Evaluation of Minimally Invasive Approaches to Achalasia in Children By Steven S. Rothenberg, David A. Partrick, John F. Bealer, and Jack H.T. Chang Denver, Colorado
Background: Achalasia is an uncommon disease in children, but when present can result in severe disabling symptoms often requiring surgical intervention. This report describes the authors’ experience with thoracoscopic (TH) and later laparoscopic Heller (LH) myotomy for definitive treatment of this disease. Methods: Nine patients with achalasia were referred for surgical therapy. Ages ranged from 5 to 17 years and weight from 23 to 78 kg. All had undergone at least one dilatation with recurrence of symptoms. The first 4 were treated by TH and the last five by LH. The 5 LH procedures also included a partial fundoplication. Results: All procedures were completed successfully using minimally invasive techniques. Operating times averaged 95 minutes for TH and 62 minutes for LH. One patient undergoing TH had a small esophageal perforation repaired primarily. The other 3 TH patients were started on clear liquids within 1 day and discharged on day 2. One patient had
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DVANCEMENTS in minimally invasive surgery (MIS) over the last decade have allowed for the application of these techniques to a wide range of diseases in children. One such area is in the treatment of achalasia. Although this is a relatively rare pediatric problem, the debate over the best course of therapy has been wide ranging.1 The discussion involves not only the role of balloon dilatation versus surgical myotomy but also the best surgical approach.2-4 There has been a significant shift in treatment of achalasia with the introduction of MIS to perform the esophagomyotomy, but the best approach (transthoracic or transabdominal) is still an area of significant debate.4 This report describes our experience with achalasia in children over the last 7 years and the evolution of our treatment protocol. From the Departments of Pediatric Surgery, Hospital for Infants and Children, Presbyterian/St Luke’s Medical Center and The Children’s Hospital, University of Colorado, Denver, CO. Presented at the 32nd Annual Meeting of the Canadian Association of Paediatric Surgeons, Chaˆteau Montebello, Quebec, Canada, September 15-18, 2000. Address reprint requests to Steven S. Rothenberg, MD, 1601 E 19th Ave, Suite 5200, Denver, CO 80218. Copyright © 2001 by W.B. Saunders Company 0022-3468/01/3605-0032$35.00/0 doi:10.1053/jpsu.2001.22967
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recurrent symptoms at 6 months and underwent a LH for an incomplete TH. All 5 LH patients were discharged on postoperative day 1. One had an esophageal perforation 4 days after operation requiring laparoscopic repair. Seven of 9 patients are asymptomatic. Studies of pH levels in 2 asymptomatic TH patients show mild gastroesophageal reflux (GER).
Conclusions: Minimally invasive Heller myotomy is a safe and effective procedure in children. TH results in a slightly longer operating time and hospital stay and, without a partial fundoplication, also may be associated with a higher incidence of silent GER. From these results, we prefer LH with a Dor fundoplication for treatment of achalasia in children. J Pediatr Surg 36:808-810. Copyright © 2001 by W.B. Saunders Company. INDEX WORDS: Achalasia, Heller myotomy, laparoscopy, thoracoscopy, minimally invasive surgery.
MATERIALS AND METHODS From February 1993 to June 2000, 9 patients were referred for surgical therapy of achalasia. The patients ranged in age from 5 years to 17 years (mean, 12 years) and weight from 23 to 78 kg (mean, 45 kg). The patients had been symptomatic from 6 months to 2 years. All had undergone upper gastrointestinal contrast studies that showed a dilated thoracic esophagus with classic “birds beak narrowing” at the lower esophageal sphincter (LES). Three patients had manometry that supported the diagnosis. All 9 patients had undergone at least 1 esophageal pneumatic dilatation that improved their dysphagia, but all had recurrent symptoms within 6 to 8 weeks. No patients had undergone botulinum injection. A thoracoscopic Heller myotomy (TH) was performed to treat the first 4 patients. This was done with the patient in a modified prone position as described previously.5 The left side was elevated 30°, and one lung anesthesia was obtained by performing a right mainstem intubation. Four ports (3 or 5 mm) were used between the posterior and anterior axillary lines and between the fifth and eighth intercostal spaces. The esophagomyotomy was performed with either a hook cautery or the hook attachment to the Harmonic Scalpel (Ethicon Endosurgery, Cincinnati, OH). The myotomy was completed proximally to the level of the inferior pulmonary vein and distally being sure to traverse the LES. No antireflux surgical procedures were performed in TH cases. A flexible endoscope was placed in the esophagus for all cases to help identify the esophagus, check for perforations, and determine completeness of the myotomy. Chest tubes were left in all 4 thoracoscopic cases. The next 5 patients were approached laparoscopically (LH) and underwent a partial fundoplication in association with a Heller esophagomyotomy. The approach used was the same as has been previously described for a laparoscopic fundoplication.6 Five trocars (3 or 5 mm)
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were placed with the camera port in the umbilicus, the right and left working ports in the midquadrants, a liver retractor in the right upper quadrant in the anterior axillary line, and the stomach/esophageal retractor in the left upper quadrant. Similar to TH patients, a flexible endoscope was placed in the esophagus in each case. The right and left diaphragmatic crura were identified and cleared, and the esophagus was mobilized intraabdominally with downward traction on the stomach. The esophagomyotomy then was performed making sure to completely cross the LES and extend the myotomy onto the stomach. Four LH patients had a Dor fundoplication, and 1 had a Toupet after completion of the esophagomyotomy. An upper gastrointestinal contrast study was obtained at 1 and 6 months in all cases to evaluate the myotomy and check for gastroesophageal reflux (GER).
RESULTS
All procedures were completed successfully endoscopically. The operating times ranged from 45 to 120 minutes. The average time for a TH was 95 minutes; the average time for LH and fundoplication was 62 minutes. One patient undergoing TH had a small esophageal perforation noted at the time of surgery. This was repaired primarily during the same operation using the thoracoscope. There were no other intraoperative complications. Oral feedings were resumed on the first postoperative day except for the patient with the perforation. Chest tubes were removed on day 1 in the first 3 patients, and they were discharged on day 2. The patient with the repaired esophageal perforation underwent a barium swallow test on postoperative day 4, which showed no leak. The chest tube was removed, she was started on oral feedings, and was discharged home on day 5. The patients undergoing LH were started on oral feedings 6 hours postoperatively. All were discharged on the first postoperative day. The patient who had a LH and Toupet fundoplication returned on postoperative day 5 with evidence of an esophageal perforation. He underwent reexploration laparoscopically, and an esophageal perforation extending the length of the myotomy was discovered. This was repaired laparoscopically, and the Toupet was converted to a Dor fundoplication. One week later he required laparoscopic exploration for drainage of multiple interloop abscesses. One patient who had a TH developed recurrent symptoms of dysphagia 6 months postoperatively. She returned to the operating room for an LH and Dor fundoplication because of an incomplete myotomy and currently is asymptomatic at 2 years out. Six patients are relatively asymptomatic with only occasional difficulty swallowing. One has mild persistent dysphagia but is gaining weight and tolerating a regular diet. Follow-up pH studies show that 2 of the patients who had a TH have mild GER that is asymptomatic. They are doing well on antireflux medication. None of the patients who had an LH and Dor fundoplication have GER on follow-up upper gastrointestinal contrast study.
DISCUSSION
Achalasia in children is a rarely diagnosed disorder making evaluation of the most efficacious therapy difficult. The largest series ever evaluated was a retrospective review of the worlds’ experience performed by Myers et al1 in 1994. They collected data on 175 patients who presented with symptoms during childhood and underwent either a transthoracic or transabdominal Heller myotomy. They found the majority of patients had good to excellent results after myotomy by either technique. However, many still advocate more conservative therapy such as repeated pneumatic dilatation and, more recently, injection of the LES with Botulinum toxin rather than surgery. These techniques, although seemingly less invasive have their own associated morbidity. Pneumatic dilatation has a recognized risk of esophageal perforation as well as a relatively high recurrence rate.7,8 Preoperative pneumatic dilatation or botulinum toxin injection can increase the risk of esophageal perforation in patients who later go on to undergo esophagomyotomy.9,10 Furthermore, a recent study in children with achalasia found repeated botulinum injections were necessary in over one half of patients.11 They therefore recommended that botulinum toxin only be used in children who were poor surgical candidates. In 1993 Pellegrini et al12 reported the first significant series of TH and LH in adults. These initial reports showed the safety and efficacy of MIS for treatment of achalasia and set the stage for earlier use of surgical myotomy. However, the debate over whether a transthoracic or transabdominal approach was better remained unanswered. The argument has been made that by using a transthoracic approach the surgeon can achieve an adequate-length myotomy and minimize the dissection at the hiatus, thereby diminishing the need for an antireflux procedure.12 Others, including Holcomb et al13 feel that a limited intraabdominal dissection also eliminates the need for a fundoplication. In 1998, Patti et al4 reported an 8-year experience with minimally invasive surgery for achalasia and found that although both groups had good symptomatic relief, 60% of the TH patients had significant GER by pH probe studies as opposed to 16% of the LH group. The majority of these patients were asymptomatic. The LH group also experienced a shorter operating time, shorter hospital stay, and slightly less postoperative dysphagia.4 Over the last 5 years there have been a few small series published using LH and TH in children with similar results to that found in the adult experience.13,14 The major points of debate seem to be the most appropriate surgical approach and the need for an associated antireflux procedure. Many of the reports claiming no signif-
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icant GER postoperatively are measuring only the patients’ clinical symptoms; pH probes have not been done routinely. The authors initially favored a transthoracic approach because it was felt this would avoid significant hiatal dissection and would insure an adequate length myotomy (up to the inferior pulmonary vein). However, the reports of silent GER prompted further follow-up in our small group of patients with pH probe studies. Two of 4 patients (50%) were found to have clinically significant GER (⬎8%) despite being asymptomatic. One patient had recurrent symptoms at 6 months and an upper gastrointestinal contrast study suggested an incomplete distal myotomy. This was despite the standard use of an esophagoscope at the time of surgery to determine completeness of the myotomy. However, this also was the patient who was noted to have the small distal perforation intraoperatively, and, after the repair, further esophageal manipulation was minimized. Her myotomy was redone laparoscopically and combined with a Dor fundoplication. The most crucial and technically demanding portion of the operation is at the lower esophageal sphincter (LES), and this probably is best approached through the abdomen. The approach for a LH is identical to that of a laparoscopic fundoplication. For the authors, as well as most surgeons, LH is a more familiar and efficient technique than that of TH. In our series, the operating time and hospital stay have been cut in half since adopting a laparoscopic technique despite the addition of a partial fundoplication wrap.
Complications associated with LH are not uncommon.15 The most devastating complication is that of an unrecognized or delayed perforation, and there was one in this series. No injury or mucosal perforation was detected during the procedure, and the patient was discharged, asymptomatic, on day 1. Incidentally, this patient had received a course of inhaled steroids, and Candida esophagitis developed 2 weeks before surgery. He was treated with oral antifungal therapy, and the esophagitis appeared to clear before surgery. The esophagomyotomy was performed using a hook cautery, and a Toupet fundoplication was performed. The exact cause of the delayed perforation is not clear, but a partialthickness mucosal injury must be suspected. This cautery injury combined with a posterior partial wrap may have put increased tension on the mucosa by pulling apart the myotomy, thus contributing to the delayed rupture. For these reasons, the authors now favor an LH with a Dor fundoplication. The Dor fundoplication provides an antireflux procedure as well as an anterior patch for the exposed mucosal wall. The use of electrocautery must be questioned as a possible source of partial thickness or delayed injury. It may therefore be better to perform the myotomy sharply and accept some mild bleeding. None of the patients in this series had undergone botulinum toxin injection, but in adult series this has been associated with an increased risk of perforation and complications.10 Botulinum toxin should thus be avoided in any patient who is a candidate for LH.
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dilatation represents a risk factor for laparoscopic Heller myotomy. Surg Endosc 11:359-361, 1997 10. Patti MG, Feo CV, Arcerito M, et al: Effects of previous treatment on results of laparoscopic Heller myotomy for achalasia. Dig Dis Sci 44:2270-2276, 1999 11. 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 12. Pellegrini CA, Leichter R, Patti M, et al: Thoracoscopic esophageal myotomy in the treatment of achalasia. Ann Thorac Surg 56:680682, 1993 13. Holcomb GW, Richards WO, Riedel BD: Laparoscopic esophagomyotomy for achalasia in children. J Pediatr Surg 31:716-718, 1996 14. Esposito C, Cucchiara S, Borrelli O, et al: Laparoscopic esophagomyotomy for the treatment of achalasia in children. A preliminary report of eight cases. Surg Endosc 14:110-113, 2000 15. Esposito C, Mendoza-Sagaon M, Roblot-Maigret B, et al: Complications of laparoscopic treatment of esophageal achalasia in children. J Pediatr Surg 35:680-683, 2000