Motility Disorders Of Esophagus

MOTILITY DISORDERS OF ESOPHAGUS PROF DR OLCAY ALVER

Cerrahpaşa Medical Faculty, Department of General Surgery

MOTILITY DISORDERS OF ESOPHAGUS PROF DR OLCAY ALVER

Cerrahpaşa Medical Faculty, Department of General Surgery

MANOMETRIC FEATURES OF ACHALASIA 

1) APERISTALSIS or HYPOPERISTALTISM

(incoordinated,feeble,simultaneous,and repetetive peristaltic pattern within the 2/3 of esophageal body) 

2)BASAL HYPERTONICITY OF LES (pressure>45mmHg)

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3)INCOMPLETE RELAXATION OF LES IN RESPONSE TO SWALLOWING (<70%)

NEUROPATHOLOGY  

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OF

ACHALASIA

Denervation of PRE-GANGLIONIC CHOLINERGIC NERVE supply to esophagus Denervation of INHIBITORY( relaxing NONADRENERGIC,NON-CHOLINERGIC) nerves to LES Absence or degeneration of GANGLION CELLS OF AUERBACH’S MYENTERIC PLEXUS Ultrastructural alterations of VAGUS NERVE(WALLERIAN DEGENERATION) Decreased and distorted motor cells of VAGAL DORSAL NUCLEUS in brainstem

FUNCTIONAL NEUROPHARMACOLOGY OF ACHALASIA 

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Supersensitivity (excessive motor response)of LES to CHOLINERGIC drugs-CANNON’S LAW of DENERVATION SUPERSENSITIVITY Supersensitivity of LES to GASTRIN EDROPHONIUM (anti-cholinesterase)↑ LES pressure(=postgn. Cholinergic nerves to LES are intact) CHOLECYSTOKININ invokes a paradoxal excitatory response of LES (=dysfunction of post-gn. inhibitory /non-adr +nonCh nerves) Marked ↓ in VIP nergic nerve fibers and NEUROPEPTID-Y (neurotransmitters of LES relaxation) Marked ↓ in NITRINERGIC NEURONS(=↓ NO concentration=inhibitory neurotransmitter) ACHALASIA=NİTRİNERGİC NEURONITIS,AUTO-İMMUNE DIS.?

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SYMPTOMS OF ACHALASIA

1)DYSPHAGIA:80-100% paradoxal

dysphagia; adoption of certain manoevres to facilitate swallowing; odynophagia 

2)REGURGITATION : 70% undigested

food,foamy mucoid saliva ; nocturnal regurgitation,and aspiration into the respiratory tract; halitosis, foul eructation 

3)WEIGHT LOSS : 40% sitophobia; odynophagia; anemia ; avitaminosis ;cachexia

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4)CHEST PAIN : 30% early stage;vigorous achalasia :odynophagia

DIAGNOSIS OF ACHALASIA

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RADIOLOGICAL FEATURES Proximal esophageal dilatation Smoothly tapered distal end Fluid level in post mediastinum Absence of gastric air bubble ENDOSCOPIC FEATURES Esophageal dilatation+retained food Retention esophagitis Passage of scope through resistant LES produces characteristic “pop” sensation Endoscopic biopsy excludes esophageal ca MANOMETRIC FEATURES

ACHALASIA

DIFFERENTIAL DIAGNOSIS OF ACHALASIA achalasıa

Diffuse spasm

Vigorous achalasia

SYMPTOM + SIGN Pain Uncommo Almost Frequent n always Sometime Nearly obstruction s Always always Frequent Regurgitat. Frequent Retention Common Rare Frequent Never Dilatation Common Never Spasm Uncommo Frequent

Occasiona l

TREATMENT OF SPASTIC MOTOR DISORDERS OF ESOPHAGUS    

PHARMACOTHERAPY

SMOOTH MUSCLE RELAXANTS NITRATES ( ISOSORBIDE DINITRATE) CALCIUM-CHANNEL BLOCKERS

(NIFEDIPINE,VERAPAMIL)    

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ANTICHOLINERGICS , SILDENAFİL HYDRALAZINE(ARTERİAL MUSCLE PSYCHOTROPIC AGENTS BOTULİNUM TOXİN

RELAXANT)

NON-PHARMACOLOGIC MEASURES

REASSURANCE, BEHAVIOR MODIFICATION/BIOFEEDBACK ESOPHAGEAL DILATATION OPEN OR LAPAROSCOPIC MYOTOMY ± PARTIAL FUNDOPLICATION ( H E L L E R + D O R procedure)

DIFFUSE ESOPHAGEAL SPASM 

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Hypertrophy and spasms of smooth musle layers of the esophageal body,Wallerian degeneration of the vagal fibers,denervation supursensitivity to cholinergic compounds MAJOR CLINICAL SYMPTOMS INTERMITTENT SUBSTERNAL PAIN-varies from slight discomfort to severe spasmodic pain mimicking ANGINA PECTORIS with radiation to back,jaws,arms aggravated by emotional upsets,very hot,cold,carbonated drinks DYSPHAGIA / HEARTBURN /ODYNDPHAGIA / no weight loss

MANOMETRIC FEATURES OF DIFFUSE ESOPHAGEAL SPASM 

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Synchronous,non-spesific , repetetive(at least three peaks) peristaltic contractions with prolonged duration (>6 sec),highamplitude (>120mm) Failure of hypertensive LES to relax completely in 30% of the cases Denervation supersensitivity to cholinergic,anticholinesterase,ergonovine compounds

DIAGNOSIS OF DIFFUSE ESOPHAGEAL SPASM  

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RADIOLOGICAL FEATURES

Delayed esophageal transit due to segmental spasms, areas of narrowing , irregular uncoordinated peristalsis described as “ C U R L I N G “ , “ C O R K S C R E W “ or “ R O S A R Y B E A D ESOPHAGUS “ and “PSEUDODİVERTICULOSIS“ involving the two-thirds of the esophageal body with coexisting small sliding hiatal hernia, epiphrenic diverticula ENDOSCOPY- Normal unless reflux esophagitis or other lesion is associated MANOMETRY

TREATMENT OF DIFFUSE ESOPHAGEAL SPASM     

MEDICALLong-acting nitrates,isosorbide,ca-channel blockers(nifedipine,verapamil) Bougienage,pneumatic dilatation SURGICAL- long esophageal myotomy(Long HELLER operation) Extending from LES to aortic arch + anterior hemifundoplication(DOR procedure )

HYPERCONTRACTING ESOPHAGUS    

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“NUTCRACKER” / HYPERTENSIVE ESOPHAGUS Hıgh-amplitude contractions(>180 mmHg) Prolonged duration contraction (>6 sec) Normal peristalsis

HYPERTENSIVE LES

Hıgh resting LES pressure (>45mmHg) Normal or incomplete LES relaxation Normal peristalsis

HYPOCONTRACTING ESOPHAGUS  

INEFFECTIVE ESOPHAGEAL MOTILITY

NONSPECIFIC ESOPHAGEAL MOTOR DYSFUNCTION

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FREQUENT NON-PROPULSIVE OR RETROGRADE CONTRACTIONS (30% or more)

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LOW-AMPLITUDE CONTRACTIONS (< 30mmHg)

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PROLONGED DURATION CONTRACTION(>6 Sec) BODY PERISTALSIS WITH NORMAL LES

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LOW RESTING LES PRESSURE (< 10mmHg )

PATHO PHYS IOLO GY OF SCLE RODERMA  

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NEURAL DYSFUNCTION OF ESOPHAGUS Normal sphincteric response to cholinergic drugs but not to gastrin or a cholinesterase inhibitor/ loss of intrinsic Ach mechanism(?) RAYNAUD’s phenomenon usually present Functional abnormalities precede morphologic changes by light microscopy OTHER POSSIBLE PATHOGENIC MECHANISMSabnormal fibrotic process ; marked smooth musle atrophy with fibrous replacement / primary muscle disease:smooth muscle atrophy with aperistalsis / evidence lacking vascular abnormality / Raynaud’s phenomenon /prolonged warming time after swallow of cold bolus

CLINICAL FEATURES OF SCLERODERMA 

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SYMPTOMS: DYSPHAGIA / HEARTBURN / REGURGITATION / SKIN + SYSTEMIC CHANGES OF SCLERODERMA / RAYNAUD’S PHENOMENON SIGNS: SKIN CHANGES / RAYNAUD’S PHENOMENON , C R E S T SYNDROME Calcinosis cutis Raynaud Esophageal involvement Sclerodactyly Telangiectasia

DIAGNOSIS OF SCLERODERMA 

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RADIOGRAPHY: aperistalsis of distal 2/3 esophagus/mild dilatation / gastroesophageal reflux / possible peptic stricture + shortening of distal esophagus/impaired esophageal clearence MANOMETRY:Upper sphincter+ 1/3 esophagusNORMAL / feeble or no peristalsis (<30mmHg)in distal 2/3 esophagus / feeble or sphincter(LES absent lower esophageal p-<10 mmHg ) ENDOSCOPY:mildly dilated,tubular esophagus /distal esophagus and LES remains open/ free reflux /peptic esophagitis with possible stricture formation(60%)

ESOPHAGEAL SCLERODERMA (LONG TUBULER STRICTURE)

TREATMENT OF SCLERODERMA  

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MEDICAL TREATMENT Proton pump inhibitors/ prokinetic drugs (metoclopramide,domperidon) SURGICAL TREATMENT Combined gastroplasty+antireflux procedure(COLLIS-NISSEN ± BELSEY MARK IV ) / DISTAL ESOPHAGECTOMY replacement with a colonic or an isoperistaltic jejunal segment

ESOPHAGECTOMY + COLONIC INTERPOSITION