A 61 y.o. sp treatment for epidural abscess with vancomycin for one month, presents with a rash.

What could be wrong?

Absolute eosinophil count was 3.5 (high), alt 78

Absolute eosinophil count was 3.5 (high), alt 78

hickey 3.JPG

Our patient had DRESS syndrome (Drug Rash with eosinophilia and Systemic Symptoms)

Cutaneous reactions to drugs are considered severe in only 2% of hospitalized patients. The reactions include: AGEP( acute generalized exanthomatous pustulosis),  DRESS, Stevens Johnson syndrome, and Toxic epidermal necrolysis. (TEN).

The cause for our patient was vancomycin

The cause for our patient was vancomycin

AGEP- Is the onset of small, sterile pustules  48-72 hrs after introduction of a drug.  Fever is uncommon.

DRESS- often presents with liver involvement and eosinophilia of over 10% anywhere from 2-8 weeks after exposure to the offending agent.  It usually presents with itching, fever and facial edema. All DRESS patients have liver involvement in several studies and 18% have kidney involvement. The maculopapular rash is not accompanied by epidermal detachment.

STEVENS JOHNSON SYNDROME-  In SJS there are often mouth lesions and skin necrosis affecting less than 10% of the body surface area. There is detachment of the surface of the skin.

There are genetic associations between human leukocyte antigen (HLA) and drug hypersensitivity. HLA B 1502 is associated with tegretol induced Stevens-Johnson syndrome and is found in a higher proportion of people of Chinese descent.

HLA-B 1508 is associated with allopurinol induced Stevens Johnson syndrome. Stevens Johnson syndrome often involves the oral mucosa.

TEN-Toxic epidermal necrolysis is  essentially a Stevens-Johnson syndrome involving more than 30% of the body surface area.  It was first described by Debre  in 1939 where it was known in French as l’erhthrodermie bulleuses avec epidermolyse.

 

necrotic epidermis seen in stevens-johnson syndrome

necrotic epidermis seen in stevens-johnson syndrome

In trying to differentiate between DRESS and SJS, the skin lesions are considered first since both conditions can have elevated eosinophils, fever, rash and elevated liver enzymes. The diagnosis of SJS is differentiated by the detachment of the surface of the skin which puts the patient at risk for sepsis. Our patient had a biopsy which did not show epidermal necrosis ( although he did have mouth lesions) so he was diagnosed as DRESS and treated with steroids. Vancomycin was the offending agent.

our patient did have mouth lesions which usually is found in SJS

our patient did have mouth lesions which usually is found in SJS

Choudhary S, McLeod M, Torchia D, Romanella P.  Drug reaction with Eosinophilia and Systemic Symptoms. 2013 J Clin Aesthet Dermatol. Jun;6(6) PMID 23882307

Hung S, Chung W, Jee S. et al. Genetic susceptibility fo carbamazepine-induced cutaneous adverse drug reactions. Parmacogenet Genomics. 2006;16:297-306.

Jeung Y, Lee J, Oh M, Choi D, Lee B. Comparison of the casues and clinical features of drug rash with eosinophilia andsystemic symptoms and Stevens-Johnson syndrome.  Allergy Asthma Immunol Res. 2010 Apr;2(2):123-6.

Casagranda A, SuppaM, Dehavay F, Mamol V. Overlapping DRESS and Stevens-Johnson Syndrome: Case Report and Review of the Literature. Cas Rep Dermatol. 2017 May-aug: 9(2):1-7.

A 40 y.o. presents with a fever and a lesion on her arm.

what is wrong?

brachial art.JPG

Our patient had a brachial artery pseudoaneurysm.  The astute ED physician recognized it immediately and did not attempt an I+D.  this condition was first described in the mid 1800’s. A right brachial artery interposition bypass with vein bypass was done with closure of the R antecubital fossa wound with an adjacent tissue transfer. 6-acetylmorphine rose to 15 on hospital admission presumably from IV drug use. She signed out AMA but continued to take her antibiotics. .  Cultures of the pseudoaneursym grew MRSA and blood cultures grew Pseudomonas. With an estimated 16 million IV drug users world wide, this problem will become more prevalent. 

femoral artery pseudoaneursym

femoral artery pseudoaneursym

Infected femoral  artery pseudoaneursyms (mycotic) are the most common arterial complications in IV drug abusers in a study from India. In this study by Saini, 40% were Staph aureus, 20% pseudomonas,  15% E coli. With the rest being Strep, and Acinetobacter. The current approaches are: ligation of the artery with local debridement, concomitant revascularization and delayed revascularization. Delayed bypasses involve a temporary bypass graft or a vein graft. Amputation was a complication of treatment in 4 of 72 patients.

Pseudosneursym of the R subclavian

Pseudosneursym of the R subclavian

pseudoaneursym of the R subclavian

pseudoaneursym of the R subclavian

Nearly one-third of patients in one survey of injection drug users admitted to licking their needles prior to injection. Int J Drug Policy 2008;19(4):342.  This is done to test the quality of the drug, but exposes IV drug users to multiple pathogens. A second source of infection is using bottled water which is not sterile and often contains gram negative organisms. Counseling on these two risks may mitigate infections.

 

 

Saini N, Luther A, Mahajan A, Joseph A. Infected pseudoaneursyms in intravenous drug abusers: ligation or reconstruction? 2014.  Int J Appl Basic Med Res. Sep;4(Suppl1):S23-S26.

Lenartoya M, Tak T. Iatogenic pseudoaneursym of the femoral artery: case report and literature review.  Clin Med Res 2003Jul;1(3):243-247.

Tufnell J. On the influence of vegetations on the valves of the heart, in the production of secondary arterial disease. Q J Med Sci (Dublin). 1853;15(2):371-382.

A 22 y.o. returns from Africa with a fever of 103 and a rash. She has a history of autoimmune hepatitis.

What could she have?

A diffuse rash is seen

A diffuse rash is seen

Our patient remained febrile for seven days.  She initially was treated with ceftriaxone by her PMD and then developed diarrhea.  She was seen in ID clinic and admitted. She was pancytopenic and had mild elevations in liver functions.  AST was 78.  In the hospital she was treated with ceftriaxone and typhoid was the presumed diagnosis. Two days later doxy was added for African tick bite fever.  Her immuran was stopped. The differential for infectious diseases acquired in South Africa is broad.

fever and africa1.JPG
fever and africa 2.JPG

African tick bite fever was first described in 1931 by Troup and Pijper.   It is transmitted by dog ticks in urban areas and cattle or game in rural areas. The incidence of infection in European visitors is 5% with visitors to game reserves at high risk of infection

Chikungunya

Chikungunya

Typhoid fever is most often contracted from exposure to water contaminated with sewage. Since one in twenty  people infected become chronic carriers, it is difficult to eradicate.

Arbovirus diseases are also common in South Africa.  Mosquitoes are the vector for Rift Valley fever, west Nile, dengue  Chikungunya. Ticks are the vector for Crimean-Congo hemorrhagic fever.  This is the most important hemorrhagic fever virus in South Africa.   Retinitis, meningitis and skin bleeds can be seen within two days of contracting the virus. 

typhoid rose spots

typhoid rose spots

When testing for arboviruses, the antibody-based assays may initially test negative as the immune system needs time to mount an immune response. In the case of our pt she had immune suppression from her immuran She was negative for malaria. Tbc, HIV,dengue, chikungunya and strongyloides tests pending at the time of discharge.

She was treated with doxy and Cipro for presumed typhoid fever and African tick bite fever.  She remained febrile although her fever was only 38 on discharge.

Delfos N, Schippers E, Rauoult D, Visser L. Fever and vesicular rash in a traveler returning from South Africa. Oxford Academia South African Fam Pract 2008 Vol 50(2) 33-35.

Prinsloo B. Arboviral diseases in Southern Africa. SA Fam Pract 2006: 48(8). 25-28

A 56 y.o. male presents with paraplegia

His MRI is shown below

what is wrong?

what is wrong?

Our patient was paraplegic because of spinal epidural lipomatosis. There is proliferation of fatty tissue in the spinal canal which compresses the cord. The first case was reported in 1975.  Many of the cases are due to long term use of steroids but it has also been associated with Cushings, hypothyroidism, prolactinoma and obesity. It has even been reported after epidural spinal injections.

lipomatosis grade II.JPG

Back pain is the initial presenting symptom. This is followed by lower extremity weakness and only rarely is incontinence reported. Since there is no fat in the cervical spine; symptoms are most often in the lower extremities.

 

Treatment can range from weight loss to surgical debulking of fat in the canal. Our patient was recognized late and was already paraplegic so no intervention was performed.

Rustom D, Gupta D, Chakrabortty S. Epidural lipomatosis: a dilemma in interventional pain management for the use of epidural steroids. J Anaesthesiol Clin Pharmacol. 2013;29:410-411

Fassett, D, Schmidt M.Spinal epidural lipomatosis: a review of its causes and recommendations for treatment. Neurosug Focus 16(4): Article 11, 2004. 1-3.

a 48 y.o. male loses control of his bicycle and skids off a slick road into a ditch. He has an obvious partial avulsion of the R ear.

because of ED overcrowding he waits hours and is never placed in a room. The triage MD orders a head and Cspine CT and a plastics consult. Plastics repairs his ear. While waiting for his ride he has an episode of syncope and is placed in a room. The head CT done previously is shown below.

do you agree with the radiologists read?

do you agree with the radiologists read?

The pt actually had a subdural along the falx

The pt actually had a subdural along the falx

There was an overread the next day on the head CT confirming the posterior subdural along the L falx.  The pt was admitted to ICU. In addition, although the CT had been read as normal the pt continued to have severe neck pain and MRI confirmed all the interspinous ligaments on the L side from C2-C5 were torn.  He was admitted to neurosurgery for both injuries and was instructed to were a cervical collar for although the anterior and posterior spinous ligaments were intact, he was unstable in lateral movement of the neck.

On T2 MRI the injury appears white over tthe posterior cervical spines

On T2 MRI the injury appears white over tthe posterior cervical spines

Cervical spine injuries occur in 3% of major trauma patients.  Morbidity and mortality from cervical injuries are high.  The litigation pay out for missed cervical spine injury on average in the US is $2.9 million. If the injury is missed on the initial presentation, the pt is ten times more likely to have neurologic sequelae.

an 84 y.o. fell out of his wheelchair and had neck pain with a negative CT of the C spine

an 84 y.o. fell out of his wheelchair and had neck pain with a negative CT of the C spine

He returned with persistent pain and an odontoid fracture was seen on MRI requiring treatment.

He returned with persistent pain and an odontoid fracture was seen on MRI requiring treatment.

In a prospective study of 767 patients who had MRI because of persistent neck pain after a negative neck CT, the MRI was abnormal in 23.6% of patients  with injuries ranging from ligamentous injuries(16%), soft tissue swelling 4%, Disc injury 1.4%,  and dural hematomas in 1.3%.  11 patients underwent cervical spine surgery after the MRI results and five of those had no neurologic findings. Another prospective study of 10,765 patients found 2% of patients with neg CT and persistent neck pain had significant neck injuries requiring intervention. The teaching point is that a neg neck CT is not able to completely rule out significant neck injury.

Most of the patients with missed injuries on CT had degenerative spine disease: our patient had a large posterior osteophyte which pushed the clinicians to order an MRI. He was discharged with a collar and neurosurgical follow up.

 

 

Maung A, Johnson D, Barre K, et al. Cervical spine MRI in patients with negative CT: A prospective, multicenter study of the Research Consortium of New England Centers for Trauma (ReCONECT). 2017 J Trauma Acute Care Surg. Feb 82(2):263-269.

Inaba K, Byerly S, Buysh L, et al. Cervical spinal clearance: A prospective Western Trauma Association Multi-Institutional Trial. 2016 J Trauma Acute Care Surg, Dec:81(6):1122-1130.

Resnick S, et al. Clinical relevance of Magnetic resonance imaging in cervical spine clearance. 2014  JAMA Surgery , vol 149 (9) jan: p 934, doiP10.1001/jamasurg.2014.867.

Diaz J, et al. The early work-up for isolated ligamentous injury of the cervical spine: does CT-scan have a role?2004  The Journal of Trauma: Injury, Infection and Critical Care, vol 57(2):453.

 

A 58 y.o. presents with an expanding abdominal wall hematoma six days after having an "abscess" aspirated

needle aspiration of an “abscess” after appendectomy; only blood returns

needle aspiration of an “abscess” after appendectomy; only blood returns

she returns six days later with an expanding hematoma; What happened?

she returns six days later with an expanding hematoma; What happened?

Our patient developed a pseudoaneursym which is  a collection of blood between the two outer layers of an artery, the tunica media and the tunica adventitia.  The damage probably happened when the interventionalist tried to aspirate what they thought was an abscess. 

aneursym.JPG

A pseudoaneursym is a common complication after a cardiac cath and occurs in 0.8 to 8% of vascular interventional procedures.

Pseudoaneursyms can occur in association with inflammation. In pancreatitis they occur in the superior mesenteric, and   pancreaticoduodenal artery.  In the heart pseudoaneursyms most often occur in the lateral wall and true aneurysms occur in the apex.

pseudoheart.JPG

Doppler flow mapping establishes the diagnosis. Diagnostic criteria include: swirling color flow in a mass separate from the main artery and a typical to and fro pattern in the pseudoaeursym neck. Ultrasound compression has been useful in occluding the pseudoaneursym neck allowing the aneurysm neck to thrombose.  Stents, percutaneous thrombin, percutaneous fibrin adhesive, coiling and surgery are also used to repair pseudoaneursyms.

In our patient, the superficial circumflex iliac artery was feeding the pseudoaneursym and was embolized.

The following is a question from USMLE

a. septal rupture  b. left ventricular free wall rupture  c. right ventricular free wall rupture  d. mitral regurgitation  e. left ventricular aneursym

a. septal rupture

b. left ventricular free wall rupture

c. right ventricular free wall rupture

d. mitral regurgitation

e. left ventricular aneursym

The answer is E. A thin fibrous scar is seen in the myocardial tissue. The patient presented with CHF which is the most common cause of death in patients with ventricular aneursyms. CHF is caused by poor contractility of the scarred myocardium and dyskinesis (paradoxical ballooning of the aneurysm with contraction). A pseudoaneursym( where there is no endocardium in the aneurysm) is more likely to rupture since only fragile scar tissue prevents cardiac wall rupture.

 

Lenartoya, M, Tak T. Iatrogenic pseudoaneursym of femoral artery: case report and literature review. Clin Med Res 2003 Jul;1(3):243-247.

Hamraoui K, Ernst S, et al. Efficacy and safety of percutaneous treatment of iatrogenic femoral artery pseudoaneursym by biodegradable collagen injection. J Am Coll Cardiol. 2002;39:1297-1304.

Messina L, Brothers T, Wakefield T, Zelenock G, et al. Clinical characteristics and surgical management of vascular complications in patients undergoing cardiac catheterization: interventional versus diagnostic procedures. J Vasc Surg. 1991;13:593-600.

A 30 y.o. male with no medical history presents for memory loss. He reports his short term memory has been gone for two weeks.

What could be wrong?

Increased T2 signal is seen in subcortical areas of the frontal lobes on MRI

Increased T2 signal is seen in subcortical areas of the frontal lobes on MRI

Our patient had encephalitis. This inflammation of the brain or meninges has many causes including viral and autoimmune. Viral causes include mosquito-borne illness as well as tick borne. RMSF is comon in Missouri but that was not the cause of our patients disease.

Rocky Mountain spotted fever

Rocky Mountain spotted fever

VIRAL ENCEPHALITIS

Varicella-zoster, Epstein-Barr virus,  CMV, measles, mumps.  Can present with  rash, lyphadenopathy, hepatosplenomegaly or parotid enlargement.

St. Louis encephalitis( mosquito borne) can present with movement disorders, dysuria and pyuria.

West Nile encephalitis( mosquito borne) can present with extreme lethargy or flaccid paralysis.

Tick borne encephalitis is an important infectious disease in Europe, the former Soviet Union and Asia. Powassan virus causes tick borne encephalitis in the US.

Rabies, Zika,  HIV, and enteroviruses also can cause encephalitis.

Any viral encephalitis can present with behavioral changes, SIADH, memory disturbance or seizures

Powassan encephalitis is also tick borne and not the cause of our patients disease..

Powassan encephalitis is also tick borne and not the cause of our patients disease..

AUTOIMMUNE ENCEPHALITIS

NMDA receptor encephalitis-40% are associated with ovarian teratomas

Leucine-rich glioma inactivated 1 and contactin-associated protein 2.

Anti-aquaporin-4 is associated with neuromyelitis optica.

HIV.

 

Different parts of the brain can be affected by encephalitis.  Limbic system involvement may result in psychosis.  Brainstem involvement may result in cranial nerve findings. Neuromyelitis optica may result in blindness. Our patient had primarily frontal lobe involvement with his encephalitis and therefore executive function; calculation and memory were affected.

Our patient had encephalitis caused by HIV.  He had a CD4 count of 90 and was started on bicegravir-emtricitabine-tenofovir and Bactrim. His JC virus in csf was negative .  The current diagnosis is HIV encephalopathy since infectious studies were negative.  

Remember West Nile as a possible cause of encephalitis

Remember West Nile as a possible cause of encephalitis

Davidson M, Williams H, Macleod J.  Louping ill in man: a forgotten disease. Journal of Infection 1991;23(30:241-49.

Ebel G. Update on Powassan virus: emergency of North American  tick-borne flavivirus . Annual Review of Entomology.  2010;55:95-110.

DubeyD, Pittock S, Kelly C, et al. Autoimmune encephalitis epidemiology and a comparison to infectious encephalitis. Ann Neurol. 2018;83(1):166-177.

 

A 39 y.o. woman persents with peritonitis, she has a history of a peritoneal inclusion cyst which has been drained by IR.

Her cyst is shown from 3/19 and her CT from 6.5 prior to the IR drain placement

cyst seen on US 3/19

cyst seen on US 3/19

CT prior to cyst drainage.

CT prior to cyst drainage.

The cyst was drained on 6/5 and alcohol was instilled. They were unable to replace a drained after several tries because the cyst had collapsed. When she returned with peritonitis the cyst was no longer visible on CT , there was no free air and no perforation noted with oral contrast on CT. She was taken to the OR by general surgery . What did they find?

Our patient was found to have a 10 cm tubovarian abscess on the L. It was not diagnosed on CT scan possibly because of abnormalities from the collapsed inclusion cyst and possibly because CT is poor for defining pelvic structures. It is not clear if the cyst drainage caused inadvertent puncture of bowel or the cyst causing peritonitis.

peritoneal inclusion cysts caused by adhesions to the ovary.

peritoneal inclusion cysts caused by adhesions to the ovary.

A peritoneal inclusion cyst is usually caused by accumulation of ovarian fluid that is trapped by adhesions to the ovary caused by previous inflammation. Previous inflammation thickens the peritoneum and makes it more difficult to absorb fluid.  Because the source of the fluid is ovarian, cysts are rare in males but have been reported in Crohn’s disease where there is chronic inflammation.

Conservative treatment of a peritoneal inclusion cyst is recommended because after surgical resection 30-50% recur. Oral contraceptives can be used to suppress ovulation, thus decreasing the formation of ovarian fluid trapped by adhesion and aspiration of the cysts can often be done transvaginally..

Our patient underwent a a L salpingo-oophorectomy and received doxy and flagyl .  Her pain improved and she was discharged.

Jain, K.  Imaging of peritoneal inclusion cysts. American Journal of Roentgenology. 2000 June Vol 174(6).

Ross M, Welch W, Scully R. Multilocular peritoneal inclusion cysts . Cancer 1989;64:1336-1364.

Sohaey R, Gardner T, Woodward P, Peterson C. Sonographic diagnosis of peritoneal inclusion cyst. J Ultrasound Med 1995;14:913-17.

Spriggs D, Melamed A, Weier L, Safdar N. A 24 y.o. woman with a pelvic mass. Case 18-2019. NEJM 2019

380:2361-9.

THE DIFFERENTIAL OF A PELVIC MASS INCLUDES:

Pregnancy and physiologic masses-endometriomas, cysts

Infections-diverticulitis, appendicitis, tuberculosis

Benign tumors-cystadenomas of the ovary or ovarian epithelial tumors

Cancer-familial tumors (BRCA or Lynch syndrome), high grade serous tumors and endometroid tumors(associated with CA-124), germ cell tumors.

A 51 y.o. presents for a swelling in the buttock

AVM presenting as a mass in the buttock

What is it?

What is it?

Our patient had an AVM of the pelvis which eroded through an ulcer in the skin. The AVM was from multiple feedings arteries from the profunda femoris and the internal iliac draining into the L common femoral vein. In the ED she initially stopped bleeding and IR was scheduled to embolize the next day but then she began to have a massive arterial hemorrhage and developed hemorrhagic shock in the ED. She was taken to IR after the massive tranfusion protocol was initiated and the BP remained 70 in spite of blood and pressors. Only 20 per cent of the AVM could be embolized but the bleeding was stopped by embolization and suturing the skin closed. She developed ATN with a creatinine was 6.0. Further definitive treatment was postponed until her kidneys recover. A REBOA was considered but general surgery felt the bleeding could be managed better with direct pressure.

AVMs are abnormal connections between arteries and veins causing high flow in the venous limb. They can occur in the brain, neck, spine, arms, legs and liver. The incidence of AVM is one in 10,000 individuals. If you have an AVM there is a 4% risk of bleeding per year.

Uterine AVM presenting with vaginal bleeding and diagnosed with US. While US is the first modality for diagnosis, MRA is often used as well.

Uterine AVM presenting with vaginal bleeding and diagnosed with US. While US is the first modality for diagnosis, MRA is often used as well.

AVMs can be congenital or acquired with the congenital form having more feeding vessels and being more difficult to embolize. Ischemia of the distal extremity and thrombosis are known complications of AVMs. One of the more common forms of acquired AVMs are uterine AVMs which occur after abortion or childbirth and cause life threatening vaginal bleeding.

Greene A, Orbach D. Management of AV malformations. 2011 Clin Plastic Surg;38(1):95-106.

Belfort M. Postpartum hemorrhage: Medical and minimally invasive management.

Sridhar D, Vogelsang R. Diagnosis and treatment of uterine and pelvic AVM: a review of etiology, clinical presentation and imaging findings, and treatment options, 2018 Endovascular Today, Jan

Yazawa H, Soeda S, Hiraiwa T, el al. Prospective evaluation of the incidence of uterine vascular malformation developing after abortion or delivery. Minimally Invasive Gynecology 2013;20:360-67.

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.