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- W2088832157 abstract "Esophageal achalasia is a rare primary motor disorder of the esophagus in children. Because the etiology of achalasia is not well defined, treatment is generally directed at symptomatic relief of the functional obstruction at the level of the lower esophageal sphincter (LES) (1,2). Even though the optimal treatment is still debated (1–3), minimally invasive esophagomyotomy has become the treatment of choice in many centers. Although the experience with this surgical procedure in children is still limited, there has been a recent increase in number of procedures and their associated complications. Dysphagia is the most common and debilitating long-term problem, and there is a paucity of published information about the approach to the child with achalasia that has dysphagia after an esophageal myotomy. In the present report, we describe our recent experience with 5 patients with severe dysphagia after myotomy, review the literature, and suggest an approach to their evaluation and treatment. CASE 1 A 12-year-old boy with achalasia underwent 2 pneumatic dilatations (PD) up to 35 mm before he experienced improvement in his dysphagia and chest pain. Four months after the last PD, there was recurrence of his symptoms, and a thoracoscopic esophagomyotomy without fundoplication was performed at our institution. He did well for 2 months, then his dysphagia and chest pain returned. A barium study showed a narrow gastroesophageal junction with marked delayed contrast passage into the stomach, with almost complete barium retention after 10 minutes. Esophageal manometry revealed a 1.5-cm-long high-pressure zone of 19 mmHg in the distal esophagus, no esophageal body peristalsis, and a 10-mmHg esophagogastric pressure gradient. A PD (30 mm) was performed with good results. Over the next 3 years, he required 2 more PDs (up to 35 mm) because of recurrence of his dysphagia. Between dilatations, he showed marked improvement for approximately 6 to 10 months. Twenty-four months after the last PD, his dysphagia worsened. A repeat barium study showed minimal retention of barium after 10 minutes, and a repeat esophageal manometry showed a maximum pressure in the distal esophagus of only 5 mmHg, absent esophageal peristalsis with no esophagogastric gradient, suggesting his mild symptoms were now related to abnormal esophageal peristalsis. He continues to have mild dysphagia with solids. CASE 2 A 10-year-old girl, diagnosed with achalasia 1 year earlier, was referred to us after she underwent a laparoscopic Heller myotomy and developed severe dysphagia that was not relieved after 2 esophageal dilatations with bougies. After arrival at our institution, she had a barium study that showed narrowing at the gastroesophageal junction, with almost complete barium retention after 10 minutes. An esophageal manometry showed a 1.5-cm high-pressure zone of 26 mmHg, an esophagogastric pressure gradient of 11 mmHg, and no esophageal body peristalsis. She responded well to PD (30 mm). Over the next few years, she required 2 more PDs (to 30 mm) because of recurrence of the dysphagia, with asymptomatic periods in between dilatations as long as 7 months. Her dysphagia improved after the last dilatation. Repeat barium studies showed easy passage of the barium into the stomach, with no barium retention. Sixteen months later, she is almost completely asymptomatic, with occasional mild dysphagia with solids. CASE 3 A 7½-year-old boy, diagnosed with achalasia when he was 3 years old, initially had a thoracoscopic Heller myotomy. Four years later, because of persistent dysphagia, he underwent a laparoscopic modified Heller myotomy with a Dor fundoplication. He continued to have dysphagia and was unable to retain any food or liquid. He lost 4 kg and required placement of a nasogastric tube for hydration and nutritional rehabilitation. After he was referred to our institution for evaluation, a barium swallow showed absent esophageal emptying after 10 minutes. Esophageal motility showed a 0.5-cm high-pressure zone of 21 mmHg in the distal esophagus, an esophagogastric pressure gradient of 8.9 mmHg, and no esophageal peristalsis. He underwent PD (30 mm) with good response, and was able to be weaned off the nasogastric tube with no more symptoms. Over the next 2 years, he required 2 more PDs (to 30 mm) because of recurrence of the dysphagia. Between dilatations (for periods of up to 9 months), he was able to eat by mouth and was asymptomatic. A few weeks after the last PD, a barium study showed easy passage of the barium into the stomach. Eighteen months after the last dilatation, he describes occasional dysphagia and is able to eat a full diet by mouth without any restrictions. CASE 4 A 15-year-old boy, diagnosed with achalasia at age 7 years, initially underwent 2 PDs with poor response. At age 8, he underwent a laparoscopic Heller myotomy and Thal fundoplication. Because of persistent dysphagia and chest pain after surgery, he underwent 3 PDs separated by approximately 6-month intervals. He did relatively well for approximately 4 years. He then developed recurrence of chest pain, heartburn, and severe dysphagia, and responded to another PD. He remained asymptomatic for 3 years, when he again developed severe dysphagia and chest pain. He then presented at our institution and a barium study showed marked esophageal dilatation and complete obstruction without any passage of contrast into the stomach after 10 minutes. An esophageal manometry showed a 2.5-cm high-pressure zone of 12 mmHg in the distal esophagus, an esophagogastric pressure gradient of 15 mmHg, and no esophageal body peristalsis. He underwent a PD to 30 mm, and experienced a significant improvement in symptoms. The dysphagia disappeared. Eight months later, he developed esophageal spasms. A repeat barium study showed easy passage of the contrast into the stomach, without any barium retention. He then responded well to the administration of calcium channel blockers and amitriptyline. Eighteen months after the last dilatation, he is asymptomatic and has only occasional dysphagia. CASE 5 A 15-year-old girl was diagnosed with achalasia at 13 years old. She was initially treated with botulinum toxin and 2 PDs without response. She then underwent a laparoscopic Heller myotomy and developed severe dysphagia that persisted after bougie dilatations. She was then referred to our institution. A barium study showed abnormal motility of the esophagus, narrowing at the distal esophagus at the site of the fundoplication, and slow passage of the contrast into the stomach, with most of the barium being retained after 10 minutes. An esophageal manometry showed a 1.5-cm-long high-pressure zone of 6 mmHg, an esophagogastric gradient of 3 mmHg, and no peristalsis. She underwent a PD (30 mm) without any change in her symptoms. Two months later, she underwent a takedown of the fundoplication. Since then, her dysphagia has disappeared. She has been aggressively treated with acid blockade with good response. Her last barium swallow showed mild esophageal dilatation, with free-flowing barium into the stomach. Twenty-four months after the surgery, she continues to do well on acid blockade and has had a normal upper endoscopy. DISCUSSION The recent advent of minimally invasive surgery has changed the traditional surgical approach to the Heller myotomy (4–6). Even though experience with this surgical procedure in children is limited, the number of procedures performed is increasing and complications are more common. There is, however, no information about which is the best treatment or approach to the child with postoperative dysphagia after an esophageal myotomy for achalasia. We describe here the evaluation and treatment of 5 pediatric patients with achalasia who presented with persistent dysphagia after minimally invasive surgical myotomy. Only 2 cases (patients 2 and 3) had surgery as primary treatment; the remaining 3 patients underwent myotomy after the failure of other multiple procedures, including PD and application of botulinum toxin. After workup, we found that 4 had persistent residual achalasia and 1 (patient 5) had wrap-induced dysphagia. The main long-term symptoms after surgery for achalasia are dysphagia, heartburn, and chest pain, with dysphagia being the most common and debilitating symptom. The overall incidence of long-term complications after surgery in children is not known, although a compilation of 504 pediatric cases that underwent open modified Heller myotomy (329 without a fundoplication and 175 with a fundoplication) reported dysphagia in 5.2%, gastroesophageal reflux disease in 3.8%, and a reoperation rate of 3.4% (1). No information about the incidence of complications after minimally invasive surgery for achalasia in children has been reported. In adults, mild dysphagia after laparoscopic myotomy without fundoplication occurs in 20%, and severe dysphagia has been reported in 1% to 9% (7). In patients in whom a fundoplication has been added to a laparoscopic myotomy, severe dysphagia has been reported in 10% to 28% (7–9). Possible causes of persistent dysphagia after laparoscopic myotomy include underlying esophageal dysmotility, incomplete myotomy, fibrosis at the distal end of the myotomy, relative obstruction from a fundoplication performed in an aperistaltic esophagus, esophageal stricture, preoperative error in diagnosis, or any combination of the above (8,10,11). Given that treatment and prognosis of the patient with dysphagia after myotomy will vary depending on the etiology, a thorough evaluation is mandatory before any additional therapy is instituted. The evaluation of the postoperative patient with dysphagia must include clinical history (12), radiological imaging (13–15), manometric studies (16,17), and esophagogastroduodenoscopy. Symptom assessment traditionally has been used to judge treatment success. However, neither the severity nor the total number of achalasia-related symptoms correlates well with the severity of radiographic abnormalities (18). Studies have shown that many achalasia patients have an altered vagal afferent response (19,20), with secondary abnormal sensation. This results in poor reliability to detect problems if only the patients' subjective reports are used. It has been reported that symptom relief and objective esophageal emptying are concordant in only about 70% of patients, whereas up to 30% of achalasia patients report near-complete symptom relief despite poor esophageal emptying of barium (21). Therefore, an objective assessment of esophageal function is important in evaluating esophageal emptying after therapy. The timed barium esophagogram is the most common objective method used to judge the effectiveness of achalasia treatment. It is not only useful to judge treatment success shortly after therapy but it also has been shown to predict poor long-term outcome if patients continue to have retained barium after therapy (21). The barium study also is useful to exclude the presence of peptic strictures or other anatomic problems after treatment. Among our 5 patients, the esophagogram showed abnormal emptying in all, suggesting a distal obstruction. After successful therapy, emptying improved and became rapid. Once there is evidence of abnormal esophageal emptying, the possibility of having an incomplete myotomy is only answered by esophageal manometry (Fig. 1). As shown in the present series, this is one of the most common etiologies of dysphagia after myotomy (22). The criteria for a successful manometric response to treatment have not been well defined (23). It has been suggested that a posttherapy LES pressure of <10 mmHg is the single most valuable factor for predicting long-term clinical response (17,24,25). A significant reduction in the esophagogastric pressure gradient to <10 mmHg also has been associated with long-term improvement (26). Residual LES pressure (LESP) after myotomy with fundoplication is more difficult to interpret because it is possible that a persistent high-pressure zone at the level of the LES could be secondary to the presence of the fundoplication and not necessarily to an incomplete myotomy. It has been shown in patients with gastroesophageal reflux disease that a fundoplication may be associated with a postsurgical increase in LES pressure, as well as a decrease in transient LES relaxation frequency and an increased mean residual pressure at the LES during swallowing (27–29). It is more complicated to assess the combined effect that a fundoplication will have on the LESP after esophageal myotomy for achalasia. A recent study showed that after a laparoscopic Heller myotomy without a fundoplication, the LES was shorter and had a lower pressure compared with those that underwent both a myotomy and a fundoplication (30). However, those differences were not statistically significant. In another study of patients who underwent laparoscopic Heller myotomy and Dor fundoplication (31), it was reported that the postoperative LES resting pressure decreased invariably below 10 mmHg, suggesting again that successfully treated patients with achalasia have a LESP of <10 mmHg after surgery (31).FIG. 1: Suggested algorithm for evaluation of the child with persistent dysphagia after surgical myotomy for achalasia.Postoperative dysphagia also may persist because of the occurrence of fibrosis at the site of the surgery, a diagnosis that is difficult to make clinically or manometrically (8,10,11). Usually the barium study will show delayed emptying in a patient with a low LESP, and the dysphagia tends to appear some time after surgery. Lack of esophageal peristalsis may contribute to the presence of dysphagia after a successful myotomy (32). This lack of peristalsis explains why dysphagia is so common even after successful treatment for achalasia (such as case 1 after final treatment) (32). This lack of peristalsis may be responsible for severe obstructive symptoms in patients with a successful myotomy, particularly if they have undergone a fundoplication (such as in case 5). In our series, the workup indicated persistent residual achalasia in 4 patients (cases 1–4; LESP > 10 mmHg) and wrap-induced dysphagia in 1 patient (case 5; LESP of 6 mmHg). Three of the patients had had pneumatic dilatations before the surgery, raising the possibility that the performance of PD before myotomy may affect outcome adversely, as has been reported recently (33,34). This is still controversial (35–39) because most patients after PD have a successful outcome (35). To establish an association between previous PD and poor outcome after myotomy will require large prospective multicenter studies. Other attempts to decrease the incidence of complications after myotomy have included the use of intraoperative esophageal manometry because it has been shown that it may identify high residual LES pressure before the end of surgery (22), or may avoid the performance of a fundoplication by determining the correct length of the myotomy (40). Both indications for intraoperative manometry are controversial (35,39). The routine performance of intraoperative manometry is not indicated at this point, given that esophageal function is profoundly affected by general anesthesia and positive pressure ventilation (41,42), the exact LES pressure and length of the myotomy that need to be achieved is not known (35,39), and there are no randomized trials that have shown that the performance of an intraoperative esophageal manometry improves outcome or avoids the need for fundoplication (35,39). The appropriate management of children with dysphagia after minimally invasive surgery for achalasia is not clear, and may vary according to the etiology (8,43–45). The recommended initial treatment of choice for an incomplete myotomy is pneumatic dilatation (46), which has been shown to be an effective procedure in nearly 80% of patients. There may be a theoretical increased risk of perforation given the previous myotomy, but most studies in adults show that the dilatation is safe, as long as it is performed a few months after surgery (46). Pneumatic dilatation also is effective in patients that have had a myotomy with fundoplication (47). In 1 report, 5 of 6 adult patients responded well to dilatation and only 1 required repeat myotomy (47). A repeat myotomy needs to be considered only when pneumatic dilatations have failed to relieve the obstruction in the distal esophagus (44,48). In our series, all 5 patients underwent multiple safe pneumatic dilatations after myotomy. Dilatations were effective in 4, although most required repeated treatments; none of the patients required a repeat myotomy. Unfortunately, there is no specific treatment for dysphagia that persists after a successful myotomy in patients with a lack of esophageal peristalsis (32), as in case 1, in which PD was successful after the myotomy. However, if the dysphagia is secondary to obstruction from the fundoplication in an aperistaltic esophagus (as in case 5), without evidence of high pressure in the distal esophagus, a takedown of the fundoplication may be necessary (44,48). Based on the above considerations and observations, we suggest an algorithm for the evaluation (Fig. 1) and treatment (Fig. 2) of the child with dysphagia after minimally invasive surgery for achalasia. The initial step should be a barium study. Repeat manometry usually is not necessary after therapy, provided that the symptoms disappear (Fig. 1). In case of worsening dilatation of the esophagus, lack of esophageal emptying, persistent complaints, or recurrence of symptoms, an esophageal manometry is indicated to establish LES pressure, the esophagogastric gradient, and the state of esophageal peristalsis (1,33,35) (Fig. 1). Depending on the results of the barium study and the esophageal manometry, a pneumatic dilatation is the recommended first step in treatment (Fig. 2) in patients with suspected residual achalasia, fibrosis, or abnormal esophageal emptying. PD is safe and effective, although multiple procedures may be necessary. Surgical treatment may be necessary for those with residual high pressure in the distal esophagus who have not responded to dilatation, if there is fibrosis that is not responding to therapy, or if there is a significant obstruction of a fundoplication in an aperistaltic esophagus.FIG. 2: Suggested algorithm for treatment of the child with achalasia with postsurgical persistent dysphagia. LES = lower esophageal sphincter; EGD = esophagogastroduodenoscopy." @default.
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- W2088832157 title "Approach to the Child Who Has Persistent Dysphagia After Surgical Treatment for Esophageal Achalasia" @default.
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