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Joel A. Ricci MDSUNY Downstate Medical Center
Lutheran Medical Center Department of Surgery
June 26, 2009
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HistoryXx year old female with worsening dysphagia and solid food regurgitation for 2 daysOther symptoms included: poor appetite and Other symptoms included: poor appetite and occasional epigastric tendernessDenied weight loss, fever, dysuria or changes in bowel habitsKnown hx of Achalasia (2006): refused Tx at the timePMH NIDDM HTN h li id i GERD?PMHx: NIDDM, HTN, hyperlipidemia, GERD?No Tobacco or EtOH useMeds: Lipitor Glipizide Zolpidem Toprol XL PrilosecMeds: Lipitor, Glipizide, Zolpidem, Toprol XL, Prilosec
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Physical ExamVital Signs: T: 97.8°F, BP: 169/81 mmHg, HR: 100 b/mGen: AAO x 3, NADHEENT: PERRL, moist mucous membranes, no icterusCV: RRR, S1S2, no murmurs or gallopsLungs: CTA b/l Abd: Soft, NT/ND, +BSExt: 2+ pulses throughout, no edema
L b k blLabs were unremarkable
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ImagingChest x‐ray: Large bullae in Right upper lobe extending into lower neck
Chest CT Scan: Diffusely dilated esophagus with food residues and mildly compressed airway residues and mildly compressed airway
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Chest X‐Ray
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CT Scan
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CT Scan
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CT Scan
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ProcedureThoracoscopic Heller myotomywith intra‐operative Esophago‐gastroduodenoscopy (EGD)
EGD: Dilated esophagus with spastic LESEGD: Dilated esophagus with spastic LESLeft side decubitus4 VATS incisions Adhesiolysis; Esophagus encircled w/ penrose drainNarrowed tapering of LES visualizedMuscular layer divided w/ scissors 5 cm onto proximal Muscular layer divided w/ scissors 5 cm onto proximal esophagus and 2 cm beyond the GEJEGD confirmed adequate passageGas insufflation confirmed no mucosal perforation
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Post‐op CoursePOD #1:
UGI Series: No obstruction or leakageTolerated clear liquids
POD #2: T l t d f ll li idTolerated full liquidsDischarged home
Currently (4 months post 0p):Currently (4 months post‐0p):Adequate relief of dysphagia, no complaints
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Upper GI Series
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Upper GI Series
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Upper GI Series
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Joel A. Ricci M.D.SUNY Downstate Medical CenterSUNY Downstate Medical Center
Department of SurgeryJune 26, 2009
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Esophageal DiseaseMotility DisordersGERD }See previous presentationsBarrett’s Esophagus Esophageal Cancer
}See previous presentations
by yours truly
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l dMotility DisordersClassification based on manometry
AchalasiaInadequate LES relaxation
Diffuse Esophageal SpasmUncoordinated contraction
N k E hNutcracker EsophagusHypercontraction
Ineffective Esophageal MotilityIneffective Esophageal MotilityHypocontraction
Spechler et al. Gut 49:145-151, 2001
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h lAchalasiaSymptomatology
Progressive dysphagia Liquids to solids
Chest painAspirationR i iRegurgitationWeight loss
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A h l iAchalasiaEtiology
Complex motor abnormality of the LES6 in 100,ooo individuals2nd most common functional disorder of the esophagus requiring surgery (GERD = 1st)C i k d diff h iCause is unknown: data suggests different theories
HereditaryDegenerativeDegenerativeAutoimmuneInfectiousInfectious
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h lAchalasiaPathophysiology
T‐lymphocyte, eosinophil, and mast cell infiltration in the myenteric (Auerbach) plexus
Myenteric neural fibrosisMyenteric neural fibrosisHypertrophy of the two muscle layers and nerve fibersDegeneration of NO and producing inhibitory neurons
Affects relaxation of LES Basal LES pressure rises
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h lAchalasiaManometry
Manometric features:Incomplete LES relaxationrelaxationElevated resting pressure (>45 mmHg)
l fAperistalsis of esophageal bodyElevated lower esophageal pressure
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h lAchalasiaWork‐up
Chest X‐rayAbsence of gastric b bblbubbleDilated fluid filled esophagusp gRight side posterior mediastinal shadow
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AchalasiaWork‐up
Barium SwallowAir fluid level“ d’ b k”“Bird’s beak”
Fl i i iFluoroscopic imagingFlaccid non‐peristaltic esophagusesophagusAbsence of “stripping” waves
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h lAchalasiaWork‐up
EGDNarrowed distal lumen“ k” l d f d“Stuck” solid food particlesRule out “pseudo‐Rule out pseudoachalasia” caused by obstructing tumor in di t l hdistal esophagus
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h lAchalasiaTreatment
Pharmacologic TreatmentIsosorbide dinitrate
Reduces LES 66% for 90 minutes
NifedipineReduces LES pressure 30‐40% for > 60 minutes
BotulinumToxin InjectionBotulinumToxin InjectionInhibits acetylcholine release60 – 80% relief50% recurrence within 6 monthsmonthsObliterates plane btwnmucosa & submucosaIncreased rate of perforation during surgeryduring surgery
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h lAchalasiaTreatment
Pneumatic DilatationSuccess increases with repeat dilatations60‐80% success rate; 5yr recurrence rate: 50%
West RL, et al. Long term results of pneumatic dilation in achalasia followed for more than 5 years. Am J Gastroenterol. 2002 Jun;97(6):1346-51.
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h lAchalasiaManagement Algorithm
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h lAchalasiaSurgicalTreatment
Heller MyotomyThoracoscopicLaparoscopic
FundoplicationGERD tiGERD preventionNissenDorDorToupet
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h lAchalasiaWhich treatment is better?
Zaninotto et al: Randomized controlled trialBotox (n = 40) vs Myotomy w/ fundoplication (n = 40)Both groups initial improvement of symptoms6 months: 45% recurrence in Botox group ( t f ) 8 % t % B t2 years (symptom free): 87% myotomy; 34% Botox
Csendes et al: Prospective randomized trialPneumatic dilatation (n 20) vs Myotomy (n 18)Pneumatic dilatation (n = 20) vs Myotomy (n = 18)3.5 years (no dysphagia): 100% myotomy
60% pneumatic dilatation60% pneumatic dilatation1. Zaninotto et al. Randomized controlled trial of botulinum toxin versus laparoscopic heller myotomy for
esophageal achalasia. Ann Surg. 2004 Mar;239(3):364-702. Csendes A, et al. Late results of a prospective randomised study comparing forceful dilatation and
oesophagomyotomy in patients with achalasia Gut. 1989 Mar;30(3):299-304
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h lAchalasiaHeller Myotomy
Ernest Heller; 1913Both ant & post LES
l fib di t dmuscle fibers disruptedModified version
Single anterior Single, anterior, longitudinal myotomy
Standard operative ptechnique
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h lAchalasiaThoracoscopic Esophagomyotomy
Port placement Division of muscle fibers
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h lAchalasiaLaparoscopic Heller Myotomy
Patient positioning Port placement
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h lAchalasiaLaparoscopic Heller Myotomy
Identification of LES Division of muscle fibers
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h lAchalasiaFundoplication
Dor (Anterior fundoplication) Toupet (Posterior fundoplication)
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h lAchalasiaExtent of Myotomy
From GEJ: Proximal (5 – 6 cm); Distal (1.5 – 3 cm)
Chen et al: 7 to 16 years post myotomy and fundoplication67% incidence of epiphrenic pseudo‐diverticulumLikely caused by absence of coverage over proximal extent of myotomy
Oelschlager et al: Standard (1.5 cm) vs Extended (3 cm) distal myotomy ( n = 110 pts)
Lower post‐op LES pressures with extended (9 5 mmHg) vsLower post‐op LES pressures with extended (9.5 mmHg) vsstandard (15.8 mmHg)Improved dysphagia24‐hr pH monitoring: no increase in GERD
1. Chen LQ et al. Long-term effects of myotomy and partial fundoplication for esophageal achalasia. Dis Esophagus. 2002;15(2):171-9.
2. Oelschlager BK et al. Improved outcome after extended gastric myotomy for achalasia. Arch Surg. 2003 May;138(5):490-5; discussion 495-7.
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h lAchalasiaAddition of Fundoplication
Myotomy:Lowers esophageal outflow resistanceImproves esophageal emptyingIncreases propensity for GERD
Is Fundoplication always needed?
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h lAchalasiaAddition of Fundoplication
Richards et al: Randomized trialHeller myotomyw/ Dor vs w/o Dor ( n = 43)Post‐op GERD (by 24‐hr pH monitoring)p y p g
47.5% in pts w/ Heller myotomy alone9.1% in pts w/ added Dor fundoplicationNo difference in LES pressure or dysphagia scores
Rice et al: Retrospective studyHeller with and w/o Dor (n = 149)Decreased incidence of GERD (by 24‐hr pH monitoring) following fundoplicationFundoplication did not decrease esophageal emptying time ( d b b i h h )(assessed by barium esophagography)
1. Richards WO, et al. Heller myotomy versus Heller myotomy with Dor fundoplication for achalasia: a prospective randomized double-bline clinical trial. Ann Surg 2004; 240(3):405-415.
2. Rice TW, et al. A physiologic clinical study of achalasia: Should Dor fundoplication be added to Heller myotomy?. J Thorac Cardiovasc Surg 2005; 130(6):1593-1600.
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h lAchalasiaWhich Fundoplication?
Complete fundoplication (Nissen) should be avoided due to aperistaltic esophagusP i l D ( i ) T ( i )Partial: Dor (anterior) versus Toupet (posterior)
Minimal differenceArain et al: No difference in relief ofArain et al: No difference in relief of
Dysphagia, Heartburn, Chest painNeed of proton pump inhibitors
Advantage DorTechnically easierP i f l i hPreservation of natural posterior attachments
Arain MA, et al: Preoperative lower esophageal sphincter pressure affects outcome of laparoscopic esophageal myotomy for achalasia. J Gastrointest Surg 2004; 8:328-334.
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h lAchalasiaThoracoscopic vs Laparoscopic
Patti et al: Thoracoscopic Heller myotomy versus Laparoscopic Heller w/ Dor fundoplication
6 ( i h )60 pts (30 in each group)Avg hospital stay: 84 hrs (thorac.); 42 hrs (lap)Relieved dysphagia: 87% vs 90%Relieved dysphagia: 87% vs 90%Abnormal reflux (by pH monitoring)
60% thoracoscopic ptsp p10% laparoscopic/fundoplication pts
Patti MG, et al. Comparison of thoracoscopic and laparoscopic heller myotomy for achalasia. Journal of Gastrointestinal Surgery. Volume 2, Number 6 / December, 1998; 561-566
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h lAchalasiaOutcomes following Laparoscopic Heller Myotomy
Author Year Patients Follow‐upmonths
Relief of Dysphagia
(%)
Length of Stay
(median)
Perforation(%) Reflux (%)
Portale 2005 248 43 (1–131) 88 5 (3–11) 4.0 7 *
Bonatti 2005 75 64 (10–131) 84 2 (1–6) 4.0 11
Khajanchee 2005 121 9 (6–48) 91 1.7 (na) 6.6 13 *
Arain 2004 78 24 (6 100) 77 na 0 17Arain 2004 78 24 (6–100) 77 na 0 17
Perrone 2004 100 26 (6–72) 96 1.2 (1–4) 3.0% na
Oelschlager 2003 110 26 (1–85) 90 na na 23
Donahue 2002 81 45 (1–70) 84 1 (na) 14.0% 4
Sharp 2002 100 11 (na) 93 1.5 (na) 8.0% 4
Patti 2001 102 25 (na) 89 1.5 (na) 5.0% na
Zaninotto 2001 113 24 (1–83) 91 na na 6
Patti 1999 133 23 (na) 89 2 5.0% 17
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llHeller MyotomyEffects on Symptoms
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h lAchalasiaOutcome Predictors
Degree of improvement in resting LES
If reduced to < 10 mmHg = long‐lasting li f f d h i ( relief of dysphagia ( 5
years or more)
> 20 mmHg = recurrent dysphagia within 12 to 24 months following 4 gsurgery
Eckardt VF, Gockel I, Bernhard G: Pneumatic dilatation for achalasia: late results of a prospective follow up investigation. Gut 2004; 53:629.
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Laparoscopic MyotomyOutcome Predictors
High pre‐operative resting LES pressure increases relief of dysphagiay p g
> 35 mmHg = 21.3 times more likely to have relief more likely to have relief than those with < 35 mmHg
The greater the decrease in LES pressure following surgery, better g y,improvement in dysphagia
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Diffuse Esophageal SpasmRapid progression of:
Abnormally high amplitude wavesLonger duration contractionsAperistalsis during more than 20 swallows
N t k hNutcracker esophagus:Variant of diffuse esophageal spasmRapid progression of esophageal pump with high Rapid progression of esophageal pump with high amplitude waves (> 180 mmHg) of the distal esophagus
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Diffuse Esophageal SpasmLES may show:
Normal resting pressure w/ relaxation after deglutitionIntermittent episodes of incomplete relaxationIntermittent episodes of incomplete relaxation
Higher risk of epiphrenic diverticulum
Treatment:Medical Tx and lifestyle adjustmentsIf persistent symptoms: surgical intervention
Long esophageal myotomy via thoracoscopic approachMyotomy throughout entire distance of manometricMyotomy throughout entire distance of manometricabnormality (from aortic arch to LES)
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Esophageal HypercontractionResting pressure in LES exceeds upper limit of normalEsophageal body peristalsis remains normalSymptoms:Symptoms:
DysphagiaChest painGERD
Occasionally 2ry to GERD and/or type III hiatal herniaAlteration of configuration of the cardiaAlteration of configuration of the cardia
Treatment: Laparoscopic myotomyw/ partial fundoplicationNi f d li i (if GERD/hi l h i )Nissen fundoplication (if 2ry to GERD/hiatal hernia)
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Esophageal HypocontractionTypically 2ry to systemic illness
SclerodermaRheumatoid ArthritisRheumatoid ArthritisSLEAlcoholism
Abnormally low amplitude (< 30 mmHg) contractionsDiscoordination leads to ineffective peristalsisNormal LESGERD due to lack of peristalsisH tb d R fl th d h iHeartburn and Reflux more common than dysphagia
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Esophageal HypocontractionNeed to rule out:
Mechanical obstructionMalignant disorders
Contrast esophagogramsMManometry24‐hr pH monitoring
Treatment: directed towards GERDSurgical Anti reflux proceduresSurgical Anti‐reflux procedures
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ConclusionLap Heller myotomy has become the standard surgical approach for patients w/ achalasia.Surgical myotomy provides superior long‐term symptom Surgical myotomy provides superior long term symptom relief compared to non‐surgical interventions.Extended distal myotomy with partial fundoplication has been found to provide greater dysphagia relief with been found to provide greater dysphagia relief with minimal development of GERD.A high pre‐operative LES pressure portends a better
i f ll i symptomatic outcome following surgery.Persistent or recurrent symptoms following myotomy can be treated effectively with pneumatic dilatationy p
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Question 1A 34 y.o man has a progressive hx of dysphagia to solids and liquids. Over the last several weeks he has regurgitated food that is several days old. Barium swallow reveals ynarrowed tapering of distal esophagus and EGD reveals retained food particles. The most appropriate management for this pt is:g p
a) Botulinum toxin injection of the LESb) Calcium channel blocker therapyb) Calcium channel blocker therapyc) Esophageal myotomyd) Esophageal myotomywith fundoplicatione) Pneumatic dilation of the LES
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Question 2A 70 y.o man presents with dysphagia and intermittent regurgitation of mucoid material. He has lost 35 lbs. since the onset of symptoms 3 months ago. Barium since the onset of symptoms 3 months ago. Barium swallow reveals a “bird’s beak” appearance. All of the following are pertinent to his work‐up EXCEPT:
a) Esophagoscopyb) 24‐hr pH monitoring) 4 p gc) Esophageal manometryd) Serum albumin
he) Chest x‐ray
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Question 3A 52 y.o woman presents c/o several year hx of progressive dysphagia to liquid and solid food. She describes a sensation of food “sticking” and not passing into her stomach. Barium swallow reveals narrow tapering of distal esophagus swallow reveals narrow tapering of distal esophagus. Manometry reveals LES pressure that remains constant thru a swallow test. You diagnose achalasia. Which of the following is TRUE regarding LES in healthy pts?
a) LES is a specific anatomic sphincterb) Gastric distention causes decreased LES tone) LES d d i th i iti ti f llc) LES pressure decreases during the initiation of a swallowd) The LES serves to prevent air from entering the stomach during a
swallowe) The LES can be visualized by upper endoscopy) y pp py
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