Childhood Achalasia Research Articles

Early myotomy and fundoplication in achalasia in childhood: a single-centre experience for 22 years

Introduction The aim of this study was to review a single institution’s experience with surgical interventions in children with achalasia and to determine treatment strategies for this rare disorder.Patients and methods This study is a retrospective analysis of 22 cases of childhood achalasia from 1991 to 2013. The patients were evaluated in terms of age, symptoms, interventions, intraoperative complications, and recurrent dysphagia.Results There were 13 boys and nine girls (7 months to 17 years old). The clinical symptoms were vomiting (68%), dysphagia (36%), wheezing (18%), coughing (13%), and weight-loss (13%). The mean duration of symptoms was 2.4 years (1 month to 6 years). A barium contrast X-ray study was performed in all of the patients. Oesophageal manometry was performed in eight patients. Six patients underwent multiple oesophageal dilatations (ED) as a first intervention. A Heller myotomy (HM) and fundoplication were performed in all the patients except two patients who recovered with dilatation. In the long term, one patient had a stricture due to the operation and had to undergo a reoperation. Of the Heller myotomy patients, one had a recurrent stricture that responded to dilatation. No other complications were present. All the patients are now asymptomatic.Conclusion Early diagnosis and prompt surgical treatment is important to prevent growth impairment in childhood achalasia cases. A Heller myotomy followed by a partial anti-reflux procedure is an effective treatment for achalasia in children. Based on our experience, it is superior to oesophageal dilatation therapy.

Achalasia Masking as Mild Intermittent Asthma: Acute Respiratory Failure, Aspiration Pneumonia, and Pneumothorax in a Teenage Girl

SESSION TITLE: Pediatrics and Congenital Cases SESSION TYPE: Affiliate Case Report Slide PRESENTED ON: Tuesday, October 27, 2015 at 07:30 AM - 08:30 AM INTRODUCTION: Primary achalasia is a rare esophageal motility disorder caused by failure of the lower esophageal sphincter to relax. Childhood achalasia is often misdiagnosed as gastroesophageal reflux, failure to thrive, eating disorder or asthma. CASE PRESENTATION: A 16-year-old girl presented with three days of vomiting, abdominal pain, and fever. She was diagnosed 3 years earlier with asthma and pneumonia, and had similar abdominal pain 6 months ago. For the past year, she sometimes felt short of breath swallowing solids. Initial vital signs were T 105.9°F, HR 176 bpm, RR 40/min, BP 74/54 mmHg, and O2 saturation 59%. After intubation for severe respiratory distress, copious thick yellow tracheal secretions grew E. coli. Labs showed leukocytosis, bandemia and elevated inflammatory markers. CXR showed bilateral infiltrates and widened mediastinum (Image 1). A follow-up CXR revealed a medial right air bubble, and a chest tube was placed. As the air bubble did not resolve, CT chest obtained showed severe esophageal distension, tracheal compression, basilar consolidations, and bronchiectasis (Image 2). She was diagnosed with achalasia with aspiration pneumonitis when a barium swallow demonstrated complete esophageal dilatation and absent peristalsis. After laparoscopic Heller's myotomy with partial fundoplication, she improved dramatically and went home with anti-reflux medicines and inhaled corticosteroids. DISCUSSION: With an incidence of 1.6/100,000, childhood achalasia presents with progressive dysphagia first to solids and then liquids. Diagnosis is delayed on average by five years. Older children may complain of reflux, sore throat, hoarseness, choking, or dypsnea, and develop food avoidance behaviors. Younger children and infants may have feeding problems and respiratory symptoms such as chronic or nocturnal cough, recurrent respiratory infections, wheezing, stridor, or acute upper airway obstruction. Preferred treatment is laparoscopic Heller's myotomy. Lung complications from chronic aspiration include obliterative bronchiolitis, abscess, empyema and fibrosis. CONCLUSIONS: Childhood achalasia should be on the differential when suspecting asthma or gastroesophageal reflux. Careful history taking is paramount. Mediastinal widening is present in achalasia but not asthma. Achalasia often results in lung injury due to chronic aspiration. Reference #1: Franklin AL, et al. Childhood achalasia: A comprehensive review of disease, diagnosis and therapeutic management. World J Gastrointest Endosc. 2014 Apr 16;6(4):105-11. DISCLOSURE: The following authors have nothing to disclose: Khanh Lai, Mini Mehra, Eliezer Nussbaum No Product/Research Disclosure Information

G446(P) An Unusual cause of Chronic Cough in a 15 year old girl

AimsCoughing is one of the most common paediatric presentations both in primary and secondary care. We present an unusual cause of chronic cough in a teenager.MethodA 15 year old girl...

Childhood achalasia: A comprehensive review of disease, diagnosis and therapeutic management.

Achalasia is an esophageal motility disorder characterized by failure of lower esophageal sphincter (LES) relaxation and is rare in children. The most common symptoms are vomiting, dysphagia, regurgitation, and weight loss. Definitive diagnosis is made with barium swallow study and esophageal manometry. In adults, endoscopic biopsy is recommended to exclude malignancy however; it is not as often indicated in children. Medical management often fails resulting in recurrent symptoms and the ultimate definitive treatment is surgical. Laparoscopic Heller myotomy with or without an anti-reflux procedure is the treatment of choice and has become standard of care for children with achalasia. Peroral endoscopic myotomy is a novel therapy utilized with increasing frequency for achalasia treatment in adults. More experience is needed to determine the safety, efficacy, and feasibility of peroral endoscopic myotomy in children.

Autism and esophageal achalasia in childhood: a possible correlation? Report on three cases

Chronic gastrointestinal symptoms are commonly reported in autistic patients. Dysphagia is often present, and it is generally related to behavioral eating disorders. The association between autism and esophageal achalasia has not been described in literature yet. We report our experience with three cases of autistic children we recently treated for esophageal achalasia. In the first case (a 14-year-old male), achalasia was diagnosed with barium swallow and esophageal manometry and was successfully treated with three pneumatic endoscopic dilatations (follow-up: 3 years). In the second case (a 12-year-old female), achalasia was diagnosed with barium swallow and esophageal manometry and was treated with Heller myotomy after two unsuccessful pneumatic endoscopic attempts (follow-up: 3 months). In the last case, a 15-year-old male underwent barium swallow and endoscopy that confirmed achalasia. He was treated with Heller myotomy, and he is asymptomatic at a 6-month follow-up. To our knowledge, this is the first report of a possible association between autism and esophageal achalasia. Because of the rarity of both diseases, their association in the same patient is unlikely to be casual even if speculation on their common etiology is impossible at present. This finding needs further confirmation, but it is sufficient, in our opinion, to indicate proper evaluation with barium swallow and/or manometry in any autistic children with eating difficulty.

Autism and Esophageal Achalasia in Childhood: A Possible Correlation? Report on Two Cases

Autism and Esophageal Achalasia in Childhood: A Possible Correlation? Report on Two Cases

Outcomes of treatment of childhood achalasia

Purpose The optimal management of achalasia in children and adolescents remains unclear. The aim of this study was to review a single institution's experience with endoscopic and surgical interventions in children with achalasia. Methods A retrospective review was conducted of the medical records of children treated for achalasia from 1978 to 2008. Patient demographics and interventions were reviewed. Outcomes after procedural intervention were evaluated. Results Thirty-five patients with achalasia were identified, and data were available for 34 (age, 13 ± 6 years; male, 62%). Eighteen patients underwent esophageal dilation (ED), and 16 patients underwent Heller myotomy (HM). Follow-up was available for 30 patients (ED, 15; HM, 15). There was symptom recurrence in 15 of 15 ED cases and 8 (53%) of 15 HM cases ( P < .01). Additional interventions were performed in 14 (93%) of 15 ED cases and 6 (40%) of 15 HM cases ( P < .01). Conclusions Heller myotomy may provide more durable long-term outcomes, as defined by symptom recurrence and need for subsequent intervention, and may be considered the procedure of choice.

Triple A syndrome mimicking ALS

We report a 22-year-old female who presented with distal muscular atrophy and weakness in all limbs for two years. Reflexes were symmetrically brisk and electrodiagnostic studies were consistent with upper and lower motor neuron involvement. A diagnosis of juvenile ALS was considered. However, surgery for achalasia in childhood and identification of alacrima and adrenal insufficiency suggested Triple A syndrome accompanied by neurological symptoms. Sequencing of the AAAS gene identified compound heterozygous mutations confirming the clinical diagnosis and demonstrating that Triple A syndrome can mimic juvenile ALS.

Intraoperative manometry during laparoscopic Heller myotomy improves outcome in pediatric achalasia

Achalasia is a rare disorder with less than 5% of patients diagnosed in childhood. Although Heller esophagocardiomyotomy is a proven intervention, incomplete myotomy can lead to clinical failure. Intraoperative esophageal manometry has been used to ensure adequacy of myotomies in adults. The purpose of the present study was to review our experience in the management of children with achalasia. A retrospective review was conducted on the medical records of patients with achalasia diagnosed between November 1999 and March 2007. Patient demographics and interventions were recorded. Outcomes after surgical intervention and esophageal dilation were assessed. Mean follow-up was 3.5 +/- 0.6 years. Intraoperative manometry was used over the past 3 years. Nineteen patients were treated for achalasia. The average age at diagnosis was 13.8 +/- 0.8 years. Most patients underwent esophageal dilation (14/19), receiving on average 2.1 +/- 0.3 dilations. One patient experienced a contained perforation that was treated conservatively. Eleven patients underwent myotomy, as primary therapy (n = 5) or after recurrence of symptoms after dilation (n = 6). Six patients underwent intraoperative manometry. More patients who underwent Heller myotomy without intraoperative manometry had recurrence of symptoms (80% vs 0%, P < .05). Inadequate myotomy is a potential cause for recurrent symptoms after esophagocardiomyotomy in childhood achalasia. Intraoperative esophageal manometry is a safe technique that may improve the success rate of surgery by confirming the adequacy of myotomy thereby decreasing recurrence of symptoms.

Achalasie im Kindesalter: Eine separate Entität?

Achalasia in childhood is rare, also the etiology and the pathogenesis of the early onset ort he disease is practically unknown. Little is known about the neuropathological changes in structure of the esophageal wall in non-hereditary, sporadic achalasia in children and ist differentiation to that in adults. The aim of our study was to examine the morphological properties or high-pressure zone of the lower esophageal sphincter in children who had undergone a Heller myotomy because of achalasia as well as to compare them with the pathological findings in adults. Muscle biopsies of the smooth musculature, a 20 x 10 mm long segment of the myenteric of the distal esophagus (lower boundary of the esophageal incision of the myotomy), were taken for histopathological and immunohistochemical studies. A conspicuous histomorphological finding was a marked reduction of the myenteric ganglion through to complete aganglionosis of the high-pressure zone of the lower esophageal sphincter. In contrast to achalasia in adults, neural inflammation was found only rarely. A pronounced fibrosis of the smooth muscle layers was found in all patients whereas muscular hypertrophy or visceral myopathy was not present. The interstitial Cajal cells were reduced, similar to those in adults. The variability of the clinical and pathological properties in cases of childhood achalasia are indicative of a complex pattern of varying etiologies and a comparison with the disease in adults does not, in principle, allow the assumption of a separate clinical entity. The present findings are compatible with the histopathological results of hereditary achalasia in children as described for Allgrove's syndrome.

Pneumatic dilation in treatment of late-onset primary gastric outlet obstruction in childhood

Late-onset primary gastric outlet obstruction in childhood is a rare condition. Only 8 cases of such were reported. Diagnosis should be considered while ruling out mechanical and structural lesions. Up to now, Heineke-Mikulicz pyloroplasty has been the standard treatment. However, we succeeded in treating this condition by using pneumatic dilation. There is no sign of recurrence for 1 year. We propose the etiology, diagnosis, and the relationship between late-onset primary gastric outlet obstruction and esophageal achalasia in childhood.

Motility disorders in childhood: Working Group Report of the First World Congress of Pediatric Gastroenterology, Hepatology, and Nutrition.

Motility disorders in childhood: Working Group Report of the First World Congress of Pediatric Gastroenterology, Hepatology, and Nutrition.

Childhood achalasia in Zaria, Nigeria

To study the presentation, management and outcome of achalasia of the oesophagus in children. A retrospective case study. Ahmadu Bello University Teaching Hospital, Zaria, Nigeria. Seven children managed for achalasia of the oesophagus in a period of 19 years. There were six boys and one girl. The median age at presentation was ten years (range three months to fifteen years). The median duration of symptoms before presentation was 20 months (range: 2-24 months). Three children were treated on several occasions for pneumonia before the diagnosis was made. Dysphagia, vomiting and regurgitation were the main presenting symptoms. Diagnosis was by barium oesophagogram. Six had modified Heller's myotomy via celiotomy and a Thal fundoplication in a three month old. Transthoracic Heller's myotomy was done in the seventh child. There was complete resolution of symptoms in six children. One patient had post-operative retrosternal pain, which was controlled by oral nifedipine. Achalasia of the oesophagus should be excluded in children with recurrent chest infection. Modified Heller's myotomy without antireflux procedure via the abdominal route is effective in relieving symptoms of achalasia in children.

Pneumatic dilatation for childhood achalasia.

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.

Achalasia in childhood: surgical treatment and outcome.

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.

Upper airway obstruction as a presenting sign of achalasia in childhood

Upper airway obstruction as a presenting sign of achalasia in childhood

Upper airway obstruction as a presenting sign of achalasia in childhood

Upper airway obstruction as a presenting sign of achalasia in childhood

Tandem balloon dilatation for childhood achalasia.

There are no previous reports of tandem balloon dilatation in childhood achalasia. To report the treatment of four cases of paediatric achalasia using tandem balloon dilatation of the lower oesophageal sphincter. A review of the literature since 1986 was undertaken to compare outcomes of balloon dilatation and surgery. A retrospective review of the patients diagnosed with this condition and treated at our institution over the past 6 years: all four patients were treated by balloon dilatation of the lower oesophageal sphincter using two or three balloons in tandem. The definition of technical success was demonstration of a waist at 1-1.5 atmospheres of inflation pressure followed by abolition of the waist at higher pressures. Where this was unable to be achieved using a single balloon, two or three balloons in tandem were used. No patient required oesophagomyotomy, and symptomatic control has been good to excellent in three of four patients. No significant side effects were encountered. Balloon dilatation and surgery have similar success rates in paediatric achalasia. Because of the low morbidity associated with balloon dilatation, the procedure should be considered as first line treatment of this condition. If the lower oesophageal sphincter is stretched insufficiently using a single balloon, tandem balloon dilatation should be utilised.

Radionuclide esophageal emptying and long-acting nitrates (Nitroderm) in childhood achalasia.

Radionuclide esophageal emptying and long-acting nitrates (Nitroderm) in childhood achalasia.

Achalasia of the cardia in children: A worldwide survey

This report summarizes a worldwide survey of achalasia in childhood. The data were obtained by circulating a questionnaire to pediatric surgeons thoughout the world. Information was received regarding 175 patients, and the analysis is presented. Childhood achalasia appears to be more common in boys, and familial cases are rare. Regurgitation of food and dysphagia are the most frequent symptoms in affected children. Although 18% of patients had symptom onset during infancy, only 6% of the patients were identified as having achalasia during infancy. The survey suggests only a fair clinical response to transthoracic esophagocardiomyotomy and a good to excellent clinical response to transabdominal esophagocardiomyotomy. A cooperative effort between several institutions will permit the development of prospective treatment protocols for children with achalasia.