NEUROMUSCULAR ESOPHOGEAL DISORDERS Dr. Abdullah Bahadi GS R2 Zenker’s Diverticula • It consist of three locations pharyngoesophageal , midesophageal , epiphrenic • Historically known to be caused by motor disturbances of ues and les • What is the difference between true and false diverticulum • Most common area for zenker diverticulum ?? • Classic patients are about 70 years old , this is has been thought to be caused by losing the tone and elasticisty with age • You name the symptoms • Most serious is • aspiration and lung abscess • Diagnosis is done by barium swallow • <2cm diverticulum myotomy is suffuciant , in frail pt do diverteculopexy due to higher risk of leak • >5 cm or good tissue >> do excision and keep pt npo 2-3 days post op • 2-5 cm >> endoscopic , needs extension of neck (not good for cervical stenosis) , 1 day stay , NPO post op day , • Study wise <3cm surgical is suprorior to endoscopic , >3cm both are same , inanition and stay is shorter in the endoscopic Midesophageal Diverticula • Historically caused by tuberculosis or fibrosing medistinitis • Nowadays belived to be from motility disorders , tend to • • • • • be right sided due to left crowding Diagnosis by barium swallow with lateral views to identify sac place before surgery CT and manometry ?? <2 cm >> no need symptomatic or 2 or more cm>> intervention required with diverteculopexy to thoracic vertebral fascia severe symptoms>> long esophagomyotomy is done Epiphrenic Diverticula • Tend to be right sided and its within 10 cm from the GEJ , • • • • its pulsion diverticula and can be caused due to motility disorder most commonly NEM , other causes like trauma or ehlers danlon can be assosciated < 2cm suspend to the vertebral fascia Severe chest symptoms and documented motor disorder >> long esophagomyotomy If there is large hiatal hernia during the repair it must be repaired Motility disorders • Primary : achalasia . DES , Hypertensive LES , Nutcracker , Ineffective esophageal motility • Secondary : collagen vascular disease , NEM disorders Achalasia • Means failure to relax • 6/100000 • Young women – middle aged man • Can be caused by nerve degeneration , trauma , infection (trypanosome cruzi) , stress • Nerveous degeneration of the LES >> makes it hypertensive and fail to relax with swallowing >> pressurization >> dilatation • After 20 years the risk is __% to develop squamous cell carcinoma but no specific survilance is there mostly occur at middle third of the esophagous below level of airfluid • Classic triad is dysphagia + regurgitation + weight loss • Patient often experience liquid dysphagia that progress to solid • Patient try to adapt by drinking water and experience relieve with LES opened • Patient present late or with complication such pneumonia and abscess • Lack of gastric bubble means advanced disease • It can lead to megaesophagous • Manometry is gold standard it will show 5 findings • 2 in les and 3 in esophageal body • LES pressure higher than 35 and failure to relax with degultation • Esophageal body will show , pressure above baseline due to air in esophagous, low amplitude due to lack of tone , mirrored simultaneous contraction with no progressive prestalsis • Endoscopy is done for esophagitis and cancer but not diagnosis • Non surgical treatment , ccb , nitrates , nitroglycerine • Botox treatment prevent acetylcholine release but recurrence is 50% in 6 month • Bougie dilatation until 54fr can be done but needs serial dilatations to sustain • Gruntzig type ballon effective in 60% and risk of perforation is 4% • Surgical heller myotomy can be done laparoscopic and is treatment of choice lower risk of perforation than ballon , pt can have reflux post surgery and its controversy to do partial antireflux surgey with it like topet or dor • Esophagectomy is endstage treatment , for those with megaesophagous , failed myotomy (<60% will benefit from repeated surgery) , reflux strictures Different treatments • 1 year remission ballon dilataion vs botox 89%vs38% • Perforation surgery vs ballon 1%vs4% • Mortality surgery vs ballon 0.2%vs 0.5% • Laparoscopic proved shorter stay , less pain , better relief of dysphagia , improved heartburn Diffuse esophageal spasms • Young women • Simultaneous contractions of esophageal body with high amplitude due to what it could be nerve degeneration and muscular hypertrophy • 5 times less common than achalasia • Chest pain plus dysphagia , squeezing CP , related to • • • • • stress Can be exacerbated by cold liquids IBS , pyloric spasm, gallstones , pancreatitis , can all trigger DES Picture of corkscrew esophagous or pseudodivertucolosis on esophagogram Manometry findings , multipeaked contraction with high amplititude >120mmhg Or long duration >2.5 • Introducing bethancol or ambulatory manometry can help catch spontenous contractions • Treatment is non surgical is the best , antireflux if needed, nitrates and ccbs , conrolloing triggers , psychiatric problems , reassurance • Botox= non sustainable • Dilatation = 70-80% of success • Peppermint of some symptomatic relief • Patient for long esophagomyotomy if failed medical tx , pulsion diverticulum, failed endoscopic therapy , incapacitating symptoms • Surgery can provide 80% relieve , extend must be determined by Manometry • Dor fundo performed with it Nutcracker esophagous • All ages , no specific gender , most painful disorder • Diagnosed by chest pain + contraction of esophagous more than 2 SD than normal up to 400 mmhg , normal LES • Mainly medical tx nitrates ,ccb, antispasmodics • Avoid triggers , dialation maybe of benefit Hypertensive LES • Hypertensive LES but normal relaxation • Hyperprestaltic esophageal body contraction but sometimes normal • Symp As usual nothing new • In manometry there is hypertensive of LES with normal relaxation • Medical or ballon or botox could be tried but procedure of choice is modified laparoscopic heller myotomy IES • Distal esophagous noncontraction leading to decrease in the clearance of gastric acid • Caused by GERD destroying the distal esophagous • Treatment is by prevention Non specific esopheageal Motility disorders • Anything else falls in this category • Conditions like dermatomyositis , scleroderma , SLE causes esophageal motility disorders • More reflux more regurgitation • Treated by treatment of underlaying disease • No specific findings in LES , but in esophageal body it will be either non transmitted , triple-peaked, retrograde , low amplititude <35mm hg, prolonged >6