Atypical Variants of Classic Achalasia Are Commona izP tuKe iebD ACSJef

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STUDY DESIGN: Records of patients undergoing high-resolution esophageal manometry between January 2008

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2Puband January 2010 were screened for diagnosis of achalasia, impaired lower esophageal sphincter(LES) relaxation, or severe peristaltic dysfunction of the esophageal body. Forty-four patientswith classic achalasia and 31 with variant characteristics were identified. Clinical and mano-metric characteristics were recorded and compared.

RESULTS: Variant achalasia was almost as common as the classic type (31 versus 44 patients). Eighty-twopercent (36 of 44) of those with classic and 48% (15 of 31) of those with variant characteristicshad dysphagia. Classic achalasia patients mean age was 62 years (SD 19 years) versus 53 years(SD 14 years) in the variant group. The classic achalasia group had 26 male patients and 18female patients and the variant group had 9male patients and 22 female patients.Two thirds (21of 31) of the variant group had impaired LES relaxation. Three variant patterns emerged:impaired LES relaxation with normal/hypertensive peristalsis (n 10), impaired/borderlineLES relaxation with mixed peristalsis/simultaneous contractions (n 14), and impaired/normal LES and aperistalsis with occasional short segment peristalsis (n 7). Mean intraboluspressure was 16.3 mmHg in variant patients with normal LES relaxation and 23.1 mmHg inthose with impaired relaxation.

CONCLUSIONS: Variants of achalasia are more common than previously recognized. LES dysfunctiondefined by high relaxation pressure occurs in two-thirds of variant achalasia patients andmight be a hallmark that could direct therapy. The notion that esophageal bodydysfunction/aperistalsis in achalasia is all or none should be reconsidered. ( J Am Coll Surg2011;213:155163. 2011 by the American College of Surgeons)

ophageal motility disorders represent a heterogeneousd poorly understood group of conditions that can over-. Of these, achalasia is the best-characterized disease.halasia is classically defined as an esophageal motor dis-

order characterized by an aperistaltic esophagus with im-paired relaxation of the lower esophageal sphincter (LES)and has been recognized and treated for more than 300years. Current guidelines require the absence of esophagealperistalsis combined with incomplete relaxation of the LESas the two key and necessary components for its manomet-ric diagnosis.1 Patients with mixed simultaneous and peri-staltic contractions and/or those with aperistalsis with nor-mal LES relaxation fail to meet these criteria and have notbeen considered to have achalasia. Although Pandolfinoand colleagues recently suggested 3 subtypes of achalasiacould be identified, which can differ in the response totherapy2 (Fig. 1), the all or nothing paradigm has not, forthe most part, been challenged.

closure Information: Nothing to disclose.sented at the Western Surgical Association 118th Scientific Session, Chi-o, IL, November 2010.

eivedDecember 6, 2010; Revised January 28, 2011; Accepted February 1,1.m the Division of Thoracic and Foregut Surgery, Department of Surgery,iversity of Rochester Medical Center, Rochester, NY.rrespondence address: Jeffrey H Peters, MD, Department of Surgery, Uni-sity of Rochester, Rochester, NY 14534. email: jeffrey_peters@urmc.hester.edu

155011 by the American College of Surgeons ISSN 1072-7515/11/$36.00lished by Elsevier Inc. doi:10.1016/j.jamcollsurg.2011.02.008nd Currently Under-Recognrevalence and Clinical Feally M Galey, MD, Candice L Wilshire, MD, Stefan Naniel P Raymond, MD, FACS, Virginia R Litle, MD, Ffrey H Peters, MD, FACS

BACKGROUND: Advances in esophageal manometry hsuggesting they are more common thaand clinical characteristics of patients wcriteria for, classic achalasia.ed: A Study ofresisch, MD, Carolyn E Jones, MD, FACS,, Thomas J Watson, MD, FACS,

facilitated identification of variants of achalasia,viously thought. This study assesses the frequencymotility abnormalities similar to, but not meeting

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156 Galey et al Variant Achalasia J Am Coll SurgThe introduction of high-resolution esophageal ma-metry has facilitated the identification of patients withiations of the features long required for the diagnosis ofssic achalasia.3 Patients with abnormal LES relaxationd occasional complete peristaltic waves, patients withxed peristaltic and simultaneous segments of the esoph-al body in a single wave, those with high amplitudeultaneous waves with or without poor LES relaxation,d those with severe esophageal body dysfunction butrderline or normal LES characteristics can be seen moredily, given the high number of pressure sensors. We be-ve that such patients are currently left largely untreatedcause they do not fit the strict criteria of achalasia. Thisdy assesses the clinical characteristics and manometrictterns of patients with motility abnormalities similar to,not meeting the definition of, classic achalasia.

ETHODSe study population was identified from records of pa-nts undergoing high-resolution esophageal manometrytween January 2008 and January 2010. Six hundred andety-five records were screened for the following charac-istics: diagnosis of achalasia (n 72), impaired LESaxation defined as a 3-second nadir or 4-second inte-ted relaxation pressure (4sIRP) of14mmHg on high-olution esophageal manometry (n 84), or severe per-altic dysfunction of the esophageal body, defined as0% abnormal contraction waves (n 44). One hun-d and thirty-eight patients met these inclusion criteriame patients matched more than one criterion), includ-50 patients with classic achalasia (impaired deglutitive

axation of the LES and aperistalsis) and 31 with variantaracteristics not meeting the criteria for classic achalasia.ty-six patients with LES or esophageal body abnormali-s thought more reflective of, or secondary to, previoustal surgery (n 20); intrathoracic stomach (n 9);troesophageal reflux disease (n 8); stricture, ring, orss (n 7); scleroderma pattern (n 5); ineffectivephageal motility as defined by a normal LES and50%ristalsis (n 3); hypertensive LES with normal relax-on pressures and normal esophageal body motility (neosinophilic esophagitis (n 1); and extrinsic compres-

Abbreviations and Acronyms

CFV contractile frontal velocityDEA distal esophageal amplitudeDES diffuse esophageal spasmIBP intrabolus pressureLES lower esophageal sphincter4sIRP 4-second integrated relaxation pressuren (n 1) were excluded. Five patients diagnosed previ-sly with achalasia and treated with myotomy were ex-ded, as well as 1 patient who was a minor. One patientused consent. Seventy-five patients constituted ourdy population. Clinical and manometric characteristics

ure 1. Classic achalasia. (A) Type I: nonrelaxing lower esopha-l sphincter, aperistaltic esophageal body, esophageal body in-luminal pressure 30 mmHg, n 24. (B) Type II: nonrelaxinger esophageal sphincter, aperistaltic esophageal body, esopha-l body intraluminal pressure 30 mmHg, n 8. (C) Type III:relaxing lower esophageal sphincter, aperistaltic esophagealy with high amplitude contractions, esophageal body intralumi-pressure 30 mmHg, n 12.

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Table 1. Clinical Characteristics of Patients with Variant andClassic Achalasia

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157Vol. 213, No. 1, July 2011 Galey et al Variant Achalasiathe 75 patients with classic and variant achalasia wereorded and compared.Manometric studies were analyzed using ManoView1 (Sierra Scientific Instruments) analysis software.anometric data included measurement of LES length,ting pressure, and residual pressures (both 3-second na-and 4sIRP) and intrabolus pressure (IBP). Peristalsis ofesophageal body was evaluated using the sensors 5, 10,

d 15 cm above the LES, as well as by measuring thentractile frontal velocity (CFV), the distal contractile in-ral, and by visually assessing morphology.Each swallow in the variant group was characterizednometrically using modified criteria described previ-sly by Pandolfino and colleagues4 as follows:

Normal: distal esophageal amplitude (DEA) 180mmHg,CFV8 cm/s, intact 30mmHg isobaric contour,distal contractile integral4300 mmHg s cm;Hypotensive: 3 cm interruption of the 30 mmHgisobaric contour in the distal esophagus;Aperistaltic: no progression of contraction through thebody of the esophagus;Hypertensive: CFV 8 cm/s, DEA 180 mmHg, ordistal contractile integral 4300 mmHg s cm;

Variant(n 31)

Classic(n 44)

ale to male ratio 22:9 19:25*an age, y (range) 54 (2377) 62 (1993)*mary or secondary symptom,n (%)

Dysphagia 15 (48) 36 (82)*Regurgitation 7 (23) 15 (34)Chest pain 9 (29) 1 (2)*Weight loss 1 (3) 2 (5)

0.05 versus variant.

ble 2. Lower Esophageal Sphincter and Esophageal Body CVariant (n

Mean SD

SResting pressure 49 mmHg 42.2 21.6Relaxation pressure 14.7 mmHg 19.2 8.717.0 mmHg 20.7 9.5phageal body (n 44)egmental simultaneous 7%imultaneous 31%ailed 5%

ree patients with classic achalasia could not be analyzed for lower esophagea, intrabolus pressure; LES, lower esophageal sphincter.Segmentally spastic: a portion of the body, but not theentire length from the transition zone to the LES withCFV 8 cm/s;Panesophageal pressurization: pressurization of 30mmHg involving the entire esophageal body from theupper esophageal sphincter to the LES.

Patients clinical and radiological data were also col-ted by chart review to assess treatment and symptomatictcomes.Categorical data was analyzed using a Fishers exact testd continuous data were analyzed with Students un-ired t-test.

SULTSventy-five patients were identified with either variant 31) or classic achalasia (n 44) during the 2-yeardy period.Table 1 compares the demographics and clin-l characteristics of the 2 groups. Mean age of the variantpulation was slightly younger (53 years variant versus 62rs classic; p 0.03), and those with variant achalasiare more commonly female (22 female and 9 male pa-nts versus nineteen female and 25 male patients; p 2). Dysphagia was the primary presenting symptom in% (12 of 31) of patients with variant achalasia, signifi-tly less than patients with classic achalasia (77%, 34 of; p 0.01). Conversely, a primary symptom of chestin more frequently occurred in the variant group, buts only approached significance (16%, 5 of 31 versus 2%,f 44; p 0.08).Table 2 summarizes the LES and esophageal body char-eristics of patients with variant achalasia.Three predom-nt variant patterns emerged (Table 3 and 4): impairedS relaxation with normal/hypertensive peristaltic con-ction waves (n 10, Fig. 2); impaired/borderline LESaxation with spastic or segmentally spastic contractions

cteristics of Patients with Variant and Classic Achalasia) Classic (n 41*)Increased

Mean SDIncreased

n % n %

8 26 39.7 12 2920 65 24.4 32 7818 58 NA NA

5 16 NA NA21 68 80% 34 779 29 30% 15 34

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Table 3. Classification of Types of Variant Achalasia(n 31)Typ

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Table 4. Manometric Classification of Types of Variant Acha-lasia (n 31)

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158 Galey et al Variant Achalasia J Am Coll Surg 14, Fig. 3); and impaired/normal LES relaxation witheristalsis with occasional short segment peristalsis with atst 20% hypotensive swallows (n 7, Fig. 4).Fourteen of 31 patients had barium esophagrams avail-le for review, 11 of 14 were abnormal. In the first sub-e, 4 patients had barium esophagrams, all were abnor-l with at least some esophageal dilation with contrastention. One demonstrated narrowing at the gastro-phageal junction and a second demonstrated delayedaxation. In the second subtype, 7 patients had bariumphagrams, 5 of 7 were abnormal, 4 had peristaltic dys-ction, and 5 had evidence of dysfunctional emptying.the third subtype, 3 patients had barium esophagrams, 2th contrast retention, including 1 with a nonrelaxingS.Most patients with variant achalasia had evidence of im-ired LES function (77%, 24 of 31), which included 20th elevated 4sIRP (14.7 mmHg), 8 with elevated rest-pressure (49 mmHg), and 18 with high IBP (17Hg). Mean IBP, a indication of esophageal outflow

struction, was higher in the variant patients with im-ired relaxation (23.1 mmHg) compared with those withrmal LES relaxation (16.3 mmHg; p 0.06). Fourre patients had borderline dysfunction (IBP 15Hg or IRP 13.5 mmHg), leaving only 3 patients

th all 3measures of LES function normal and 90% (28 of) of patients with some evidence of LES dysfunction.The majority of patients (23 of 31) did not receive anycific treatment directed at their motility disorder. Threetients received nitroglycerine for their symptoms. Onetient received onabotulinumtoxinA (Botox) with someeviation of symptoms. Four of the 31 patients underwentesophageal myotomy; 2 underwent an open transtho-ic long myotomy and 2 underwent laparoscopic distalphageal myotomy, all with partial fundoplication. Dys-agia was relieved in all 4 at a mean follow-up of 11.5nths.

SCUSSIONthese data confirm, the introduction of high-resolutionnometry has facilitated the recognition of considerablynormal patterns of LES and esophageal body motility

e Description n %

Abnormal LES with normal/hypertensive peristalsis 10 32Abnormal/borderline LES with spastic or partiallyspastic contractile waves

14 45

Borderline/normal LES and aperistalsis withoccasional short segment peristalsis

7 23

S, lower esophageal sphincter.t have until now been largely unrecognized or classifiednonspecific. Among these are motility patterns with fea-es of esophageal outflow resistance, including elevatedS residual pressures and/or esophageal IBP often com-ed with swallows with either complete or segmentalultaneous contraction waves. These variants are rela-ely common, we identified 31 patients during a 2-yeare frame and they commonly presented with a primaryort of dysphagia. Most (77%) have evidence of LESsfunction, and all have substantial abnormalities ofphageal body peristalsis, although sometimes isolated toly a portion of the esophageal body or some swallows.ternatively, some patients had predominantly spasticallows (CFV8 cm/s), but with segmental peristaltis ine swallows peristaltic, distinguishing them from classicalasia (Fig. 5). We propose that these abnormalities areere enough to consider these patterns as variants of acha-ia, and that the all or none dogma associated with thetility diagnosis of achalasia might be in error.The motility patterns described here should not be con-ed with diffuse esophageal spasm (DES). Importantly,S is characterized by normal LES resting and residualssures, normal peristalsis in a substantial proportion ofswallows (usually 30%), and no signs of esophageal

tflow obstruction, such as an increased IBP. We haveluded a few patients in type III with normal LES char-eristics but virtually absent esophageal body function.

Typearacteristic I II IIIwer esophageal sphinctern 10 14 7ean age, y 52 52 58s IRP, mmHg (14.7 mmHg) 23.3 19.8 12.2esting pressure (48 mmHg) 58.4 38.3 28.5BP, mmHg (17 mmHg) 21.8 22.1 16.2phageal body (swallow characteristics)pastic 0.4 5.6 1.7egmentally spastic 0.4 1.3 0.1ailed 0.1 0.4 1.4ypotensive 0.1 0.1 3.9ypertensive 4.0 1.1 0.0ormal 5.0 1.5 2.9ncreased IBP 17 mmHg 7.2 6.0 4.0Multiple peaks 1.0 2.7 0.9DEA 162 159 46DCI 4,735 4,518 498

allows represent average number of swallows out of ten with eachracteristic.I, distal contractile integral; DEA, distal esophageal amplitude; IBP, in-olus pressure.

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159Vol. 213, No. 1, July 2011 Galey et al Variant Achalasiais small but interesting group is arguably the most dif-ult to classify, although we believe they more closelyemble achalasia than DES.The majority of patients in the third subtype have eithertle or borderline LES abnormalities. For example, al-ugh some of them might have had normal relaxationssures, relaxation was not normal. Others have border-e abnormal LES values in which a 1 or 2 mmHg differ-ce in the reading would have changed the classification.mbined with concomitant esophageal body abnormali-s, these findings were similar enough to classic achalasiaindicate that although quantitatively their LES is func-ning, it is qualitatively dysfunctional and might play anportant role in their symptomology and might contrib-to their dysmotility.Achalasia is a primary esophageal motility disorder ofclear etiology. It is an uncommon disease affecting ap-ximately 1 in 100,000 individuals per year. It occursually in male and female patients and is an acquiredndition usually diagnosed between 20 and 50 years of, but can occur at any age. The original description isributed to SirThomasWillis in the mid-1600s. Steichend colleagues outlined the history of the disease and itsatment to date in an excellent review published in 1960.5

veral classic clinicopathologic descriptions appeared in1700s and 1800s. Von Mikulicz described the endo-pic findings in 1882, and Heller and others, its surgicalatment in 1914.With the introduction of water perfused esophagealnometry in the late 1960s and early 1970s, the mano-tric features of achalasia became evident. Four classicnometric characteristics have been described: hyper-sive LES; nonrelaxing LES; esophageal body aperi-lsis; and elevated esophageal baseline pressure. Pres-

ure 2. Variant subtype I, abnormal lower esophageal sphincterh normal/hypertensive peristalsis, n 10. This patients studyrall: resting pressure: 70.4 mmHg; 4-second integrated relax-n pressure: 20.3 mmHg; distal esophageal amplitude: 146.6;abolus pressure: 22.9 mmHg; 9 peristaltic swallows, 1ertensive.ce of a nonrelaxing LES and aperistalsis of thephageal body became the sine qua non of manomet-diagnosis. A subset of achalasia patients characterizedsimultaneous contraction waves of variable ampli-

des consistent with preserved muscle function was alsoscribed and termed vigorous achalasia. These charac-istic manometric features have remained virtually un-anged for 30 to 40 years. The dogma that completeeristalsis of the esophageal body is a hallmark of theease has been accepted virtually without challenged perhaps in error. Variability in the resting pressurethe LES was well recognized by the end of the 1970s.elsen and Bremner wrote in 1979 that although manyorts noted the failure of relaxation of the LES inponse to swallowing, measures of the resting pressurethe LES and swallow responses in achalasia were con-ting, with both normal and high resting pressuresorted.6

Manometric evaluation of patients with structuralsons for esophageal outflow obstruction, includingsfunctional gastric bands, fundoplication, tumors, or

ure 3. Variant subtype II, abnormal/borderline lower esophagealincter with (A) mixed peristalsis and (B) simultaneous waves, n . This patients study overall: resting pressure: 40.4 mmHg;econd integrated relaxation pressure: 25.8 mmHg; intrabolus pres-e: 50.9 mmHg; 4 spastic swallows, 5 partial spastic, and 1 failedallow.

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160 Galey et al Variant Achalasia J Am Coll Surgraesophageal hernia, strongly suggest that variationsboth hiatal outflow resistance and esophageal bodysfunction occur as a spectrum of dysfunction ratheran all or none. For example, high-resolution mano-tric analyses of patients with adjustable gastric bandseals a wide spectrum of esophageal body abnormali-s, particularly with overfilling of the band.7 Theseta suggest that esophageal outflow obstruction canult in esophageal body abnormalities similar to thosen in the patients described in our study, in which LESnormalities presumably caused the outflow resistance.note, and perhaps an important insight into the truenicopathologic characteristics of the disease, a mano-tric pattern characterized by 100% simultaneousntractions with a normal sphincter and no evidence oftflow obstruction is not seen.The pathophysiology of achalasia is incompletely un-rstood but thought to result from an absence of inhib-ry ganglion cells in the myenteric plexus of the esoph-us. This loss creates an imbalance between excitatoryd inhibitory neurons, impairing LES relaxation.hether esophageal body dysfunction is a consequencethe outflow resistance created by LES abnormalities,urologic damage affecting the esophageal circulard/or longitudinal muscle function or a combinationthese is unknown. Given what we know of its patho-ysiology, it seems quite plausible that patchy, seg-ntal, and incomplete dysfunction might well occur. Ifthe concept of variant achalasia would be the clinicalnifestation of partial damage.The concept of manometric heterogeneity in patientsth achalasia is not new. Hirano and colleagues empha-ed the likelihood in 2000.8 Fifty-eight consecutive pa-nts seen between 1996 and 1999 in whom the diagnosis

ure 4. Variant subtype III: abnormal/borderline lower esopha-l sphincter and aperistalsis with occasional short segment peri-lsis, n 7. This patients overall study: resting pressure: 42.2Hg; 4-second integrated relaxation pressure: 10.9 mmHg; in-bolus pressure: 14.1 mmHg; 4 spastic, 4 hypotensive, and 2mal swallows.achalasia was not established by manometry were char-erized manometrically. Confirmatory clinical featuresluding endoscopy, barium swallow, histopathology, andponse to therapy were used to establish the presence ofalasia. The authors described 4 manometrically distinctiants: simultaneous high-amplitude esophageal bodyntractions; short segment aperistalsis; retained completeglutitive inhibition; and intact transient LES relaxation.ey concluded that significant heterogeneity exists inmanometric features of achalasia and that this mightvide important insights into the pathophysiology of theease. We agree.Arledge and colleagues9 suggested the existence ofriant achalasia in an abstract published in 2008.9 Theyrospectively reviewed 900 motility studies performedthe University of South Florida selecting those withe diagnosis of achalasia in which a peristaltic segmentcm was found. Seventeen such patients were identi-

d. Blinded review confirmed 8 of these, including 5le patients and 3 female patients averaging 65 years ofe. Symptoms consisted of dysphagia, bolus impaction,est pain, and heartburn. They concluded that variantalasia with retained short segments of peristalsis

ould be acknowledged. Kahrilas10 has recognized anometric pattern similar to what we have described ase II and termed it spastic nutcracker.The natural history and ideal treatment of patientsth the motility patterns described in this study is un-own. The entire clinical picture, including an assess-nt of bolus transport radiographically or via imped-ce manometry, is an important adjunct to thenometric findings and might help the clinician inerapeutic decision-making. Most of our patients didt undergo esophageal myotomy, pneumatic dilation,

ure 5. Spastic morphology with peristaltic element. Contractilental velocity 8 cm/s.

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161Vol. 213, No. 1, July 2011 Galey et al Invited Commentarylly responded well. The recognition of these patternsvariants of achalasia, particularly when coupled withidence of LES dysfunction, argues for consideration ofer esophageal myotomy or endoscopic therapy. The

efulness of such an approach and the symptomatictcomes await additional study.

NCLUSIONSe notion that esophageal body dysfunction/aperistalsisachalasia is all or none might be wrong and should beonsidered. We believe sphincter dysfunction should de-e the disease currently best measured by high 4sIRP onh-resolution esophageal manometry.This is particularlye given that therapy for achalasia is directed at relievingphageal outflow resistance. Variants of classic achalasiamore common than previously recognized and deservetailed study.

thor Contributions

dy conception and design: Galey, Wilshire, Niebisch,Jones, Raymond, Litle, Watson, Petersquisition of data: Galey, Wilshire, Niebisch, Petersalysis and interpretation of data: Galey,Wilshire, Niebisch,Jones, Raymond, Litle, Watson, Petersafting of manuscript: Galey, Wilshire, Niebisch, Jones,Raymond, Litle, Watson, Petersitical revision: Galey, Wilshire, Niebisch, Jones, Raymond,Litle, Watson, Peters

FERENCES

. Vaezi MF, Richter JE. Diagnosis and management of achalasia.Am J Gastroenterol 1999;94:12 34063412.

. Pandolfino JE, Kwiatek MA, Nealis T, et al. Achalasia: a newclinically relevant classification by high-resolution manometry.Gastroenterology 2008;135:15261533.

. Fox MR, Bredenoord AJ. Oesophageal high resolution manometry;moving from research into clinical practice. Gut 2008;57:405423.

. Pandolfino JE, Kwiatek MA, Ho K, et al. Unique features ofesophagogastric junction pressure topography in hiatus herniapatients with dysphagia. Surgery 2010;147:5764.

. Steichen FM, Heller E, Ravitch MM. Achalasia of the esopha-gus. Surgery 1960;846876.

. Nielsen, IJ, Bremner CG. Lower oesophageal sphincter restingpressures in achalasia and the response of the sphincter to swal-lowing and drugs. S Afr Med J 1979;50:18221825.

. Burton PR, BrownW, Laurie C, et al. The effect of laparoscopicadjustable gastric bands on esophageal motility and the gastro-esophageal junction: analysis using high resolution video ma-nometry. Obesity Surg 2009;19:905914.

. Hirano I, Tatum RP, Shi G, et al. Manometric heterogeneity inpatients with idiopathic achalasia. Gastroenterology 2001;120:789798.criteria. Am J Gastroenterol 2008;103:S9.. Kahrilas PJ. Esophageal motor disorders in terms of high reso-lution esophageal pressure topography: what has changed. Am JGastroenterol 2010;105:981987.

vited Commentary

ilip O Katz, MDiladelphia, PA

gh-resolution esophageal manometry (HRM), a tech-logy that uses an increased number of transducers placedse together, plus enhanced software that allows for visu-zation with color contour plots as well as line tracings,s allowed investigators to evaluate esophageal body con-ction and lower esophageal sphincter (LES) functionways not previously possible even with solid-state trans-cers.This has led to a reclassification (Chicago) of esoph-al motility abnormalities,1 in particular 3 manometrictypes of achalasia,2 the latter study suggesting a differ-t therapeutic outcome for each. Although HRM allowseasier performance of manometry and the opportunityfind new contraction and LES abnormalities, outcomedies are still needed to determine if these new classifi-ions are more clinically relevant than previous.In this issue of the Journal, Galey and colleagues3 chal-ged all of us to reexamine the manometric diagnosis ofalasia and disorders of LES function by highlightingclassified manometric abnormalities that they termedhalasia variants. They presented the results of a carefuliew of a large cohort (N 695) of patients studied withM.They identified 31 patients with 3 manometric pat-ns of what they called variant or atypical achalasia; 10d high residual LES pressure (abnormal relaxation) andrmal/hypertensive peristalsis (type 1), 13 had high resid-l LES pressure with mixed peristalsis/simultaneous con-ctions (type 2), and 8 had borderline/abnormal LES anderistalsis with occasional short segment peristalsis (typeThey strongly suggested that these patients are repre-tative of part of the spectrum of achalasia and that wewithholding potentially good treatment by leavingse patients in this currently unclassified group. Unfor-ately, the authors did not have information on howny had other clinical evidence of outflow obstructionrium, impedance, nuclear emptying) or many data onnical outcomes because only 4 of the 31 received defin-e treatment aimed at relief of outflow obstruction. It isinterest, however, that all 4 were reported improved.

Atypical Variants of Classic Achalasia Are Common and Currently Under-Recognized: A Study of Pre ...MethodsResultsDiscussionConclusionsAuthor Contributions

References