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Query: UMLS:C0014848 (achalasia)
 2,804 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Achalasia is a rare disorder of the esophagus in children. From 1971 to 1999, 20 children with achalasia of the esophagus have been treated at our institution including two patients who were referred to us after esophagomyotomy. There were 13 boys and 7 girls (average age, 8.2 years; range 2 to 15 years). Presenting symptoms were vomiting (n = 18), dysphagia (n = 11), loss of weight (n = 5), recurrent respiratory infections (n = 3), cough (n = 2) and noisy respiration (n = 1). Barium swallow established diagnosis in all patients. Esophagoscopy was used as a supportive investigation in some patients (n = 10). Nineteen patients underwent Heller-Zaiger operation (modified Heller esophagomyotomy) either by transabdominal (n = 16) or transthoracic approach (n = 3) with (n = 6) or without concomitant antireflux procedure. The postoperative period was uneventful in all patients. Follow-up ranged from 2 months to 16 years. Decreased or absent peristalsis persisted in initial control esophagograms in all patients. Gastroesophageal reflux was encountered in only one patient. Complete relief of symptoms was noted in 14 patients. Mild to moderate dysphagia was encountered in 5 patients and all of them were evaluated by endoscopy and upper gastrointestinal series. Dysphagia resolved spontaneously in one child and following two dilations in another child. One child has moderate dysphagia after a short follow-up period. Esophageal stenosis was seen in the remaining two and subsequently treated by esophagocardioplasty (Heyrowsky and Wendel operations). Achalasia should be considered in the differential diagnosis in any children with persistent dysphagia, recurrent respiratory tract infections and vomiting, including children treated for clinically suspected gastroesophageal reflux. The obvious mode of treatment is surgical myotomy in children. Modified Heller esophagomyotomy is the procedure of choice, which can be performed either by an abdominal or a thoracic approach. The need to carry out a concomitant antireflux procedure remains controversial. The most frequent postoperative problem is persistent dysphagia. It may be self-limited in some cases and disappear during follow-up. Resistant stenosis following esophagocardiomyotomy can be treated by esophagocardioplasty procedures such as Heyrowsky and Wendel operations.
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PMID:Achalasia in childhood: surgical treatment and outcome. 1155 10

Treatment of achalasia by pneumatic balloon dilatation (PBD) is well established in adults. Due to limited experience and the rarity of the condition in children, there are relatively few reports in the paediatric literature. Although PBD has been reported as a primary method of treatment, there are no reports of secondary PBD for childhood achalasia. Between 1995 and 1999, five patients underwent treatment for achalasia (age: 9-14 years, M:F = 4:1). The presenting symptoms were dysphagia (5). vomiting episodes (2), aspiration (1), food-bolus obstruction (1), and failure to thrive (1). In all patients a barium swallow and manometry were used to confirm the diagnosis. Three underwent primary PBD. Two who had previously undergone surgical myotomy underwent secondary PBD for recurrence of symptoms. Dilatation was performed using a 35-mm balloon with the child under general anaesthesia. Technical success was defined as demonstration of a waist under screening at lower pressures followed by abolition of the waist at higher pressures. In addition to reviewing our results, a systematic review of the literature was performed (Medline, Cochrane Library, Pubmed, Embase). Three patients (primary dilatation) showed excellent improvement after a single dilatation. In two cases (secondary dilatation) three and five attempts were required. No complications were encountered. The mean follow-up period was 2 years (1-3.5 years) and four patients remained asymptomatic, an overall success rate of 80%. The literature review revealed similar good results in most of the recent reports. Thus, PBD as a primary treatment for childhood achalasia has a success rate of 70%-90% with minimal side effects, short hospital stay, and good patient acceptability over an operation. We have also established the usefulness of this method as a secondary treatment when symptoms recur after surgery.
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PMID:Pneumatic dilatation for childhood achalasia. 1166 45

Achalasia is rare in children, more so familial. We report two siblings with familial achalasia who presented in their infancy with vomiting and failure to thrive. Achalasia can be misdiagnosed as upper gastrointestinal obstruction as happened in one of our siblings. Esophageal contrast roentgenography is diagnostic. Both the children were treated successfully by transabdominal esophagomyotomy with fundoplication.
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PMID:Achalasia in siblings in infancy. 1166 40

To many nonsurgeons myotomy is considered an excessively invasive treatment for achalasia and has become a salvage procedure when esophageal dilation and botulinum toxin (botox) injections fail. We sought to examine our experience with videoscopic Heller myotomy to determine whether preoperative therapy predicts perioperative complications and long-term outcome. Videoscopic Heller myotomy was undertaken in 111 patients with achalasia between June 1992 and May 2000. Intraoperative endoscopy was used in all patients. Fundoplication was used selectively for patients with large hiatal hernias or as part of repair of esophageal perforation. Patients were asked to grade their dysphagia and reflux symptoms before and after myotomy on a scale of 0 (no symptoms) to 5 (severe symptoms). Patients were also asked to rate their outcome as excellent (no symptoms), good (greatly improved), fair (slightly improved), or poor (not improved) compared with their preoperative status. Patients were stratified on the basis of preoperative intervention (botox, pneumatic dilation, botox and pneumatic dilation, or no botox or dilation) and compared. Previous pneumatic dilation and/or botox injection had been undertaken before operation in 88 (79%) patients whereas 23 (21%) patients had no invasive preoperative therapy. The overall mean preoperative dysphagia score was 4.8+/-0.8 and mean preoperative reflux score was 3.3+/-2.1. Groups of patients undergoing preoperative interventions were similar to those patients not undergoing preoperative interventions in terms of preoperative symptoms, dysphagia scores, and reflux scores. Postoperative complications (13%) and perforations (8%) were slightly more common in patients who had undergone preoperative botox or dilation (P = not significant). Subjectively, operative myotomy was more difficult in patients who had preoperative botox or dilation. Patients had significant improvement in dysphagia, dysphagia score, reflux score, emesis/ regurgitation, and chest pain (P < 0.05) regardless of preoperative intervention. After myotomy patients who had never undergone botox or pneumatic dilation were less likely to have mild dysphagia compared with those with previous botox injections (30% vs 53%; P = 0.09), previous dilations (30% vs 54%; P = 0.09), or both (30% vs 59%; P = 0.04). As well, dysphagia scores were better if no preoperative therapy had been undertaken: botox 0.8+/-1.3, dilation 1.0+/-1.4, botox and dilation 1.0+/-1.3, and no therapy 0.3+/-0.7 (P < 0.05). Overall 97 per cent of patients stated that their symptoms were improved although more patients tended to have excellent or good outcomes if no preoperative intervention was undertaken (91%) compared with patients undergoing preoperative botox (86%), dilation (83%), or both (82%) (P = not significant). We conclude that videoscopic Heller myotomy is safe and efficacious particularly in patients who have not undergone previous endoscopic interventions. The difference in patients' outcomes based on preoperative therapy may be related to a less difficult operation in patients who forgo endoscopic therapy and elect to undergo early myotomy. Although videoscopic Heller myotomy provides good outcomes as a salvage procedure after failed dilations and/or botox injections for achalasia we advocate it as first-line therapy in reasonable operative candidates.
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PMID:Videoscopic heller myotomy as first-line therapy for severe achalasia. 1173 Feb 31

Disorders of the oesophagus present a diagnostic and therapeutic challenge. The presenting symptoms of dysphagia, reflux, pain and vomiting are almost universal, irrespective of the underlying pathology. A combination of endoscopy, barium studies, pH studies and manometry are often required to determine the exact diagnosis and to plan the most effective treatment. Paraoesophageal hiatal hernia is an uncommon condition, present in 14% of all hiatal hernias, which requires urgent correction to prevent life-threatening complications. It is unusual for other oesophageal disorders to coexist. We present a case where achalasia and a paraoesophageal hiatal hernia probably coexisted.
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PMID:Coexisting achalasia and paraoesophageal hiatal hernia. 1178 83

Triple-A syndrome is a very rare pathology in childhood, consisting of achalasia, alacrima and adrenal insufficiency. The order of manifestation of these three main symptoms is variable. Here we present a case of Triple-A syndrome with recurrent vomiting caused by achalasia as first symptom. At the same time, alacrima was diagnosed. Several years later adrenal insufficiency occurred and substitution therapy with hydrocortisone was started. The follow-up of patients with achalasia should pay attention to symptoms of adrenal insufficiency.
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PMID:Recurrent vomiting as first symptom of triple-a syndrome--a case report. 1180 74

We report an unusual hepatoid adenocarcinoma in Barrett's esophagus with achalasia, which developed in a 44-year-old Japanese woman. The patient received an esophago-gastrectomy after diagnosis of the tumor and achalasia at the lower esophagus, 4 months before her death due to multiple metastatic tumors of the liver. The main granular tumor removed surgically was a hepatoid adenocarcinoma, mainly composed of clear cancer cells (alpha-1 antitrypsin, albumin and alpha-fetoprotein positive), with elements of choriocarcinoma and tubular adenocarcinoma. Non-neoplastic specialized columnar epithelium was present extensively near the oral side of the tumor edge in the esophagus, indicating Barrett's esophagus. This unusual tumor was therefore considered to have originated in Barrett's esophagus. The gastroesophageal reflux was presumed to have occurred for a long period, as there was a well-preserved fundic gland in the stomach and a history of frequent vomiting from the patient's youth, accounting for the appearance of achalasia.
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PMID:Hepatoid adenocarcinoma in Barrett's esophagus associated with achalasia: first case report. 1194 Feb 19

We reviewed our experience in the diagnosis and management of esophageal achalasia in 33 children over a 25-year period at a single center by a retrospective chart review of all patients diagnosed with achalasia between December 1, 1975 and January 30, 2001. There were 33 cases ranging from 5 months to 16 years of age at the time of presentation (17 boys and 16 girls). Although dysphagia and vomiting were the commonest presenting symptoms, weight loss, chest pain, coughing, and recurrent pneumonia also occurred in many patients. Barium contrast study of the esophagus was the initial diagnostic modality followed by esophageal manometry. An upper endoscopy was also performed in 78.7% of cases. Management was predominantly surgical; however, seven recently diagnosed patients opted for botulinum toxin (botox) injection as the first line of treatment. The follow-up duration varied from 10 months to 10 years (mean 4.71 +/- 3.2 years). Postsurgical complications included gastroesophageal reflux disease in five patients who had not received a simultaneous antireflux procedure and "residual achalasia" in two patients, who both responded to a single botox injection.
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PMID:A review of achalasia in 33 children. 1245 92

A 9-year-old girl presents for continuing weight loss of 10 kg over the course of 1 year. Medical history showed three episodes of pneumonia requiring hospital admission in the 6 months before presentation and 4 months of weekly psychotherapy for anorexia nervosa. A thorough history of eating behavior and a review of systems revealed not only typical aspects of prepubertal anorexia nervosa but also vomiting at night while asleep, difficulty drinking liquids, epigastric pain, and a frequent experience of "a lump in the throat"; these symptoms were not suggestive of a diagnosis of anorexia nervosa but rather of esophageal achalasia. The patient was transferred to the Department of Pediatrics, and a diagnosis of esophageal achalasia was made by chest x-ray and barium swallow. After dilatation and botulinum toxin application, the patient regained weight easily and was discharged in stable condition. In this case, esophageal achalasia mimicked prepubertal anorexia nervosa.
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PMID:Achalasia mimicking prepubertal anorexia nervosa. 1265 33

Cholangiocarcinoma most commonly presents as painless progressive jaundice. We report a case occurring in a 56-year-old Chinese woman with an unusual presentation of progressive dysphagia and vomiting. Oesophageal manometric and barium studies were indicative of achalasia, and computed tomography confirmed the presence of cholangiocarcinoma extending to the gastroesophageal junction and proximal lesser curve of the stomach. In this case, a constricting tumour at the gastroesophageal junction with probable invasion of the vagus nerves led to features of achalasia and gastroparesis.
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PMID:Cholangiocarcinoma presenting as pseudoachalasia and gastroparesis. 1290 20


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