A 30 year old female presents with dysphagia and heart burn. The present complaint started progressing gradually for the past few months. Now dysphagia is present for both solids and liquids. There is a progressive weight loss of about 6 kg in 2 months. Barium swallow showed bird beak deformity. What is the most likely cause for this presentation?
High-Yield Explanation
Answer: b) Non relaxing lower esophageal sphincter (BAILEY 26TH ED, P-1014)ACHALASIA CARDIAMotor disorder of esophagus where the LES does not relax normally while swallowingEsophagus undergoes non peristaltic contractionsDue to loss of ganglions in the Auerbach's myenteric plexusHistology reveals reduction in the number of ganglion cells - mainly inhibitory neuronsAssociated with stress, vitamin B1 deficiency, Chaga's disease(Trypanosoma cruzi)Premalignant condition for esophageal carcinomaPseudo achalasia - produced by adenocarcinoma of cardia (sometimes ca of bronchus & pancreas)Clinical featuresCommon in femalesProgressive dysphagia to both solids and liquids (liquids > solids), pain, regurgitation, aspiration DiagnosisPlain X-ray abdomen erect: absence of gastric air bubbleX-ray chest lateral view: Retro-cardiac air-fluid levelBarium swallowUniformly dilated proximal esophagus with a smooth tapering segment below - Birds beak or Pencil tip deformity or Cucumber esophagusSigmoid esophagus or mega esophagusAn air fluid level in the mediastinum in upright positionHurst phenomenon - temporary transit through the cardia when the hydrostatic pressure barium column is more than the pressure of the esophageal sphincterManometryA firm diagnosis is established by oesophageal manometryIncomplete relaxation of LESElevated LES pressureLoss of peristaltic wave in esophageal bodyIncreased intra esophageal baseline pressureCCK test - Cholecystokinin normally causes relaxation of LES, paradoxically causes contractionRat tail appearance and Apple core lesion - Esophageal carcinomaGERD may result in endoscopic changes but not a bird's beak appearance on barium swallow.Rosary bead or corkscrew esophagus on esophagogram is characteristic of esophageal spasmTreatmentModified Heller's myotomy is the treatment of choiceFundoplication is usually done along with this procedure to prevent reflux (Heller-Dor's operation)Pneumatic dilatationBotulinum toxin - has to be repeated every few months, repeated injections cause fibrosisDrugs - calcium channel blockers, nitrates