The 2 primary therapeutic modalities used to treat achalasia are pneumatic dilation (PD) and esophagomyotomy (EM) (Fig. 26-1C). Although there have historically been proponents of each of these procedures, numerous reports have been published that document clinical improvement in adult patients ranging from 65% to 85% with either modality. Moreover, a comparative evaluation is difficult to make in that criteria for classifying results differ from one study to another and follow-up studies are often inadequate. Despite the controversy, in many cases of adult achalasia, PD is frequently performed as the initial treatment modality; it has low morbidity and fair clinical success and involves a short hospital stay with a rapid return to normal activity. There is also general agreement that if 1 or 2 attempts at nonoperative treatment fail to provide satisfactory long-term relief of dysphagia, surgical treatment is then initiated. An esophagectomy should be considered for patients who have a tortuous or severely dilated esophagus and for those in whom symptoms are not relieved by an EM; however, this is rarely seen in children.
While the pediatric experience with achalasia is much more limited, outcomes differ from those in the adult population. Several studies have revealed an age-related correlation with successful treatment. In a study conducted by Azizkhan et al, only 25% of children showed significant improvement with PD alone, and no child under age 9 responded to this treatment. In general, the younger the patient, the less the success achieved by one or multiple dilations. However, a recent study by Lee et al found that all of the children treated with PD as the initial mode of therapy had recurrent symptoms and 93% required additional intervention. This group was compared with 53% of patients receiving EM as initial treatment that developed recurrent symptoms and 40% required additional intervention. The study also showed that 47% of children initially treated PD went on to have EM performed. The authors concluded that patients treated with EM had a statistically significant longer symptom-free duration, fewer hospital admissions with similar complication rates compared with the PD treated patients. Two case series of laparoscopic Heller myotomy and Dor fundoplication in children with achalasia (Patti et al and Mattioli et al) showed greater than 90% relief of dysphagia with low complication rates. In light of multiple studies and the authors' experience, we recommend that EM should be the initial therapy for achalasia in children of all ages. PD should be reserved for children who may not tolerate an operation or for EM patients who developed recurrent dysphagia. Additionally, endoscopic botulinum toxin injection of the LES has been shown to have only transient relief of symptoms, and therefore has essentially no role in treatment of children.
While the modified Heller EM is the standard surgical treatment for achalasia, controversy exists over whether this is best performed through a laparoscopic versus a thoracoscopic approach. While both approaches show similar overall effectiveness and rates of complications, the complication profiles differ somewhat. The initial minimally invasive approach was a left thoracoscopic approach, based on the traditional left thoractomy used to perform the modified Heller EM. The thoracoscopic approach has limited hiatal access with suboptimal visualization of the gastric cardia leading to an elevated rate of incomplete myotomy and recurrent dysphagia. Currently, the laparoscopic approach is preferred because of the lower rate of incomplete myotomy owing to easy gastric access, but a higher incidence of postmyotomy GERD from extension of the myotomy onto the cardia. Consequently, most pediatric surgeons performing the laparoscopic approach also perform a fundoplication as well. Debate continues regarding whether a fundoplication should be performed at the time of the EM, and if so, which type of fundoplication should be performed. Regardless of which approach is used, after the operative procedure, respiratory symptoms diminish and there is a rapid resumption of normal diet, normal growth, and normal activity.
Both PD and EM entail potentially serious risks to patients. The reported incidence of esophageal perforation after forceful dilation is between 1% and 5%, and treatment for perforation generally necessitates surgical intervention. Gastroesophageal reflux (GER) and peptic esophagitis are significant problems with the modified Heller EM, occurring in approximately 10% to 30% of pediatric patients. These disorders are also difficult to treat medically. It appears that the absence or very low incidence of postdilation reflux is related to the fact that it is difficult to disrupt the oblique fibers of the gastroesophageal junction with a dilator.
Regardless of the type of treatment, all patients require close long-term follow-up. Although the initial results may be good or even excellent, symptoms often reappear. Moreover, achalasia is associated with a slightly increased risk of esophageal cancer.
Pneumatic Dilation in Children
The aim of treatment with PD is relief of obstruction by successfully reducing LES tone while maintaining an effective barrier to GER. This procedure is performed under intravenous sedation or general anesthesia. Fluoroscopy facilitates the placement of a pneumatic dilator in the distal esophagus. Air or contrast is forced into the balloon until the pressure reaches the desired amount. Although the technique of PD is similar in all patients, the number of dilations and the maximal diameter of the dilating balloon are individually determined according to clinical, radiologic, and especially manometric criteria. The patient is held NPO for a 24-hour period, with frequent checks of vital signs. A barium swallow is subsequently performed to rule out the possibility of esophageal perforation and evaluate relief of obstruction. If there are no complications, a soft diet is begun the next morning and the patient is discharged.
Laparoscopic Heller Myotomy with Dor Fundoplication
The patient is positioned in the lithotomy position with the table tilted in reverse Trendelenburg. The patient is prepared by emptying the esophagus and stomach with an orogastric tube. Flexible esophagoscopy may be required to confirm that there is no retained food material in the esophagus.
A 5-port technique employing 4- and 5-mm trocars as shown in Fig. 26-2. A pneumoperitoneum is established with a Veress needle, and standard CO2 insufflation. A 5-mm trocar is placed through the umbilicus; a right upper quadrant trocar 4 or 5 mm is placed for the articulated liver retractor secured to a holder; a 4- or 5-mm trocar is placed at the left costal margin as the right hand working port; a 4- or 5-mm trocar in the epigastrium right of midline for the left-hand working port; supraumbilical, left of midline 4- or 5-mm trocar for the laparoscope. A tapered Maloney dilator is passed into the distal esophagus for ease of identification of the esophagus. The liver is retracted to identify the gastroesophageal junction, next the phrenoesophageal ligament is divided mobilizing only the anterior and lateral crural attachments (Fig. 26-3A). Next, the anterior vagus is identified, gently mobilized, and pushed to the right to avoid injury. A longitudinal myotomy is started on the left anterior aspect using hook cautery to coagulate the muscular layer (Fig. 26-3B). A Maryland dissector is used to spread the longitudinal muscle fibers; once the circular fibers are encountered, these are elevated with the hook cautery and divided. Once the submucosal layer is identified, it protrudes through the myotomy. Great care should be taken to avoid bleeding and mucosal perforation. Intraoperative esophagoscopy can be helpful to reduce the risk of mucosal perforation, especially in patients who have had previous esophageal dilatations. The edges of the myotomy are grasped with the each hand and gently pulled apart to widen the myotomy and facilitate submucosal herniation (Fig. 26-3C). The myotomy is extended proximally well onto the dilated esophagus and distally onto the cardia to the transverse esophageal vessels. The esophageal muscle is separated from the mucosa for at least one third of the circumference of the esophagus. To check for mucosal perforation, air is insufflated into the esophagus using a nasogastric tube. If a mucosal perforation occurs, this can be closed with fine suture and covered with an anterior fundoplication (Dor or Thal).
The location of the 5 trocars in order of placement. A 5-mm port is placed in the umbilicus using an open technique (1). A second port is placed near the liver edge and in the right midclavicular line for the liver retractor (2). The third trocar is placed at the left costal margin in the anterior axillary line (3). The fourth trocar is placed in the supraumbilical region, left of midline (4); and the fifth trocar is placed in the epigastrium to the right of midline (5). Ports 3 and 5 (left and right hands, respectively) should be placed approximately 60° apart in relation to the hiatus, as this provides an optimal working angle.
A. The phrenoesophageal ligament is divided and anterior and lateral attachments mobilized. B. A longitudinal mytomy is started on the left anterior aspect of the distal esophagus using an L-hook cautery. C. The edges of the myotomy are grasped and gently pulled apart to widen the myotomy.
Although controversial, an antireflux procedure is often added to minimize the risk of postoperative reflux. If an antireflux procedure is added, the general consensus is that a partial wrap (Dor or Toupet procedure) should be performed. We routinely perform a 180° anterior Dor fundoplication to cover the exposed submucosa and to mitigate risk of GERD. The fundus is sutured to the left and right edges of the myotomy with nonabsorbable 3-0 sutures (Fig. 26-4A and B). This can be accomplished by either intracorporeal or extracorporeal knot tying.
A. Completed myotomy extending down just beyond the gastroesophageal junction. The numbers show suture placement for 180° anterior Dor fundoplication. B. Two nonabsorbable sutures are placed to secure the anterior stomach to each edge of the myotomy.
Most patients have the nasogastric tube removed prior to emergence from anesthesia and are kept NPO on the day of the operation. A liquid diet is started the day after surgery, and, if tolerated, can be advanced to a soft diet for 2 weeks. If there is any concern regarding mucosal perforation, an esophagram with water-soluble contrast should be performed prior to starting a diet. Pain control with acetaminophen and low dose morphine sulfate is usually adequate. Most patients are discharged on the first or second postoperative day.
Thoracoscopic Heller Myotomy
The preoperative preparation is essentially identical to that described above for the laparoscopic procedure. During this procedure, the anesthetized patient is secured in the Trendelenburg position with the left side up. Four ports are needed (Fig. 26-5). One 5-mm port for the thoracoscope is placed in the superior portion of the midclavicular line; two 4- or 5-mm ports for the dissector and grasper are placed 1 to 2 interspaces below the thoracoscope anteriorly and posteriorly to the midclavicular line; and one 5-mm port for the cautery and scissors used to divide the esophageal muscle and free it from the underlying mucosa is placed close to the diaphragm. A tapered Maloney dilator is passed into the distal esophagus for ease of identification and mobilization from the mediastinum. Some surgeons have found that a flexible endoscope placed transorally into the distal esophagus permits easy identification with transillumination of the esophagus.
The location of the 4 trocar sites on the left lateral chest wall for a thoracoscopic EM. The camera is usually held at the most cephalad site for optimal viewing of the procedure, which is performed near the diaphragm.
The pleura is incised overlying the esophagus, and dissection is carried both superiorly and inferiorly to free it from the posterior mediastinum. The esophagus must be mobilized superiorly to the level of the inferior pulmonary vein and inferiorly to the hiatus of the diaphragm; this mobilization step is best accomplished using blunt dissection. Umbilical tape is then wrapped around the esophagus for retraction. The esophagus is vertically incised using the laparoscopic shears, and the esophageal muscle is dissected from the underlying mucosa on the distal third of the esophagus inferiorly to the cardioesophageal junction (Fig. 26-6A). Graspers are used to hold the cut muscle edges, and the mucosa is pushed away from the muscle using a Kittner dissector (Fig. 26-6B). The muscle should be stripped away from the mucosa approximately one third of the circumference of the esophagus, with half of this circumference on either side of the incision. Esophagoscopy is also helpful at this stage to gauge the distal extent of the obstruction and when the EM has been anatomically successful.
A. After the parietal pleura has been opened, the myotomy is begun in the distal third of the esophagus with an endoscopic J hook connected to electrocautery. A large dilator or esophagoscope is prepositioned in the distal esophagus to facilitate the myotomy. B. The muscle is elevated on either side of the myotomy with graspers to anchor the edge of the muscle, while the endoscopic scissors are used to dissect the muscle from the underlying mucosa.
The most significant risk at the time of myotomy is perforation through the mucosa. Repairing the small hole with fine absorbable sutures and buttressing the repair site with a vascularized tissue pedicle of pleura is usually all that is required. A thoracostomy tube is placed and positioned near the myotomy at closing, and usually removed on postoperative day 1 or 2 once a diet is resumed. The postoperative management is the same as that for the laparoscopic technique.
The minimally invasive procedures offer the advantages of smaller incisions, less postoperative pain, and a shorter hospital stay than the open approaches. The decision regarding appropriateness of which approach to choose should, of course, be based on the individual patient and the surgeon's skill set.