Journal of Pediatric Surgery (2009) 44, 909–911
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Minimally invasive Heller's myotomy in children: safe and effective Johanna R. Askegard-Giesmann, Jayleen M. Grams, Angela M. Hanna, Corey W. Iqbal, Swee Teh, Christopher R. Moir ⁎ Division of Pediatric Surgery, Department of Surgery, Mayo Clinic, Rochester, Minn 55905, USA Received 5 January 2009; accepted 15 January 2009
Key words: Laparoscopic; Heller's myotomy; Pediatric; Minimally invasive; Achalasia
Abstract Purpose: The aim of the study was to review a single institution experience of minimally invasive Heller's myotomy in pediatric patients with achalasia. Methods: An institutional review board-approved retrospective review from 1999 to 2005 identified patients 18 years old and younger who underwent a minimally invasive Heller's myotomy for achalasia. Results: Twenty-six patients were identified with a mean age of 15 (range, 4-18 years). There were 11 female and 15 male patients. There were 3 intraoperative complications (2 esophageal mucosal injuries and 1 aspiration). There was no mortality. All 26 surgeries were completed laparoscopically. Two patients had Dor fundoplication, whereas 23 patients had Toupet fundoplication. Average length of hospital stay was 2.7 days (range, 1-4 days) excluding the 3 patients with intraoperative complications and 3.5 days for all patients (range, 1-17 days). Postoperative follow-up ranged from 0 to 75 months (mean, 20 months). Postoperatively, one patient developed reflux symptoms (incidence 4%). Seven patients (27%) had recurrence of symptoms at a mean of 13 months (range, 1-66 months) after their operation. Conclusions: Laparoscopic Heller's myotomy with fundoplication is a safe and effective treatment of symptomatic achalasia in the pediatric population. Complications were low in this group of patients and comparable to other published reports in the literature. © 2009 Elsevier Inc. All rights reserved.
Achalasia is a primary motility disorder of the esophagus that results in poor relaxation of the lower esophageal sphincter (LES) and eventual loss of esophageal peristalsis. This is a rare disease in children with an estimated incidence of 0.11 cases per 100,000 children [1-3]. The treatment of achalasia has evolved over the years from medical treatment to attempt to relax the LES, pneumatic dilation to stretch the Presented at the 40th Annual CAPS Meeting, August 21-24, 2008, Toronto, Ontario, Canada. ⁎ Corresponding author. Tel.: +1 507 284 2623; fax: +1 507 284 0058. E-mail address:
[email protected] (C.R. Moir). 0022-3468/$ – see front matter © 2009 Elsevier Inc. All rights reserved. doi:10.1016/j.jpedsurg.2009.01.022
LES, botulinum toxin injections to paralyze the LES, and surgical myotomy to disrupt the muscle fibers of the LES. Although the underlying disease cannot be eradicated, these interventions have improved the symptoms associated with achalasia in many children. The mode of surgical treatment has evolved over the years, and most recently, laparoscopic Heller's myotomy has been indicated for the treatment of achalasia in children. This article examines a singleinstitution experience of laparoscopic Heller's myotomy in children younger than 18 years of age with combined antireflux procedure to identify efficacy and safety of the laparoscopic procedure.
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1. Methods With the approval of the institutional review board, we identified 26 patients younger than 18 years old who were treated at our institution for achalasia and had undergone a laparoscopic Heller's myotomy from 1999 to 2005. Information collected for each patient included age, sex, medical and family history, presenting symptoms, diagnostic methods, previous treatments (such as pneumatic dilation, botulinum toxin injections or prior surgery, surgical treatment), and outcomes.
2. Results Twenty-six patients were identified with a mean age of 15 years (range, 4-18 years). There were 11 female and 15 male patients. Fourteen patients underwent pneumatic dilation, and 6 patients underwent botulinum toxin injections before seeking definitive surgical treatment. There were 3 intraoperative complications: 2 esophageal mucosal injuries repaired primarily at the time of surgery (one occurred in a reoperative situation) and 1 aspiration that occurred upon induction of anesthesia. There was no mortality. All 26 surgeries were completed laparoscopically with no conversions to open procedures. One patient did not have a simultaneous antireflux procedure because of severe esophageal dysmotility and a reoperative field. Two patients had Dor fundoplication, whereas 23 patients had Toupet fundoplication. Average length of hospital stay was 2.7 days (range, 1-4 days) excluding the 3 patients with intraoperative complications and 3.5 days for all patients (range, 1-17 days). Postoperative follow-up ranged from 0 to 74.5 months (mean, 20.4 months). One patient (4%) had reflux symptoms that were treated medically and did not require additional surgery. Seven patients (27%) had recurrence of symptoms between 1 month and 5 years after their operation, as follows: 3 patients required reoperation, 3 were treated with pneumatic dilation, and/or botulinum toxin injection, and 1 treated elsewhere (therapy unknown). In the patients who required reoperation, the fundoplication was taken down with extension of the myotomy proximally and distally in 2 patients, and one patient only required extension of the myotomy onto the stomach wall.
3. Discussion Achalasia is a rare idiopathic disorder of esophageal motility that results in near complete disruption of esophageal function. It affects both the LES and smooth musculature of the esophagus and is thought to result from smooth muscle denervation [3]. The diagnosis of achalasia can be difficult in children as the symptoms can be confused
J.R. Askegard-Giesmann et al. with feeding aversion, failure to thrive, or gastroesophageal reflux [4]. However, in patients older than 5 years, the clinical presentation is very similar to adults, as follows: vomiting, dysphagia, weight loss, recurrent pneumonia, failure to thrive, or nocturnal cough. All of our patients presented with dysphagia, and more than 50% also had reflux and/or vomiting. All patients underwent manometric and barium studies to confirm the diagnosis. Treatment of achalasia is focused on improving symptoms by relaxing the LES, which in turn may prevent worsening of esophageal motility. The earlier the obstruction can be relieved by myotomy or other means, the less likely the patient will need to progress to esophagectomy [5]. Medical treatment has been attempted in the form of calcium channel blockers and nitrates, with minimal effect, especially in children, and this therapy is only recommended for short-term symptomatic relief pending definitive surgical treatment [4]. The use of pneumatic dilation and botulinum toxin injection over surgery has been debated in the literature, especially in children. Both pneumatic dilation and botulinum toxin injection have demonstrated improvement in the symptoms related to achalasia, but neither treatment has long-term efficacy and require repeated procedures with their associated risks [4,6]. Pneumatic dilation has a reported success rate of 78.7% [4] but requires repeated endoscopy and carries a risk for esophageal perforation with each procedure. The incidence of esophageal perforation in the pediatric population is not known, and one study reported 3 (6%) perforations in 50 procedures [4]. For adults, the incidence ranges from 0% to 21%, with most studies not exceeding 5% [4]. Fourteen of our patients had received pneumatic dilation before surgery without any complications. Botulinum toxin injection has been shown to improve symptoms in patients for as long as 6 months from a single injection, but repeated injections are often necessary [6]. Botulinum toxin injection has been associated with increased scarring that may make the subsequent surgical myotomy more difficult [7]. However, this was not seen in our population. Six patients had undergone botulinum toxin injections before their operation, and all underwent laparoscopic Heller's myotomy with fundoplication without an intraoperative complication. Only one patient developed reflux postoperatively that was treated medically. Minimally invasive Heller's myotomy with or without fundoplication is quickly becoming the accepted surgical treatment of pediatric achalasia. There are several series in the pediatric literature that advocate the thoracoscopic or laparoscopic approach for Heller's myotomy in the treatment of achalasia [5,7-11]. At our institution, nearly all adult and pediatric patients with achalasia are treated laparoscopically and nearly always in conjunction with an antireflux procedure. There has been very little published about the complications of a minimally invasive approach to this procedure. Perforation of the esophageal mucosa has been
Minimally invasive Heller's myotomy in children reported both during the myotomy as well as during the fundoplication portion of the procedure [10]. When detected at the time of surgery, the perforation can be repaired primarily. Delayed perforation may be treated with drainage, antibiotics, fasting, and parenteral nutrition or may be managed by reoperation. Dysphagia or recurrent symptoms may be troubling complications after minimally invasive surgery as the inherent esophageal dysmotility may be a confounding variable. Incomplete myotomy or a constricting fundoplication should also be considered in patients with recurrent symptoms. In our series, there were 2 intraoperative perforations of the esophageal mucosa, one of which occurred in a reoperative field. One patient aspirated upon induction of anesthesia, a complication that could have been prevented with preoperative esophageal suctioning using a nasogastric tube. Although laparoscopic Heller's myotomy is becoming the treatment of choice for patients with achalasia, debate still exists regarding a concomitant antireflux procedure and if so the type of antireflux procedure to choose. Opponents of using an antireflux procedure claim that avoiding excessive posterior dissection and any closure of the crura will help prevent reflux symptoms [14]. However, the adult literature supports the use of an antireflux procedure with Heller's myotomy, as the risk of developing gastroesophageal reflux has been reported to be as high as 48% in long-term follow-up [12,13]. Dor fundoplication has been combined with Heller's myotomy in several series in the pediatric population with adequate results [5,7,10]. The posterior partial fundoplication (Toupet fundoplication) has been more effective in controlling postoperative dysphagia in the adult literature [13]. Katada et al [13] performed laparoscopic Heller's myotomy with Toupet fundoplication in 30 patients who had achalasia with severe dysphagia. They found that the combination of the laparoscopic Heller's myotomy with Toupet fundoplication helped to straighten the esophagus, reduced LES pressure, and relieved dysphagia. Our patient population did well with the Toupet fundoplication with only one patient experiencing postoperative gastroesophageal reflux symptoms that were treated medically. Postoperative pH testing was not performed on our patients and may be necessary to determine the true incidence of gastroesophageal reflux after Heller's myotomy. Recurrence of symptoms, especially dysphagia, has been reported from 0% to 20% in the pediatric literature [5,7,10] and 6% to 23% in the adult literature [14]. It is difficult to determine if the postoperative dysphagia is directly related to failure of the surgery or the result of an inherent motility disorder of the distal esophagus. The incidence of symptom recurrence in our patient population was somewhat higher than reported in the literature (27%). This may have been partially because of the duration of symptoms before
911 proceeding with surgery in our patients (range, 0-6 years between diagnosis and surgery) as well as the number of patients seeking care at our institution after failing medical and surgical treatment elsewhere.
4. Conclusions Laparoscopic Heller's myotomy with fundoplication is a safe and effective treatment of symptomatic achalasia in the pediatric population [15]. Complications were low in this group of patients and comparable to other published reports in the literature. However, the recurrence rate of symptoms was somewhat higher than reported in the literature.
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