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Dr W.J. Conradie Department of Diagnostic Radiology March 2012 Housewife No previous major surgery Medical history: Hypertensive with mild CCF on medication. Irritable bowel syndrome Medication: Fosamax Disprin Adco Dol Enalapril and Lasix Family history: Eldest son died of esophageal Ca in 2007 Progressive dysphagia (solids/fluids) over couple of months. Episodes of coughing while eating/drinking Intermitted regurgitation of undigested food. Feeling of “fullness” in neck Weight loss ± 5kg Large para-tracheal mass on the left extending into/through thoracic inlet Moved with swallowing No features of thyrotoxicosis No cervical lymphadenopathy Severe kypho-scoliosis CXR: Degenerative spine Clear lung fields Bloods: Normal FBC, U&E, LFT CRP and ESR Thyroid functions S-albumin Large irregular mass from left thyroid lobe Extends deep into superior mediastinum Cyst with calcifications inferiorly Nodule in superior aspect of lobe with central breakdown No mediastinal lympnodes Lung fields clear Incidental: Aorta arch anomaly Aorta arch anomaly: 1. Main stem for right and left common carotid 2. Left subclavian artery 3. Aberrant right subclavian artery Differential diagnosis for dysphagia 1. Thyroid mass 2. Zenker ‘s Diverticulem 3. Aberant right subclavian artery (dysphagia lusoria) 4. Achalasia Named after Friedrich Albert von Zenker who was a German pathologist (1825 – 1898) Definition: Mucosal outpouching of posterior hypopharyngeal wall. Proximal to upper esophageal sphincter (Cricopharyngeal muscle) Pathophysiology: Pulsion-pseudodiverticulum with herniation of mucosa and submucosa through Killian’s dehiscence. Focal weakness in cleavage plane between the fibers of inferior pharyngeal constrictor and cricopharyngeus muscles. Due to cricopharyngeal dysfunction luminal pressure Prevalence <0.2% of general population Elderly woman >50% occur in 7th -8th decade Complications Aspiration Perforation Ulceration Carcinoma Clinically: globus feeling dysphagia halitosis regurgitation Associated with: Hiatus hernia GER / Reflux oesophagitis Achalasia Differential diagnosis 1. Killian-Jamieson diverticulum (K-J) 2. Esophageal web 3. Lateral pharyngeal pouch 4. Epidermolysis bullosa dystrophica General features: Location: Killian’s dehiscence Posterior above cricopharyngeus C5-6 Size: 0.5-8cm (average 2.5cm) Best diagnostic clue: Barium filled sac! Radiographic findings: CXR/CT: Air-fluid level in superior mediastinum Barium swallow AP: Barium-filled sac below the level of hypopharynx Lateral/oblique view: Barium-filled sac posterior to cervical esophagus Neck opening into posterior wall above cricopharyngeus m. Prominent or thickened cricopharyngeal muscle Luminal narrowing at upper pharyngoesophageal junction ± Nasopharyngeal regurgitation Dysphagia secondary to extrinsic esophageal compression by an aberrant right subclavian artery Described by Bayford in 1794 lusoria - Greek phrase lusus naturae, meaning “ freak or zest of nature”, which refers to the freaky course of the artery (lusoria artery) Prevalence of 1.8% 1/3 experience symptoms (90% = dysphagia) Any age Old age: atherosclerosis or aneurysmal dilatation of ARSA. Associated: Dyspnoea Lower right arm BP/pulse volume Diverticulum of Kommerell. Management: Conservative Carotico-subclavian bypass Definition: Primary motility disorder of esophageal smooth muscle Failure of LES to relax “Failure to relax” Sir Thomas Willis in 1672. 1929: Hurt and Rake Discovered failure of LES to relax. Pathophysiology Degeneration of Auerbach’s plexus Primary(classic) Secondary Infectious - idiopathic (number decrease, CNX – nucleus or nerve) metastases, adenocarcinoma, vagotomy, scleroderma Chagas disease (trypanosoma cruci neurotoxin destroys ganglia) Prevalence Primary: younger (20-50) Secondary: older Male=female Clinically: Dysphagia (solids and liquids) Regurgitation Weight loss in 90% Diagnosis Exclude malignancy Exclude oesophageal spasm Manometry Complications: Coughing Aspiration Pneumonia Lung abscess Esophageal carcinoma (2-7%) Management: Aimed at improving esophageal emptying Calcium channel blockers Botulinum toxin injection Pneumatic dilatation Heller myotomy General findings 2 criteria: Absent primary/secondary peristalsis LES fails to relax when swallowing Tertiary waves "Bird-beak" deformity Dilated esophagus with smooth, symmetric, tapered narrowing at GEJ CXR: Mediastinal widening Double contour Anterior tracheal bowing Air-fluid level in mediastinum Small or absent gastric air bubble Classic Achalasia Secondary Achalasia Dilated esophagus (>4cm) Less dilated (<4 cm) Absent peristalsis Decreased or absent peristalsis Distal segment "Bird-beak" deformity Distal segment: Eccentric, nodular, shoulder smooth, symmetric, tapered Hurst phenomenon: transit when hydrostatic pressure of barium column is above tonic LES pressure Narrowed segment: <3.5 cm in length Narrowed segment: >3.5 cm Differential diagnosis 1. Scleroderma 2. Esophageal carcinoma 3. Gastric carcinoma 4. Esophagitis with stricture 5. Diffuse esophageal spasm Cause for dysphagia: Thyroid mass Surgicaly removed 16-07-2008 Histology: Benign, Non toxic Nodular goitre Outcome (2012): Improved but still suffers from dysphagia!! Zenker’s divertikulem? ARSA? THANK YOU 1. Weissleder, Wittenberg, Harisinghani, Chen. Primer of Diagnostic Imaging. Fifth edition. 2011. 2. Federle, Jeffrey, Desser, Anne, Eraso. Diagnostic Imaging of the Abdomen. First edition. 2004. 3. (PPP) ZENKER’S DIVERTICULUM. N. D’Souza,Underbrink. 2010 4. J. Dandelooy, J.P.M. Coveliers, P.E.Y. Van Schil, S.Anguille. Dysphagia lusoria. CMAJ • October 13, 2009 • 181(8) 5. P.D. Kent, T.H. Poterucha. Aberrant Right Subclavian Artery and Dysphagia Lusoria. N Engl J Med, Vol. 346, No. 21 May 23, 2002