Achalasia, an uncommon condition in children, is characterized by an increased basal resting pressure and failure of complete relaxation of the lower esophageal sphincter, combined with an absence of normal esophageal peristalsis. The incidence of achalasia in children is 0.02 to 0.31 per 100,000 children per year (1). The estimated manifestation of symptoms before age 15 is less than 5%. These symptoms may include dysphagia, regurgitation, cough, and chest pain with subsequent failure to thrive (2).
Treatment for achalasia includes medical management with nifedipine (3–6) or nitroglycerine (7), endoscopic management with pneumatic or bougie dilators (8–15) and botulinum toxin injection (16–20), and surgical intervention with esophagomyotomy (21–27). The optimal treatment for achalasia remains unclear. Few articles compare different treatment modalities for achalasia.
For many years, the use of open esophagomyotomy (OE) was the only surgical option for adults and children with achalasia (28–33). More recently, however, the development of minimally invasive esophagomyotomy (MIE), with or without fundoplication, has become a popular management strategy for adults (34–45). The minimally invasive surgical approaches include transabdominal laparoscopic esophagomyotomy (TLE) and thoracoscopic esophagomyotomy (TE).
In the current study, we used multicenter data collection to examine the safety and efficacy of MIE with or without fundoplication in the largest known group of children who have undergone one of these procedures. We then compared the postoperative progress in patients based on the type of MIE performed.
MATERIALS AND METHODS
Via the Internet, patients were identified from an approximately 800-member listserve of pediatric gastroenterologists. Patients were included if they underwent MIE after a diagnosis of achalasia was established before 18 years of age. Participating physicians were given a patient data form for each patient via electronic mail or facsimile. Patient charts were evaluated, and pre-and postoperative data were collected. Patients were diagnosed using established criteria from upper gastrointestinal contrast radiography, esophageal manometry, or both (46,47).
Each form summarized patient information, including age, preoperative and postoperative symptoms, presurgical treatments, type of MIE, addition of fundoplication and type of fundoplication, length of postoperative stay, number of postoperative days to refeeding, and length of follow-up. Preoperative and postoperative symptoms included dysphagia, regurgitation, “heartburn,” and chest pain. Symptoms were subjectively rated from 0 to 3 (0 = none, 1 = mild, 2 = moderate, 3 = severe).
Transabdominal laparoscopic esophagomyotomy was performed using the same principles as an open esophagomyotomy, but laparoscopically. Briefly, a pneumoperitoneum was obtained through the umbilicus using a Veress needle (Storz, Culver City, CA) or open cutdown. Trocar positions were the same as those used for a laparoscopic fundoplication. Spreading the longitudinal muscle and cutting the circular muscle of the esophagus with harmonic scalpel or hook achieved separation of the submucosal plane from the muscularis. A blunt dissector was used to separate the submuscular plane of the myotomy. A harmonic hook was then used to complete the myotomy with precision. The myotomy was completed while protecting the anterior vagus nerve. The myotomy was made from the beginning of the esophageal dilatation through the gastroesophageal junction and continued to 1 cm below the gastroesophageal junction.
Thoracoscopic esophagomyotomy was performed after the patient was intubated selectively into the right mainstem bronchus with a double lumen endotracheal tube and the left lung was collapsed. The esophagus was brought up out of the aortic groove. After the parietal pleura over the esophagus was opened, spreading the longitudinal muscle and cutting the circular muscle was completed with scissors or harmonic scalpel, as with the laparoscopic approach. A harmonic hook was then used to complete the myotomy. A longitudinal esophageal myotomy was performed 6 cm cephalad to the gastroesophageal junction and carried inferiorly until the sling arrangement of gastric smooth muscle. The approach to both surgical procedures has been detailed by Hunter and Richardson (48).
Several types of fundoplications were performed. A Nissen fundoplication (49) was performed with placement of a bougie within the esophagus and pulling the fundus posteriorly behind the esophagus, so that the two edges of the stomach circumferentially encircle the esophagus just above the gastroesophageal junction. The stomach is then sutured laterally to the esophagus above the gastroesophageal junction. This method completed a 360° wrap. A Dor fundoplication (50) was constructed with the use of the anterior wall of the gastric fundus that was secured to the upper border and to the edges of the myotomy.
The most proximal stitches were fixed to the border of the anterior diaphragm. This was a 180° anterior wrap. Grasping and retracting the fundus medially behind the esophagus performed a Toupet fundoplication (49). Three sutures on each side of the esophagus, with full-thickness bites of the stomach and partial thickness bites of the esophagus, were placed. Additional sutures were placed between the posterior fundus and the crura. This maneuver completed a 270° posterior wrap. A Belsey fundoplication (51), using a thoracoscopic approach, attached the gastroesophageal junction inside a collar of the stomach and then attached both structures to the anterior arch of the hiatus.
Results from the patients were expressed as median or mean ± standard deviation. Comparison of mean duration of hospital stay and mean time to resumption of soft feedings were performed using the Student t test. A comparison of the mean severity score with regard to dysphagia and regurgitation was performed using the paired t test.
Twenty-two patients identified from the listserve of pediatric gastroenterologists met inclusion criteria. Exclusion occurred once because of inability of researchers to rate symptoms subjectively in a 5-month-old patient. Fifteen patients were diagnosed with both upper gastrointestinal contrast radiography and esophageal manometry. Six were diagnosed with upper gastrointestinal contrast radiography alone, and one was diagnosed with manometry alone. Ten females and 12 males underwent MIE between 1995 and 2000. The median age was 11.3 ± 3.4 years (range, 5.3–18.5 years). Transabdominal laparoscopic esophagomyotomy was performed in 18 patients, with conversion to OE in 2 patients because of intraoperative perforation. A fundoplication was completed in all patients who underwent TLE. Of the 16 patients who underwent TLE, type of fundoplication was as follows: eight had Dor fundoplications, seven had Toupet fundoplications, and one had a Nissen fundoplication. Of the patients who underwent conversion from TLE to OE, one underwent a Nissen fundoplication and one underwent a Toupet fundoplication. Thoracoscopic esophagomyotomy was performed in four patients. Only one of the four TE patients underwent a Belsey fundoplication. Preoperative interventions included 17 pneumatic dilatations in 12 patients, 9 botulinum toxin injections in 7 patients, and 2 nifedipine treatments in 2 patients. Mean follow-up time for all patients was 17.2 ± 16.1 months (range, 1–54 months). Repeat balloon dilatation was required in one patient who underwent OE and in one patient who underwent TE. Three patients experienced heartburn after surgery and one patient experienced chest pain after surgery.
Mean duration of hospitalization (days + standard error of mean) was less for TLE than for TLE converted OE (2.7 + 0.3 days vs. 9.0 + 3.0 days;P < 0.05) or for TE (4.8 + 1.7;P = not significant;Fig. 1). Mean time to resumption of soft feedings (days + standard error or mean) occurred sooner after TLE than after OE (2.0 + 0.2 days vs. 5.5 + 0.5 days;P < 0.001) or after TE (4.0 + 1.3 days;P = not significant;Fig. 2). One patient who underwent TE and one patient who underwent TLE converted to OE required repeat balloon dilatation 4 and 2 months after surgery, respectively. Patients experienced significant pre-to postoperative improvement in mean severity score from 0 (no symptoms) to 3 (severe) with regard to dysphagia (2.6 vs. 0.4;P < 0.001) and regurgitation (1.7 vs. 0.2;P < 0.001).
There is a dearth of literature on MIE in children with achalasia, primarily because of the rare nature of the condition. Our study, consisting of 22 patients, is the largest to date. With our multicenter data pool, we were able to determine that these children experienced a statistically significant improvement in dysphagia and regurgitation after surgery. Although variability of experience and different types of fundoplications among the different surgeons may confound our findings, improvement of symptoms occurred in all patients. A limitation of this study is the lack of comparison of our data with randomization to other methods of treatment. Given the available literature on achalasia in children, it is difficult to compare statistically the long-term results of dilatation, botulinum toxin treatment, or exclusive open myotomy studies with those of our data. However, comparison of other invasive treatment methods for achalasia in children with data from previous studies may assist in choosing an appropriate method for the management of these patients.
Six studies examining the clinical outcome after esophageal dilatation (8–13) were published between 1980 and 1999 (Table 1). Each study described at least eight children with achalasia. The percentage of patients with “good” results improves with each subsequent study, with the exception of the study by Boyle et al. (9), in which the percentages of patients with good outcome were greater than those in the subsequent studies. Good results are defined as minimal residual or complete resolution of symptoms after dilatation. Other than the study by Boyle et al., the improvement in patients with good outcome may signify an improved technique over time. Patients who required surgery in these studies had a mean of 2.8 sessions (range, 2–3 sessions) of dilatation before surgery. The patients who did not go on to surgery had a mean of 1.7 sessions (range, 1–2.4 sessions) of dilatation at the end of each study. In general, patients who underwent surgery did not experience improvement of symptoms even with a greater number of dilatation sessions. We recommend a study of long-term experience of the patients who do not require further treatment beyond these sessions. Such patients may require further management with repeat dilatation or eventually would have undergone surgery with longer follow-up. Most of these studies do not delineate a predictive factor to assist in determining which patients will require surgery. However, if symptoms recur quickly or there is only minimal response after initial dilatation, some authors recommend direct referral for surgery (8,9).
Three studies evaluating the use of botulinum toxin in children with achalasia have been published between 1997 and 2000 (17–19). The largest series of pediatric patients in a retrospective study by Hurwitz et al. (18) included 23 patients undergoing botulinum toxin therapy between 1995 and 1998. The study concluded that 50% of the patients were expected to receive further intervention, such as dilatation, repeated botulinum toxin injections, or surgery, 7 months after treatment. The mean duration of effect after the first treatment was only 4.2 months ± 4.0 (standard deviation). Recently, Ip et al. (19) confirmed these results. Seven of seven patients had recurrence of their symptoms 4 months after their initial botulinum toxin treatment. All seven patients in the series also required further treatment, and sustained response was found in only three of the patients at 6 months after the procedure. In these reports, botulinum toxin provided only temporary relief of symptoms, allowing the patients to gain weight before surgical treatment (17), or was reserved for poor dilatation or surgery candidates (18). A low pretreatment lower esophageal sphincter pressure is a positive predictor of sustained response (19). Botulinum toxin, therefore, should not be considered a definitive treatment for achalasia in the pediatric population.
Seven studies evaluating the clinical outcome after open myotomy (21–27) were published from 1985 to 1997 (Table 2). Each study had at least 10 children with achalasia. Overall, the patients had a good to excellent outcome of their symptoms with regard to dysphagia in 71% to 97% of cases. The largest study, by Myers et al. (26), described the worst outcome percent of 71%, but delineation of various surgical methods within the study had differing results. One hundred sixty-four of 175 pediatric patients underwent esophagomyotomy through a variety of approaches. These included transabdominal myotomy without an antireflux procedure, transabdominal myotomy with an antireflux procedure, transthoracic myotomy without an antireflux procedure, and transthoracic myotomy with an antireflux procedure. The transabdominal myotomy with an antireflux procedure was completed in 66 patients and resulted in the best outcome, with a 91% symptom resolution rate. After transabdominal myotomy without an antireflux procedure (22 patients), transthoracic myotomy with antireflux procedure (13 patients), and transthoracic myotomy without antireflux procedure (63 patients), 25%, 50%, and 53% of patients experienced complete symptom resolution, respectively. Only two of the seven studies evaluated a median length of hospital stay of at least 7 days (range, 4–47 days) (24,27). None of the studies examined time to refeeding.
Recommendations from all of these studies include the transabdominal approach with a concomitant antireflux procedure as the preferred procedure for the treatment of achalasia in children (21–27).
Although open esophagomyotomy in children with achalasia is associated with a better outcome than are dilatation or botulinum toxin treatments, as suggested by the previous short-term studies, until now dilatation remains the first line of treatment. Surgical treatment for achalasia in children has been reserved for patients who experienced perforation during dilatation, residual dysphagia after multiple dilatations, or for those who are poor candidates for dilatation or botulinum toxin injection. Further concerns about operative risk, postoperative pain, and prolonged hospitalization have made surgery a final option for most patients. If laparoscopic or thoracoscopic approach decreases the concerns involved in the open surgical approach, then it may be a viable first option, especially for children. Children with achalasia differ from adults, in that earlier surgical treatment may be more appropriate than multiple dilatations or botulinum toxin injections over the course of a lifetime. A multicenter database with patients who are studied thoroughly before surgery, randomized to treatments, and studied thoroughly after surgery is recommended to compile information on children with achalasia to assist in choosing the most appropriate method of treatment.
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