Esophageal achalasia (EA) was first described in 1694 by Willis and is an idiopathic motor alteration of the smooth esophageal muscle characterized by absent postdeglutition relaxation of the lower esophageal sphincter (LES) and replacement of physiological peristalsis by repetitive, simultaneous, and propulsive contractions (ie, tertiary contractions). Liquid and solid food stagnation in the visceral body causes progressive dilation at the top of the achalasic segment (ie, megaesophagus); the food bolus enters the stomach only when hydrostatic pressure can overcome functional obstruction (1).
Achalasia is the best known and most common disorder among primary and specific esophageal motility alterations, has an incidence of 1 case per 1000 yearly in the age range of 30 to 50 years and a slight prevalence in males; however, it must be considered a rare condition in children (1.6%–2% of patients are children) (2). The low incidence in childhood makes it all the more necessary to study larger pediatric populations and create multicenter studies to unify patient cohorts from different centers.
The aim of the present study is to present results of minimally invasive surgery of EA through the Heller procedure and Dor anterior fundoplication at the Unit of Paediatric Surgery at the University of Siena, Italy, and the Lenval Foundation in Nice, France.
PATIENTS AND METHODS
Between January 1997 and October 2005 at 2 institutions, 14 young patients were treated for EA (Table 1). Two patients were not included in the study because 1 had apparently benefited from pneumatic dilations and the other was on the waiting list for surgery.
The patients group included 5 female and 7 male patients with ages ranging between 3.5 and 16 years (mean age, 11 years). All of the patients had symptoms of advanced EA: regurgitation or vomiting, dysphagia, and marked weight loss. Seven of 12 patients (58.3%) had been seen by a pneumologist for persistent respiratory impairment.
All patients underwent chest radiograph, barium radiograph, esophageal manometry, and esophageal gastric duodenoscopy, which allowed for a correct diagnosis. In a few cases, achalasia was diagnosed during computed tomography for suspected respiratory diseases.
A 5-year-old patient showed severe neuropsychomotor retardation for perinatal suffering associated with growth retardation (below the third percentile). An 8-year-old girl had triple A syndrome, a disorder caused by alteration of the whole sympathic and parasympathic autonomous innervation and characterized by contemporary presence of achalasia, alacrima and asthenia (3). One patient had Rett syndrome and one had juvenile parkinsonism.
All of the patients underwent modified Heller extramucosal myotomy systematically associated with Thal/Dor antireflux plastic surgery by open laparoscopy. During this procedure, the patient is placed in the supine position with reverse Trendelenburg maneuver and divaricated legs. The first operator stands between the patient's legs, with the cameraman on the right side of the patient and the second operator on his left side. Five 5-mm trocars are placed as follows: 1 on the left flank on the hemiclavear line, 1 transumbilically for the camera, 1 left subcostally, and 1 left and 1 right periumbilically. A pneumoperitoneum is created through CO2 insufflation with a 12-mmHg pressure. After retracting the stomach and liver and detecting the gastroesophageal junction, the phrenoesophageal ligament is cut and isolation of the esophagus is achieved anteriorly. A myotomy is then carried out, leading to mucosal herniation, with a muscle dissection of approximately 7 cm. Air insufflation through a nasogastric tube aims at excluding perforation of the herniated esophageal mucosa, and the presence of air bubbles at the myotomy site must be ruled out as well. The procedure is completed with a 180° anterior fundoplication to protect the herniated mucosa and prevent gastroesophageal reflux. Follow-up ranged from 18 to 60 months.
A total of 12 Heller procedures were performed: 6 Thal and 6 Dor fundoplications. Duration of surgery ranged from 90 to 180 minutes (average, 120 min). Esophageal mucosa perforation occurred in one case, which was promptly visualized and repaired via laparoscopy with no consequences in the immediate and later postoperative phases. The nasogastric tube inserted at the moment of surgery was removed after 24 to 48 hours, and patients were restarted on oral feeding on day 3 postoperatively and discharged between day 5 and day 8.
All of the patients were seen at the first and third months after surgery for clinical examination; at 6 months a barium radiograph of the upper digestive tract was performed as part of the clinical examination. Not all of the parents consented to the esophageal manometry to be carried out postoperatively in the absence of symptoms; therefore, only 2 patients underwent the procedure.
All of the patients showed complete regression of digestive and respiratory symptoms from the first examination, with normal oral food intake and an improvement of weight and height parameters. In 3 cases painful symptoms persisted despite resolution of respiratory problems, weight gain, and normal pH studies and endoscopy findings. As a result of persisting later pain symptoms, one patient underwent esophageal manometry, which proved normal, whereas the barium radiograph showed residual narrowing of the gastroesophageal tract; a surgical repeat intervention showed an insufficient myotomy on the stomach wall.
Given the low incidence of achalasia in children, few studies have been published on the treatment of the megaesophagus in the pediatric population. However, the treatment of choice for achalasia in children is undoubtedly extramucosal esophagomyotomy (4,5).
Administration of nitrates, calcium channel blockers, and botulinum toxin in children has not been extensively studied. Today, botulinum toxin is primarily administered to patients at high risk for complications from endoscopic dilations and surgery (6). None of our patients were given pharmacological treatment.
Endoscopic dilations—mechanical, hydrostatic or pneumatic, which are the most common ones—achieved optimal results in adult patients. Botzas et al reported the results of a long-term follow-up (mean follow-up >3 years) in adults (mean age, 41.42 ± 18.07 years) with EA treated with endoscopic pneumatic dilations (7), in which a 12% rate of esophagitis and no esophageal perforation were reported. In the literature the reported rate of gastroesophageal reflux and postdilation esophagitis ranges from 1.5% to 26.5%; the rate of postendoscopic pneumatic dilation esophageal perforation is 1.5% to 3.0%, although, thanks to the so-called safe probes, the most recent studies do not describe cases of postendoscopic pneumatic dilation esophageal perforation (7,8).
The choice between endoscopic dilation and myotomy is still debated because both techniques seem to produce the same symptom resolution rate in the adult population. Two randomized trials in adult patients have been published that comparatively analyze the endoscopic pneumatic dilation of achalasic LES and modified Heller myotomy with open access; we are aware of no references to the minimally invasive approach in pediatric patients (9–11). Surgery is pursued by some investigators to avoid emergency thoracotomy, notwithstanding a low risk of perforation and secondary postdilation gastroesophageal reflux (12).
The technique applied at both institutions in the present study was a modified Heller extramucosal cardiomyotomy with anterior 180° fundoplication. Among the first pediatric patient series, that of Esposito et al highlights the optimal resolution of the disorder following laparoscopy associated to antireflux plastic repair (13). The advantages of laparoscopy are several (13): image magnification, minimal traction, and tissue dissection with decrease of trauma, postoperative pain, and administration of third-level analgesics. All of this allows for quicker recovery in the immediate postoperative phase of intestinal transit and oral food intake with shorter hospitalization time and faster return to daily activities. The aesthetic result is also worth mentioning as it modifies the patient's psychological and relational development (14). The myotomy's length should be approximately 6 to 8 cm long, whereas the cut of the gastric wall fibers should be more than 2 to 3 cm. Muscles were dissected on the esophageal wall up to the point where the vagus nerve passes anteriorly from right to left. Oelschlager et al described a dysphagia rate of 17.3% in a patient group with dissection extended to approximately 1.5 cm on the gastric wall, with the rate decreasing to 3.4% in the patient group with myotomy extended to 3 cm on the gastric wall (15). In our case series 1 case (8.3%) of postoperative dysphagia was caused by insufficient myotomy on the gastric wall.
Surgery can be performed via a transabdominal or transthoracic approach. The approach selected at both treatment centers in the present study was transabdominal because it enables the surgeon to associate antireflux plastic surgery and is less traumatizing for the patient. Other authors, including Holcomb et al, state that the abdominal approach allows restriction of surgical dissection to the esophageal hiatus, diminishing the number of antireflux plastic repair interventions (16).
Patti et al describe their 8-year experience in minimally invasive treatment, highlighting a shorter duration of surgery in the group treated via abdominal approach, along with shorter hospitalization time and a lower rate of postoperative dysphagia. In the same study, the authors encountered a 60% rate of postoperative gastroesophageal reflux in the transthoracic approach patient group, compared with 16% in the group treated with an abdominal approach (6).
Antireflux plastic surgery has been systematically associated with our procedures and to date (60-month maximum follow-up) no case of gastroesophageal reflux has been reported in the short or long term. In fact, several studies show how patients undergoing a modified Heller procedure may subsequently develop gastroesophageal reflux (20%) (13,17). At present, anterior plastic repair is most commonly associated with surgery for achalasia (18): it protects the exposed mucosa after myotomy, but the antireflux valve exerts a slight containment of the gastroesophageal area, which presents an inactive and resected LES. Moreover, 180° antireflux plastic surgery has a lower associated risk of secondary stenosis at the level of LES compared with 360° surgery (eg, Nissen procedure) (19,20).
Zaninotto et al report a rate of dysphagia following Heller procedure associated with anterior plastic surgery (ie, Dor procedure) of 8.8% as a result of incomplete myotomy and stenosing and retracting scars between the herniated mucosa and the anterior antireflux valve (2). Lyass et al support this hypothesis, thus explaining their exclusion of antireflux plastic repair (21). Oelschlager et al described an incidence rate of postoperative dysphagia of 17.3% in the patient group treated with Heller/Dor technique, as opposed to 3.4% in the group treated with the Heller/Toupet procedure (15). All of the mentioned case series comprise adult patients. In our experience 1 case required repeat intervention for recurring disease; during surgery, no special difficulty was encountered in the performance of a further myotomy on the gastric wall, and no occurrence of retracting scars between the esophageal mucosa and the plastic surgery site were seen. Rothember et al described 1 case of late and unknown perforation in 1 patient with achalasia treated with the Heller/Toupet procedure (22). The authors consider the perforation to be a result of the use of hook cautery and a posterior antireflux plastic repair, which causes late rupture by exerting tension on the muscle strips and mucosa (22).
In our case series 2 children (8.3%) reported dysphagia in the first 6 months after surgery, which was not caused by intraoperative complications. Twenty-four–hour pH-metric evaluation did not show any gastroesophageal reflux; therefore, antireflux drugs were not administered to any patient at either treatment center because the symptoms were attributed to typical persistent disease-related incoordination of esophageal motility. At 1 year after surgery, in both cases, dysphagia symptoms were attenuated with improvement of the weight growth curve.
Possible complications of these procedures are intraoperative lacerations of the herniated esophageal mucosa or nearby organs (22). In our series 1 case of macroscopic mucosal perforation was diagnosed (8.3%), which was promptly repaired during surgery. Noticeably, the patient with perforation and the one with insufficient myotomy were the first cases of achalasia treated with the minimally invasive approach. The interventions were carried out during the “learning curve” period for this specific intervention, which is one of the most important problems for minimally invasive techniques.
Our data, compared with the literature, confirm that modified extramucosal Heller cardiomyotomy associated with 180° anterior antireflux plastic surgery (according to Thal and Dor's procedures) is a useful and safe procedure in the treatment of EA in pediatric patients. Minimally invasive surgery allows reduction of postoperative trauma, even though it is highly affected by the operator's learning curve. Our data, which are supported by long-term follow-up, also stress the relevance of anterior fundoplication in preventing postoperative gastroesophageal reflux.
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