Pneumatic Balloon Dilatation for Achalasia Cardia; Early & late results, a single center study.

OBJECTIVE: Achalasia Cardia is treated by Pneumatic balloon dilatation, Heller's Myotomy and recently, by Peroral Esophagaeal Myotomy. This study reports the efficacy of pneumatic balloon dilatation as a non-surgical motility in achieving relief of dysphagia, clinical improvement and recurrence. Long-term complications were reported. METHODS: Eight hundred ninety two adult achalasia patients of both genders were treated from January 1988 till December 2011, with pneumatic balloon (Rigiflex Microvasive®) dilatation, under fluoroscopy Barium swallow was obtained prior to and five minutes after dilatation to evaluate for efficacy of dilatation as well as for complications. Patients not responding to 30 mm balloon had repeat dilatation with 35 mm balloon after 8 weeks. All patients were enrolled in regular follow up at one, six months and yearly intervals up to a period of five years. Recurrence was defined as an increase in symptom score at 8 weeks greater than 50% of their baseline value. These patients were treated with 35 mm balloon or referred for surgical intervention. RESULTS: Of 892 patients, follow up was obtained in 50% for 5 years, 9.2% for 4-years), 9.3% for 3-years, 10% for 2-years and 21.5% for 1-year of patients. One patient died after repeat dilatation. Eighty-eight patients were excluded from this analysis (20 died due to non-procedure related causes and another 68 were lost during follow up). Statistically significant improvement was noted in reduction in height and width of barium column and symptom score coupled with weight gain during follow up. Forty-eight patients were subjected to repeat dilatation with 35 mm balloon, two of these developed post-procedure perforations with one mortality. Three non-responsive patients required surgical laparoscopic myotomy. No carcinoma of esophagus was reported during follow up. One patient post dilatation, developed esophageal bezoar. A single pneumatic dilatation achieved a remission rate of 93% at four years, 90% at three years, 95% at two years and 92% at one year post dilatation. CONCLUSION: Achalasia of esophagus can be effectively and safely treated with balloon dilatation to achieve adequate short and long-term symptomatic relief with a low complication rate.

AAA Syndrome, Case Report of a Rare Disease.

Triple A (Allgrove) syndrome, an autosomal recessive disease is characterized by achalasia, alacrimia and ACTH-resistant adrenal failure with progressive neurological syndrome including central, peripheral and autonomic nervous system impairment, and mild mental retardation. The triple A syndrome gene, designated AAAS, localized on chromosome 12q 13 encodes for a 546 amino acid protein called ALADIN (Alacrimia-Achlasia-Adrenal Insufficiency and Neurologic disorder). This report relates to two sisters, aged 8 and 12 years, who had vomiting, muscle weakness, alacrimia, excessive fatigue and dysphagia. Abdominal sonography, esophago-gastroduodenoscopy, barium swallow, esophageal manometry, CT scan abdomen and brain, biochemical profiles, as well as neurologic and ophthalmic evaluations were consistent with Allgrove's syndrome. Management consisted of pneumatic balloon dilatation for achalasia and initiation of cortisone therapy with successful resolution of dysphagia and other symptoms.

Achalasia esophagus; presenting as acute air way obstruction.

Acute airway obstruction from mega-esophagus is a rare presentation of achalasia. Mega-esophagus is generally considered when the transverse width of the esophagus is more than 7 cm. A 78 year old lady presented with longstanding history of productive cough and nocturnal dyspnoea. She was seen in the emergency department with acute exacerbation of dyspnoea after a bout of vomiting, containing semi-solid food with foetid smell. Her respiratory status deteriorated rapidly with onset of stridor, and cyanosis. Chest x-ray showed widening of mediastinum due to dilated esophagus with air-fluid level. Prompt, repeated, upper respiratory tract suction was carried out. A wide bore nasogastric tube was introduced, esophagus was decompressed with a gush of air and fluid, relieving the respiratory distress. This case illustrates an unusual presentation of achalasia underscoring the need for urgent, life-saving esophageal decompression. Hypotheses, regarding the mechanism of airway compromise, as well as, treatment options are reviewed.

Efficacy of Rigiflex balloon dilatation in 12 children with achalasia: a 6-month prospective study showing weight gain and symptomatic improvement.

Achalasia cardia is a disease of adolescents and is rare in children. In total, 12 children with primary achalasia, with a mean age of 10.8 +/- 2 years, were prospectively evaluated for the efficacy of a 30-mm-diameter Rigiflex balloon for relief of symptoms and weight gain after 1 and 6 months of follow up. The 12 children were evaluated and treated for achalasia, with pneumatic balloon dilatation, from January 1998 to December 2000. They were studied for basal, 1-, and 6-month post-dilatation composite symptoms for dysphagia, regurgitation, night cough and heartburn. Basal and 5-min post-dilatation barium swallow were obtained to compare barium height and width for efficacy of dilatation and to evaluate for complications. There were no complications. Barium height, width, composite symptom score and weight improved significantly up to the 6-month follow up. Rigiflex balloon dilatation of 30-mm diameter is safe and effective in children with achalasia.