Bird beak appearance on Ba swallow is diagnostic of-
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Correct Answer:
Achalasia
Description:
Ans. is 'b' i.e., Achalasia Achalasiao Achalasia cardia is a motor disorder of the esophageal smooth muscle in which the LES does not relax normally with surallowing, & the esophageal body undergoes nonperistaltic contractions. (Primary peristalsis is absent or reduced).o 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 Aurebach's myentric plexus) or extrinsic (extraesophageal 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 clolinergic innervation of the LES is intact or affected only in advanced disease.o 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 findingso Both sexes are equally affected (Ref: Maingots 10/e, p 846)o May develop at any age but peak years are from 30 to 60.o Classical clinical symptom is progressive dysphagia for both solids and liquids. Dysphagia is worsened by emotional stress and hurried eating.o Regurgitation and Pulmonary aspiration occur because of retention of large volumes of saliva and ingested food in the esophagus.o Esophagitis with ulceration may occur with chronic retention of food.o Pain is infrequent in classical achlasia but a variant called vigrous achlasia is characterized by chest pain and esophageal spasms that generate non-propuloine high-pressure waves in the body of the esophagus.Diagnosiso Chest x-ray - shows absence of gastric air bubble, an air-fluid level in the mediastinum in the upright position representing retained food in oesophagus.o Barium swallow - shows dilated esophagus with tapering narrowing in the terminal end of esophagus described as 'bird's beakr appearance. Fluoroscopy shows loss of normal peristalsis in the lower two thirds of esophagus,o Endoscopy may be done to rule out any secondary cause of achlasia eg. carcinoma, stricture at LES.o ManometryIt's the most confrmatory> investigationIt is able to distinguish between various forms of motor disorders of esophagusManometric characteristics of Achalasiao Incomplete lower esophageal sphincter relaxation (< 75% relaxation)o Elevated LES pressureo Loss ofprimary peristaltic waves in the esophageal body, but disorganized muscular activity may be present,o Increased intraesophageal baseline pressure relative to gastric baseline,o Cholecystokinin (CCK) which normally causes a fall in the sphincter pressure, paradoxically causes contraction of the LES (the CCK test). This paradoxical response occurs because, in achlasia the neurally transmitted inhibitory effect of CCK is absent owing to the loss of inhibitory neurons.Also know"Although complete absence of peristalsis in the esophageal body has been proposed as the major abnormality,present evidence indicates achalasia is a primary disorder of the LES Schwartzo The LES fails to relax on deglutition, - elevation of intraluminal esophageal pressure - esophageal dilatation - subsequent loss of primary peristalsis in the body of esophagus,o Other options are described ahead.
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