PHOTO ROUNDS

Drew C. Baird, MD Bilateral leg edema Department of Family and Community Medicine, and diffi culty swallowing D.D. Eisenhower Army Medical Center, Fort Gordon, Ga [email protected]

This patient’s leg pain was the tip of the iceberg. He also EDITOR Richard P. Usatine, MD had diminished breath sounds and a cachectic appearance. University of Texas Health Science Center at San Antonio

70-year-old man came to our and lower lung fi elds, and pitting edema medical center for treatment of up to the knees bilaterally. A painful bilateral leg swelling that Lab studies showed a normal had gotten progressively worse over the natriuretic peptide of 16 pg/mL, nor- past week. He had no signifi cant past® Dowdenmocytic anemia Health (hemoglobin, Media 9.5 g/dL; medical or surgical history, took an as- hematocrit, 29.6%; and a mean corpus- pirin daily, did not smoke tobacco or cular volume of 82.6 fL), potassium of drink alcohol, and hadCopyright not takenFor any personal2.4 mEq/L, calcium use of only 5.4 mg/dL, and al- trips recently. He denied any chest pain, bumin of 1.4 g/dL. An electrocardiogram dyspnea, or orthopnea, but indicated (EKG) revealed a bifascicular heart block, that he’d been having diffi culty swal- which was not new based on older EKGs. lowing food for the past month. A plain chest radiograph was performed FAST TRACK The patient had a cachectic appear- (FIGURES 1A AND 1B). In addition to ance, diminished breath sounds and dull- ness to percussion over the right middle ❚ What is your diagnosis? having diffi culty swallowing, FIGURE 1 the patient had Chest x-rays reveal large mass diminished breath A B sounds and dullness to percussion over the right middle and lower lung fi elds.

A posterior-anterior chest radiograph (A) revealed a large mass in the right hemithorax with an air-fl uid level. A lateral

PHOTOS COURTESY OF DREW C. BAIRD, MD PHOTOS COURTESY chest radiograph (B) showed the same large mass with an air-fl uid level that displaced the anteriorly.

www.jfponline.com VOL 58, NO 2 / FEBRUARY 2009 89

For mass reproduction, content licensing and permissions contact Dowden Health Media.

089_JFP0209 089 1/22/09 11:16:53 AM ROUNDS ❚ Diagnosis: Achalasia and esophageal cancer.3 This case is re- Achalasia is characterized by an incom- markable for how far the patient’s dis- plete relaxation of the lower esophageal ease progressed before he presented with PHOTO sphincter (LES) accompanied by aperi- signs and symptoms more indicative of stalsis of the esophageal body. The etiolo- secondary malnutrition than the primary gy of primary achalasia is unknown, but disease. involves the degeneration of inhibitory neurons within the re- sponsible for LES relaxation and esopha- ❚ Differential Dx geal . includes hiatal hernia Many potential causes. Infectious, The differential diagnosis for achalasia infl ammatory, autoimmune, and genetic includes hiatal hernia, right middle lobe causes have all been proposed. Achalasia pneumonia, empyema, and lung abscess. has a prevalence of less than 1 in 10,000; Most patients with hiatal hernias are its incidence is 0.3 to 1 per 100,000 per asymptomatic, and the diagnosis is made year, with peaks in the third and seventh incidentally on chest x-ray as a retro- decades of life. Men and women are af- cardiac air-fl uid level or on upper gas- fected equally.1 trointestinal studies. Right middle lobe Conversely, pseudoachalasia—or pneumonia would appear on chest x-ray secondary achalasia—has a known cause as a well-demarcated opacity within the that either destroys the neurons of LES confi nes of that lobe, but would not have relaxation or has a mass effect that limits an air-fl uid level. Empyema and lung ab- LES relaxation. Its most common cause scess are complications of pneumonia, is malignancy involving the gastroesoph- and patients present with persistent fever, ageal junction. Other causes include Cha- cough, dyspnea, and malaise. With an em- gas disease, an infection by the parasite pyema, chest x-ray reveals a pleural effu- Trypanosoma cruzi that affects the my- sion. A lung abscess is an intrapulmonary enteric plexus, and amyloidosis.2 cavitary lesion with an air-fl uid level, most FAST TRACK commonly due to . To aid in the A disorder that goes This patient’s chest x-ray showed a undiagnosed for years mass outside the lung tissue and pleu- transit of food In primary achalasia symptom onset is ral space that had an air-fl uid level, was boluses, patients insidious, with the disorder typically go- within the mediastinum, and displaced will eat slowly, ing undiagnosed for several years. Pa- the trachea anteriorly, all suggestive of a stretch, move tients experience a gradual for dilated fi lled with undigested solids, progressing to liquids. Over time, food unable to pass the LES. side-to-side, and patients adopt particular behaviors to walk after meals. aid the transit of food boluses down the esophagus and through the contracted ❚ Esophageal manometry LES, including slowly, stretching, clinches the diagnosis moving side-to-side, or walking after Esophageal manometry is the gold stan- meals.1 Regurgitation of food, chest pain, dard for diagnosis. In achalasia, ma- weight loss, heartburn, and diffi culty nometry shows poor peristalsis of the belching are also common complaints.3 esophageal body and a constricted LES A more rapid onset and progression of that does not relax suffi ciently with swal- symptoms (less than 6 months) is sugges- lowing.3 Manometry cannot, however, tive of pseudoachalasia and cancer. reliably distinguish primary achalasia Left untreated, primary achalasia from pseudoachalasia. leads to a progressively dilating esopha- Barium swallow studies and endos- gus with increased risk of aspiration, copy can assist in the diagnosis and perforation, malnutrition, weight loss, help rule out pseudoachalasia. Timed

90 VOL 58, NO 2 / FEBRUARY 2009 THE JOURNAL OF FAMILY PRACTICE

090_JFP0209 090 1/22/09 11:16:58 AM Bilateral leg edema and diffi culty swallowing

barium studies can show a lack of peri- FIGURE 2 stalsis, an air-fl uid level at the top of a Massively distended esophagus barium column retained within the di- lated esophagus, and the narrowing of the distal esophagus into the pathogno- monic “bird’s beak” of primary acha- lasia.4 Endoscopy can visualize a mass beyond the LES, but cannot adequately assess esophageal peristalsis or LES relaxation.1 In our patient’s initial workup, a CT (FIGURE 2), barium study (FIGURE 3), and endoscopy were all used to rule out an obstructive lesion. A chest CT revealed a distended esophagus—at times 8 cm in diameter—from the thoracic inlet to the gastric inlet, strongly suggestive of achalasia. The patient’s ❚ Treatment targets trachea was displaced anteriorly, his posterior right lung was compressed, he had bilateral pleural effusions, the constricted LES and had generalized edema that was suggestive of The goal of treatment in achalasia is to anasarca. help food move through the constricted FIGURE 3 LES. Pharmacologic therapy, used to decrease LES resting tone, has limited The “bird’s beak” of achalasia benefi t and becomes less effective as the disease progresses. Calcium channel blockers, particularly nifedipine, and nitrates are the most commonly used agents.5 Pneumatic dilation is the most effec- tive nonsurgical treatment of achalasia. FAST TRACK It involves the controlled rupture of LES It was remarkable muscle fi bers to dilate the sphincter and allow gravity to move food from the how far the esophagus into the .6 Symptom patient’s disease improvement lasts for 60% of patients had progressed at 1 year, 50% at 5 years, and 36% at before he sought 10 years. Repeated dilations are possible and sometimes necessary, but have di- treatment. A barium swallow study showed diffuse enlargement of minishing success rates. The most worri- the esophagus with a narrowing “bird’s beak” near the some complication of pneumatic dilation esophagogastric junction, consistent with achalasia. is esophageal rupture, which occurs 2% of the time.5 third at 1 year. Patients may require re- Botulinum toxin injection is another peat injections every 3 to 4 months, but accepted nonsurgical therapy and is the toxin’s effectiveness diminishes with recommended for patients who would each injection.6 like to avoid more invasive treatment. The laparoscopic Heller myotomy in- When injected into the LES, the toxin volves cutting the LES muscle, making it causes temporary atrophy of the neu- incompetent and allowing food boluses rons responsible for LES contraction. to pass through the LES. Ninety percent After the procedure, 70% of patients of patients experience symptom relief report improvement in symptoms, with after surgery,5 with 10-year remission half in remission at 6 months and one- rates of 67% to 85%.1 Comparisons to

www.jfponline.com VOL 58, NO 2 / FEBRUARY 2009 91

091_JFP0209 091 1/22/09 11:17:02 AM 0092_JFP0209 092 92_JFP0209 092 PHOTO ROUNDS READ ABOUT ABOUT READ

92 FACULTY SmithKline. asaconsultant forand serves AstraZeneca, EliLilly&Co, andGlaxo Pharmaceuticals andreceives grant(s) from Forest Laboratories. No off -label use of drugs or devices are discussed in this supplement. mercial interests. haveMedia disclosednorelevant fi nancial any with relationship(s) com- Georgi, Charles Williams, andKatherine Wandersee for Dowden Health the speakersbureau ofAstraZeneca, Janssen,andPfi zer. America Pharmaceutical Inc., Pfi zer, Roche, andsanofi -aventis; and is on receives grants from AstraZeneca, Forest Laboratories, Janssen,Otsuka consultant for AstraZeneca, Janssen,Pfi zer,and Vanda Pharmaceuticals; traZeneca, Cephalon, Janssen,Pfi zer,and Vanda Pharmaceuticals;a is Journal ofFamily Practice. wasedited andpeerreviewedIt by The of CincinnatiandDowden HealthMedia. the jointsponsorshipofUniversity wasdeveloped throughThis CMEactivity by aneducationalgrant fromSupported AstraZeneca. speakers bureau for AstraZeneca, GlaxoSmithKline, andPfi zer. levels ofevidence. reviewed for fairbalance, scientifi studies of and reported, objectivity c decreasing thevalueofpresentation. All educationalmaterials are tence oftheseinterests orrelationships isnotviewed asimplyingbiasor of Cincinnatito initiate amechanismto resolve theconfl ict(s). exis- The ships thatare identifiare ed reviewed potential for conflicts of interest.If the commercial companies related to thisactivity. Allrelevant relation- content are required to discloseany relevant fi nancial with relationships editors, managers, andotherindividualswhoare inapositionto control According oftheUniversity ofCincinnati,faculty, to thedisclosure policy ing medicaleducationfor physicians. nati College isaccredited ofMedicine by theACCME to provide continu- the University ofCincinnatiCollege ofMedicine. The University ofCincin- for Continuing Education(ACCME) Medical through thesponsorshipof dance withtheEssentialAreas andPolicies oftheAccreditation Council activity. hasbeenplannedandimplemented inaccor-This CMEactivity inthe claim credit commensurate oftheirparticipation withtheextent maximum of2.5 forThe University ofCincinnatidesignates a thiseducationalactivity als whotreat patientswithpsychotic andmooddisorders Psychiatrists, care physicians, primary andotherhealthcare profession- themostappropriate• andlong-term treat- agent(s)forSelect short- • Develop aneff treatmentective plan that includes monotherapy or • hepaticeffUnderstand themechanismsofaction, other and ects, Utilize available screening tools eff ectively • Achieve earlyandaccurate diagnosis ofpatientswithmooddisorders • After reviewing thismaterial, cliniciansshouldbebetter ableto: tol-Myers Squibb, Pfi zer,Sepracor, and Wyeth. Bristol-Myers andisonthespeakersbureau Squibb; ofAstraZeneca, Bris- a confl of interest ict is identifi ed, itisthe oftheUniversity responsibility PLANNING COMMITTEE: DR MUZINA DR PARISER DR GOLDBERG DR BLACK DR NASRALLAH ACKNOWLEDGEMENT SUPPORT THE FACULTY HAS REPORTED THE FOLLOWING: FINANCIAL DISCLOSURES AND CONFLICTS OF INTEREST CME ACCREDITATION STATEMENT TARGET AUDIENCE LEARNING OBJECTIVES EXPIRATION DATE: RELEASE DATE: Columbus, Ohio Ohio State University College ofMedicine Director,Medical Neuropsychiatry Director,Medical Psychiatry Clinics Professor ofPsychiatry, Obstetrics andGynecology Cleveland, Ohio ofPsychiatry &PsychologyCleveland ClinicDepartment Associate Professor ofMedicine Vice Chairfor Research &Education Hospital,Silver NewCanaan, Hill Connecticut ofMedicine,Mt SinaiSchool New York, New York Associate ClinicalProfessor ofPsychiatry Iowa City, Iowa CollegeUniversity ofIowa Carver ofMedicine Professor ofPsychiatry Cincinnati, Ohio University ofCincinnatiCollege ofMedicine Professor ofPsychiatry, Neurology, andNeuroscience STEPHEN F. PARISER, MD DAVID J. MUZINA, MD JOSEPH F. GOLDBERG, MD DONALD W. BLACK, MD HENRY A. NASRALLAH, MD, ment to meetindividualpatientneeds combination therapy treatment metabolic eff of available ects onagents and their potential impact jfponline.com reports thatheisaconsultant for reports Forest Laboratories andJazz reports that he is on the advisory board ofAstraZeneca thatheisontheadvisory and reports reports thathereceives reports grants from Pfi the on is and zer © 2008 AMERICAN ACADEMY OF CLINICAL PSYCHIATRISTS PSYCHIATRISTS CLINICAL OF ACADEMY AMERICAN © 2008 reports that he is on the advisory board, speakersbureau, thatheisontheadvisory reports reports that he is on the advisory board ofAbbott,As- thatheisontheadvisory reports DECEMBER 1,2008 AMA PRA Category 1credits Category AMA PRA DECEMBER 1,2009 Kay Kay Weigand, University ofCincinnati;andKristen AND DOWDEN HEALTH MEDIA and currentclinicalpractice.com PROGRAM CHAIR ™. Physicians shouldonly 2008 issueofC A larger version ofthispaneldiscussionappearsinasupplement to theDecember to criticalcluesinapatient’s ofbeingalert orthefamily’simportance history history. in bothinpatientandoutpatientsettings. particular, In thesecasesunderscore the insightsinto thenuances practical ofmanagement ofsuchpatients lends further followed by apaneldiscussioninwhichthecollective experience ofallthefaculty mood disorders, membersto patientcases we present faculty invited actual 4expert sion accompanied by symptoms ofmaniaorhypomania. care, farlessinformationmary isavailable aboutpatientsinthissettingwithdepres- existontheprevalenceAlthough many reports andtreatment ofdepression inpri- 1. Olfson M,Gameroff Das AK, REFERENCE T with psychotic andmooddisorders. will enhance andconfi rm your own approach to diagnosing and treating patients in themanagementofmooddisorders: truth ofasignifi defect. cant birth preoccupied Healsobelieved histhinking. hisparents were hidingfrom himthe and theyhadledto abreak-up withhisgirlfriend, whichdistressed him greatly and Profound, persistent sadnessandfeeling “dead inside” were hischiefcomplaints, smallness.gram andsupplementuseto ofweight changehisself-described lifting symptoms beganrather abruptly14monthsearlier, coinciding withanintense pro- andthenvenlafaxine,sertraline butdiscontinued bothmedications onhisown. His chologist for treatment ofmoodswings, anxiety, andconfusion. Hehadbeengiven a 20-year-old manwhowasreferred for psychiatric evaluationby hisPCPandpsy- careof mostinterest providers. to primary in primary care in primary psychotic andmooddisorders Recognizing andmanaging 2005;293:956-963. ing process and an important clinical topic for primary care clinicaltopic clinicians(PCPs).ing process for primary andanimportant he diagnosis andmanagement ofpsychotic andmooddisorders isanevolv- 2.5 CME FREE To facilitate adialogueontheidentifi cationandtreatment ofpsychotic and We hopetheinsights you gleanfrom thisexchange clinicalissues ofpractical • Suggestions for enablingpatientcompliance withprescribed regimens • ofavailable ofaction medications andimplications forMechanisms aneffec- • Pros andcons ofmonotherapy andcombination therapy • Pitfalls to avoid duringthediagnostic evaluation Following thecasepresentation discussionofseveral isafaculty pivotal issues The for caseselected presentation here isby David Muzina,MD, andconcerns CREDITS tive treatment plan Supplement to The Journal ofFamilyPractice Supplement toTheJournal INTRODUC AND URRENT P MJ, etal. Screening care for practice. JAMA. bipolardisorder inaprimary SYCHIATRY TION .

We’ve thatwe felt would theportion be extracted › HENRY A. NASRALLAH, MD ❙ Vol 57,No12s 1 EARN 2.5 FREE CME CREDITS CREDITS CME 2.5FREE EARN ❙ December2008 although most studies favor myotomy although moststudiesfavormyotomy cant differencesinoutcomesovertime, pneumatic dilationdonotshowsignifi - treatment. this diseaseandtherisksinherenttoits ration, highlightingtheseriousnessof hemorrhage afteranesophagealperfo- ultimately diedfromanuncontrollable riod hehadseveralcomplications. He formed. Duringthepostoperativepe- achalasia, aHellermyotomywasper- Due totheseverityofpatient’s ❚ risk ofesophagealperforation. nonsurgical therapies, andthoseathigh of age, thosewhohavefailedrepeated patients whoareyoungerthan40years over dilation. VOL 58,NO2/FEBRUARY 2009 Patient’s course BIPOLAR SPECTRUM DISORDERS BIPOLAR highlights Txrisks S5 THROUGH THE JOINT SPONSORSHIP OF OFTHE CINCINNATI UNIVERSITY AND DOWDEN HEALTH MEDIA. THIS CME ACTIVITY IS SUPPORTEDTHIS BY CME ACTIVITY AN EDUCA Surgical treatment is indicated for Surgical treatmentisindicatedfor and mood disorders in primary care and mooddisorders inprimary andmanagingpsychotic Recognizing and mooddisorders. of patientswithpsychotic managethecarehow experts care andfiby primary nd out Read acasereferred to psychiatry DON’T MISS THIS 12-PAGE CME SUPPLEMENT ■ AVAILABLE ONLINE AT JFPONLINE.COM THE JOURNALOFFAMILY PRACTICE 5 TIONAL GRANT FROM ASTRAZENECA AND DEVELOPED 1. TutuianD, Pohl di- of overview an Achalasia: R. References Department ofDefense. be construedasoffi cialoras refl ectingtheviewsof herein are theprivateviewsofauthorandare notto vant tothisarticle.Theopinionsorassertionscontained The authorreported nopotentialconfl ict of interest rele- Disclosure [email protected] ter, 300HospitalRoad,FortGordon, GA30905;drew. munity Medicine,D.D.EisenhowerArmyMedicalCen- Drew C.Baird, MD,DepartmentofFamilyandCom- Correspondence 2. Spechler SJ, Castell DO. Classifi cation of oesopha- 3. Farrokhi F, Vaezi MF. Idiopathic (primary) achalasia. 4. Levine MS, Rubesin SE. Diseases of the esopha- 6. of V,treatmentNon-surgical Annese G. Bassotti 5. Roberts KE, Duffy AJ, Bell RL. Controversies in the } } } } } FACULTY

2007;16:297-303. agnosis andtreatment. geal motilityabnormalities. Orphanet J Rare Dis. Orphanet JRare 2005;237:414-427. gus: diagnosiswithesophagography. . sia. treatment ofgastroesophageal refl achala- and ux 2006;12:5763-5766. STEPHEN F. PARISER, MD DAVID J. MUZINA, MD JOSEPH F. GOLDBERG, MD DONALD W. BLACK, MD HENRY A. NASRALLAH, MD, PROGRAM CHAIR World JGastroenterol. 2007;2:38. J GastrointestinLiverDis. World JGastroenterol. 2006;12:3155-3161. Gut. 2001;49:145-151. Radiology. 1/22/09 11:17:07 AM AM