Basic Science Lecture 3/8/11 Marcie Dorlon, PGY3

Blood Supply: Inferior thyroid, aortic branches, bronchial branches, left gastic artery branches, inferior phrenic branches Venous drainage: inferior thyroid vein, bronchial veins, hemiazygous and azygous veins, coronary vein Lymphatic Drainage: submucosal, dense single-plexus Innervation: mainly vagus

Antireflux mechanisms: Antireflux mechanisms: mechanically effective LES mechanically effective LES efficient esophageal clearance efficient esophageal clearance adequately functioning gastric reservoir adequately functioning gastric reservoir Tests to detect: Tests to detect: structural abnormalities structural abnormalities functional abnormalities functional abnormalities increased exposure to gastric juice increased exposure to gastric juice duodenogastric function as related to esophageal disease duodenogastric function as related to esophageal disease

Radiographic evaluation (barium swallow) Radiographic evaluation (barium swallow) endoscopic evaluation (with or without biopsy) endoscopic evaluation (with or without biopsy) stationary manometry stationary manometry high-resolution manometry high-resolution manometry esophageal impedance esophageal impedance esophageal transit scintigraphy esophageal transit scintigraphy video- and cineradiography video- and cineradiography 24-hour ambulatory pH monitoring 24-hour ambulatory pH monitoring gastric emptying study gastric emptying study gastric acid analysis gastric acid analysis cholescintigraphy cholescintigraphy 24-hour gastric pH monitoring 24-hour gastric pH monitoring

Chronic disease often requiring life-long medical tx Chronic disease often requiring life-long medical tx Dx: Lack of universally accepted definition Dx: Lack of universally accepted definition Sx: heartburn (substernal burning worsened by spicy and fatty foods, etoh, coffee, chocolate, etc) and regurgitation, cough, hoarseness, asthma, recurrent pneumonia, bronchospasm, dysphagia, odynophagia Sx: heartburn (substernal burning worsened by spicy and fatty foods, etoh, coffee, chocolate, etc) and regurgitation, cough, hoarseness, asthma, recurrent pneumonia, bronchospasm, dysphagia, odynophagia Complications: esophagitis, stricture, Barrett’s esophagus (metaplasia), adenocarcinoma (neoplasia), repetitive aspiration and pulmonary fibrosis Complications: esophagitis, stricture, Barrett’s esophagus (metaplasia), adenocarcinoma (neoplasia), repetitive aspiration and pulmonary fibrosis Tx first line: H2 blockers, PPI, surgery if medical management fails Tx first line: H2 blockers, PPI, surgery if medical management fails Surgical Tx Indicated: Barrett’s metaplasia, ulcer, stricture, failure of medical tx, structurally defective LES Surgical Tx Indicated: Barrett’s metaplasia, ulcer, stricture, failure of medical tx, structurally defective LES Anti-reflux surgery restores gastroesophageal barrier Anti-reflux surgery restores gastroesophageal barrier Laparoscopic Nissen Fundoplication, Transthoracic Nissen Fundoplication Laparoscopic Nissen Fundoplication, Transthoracic Nissen Fundoplication

Type I sliding hernia, upward dislocation of cardia in the posterior mediastinum Type I sliding hernia, upward dislocation of cardia in the posterior mediastinum Type II rolling/paraesophageal hernia, upward dislocation of gastric fundus alongside normally positioned cardia Type II rolling/paraesophageal hernia, upward dislocation of gastric fundus alongside normally positioned cardia Type III combined sliding-rolling/mixed hernia, upward dislocation of cardia and gastric fundus Type III combined sliding-rolling/mixed hernia, upward dislocation of cardia and gastric fundus Type IV: additional organ (colon) herniates along with stomach into chest Type IV: additional organ (colon) herniates along with stomach into chest Sx: same as GERD, anemia Sx: same as GERD, anemia Dx: Barrium swallow, endoscopy Dx: Barrium swallow, endoscopy Indications for surgery: paraesophageal hernia (prevent bleeding, infarction, perforation) Indications for surgery: paraesophageal hernia (prevent bleeding, infarction, perforation) Approach: transabdominal or transthoracic to repair diaphragm +/- fundoplication Approach: transabdominal or transthoracic to repair diaphragm +/- fundoplication Recurrence rates 10-15% Recurrence rates 10-15%

Thin circumferential submucosal ring in the lower esophagus at the squamocolumnar junction, often associated with hiatal hernia Thin circumferential submucosal ring in the lower esophagus at the squamocolumnar junction, often associated with hiatal hernia Significance and pathogenesis unclear, prevalent in % of population Significance and pathogenesis unclear, prevalent in % of population Symptoms: dysphagia (solid food), reflux Symptoms: dysphagia (solid food), reflux Tx: dilation, incision or excision of ring, antireflux procedure Tx: dilation, incision or excision of ring, antireflux procedure

Esophageal abnormalities in 80% patients with this diagnosis Esophageal abnormalities in 80% patients with this diagnosis Perivascular deposition of collagen leads to smooth muscle atrophy in GI tract Perivascular deposition of collagen leads to smooth muscle atrophy in GI tract Primary neurogenic disorder in regards to esophageal symptoms as methacholine and edrophonim relieve symptoms Primary neurogenic disorder in regards to esophageal symptoms as methacholine and edrophonim relieve symptoms Dx: manometry demonstrates absence of peristalsis in distal smooth muscle portion, progressively weakened LES Dx: manometry demonstrates absence of peristalsis in distal smooth muscle portion, progressively weakened LES Can lead to strictures and severe esophagitis Can lead to strictures and severe esophagitis Tx: medical, serial dilations, partial fundoplication for severe cases Tx: medical, serial dilations, partial fundoplication for severe cases

: Zenker’s diverticulum- most common sign of pharyngoesophageal dysfunction : Zenker’s diverticulum- most common sign of pharyngoesophageal dysfunction Sx: dysphagia, regurgitation of undigested bland material, chronic aspiration, weight loss Sx: dysphagia, regurgitation of undigested bland material, chronic aspiration, weight loss Dx: barium swallow Dx: barium swallow Tx: open cricopharyngeal myotomy, diverticularpexy, diverticulectomy, endoscopic Tx: open cricopharyngeal myotomy, diverticularpexy, diverticulectomy, endoscopic Achalasia- primary disorder of LES not relaxing Achalasia- primary disorder of LES not relaxing Sx: pain, regurgitation, weight loss Sx: pain, regurgitation, weight loss Dx: 24-Hr motility monitoring, radiographic “bird’s beak” narrowing distal esophagus Dx: 24-Hr motility monitoring, radiographic “bird’s beak” narrowing distal esophagus Tx: dilation, medication, Botox injection, surgery Tx: dilation, medication, Botox injection, surgery Diffuse esophageal spasm (DES), nutcracker esophagus, hypertensive LES Diffuse esophageal spasm (DES), nutcracker esophagus, hypertensive LES

Long Myotomy- thoracic approach, myotomy extends from 1-2cm below GEJ proximally to level of dysmotility Long Myotomy- thoracic approach, myotomy extends from 1-2cm below GEJ proximally to level of dysmotility Heller Myotomy- myotomy of LES via thoracic or abdominal approach Heller Myotomy- myotomy of LES via thoracic or abdominal approach Open Esophageal Myotomy- used for reoperation Open Esophageal Myotomy- used for reoperation Laparoscopic Heller Myotomy and partial fundoplication- beat pneumatic dilation and Botox injections in several RCT for esophageal motility disorders Laparoscopic Heller Myotomy and partial fundoplication- beat pneumatic dilation and Botox injections in several RCT for esophageal motility disorders

Most common worldwide: squamous carcinoma, associated with etoh and tobacco, lye ingestion, long standing achalasia, HPV Most common worldwide: squamous carcinoma, associated with etoh and tobacco, lye ingestion, long standing achalasia, HPV Increasing incidence of adenocarcinoma: associated with GERD and Barrett’s esophagus Increasing incidence of adenocarcinoma: associated with GERD and Barrett’s esophagus Sx: Dysphagia, asymptomatic found in EGD, stridor, cough, choking, recurrent aspiration or pneumonia, pain swallowing rough or dry food, vocal cord paralysis, TEF Sx: Dysphagia, asymptomatic found in EGD, stridor, cough, choking, recurrent aspiration or pneumonia, pain swallowing rough or dry food, vocal cord paralysis, TEF Staging by CXR, CT, PET, EUS Staging by CXR, CT, PET, EUS Tx: chemoradiation and surgery Tx: chemoradiation and surgery

Rare % of all esophageal tumors Rare % of all esophageal tumors Sx: Dysphagia, same as carcinoma Sx: Dysphagia, same as carcinoma Dx: barium swallow shows large polypoid intraluminal mass causing dilation and obstruction of proximal esophagus (carcinomas tend to ulcerate and stenos) and EGD with biopsy (must get to bleeding tissue or bx only demonstrate necrosis) Dx: barium swallow shows large polypoid intraluminal mass causing dilation and obstruction of proximal esophagus (carcinomas tend to ulcerate and stenos) and EGD with biopsy (must get to bleeding tissue or bx only demonstrate necrosis) Tx: resection, little role for radiation as tumors remain superficial with rare metastasis or spread to LN Tx: resection, little role for radiation as tumors remain superficial with rare metastasis or spread to LN

Uncommon, divided into within lumen or within muscular wall Uncommon, divided into within lumen or within muscular wall Leiomyoma: constitute more than 50% benign esophageal tumors, average age at presentation is 38, more common in males, smooth muscle origin so >90% are found in lower 2/3 of esophagus Leiomyoma: constitute more than 50% benign esophageal tumors, average age at presentation is 38, more common in males, smooth muscle origin so >90% are found in lower 2/3 of esophagus Dysphagia and pain most common complaints, followed by bleeding Dysphagia and pain most common complaints, followed by bleeding Dx: barium swallow classical smooth, contoured, punched-out lesion Dx: barium swallow classical smooth, contoured, punched-out lesion Tx: enucleation with closure and reconstruction of muscular layer Tx: enucleation with closure and reconstruction of muscular layer Congenital or acquired cysts Congenital or acquired cysts Tx: excision Tx: excision

True Emergency, cause is most commonly iatrogenic True Emergency, cause is most commonly iatrogenic Spontaneous: Boerhaave’s Syndrome (15%), Foreign body (14%), trauma (10%) Spontaneous: Boerhaave’s Syndrome (15%), Foreign body (14%), trauma (10%) Sx: Pain Sx: Pain Dx: CXR- mediastinal emphysema and widening, pneumothorax (pleural rupture) Dx: CXR- mediastinal emphysema and widening, pneumothorax (pleural rupture) Contrast esophogram with gastrograffin confirms in 90% patients (position in right lateral decubitus position for best result) Contrast esophogram with gastrograffin confirms in 90% patients (position in right lateral decubitus position for best result) Tx: Key is early dx! Radiographic signs could take hours to show up Tx: Key is early dx! Radiographic signs could take hours to show up Primary closure within 24 hours  80-90% survival rate Primary closure within 24 hours  80-90% survival rate Repair after 24hrs < 50% survival rate Repair after 24hrs < 50% survival rate

Syndrome characterized by acute upper GI bleeding following repeated vomiting is considered to be cause to up to 15% of all severe upper GI bleeding Syndrome characterized by acute upper GI bleeding following repeated vomiting is considered to be cause to up to 15% of all severe upper GI bleeding Caused by arterial bleeding, which may be massive Caused by arterial bleeding, which may be massive Can also be caused by paroxysmal coughing, seizures, or retching Can also be caused by paroxysmal coughing, seizures, or retching Dx: Upper endoscopy Dx: Upper endoscopy Tx: nonoperative management in majority of patients (bleeding stops spontaneously) Tx: nonoperative management in majority of patients (bleeding stops spontaneously) Resuscitate, stomach decompression, antiemetics, endoscopic injection of epinephrine Resuscitate, stomach decompression, antiemetics, endoscopic injection of epinephrine Surgery as last resort Surgery as last resort Mortality uncommon and rare recurrence Mortality uncommon and rare recurrence

Occurs mainly in children, in adults/teenagers associated with suicide attempts Occurs mainly in children, in adults/teenagers associated with suicide attempts Alkalies more frequently accidentally swallowed because strong acids cause immediate burning pain in mouth that prevents swallowing Alkalies more frequently accidentally swallowed because strong acids cause immediate burning pain in mouth that prevents swallowing Alkalies cause liquefaction necrosis and acids cause coagulation necrosis Alkalies cause liquefaction necrosis and acids cause coagulation necrosis Lye injury: acute necrotic phase (1-4 days), ulceration and granulation phase (3-12 days), and cicatrization and scarring phase (three weeks +) Lye injury: acute necrotic phase (1-4 days), ulceration and granulation phase (3-12 days), and cicatrization and scarring phase (three weeks +) Sx: depend on extend of lesion Sx: depend on extend of lesion Tx: immediate neutralizing agents (within 1 hour), then depends on extent of lesion Tx: immediate neutralizing agents (within 1 hour), then depends on extent of lesion NO sodium bicarb (produces carbon dioxide in increases risk perforation) NO sodium bicarb (produces carbon dioxide in increases risk perforation)

Result of esophageal or pulmonary malignancy, less common trauma or related to diverticula Result of esophageal or pulmonary malignancy, less common trauma or related to diverticula Sx: Paroxysmal coughing after ingestion of liquids, recurrent or chronic pulmonary infections Sx: Paroxysmal coughing after ingestion of liquids, recurrent or chronic pulmonary infections Tx: Benign fistula: Division of fistula tract, resection of abnormal pulmonary tissue involved, repair of esophageal defect, interposition of pleural flap Tx: Benign fistula: Division of fistula tract, resection of abnormal pulmonary tissue involved, repair of esophageal defect, interposition of pleural flap Malignant fistula: difficult usually due to radiation tx, palliative stent or surgery (esophageal diversion and feeding jejunostomy) Malignant fistula: difficult usually due to radiation tx, palliative stent or surgery (esophageal diversion and feeding jejunostomy)