Ans. a. Heller's cardiomyotomy (Ref: Sabiston 19/e p1025-1028; Schwartz 9/e p850-851, 857-860: Bailey 26/e p1014- 1017, 25/e p1036-1038; Shackelford 7/e p349-352, 354-361)The features of dysphagia, intermittent chest pain and dilation of esophagus with narrowing of its distal end on radiology in a young female suggest a diagnosis of achalasia. Treatment of choice for achalasia cardia is Heller's cardiomyotomy.Achalasia CardiaAchalasia means "failure to relaxQ," (sphincter remains in a constant state of tone with periods of relaxation)Both the muscle of the esophagus and LES are affectedQ.Prevailing theory: Destruction of the nerves to LES is primary pathology and degeneration of neuromuscular function of the body of esophagus is secondaryQ.Premalignant condition leading to squamous cell carcinomaTriple A-syndrome or Allgroves diseaseQ: Achalasia, Alacrima and ACTH-resistant Adrenal insufficiency.Pathogenesis:Progressive inflammation and selective loss of the inhibitory myenteric neurons in Auerbach'splexus of the esophagus that normally secrete VIP and nitric oxideQ.This results in failure of relaxation of the LES and aperistalsis of the esophageal body with subsequent functional obstruction at the level of the GE junction and gradual dilatation of the esophagusQ.Clinical Features:The classic triad of symptoms consists of dysphagia, regurgitation, and weight loss.Heartburn, postprandial choking, and nocturnal coughing are seen commonly.Men and women are equally affected, with no ethnic predisposition to the diseaseQ.Regurgitation of undigested, foul-smelling foods is common, and with progressive disease, aspiration can become life-threateningQ.Pneumonia, lung abscess, and bronchiectasis often result from long-standing achalasia.Dysphagia progresses slowly over yearsQ.Diagnosis:Barium swallow:Dilated esophagus with a distal narrowing"Bird's beak" ,"Pencil-tip" or "Rat's tail" appearanceQSphincter spasm and delayed emptying through the LESA lack of peristaltic waves in the body and failure of LES relaxationLack of a gastric air bubbleQ on the upright portion is a result of the tight LES not allowing air to pass easily into the stomach.Massive esophageal dilation, tortuosity, and a sigmoidal esophagus (megaesophagus) in advanced stageQMecholyl test is positive in AchalasiaQCCK test is positive in AchalasiaQManometry is gold standard test for diagnosis.Absence of body peristalsis and poor LES relaxation is mandatoryQ for diagnosis.Manometry Findings of Typical AchalasiaAbnormalities of LESAbnormalities of esophageal body* Incomplete or absent LES relaxationQ* Elevated LES pressureQ* Elevated intraesophageal pressureQ (pressurization of the esophagus) from incomplete air evacuation* Simultaneous mirrored contractions with no evidence of progressive peristalsisQ* Low-amplitude waveformsQ indicating a lack of muscular toneTreatment:Early stage: Sublingual nitroglycerin, nitrates, or calcium channel blockersQ may offer hours of relief of chest pressure before or after a meal.Bougie dilationQ up to 54 French may offer several months of relief but requires repeated dilations to be sustainable.Botulinum toxin:Injection of botulinum toxin (Botox) directly into the LES blocks acetylcholine release, preventing smooth muscle contraction, and effectively relaxes the LESQ.With repeated treatments, Botox may offer symptomatic relief for yearsSymptoms recur more than 50% of the time within 6 monthsQ.Laparoscopic Heller myotomy is now the operation of choiceQ.Extent of Heller's myotomy: 2 cm above GE junction to 1 cm belowQ, over stomach.Partial antireflux procedure (Toupet or Dor fundoplication)Q will restore a barrier to reflux and decrease postoperative symptoms.Esophagectomy is considered megaesophagus, sigmoid esophagus, failure of more than one myotomy, or an undilatable reflux strictureQ.