A 60 y.o male with altered mental staus is found wandering; you notice something in his chest.

what happened to him?

what happened to him?

Our patient had an implantable loop recorder that eroded through the skin. The device is covered by Medicare and can stay implanted for three years. The European College of Cardiology recommends implantation in the early stages of a syncope evaluation so they may be seen more frequently in the ED.

placement of a loop event monitor

placement of a loop event monitor

Kaoru Tanno’s article reviews the studies which have been done comparing implantable loop event monitors with conventional syncope evaluations which include 2-4 weeks of external heart monitoring, electrophysiological studies and tilt table testing. The implantable monitor was more successful in making a diagnosis than conventional testing. There have been no studies showing that making the diagnosis improves quality of life or length of life.

2.JPG

The complications of the implantable loop event monitor include infection of the pocket and misdiagnosis since it has difficulty distinguishing at times between VT and SVT. The complications occur in 1-5% of patients.

Our patient had his event monitor removed in the ED.

Krahn A, Klein G. et al. The high cost of syncope: cost implications of a new insertable loop recorder in the investigation of recurrent syncope. Am Heart 1999. may, 137(5)870-87

Tanno K. Use of implantable and external loop recorders in syncope with unknown causes. Arrhythm 2017 v33(6) . This reference includes the RAST study comparing external recording x 2-4 wks, with EP testing and tilt table vs 1 yr of an implantable recorder.

Onuki T, Ito H. Ochi A. Single center experience in Japanese patients with syncope. J Cardio 2015;66:395-402.

Ruwald M, Zereba W. ECG monitoring in Syncope. Prog Cardiovas Dis. 2013. 56:203-210.

A 23 y.o. female involved in a head on MVC, is awake and alert. She is taken to the OR and post op does not wake up.

A chest Ct looking for PE is neg for PE but shows the findings below.

patchy ground glass infiltrates are seen HInt: she had a leg fracture

patchy ground glass infiltrates are seen HInt: she had a leg fracture

What could be wrong?

our patient had fat emboli from a femur fracture

our patient had fat emboli from a femur fracture

Our patient had a fat emboli syndrome. Fat emboli occur in nearly   all patients that have fractures of the long bones but in a small percentage this results in fat emboli syndrome (FES). This occurs in 3-10% of ortho trauma patients  and the mortality is 10-20%. Manifectations occure 24-72 hours after injury  Embolized fat is degraded into free fatty acids, which cause toxic injury and inflammation causing edema. 

mini infarct in the brain on her T2 weighted images.

mini infarct in the brain on her T2 weighted images.

The classic triad involves lung, brain and skin. It was first described by Zenker in 1862. The pulmonary manifestations are the most common with hypoxia as the main presentation.  The neuroabnormalities include confusion, focal deficits and seizures.  There is a petechial rash in 20 per cent of cases often on the head and neck.  Lipiduria may also be present.  Fat globules can be seen in bronchoalveolar lavage specimens.

fat globues with Oil red O stain in macrophages on bronchoalveolar lavage.

fat globues with Oil red O stain in macrophages on bronchoalveolar lavage.

In the correct clinical setting, as in our patient the diagnosis can be made.  Other forms of trauma have been associated with fat embolism syndrome including liposuction and CPR.  There are also rare cases of nontrauma-related FES including sickle cell anemia, pancreatitis and lipid infusions.

 

Albumin has been recommended for treatment since it binds fatty acids. It is not clear if steroids are effective.

Our patient was unable to speak after surgery secondary to cerebral fat emboli and mini strokes.  She has gradually improved and now is able to speak although she is very slow to respond to questions and will be discharged to rehab.

petechiae in fat embolism

petechiae in fat embolism

Shaikh N, Emergency management of fat embolism syndrome. 2009 J Emerg Trauma Shock. Jan-April 2(1);19-33.

Fourme T, Viellard-Baron. Et al. Early fat embolism after liposuction. Anaesthesiology 1998 ;89:782-4.

Verdrinne J, Guillauma C, Gagnieu M. et al. Bronchoalveolar lavage in trauma patients for diagnosis of fat embolism syndrom. Chest 1992;102:1323-7.

Gangaraju R, Reddy V, Marques M. Fat embolism syndrome secondary to bone marrow necrosis in patients with hemoglobinopathies. South Med 2016;109-549.

A 49 y.o. woman presents with diarrhea and abdominal pain.

what do you see on her CT?

tera.JPG

Our patient had a mature teratoma with a malignant transformation.  She had had a previous US in 2017 which showed a bicornuate uterus and a mature teratoma with a second cystic mass near the L ovary.  Mature cystic teratoma is the most common benign ovarian tumor. Rarely, they undergo malignant transformation. The term teratoma comes from the

Greek words for “monster” and “tumor”.

teratomapath.jpg

Teratomas are germ cell tumors which contain fat, hair , teeth and cartilage. As a result when they become malignant they are often squamous cell tumors but can be adenocarcinomas or melanomas. A teratoma can be non-malignant and yet highly aggressive, growing rapidly.  This happens in the “growing teratoma syndrome” where  chemotherapy eliminates the malignant elements of a mixed tumor and the pure teratoma which is left grows very rapidly.

A nine y.o. with headache has a teratoma with teeth in the brain

A nine y.o. with headache has a teratoma with teeth in the brain

Teratomas are thought to originate in utero but many are not diagnosed until adulthood. They can be diagnosed in utero on US and if they are large can steal the blood from the fetus  causing heart failure.  Later in life, ovarian teratomas can be responsible for anti-NMDA receptor encephalitis.(N-methyl-D-aspartate receptor  antibody encephalitis) They can also secrete thyroid hormone or alpha feto protein.  While they most often occur in the ovaries, testes and sacrum they can occur in the spinal cord and brain.

Scavuzzo A, Rios S, Arturo X, et al. Growing teratoma syndrome. 2014 Case Reports in Urology. 139425. Doi:10/1155/2014/139425. PMID 25197607.

Chang A, Ganz P, Hayes D, Kinsella T, et al.  Oncology: An Evidence-Based Approach. 2007. Springer Science & Business Media  p. 848.

Gonzalez-Crussi F. Extragonadal teratomas. Atlas of Tumor Pathology. Second series. Fascicle 18;Armed Forces Institute of Pathology, Washington D. D.

Jitsumori M, Munakata S, Yamamoto T. Malignant transformation of mature cystic teratoma after a 10-year interval. 2017. Case Resports in Obstetrics and Gynecology.  Article ID 2947927.

A 61 y.o. woman is depressed because her cat died; she is drinking alcohol and is sent to the Psych pod.

She has snoring respirations and a rhythm strip is shown below.

torsades.JPG

What is the problem here and what is the treatment. Her EKG post resuscitation is shown below.

A toxicologist happened to be in the psych pod and suggested amiodarone not be used. Why?

A toxicologist happened to be in the psych pod and suggested amiodarone not be used. Why?

BEWARE AMIODARONE

Amiodarone is marketed as a class III antiarrhythmic but actually is also a class I and blocks sodium channels.  It should not be given in several situations where there is a wide complex tachycardia.

1.   WPW with a fib( use procainamide)

2.   Wide complex tachycardia in a patient on methadone. Methadone prolongs QT

3.   Anyone with “slow v tach” (rate < 120). This kind of idioventricular rhythm can be due to reperfusion arrhythmias, TCA , cocaine OD or hyperkalemia. If amiodarone is given  it can cause cardiac arrest.

Lidocaine is also contraindicted in an idioventricular rhythm and also can cause an arrest.

SO USE AMIODARONE:

amio.JPG
ANd EXCEPT in patients at risk for hypomagnesemia

ANd EXCEPT in patients at risk for hypomagnesemia

AND EXCEPT when the patient is in “slow v tach” because it might be a reperfusion arrhythmia, hyperkalemia or sodium channel blocker OD.

AND EXCEPT when the patient is in “slow v tach” because it might be a reperfusion arrhythmia, hyperkalemia or sodium channel blocker OD.

This is a case of v tach with av dissociation and fusion beats. courtesy of Amal Mattu

This is a case of v tach with av dissociation and fusion beats. courtesy of Amal Mattu

Marill K, deSouza IS, et al. Amiodarone is poorly effective for termination of ventricular tachycardia.  2006. Ann Em Med. 47(3):217-24.

Ortiz M, Martin A, Arribas F. et al. Randomized comparison of IV procainamide vs IV amiodarone for acute treatment of wide QRS tachycardia. Eur Heart J. 2017 38:1329-35. Doi 10.1093eurheartj/ehw230.

ACC/AHA/ESC. Guidelines for management of patients with ventricular arrhythmias and prevention of sudden death. Executive summary. 2006. Circulation.

The EKGs except for our patient were taken from Amal Mattu’s lecture which is available free on the ACEP bonus free eCME web site. Thanks to Dr. Liss for the case.

A 44 y.o. woman presents with R chest pain

she has been treated for three weeks for pneumonia with azithromycin and cefdinir but continues to worsen.

because of a persistent infiltrate on cxr a PE protocol CT was done.

because of a persistent infiltrate on cxr a PE protocol CT was done.

what do you notice on the PE protocol ct?

what do you notice on the PE protocol ct?

Our patient did not have a pulmonary embolus although there is a blockage in the pulmonary artery, a mass extends beyond the vessel itself. 

The mass extends beyond the pulmonary artery

The mass extends beyond the pulmonary artery

Our patient had fibrosing mediastinitis which is thought to be the sequela of infection with Histoplasma capsulatum.  It begins as an asymptomatic pulmonary infection and spreads to mediastinal nodes. It can result in a granuloma commonly, but depending on the host response,  some patients develop fibrosis with involvement of vessels, heart or esophagus.  The organism is ubiquitous in the Ohio and Mississippi river valleys. People living in these areas are exposed in childhood and most have an asymptomatic illness.

fibrosing mediastinitis with bronchial narrowing

fibrosing mediastinitis with bronchial narrowing

Whenever soil is disturbed the spores become airborne and can be sucked into airconditioning systems. An outbreak of histoplasmosis occurred in a school in Ohio.  On Earth Day, a courtyard was raked and the entire school building was contaminated with spores, infecting 384 students.

 

While histoplasmosis accounts for 80% of cases, rarely, tuberculosis, aspergillus, sarcoid and Wegeners have all been reported as causes. There is no treatment other than palliative care: stenting of airways, coiling bronchial vessels that develop because of SVC syndrome or debulking . Our patient had a resection of the fibrotic part of the pulmonary artery on the R with a bovine patch.  She is doing well, although her cxr still shows atelectasis of the RML.

Goodwin RA, Nickell JA, Des Prez RM. Mediastinal fibrosis complicating healed  primary histoplasmosis and tuberculosis . Medicine (Baltimore 1972;51:227.

 Wheat LJ, Slama TG, Eitzen HE, et al. A large urban outbreak of histoplasmosis; clinical features. Ann Intern Med 1981;94:331.

 Kern R. Peikert T, Edell E, et al. Bronchoscopic mangement of airway compression due to fibrosing mediastinitis.  Ann Am Thorac Soc  2017;14:1353.

A 40 y.o. male complains of wrist pain after a fall.

what do you see?

signet ring of the scaphoidf.JPG

The signet ring sign is present on the xray.

The  signet ring of the House of Targaryen, the three headed dragon.

The signet ring of the House of Targaryen, the three headed dragon.

The  signet ring sign can be seen in the scaphoid when it demonstrates  a cortical ring as a result of foreshortening on a PA projection.  This suggest volar rotation of the scaphoid with scapholunate dissociation.  It can occur in normal wrists if the xray is taken with the wrist flexed. If this happens the lunate would appear more triangular as it would be flexed as well.

The scaphoid is rotated causing the “ring” sign

The scaphoid is rotated causing the “ring” sign

 In addition to the signet ring of the Game of Thrones and the signet ring of the scapholunate dissociation, the ring can be see in bronchiectasis and papillary necrosis. The signet ring can be seen in bronchiectasis when the dilated bronchus and pulmonary artery branch are seen in cross section.  Normally, the bronchus and pulmonary artery branch are the same size but in bronchiectasis the bronchus is much larger.

signet ring in brochiectasis

signet ring in brochiectasis

The signet ring appearance can also be seen in an IVP with renal papillary necrosis where contrast fills the circumferential fornix of the necrotic papillae.

signet ring sign of papillary necrosis  where the sloughed papilla is surrounded by contrast in the calices.

signet ring sign of papillary necrosis where the sloughed papilla is surrounded by contrast in the calices.

Abe T, Doi K, Hattori Y. the clinical significance of the scaphoid ring sign:  a study of normal wrist xrays. 2008. Journal of Hand surgery, https://doi.org/10.2277/1753193407087572.

Ouellette H, the signet ring sign. Radiology. 1999;212(1):67-8Doi:10.1148/radiology.212.1.r99jl2067

Burgener F, Kornano M,Pudas T. differential Diagnosis in conventional Roadiology. Thieme. (2008) ISBN:1588902757

Jung D, Kim S, Jung S et al.  Renal papillary necrosis: review and comparison of findings at multi-detector row CT and intravenous urography. Radiographics. 26(6): 1827-36.

Doi:10.1148/rg.266065039

A 19 y.o. woman falls forward on to her chin; she has a 1 cm lower lip laceration and severe L ear pain

what do you see?

1brent.JPG
Hint the ear pain is her major complaint

Hint the ear pain is her major complaint

Our patient had a fracture of the condylar head of the mandible which was dislocated from the glenoid fossa into the sigmoid notch. Mandibular fractures are a common result of facial trauma with 19-52% involving the condyle. The condyle can fracture into the external auditory canal as happened in this case.

brent.JPG
condylar fracture on the L

condylar fracture on the L

In adults the repair of condylar fractures often involves closed reduction and intermaxillary fixation with arch bars for six weeks.  Surgery is complicated and not only risks damage to the facial nerve but prolonged jaw immobilization can result in muscle atrophy.   In children these are managed without surgery and often in novel ways.  The Kirschner wire is a threaded wire that can applied percutaneously into the displaced fragment and it is pulled into alignment.

intermaxillary fixation.JPG

Intermaxillary fixation is maintained for six weeks. The picture below shows how a Kirschner wire is used to align the displaced condylar fracture.

condylar fr kursh wire.JPG
condylarkirschboy.JPG

Our patient had near normal occlusion .  It was thought that reduction of the fracture could not be maintained  with a plate because there was not enough bone on the proximal fragment and she  did not want to pursue maxillomandibular fixation. The complication of ankylosis (fusion of the joint with inability to move the jaw) was addressed by physical therapy

Valiati R, Ibrahim D, Abreu M, et al. The treatment of condylar fractures: to open or not to open? A critical review of theis controversy.  Int J Med Sci 2008;5(6): 313-316.  

Choi K, Yang J, Chung H, Cho B. Current concepts in the mandibular condyle fracture management Part II: open reduction versus closed reduction. Arch Plast Surg 2012. 39(4);

Kim J, Nam H, closed reductionof displaced or dislocated mandibular condyle fractures in children using threaded Kirschner wire and external rubber traction. 2015 Oral & Maxillofac Surg . 44:1255-59.

anatomy of the jaw

anatomy of the jaw

A 20 y.o. woman presents with a cardiac arrest; ROSC is obtained.

Would you activate the cath lab based on her EKG?

ekg1.JPG

The cath lab was called for this patient.

The second EKG however convinced the cardiologist not to cath the patient.

Ischemic changes have resolved

Ischemic changes have resolved

. The likelihood of an acute coronary occlusion in a 20 y.o. with presumably a hypoxic arrest from a fentanyl OD is small.  These EKGs more likely represent demand ischemia from prolonged hypoxia.

Cardiac arrest accounts for 500,000 deaths in the US and Europe annually, with out-of-hospital arrest survival at <15%. It is being debated whether cath should be done urgently on patients with return of spontaneous circulation whether or not they have ST elevation. Current studies often demonstrate selection bias in studies of cardiac cath post arrest with only those with a perceived favorable neurologic outcome being taken for cath. Unwitnessed arrests, CPR for 30 minutes or longer prior to ROSC and lack of spontaneous respirations are predictors of poor outcome.

 

The current points to remember are:

1.   Patients presenting with out of hospital cardiac arrest in a shockable rhythm have a high probability of coronary disease

2.   Observational case series suggest that among patients resuscitated with persistent ST elevation, the prevalence of CAD has been shown to be 70-85%.  In patients resuscitated with no persistent ST elevation, the incidence of coronary disease is 25%.

3.   The ongoing ARREST trial will address cost per life saved and should provide further guidance.

Our patient was declared brain dead and the family donated her organs.

Yannopoulos D, Aufderheide T, et al. The evolving role of the cardiac catheterization laboratory in the management of patients with out-of-hospital cardiac arrest: A scientific statement from the American Heart Association. 2019 Circulation Feb 14.

Reddy S, Lee K.  Role of cardiac catheterization lab post resuscitation in patients with ST elevation MI. Curr Cardiol. Rev. 2018;14(2)85-91.

Chan P, McNally B, Tang F, Kellermann A. Group CS. Recent trands in survival from out-of –hospital cardiac arrest in the United States. Circulation 2014;130(21):1876-82.

A 25 y.o. male with cushing's syndrome presents with abdominal distention.

do you see a cause for his abdominal complaints?

intussuseption.JPG

As observed by the commentators, our pt had an intussusception of the small bowel.  A Dutch physician, Barbett, was the first person to refer to intussusception in 1674.

intussuseptionf.jpg

 This is an important etiology of abdominal pain to consider in adults.  The classic triad of abdominal pain , vomiting and currant-jelly stool found in children is rarely seen, leading to delays in diagnosis. In the case presented by Teng referenced below, a 37 y.o. male had symptoms for five years before the diagnosis was made on CT. When he underwent laparotomy,  no intussusception was found; just thickened bowel loops.  Presumably it had spontaneously reduced each time he presented. Intussusception accounts for 1-5% of bowel obstructions in adults

 In contrast to pediatric intussusceptions which are managed with air contrast enemas, treatment in adults can involve surgery. There is often a lead point for intussusception in adults which can be a tumor and enemas are not recommended because of the fear of perforation and spread of tumor cells.

intussusception in a pt with cancer

intussusception in a pt with cancer

lead point for the intussusception shown above, small bowel tumor

lead point for the intussusception shown above, small bowel tumor

In children,  Henoch Schonlein purpura or hypertrophy of Peyer’s patches can be the lead point of the intussusception.  Peyer’s patches hypertrophy in several diseases including prion disease, polio and salmonella. 11% of pediatric intussusceptions reduce spontaneously.

Intussusceptions also reduce spontaneously in adults and that was the case in our patient.  A repeat CT showed no abnormality and his symptoms resolved.

 Teng L, Chng, U.  Adult Intussusception, Perm J. 2015 Winter;19(1):79-81.

Spiridis C, Kambaroudis A, et. al. Intussusception of the small bowel secondary to malignant metastases in two 80 year-old people: a case series. J Med Case Resports 2011;5:176.

Barbette P. Ouevres Chirurgiques at Anatomiques. Geneva: Francois Miege; p. 1674.

Stewardson R, Bombeck C, Nyhus L. Critical operative management of small bowel obstruction. Ann Surg. 1978;187:189-193.

A 48 y.o. complains of inability to urinate and lower abdominal pain

What is wrong?

stercoral colitis f.JPG

Our patient had stercoral colitis.This is an inflammatory colitis caused by increased intraluminal pressure from impacted fecal material in the colon.  The rectosigmoid is the most frequently involved. The most important complication is perforation and mortality for the condition is 32-57%.

The condition is almost always found in cases of chronic constipation, advanced age and medical cormorbidities. Colonic wall thickening is > 3 mm, and air migrates from the colon lumen into the wall of the colon. In addition to constipation; diseases which cause obstruction of transit such as Hirschprung’s, hypercalcemia, and Chagas disease can cause stercoral colitis.

stercoral colitis causing perforation with free abd fluid, and discontinuity of the sigmoid in an opiod user

stercoral colitis causing perforation with free abd fluid, and discontinuity of the sigmoid in an opiod user

Our patient presented with abdominal distention and no urine output.  She was found to have urinary retention with a creatinine of 15  and a K of 5.0. She had bladder obstruction with hydronephrosis bilaterally.  A Foley was placed and 4L of urine was obtained. She was disimpacted at the bedside by general surgery. On the second day after disimpaction her Cr was 1.0 and she was discharged.

toxic megacolon occurs in 1% of patients with c diff as in this patient.

toxic megacolon occurs in 1% of patients with c diff as in this patient.

Unal E, Onur M, Baci S, et al. Stercoral colitis: diagnostic value of CT findings. 2017 Diagn Interv Radiol. Jan;23 (1):5-9.  

Serpell J, Nicholls R. Stercoral perforation of the colon Br J Surg 1990;77:1325-1329.

Core Topics in General and Emergency Surgery (6 ed.). Elsevier. 2019. Pp216-233.

A 33 y.o. male presents with leg swelling(and Oh, by the way, a rash on the penis)

Why are his legs swelling?

Our patient had nephrotic syndrome from membranous glomerulonephropathy caused  by syphilis.

 

Membranous nephropathy can be caused by hep B, HIV,  hep C, NSAIDS, Li,  and gold salts.  It is also caused by autoimmune diseases like lupus and occasionally can be caused by solid cancers.  In the case of our patient, all other causes were ruled out and he improved with penicillin alone. 

pathologic findings of membranous glomerulonephropathy

pathologic findings of membranous glomerulonephropathy

Renal disease is also caused by heroin and cocaine.  7 of 8 heroin addicts that present with nephrotic syndrome have membronoproliferative glomerulonephritis in one study.  Blacks are 3-7 times more likely to suffer renal disease. 

Cocaine releases endothelins which induce endogenous angiotensin converting enzyme causing vasoconstriction.   This can result in renal infarct, endothelial injury  or acute rhabdo from muscle breakdown.  These effects can be  partially blocked by ACE inhibitors. The final common pathway of endothelins is to cause fibrosis and renal failure.

endothelins produce fibrosis.JPG

Crowe A, Howse M, Bell G, Henry J. Substance abuse and the kidney  2000 QJM 93:147-152.

Van Assen S, Bakker S. Did syphilis truly strike the kidneys this times. 2005. Nephrology, dialysis transplantation Vol. 20 Issue 6 1029-1031.

Zhang Z, Hever A, Bhasin N, et al. Secondary syphilis associated with membranous nephropathy. 2018 Perin J. 22:17-062.

 

A 39 y.o. woman with lupus presents with swollen breasts and no other swelling

What could be wrong?

svc.JPG

Our patient had classic peau d’orange, but had superior vena caval syndrome and not inflammatory breast cancer. She did not have swelling of her face because of extensive chest wall collaterals. 

collaterals on the chest wall with svc thrombosis.

collaterals on the chest wall with svc thrombosis.

It was first described in 1757 by William Hunter in a patient with syphilitic aortitis compressing the SVC. The syndrome occurs in 15,000 people in the US a year.  The most common cause is malignancy with 85% reported to be caused by malignancy in 2008 but now 40% are reported in non malignant cases due to the prevalence of ports.   Other causes of SVC syndrome include: fibrosing mediastinitis, tuberculosis, histoplasmosis , actinomycosis  and thrombosis caused by pacemaker leads.

fibrosing mediastinitis causing svc obstruction

fibrosing mediastinitis causing svc obstruction

Shaikh I, Berg K, Kman N. Thrombogenic Catherter-Associated Superior vena cava syndrome. 2013, Case Resports in Emergency Medicine Volume 2013, article ID 79054, 3 pages.

Higdon M, Higdon J. The treatment of oncologic emergencies. Am Fam Physician 2006;74:1873-80.

Wilson L, Dettebach F, Yahalom. Superior vena caval syndrome. 2007 NEJM 356:1862-1869.

Cohen R, Mena D, Carbajal-Mendoza A, et al. Superior vena caval syndrome: A medical emergency? Int J. Angio 2008 spring 17(1):43-46

 

A 62 y.o. male from St. Louis presents with fever, hypoxic respiratory failure and tender inguinal nodes.

Is there a unifying diagnosis?

tu.JPG
tu2f.jpg

Our patient had tularemia or “rabbit fever”, blood cultures were positive for Francisella tularensis. He lives in south St. Louis county and was scraping a dead rabbit from his driveway and presumably inhaled aerosolized bacteria.  Ten days later he presented with fever and hypoxia.  He had painful nodes in the R groin and they were biopsied. In addition to rabbits, voles and feral hogs in Texas have been reported as reservoirs of disease.

tular2.JPG

 Tularemia is also transmitted by tick bites and deer fly bites. The disease is named after Tulare county in California where the disease was first discovered in 1911. Although generally rare, the disease is present in sporatic outbreaks in Finland and Sweden with   4,830 cases reported in Sweden  from 1984-2012. 

Cases in the US in 2016

Cases in the US in 2016

Types of tularemia include:

Ulceroglandular- the most common type where an ulcer appears at the site of the infection.

Oculoglandular- when the bacteria enter through the eye

Oropharyngeal- from eating contaminated food where the bacteria causes mouth ulcers

Pneumonic- results either from breathing the organism or bacteremia  from ulceroglandular with dissemination.

Treatment is usually with gentamycin although doxycycline can also be used. Interest in tularemia increased in modern times since it is a potential biologic weapon with only 10 to 50 bacteria being sufficient to infect a person.

Ulceroglandular tularemia

Ulceroglandular tularemia

Desvars A, Furberg M, Hjertquist M, et al. Epidemiology and ecology of tularemia in Sweden. 1984-2012. 2015 emerg Infect Dis 21(1) 32-39.

Feldman K, Enscore R, Lathrop S., et al. An outbreak of primary pneumonic tularemia on Martha’s Vineyard.2001.  NEJM 345(22): 1601-6.

Dennis D, Inglesby T, Henderson D, et al. Tularemia as a biological weapon: medical and public health management. 2001 JAMA 285(21)2763-73.

Random rabbit facts:

In the wild rabbits don’t eat carrots or any root vegetables.

Their warrens can extend 10 feet underground.

Rabbits can see nearly 360 degrees.

Ovulation is triggered by intercourse and after a thirty day gestation 4-12 kits are born.

A 69 y.o. presents with shortness of breath

What do you see on the CT of the chest?

leio.JPG

Our patient had lymphangioleiomyomatosis or LAM.  It is a kind of cystic lung disease which can be associated with tuberous sclerosis and affects women in their childbearing years. In 82% of patients the initial presentation is with pneumothorax.  There are multiple complications of lymphatic obstruction including chylothorax, chylous ascites and chylopericardium. These complications are caused by smooth muscle cells that invade vessels and airways.

Kidney tumors, angiomyolipomas are present in 30% of the patients with LAM not associated with tuberous sclerosis and int 90 % of patients with tuberous sclerosis.

skin manifestations of tuberous sclerosis include adenoma sebaceum

skin manifestations of tuberous sclerosis include adenoma sebaceum

The other three types of cystic lung disease are Langerhan’s cell histiocytosis (eosinophilic granuloma), Birt Hogg Dube (which presents with lung cysts and renal CA caused by an abnormality of folliculin) and lymphoid interstitial pneumonia.  The latter is found in autoimmune diseases like Sjogrens and is caused by an overgrowth of lymphoid tissue.

more skin manifestations of cystic lung disease

more skin manifestations of cystic lung disease

Cystic lung disease does not include: cystic fibrosis(where the cysts are found in the pancreas), emphysema, metastatic cystic lesions  or bronchiectasis where air is trapped in spaces without true walls.  Any cystic lung disease can present with pneumothorax.

 

McCormack FX. Lymphangioleiomyomatosis: a clinical update. 2008 Chest 133(2):507-16.

Lampert S, Schmid A, Wiest O, Hahn E, Ficker J. Fire-eater’s lung. Two cases and review of the literature. 2006. Disch Med Wochenschr  131(7):319-22.

Koo H, Yoo C.  Multiple cystic lung disease. 2013 Tuberc Respir Dis 74(3) 97-103

A bust in the Musee l'Homme in Paris depicts a disease. What is it?

Screen Shot 2019-03-11 at 3.21.46 AM.png

This bust depicts gumma associated with tertiary syphilis. The gumma are granulomas attempting to wall off the treponema pallidum. Gummas have are a proteinaceous mass with a necrotic center. They eventaully become fibrotic.

Electron micrograph of spirochetes; similar spirochetes cause leptospirosis.

Electron micrograph of spirochetes; similar spirochetes cause leptospirosis.

Tertiary syphilis occurs either as gummas, neurosyphilis or cardiovascular syphilis with aortitis being the most serious feature. There are two main tests for tertiary syphilis: nontreponemal and treponemal. The non treponemal are the VDRL and RPR. These vary can be used to assess treatment status since they decrease with treatment. They are also prone to false positive results and can be positive in measles, lymphoma, tuberculosis and even pregnancy. For this reason the treponemal tests are also done. The FTA-Abs(fluorescent treponemal antibody absorption test) is the most sensitive test for detection of syphilis but is always positive after infection and cannot be used to follow the course of treatment.

There are several non-venereal types of treponema pallidum including yaws shown above.

There are several non-venereal types of treponema pallidum including yaws shown above.

The good news is that treponema pallidum pertenue and nonvenereal endemic syphilis are easily treated with penicillin. If untreated yaws can cause bony destruction and disfigurement.

Kent ME, Romanelli F. Reexaming syphilis: an update on epidemiology, clinical manifestations and management. 2008. Anneals of Pharmacology 42(2):226-36.

Eccleston K, Collins L, Higgins SP, Primary syphilis: the classical triad of skin rash, mucosal ulceration and lymphadenopathy. 2010. Int J of STD &AIDS 21(8) 537-45.

Bhatti MT. Optic neuropathy from viruses and spirochetes. 2007. Int Ophthalmol Clin 47(4) 37-66.

A 67 y.o. male arrests in front of EMS and his EKG is shown below

Would you call the cath lab?

lane first ekg.JPG

Our patient had hyperkalemia.  His second EKG is shown below. 

ekg2.JPG

The pt had a hx of seizures and was found in an empty bath tub.  He initially had agonal respirations and a weak pulse but arrested in front of EMS.   K was 7.9 and lactate was 9.7  He was found to have a SDH on CT. The cath lab was called but felt the changes were due to electrolyte abnormalities and did not cath the pt.

Although this case was hyperkalemia AV blocks do exist with MIs.

High degree AV block occurs in 9.8% with inferior MI and 3.2% with anterior MI in large studies.  High degree AV block associated with inferior MIs is located proximal to the bundle of His and the QRS is usually narrow.  High degree AV associated with anterior MI is usually distal to the AV node and is associated with high mortality and a widened QRS.

Inferior MI  can be associated with sinus brady, Mobitz I, and complete heart block since the SA node, AV node and his bundle are most often supplied by the RCA. Anterior MI is associated most often with a Mobitz II block and can cause complete heart block.

Pacing is recommended for symptomatic complete heart block, alternating right and left bundle branch block, symptomatic bradycardia and Mobitz I second-degree AV block.

 

blood supply.JPG

Meine TJ, Al-Khatib SM, Alexander JH, et al. Incidence, predictors and outcomes of high-degree atrioventricular block complicating acute myocardial infarction treated with thrombolytic therapy. Am Heart J 2005;149;670.

Hunt D, Sloman G, Bundle-branch block in acute myocardial infarction. 1969. Brit. Med J. 1(5636):85-8.

Sgarbossa EB, Pinski SL, Barbageleta A, et al. Electrocardiographic diagnosis of evolving acute myocardial infarction in the presence of left bundle branch block. GUSTO-1

N Engl J Med 1996;334:481-7.

Wilner B, deLemos, J, Neeland I. LBBB in patients with suspected MI: an evolving paradigm. 2017. American College of Cardiology.

 

A 22 y.o. woman presents with vomiting and weight loss

What does her upper GI show?

the arrows point at the problem

the arrows point at the problem

Our patient had a duodenal obstruction from superior mesenteric artery syndrome. She had compression of the third portion of the duodenum between the superior mesenteric artery and the aorta. The syndrome was first described in 1861 by Von Rokitansky.  The incidence is reported to be 0.1-.3%
It can occur after scoliosis surgery, anorexia with weight loss, rapid growth in adolescence,  or trauma.

an angle of 20 degrees between the sma and aorta is considered likely to cause sma syndrome

an angle of 20 degrees between the sma and aorta is considered likely to cause sma syndrome

The greatest barrier to making the diagnosis is not to think of it.  Delay in diagnosis can result in malnutrition, electrolyte abnormalities, a duodenal bezoar or even gastric perforation. The symptoms are typically relieved when the patient is in the L lateral decubitus, prone or knee to chest position.

surgical correction of sma syndrome with a duodenojejunostomy

surgical correction of sma syndrome with a duodenojejunostomy

Surgical correction is recommended if conservative measures fail. The standard procedure is a duodenojejunostomy  where an anastomosis is created between the duodenum and jejunum anterior to the superior mesenteric artery.

sma syndrome can be diagnosed on Ct as well. the large white arrow is the dilated duodenum, the small black area is the compressed distal duodenum and the two small black arrows show the closeness of the sma and aorta. with the duodenum compressed between.

sma syndrome can be diagnosed on Ct as well. the large white arrow is the dilated duodenum, the small black area is the compressed distal duodenum and the two small black arrows show the closeness of the sma and aorta. with the duodenum compressed between.

Our patient underwent a duodenojejunostomy which was complicated by a wound infection and contained duodenal leak. She recovered and is now able to eat whatever she wants without vomiting.

Gerasimidis T, George F. superior Mesenteric Artery Syndrom.  Dig Surg 2009. May 20. 26(3):213-14.

Saraya T, Kurai D, Ariga M, Nakamoto K, Koide T, Tamura M. Superior mesenteric artery syndrome caused by huge mycotic abdominal aortic aneursym. Intern Med. 2009. 48(12):1065-8.

Pottorf BJ, Husain FA, Hollis HW Jr, Lin E. Laparoscopic management of duodenal obstruction resulting from superior mesenteric artery syndrome.  JAMA Surg. 2014 Dec. 149(12):1319-22.

Rokitansky C. Handbuch der Pathologischen Anotomie. Vienna: Branmiller and Siedel. 1842.

 

A 36 y.o. woman presents with shortness of breath. What is the most likely cause?

what causes cannonball lesions of the lung?

what causes cannonball lesions of the lung?

Our patient had metastatic melanoma in the lungs. The differential of “cannon ball” lesions in the lungs includes metastatic disease, infection, vasculitis, autoimmune disorders  and sarcoid.  

METASTATIC DISEASE

The most common malignancies associated with cannon ball lesions are: renal cell ca, choriocarcinoma, endometrial carcinoma, prostate cancer, colon cancer,  and synovial sarcoma. While these are the most common; any cancer can produce lung nodules.

renal cell ca

renal cell ca

INFECTIOUS DISEASE

While tuberculosis classically presents in the upper lobes, in immunosuppressed patients including diabetics it can present as nodules.  Histoplasmosis, cryptococcosis , nocardia , and echinococcus can all present as nodules.

pulmonary tbc in a diabetic pt

pulmonary tbc in a diabetic pt

VASCULITIS/AUTOIMMUNE/SARCOID

Wegener’s, is often diagnosed by c-ANCA . These are antibodies to proteinase 3 and myeloperoxidase which occur in 90% of patients with Wegener’s. Necrotic nodules can be found in the lungs. Sarcoid and rheumatoid nodules can also appear as lung nodules.

rheumatoid nodules are composed of B lymphocytes and often occur after methotrexate is started.

rheumatoid nodules are composed of B lymphocytes and often occur after methotrexate is started.

Kshatriya R, Patel V, Chaudhari S, Patel P, et al.  Cannon ball appearance on radiology in a middle-aged diabetic female.  2016. Lung India. Sep-Oct 33(5):562-568.

Wheat LJ, Kaufmann C. Histoplasmosis. Infect Dis Clin N Am 2003;17:1-19.

Vohra P, Sharma M, Yadav A, Chaudhary U. Nocardiosis: A review of clinic-microbiological features, Int J Life Sci Biotechnol Pharma Res. 2013;2:20-29.

A 27 y.o. with a hx of sinusitis for two months presents with a seizure

What do you see?

IGG4sinusitis.JPG
and what is going on the in the L frontal lobe?

and what is going on the in the L frontal lobe?

Our patient had IgG4-related disease.   This is not a brain abscess but rather a systemic inflammatory condition where tissue is infiltrated by plasma cells that secrete IgG4.  IgG4 related disease can involve multiple sites in the body. It can be found in the heart, lung, GI tract, prostate and brain. There can be considerable organ destruction related to the disease.

IgG4 in the prostate with a dense lymphocytic infiltrate

IgG4 in the prostate with a dense lymphocytic infiltrate

The pathologic process was first described in 1977 when histologic research was done on 349 cases of Kuttner’s tumor( now known as IgG4-related sialadenitis).  Diffuse infiltration of lymphocytes was noted with plasma cell infiltration and eventual fibrosis. 

IgG4 related disease in the parotid on the R, Mikulicz disease

IgG4 related disease in the parotid on the R, Mikulicz disease

 Several diseases that have been thought to be unrelated are now linked by the fact that they contain IgG4 secreting plasma cells.  Some of these diseases include:  fibrosing mediastinitis, interstitial nephritis, autoimmune pancreatitis, retroperitoneal fibrosis, sensorineural hearing loss,  and inflammatory pseudotumors.

 

Our patient received rituximab.  Research is underway to evaluate plamablast-directed therapy with a monoclonal antibody(XmAb5871) which inhibits B-cell function.

 

Perugino C, Stone J. Treatment of igG4-related disease: Current and future approaches.2016.  Zeitschrift fur Rheumatologies. 75(7):681-686. PMID.27431746(https://www.ncbi.nlm.nih.gov/pubmed/27431746)

Seifert G, Donath K. Zur Pathogenese des Kuttner-Tumors der Submandibularis-Analyse von 4347 Fallen mit chronischer Sialadenitis der Submandibularis  ) on the pathogenesis of the Kuttner tumor of the submandibular gland-analysis of 349 cases with chronic sialadenitis of the submandibular . 24(3):81-92.  PMID 856776( https://www.ncbi.nlm.nih.gov/pubmed /856776

 

Wallace Z, Deshpande V, et al.  IgG4-related disease: Clinical and Laboratory Features in 125 Patients. 2015. Arthritis and Rheumatology 67(9): 2466-75. PMID 25988916(https://www.ncbi.nlm.nih.gov/pubmed/25988916)