eventually will require an additional procedure (dilation or esophagomyotomy), the authors recommend this technique only in children who are poor candidates for either dilation or surgery.

Patti MG, Albanese CT, Holcomb GW III, et al: Laparo- scopic Heller myotomy and Dor fundoplication for esophageal achalasia in children. J Pediatr Surg 36:1248-1251, 2001.
A small series of children underwent laparoscopic esophagomyotomy and anterior fundoplication for achalasia. All patients were discharged within 2 days of the procedure.

Patti MG, Pellegrini CA, Horgan S, et al: Minimally invasive surgery for achalasia: An 8- year experience with 168 patients. Ann Surg 230:587-593, 1999.
In this large series of 168 adults, the authors describe the evolution in their surgical technique and their clinical results.

Storr M, Allescher HD, Classen M: Current concepts on pathophysiology, diagnosis and treatment of diffuse esophageal spasm. Drugs 61:579-591, 2001.
This article describes current concepts regarding the physiology, diagnosis, and treatment of patients with diffuse esophageal spasm.

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Achalasia