A female patient has dysphagia, intermittent epigastric pain. On endoscopy, esophagus was dilated above and narrow at the bottom. Treatment is:
Ans is 'd' i.e. Heller's Cardiomyotomy Ref: CSDT 13/ep430 This patient is having Achalasia cardia."A laparoscopic Heller myotomy and partial fundoplication is the procedure of choice for esophageal achalasia..... A laparoscopic Heller myotomy allows excellent relief of symptoms in the majority of patients and should be preferred to pneumatic dilatation whenever surgical expertise is available. Botulinum toxin and medications should be used only in patients who are not candidates for pneumatic dilatation or laparoscopic Heller myotomy. ''Achalasia Achalasia cardia is a motor disorder of the esophageal smooth muscle in which the LES does not relax normally with swallowing, & the esophageal body undergoes non peristaltic contractions. (Primary peristalsis is absent or reduced)The pathogenesis of Achalasia is poorly understood It involves:neurogenic degeneration either idiopathic or due to infection. The degenerative changes are either intrinsic (degeneration of ganglion cells of Auerbach's myenteric plexus) or extrinsic (extra esophageal vagus nerve or the dorsal motor nucleus of vagus)Pharmacologic studies suggest dysfunction of inhibitory neurons containing nitric oxide and vasoactive intestinal polypeptide in the distal esophagus (LES). The cholinergic innervation of the LES is intact or affected only in advanced disease.As a result of the abnormality, the LES fails to relax, primary peristalsis is absent in esophagus which dilates. As the disease progresses the esophagus becomes massively dilated and tortuous.Clinical findingsBoth sexes are equally affected (Ref: Maingot's 10/e, p 846)May develop at any age but peak years are from 30 to 60.Classical clinical symptom is progressive dysphagia for both solids and liquids. Dysphagia is worsened by emotional stress and hurried eating.Regurgitation and Pulmonary aspiration occur because of retention of large volumes of saliva and ingested food in the esophagus.Esophagitis with ulceration may occur with chronic retention of food.Pain is infrequent in classical achalasia but a variant called vigorous achalasia is characterized by chest pain and esophageal spasms that generate non-propylamine high-pressure waves in the body of the esophagus.DiagnosisChest x-ray - shows absence of gastric air bubble, an air-fluid level in the mediastinum in the upright position representing retained food in oesophagus.Barium swallow - shows dilated esophagus with tapering narrowing in the terminal end of esophagus described as 'birds' beak' appearance. Fluoroscopy shows loss of normal peristalsis in the lower two thirds of esophagus.Endoscopy may be done to rule out any secondary cause of achalasia eg. Carcinoma, stricture at LES.ManometryIt's the most confirmatory investigationit is able to distinguish between various forms of motor disorders of esophagusManometric characteristics of AchalasiaIncomplete lower esophageal sphincter relaxation (<75% relaxation)Elevated LES pressureLoss of primary peristaltic waves in the esophageal body, but disorganized muscular activity may be present.Increased intra esophageal baseline pressure relative to gastric baseline.Treatment of AchalasiaThe aim of treatment is to be relieve the functional obstruction at the cardiaThe two main methods to achieve this areForceful dilatation orEsophageal myotomy (Heller's myotomy) with or without an antireflux procedureForceful dilatation (pneumatic dilatation)The aim is to weaken or rupture the circular muscle fibres of the LES by forceful stretch.Perforation and bleeding are potential complicationsExtra mucosal cardiomyotomy {Heller's myotomy)this involves surgical division of the muscle fibres of the lower esophageal sphincter.this procedure can be performed through a laparoscopic or thoracoscopic approach {Open surgical procedure i.e. Laparotomy or thoracotomy can also be done, but the videoscopic procedure is better)major complication is gastro-esophageal reflux.partial fundoplication is done to prevent reflux "modified laparoscopic Heller myotomy is the operation of choice"- SabistonOther methods of treatmentDrugsNitrates and calcium channel blockers can be used, but are ineffective for long term use. They can be used for transient relief of symptoms and in patients unfit for surgery and pneumatic dilatation.Botulinum toxinBotulinum toxin is given by endoscopic injection into the LES. It reduces LES pressure by blocking the cholinergic excitatory nerves in the sphincter.Its effect is only short-lived and repeated injections have to be given.Used only in patients unfit for surgery and pneumatic dilatation.
PPIs
Esophagectomy
Dilatation
Heller's Cardiomyotomy
Esophageal Motility Disorders