Boerhaave\u27s syndrome is a potential lethal condition which presents not only a diagnostic but also a therapeutic challenge. Errors in diagnosis are usually caused by unawareness of its varied and atypical presentations. All clinicians need to be aware of this lethal disease, its frequent unusual presentations and the importance of early diagnosis

Fatimi, Saulat H.

Hussain, J

Khalid, I

Salahuddin, N

Zubairi, AB

English

eCommons@AKU

eCommons@AKUDepartment of Medicine Department of MedicineJanuary 2004Boerhaave's syndrome: a diagnostic dilemma in theemergency roomAB ZubairiAga Khan UniversityI KhalidAga Khan UniversitySaulat H. FatimiAga Khan University, saulat.fatimi@aku.eduJ HussainAga Khan UniversityN SalahuddinAga Khan UniversityFollow this and additional works at: https://ecommons.aku.edu/pakistan_fhs_mc_med_medPart of the Surgery CommonsRecommended CitationZubairi, A., Khalid, I., Fatimi, S., Hussain, J., Salahuddin, N. (2004). Boerhaave's syndrome: a diagnostic dilemma in the emergencyroom. Journal of Ayub Medical College, 16(2), 75-77.Available at: https://ecommons.aku.edu/pakistan_fhs_mc_med_med/570J Ayub Med Coli Abbottabad 2004;16(2) CASE REPORT BOERHAAVE'S SYNDROME: A DIAGNOSTIC DILEMMA IN THE EMERGENCY ROOM Ali Bin Sarwar Zubairi,* Imran Khalid,** Saulat H. Fatimi,*** Javed Hussain,* Nawal Salahuddin* Departments of*\1edicine, **Biologicaland Hiomedical Sciences, and ''"'*Surgery, The Aga Khan L'niversity, Kar:1chi Bocrhaavc·s syndrome is a potential lethal condition which prc:-,cnts not only a diagnustic but also a therapeutic challenge. Errors in diagnosi:-> are usua!ly caused by unawareness of its varied and atypical presentations. All clinicians need to be aware of this lethal disease, its frequent unusual presentations and the importance of early diagnosis. Key\Yords: Hoerhaavc 's Syndrome, oesophageal mpturc, 1:-.iTRODUCTION Boe-r! wave· s syndrome or spontaneous esophageal rupture is c1 rare, but potentially lethal condition. It usually foliO\VS forceful vomiting with subsequent mediastinitis am\ in the absence of therapy is as~ociatcd with high morbidity and mortality. lkeause of the lack of <Jwarencss about this rare condition, it may go unrccognizcd. 1 The prc~cnting picture mimic.s other common conditions and as a result crucial intervention is delayed. Mortality rates as high as 100 percent have been reported in some case-series artcr one week or nonsurgical ml:dical carc.n \Vc present the case of a ~patient with spont:lncous esophageal rupture !Tl \vhich the diagnosis W<ls delayed because of unusual presentation. CASE REPORT An X2-ycar-old retired electrical engineer presented to the emergency room with severe left sided chc~t pain and difficulty breathing for the past three hours. His past history included mild asthma and ischemic heart disease. He had had coronary artery bypass surgery about 15 years back. His attendant, gave the history· that \vhile having lunch, the patient had three episodes of vomiting follmvcd by intense chest pain and shortness of breath. He \Vas managed as angina in another hospitaL and \vas tre<'ltcd with sublingual nitroglycerine without any relief in symptomatology. Physical examination revealed an anxious gentleman in mild respiratory di$trcss. Vital signs revealed blood pressure of I 36/70 111111 Hg, pulse rate XX beats per minute, re:-:;piratory rate 28/min, and temperature of 36.5" C. Chest atLSCtdtation revealed din1inishcd breath sounds, egophony, dccn:ascd tactile fremitus und dullnc;-;s to percussion at the left hmg basL'. The abdominal examination revealed mild epiga~tric tenderness but no guarding or rebound tcndcmcss. Laburatory studies showed a whi1l' blood cell count or I I ,000 cells per micro liter, with 65?--0 ncutrophils n11d 26.3 1;.-;, lymphocytes. J·:!cctrocardio-gram showed no new ischemic changes. The chest x-ray revealed left-sided alveolar infiltrates along vv·ith pneumothorax (Figure-1 ).The arterial blood gases on 3 !itc.-s or oxygen, ITvealcd a Pa02 or 51.1 mml !g, PaC02 of 34.2 mmi lg, pll of 7.44 and artcfial oxygen saturation of 87 .7%. Fig11rc I Chest radiogr·aph showing left sided infiltrates n·ith pneumothorax Based on clinical symptomatology and radiological findings, the diagnosis of left sided aspiration pneumonia with small pneumothorax was made ami patient wa.'> admitted under pulmonology scrvtce. Ultrasound guided thoracentesis \Vas suggested but the attendant refused it. The patient was started on empiric antibiotic therapy with high flow supplemental oxygen. The patient's condition deteriorated the next morning:. He \Vas found to be hypotensive and in respiratory distress. He vva~ intubated with size X.O endotracheal tube and a len chest tube was urgently placed. The chest tube drained about 400 ml of dirty grey, foul smelling fluid which also contained food particles. The biochemistry or fluid showed glucose of l5mg/dL protem of 788mg/dl. lactate dehydrogenase ol" 4hSO lUlL and amylase of 65770 IU/L. The pleural fluid was sent for culture and sensitivity. Cardiothoracic surgery was consulted and the patient wa$ urgently taken to thL operating room. He undcn\ cnt a lcfi posteroi;Hcral thoracotomy and the pleural cavity \vas entered through the sixth intercostal space. Approximately one liter of greyish brown tluid \Vas drained. A 6-cm perforation wa:-> identified just proximal to the gastroesophageal junction and extcnsi\·c mediastinul necrosis \\'il'i seen. The perforation \Vas closed primarily in two layers with an intercostal nap overlay. Diversion surgery by esophagostomy \Vas al.-.o done and a feeding jc_junostomony was created (Figurc-2). Figurc-2: Chl•st radiograph after surgical intervention The patient was tran\>ferred to the intcn~ive care unit. The post operative course \Vas complicated by severe sepsis I shock requiring multiple inotropic drugs. Blood cultures grew E. Coli. Pleural fluid grew gram negative rods and gram positive cocci. After 3 da_ys, he ckvclopcd acute renal failure requiring dialysis. His condition deteriorated further and he developed multi organ failure. He died after 20 days in the !CU. DISCUSSION This syndrome was first described by !-Ierman Bocrhaavc ( 1669-173S) from L~idcn, The Netherlands (A troscis, nee dcscripti prui:-;, morbid historica). 4 !lis patient was a 50-year-o!d Gmnd Admiral vvho died in 1723 after I 8 hours of self induced vomiting which resulted in esophageal rupture. Since then, the term Boerhaave's syndrome is used to describe a full thickness perforation of the esophagus not associated with external trauma, instrumentation or foreign bodies. The diagnosis of this uncommon condition is often missed or delayed, because of a non-specific 7(l J Ayub Med Coil Abbottabad 2004;16(2) presentation in the majority of cases. This syndrome can be confused with pneumonia, pneumothorax. lung abscess, myocardial tnfarctinn, pancreatitis, dissecting aortic aneurysm, pu[monury embolus, pericarditis or pcrfor<ttcd ulcer. 1 Early diagnosis and prompt surgical intervention is life saving. ;\ delay of 12 hours or more bct\\·ecn symptoms and operative repair is associated vvith a reported J6 percent mortality; with a delay of .24 hour:; this almo.o;t doubles to 64%.' It may apprnttch nearly l 00 percent \Vithout any ,o;urgical intervention.~ Bocrhaave's syndrome is traditionally associated with the triad of vomiting, chest pain and subcutaneous emphy.-,cma as described by Mccklcr1', but the patient may present with atypical ,o;ympto111s so reliance on a 'classic' presentation may be mis!eading 7• Unusual clinical features should also be kept in mind while evaluating patients with a sw>picion of esophageal rupture. The mechani.''dll of injury as c!ucitbtcd by Rogers and associates ~ is an increase in intragastric pressure coupled with opening of lmver csophagcul sphincter to allow entry of gastric contents into the lower esophagus. If the upper sphincter docs not open, hydrostatic pressure within the esophagus rise~ to a pnint \vhen the wall gives way at its weakest point; the po~terolatcr;ll wall nn the !eft. Spillage uf corrosive gastric acid. cn7ymes, food ;md bacteria into the mediastinum le;_rd.c., to SC\"Cre 111ediastinitis and empyema caw.,ing a sepsis sync!rorne and shock Chest radiograph may show <-l pleural effusion, pneumothorax or the ·y' sign of Naclerio, a density behind the left cardiac border. Pate ct al~ in their series found that the initial chest roentgenogram was abnormal in 97(;_-;) of the patients but was interpreted as comrxrtiblc with perforation of esophagus in only 27%. Contrast studies arc positive in 75 percent of cases; however. a lack of extra\'asation docs not necessarily exclude a perforation. Pleural fluid C}.;amini.ltion can be an adjunct to- diagnosing Bocrhaavc' s syndrome as described by Drury et aL'J Their patient had a negative barium swallow hut subsequent pleural fluid testing showed an acidic pl-f with an increased amylase level of -salivary origin. Plcur;_d effusion cytnlogy showed undigested food particles. The role of upper CJ! endoscopy in the presence of esophageal rupture is contro\'crsia! because .'mille authorities believe that insufflution of air can extend the perforation. Various treatment options are available, both surgical and non-surgicaL Non-surgical treatment is recommended for patients with sma!! perforations, contamination confined to the mediastinum and late recognition (more thun 24 hours) of an esophageal perfOration, as the surgical mortality at 24 hour~ becomes equal to that of a more conservative approach. 111 Sucgical intervention which includes open thoracotomy. debridement il!ld surgical clo~urc or the perforation is recommended if' the diagnosis is made early. Divcr~ion surgery by csophagostomy, csophagecto111y or T-tubc di\'crsinn 11 ts usually rc,crvcd for critically ill patients. Use of self expanding metallic stcnt has been reported to be successful if diagnosis is made within 24 hours. 11 The truism that a diagnosis will only be made if it is borne in mind applies with particular force to this life threatening condition that charactcri;.cs spontaneous esophageal perforation. All clinicians need to be aware of the manifestations of Boerhaavc's ~yndromc and the importance or early diagnosis. REFERENCES lkndcrson JA\t Peloquin AJ. Boerhcwve revisited: sp<llll<meous ocsophage;d per!\mtlion as a di;q,!:nostic 1msqueradc1·. Am J :vlcd 1 'JI\9: .S(1 5~9-(l 7. 2. Pate J\V, \\'alker \VA, Cok Fl-1 Jr. Owen FW, Jobnson WH. Spontaneous rupture or c·sopbagus: a .10-ycar expl·ricnce. \nn Thorae Surg 19R9; 47:689-n ·\tlar S. l!ankins JR. Suter C:vl. Coughlin TR. Sequeira A. \1cl.;ntghltn .IS Fsopktgral perforation a thrrapcultc eil;tlknge_ Ann ThonJC Surg 19')0~ 50"45-51 4 8ocrha:l\T H_ ,\[rons. twc dcset·ip!i prius, morbid histonca. Secundum medicac art is leges conscnpta. I .ugduni Address for Correspondence: J Ayub Med Coli Abbottabad 2004;16(2) 8at:lVorum Buutes klll:llla, 17:::'4 {English translation Derbes V .J, Mitchell RF Bull Mcd 1.ibt· Ass 10S.~_ 43·217-40. 5. Blichcn-Toll \1 Sp()nt;mcous Ol'sophageal ntpturc An cv;duatmn or the results or treatmcn! I ')44-1 ()(J9. Scand J Thorac CardiD\asc Surg 1971.:-:111-5 6. Bo:rym;,k1 LM. London .IF __ Vlisccllancous diseases of the esophagus: esophageal in_itu·y and rupture. '{amada 'I, editor Tl·xtbook uf G:~strocn!crology_ Phil:uklphia. Pi\: 1 .ippincott. 1()')1, pp.lll/2-5. 7. \Valkcr WS, Cameron l:::WJ, \Va1baum PR. Diagnosis and m;magemcnt or spontaneous transnmral ruptmc or !he oesophagus (13oerhaave's syndrome) !~1 .I Surg 19S5: 72:204-7. R. Rogers LF. Puig A W, Dooley BN. Cuello I. Ui'-lgnostic cnn~iderations in mcdi'-lstinal emphysen1a: a pathophy~io­logic-rocntgcnologic appruach to Bocrhaave·:.. syndrome :md spontaneous pncumomcdwslinum. Am .I Rocntgcnol Radium Thtr Nucl Mcd !972: 115:4 1)5~511. 9. Drury M, Anderson W, HeiTner .JL Dic1gnostic valut of pkur;d lluid cytolugy in occult I3Dcrhaa\·e"s syndrome. Chest !9S7: 92:tJ95-8. 10. Lyons WS, Sercmetis \1G, deGuzman VC, Pc;!body J\V Jr. Ruptures ;md pcrf"oration;. or the oesophagus: the care l"or con~crva!ive supportivL· management Ann tho rae surg I rns: 25:346-50. II. Ojima l !, Kuwano H. Sasaki S, Fujisawa T. Ishibashi Y Successful late management or spontaneous esophageal rupture using T-lubc mcdiastinoabJomin<ll dr:1inage. Am J Surg200l: 1S2:192-(). 12. Chung \Hi. Kang Dll, Park DK, Park JL Park II C. Kim Jll Successful 1reatmcnt uf Bocrhaave·s synd1·ome \vith endoscopic insertion or a self expandable metallic ;.tent: report or three cases :md a n~\·iew or the literature. Endoscopy 2001; 33:894-7. Dr Ali Bin Sarwar Zubairi, Department of Pulmonology, Aga Khan University Hospital, Stadium Road, Karach-74800, Pakistan. Email: aliJ.ubairi(!::1laku.cdu 77 

Boerhaave\u27s syndrome: a diagnostic dilemma in the emergency room

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Boerhaave\u27s syndrome: a diagnostic dilemma in the emergency room

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