Oesophagus

Question 1

Types of Achalsia

Answer 1

Type 1 (classic): absence of any contractions or pan-oesophageal pressurisations that fail to reach 30mmHg.
Type 2 (most): pan-oesophageal pressurisations that occur in ≥20% of swallows, reaching ≥30mmHg. These are due to residual contractile activity in both circular and longitudinal muscles
Type 3 = spastic activity, shortened latency =\> premature/spastic contraction

Question 2

T score Oesaphgeal Cancer

Answer 2

T-factor
- Tis: carcinoma in situ, high-grade dysplasia
- T1: invades lamina propria or submucosa
o T1a: lamina propria or muscularis mucosa
o T1b: submucosa
- T2: Invades muscularis propria
- T3: invades adventitia
- T4: invades adjacent structures
o T4a: resectable tumour invading pleura, pericardium, azygous vein, diaphragm, peritoneum
o T4b: unresectable tumour invading other structures (aorta, vertebrae, airway)

Question 3

N/ M score Oesophageal Cancer

Answer 3

● N-factor (at least 6 nodes should be assessed)
- N0: no regional LN mets
- N1:mets in 1-2 regional LNs
- N2:mets in 3-6 regional LNs
- N3:mets in 7+ regional lymph nodes
● M factor
- M0: no distant mets
- M1: distant mets (most common = liver, lung, bone, adrenal)

Question 4

Management of Patients with metastatic oesophageal cancer

Answer 4

o combination chemo e.g. FOLFOX (leucovorin, 5FU, oxaliplatin) as first line o HER2 assay for consideration of trastuzumab (herceptin)
o enroll in clinical trial
o tumours with dMMR, MSI-H, or overexpression of programmed cell death
ligand 1 (PD-L1) is immunotherapy with an immune checkpoint inhibitor e.g.
pembrulizumab or VEGF inhibitor (ramucirumab)
o If not fit => supportive mx and palliation

Question 5

Oesophageal Embryology

Answer 5

Develops as part of the foregut from the endodermal primitive gut tube.
The gut tube forms during weeks 6-8 (abnormalities lead to atresia or stenosis.

The region of the foregut just caudal to the pharynx develops two longitudinal ridges called the tracheoesophageal folds that divide the tube ventrally into the trachea (and subsequent lung buds), and dorsally into the oesophagus.
As with the rest of the gut tube, the lumen of the oesophagus becomes temporarily OCCLUDED around the 5th week of development and recanalises by around the 9th week.
The oesophagus is initially short and must grow in length to “keep up” with the overall growth in length of the embryo The heart and lungs descend with the oesophageal lengthening and reach their final position at week 7

Question 6

Clinical consideration of oesophageal embryology?

Answer 6

Oesophageal atresia

○ occurs when the tracheoesophageal ridges deviate too far dorsally causing the upper oesophagus to end as a closed tube.

○ usually is accompanied by a tracheoesophageal fistula, in which case gut contents can be aspirated into the lungs after birth causing inflammation (pneumonitis) or even infection (pneumonia).

○ typically associated with polyhydramnios prenatally (the fetus cannot swallow amniotic fluid and it accumulates in the amniotic cavity). Postnatally, the child will regurgitate IMMEDIATELY upon feeding and, if a tracheoesophageal fistula is present, there will be congestion in the lungs.

Oesophageal stenosis

○ occurs when the oesophagus fails to recanalise (week 7)

○ also typically associated with polyhydramnios prenatally. Postnatally, the child will regurgitate IMMEDIATELY upon feeding. However, there is usually NOT a tracheoesophageal fistula, so the lungs will usually NOT be congested.

Congenital hiatal hernia

○ occurs when the oesophagus fails to grow adequately in length. As a result, the oesophagus is too short and therefore pulls the cardiac stomach into the oesophageal hiatus in the diaphragm. The resulting compromised structure of the hiatus can allow gut contents (usually loops of small bowel) to herniate up into the thoracic cavity.

Question 7

Oesophageal Anatomy

Describe the anatomy and relations of the oesophagus

Answer 7

• The oesophagus is a muscular tube between the cricoid (C6) superiorly and the gastric cardia inferiorly. It measures 25 cm in length. It passes through the oesophageal hiatus at T10. During its descent it inclines initially to the left, returns to the midline, then deviates left again to sit 2.5cm to the left of the midline on entry to the abdomen. Its lumen is indented at 4 points; its origin by cricopharyngeus (15cm from incisors), by the aorta (22cm from incisors), and by the left main bronchus (27cm from incisors) – also described as the bronchoaorticconstriction- and finally at the diaphragmatic hiatus (38cm from incisors). It is* often described as having 3 parts: the cervical, thoracic, and abdominal oesophagus. The intra-abdominal part of the oesophagus is only 1-2cm in length.
• Posteriorly it is related to the trachea until the trachea bifurcates at T4. At the same point, the azygous is to its right and the aortic arch to its left and the thoracic duct crosses from the right to the left side of the oesophagus. Between the tracheal bifurcation and the diaphragm the left atrium is the anterior relation. On either side the mediastinal pleura touches the oesophagus.*

Question 8

Oesophageal Blood supply?

Answer 8

Inferior thyroid (cervical)

Aortic branches (thoracic)

Left Gastric (abdominal)

Arterial supply is via branches of the _inferior thyroid artery_ superiorly, direct _aortic branches_ in its mid-portion, and from ascending oesophageal branches from the _left gastric_ distally. Venous drainage corresponds to arterial supply, _importantly the left gastric-ascending oesophageal veins are a site of porto-systemic shunt and possible varices_.

• Lymphatic drainage is via a rich submucosal plexus throughout its length; the oesophagus may drain to the cervical, mediastinal, or coeliac lymphatic plexuses.*
• Nerve supply is segmental; its upper portion is supplied by the recurrent laryngeal nerves, the vagus and thoracic splanchnics supply autonomic nerves for the remainder.*

Question 9

Epithelium??

Muscularis?

Answer 9

Stratified squamous, cuboidal at GOJ

Striated proximally, mixed mid, smooth distally.

Thicks muscular mucosal + no adventitia/serosa - therefore join to this

Question 10

Oesophageal nerve supply

Answer 10

PARASYMPATHETIC - VAGUS

Sympathetic - middle cervical ganglion (proximal) + upper 4 ganglia (distal)

Question 11

GOJ: What contributes to the anti reflux barriers

Answer 11

1. The intrinsic musculature of the distal oesophagus is in a state of tonic contraction - relax after swallowing to allow the passage of food/liquid
2. Sling fibres of the gastric cardia are oriented diagonally from the cardia funds junction to the lesser curve of the stomach (insert into submucosa) → located same anatomic depth as the circular muscles of oesophagus
3. The crura of the diaphragm surrounds the oesophagus - compressed the oesophagus during inspiration
4. Increased intra-ado pressure transmitter to GOJ which increases the pressure in the distal oesophgus

• Normal pressure gradient between the stomach and the oesophagus. The oesophagus is -5mm and the stomach is +5mmhg
• Neurological control of the lower oesophageal sphincter.

Central – dorsal efferents-> vagal efferents (sensory via tractus solitarus, neurotransmitters include glutamate, adrenaline, dopamine, acetylcholine and nitric oxide).

Peripheral – vagus fibres synapse in the myenteric plexus using Ach

Inhibitory control via nitric oxide and the interstitial cells of cajal

Question 12

Physiology of swallowing??

Answer 12

Relaxation of upper and lower oesophageal sphincters

Food bolus is propelled by peristaltic wave due to sequential oesophageal muscle segmental contraction
- Primary peristalsis initiated centrally after swallowing: Wave starts in pharynx
o Initiated centrally (via vagus) - Nucleus ambiguous (skeletal muscle) + Dorsomotor complex (smooth muscle)
o Modified peripherally (local myogenic mechanisms)→ Affected by temperature, volume, acidity. Warm boluses exaggerate peristaltic wave

Secondary peristaltic wave
o Persistent bolus triggers local mechanisms by distending oesophagus

Tertiary contractions - Aberrant + No role in peristalsis
Peristaltic wave can be interrupted by subsequent peristaltic wave. This allows multiple swallows for same bolus. First swallow peristaltic wave is aborted. Mediated by intrinsic nitric oxide inhibition

Question 13

Factors leading to GORD?

Answer 13

1. GOJ incompetence due to … increased transient LOS relaxation (65%), incompetent/hypotensive LOS (18%) and Anatomic disruption of the GOJ and HH (17%)
2. Abnormal oesophageal motility/laxity (achalasia, scleroderma) - relfux peristalsis is induced by oesophageal acid receptors
3. Gastric abnormality - gastro paresis, gastric outlet obstruction, acid hyper secretion

Question 14

Other contributers to antireflux…

Answer 14

1. Mucosal rosette
2. Oesophageal clearance - reflux peristalsis when acid high (reduced in motility disorders/ scleroderma)
3. tissue resistance
   
   1. preepithelial - buffer layer/saliva
   2. epithelial - tight junction, pH dependant cation channels, intracellular buffers, transmural electrochemical gradients
   3. post-epithelial - adapter perfusion and epithelial repair

Question 15

What is GORD?

Answer 15

GORD is a disorder where gastric contents reflux into the oesophagus resulting in symptoms and/or mucosal damage.

It is a risk factor for oesophageal carcinoma

Extent of symptoms and mucosal injury is proportional to frequency of reflux, duration of acid exposure and caustic potency of refluxed fluids.

Question 16

Objectives of Investigation in GORD?

Answer 16

1. Confirm the diagnosis of GORD
2. Look for complications
3. Define the anatomy of the oesophagus
4. Exclude a motility disorder

Question 17

Indications for endoscopy in GORD

Answer 17

Severity of symptoms: >4weeks, persistent symptoms despite treatment, relapsing symptoms

Alarm symptoms: new onset age 40-50, dysphagia, odynophagia, persistent vomiting, anorexia, weight loss, anaemia, GI bleeding, first degree relative with cancer

Abnormal imaging

Question 18

Los Angeles Classification

Answer 18

Grade A: 1 or more mucosal breaks <5mm in length

Grade B - 1 of more mucosal breaks > 5mm but not continuous between the tops of adjacent folds

Grade C - 1 or more mucosal breaks that is continuous between the tops of folds but not circumferential

Grade D - mucosal breaks involving > ¾ of the oesophagus circumference

Question 19

What is the Gold standard for the investigation of GORD?

Answer 19

pH studies

Indications: refractory typical symptoms, atypical symptoms, motility disorder suspected

Atypical reflux symptoms or non erosive disease for whom antireflux surgery is being considered.

Question 20

What parameters are generated by pH studies

Answer 20

1. Number of episodes
2. Episodes > 5mins
3. Total reflux time
4. Longest episode
5. Upright and supine times

Reflux episodes start when the pH is less than 4 and ends when it is above 5

Question 21

Pathogenesis of GORD

Answer 21

The development of gastroesophageal reflux disease (GORD) reflects an imbalance between injurious or symptom-eliciting factors (reflux events, acidity of refluxate, oesophageal hypersensitivity) and defensive factors (oesophageal acid clearance, mucosal integrity). The extent of mucosal injury is proportional to the frequency of reflux events, the duration of mucosal acidification, and the caustic potency of refluxed fluid

Question 22

What is the DeMeester score?

Answer 22

% of total time pH is <4 in 24 hours

Positive if above 14.72 (a bit more than 3.5 hours)

Question 23

What are the risk factors for Barrett’s Oesophagus

Answer 23

GORD symptoms of 5 years

Nocturnal reflux

FH, obesity, smoking

Demographics: older white males.

Question 24

WHAT IS THE GOLD STANDARD FOR MOTOR FUNCTION?

Answer 24

Manometry

Measures: baseline sphincter pressure and length of oesophageal sphincter, contraction amplitude, pressure wave duration, peristaltic velocity, LOS function and position

Question 25

Medical Management of GORD

Answer 25

* *Suggested approach** - Usually start with an **8-week trial of PPI + general measures (scope if alarm symptoms)** - If symptoms **controlled -\> stop** - If recurrence **within 3 months** -\> continue medical Rx **long-term + scope** (needs to be off PPI prior to Dx H pylori) - If recurrence **after 3 months** -\> **repeat 8-week trial + scope** (needs to be off PPI prior to Dx H pylori) - If any evidence of **severe oesophagitis or barrett's**, continue **long-term** - In **pregnancy** -\> diet and lifestyle modification, **antacids + sucralfate** (antacids containing **sodium bicarbonate and magnesium trisilicate should be avoided**) -\> fail to respond = **H2B** -\> PPI

Question 26

PPI MOA?

Answer 26

Binds irreversably to the hydrogen/potassium ATPase enzyme on gastric parietal cells and block the secretion of hydrogen ions.

Question 27

GORD: Surgical indications and Aims

Answer 27

Indications: 1. Chronic GORD and want to avoid lifelong medications 2. Symptoms on medical management 3. HH and GORD AIMS: Restore the anatomy and function of the anti reflux barriers by reducing any hiatus hernia and GOJ into the abdomen and creating a competent LOS by fundoplication + tightening the oesophageal hiatus

Question 28

What is Barrett's Oesophagus

Answer 28

The adaptive replacement of the normal stratified squamous epithelium of the distal oesophagus with metaplastic columnar epithelium as a result fo sustained exposure to reflux. The z-line is situated more that 1cm from the GOJ. If less than 3cm the short segment, if more then long segment. 2% prevalence White dudes → white to african american 20:1, M:F 1.7:1 histopath: intestinal metaplasia = intestinal type crypts lined by goblet and columnar cells

Question 29

Risk Factors for Barrett's Oesophagus

Answer 29

Symptoms - nocturnal reflux and GORD symptoms for \> 5 yrs Demographics: age\>50, male, white, hiatus hernia Lifestyle: obesity and smoking Family Hx - of barretts or oesophageal cancer

Question 30

Pathogenesis of Barrett's

Answer 30

**Develops as an adaptive response to cell loss secondary to chronic inflammation in the setting of GORD.** ## Footnote Clonal expansion of metaplastic cell population that can survive in low pH Inflammation drives cycle of proliferation and accumulation of genetic defects - **p53 and p16** in submucosal oesophageal glands + loss of e-CADHERIN Leads to dysplasia adenocarcinoma sequence - high grade dysplasia has nuclear pleomorphism and loss of crypto architecture, low grade has loss of cell differentiation and loss of goblet cells. Carcinoma when invasion past basement membrane. 0.25% per year progress to cancer

Question 31

Natural History of Low Grade dysplasia

Answer 31

Characterised by mild to moderate atypic, loss of cellular differentiation, crowded elongated and hyperchromatic cells, loss of goblet cells Majority will regress. 0.5% per year progress to cancer

Question 32

Natural History and Features of High grade dysplasia

Answer 32

15-59% progress. **4-8%/yr progress to cancer** **(there is a 40% rate of occult cancer in resected specimens)** Severe atypia, loss of crypt architecture, nuclear pleomorphism, and increased mitoses. Histopathology of nondysplastic Barrett's esophagus (a, NDBE), low-grade dysplasia (b, LGD), high-grade dysplasia (c, HGD), and (d) cancer. Yellow arrow in a indicates goblet-cell-positive mucosa adjacent to the squamocolumnar junction. **Goblet cells are the hallmark for the diagnosis of Barrett's esophagus without dysplasia**

Question 33

Risk factors for progression to cancer in Barrett's Oesophagus

Answer 33

**Length of Barretts segment** **Extent of high grade dysplasia and Low grade Dysplasia** ?aspirin with PPI is protective

Question 34

Describe the Prague Criteria for Barretts Oesophagus? Barrett's on endoscopy - proximal displacement of the squamous-columnar epithelium, salmon pink columnar mucosa with tongue extensions proximally or columnar islands.

Answer 34

C and M criteria C - extent of the circumferential involvement in comfort OGJ M - maximal length (including tongues but not islands) in cm from the GOJ

Question 35

What is the Seattle protocol?

Answer 35

**Seattle protocol**: biopsy of any **mucosal irregularity** and **four quadrant** biopsies **every 2 cm** including normal mucosa proximally, unless known or suspected dysplasia then **four quadrant biopsies every 1 cm** ## Footnote **Every 2 years**

Question 36

Management of Barretts

Answer 36

1. Indefinite PPI 2. Consider fundoplication 3. Surveillance, recommendations vary according to presence of dysplasia American Gastroenterology association * Barretts without dysplasia-short repeat in 3-5, long 2-3 * indefinite - repeat after 8-12 weeks high dose PPI * LG dysplasia: **Repeat OGD** with 4 quadrant bx every 1cm in **8-12 weeks** after **high dose PPI -** if confirmed to be LGD =\> can either continue to **survey every 6months** with **4 quadrant bx every 1cm + high dose PPI** or refer for **endoscopic eradication**(resection/ablation) or **anti-reflux surgery. Surveillance 2yearly if regresses to non dysplasia on two consecutive examinations.** * **HG dysplasia: Repeat OGD** with 4 quadrant bx every 1cm in **8-12 weeks** after **high dose PPI if confirmed by 2nd expert pathologist needs endoscopic eradication**

Question 37

Treatment of High Grade Dysplasia in Barretts

Answer 37

Goal - removal of all dysplastic and metaplastic tissue * Endoscopic resection of an =visible mucosal irregularities prior to ablation. If specimen shows invasion of submucosa requires oesophagectomy. If mucosal - RFA (radiofrequency ablation) remainder of disease. * Oesophagectomy (5yr survival 90%) only therapy that removes all neoplastic epithelium + occult malignancy + regional lymph nodes → risk of LN mets only 1% in HGD. BUT significant morbidity and mortality (2-5% at high volume centres) → CONSIDER with l**ong segment/multifocal disease** not amenable to endoscopic Mx

Question 38

Treatment of Intramucosal Cancer

Answer 38

* **Stage patient: CT CAP, PET, diagnostic lap, EUS for ?EMR** * Risk of LN mets → 3% with T1a (invades lamina propria or muscularis mucosa) → **13% with T1b (invades submucosa)** * **Oesophagectomy OR** * **Endoscopic mucosal resection** - Indicated for **T1a** lesions with no adverse histological features → **\<2cm, Well-differentiated, No lymphovascular invasion, Not ulcerated** - Extensive submucosal invasion (in T1b) carries high risk of LN mets and is a contraindication unless the patient is not a candidate for curative surgery and chemotherapy - Contraindicated in patients with a bleeding tendency, untreated oesophageal varices, lesions associated with an oesophageal diverticulum - In appropriately selected patients, long-term outcomes are comparable to surgical treatment, fewer complications - Requires **3 monthly endoscopies and biopsies** post-op - Higher rate of recurrent/metachronous tumours (20%) → can usually be symptoms prior treated with further EMR (less if combined with ablative therapy)

Question 39

Type of Hiatus Hernia?? Hiatus hernia results from derangements in the normal anatomy - attenuation of the **phreno-oesophageal ligament** and laxity of the hiatus. The phrenology-oesophageal ligament is the fused end-thoracic and endo-abdominal fascia that circumferentially inserts into the oesophageal musculature close to the squamous-columnar junction.

Answer 39

Type 1 - sliding (90%)L gastric cardia and LOS herniates into chest + phrenology-oesophageal ligament is attenuated but intact Type 1 - true para-oesophageal hernia (3%).GOJ remains in usual position and gastric fungus herniates through the hiatus Type 3 - mixed (7%). type one defect enlarges and fungus and upper stomach herniate into the chest. Predisposes to gastric volvulus. Type 4 - large hiatus hernia with other organs in the hernia sac (1%) → 5% incidence of acute symptoms and volvulus Giant Paraoesophageal hernia: ½ stomach + hernia \>6cm on endoscopy + 5cm crural distance

Question 40

Indications for Surgery in Hiatus Hernia

Answer 40

Type 1 - if symptomatic refractory to medical therapy or patient prefers surgery All other types should be offered an operation if surgically fit due to high rate of complications if needing emergency repair (gastric volvulus, uncontrolled bleeding, strangulation, perforation or respiratory compromise). There is a 30% mortality after emergency surgery vs 1% for elective cases ***30%*** ***of asymptomatic patients w/ para-oesophageal hernias will potentially develop devastating complications - such as strangulation or perforation***

Question 41

Principles of Hiatus hernia Surgery?

Answer 41

1. Complete reduction and excision of the hernia sac 2. Mobilise and reduce herniated stomach and 2-3 cm of distal oesophagus without tension 3. Repair of the diaphragmatic hiatus (close crural defect) 4. Fixation of the stomach into the abdomen with fundoplication or gastropexy Fundoplication → division of short gastric will increase mobilisation and improve exposure. Benefits decreased port op GORD and recurrence Gastropexy → allows permanent fixation in the abdominal cavity and decreased possibility of reherniation

Question 42

Types of Hiatus Hernia Repair Failure? MORTALITY 1.4%, leak rate 2.5%

Answer 42

1A: fundoplication slips into chest 1B: Hiatus hernia recurs but wrap remains in abdomen 2: paraoesophagheal hiatus hernia 3: Wrap malpositioned Patients with long standing HH have delayed gastric emptying after repair due to atrophic gastric musculature and vagal neuropraxia during dissection

Question 43

What is a cameron's Ulcer

Answer 43

Ulceration on the mucosal folds lining the stomach where it is constricted by the thoracic diaphragm Delay surgery until healed - 6 weeks, high dose PPI

Question 44

What is Gastric volvulus

Answer 44

Rotation of the stomach \>180 degrees around a fixed axis → associated with strangulation in up to 30% and mortality in 15-20%

Question 45

Anatomical Classification of Gastric volvulus?

Answer 45

Organoaxial (60%) around longitudinal axis - strangulation in up to 30%. Predisposed by large paraoesophagheal hernia Mesoentericoaxial - 30%: around the transverse axis

Question 46

What is Borchardt's Triad

Answer 46

1. **Severe epigastric pain** 2. **Retching and inability to vomit** 3. **Inability to pass an NG tube** Other symptoms of gastric volvulus - chest pain, dysphagia, high gastric outlet obstruction. **Hypokalaemic, hypochloraemic, metabolic alka**losis (vomiting)

Question 47

What does this XR show?

Answer 47

CXR demonstrating a retro cardiac air fluid level and another one below the diaphragm. Barium swallow with obstruction at the level of the volvulus would confirm the diagnosis of **GASTRIC VOLVULUS** ## Footnote ▪ organoaxial volvulus -\> lies in a **horizontal plane** * *▪** mesenteroaxial volvulus -\> **spherical stomach on supine** images but- Due to **anatomical abnormalities** that result in abnormal mobility of the stomach **two air-fluid levels on upright** films, with the **antrum positioned superior to the fundus** * *▪ Abnormal course of NGT**

Question 48

Management of Gastric Volvulus

Answer 48

**Goals**: 1. Resuscitate 2. Restore anatomy 3. Repair defects 4. Prevent future rotations 1. IV FLUIDS 2. NG decompression, if fails endoscopic. 3. Emergency OT if decompression unsuccessful → reduce hernia, release volvulus, debride non viable tissue, hiatal closure, anterior gastropexy/fundoplication to prevent recurrence

Question 49

Peptic Stricture pathophysiology?

Answer 49

Complication of chronic GORD that occurs in 5% of people with oesophagitis. Progressive inflammation and ulceration involving the submucosa and muscular mucous → damage to muscle and intrinsic nerves → collagen deposition → formation of scarring and structuring Usually short and contiguous with GOJ Mx dilate + PPI

Question 50

Caustic Oesophageal Injury

Answer 50

* 30-50% will develop strictures after caustic injury * Manage with endoscopic dilatation - delay 6 weeks post injury to minimise risk of perforation * 0.5% risk of perforation at dilatation * Consider surgery if young patients as likely to require multiple dilatation with cumulative risk of perforation + RISK of oesophageal Cancer is increased 1000x after caustic injury + 16% risk of SCC *

Question 51

What is an A ring?

Answer 51

Normal smooth **muscle** contraction of the oesophagus just proximal to the squamocolumnar junction (thickened symmetrical band of muscle) → strongest part of LOS.

Question 52

What is a B ring?

Answer 52

A B ring is a **mucosal** structure (smooth and thin) at thsquamocolumnar junction. A Schatzki's ring is a narrowed B ring. 6-14% of barium swallows for dysphagia Schatzki's ring found on 13% of endoscopies for dysphagia.

Question 53

What is an oesophageal Web?

Answer 53

* Thin \<2mm eccentric membrane protruding into the oesophageal lumen * covered in squamous epithelium - mucosa + submucosa * usually cervical oesophagus * Causes focal narrowing, most commonly in the post cricoid area

Question 54

What is Plummer Vinson Syndrome?

Answer 54

Characterised by iron deficiency anaemia, dysphagia and cervical oesophageal webs May also have glossitis, angular chelitis, koilnychia, splenomegaly, enlarged thyroid and dermatologist findings of bullies pemphigus.

Question 55

How do you diagnose Eosinophilic Oesophagitis?

Answer 55

All of… 1. Symptoms of oesophageal dysfunction 2. Eosinophil predominant inflammation on biopsy **(\>15/HPF)** 3. Other causes excluded Endoscopic findings include: stacked circular rings (44%), strictures (21%), linear furrows and white papules.

Question 56

Key Findings in Achalasia

Answer 56

Increased LOS resting pressure (\>45mmHg) Fails to relax completely after swallowing peristalsis in distal ⅔ or simultaneous non peristaltic contractions

Question 57

What is diffuse oesophageal spasm?

Answer 57

A motility disorder where normal peristalsis is replaced by simultaneous, repetitive high amplitude disordered contraction Physiology unclear. F\>M, 5 x less common than achalasia. Worsened by emotional stress(associated with IBS/pyloric spasm) - psych input + cold liquids, acid reflux and biliary colic Oesophagogram - corkscrew oesophagus (tertiary contractions and late disease), distal bird beak narrowing **Manometry: simultaneous high amplitude or long duration, multi peaked contractions (120mmHg, \<2.5sec) occurs after \>10% wet swallows**

Question 58

Treatment of Diffuse Oesophageal Spasm

Answer 58

1. Psych Evaluation 2. Eliminate Food Triggers → acid suppression if trigger 3. Meds - nitrites, Ca channel blockers, sedatives, anticholinergics + peppermint 4. Endoscopy - bougie dilatation - up to 50-60F provides relief from dysphagia in 70-80% + botox 5. Surgery - Indications are failure of endoscopic/medical mx with ongoing severe symptoms or pulsing diverticulum in thoracic oesophagus - **Long oesophagomyotomy encompassing entire length manometric abnormality to LOS + Dor fundo** (surgery interrupts phrenology-oesophageal ligament. OR POEM

Question 59

What is Nutcracker/ Jackhammer Oesophagus?

Answer 59

Hyper contractile oesophagus. Affects oesophageal body; hypertensive peristalsis or high amplitude peristaltic contractions. High-amplitude contractions (\>180mmHg) with normal peristalsis during standard manometry → normal LOS, relaxation after every wet swallow, distal contractile integral \>8000 Commonest motility disorder. M=F oesophagogram may be normal Mx: avoid triggers, muscle relaxants, endoscopic bogie dilatation

Question 60

Systemic Sclerosis? CREST?

Answer 60

Autoimmune collagen vascular disease characterised by thickening, oedema and sclerosis of the skin associated with subcutaneous calcinosis * C-Calcinosis * R - Raynaud's phenomenon * E-oesophageal Dysmotility * S - sclerodactyly * T - Telangiectasia Visceral involvement is rare apart from the oesophagus → affected in 80%: smooth muscle atrophy affecting LOS, weak peristalsis, reflux is common Manometry - almost no peristalsis or loss of tone Mx - PPI, manage strictures and complications

Question 61

Integrated Relaxation Pressure (IRP) is the mean of the 4 seconds of maximum deglutive relaxation in the 10 second window beginning at the upper oesophageal sphincter relaxation referenced to gastric pressure Distal contractile integral is the amplitude x duration x length of the distal oesophageal contraction exceeding 20mmHg from the transition zone to the proximal margin of the lower oesophageal sphincter.

Answer 61

Question 62

What is hypertensive Lower Oesophageal Sphincter

Answer 62

This is when the resting pressure of the LOS \> 45mmHg with normal relaxation and peristalsis. It is though to be evolving achalasia. Oesophagogram demonstrates narrowing at the GOJ and delayed flow. Manometry shows a median integrated relaxation pressure of 15mmHg, elevated LOS pressure \>26mmHg w incomplete relaxation + normal(50%)/hypertensive peristalsis Mx: Endoscopy + botox/dilatation or Laparoscopic hellers myotomy + fundo as anti reflux

Question 63

What is Allgrove Syndrome (or AAA/triple A syndrome)

Answer 63

Autosomal recessive genetic disorder Achalasia, adrenal insufficiency and absence of tears (alacrimia) :( Associated with progressive neurological impairment

Question 64

What is the Aetiology of Achalasia? **Achalasia is associated with a 40x risk of SCC**

Answer 64

**Unknown/ incompletely understood** Hypothesis is that there is progressive loss of ganglion cells in the myenteric plexus of auerbach. Inhibitory NO-producing neurons most severely affected → failure to relax oesophageal smooth muscles + aperistalsis. The cholinergic neurons that contribute to LOS tone are relatively spared. As the syndrome is often accompanied by an inflammatory infiltrate - theatrics are that there may be an underlying viral or autoimmune pathology. Secondary achalasia occurs due to chagas (trypanosoma cruzi) or pseudoachalasia due to a tumour at the LOS/cardia or invasion of the neural plexus/ following reflux surgery if wrap too tight.

Question 65

What is achalasia

Answer 65

Achalasia is the failure of the lower oesophageal sphincter to relax with the absence of peristalsis. Primary form is uncommon - 1/100,000 people/yr in western countries idiopathic or infectious neurogenic degeneration due to emotional stress, trauma, post viral, rapid weight loss or Chagas disease. 8% chance of developing adenocarcinoma over 20 yrs. Likely due to long standing retained undigested fermenting foods causing mucosal irritation. SCC not adeno. But can be adenocarcinoma in the middle ⅓ at the site of the greatest mucosal irritation. No guidelines for surveillance as absolute risk is still low despite 40x increased risk.

Question 66

Achalasia Oesophagogram (Barium swallow)

Answer 66

* Hold up in distal oesophagus, dilatation of oesophageal body, peristaltic dysfunction, tapering to ‘birds beak’ stricture of distal oesophagus * **dilated oesophagus with distal narrowing** → birds beak * sphincter spasm, dilated oesophageal body, delayed emptying * Lack of gastric bubble (tight LOS not allowing air to pass) * **LATE PHASE → megaoesphagus, (\>6cm) sigmoidal oesophagus, massive oesophageal dilatation**

Question 67

Achalasia Manometry

Answer 67

Lack of peristaltic waves and failure of LOS to relax 5 classic findings: 2 regarding the LOS and 3 regarding the oesophageal body… 1. Hypertensive LOS → pressure \>35mmHg 2. LOS does not relax with swallowing 3. Above baseline pressure in oesophageal manometry 4. Simultaneous mirrored contractions in the oesophageal body without progressive peristalsis 5. Low amplitude wave form in the body indicating a lack of muscle tone.

Question 68

Achalasia Management

Answer 68

GOAL of management is to **reduce the pressure** at the lower oesophageal sphincter to a level that the sphincter **no longer impedes the passage of digested material.** Can be done by .. * **mechanical disruption of muscle fibres** of LOS from * **pneumatic** **dilation** * **heller's myotomy** * peroral endoscopic myotomy (**POEM**) * **pharmacological reduction** in LOS pressure through * **botox** **injection** * **oral** **nitrates** * **No treatment to reverse** primary pathology * All treatments only improve swallowing and **efficacy reduces** over time * Thus pts need **longterm f/up** and **alternative mx**

Question 69

Zenker's Diverticulum - Pharyngo-oesophageal

Answer 69

Due to loss of tissue elasticity False diverticulum - doesn't contain all layers of oesophageal wall (mucosa and submucosa) Found at Killian's triangle → between oblique fibres of the thyropharygeus and horizontal fibres of cricopharyngeus (2 parts of inferior constrictors). Mucosa/submucosa dissect down left side of oesophagus into the superior mediastinum and then posteriorly into the prevertebral space. Treatment - open repair through the side of the neck. \<2cm → myotomy of proximal and distal thyropharyngeus and cricopharyngeus. \>2cm myotomy and the excision of sac. Diverticulopexy → fixing sac to posterior pharynx (not prevertebral fascia)

Question 70

What is the Aetiology of Oesophageal Varices?

Answer 70

A rise in portal system pressure causes reversal of flow from the portal to systemic circulation. Venous bypasses develops wherever portal & systemic circulations share common capillary beds. Varices do not develop until hepatic pressure gradient \> 10mmHg and bleeding at \>12mmHg → primary endpoint for therapy. 30% of patients with cirrhosis and portal hypertension develop varices 30% of patients with varies develop bleeding. Every episode of bleeding is associated with a 30% mortality