A 28 y.o. male has lesions in the liver; what could cause this?

Hint; he has hilar adenopathy shown below

sarcoid1.JPG
 hilar adenopathy

hilar adenopathy

Our patient had neurosarcoidosis.  The lesions in the liver and lymph nodes were granulomas.  He had presented initially with progressive leg weakness over 3 months and urinary retention.  The pt had cauda equina syndrome with involvement of the sacral nerve roots on MRI as well as diffuse leptomeningeal enhancement of the entire spine. 5-10% of patients with sarcoid develop neurosarcoidosis.

 

 involvement of sacral roots on MRI

involvement of sacral roots on MRI

Sarcoidosis is a poorly understood disease thought to be an immune response to something; possibly environmental, possibly infectious, possibly genetic, and possibly related to other immune diseases.  It is more common in celiac disease and common variable immune deficiency. The disease has a prevalence of 40/100,000 in the general population but seems to be dependent on race.  It is more common in Sweden and Iceland where the prevalence is 60 per 100,000. The disease is also more common among those of African American descent.  In Japanese patients ophthalmologic and cardiac involvement are more likely.

The presentation is widely variable.  It can present as cranial nerve palsies with vestibulocochlear nerve involvement causing  deafness or vertigo, optic nerve involvement causing  loss of vision, glossopharyngeal nerve involvement causing difficulty swallowing or facial nerve involvement. The granulomas can form in the pituitary and cortex of the brain.  10% of patients presenting with sarcoid present with seizures due to brain involvement.

It can present as mediastinal adenopathy with no other involvement. It can also present as e nodosum which is an inflammation of fat cells causing raised nodules on the shins.

  e nodosum

 e nodosum

Our patient had a biopsy of a lymph node confirming sarcoid and was treated with high dose steroids. His symptoms greatly improved and he was discharged. Long term treatment is anticipated.

 

Joseph FG, Scolding  NJ. Sarcoidosis of the nervous system. 2007 Practical Neurology 7(4) 234-44.

Li Y, Pabst S, Kubisch C, Grohe, Wollnik B. First independent replications study confirms the strong genetic association of ANXA11 with sarcoidosis. Thorx 2010. 65(10) 939-40.

Syed J, Myers R. sarcoid heart disease. Can J Cardiol 2004. 20 (1) 89-93.

A 24 y.o. presents to the ED with a three week hx of a neck mass and sore throat.

What does he have?

branchial cleft abs.JPG

Our patient had an infected branchial cleft cyst involving the L lobe of the thyroid; so probably the 4th branchial cleft. Although  branchial  cleft abnormalities are congenital abnormalities, they present in early adulthood with fistulas and cysts. Phylogenetically, the branchial apparatus is related to gill slits.  In fish and amphibians  these slits do not fuse and become gills.  The name branchial comes from branchia which is Greek for gills.

 common sites for branchial cleft cysts

common sites for branchial cleft cysts

The second branchial cleft accounts for 95% of branchial cleft abnormalities. They are most frequently found on the anterior border of the upper third of the sternocleidomastoid.  The cysts can become infected after a viral infection  because they contain lymphoid tissue beneath their epithelium.  Any patient who presents with an abscess of the thyroid probably has an underlying branchial cleft fistula communicating with the pyriform sinus. 

 Other branchial cleft sites

Other branchial cleft sites

Our patient underwent drainage of his abscess which grew strep constellatus.  Since 20% recur if infection is present he is being followed by ENT.

 MRI of our patients neck

MRI of our patients neck

Doi O, Hutson JM, Myers NA, McKelvie PA. Branchial remnants: a review of 58 cases. J Pediatric Surg. 1988 Sep23(9):789-92.

Goff CJ, Allred C, Glade  RS. Current mansgement of congenital branchial cleft cysts, sinuses and fistulae. Curr Opin Otolaryngol Head and Neck Surg 2012. Dec 20(6) 533-9.

Valentino M, Quiligotti  C, Carone L,Branchial cleft cysts. J Ultrasound 2013  16(1) 17-20.

Benson MR, Dalen K, Mancuso A et al.  congenital anomalies of the branchial apparatus; embryology and pathologic anatomy. Radiographics 1992 Sept 12(5) 943-60.

A 23 y.o, presents to the ED with carpopedal spasms, you notice something unusual on exam of his skin.

What condition does he have?

melanosis.JPG

Our patient had neurocutaneous melanosis.  This is a rare disease characterized by pigmented nevi that affect not only the skin but the leptomeninges.  It was first described in 1861 by Rokitanski.  About 1/3 of the patients diagnosed are symptomatic often presenting with seizures, cranial nerve palsies, hydrocephalus or spinal cord involvement.  It is associated with Dandy-Walker malformation  in 10% of the cases. Our patient had presented with seizures as a child and on imaging was found to have melanosis in the R temporal lobe.  Because of intractable seizures he underwent a R temporal lobectomy in 2015.

 melanosis in the R temporal lobe in our patient prior to surgery

melanosis in the R temporal lobe in our patient prior to surgery

The diagnosis is made on MRI with the melanocytes being hyperintense on T1 images. The deposits of melanocytes are most often seen in the amygdala, cerebellum and upper cervical cord. The pathogenesis is believed to be dysplasia of the neuroectodermal melanocyte precursor cells leading to proliferation of melanin in the skin and leptomeninges.  Patients with neurocutaneous  melanosis are at risk for malignant transformation to melanoma.  Malignant transformation occurs in 40-60% of symptomatic cases.

 carpopedal spasm

carpopedal spasm

Carpopedal spasm occurs when acute hypocarbia causes reduced ionized calcium and phosphate levels resulting in involuntary contraction of the feet and hands. This can occur with dehydration, hyperventilation (from any cause including MI, infection or bleeding), hypothyroidism, tetanus,  and brain disorders (Parkinsons, MS, dystonia and huntingtons).  

 

Our patient had normal Mg, Ca and phosphorus. His CO2 was 20.  The cause for his carpopedal spasm was thought to be dehydration from a night of drinking preceding his presentation.  He had an elevated ddimer and underwent a PE protocol CT which showed no PE but a small R to L shunt.  His spasms resolved with hydration. 

 

Gocmen R, Guler E, Arslan E. A case of neurocutaneous melanosisand neuroimaging findings. 2015 Journal of Radiology Case Reports. 9(3) 1-6.

Rokitanski J, Ein ausgezeichneter Fall von Pigment-Mal mit ausgebreiteter Pigmentierung der inneren Hirn-und Ru chenmarkhaute. Ally Wien Med Z 1861(6):113-16.

Ginat DT, Meyers SP.  Intracranial lesions with high signal intensity of T1-weighted MR images: differential diagnosis. Radiographics 2012 32(2) :499-516.

A 41 y.o. male presents with anemia and shortness of breath. His peripheral smear appears below.

 What is the differential?

What is the differential?

Our patient had atypical hemolytic uremic syndrome. This was called atypical because in most cases the syndrome is preceded by an episode of diarrhea which is caused by E Col, O157:H7,  other non -O157 E. coli, shigella and campylobacter.  It can also be preceded by a virus.  Another atypical feature of this case was that HUS most commonly occurs in children where it remains the most common cause of acquired renal failure.  5% of the cases result after infection by Strep pneumo. In atypical HUS, genetic defects cause uncontrolled complement activation.

HUS was first described in 1955. The toxin binds to GB3 receptors which are more common in children and in adults are concentrated in the kidneys.   The virus or bacteria cause endothelial damage, platelet activation , and widespread inflammation leading to  thromboses in small blood vessels. Stroke, MI , renal failure in 55-70%, liver necrosis, pancreatitis, and seizures may result .

rbc.JPG

The mechanism of action includes binding of the toxin to BG3 receptor on the surface of glomerular endothelium, and binding of leukocytes to endothelial cells.  The binding of toxin inactivates  a metalloproteinase call ADAMTS13 and multimers of von Willebrand Factore  form and initiate platelet activation, causing microthrombi.  These thrombi lodge in small vessels and break red blood cells as that try to squeeze through.  Lastly, the toxin activates the alternative complement pathway and interferes with complement factor 5, an inhibitor of complement activation.  In contrast to DIC where coagulation factors are consumed, in HUS fibrinogen and DDimers are normal.

 causes of hemolysis

causes of hemolysis

The country with the highest incidence of HUS is Argentina possibly because of the association between E coli and contaminated meat.  In the 1990’s Jack in the Box restaurants served contaminated meat causing an outbreak of HUS. Outbreaks have also been linked to cookie dough and spinach.

If you see schistocytes you may have a thrombotic microangiopathy.

HUS-  positive shiga-toxin confirms the diagnosis, plasmapheresis is contraindicated, use eculizumab

TTP  ADAMTS13 deficiency <5% of normal; plasmapheresis is indicated.

Our patient was treated with Eculizumab , a monoclonal Ab against CD5( blocks complement)  In one week,  his platelet count increased from 56,ooo to 122,000,  his hgb increased from 7.3 to 8.7 and he began making urine.   One year of treatment costs $500,000.

Benz K, AmannK. Thrombotic microangiopathy:new insights. Current Opinion in Nephrology and Hypertension. 2010 19(3) 242-247.

Shimizu M, Yokoyama T, et al. Thomsen-Friedereich antigen exposure aas a cause of Strep pyogenes-associated hemolytic-uremic syndrome.  Clinical Nephrology 78(4):328-31.

Noris M, Remuzzi G. Atypical hemolytic-uremic syndrome. 2009. NEJM 361(17):1676-1687.

thrombocy.JPG

A 31 y.o. male comes in with bilateral heel pain. His xray is shown below

 what do you notice when compared to the normal image below

what do you notice when compared to the normal image below

kagers normal.JPG

Our patient had inflammation of Kager’s fat pad.  Kager’s fat pad is a lipomatous structure located in the posterior ankle joint anterior to the Achilles tendon. On lateral radiographs of the ankle the fat pad is radiolucent and triangular with boundaries formed by the flexor hallucis longus muscle and tendon anteriorly, the superior cortex of the calcaneus inferiorly and the Achilles tendon posteriorly.

kagers fat pad.JPG

What sorts of conditions cause inflammation of the fat pad?  Fractures, joint effusions, Achilles tendon injury, arthritis  and  autoimmune diseases all can cause the fat pad to be opaque on xray. . The most common autoimmune diseases causing this enthesitis ( inflammation where the tendon inserts on the bone) are ankylosing spondylitis and Reiters syndrome. . Reiter’s syndrome( now called 'reactive arthritis" because Reiter experimented in Nazi concentration camps) consists of conjunctivitis,  non-gonococcal urethritis and arthritis. It affects the heel in 50% of patients and is often caused by chlamydia. For this reason it has been known as “lover’s heel.”  It usually occurs 1-3 weeks after infection  and is most commonly associated with Chlamydia.however it can also occur after diarrheal illness  with Salmonella, Shigella, or Camphylobacter..

Another cause of inflammation is a retrocalcaneal exostosis  called a “pump bump”.  This is more common in women and thought to be due to wearing high heeled shoes.  This is also called Haglund’s deformity.  The cure for this inflammation which leads to the exostosis is to change the height of the heels being worn.

calcaneus haglands..JPG

 

Our patient had conjunctivitis and bilateral heel pain. The formal ultrasound did not show Achilles tendon injury.   He was treated for Reiter’s with NSAIDS and antibiotics for Chlamydia.

Ly J, Bui-Mansfield L. Anatomy of and abnormalities associated with Kager’s fat pad. AJR 2004, 182(1).

Pavlov H, Heneghan MA, Hersh A, Goldman AB, Vigorita V. Haglund’s syndrome: intial and differential diagnosis of posterior heel pain. Radiology 1982;144:83-87

Frey C, Rosenberg Z, Shereff MJ, Kim H. The retrocalcaneal bursa: anatomy and burography. Foot Ankle 1992;13:203-207.

McGahan JP, Graves DS, Palmer PES. Coccidioidal spondylitis : usual and unusual radiographic manifestations. AJR 1980;136:5-9.

A 64 y.o. male comes to the ED with abdominal pain and hypotension.

 you notice some gallstones ....and

you notice some gallstones ....and

 and a discontinuity at the end of the gall bladder.&nbsp; What happened?

and a discontinuity at the end of the gall bladder.  What happened?

Our patient had a rupture of the gallbladder with stones spilling out into a cavity under the liver. The reason the stones were not free in the abdomen was because of adhesions found at surgery.

 perforated gallbladder at surgery( initially done laparascopically and converted to open

perforated gallbladder at surgery( initially done laparascopically and converted to open

 

About 4% of patients with acute cholecystitis have gallbladder perforation.  This is somewhat dependent on time between onset of symptoms and surgery.It is not surprising that there is a delay in diagnosis since  the symptoms of a perforation may be very similar to uncomplicated cholecystitis.  There are several types of gallbladder perforation according to Niemeier’s classification in 1934. In type I there is free gallbladder perforation and bile peritonitis, type II is localized peritonitis with an abscess and type III is chronic gallbladder perforation which results in a cholecystoenteric fistula.   

A perforation can occur as early as two days after the onset of acute cholecystitis or as long a several weeks after.  The usual mechanism of perforation is blockage of the cystic duct with a gallstone causing a rise in intraluminal pressure which impedes venous and lymphatic darainge leading to necrosis. Gallbladder perforation can also occur with acalculous cholecystitis where  there is increased bile viscosity due to fever and dehydration and prolonged absence of oral feeding resulting in a decrease in cholecystokinin-induced gall bladder contraction. Edema of the gallbladder wall can also occur with chf.

The treatment of a gangrenous gallbladder is cholecystectomy  after the infection is relieved by US guided percutaneous drainage. Our patient underwent surgery. 

 look for gall bladder perforation on US

look for gall bladder perforation on US

Derici H, Kara C, Bozdag A,  et al. Diagnosis and treatment of gallbladder perforation, 2006 World J Gastroenterol. 12(48): 7832-7836.

Niemeier OW. Acute free perforation of the gall-bladder. Ann Surg 1934;99:922-924.

thanks to Dr Baumgartner

A 30 y woman, G8P1 comes to the ED with vaginal bleeding. You notice two things in the uterus.

an apparently normal 7 wk gestation

higgins2.JPG
 and you also notice this mass;&nbsp; what is it?

and you also notice this mass;  what is it?

Our patient had a twin pregnancy where one twin was a molar pregnancy.  The term hydatidiform mole comes from the Greek hydatisia(a drop of water) and mola(from the Latin meaning millstone/false conception).  Molar pregnancies occur in 1 in 1000 pregnancies. They are more frequent in Mexico , SE Asia and the Phillipines.  They are more frequent in white women than black women. How a mole develops  is a subject of debate although it is thought to occur when an egg without DNA  is fertilized by a sperm which then creates a fetus with only male DNA patterned methylation and this stimulates syncytiotrophoblast growth.  If the egg DNA is present this rapid growth is normally curtailed.

 molar twin pregnancy at 19 wks.

molar twin pregnancy at 19 wks.

More than 80% of moles are benign and in those cases women are counseled to avoid pregnancy for 6 to 12 months after a D+C. In 10-15% of cases hydatidiform moles may develop into invasive moles. This is called persistent trophoblastic disease and can result in invasion of the uterine wall with hemorrhage.  In 2-3% of cases, moles develop into choreocarcinoma which is an aggressive malignancy resulting in widespread metastatic disease and death.

There are numerous reports of twin pregnancies where one “fetus” is a molar pregnancy.  It is estimated that 20% of these pregnancies will result in a viable term infant. On the other hand, the mole can be aggressive and cases have been presented where the HCG (which normally peaks at 100,000 in the 10th week of pregnancy) can be over 1,000,000 and widespread metastases occur.  HELLP syndrome can develop as well as hyperthyroidism and severe bleeding.

 The ""bunch of grapes" appearance of a molar pregnancy

The ""bunch of grapes" appearance of a molar pregnancy

Our patient elected to undergo a D+C and had no complications.

Renard N, BijvankS, deGroot J, Verheijen R, et al. Aggressive complete hydatidiform mole coexistent with a normal fetus during pregnancy: is there a correlation between outcome and serum HCG levels? A report on 2 cases and review of the literature.  Obstetrics and Gynaecology cases=reviews 2016. 3(5) 089 ISSN : 2377-9004

Jones WB. Lauersen NH Hydatidform mole with coexistent fetus. Am J Obstet Gynecol 1975. 122:267-272.

Woo J, Hsu C, Fung L, Ma H. Partial hydatidiform mole: ultrasonograqphic features. 1983. Aust N Z J Obstet Gynaecol. 23 (2 ):103-7.

A 22 y.o. woman fell from her horse; she has pain over the L shoulder and is sent from an urgent care

What is wrong?

 what do you want to do?

what do you want to do?

Our patient had a pseudodislocation of the shoulder.  This results from an occult fracture that distends the joint due to blood.  It can be recognized by viewing the transcapular or axillary view.  As seen below where the humeral head in in the glenoid.

 humeral head in the glenoid on axillary view; not dislocated.&nbsp;

humeral head in the glenoid on axillary view; not dislocated. 

Proximal humerus fractures are common injuries in the elderly. They represent 4-5% of fractures; with 80-85% being minimally displaced.  Fairbank reported a 10% occurrence of pseudodislocation following proximal humerus fractures.   In some cases fat can be seen along with the fluid in the joint space.

sdlipohemarthrosisf.JPG
 from Magid

from Magid

 Us showing fluid in the joint&nbsp;

Us showing fluid in the joint 

 

http://teamrads.com/index.php/radiology-elective/musculoskeletal/shoulder

Hussein MK, the Kocher technique J Bone Joint Surg. . 50B 1968 669-671.

Qamamoto T, Yoshiya S, Kurosaka M et al. Luxatio erecta (inferior dislocation of the shoulder) a report of 5 cases and a review of the literature. American Journal of Orthopedics. 32(12) 601-3.

Hawkins RJ, Neer CS, Pianta RM, Mendoza FX Locked posterior dislocation of the shoulder 1987 J Bone Joint Surg AM 69(1)9-18.

shoulderdis.JPG

A 61 y.o. female has had a COPD exacerbation x several weeks

She was watching the superbowl, using crack cocaine and heroin and on returning home developed lightheadedness and tingling on the L side of her body.  She passed out on her lawn when arriving home and was incontinent of stool.  A TPA page was called on arrival in the ED. Head CT was negative.

 What do you notice on her chest CT?

What do you notice on her chest CT?

Our patient had a saddle embolus. In addition to her strange presentation, more history was obtained .  She had a myelodysplastic disorder and was hypercoagulable.  She had a previous PE and was supposed to be on Coumadin  which she had not taken in over a week.  She also had a clot while therapeutic on Coumadin so an IVC filter was placed on this admission. 

saddlef.jpg

The patient had persistent L sided weakness and tingling and although her MRI was neg she was felt to have a stroke.  How common is a stroke with negative diffusion weighted MRI? An “invisible stroke?”

There has been a significant increase is the use of MRI in the diagnosis of stroke.  It was thought at one time to be 88-100% sensitive for diagnosing stroke but recent evidence shows diffusion weighted MRI fails to identify stroke in 30% of cases.  The specific areas where it fails are in three categories: the posterior fossa, the brainstem, and in hyperacute ischemia( within six hours of symptoms). These patients with negative imaging have the same outcomes as strokes with positive imaging; persistent deficits and risk of recurrent strokes.

The reason for “invisible strokes” may be that the reduction in cerebral blood flow required to initiate cell swelling( and a positive MRI)  is more severe than that required to produce neurologic symptoms.

stroke ,blood flow.JPG

Case thanks to Dr. Ruoff, with stroke information supplied by Dr. Panagos.

A 44 y.o. male presents to an outside ED with penile pain and forceful urination that "sprays" per EMS.

The CT is shown below and was read as "mild stranding suggestive of pyelo"   What was missed by the teleradiologist?

 The urethra is very dilated suggesting a stricture

The urethra is very dilated suggesting a stricture

The pt initially had  2+ LE , 3+ blood and WBC of 23K. he was treated with rocephin , diagnosed as mild pyelo and elected to be treated at home with cipro. He returned in 7 days with the CT showing the image below. 

What happened?

urethra, rupture with nec.JPG

Our patient had a urethral stricture, and resultant urethral abscess leading to rupture of the urethra and necrotizing fasciitis of the perineum.

Urethral strictures can be difficult to diagnose because they can present with a variety of different symptoms. While about half present with symptoms of a UTI,  a quarter present with urinary retention and another quarter have urethral “spraying”, abscess, or urethral fistulae. The most frequent cause of strictures are infections with Neisseria gonorrhea and Chlamydia trachomatis. It is important to diagnose because in a large study 7% of people with strictures end up with abscess and fournier’s gangrene( necrotizing fasciitis of the perineum).

 Gas in the soft tissues from necrotizing fasciitis.

Gas in the soft tissues from necrotizing fasciitis.

 Necrotizing fasciitis  affects 0.4 to 1 person per 100,000 per year. Fascia come from the latin meaning a band or bundle;  referring to the thick connective tissue  covering muscles and internal organs.   The term came into use in 1952 although the disease has been recognized since the time of Hippocrates.

Affected patients usually complain of intense pain, often out of proportion for what looks like mild cellultitis. Patients often have a fever and appear sick.  The mortality is estimated at 73% if untreated. Common organisms include Group A Strep, Klebsiella, Clostridium, E. coli, Staph aureus and Aeromonas hydrophila.  Vibrio vulnificus can cause necrotizing fasciitis if an injury occurs in salt water. Gas may be found in the soft tissues.

Our patient underwent extensive debridement.  And 8 cm abscess was drained that grew anaerobes, a suprapubic tube was placed and a urethral tear was found 8 cm posterior to the meatus.  He did well post op and has been seen in clinic since his surgery.  Case courtesy of Dr. Steen.

 The fasces appears on the left.

The fasces appears on the left.

Useless trivia:

Fascia is related to fasces( a bundle of rods with a band around it and an axe projecting) which was used by the Romans to represent the power to rule.  This was adopted by the United States (it appears on the dime) and also by the French; progressive republics that imitated the Roman constitution. 

Fascio is the Italian word for labor unions and Mussolini used the term fascist as a play on two words: fascio and fasces.  He implied his movement was linked to the progressive government of the Romans. 

Rourke K, Hickle J.  The clinical spectrum of the presenting signs and symptoms of anterior urethral stricture: detailed analysis of a single institutional cohort.  Urology 2012 May;79(5):1163-7.

Paz Maya, S, Beltran D, Lemercier P, Leiva-Salinas C. Necrotizing fasciitis: an urgent diagnosis. 2014. Skeletal radiology. 43(5):577-89.

Hakkarainen T, Kopari N, Pham T, Evans H. Necrotizing soft tissue infection: Review and current concepts in treatment , systems of care, and outcomes. Current Problems in Surgery 51(8):344-62.

Trent J, Kirsner R. Necrotizing fasciitis Wounds. 2002  14(8): 284-92.

A 30 y.o. male presents with SOB, his friend says he "turned blue". Why is his trachea so severely narrowed?

 arrow points to the trachea; the answer is on the image below.

arrow points to the trachea; the answer is on the image below.

goiter2f.jpg

Our patient had a very large goiter with a substernal component.  His airway was severely narrowed and he was admitted to the ICU and placed on ECMO rather than trying to intubate him initially.  The thyroid was removed after a sternotomy and parathyroids were reimplanted.

A goiter is  most often caused often by low iodine intake. 29% of the world’s population  resides in areas where the soil is  deficient  in iodine.  These areas include Africa, eastern Europe, the Himalayas, the Alps and the Andes.  

 goiters in the Himalayas

goiters in the Himalayas

In the United States there are areas where the soil is deficient in iodine.  This led to maps of the regions where goiters are endemic;  the “goiter belt”. Many of these individuals are hypothyroid. 

goiter belt.JPG

In the 1920’s salt was iodized as well as chicken feed.  Milk cows were also treated with iodine.  So from 1971-74 iodine intake averaged 320 mcg/L. Subsequently, iodate conditioners were removed from bread and many cows were no longer treated.  There was a general reduction in salt and egg consumption because of concern about hypertension and cholesterol.  Kosher salt became popular which DOES NOT contain iodine.  As a result from 1988-94 consumption dropped to 145 mcg/L.   ( lactating women need 290 mcg/day) Other compounds which compete for iodine receptors include bromides ( in fire retardants), fluorides  and chlorides were found contaminating water supplies.  As a result the incidence of hypothyroidism is not dropping.

 cretinism(severe iodine deficiency in three children in the Congo

cretinism(severe iodine deficiency in three children in the Congo

Our patient was extubated and then had to be reintubated for tracheomalacia.  He was eventually extubated and was noted to have some vocal cord paresis ,however he was able to pass a swallow test and was discharged home on a regular diet.

Zimmermann MB, Jooste PL, PandavCX. Iodin-deficiency disorder. Lancet. 2008 Oct 4.372(9645):1251-62.

Zimmermann MB. Iodine deficiency in pregnancy and the effects of maternal iodine supplementation on the offspring: a review . Am J Clin Nutr. 2009 Feb. 89(2)668s-72s.

Caldwell KL, Miller GA, Wang RY, Jain RB, Jones RL. Iodine status of the US population , National Health and Nutrition Examination Survey 2003-4. Thyroid. Nov 18(11):1207-14.

DeGroot L, Abalovich M, Alexander EK, et al. Management of thyroid dysfunction during pregnancy and postpartum: an Endocrine Society clinical practice guideline. J Clin Endocrinol Metab. 2012 Aug 97(8)2543-65.

A 59 y.o. male being treated for melanoma comes in with shortness of breath. His CT is shown below.

What is the diagnosis?

nivolumab.JPG

Our patient had a non infectious pneumonia(organizing pneumonia) which presents with a specific pattern called the” atoll” sign in 10% of patients on nivolumab. There is a ring of inflammation around an area of central clearing.   Nivolumab is the current treatment for melanoma. Organizing pneumonia (also called BOOP- bronchiolitis obliterans or COP-cryptogenic organizing pneumonia) is also found to  association with rheumatoid arthritis, dermatomyositis and amiodarone.  It was first described by Gary Epler in 1985.  Bronchoscopy shows up to 40% lymphocytes in the bronchoalveolar lavage.

 The atoll is a coral reef around a sunken istand

The atoll is a coral reef around a sunken istand

Rather than treating these individuals with antibiotics, they are treated with steroids. Below is the CT of our patient after treatment with steroids.

niv improv with steriods.JPG

Patients on nivolumab can also have other non infectious pneumonias including NSIP, USIP and AIP. The definitions are included below (NOT required reading)

niv org pneumf.jpg

Non-specific interstitial pneumonia is a form of interstitial lung disease resulting in fibrosis.  It includes lung disease that cannot be classified into UIP, AIP, organizing pneumonia, lymphoid interstitial pneumonia or desquamative interstitial paneumonia.

UIP- usual interstitial pneumonia is characterized by progressive scarring of both lungs.  It is the most common form of interstitial fibrosis.

AIP- acute interstitial pneumonitis or Hamman-Rich appears as ARDS with diffuse alveolar damage and fluid in the alveoli.. 

 

Our patient has been on nivolumab intermittently with recurrence of lung disease every time he is placed on the drug. Case courtesy of Dr. Bhalla. 

 

Clin Cancer Res 2016 Dec 15;22(24) 6051-6060 Epub Aug1

Radswiki et al. Reversed hal sign. Radiopaedia.

Epler GR.  Bronchiolitis obliterans organiaing pneumonia. 25 years: a variety of causes, but what are the treatment options? 2011. Expert Rev Respir Med 5(3):353-61.

Li X, Pen S, Wei L. Nonspecific interstitial pneumonia overlaps organizing pneumonia in lung-dominant connective tissue disease. International Journal of Clinical and Experimental Pathology 2015;8(9);11230-11235.

A 37 y.o. male fell off his segway one week pta. a ct of the abdomen is shown below.

 Hint: It is normal

Hint: It is normal

delayed rup.JPG

He returns 12 days later with one day of shoulder pain and flank pain.  What happened?

Our patient had a delayed rupture of the spleen.  This is defined as an injury followed by a negative CT and then a proven rupture of the spleen at a later date. Our patient  was riding on a Segway when the R wheel caught on a wall.  He had to step off in front of it.  The Segway hit his leg and and he swung around so it hit his upper abdomen causing him to fall to the ground.  He had the CT shown which was read as normal.  Three days later he began to complain of “muscle spasms” .  Ten days after the injury he came in with L flank pain and had the second CT showing a large subcapsular hematoma of the spleen.

Delayed splenic rupture was first described in 1902 by Baude who noted its occurrence 48 hours after trauma. Most often it occurs 4-8 days after trauma. Potential mechanisms include expansion of a subcapsular hematoma, clot disruption, and rupture of a pseudoaneursym or pseudocyst.  Splenic abscesses and pancreatic pseudocysts may result as well after trauma.

 splenic pseudocyst

splenic pseudocyst

Our patient was admitted to surgery and watched  over night.  He had Kehr’s sign with pain in the L shoulder. He remained stable and was discharged the next day. Segway accidents are on the rise with 40% of the admitted patients admitted to the ICU according to a study from George Washington Hospital in DC.

Ironically, James Heselden, owner of the company that makes the Segway died in a Segway-related mishap where he fell off a 30 foot cliff in England.

 

Ashurst J, Wagner B.  Injuries following Segway personal transporter accidents: case report and review of the literature. West J emerg med. 2015. 16(5):693-695.

Freiwald S. Late-presenting complications after splenic trauma. Perm J. 2010 14(2): 41-44.

Berlatzky Y, Shiloni E, Anner H, Weiss Y. Delayed rupture of the spleen or delayed diagnosis of the splenis injury? Isr J Med Sci 1980 sep-oct;16;(9-10):659-65.

Schwartz SI editor-in-chief, Shires GT, Spencer FC, editors. Principles of Surgery . 5th edition. New York: McGraw-Hill;1989.

A 61 y.o. woman on steroids for COPD comes in with back pain; she is sent in to rule out cauda equina.

this is her MRI. What does she have?

 hint: it has something to do with the edema near the arrow

hint: it has something to do with the edema near the arrow

sacral insuarr.jpg

Our patient has a sacral insufficiency fracture.  This is a stress fracture of the sacrum and is a common cause of low back pain in the elderly with osteoporosis who do not experience trauma.   Bone scan and MRI are the most sensitive means of detection. Patients are managed conservatively  with bed rest and rehabilitation. More recently, sacroplasty( a minimally invasive procedure similar to vertebroplasty)  has emerged as an alternative therapy.  The sacral insufficiency fracture is found in the sacrum parallel to the SI joint. 

 The fractures can form an H across the body of the sacrum

The fractures can form an H across the body of the sacrum

The  sacral insufficiency fracture was first described by Lourie in 1982. Although they are found most commonly in osteoporosis they can be found in steroid-induced osteopenia, rheumatoid arthritis, paget's and renal osteodystrophy.  The incidence  may be as high as 89% in patients undergoing pelvic radiation for cervical cancer.

Patients present with low back pain and sacral radiculopathy mimicking cauda equina syndrome.  Pain is often in the buttock , hip, or groin not necessarily over the sacrum. They are often associated with pubic rami fractures.

Elderly patients with trauma can also fracture in the same place as those without trauma, due to weakening of the bone. Below, is a patient sp an mvc with a negative pelvic xray and a sacral fracture on CT.

sif.jpg

Our pt was managed conservatively.

Lyders EM, Whitlow CT, Baker MD, Morris PP. Imaging and treatment of sacral insufficiency fractures. 2020 AJNR 31 202-210.

Lourie H. Spontaneous osteoporotic fracture of the sacrum: an unrecognized syndrome in the elderly. JAMA 1982;248:715-717.

Blomlie V, rofstad E, Talle K, et al. Incidence of radiation-induced insufficiency fractures of the female pelvis: evaluation with MRI imaging AJR Am J Roentgenol 1996;167:1205-10.

Butler CL, Given CA, Michel SL, Tibbs  PA. Percutanwous sacroplasty for the treatment of sacral insufficiency fractures. AJR 2005 184:10956-59.

An 85 y.o. male walks into the the ED with a complaint of neck pain, he is s/p a cervical epidural injection three weeks earlier.

atlantoax.JPG

Our patient had atlantoaxial rotatory subluxation as Dr. Noelker points out. The inferior facet of C1 is displaced completely anterior to the L facet of C2.  The  right inferior facet of C1  is also displaced anteriorly. This resulted in severe spinal canal stenosis at the level of C1-2 with severe cord impingement and impingement on the R vertebral artery.  Our patient however, was neurologically intact.  He had an xray prior to his corticosteroid injections showing this was not present.

atlantoaxialprev.JPG

Did the steroid injections contribute in anyway to the problem?  It is known that steroids can  cause destruction of joints and ligaments by preventing the inflammation which is necessary for healing. This is why steroid injections are given with three week intervals between them.  Steroids also inactivate vitamin D, limit Ca absorption by the GI tract, and increase urinary excretion of calcium. This leads to a decreased uptake of Ca by the bone and ultimately weakness of the fibro-osseous junctions. In addition to delayed healing, the complications of  cervical steroid injections as found in the malpractice literature include:

Quadriplegia, respiratory arrest, cardiac arrest, dural puncture, vertebrobasilar stroke, epidural hematoma, and cervical cord infarct.

Quadriplegia which was permanent has been reported with an intravascular injection. Transient quadriplegia has been reported with injection into a cervical radicular artery.  In this report, the patient was quadriplegic for only 20 minutes .  The authors state although injecting a local anesthetic may have only a temporary effect on spinal cord function, particulate steroids may act as an embolus and cause permanent impairment.

Respiratory arrest can be transient if lidocaine causes a high cervical block, so most physicians who give the injections just numb the skin and give steroids alone after that to avoid respiratory arrest.

Causes of cardiac arrest have been reported including blockade of the sympathetics leading to a bradycardic arrest and involvement of the phrenic nerve leading to apnea.

atlantoax fixed.JPG

Our patient had surgery and is doing well. It is not known whether the steriods had any role in his condition.  Thanks to Dr. Baumgartner for the case.

 

Sofat N, Kuttapitiya A. future directions for the management of pain in osteroarthritis.  Rheumatol. Apri 2014;9(2):197-276.

Epstein NE. The risks of epidural and transforaminal steroid injections in the Spine: Commentary and a comprehensive review of the literature. Surg Neurol Int 22-Mar-2013;4:. 

Abram SE, O’Connor TC.:: Complications associated with rpidural sterid injections. Reg Anesth 1996;21:149-62.

Karasek M, Bogduk N. Temporary neurologic deficit after cervical transforaminal injection of local anesthetic. Pain Med. 2004;5:202-5

A 55 y.o. woman who has had sinus surgery comes in for epistaxis. When you scope her you see what is pictured below.

 what is it?

what is it?

This is a remnant of our reptilian  ancestry called the vomeronasal organ(VNO)  or Jacobson’s organ. It is auxillary olfactory sense organ useful for detecting pheromones.  It was discovered in 1732 by Frederick Ruysch and later rediscovered by Ludwig Jacobson in 1813. In humans the organ, which is found along the nasal septum,  regresses during fetal development but can be seen in about 25% of adults.  Image below is from Chapter 41. Physiology of olfaction. DA Leopold and EH Holbrook. Cummings Otolaryngology. 5th edition.  

 close up of the Vomeronasal organ on the septum

close up of the Vomeronasal organ on the septum

The organ has been widely studied in animals. The vomeronasal organ connects to the olfactory bulb and eventually to the amygdala and hypothalamus. This is extremely important  in reproduction and social behavior in animals since it triggers release of hormones needed for mating. The organ can detect both odorants and nonvolatile proteins such as urinary tract proteins. This explains why your dog is constantly sniffing urine from other dogs.

VNO snake.JPG

Snakes use this organ to sense prey, sticking out their tongue to gather scents and then touching it to the VNO. Elephants transfer scents from the tip of their trunks to the VNO connection which is located in the roof of their mouths. Cattle and pigs also have a  connection to the VNO  in the roof of their mouths behind their teeth and they often exhibit the Flehmen response in response to an odor.  They lift their heads , wrinkle their noses and cease to breathe momentarily allowing the odor to go through the mouth into the VNO.

 tiger catching a scent

tiger catching a scent

Our patient was being scoped  for possible bleeding after sinus surgery.  Her vomeronasal organ is just a vestigial remnant  of evolution. It does not function in humans.  Our patient had no nasal bleeding and was found to have an ulcer.

Jacobson L Anatomisk Beskrivelse over et nyt Organ I huusdyrenes naese veternaer-selskapets skrifter 1813. 2, 209-246.

Stoyanov G, Moneva K, sapundzhiev N, Tonchev AB. The vomeronasal organ incidence in a bulgarian population. 2016. The Journal of Laryngology & Otology. 130:1-4.

Trotier D. Vomeronasal organ and human pheromones. Annales francaises et de Pathologie Cervico-faciales. 2010. Vol 128 (4)222-28

A 25 y.o. woman is brought to the ED after a high speed MVC, she has a GCS of 11 and appears to be intoxicated

What do you notice on her C-spine CT?

 hint it's near C1,C2

hint it's near C1,C2

Our patient had an occipital condyle fracture seen in the figure below.  Occipital condyle fractures are uncommon but can have fatal outcomes if missed. The first description of a death reported from  a missed fracture was in a patient who was being discharged and turned his head suddenly.  He arrested and autopsy showed a fracture of the occipital condyle which had been displaced into the brainstem. In large autopsy series of fatal car crashes, occipital condyle fractures have been reported at 4.2% .

occ conarr.jpg
occ con3.JPG

 Occipital condyle fractures are often seen  in association with palsies of the 12th cranial nerve due to fracture through the hypoglossal canal. Because of the proximity of the jugular foramen, nerves IX,X, and XI can also be involved. Delayed nerve damage has been reported up to nine weeks after the injury because of dislocation of bony fragments.

 A more obvious occipital condyle fracture.

A more obvious occipital condyle fracture.

Conservative management is the standard of care unless there is severe displacement of fragments. The duration of immobilization is three months.  Unstable fractures may be treated with a Halo or occipito-cervical fixation.   Interestingly, the MRI did not show the bony fragment but showed edema centered around the L occipital condyle and surrounding apical odontoid ligament and tectorial membrane consistent with an acute fracture.  There was no cord signal abnormality. Our patient was treated with a hard collar.

 

Bell C. Surgical observations. Middlesex Hosp J. 1817;4:469-470.

Caroli E, Rocchi G, Orlando ER, Delfini R. Occipital condyle fractues: report of five cases and literature review 2005 Eur Spine J. Jun;14(5) 487-92.

Alker GJ, Oh US, Leslie EV. High cervical spine and cranio-cervical junction injuries in fatal traffic accidents: a radiological study. Orthop Clin North Am. 1978;0:1003-1010.

Bucholz RW, Burkhead WZ. The pathological anatomy of fatal atlanto-occipital dislocations. J Bone Joint Surg Am. 1979;61:248-250.

A 35 y.o. male presents with decreased vision in the R eye and pain.

What caused this problem?

meth eyef.jpg

Our patient had corneal destruction from chronic methamphetamine use.  This resulted in descemetoceles ( outpouching of the inner layer of the  cornea).   Drug induced ocular symptoms also include  retinal vasculitis, episcleritis, panophthalmitis, endophthalmitis, scleritis, and globe rupture.

descem.JPG

In the initial stages of chemical eye burns irrigation and correction of the pH to between 7.0 and 7.2  should be accomplished. Fluorescein staining may be absent with a complete epithelial defect.  In mild burns a topical steroid is applied four times a day for one week to control inflammation. A cycloplegic is also given three times per day.  In more severe burns prednisolone is given q 1 hr while awake and tapered rapidly between days 10 and 14 to minimize the risk of corneal melting. Amniotic membrane is often used to cover the cornea in severe burns.   In some cases limbal stem cell transplants are performed from the contralateral eye.

 a more subtle descemetocele

a more subtle descemetocele

 the eye exam after a corneal transplant.

the eye exam after a corneal transplant.

Our patient will be receiving a corneal transplant.

 

Hazin R,Cadet JL, Kahook MY, Saed. Ocular manifestations of crystal methamphetamine use. 2009 Neurotox Res. 15(2):187-91.

Hemmati H, Colby K, edited by Scott I and Fekrat S. Treating acute chemical injuries of the cornea. 2017 Eyenet Magazine.

A 62 y.o. male complains of persistent pain in the wrist after a fall.

 What do you notice on imaging?

What do you notice on imaging?

Our patient has wrist pain because of a scapholunate ligament tear.  This is the most common ligament injury in the wrist. Normally the scaphoid and lunate move together but when a scapholunate tear occurs the scaphoid  flexes and the lunate extends.  This causes pain in the wrist. There are four classes of injury: 1 occult seen only on MRI, 2.  Dynamic showing up on stress xrays or MRI, 3. Static visible on plain films as a gap between the scaphoid and lunate and 4. Advanced collapse where the ligament is completely torn and the injury has been there for years causing arthritis.

scapholundiagram.JPG

The pt may complain of pain while doing pushups or any activity that loads the wrist. A stress xray may be performed by making a tight fist for one xray and then bending the wrist towards the small finger for another xray and then bending toward the thumb for a final xray. A gap will occur at the scapholunate junction during activity if there is an injury.  If there is a widening of 3-4 mm this is diagnostic and referred to as the Terry Thomas sign named for a British comedian who had a gap between his two front teeth.

World class tennis players are at risk for these wrist injuries .  It is estimated that serves pack rotational velocities of 1,500 degrees per second which  is nearing the limits of human physiology. Richard Berger of the Mayo clinic is currently operating on microscopic tears  of the scapholunate ligament which are not even visible on MRI in these elite athletes so continued pain is a reason for referral even if there is no radiologic abnormality.  These injuries also occur in the more common “fall on outstretched arm” injuries that we see associated with distal radius fractures.

scapholunate dislocation2.JPG

Kuo CE, Wolfe SW Scapholunate instability current concepts in diagnosis and management J Hand Surg 2008;33A :998-1013.

 

Lew DM Osterman AL. Scapholunate instability in athletes. Clin sports Med 2001;10(1):131-40

Fufa D, Goldfarb C.  Sports injuries of the wrist. Curr Rev Musculosketat Med 2013 m6(1) march 35-40

A 20 y.o. male with C6 fracture and tetraplegia presents with severe HA.

What do you see on the MRI?

presf.jpg

Our patient had PRES( posterior reversible encephalopathy syndrome) which was first described in 1996.  The image of the MRI shows edema posteriorly on the T2 weighted MRI.  The reason for PRES in this case was autonomic dysreflexia secondary to spinal cord injury.  In patients with spinal cord injury above the T6 level there is an imbalance of sympathetic discharge since sympathetic control of the splanchnic vascular bed is no longer functioning.

When there is a noxious stimulus, for example, an enema, there is unopposed activity of the  spinal reflex mechanisms that remain intact in spite of the spinal cord injury. The splanchnic bed vasoconstricts and there is a sudden increase in blood pressure.  

In our patient this resulted in extreme hypertension with enemas or urologic procedures leading to a seizure and PRES.  This can be avoided by applying a topical anesthetic to the rectum prior to an enema or giving a dose of nifedipine. 

preschart.JPG

PRES has also been reported with duloxetine, lupus, pregnancy, chemotherapeutic agents and anti-rejection drugs. In the case of chemotherapeutic agents or antirejection therapy this is thought to be a direct  toxic effect on vascular endothelium in the brain.

 

Garg RK. Posterior leukoencephalopathy syndrome. 2001 Postgrad Med J. 77(903) 24-28.

Fugate J. Rabinstein A. Posterio reversible encephalopathy syndrom. Clinical and radiological manifectations, pathophysiology, and outstanding questions. 2015.  The Lancet Neurology 14(9) 914-925.

Hinchey J, Chaves C, Appignani B, et al.  A reversible posterior leukoencephalopathy syndrome. 1996. N Engl J Med 334(8) 494-500.

Kur JK, Esdaile JM. Posterior reversible encephalopathy syndrome—an underrecognized manifestation of systemic lupus erythematosus. 2006.  The Journal of Rheumatolgy 33(11):2178-83.

Rajasekhar A, George TJ. Gemcitabine-induced reversible posterior leukoencephalopathy syndrome. A case resport and review of the literature. 2007 Oncologist 12(11)1332-1335.

Zappella N, Perier F, et al duloxetine-related posterior reversible encephalopathy syndrome. Medicine 2016 95:33(e4556)