Cardiac Achalasia in Children. Dilatation or Surgery?

Abstract

Surgical treatment of cardiac achalasia in children is still the main line of treatment with a success rate of 70-80%. Balloon dilatation is less widely used due to inappropriate size of balloons. The authors report on their experience in 11 children with cardiac achalasia over the last 7 years using balloon dilatation as the treatment of choice, 8 boys and 3 girls with ages ranging from 1.5-14 years (average 7.5 years) were investigated. One family (brother and sister) presented with no glucocorticoid deficiency or other anomalies, one patient had mental retardation, the rest had no associated anomalies. All patients presented with vomiting, 7 with dysphagia, 6 with loss of weight, 5 with recurrent chest infection and 2 with retrosternal pain. Radiological diagnosis was accurate in all patients, endoscopy with biopsy were done to confirm diagnosis and exclude other pathology, manometry yielded positive results in 4 patients. Dilatation was done under general anesthesia with fluoroscopic control, balloons were used over a guide wire (balloon sizes were 18-35 mm). Seven patients had 2 sessions and 4 had 3 sessions with radiological follow-up after the second dilatation. Follow-up ranged from 2-7 years: excellent results were achieved in 8 patients (72.7%) with disappearance of symptoms and marked radiologic improvement, 2 still have mild symptoms with overall success (90.9%), one had mild gastroesophageal reflux, controlled medically, and one had mild dysphagia but his status was improved compared to that before dilatation. One patient had recurrent dysphagia necessitating cardiomyotomy (9.1%). Results were not related to age or sex. The authors recommend balloon dilatation in children with cardiac achalasia as the treatment of choice or even as the only feasible treatment.

Acalasia de esôfago de causa desconhecida na infância

“…In the dilated section, tertiary contractions of the esophageal body are found together with an air-fluid level, causing the observed difficulties in the passage of the contrast from the esophagus to the stomach (Figure 1), in common with reports in the literature. [2][3][4][5][6] During endoscopic examination the endoscope encountered resistance to its progress, [1][2][3]6 also in agreement with published literature. This examination is of extreme importance to confirm the presence of esophagitis and other types of injury present with GERD, such as esophageal stenosis.…”

“…[2][3][4][5] The main theories that have been developed to explain the onset of this disease are: 1) a primary neurogenic abnormality with a failure of the inhibitory nerves and progressive degeneration of ganglion cells; 2) an acquired deficiency of the myenteric plexus ganglion cells, secondary to GERD, Chagas disease, or viral process. 1,4 Clinical presentation varies from regurgitation of ingested food, described as vomiting immediately after meals, (associated or not with stasis, a deficit in pondero-statural growth or weight loss, repeated aspiration pneumonia, and, in older children, complaints of dysphagia. [1][2][3]6,7 Because of these symptoms and because it is related to GERD, diagnoses of achalasia of the esophagus are often underestimated.…”

“…1,4 Clinical presentation varies from regurgitation of ingested food, described as vomiting immediately after meals, (associated or not with stasis, a deficit in pondero-statural growth or weight loss, repeated aspiration pneumonia, and, in older children, complaints of dysphagia. [1][2][3]6,7 Because of these symptoms and because it is related to GERD, diagnoses of achalasia of the esophagus are often underestimated. With respect of the present case we observed regurgitation and weight loss.…”

“…1 Achalasia involves a failure of the lower esophageal sphincter (LES) in conjunction with reduced motility in esophagus itself. The passage of food via the esophagogastric junction is extremely difficult without true organic stenosis or extrinsic compression.…”

Per Oral Endoscopic Myotomy in Children with Achalasia Cardia

“…PBD has a reported success between 70-90% in published case series. 13,14 Many experts prefer PBD as initial therapy for older children and adolescents with achalasia. The success rate after a single dilatation was only 67% in a recent study.…”