A 58 y.o. woman who works as a park superintendent in Sullivan, MO, comes in with somnolence, fevers and a rash. She reports several tick bites.

What illness could she have?

Our patient had an illness caused by the Bourbon virus.  This is a tick borne illness discovered in 2014 and named after Bourbon County in eastern Kansas where a man developed the index case after being bitten by ticks.  He presented with a flu-like illness and later died.  Our patient removed two ticks from her body several weeks before she was admitted to the hospital with somnolence, fevers, myalgias and a rash.

Rocky Mountain spotted fever map  above. .

Lyme disease map above.

Tick borne diseases in Missouri  also include Rocky Mountain spotted fever , tularemia,,heartland virus,  Ehrlichia, and lyme disease. Where Rocky Mountain spotted fever had been reported most often in the southeast; there were 229 cases reported in Missouri in 2017. The number of cases of Erhlchia is also rising  with 149 reported cases. Tularemia is not only found in ticks but also in rodents and rabbits.  It can be aerosolized with reported illness in a person who ran over a dead rabbit with a lawn mower. ( Francisella tularensis is very infectious with 10-50 organisms causing disease and is a candidate for possible weaponization) If contaminated animals inhabit a site, even a lawn, there have been cases of tularemia reported after mowing the lawn. Of the 200 or so cases reported each year, Arkansas, Missouri, Kansas and Oklahoma claim the most cases.

Heartland virus was discovered in Missouri at Heartland hospital near St. Joseph in 2009 when two farmers were infected and did not respond to doxycycline. Their sera was sent to the CDC and a new virus was confirmed. 

rocky mountain spotted fever rash. 

rocky mountain spotted fever rash. 

 None of the reported  incidence of tick borne disease is likely to be accurate since most people have a mild illness and do not seek medical attention.  It is a good thing that tick borne illness is often mild since ticks are a world wide vector of disease.  Two cases of Crimean- Congo hemorrhagic fever were reported from near Madrid in the July 13, 2017 New England Journal of Medicine.  This is a particularly virulent tick borne illness in which the first patient presented with fever and malaise, developed a coagulopathy and died.  The second case reported was the nurse who assisted with the intubation  and central lines of the first  patient. She developed fever and coagulopathy.  She was treated with ribavirin and developed hemolytic anemia but  eventually recovered after 22 days.

Our patient had a complicated course .  Her past history included follicular lymphoma which had been treated.  She developed encephalopathy, mouth ulcers, acute respiratory failure, hemophagocytic lymphohistiocytosis, pancytopenia and shock.  She died three weeks after admission.

 

 

Feldman KA, Enscore RE, Lathrop SL, et al. an outbreak of primary pneumonic tularemia on Martha’s Vineyard.  N Engl . J Med 2001.  345(22): 1601-6.

http://www.daily camera.com/Lafayette-news/ci-28203252/Lafayette-resident-contracts-tularemia-after-mowing-lawn-dies.

Negredo A, de la Celle-Prieto F, et al. Autochthonous Crimean-Congo Hemorrhagic Fever in Spain. NEJM 2017 377;2: 154-161.

 

A 45 y.o. construction worker states he has an infection in his hand. His hand is pictured.

What is wrong?

What is wrong?

Our patient had Sweet’s disease or acute febrile neutrophilic dermatosis.  It is characterized by tender red, papules and plaques that show dense infiltrates of neutrophils.  It was first described by Robert Sweet in 1964 and four criteria have to be met to diagnose the disease: fever, leukocytosis, tender red plaques, and a dermal infiltrate of neutrophils. Often patients with the disease are treated several times with antibiotics for cellulitis/abscess and they do not improve because the treatment is steroids. In addition to the raised plaques pts present with fever in 50% ofcases,  arthritis in 62% of cases and eye findings in 38% of cases.

skin biopsy of a pt with sweets showing neutrophils

skin biopsy of a pt with sweets showing neutrophils

It is classified by the setting in which it occurs either malignancy associated,  drug induced or idiopathic.

20% of the cases are associated with malignancy. It can also be associated with strep infections, inflammatory bowel disease, rheumatoid arthritis,  and pregnancy. Drugs that cause it include granulocyte colony-stimulating factor and granulocyte-macrophage colony-stimulating factor.  It is thought to represent a hyperactive immune response in response to a systemic process.   Steroids dramatically improve the condition in 72 hours presumably suppressing dysregulated cytokines.

The differential of Sweet’s includes:  pyodermagangranosum which appears as purple colored ulcers,  erythema multiforme which appears as red spots on the hands, feet and face( often in rings) and erythema nodosum which appears as raised plaques.

pyoderma gangranosum in the setting of crohns

pyoderma gangranosum in the setting of crohns

The process usually lasts 2-4 weeks and can recur in one third of the patients.  In our patient, treatment with steroids caused resolution of the rash and he has not had a recurrence.

 

Mustafa MN, Lavizzo M. Sweet’s syndrome in a patient with Crohn’s disease: a case report.  J Med Case Reports 2008 2:221.

Cohen PR. Sweet’s syndrome-a comprehensive review of an acute febrile neutrophilic dermatosis. Orphanet Journal of Rare Diseases 2(34).

Sweet RD An acute febrile neutrophilic dermatosis. Br J Dermatol. 1964 76:349-56.

A 33 y.o. with a history of depression is found on the basement floor wearing a swim suit and in a pool of urine. Pictured are her urine and vomitus.

She is somnolent; what could she have taken?

She is somnolent; what could she have taken?

Our patient had nephrogenic diabetes insipidus from Lithium.  She had texted her mother and told her she had taken 160 pills.  Her mother found her in the basement in a pool of urine with empty bottles  of Lithium( which she had been prescribed a year earlier) and clonazepam.  The reason she was in a pool of urine is that  Li causes nephrogenic diabetes insipidus.  Her urine was very dilute with a specific gravity of 1.004.  In the ED  she was rousable to painful stimuli and began to vomit so was intubated for airway protection.  She continued to make large volumes of dilute urine after her admission to the ICU.

 

The name insipidus comes from the old practice of tasting urine to determine the cause of illness.  Unlike diabetes mellitus which results in sweet urine, diabetes insipidus creates watery flavorless urine. (tasting urine is no longer recommended).  The word diabetes comes from the Greek diabainein ”to pass through” and refers to the excessive amounts of urine in both conditions. In diabetes insipidus more than 2.5 L of urine is produced per day.  Li is the most common drug implicated in nephrogenic DI with50% ofpeople on Li chronically developing dilute urines.  It is more common in chronic overdose but has been reported with acute overdose.

Nephrogenic DI is caused by conditions affecting aquaporin(porous to water) channels in the tubules which allow water resorption.  Aquaporin 2 (AQP2) in the kidney is usually dormant in the intravesicular membrane. When it is needed, vasopression ( antidiuretic hormone) cases the vesicles containing aquaporin to fuse to the membrane and release aquaporin which happens in water retaining states like pregnancy and chf.  Li, however, decreases the expression of the AQP2 gene preventing the absorption of water.

Persons with nephrogenicDI need to consume amounts of fluid equal to the amount excreted but they excrete more water than sodium so serum osmolarity increases which stimulates thirst.  Thiazide diuretics are often given to allow increased excretion of sodium, decreasing serum osmolarity and therefore decreasing thirst.  This breaks the polydipsia-polyuria cycle.  In the case of our patient she was producing 300 cc of urine per hour for several days and these fluids were replaced.

Our patient remained somnolent/intubated because of the clonazepam overdose and although her initial nasal swab was negative for staph, she developed MSSA pneumonia and remained intubated for 8 days.  She was extubated on the 8th  day and is now alert and oriented agreeing to psychiatric admission.

 

Clinical pearls: serum lithium levels do not correlate well with clinical findings. Our patient’s Li level rose only to 1.5and yet she had profound polyuria.  If sodium rises desmopressin can be given to correct the electrolyte abnormalities. Dialysis is helpful with neurologic toxicity which usually occurs with a Li level of 3 or greater.

Wildin R. What is nephrogenic diabetes insipidus? 2006.  The diabetes Insipidus Foundation http://www.diabetes insipidus.org/4-types-nephrogenic-di.htm

Erden A, Karagoz H, et al. Lithium intoxication and nephrogenic diabetes insipidus: a case report and review of literature. Int J Gen med 2013;6:535-539.

Alexander MP, Farag YMK, et al. Lithium toxicity: a double-edged sword.  Kidney Int. 2008;73:233-7.

A 44 y.o. woman presents with memory loss, headaches and "twitching" of her arms.

What does her MRI show?

What does her MRI show?

Our unfortunate patient had familial prion disease which results in the destruction of the thalamus and corpus striatum.  There is no cure for this condition and it is uniformly fatal.  

The gene for  prion protein (PRPc)  isfound in all mammals and appears on chromosome 20.  It can spontaneously mutate as in our patient or a malformed protein can be acquired by eating infected protein.  When prion proteins are  malformed  (PRPsc)  they act like viruses and cause other  prion proteins( or alpha-synuclin)  to be misfolded as well.  This makes them unique since all other infectious agents contain nucleic acids ( DNA or RNA) . 

Prions were initially identified as the causative agent in acquired prion diseases in animals such as scrappie in sheep  and bovine spongiform encephalopathy ( mad cow disease). In humans it causes Creutzfeldt-Jakob disease , fatal familial insominia and kuru.  After crossing the blood brain barrier, the abnormal prion proteins  are reproduced in the endoplasmic reticulum of the cell and cause misfolding of other normal prion proteins.   They  then migrate to the surface of cells where they form  amyloid plaques which disrupt structure and cause “holes” in the tissue because of vacuoles in the neurons.

The acquired form of prion disease kuru( which means to shake) was frequent in New Guinea where the Fore people historically ate the brains of dead relatives.  When the Fore people stopped eating human meat about 50 years ago, the disease lingered due to its long incubation period.  The last known kuru victim died in 2009.  Interestingly,  in studying the epidemic, some people were found to be immune, suggesting that a long period of cannibalism had allowed the development of resistance.

 

The incubation period for prion disease is 5-20 years but once symptoms occur it rapidly leads to brain damage and death.. The striatum coordinates cognition including decision making, motivation and action planning. The loss of these parts of the brain results in dementia, ataxia, memory loss and seizures.  Our patient was counselled as to her prognosis .

The disease affects one in a million people.  Although rare, it is thought that alzheimer’s may also act in a prion like fashion which makes a cure for prion disease the “holy grail” of neurodegenerative research.

 

Alberti S, Halfmann R, King O, Kapila A, Lindquist S  A systematic survey identifies prions and illuminates sequence features of prionogenic proteins Cell 2009.  137(1) 146-58.

Prusiner SB Molecular biology of prion diseases Science. 1991 252 1515-1522.

Alpers, M. A history of kuru. 2007 Papua and New Guinea Medical Journal 50 (1-2) 10-1p

A 70 y.o. man presents with a one day history of bilateral parotid swelling

What is the differential

Our patient has bilateral parotid enlargement from Sjogren’s syndrome . He had painless bilateral enlargement of the parotids over one day.  He had a similar unilateral event several years pta which resolved spontaneously.   On arrival he spiked a temp to 39 and was treated as bacterial parotitis although cultures from Stensen’s duct were negative. He had ANA + 1:2500, RF 250, sed rate 99, ENA +, ENA SSA+ and anti Jo -.  

 

The differential of bilateral parotid swelling is extensive although it can be divided into obstructive(related to stones), infectious , malabsorption related (pancreatitis) and immune. Bacterial causes that are  related to a stone in the ductare painful and most commonly in females.  Viral infections are often caused by mumps , cocksackie, or HIV.   There is a chronic parotitis which occurs in children age 2-7 which is painless.

sarcoid is also a cause of bilateral parotid swelling

sarcoid is also a cause of bilateral parotid swelling

 Wegeners and Sjogrens are the immune diseases most commonly causing parotid enlargement. Kimura’s disease is bilateral parotid enlargement with elevated IgE and eosinophilia. Tumors can also cause bilateral parotid enlargement specifically Warthin’s tumor,  as well as benign masseteric enlagement .

Radiation, specifically radioactive iodine and pneumoparotid caused by blowing a wind instrument can also cause parotid swelling.

air in parotids

air in parotids

 Sjogrens syndrome  as in our patient, usually results from parotid and salivary gland involvement with dry eyes and dry mouth. Extraglandular manifestions of Sjogrens can includebiliary cirrhosis, pulmonary interstitial disease, renal disease and peripheral neuropathy. 5% of these patients develop B cell lymphoma.

Treatment of the disease is symptomatic , with artificial tears and throat lozenges for dry mouth.  Immunosuppression is not recommended.  Our patient was treated symptomatically after a short course of antibiotics and has remained asymptomatic.

Mandel L, Surattanet F, Bilateral parotid swlling : a review. Oral Sug Oral Med Pathol Oral radiol 2002.93: 221-232.

Gadodia A, Bhalla A, Sharma R, Thakar A, Parshad R.  Bilateral parotid swelling: a radiologic review. Dentomaxillofac rad 2011Oct 40(7) 403-414.

Yousem DM, Krout MA, ChalienA. Major salivary gland imaging Radiology 2000;216:  19-29

 

A 21 y.o. avid outdoorsman complains of R pleuritic chest pain

his cxr is shown below

He was treated for RML pneumonia with levofloxacin and symptoms resolve but return in 3 weeks with the xray below.

What is wrong with this patient?

What is wrong with this patient?

Our patient had coccidiomycosis in a pulmonary form which led to “phantom” infiltrates. These repeated pneumonias are a classic pulmonary presentation for the fungal infection.

Coccicdiomycosis is a fungal disease native to the Americas. It is caused by two fungi: C. immitis and C. posadasii which are endemic to the southwestern US, northern Mexico and central and South America.

 

It was first described in 1892 in a soldier in Argentina.  The illness in the US was found with increasing prevalence when immigrants moved to California after the dustbowl in the 1930’s.  The first effective therapy was not found until 1957 when IV amphotericin was used.

 

Initial infection can occur from inhaling a single spore of the fungus which then rapidly multiplies in the lung over 48-72 hours.  60% of people are asymptomatic with this infection because the fungus is destroyedby macrophages.  It can , however, cause a flu-like illness or pulmonary disease including infiltrates as in our patient, nodules or even cavitary lesions which can rupture into the pleural space causing pneumothoraces or effusions.  Only 1% of healthy individuals go on to develop disseminated coccidiomycosis(although this number is higher in the immunocompromised) .  Disseminated disease can result inosteomyelitis, arthritis, meningitis or transplacental infection in a pregnant woman. Disseminated disease can also present with skin infections including abscesses, granulomas , ulcers or rashes often of the nasolabial folds.  Disseminated infection may require years of antifungal treatment at a cost of $5,000-12,000 per person per year.

Coccidiomycosis is found with increasing frequency in Arizona where 80% of the population who have lived there for five years is positive for coccidiomycosis.

 

 Our patient had been hiking in Arizona and presumably this is where he acquired the disease.  He was treated successfully with antifungals and had no recurrence of the disease.

Amstead GM, Graybell JR. Coccidiomycosis Infect Dis Clinic N AM 2006 20: 621-43.

Gaidici A, Saubolle MA. Transmission of coccidiomycosis to a human via a cat bite. Microbiol Feb47(2):505-6.

CDC From the Centers for Disease Control and Prevention. Coccidiomycosis following the Northridge California Earthquake.  JAMA 1997 March 19 277(11) 904-8.

Increase in reported coccidiomycosis in the United States 1998-2011.  MMWR. Morb Mortal Wkly Rep 2013 March 29 62(12): 217-21.

 

A 79 y.o. woman is a restrained passenger in a high speed MVC. + LOC . her only complaint is that she is confused.

What do you see on the CXR that might explain her confusion?

Our patient had a stroke most likely caused by embolic phenomenon after an aortic injury.  This is not the usual transection since it also involved the subclavian artery with non occlusive thrombus in the proximal vessel. Strokes are more commonly associated with aortic dissection

than transection and have even been reported after being struck in the chest with the recoil after firing a rifle.

The aortic injury is seen in this image with a mediastinal hematoma surrounding.

Acute traumatic rupture of the aorta accounts for 18% of all deaths in auto accidents.  The most common site of rupture is the proximal descending aorta near where the subclavian branches off. This is where the remnants of the ductus arteriosus are located; the structure that shunts fetal blood from the pulmonary artery to the aorta . Those who do not die immediately(80%)  can have an incomplete tear and a false aneurysm.  In our patient there was an injury to the aorta and blood dissected both into the mediastinum causing the widened mediastinum on xray and into the pericardium.  Probably because of her strokes, the patient did not report chest pain or back pain until later in her course. 

Other injuries includedR rib fractures 3-7, a C2 tear drop fracture, and three punctate foci of diffusion restriction on MRI (one in either occipital lobes and one in the R hippocampus) compatible with embolic strokes.

C2 fracture

Our patient had a TEVAR ( thoracic endovascular aortic repair) stent placed and a bypass of L carotid to the L subclavian. A nurse noted that the bp in the R arm was 155/52 and in the L was 70/52  post op.   This was due to the vascular procedure and expected; so BP in a patient like this should be taken only in the R arm.  She is currently intermittently alert.

 

Khoynezhad A, Donayre C, Bui H. et al. Risk ofneurodefecit after thorcic aortic endografting. Annals of Thoracic Surgery, 2007. Vol 83(2).

Lobay K, Macgougan C. Traumatic ooronary dissection: a case report and literature reviews.  J Em Med 2012 43 e239-43.

Phillips BJ. Traumatic rupture of the thoracic aorta; an endoluminal approach.  2001. Internet Journal of Thoracic and cardiovascular surgery 4(1) 2001do1: 10.5580/2563.

McKnight J, Meyer J, Neville J.  Non penetrating traumatic rupture of the thoracic aorta. 1964. Ann Surg 160(6); 1069-1072.

Rao M, Panduranga P, Al-Mukhaini M et al. Ischmic stroke secondary to aortic dissection following rifle butt recoil chest injury.  Oman Med J. 2011 26(8) 438-440.

A 51 yo male was working on his car in the rain and was found unconscious with the rash below.

What happened?

This patient presents with Lichtenberg figures from a lightning strike.  They are thought to be due to the rupture of capillaries under the skin caused by the currentor flash overs just above the skin causing bruising. They were first described by Georg Lichtenberg in 1777 when he noted that sulfur granules on the surface of insulating materials could form fractal like images if a current was applied. 20-30% of those struck by lightning have these figureswhich last 1-3 days.

Benjamin Franklin flew his kite on a stormy day in Pennsylvania in 1752 and  learned that lightning is an electrical discharge creating a current that flows either within a cloud or between a cloud and the ground. Since the upper part of a thunderstorm cloud becomes positively charged while the middle becomes negatively charged a current can flow between the two areas.  

There are several ways to be struck by lightning. In a direct strike the person becomes part of the main lightning discharge channel.  A portion of the current moves along the skin surface and a portion moves through the body along the cardiovascular or nervous systems. Asystole is a common result, however the natural automaticity of the heart may cause a rhythm to resume.    A side flash occurs when lightning strikes a taller object near the victim anda portion of the current jumps to the person. Less current is transmitted to the person and this is probably what happened to our patient since a tree next to his car was struck first and he did not have an exit wound .Ground current is the third way you can be effected by lightning. This is where the lightning hits the groundand travels along the ground.  It enters the body at the closest point to the strike and exits at the point furthest away.  This often kills farm animals.  Conduction  of the lightning strike can also kill people.  In this case the current travels along a metal surface like plumbingor electrical cords and can cause an electric shock. The final way you can be injured by lightning is with a streamer.  Streamers develop as the downward-moving leader approaches the ground.  Only one streamer will rise up to contact the leader but all other streamers in the areacan discharge.  This gives rise to numerous stories of being hit by a “bolt from the blue” since you can be at a distance from the main discharge. A famous case of a bicycle rally in Vail described a participant being hit by lightning under a cloudless sky. 

Lightning causes 4,000 deaths per year worldwide and extensive property damage. Lake Maracaibo in Venezula is the number one spot for lightning in the world (252 strikes per kilometer per year) with the Democratic Republic of Congo also reporting frequent lightning strikes.  Since ideal conditions for lightning include areas where warm moist air mixes with cold air above; these two areas would be ideal.  More lightning occurs over land than over the ocean because sunshine heats land surfaces faster than water.  In the US Florida has the highest number of lightning strikes

Our patient did well.  He initially had fallen from his truck to the ground.  His troponin peaked at 0.35, and CK at 401. He was discharged in two days with no treatment. 

 

http://en.blitzortung.org/live_lightning_maps.php  real time lightning map

Cherington M, Olson S, Yarnell P.  Lightning and Lictenberg Figures. InternationalJournal of the Care of the InjuredVol 34(5) 2003; 367-371

Domart Y, Garet E.  Lichenberg figures due to a lightning strike. New Englan Journal of Medicine. 343:1536 2000.  Images in Clinical Medicine.

Theodore Gray, TTheo Gray’s Mad Scienc Experiments You Can Do At Home-But Probably Shouldn’t Blanck Dog & Leventhal Publishers, 2009 ISBN 978-1579127916.

A 52 y.o. pedestrian was struck by a car. She was unresponsive at the scene and brought to the ED. Her imaging appears below.

Why is she unresponsive?

Our patient had an atlanto-occipital dissociation with a basion fracture. 

 

Tectorial membrane and alar ligaments provide the stability of the atlanto-occipital joint.  When the basion-dens interval is > 10 mm suspect disruption of the joint.  The injury is immediately fatal in 70% of cases with an additional 15% surviving to the ED.  The injury is more common in children because of the large size of their heads relative to their bodies and the more horizontal occipital condyles in children.    The injury represents 1% of all cervical spine injuries.  Rare survivors usually have a neurological deficit with damage to  cranial nerves VII to X.

The Wackenheim line can be drawn from the clivus and should intersect the dens posteriorly if ligaments are intact. 

In our patient a CT angio showed there was no flow to the brain and care was withdrawn.  She did not have an increased basion to dens distance and it is presumed that the fracture was reduced when a collar was applied. The treatment of atlanto-occipital dislocation is surgical and we happened to see a survivor of an atlanto-occipital dislocation in the same week.  He had the base of the skull fused to the vertebral column.  He had no lateral movement of his head but was neurologically intact.

 

Theodoere N, Aarabi B, Dhall SS, Gerb, DE, et al. The diagnosis and management of traumatic atlanto-occipital dislocation injuries. 2013 Neurosurgery 72 Supple 2: 114-26.

CooperZ, Gross JA, Lacey JM, Traven N, Mirza SK, Arbabi S. Identifying survivors with traumatic craniocervical dissociation: a retrospective study.  2010.  Journal of surgical research 160: (1 ) 3-8.

A 55 y.o. diabetic presents with a chief complaint of ," I am going blind in my R eye"

You are unable to see the fundus and see what is pictured below on eye exam. What does the patient have?

Our patient had mutton fat precipitateson the inside of the cornea.  It was impossible to see the fundus, not because of a vitreous hemorrhage but because the deposits did not allow light through. She had pronounced photophobia which is a key finding in uveitis.

Uveitis is inflammation of the pigmented layer that lies between the retina and the outer fibrous layer which is made up of thesclera and cornea.   It includes the iris, ciliary body and choroid.  Uveitis  is classified anatomically by the part of the eye involved into anterior chamber which is similar to iritis (occurring frequently  with a corneal abrasion), intermediate involving thevitreous, or posterior uveitis  (chorioretinitis)  involving the retina.    90% of uveitis is anterior.

Mutton fat  precipitates are white blood cells deposited on the endothelium of the cornea which occur because of inflammation of the iris or ciliary body.  They are “greasy” in appearance; hence, the name mutton fat. They can occur in uveitis caused by herpes, sarcoid, syphilis, tuberculosis HLA-B27, and autoimmune diseases.

T cells must normally be suppressed by dendritic cells in the eye which produce TGF beta . If T cells proliferatesystemically, they can migrate to the eye and cause release of cytokines and inflammation.

Globally,  many infectious diseases also result in uveitis including Lyme disese, syphilis,  parasitic disease and even Zika virus.  Our patient was felt to have uveitis from a previous herpes infection.

Teaching point:  Consider the causes of uveitis when evaluating the red eye.

 

Caspi, R A look at autoimmunity and inflammation in the eye. Journal of Clinical Investigation 2010.  120(9) 3073-83.

Chang JH, Wakefield D.  Uveitis: a global perspective.  Ocular immunology and inflammation  2002. 10(4) 263-79.

Ruggieri S, Frassanito MA, Dammacco R. Treg lymphocytes in autoimmune uveitis. 2012 Ocular immunology and inflammation 20(4): 255-61.

A 27 y.o. female presents with RLQ pain. The US appears below.

What is the differential

What is the differential

Our patient had been doing sit-ups on an incline and suffered a rectus sheath hematoma.  Her initial Her hemoglobin was 11 which dropped to 8 when she arrived in our ED. 

The rectus muscles attached to the ventral aspect of the 5,6,7 costal cartilages and extend to the superior pubic ramus.  The arcuate line is located 5 cm below the umbilicus and separates the rectus muscles into upper and lower sections.  The upper section has three or four transverse tendinous insertions attaching the muscle to the under lying fascia, creating the “six pack” seen in body builders.

During contractions of the rectus muscle, the length of the muscle changes and the superior and inferior epigastric arteries must glide with the muscle.  The arteries themselves are loosely attached and move easily but their branches are more fixed and shear stress is created during contraction of the muscle.  This is the area most likely to tear.

Rectus muscle hematomas may have a positive Carnett’s sign.  This was first described in 1926 by John Carnett.  When the muscles of the abdominal wall are tensed by asking the patient to lift the head and shoulders from the bed; pain is worsened with abdominal wall pathology.  The test will be positive with hernias, nerve entrapment syndrome, and irritation of intercostal nerve roots.

The treatment of rectus hematomas is almost always conservative.   Our patient was transfused and watched.  She was seen several months later still complaining of abdominal pain although it had improved.  This is the usual course of rectus sheath hematomas with resolution occurring over 2-3 months.  Clinical pearl: consider the rectus sheath hematoma in patients with abdominal pain.

 

Suleiman S, Johnston DR. The abdominal wall: an overlooked source of pain. 2001 Am
Fam Physician 64(3) 431-8.

Gray DW, Dixon JM. Seabrook G, Collin J. Is abd wall tenderness a useful sign in the diagnosis of non-specific abdominal pain? Ann R Coll Surg Engl. 1988.  70(4):233-4.

Fitzgerald JEF, fitzgerald LA, Anderson FE, Acheson AG The changing nature of rectus sheath haematoma: case series and literature review.  International Journal of Surgery 2009. 7 (2) 150-54.

A 64 y.o. diabetic presents with L flank pain and elevated blood sugar. His CT is below.

What is the reason for his pain?

Our patient had a rupture of his L kidney caused by obstruction. Forniceal rupture occurs from several causes.  In a study of 100 cases of kidney rupture:  obstructing stones caused 74% of ruptures, with malignant external compression of the ureter causing 8.3%,  Benign extrinsic compression caused 1.9%, pelvic ureteral obstruction caused 1.9% with vesicoureteral obstruction causing the same number.  They listed 3.7%  as iatrogenic (i.e. during stent placement)  and bladder obstruction in 0.9%.  Bladder obstruction was the cause in our patient.  Several patients had unusual causes of obstruction, for example, an aortic aneurysm. In children obstruction is often at the pelvis of the kidney.

The most common cause of kidney rupture, obstructing stones were also studied and the average stone size was 4 mm.  Once the kidney ruptures, either the urine becomes encapsulated forming a urinoma or becomes free fluid in the peritoneal cavity, urinary ascites.

The treatment is relieving the obstruction; in the case of our patient a foley was placed.  In cases where a stone is present, a stent is often placed. Doppler US can indirectly provide a measure of renal pelvis pressure.  Thanks to Drs. Schwarz, Ruoff and Kane for the case.

Allin B, Chetwood A, Khoubehi B, DasGupta R. Ruptured renal calyx mimicking leaking abdominal aortic aneurysm.  BMJ Case Reports.

Mostbeck GH, Zontsick T, Turetschek. Ultrasound of the kidney: obstruction and medical diseases. European J of Rad Vol 11(10) 2001, 1878-89.

Tsai, J, Huang F. Intermittent hydronephrosis secondary to ureteropelvic junction obstruction: clinical and imaging features.  Pediatrics Jun 2006, vol 117(4).

Gershman B, Kulkarni N, Sahani D. Eisner BH. Causes of forniceal rupture.  2011. BJU. Discussion 1912.

 

A 61 y.o. Hispanic male presents with a headache.

Why does he have a headache?

Our patient had a benign condition; a mucocele. The term mucocele was first coined by Rollet in 1896. This is a collection of mucous which cannot drain because of sinus ostia  blockage and results in bony expansion due to continued production of mucous. This either occurs because of chronic infection/chronic allergies or as a result of trauma.

In this case the mucocele was in the maxillary sinus but they can occur in the frontal sinus and extend into the orbit, the ethmoid sinus compressing the optic nerve, and the sphenoid sinus compressing the pituitary and brainstem or causing cavernous sinus thrombosis.  Two thirds of all mucocles occur in the frontal and ethmoid sinuses.  Mucoceles are rare in the pediatric population except in cystic fibrosis. The mucocele below is in the frontal sinus and causing proptosis. 

If a mucocele becomes infected, there can be extension into adjacent spaces: intracranial extension can occur with empyema, meningitis or abscess, orbital extension can occur with subperiosteal abscess or subcutaneous extension can occur as in Pott’s puffy tumor of the forehead.

It is essential to differentiate a mucocele from a mucus retention cyst. A mucus retention cyst is just a fluid filled sac along the sinus lining which does not expand and push into the eye socket, nose or brain and does not cause problems in the vast majority of cases. It does not fill the whole sinus.  Mucus retention cysts in the sinuses are  common. They occur in 30-40% of the population with absolutely no complaints of sinus problems and are discovered incidentally on CT scans done for another reason.

The pictures above show the sphenoid sinus in a diagram and on CT. Sphenoid mucoceles can cause compression of the pituitary.  

Our patient underwent endoscopic sinus surgery with opening of the maxillary sinus antrum.  There were no complications.

Mafee MF, Valvassori GE, Becker M. Imaging of the head and neck. George Thieme Verlag. 2004 ISBN :15889000096.

Guttenplan MD, Wetmore RF. Paranasal sinus mucodele in cystic fibrosis. Clin Pediatrics ( phila_. 1989;28(9): 429-30.

Har-El G. Endoscopic management of 108 sinus mucoceles. The Larynoscope.  2001;111:2131-4.

A 42 y.o. woman who had a Roux-en-Y gastric bypass in 2004 presents with a rash on her hands.

What is the cause of the rash?

What is the cause of the rash?

Our patient had zinc deficiency  also known as acrodermatitis enteropathica if it is a congenital defect in zinc carrier protein ZIP4 .  Zinc deficiency may have been caused  in our patient by a combination of factors since she was an alcoholic, had a Roux-en-Y gastric bypass,  and had chronic pancreatitis causing malabsorption.  Her zinc level was documented to be low on several occasions.

Zinc deficiency is most commonly caused by decreased dietary intake and 25% of the world’s population is at risk. Specifically,  zinc deficient soils are found in several areas of the world, notably in central Turkey.  Lack of zinc is responsible for increased rates of malaria, diarrhea and pneumonia since it plays a role in immune function.  Zinc deficiency may lead to anorexia nervosa.  A 1994 trial showed that zinc doubled the rate of body mass increase in the treatment of anorexia nervosa. Wilson’s disease, sickle cell disease, chronic kidney disease, and chronic liver disease have all been associated with zinc deficien

The highest concentrations of zinc are found in animal proteins with the number one concentration of zinc being found in oysters.  Exercising and excessive alcohol  intake decrease the levels of zinc in the body

Vitamins as well as trace elements like zinc can be deficient in the alcoholic population.  Our patient was found to be Vit A deficient as well.  The chart below reviews the findings in vitamin deficiencies and the one below that shows the amount of time it takes to become deficient in various vitamins.

Our patient suffered a coagulopathy with her malnutrition and had a retroperitoneal bleed with BP of 66 at one point. On CT blood was found in the R psoas muscles and bilateral iliacus muscles with active extravasation in the left iliacus.  She required emergent transfusion.  In the hospital she continued to have abdominal pain and poor po intake so TPN was started.  She remains hospitalized.

Foster M, Samman S. Zinc and regulation of inflammatory cytokines. Implications for cardiometabolic disease. Nutrients 4(7):676-94.

Suzuki H, Asakawa A, Li JB et al. (sept 2011) Zinc as an appetite stimulator-possible role of zinc in the progression of diseases such as cachexia and sarcopenia.  Recent patents of food, nutrition and agriculture 3(3):226-31.

Prasad AS (2003) Zinc deficiency: has been known of for 40 years but ignored by global health organizations BMJ 326(7386): 409-10.

A 50 y.o. man presents with sinus congestion and deafness in the R ear

When you look in his mouth you see what is pictured below.

you notice an ulcerated mass on the palate directly in the midline.  What is this?

Our patient had a basaloid poorly differentiated squamous cell carcinoma. The mass extended through the palate into the sinus. 

palate tumor.JPG

The differential for lesions of the palate is interesting and ranges  from benign to malignant.  Nicotine stomatitis is common and caused by nicotine irritation of salivary glands. It appears initially as whitish patches of hyperkeratosis and keratotic papules with red centers.  A second benign lesion is necrotizing sialometaplasia.  This is caused by ischemia of the salivary glands of the palate ( often caused by dentures or vasoconstriction from cocaine)  It is thought that long term use of salbutamol might lead to thinning of the mucosa making it more susceptible to trauma.  A biopsy is often necessary to prove this is not cancer.

nicotine stomatitis

nicotine stomatitis.JPG
necrotizing sialometaplasia

necrotizing sialometaplasia

Lesions of the palate can also be malignant. 50% ofall hard palate cancers are squamous cell carcinoma.  In addition to squamous cell CA , sarcomas, adenoid cystic carcinoma, mucoepidermoid ca, adenocarcinoma,  melanomas and  salivary gland tumors are found in the palate. .  Lethal midline granuloma   renamed NK-T cell lymphoma is a particularly devastating form of cancer leading to destruction of the midface.  It can be treated with radiation but has a very poor prognosis.

lethal midline granuloma

Our patient is being prepared  for surgery. Clinical pearl:  Remember to look in the mouth on your patients. 

 

Fowler CB. Brannon RB. Subacute necrotizing sialadenitis: Report of 7 cases and a review of the literature. Oral SurgOral Pathol Oral Radiol Endod. 200:89:600-9.

Carlson DL. Necrotizing sialometaplasia: A practical approach to the diagnosis. Arch Pathol Lab Med 2009;133:692-8. 

Ghosh, SN, Dey SK, Chattergee C. Lethal Midline Granuloma.  Indian J Derm 1995;40:53-4.

A 51 y.o. man comes in after being treated for bronchitis with the urine shown below.

dark urineapril.jpg

What do you think is wrong?

Our patient had received augmentin for his bronchitis.  Augmentin is the most frequent cause of idiosyncratic drug-induced injury in the US. Augmentin causes a moderately severe mixed hepatocellular-cholestatic injury, particularly in older men. In a study from 2016by deLemos et al,  of 117 patients reviewed only 3 required liver transplants. The mean time to injury was 31 days and average time to return to normal was 55 days.

Levels of bilirubin in the blood greater than 3 results in jaundice. Jaundice is from the French jaunisse, meaning “yellow disease”.   Elevated bilirubin results in yellowing of the whites of the eyes which is termed scleral icterus although it is actually the conjunctiva that is discolored. High bilirubin is divided into two types: conjugated and unconjugated. 

Whether  the bilirubin is conjugated or unconjugated dependson the underlying cause of the jaundice. 

Pre-hepatic    Unconjugated bilirubin most commonly occurs with the breakdown of RBCs and release of heme.   This is most often caused by malaria in third world countries as well as thalassemia, Gilbert’s, neonatal jaundice and genetic disorders like Crigler-Najjar syndrome. Bilirubin is not found in the urine because unconjugated  bilirubin is not water soluble but there is an increase in urine urobilinogen and serum unconjugated bilirubin.

Hepatic   Jaundicethat is caused by a process in the liver is most commonly seen in cirrhosis, primary biliary cirrhosis ( impaired excretion of conjugated bilirubin into bile), leptospirosis, drug reactions and hepatitis.  This results in both conjugated and unconjugated bilirubin in the blood. The congested bile canaliculi rupture releasing conjugated bilirubin into the lymph and ultimately blood (bypassing the intestine) where it is excreted by the kidney causing the urine to be dark as in our patient.

Post Hepatic   Jaundice that is caused by a blockage after bilirubin is conjugated is most commonly due to gallstones or pancreatic cancer.  In the developing world liver flukes would be a common cause.  In complete obstruction of the bile duct, no urobilinogen is found in the urine, since bilirubin has no access to the intestine and cannot be broken down into urobilinogen.

Our patient was admitted . the Liver service was consulted the next day and recommended imaging to ro pancreatic cancer.   No cancer was found , no eosinophilia was present to suggest DRESS and the patient was discharged.  He continued to complain of pruritus but refused further medication that was offered: cholestryramine.  Case courtesy of Dr. Aubinwho would like to remind everyone that unnecessary medications can cause problems.

deLemos AS, Ghabril M,Rockey DC, Gu J, Barnhart HX, et al. Amoxicillin-Clavulante-Induced Liver Injury. 2016. Dig Dis Sci Aug;61(8):2406-16.

Dr. Chase’s Family Physician, farrier, bee-keeper, and second receipt book: Chase publishing company. 1873. P. 452.

Hall, JE, Guyton A. 2011 Textbookof Medical Physology, Aunders/Elsevier , p841, ISBN 1416045740.

O’Keefe L. Increased vigilance needed to prevent kernicterus in newborns. May 2001.  American Academy of Pediatrics. 18(5): 231.

 

A 66 y.o. farmer presents with an abnormal cxr and no symptoms.

What do you see on the xray?

Our patient has a paraesophageal hernia. 

There are two kinds of hernias through the hiatus of the diaphragm:  sliding hernias where the GE junction slides into the chest and paraesophageal hernias where the GE junction stays in place but the stomach herniated alongside it. This type of hernia is is a true hernia because it includes a peritoneal layer. These hernias account for only 1% of midline hernias but are significant because the stomach can then become twisted and ischemic causing perforation of ulcers called Cameron’s ulcers.  .  A patient with a paraesophageal  hernia with chest pain is a medical emergency and ischemia of the stomach must be ruled out.

In the sliding type of hernia, gastroesophageal reflux is the most common complaint.  In the paraesophageal hernia, they can be asymptomatic or develop, vomiting, chest pain, anemia and Barrett’s esophagus.

Our patient became symptomatic with vomiting and a five port approach to repair his hernia with a laparoscopic repair.  Gastric reduction, excision of the sac, esophageal lengthening by wedge gastroplasty creating a “neoesophagus”, crural repair and Nissen fundoplication were done.  The patient recovered without incident.

 

The congenital diaphragmatic hernias that appear on the ER boards are Bochdalek and Morgagni.  The Bochdalek is most common on the posterior left side of the diaphragm ( Bochdalek is back to remember it easily) and the Morgagni is on the anterior right.    Case courtesy of Keith Naunheim.

Skinner DB, Belsey RH/ Surgical management of esophageal reflux and hiatus hernia: longterm results in  1.030 patients. J Thorac Cardiovasc Surg 1967;53:33-54.

Luketich JD, Raja S, Fernando HC, et al. Laparoscopic repair of giant paraesophageal hernia: 100 consecutive cases. Ann Surg 2000;232: 608-18. 

 

https://www.ctsnet.org/article/laparoscopic-repair-giant-paraesophageal-hernias

A 35 y.o. male has just started a job at a sushi restaurant and comes in with a rash on his hands.

What could cause this?

Our patient had severe dermatitis secondary to urushiol, the active ingredient in poison ivy which causes a rash.  How, you might ask, is this related to working in a sushi restaurant?   Urushiol is also found in the skin of mangoes which the patient had been peeling in the restaurant.  He knew he was very allergic to poison ivy because he had previously worked in landscaping and on several occasions his eyes swelled shut and he required steroids.  He had no idea that mangoes contain the same toxin.  The differential would also include Mycobacterium marinum.

The family Anacardiaceaeare a family of flowering plants containing mango, poison ivy, sumac, and cashews.  The skin of mangos contain urushiol as well as the shells of cashews; which is why cashews are always sold after they are shelled.

Poison ivy is most likely to transmit urushiol in the spring when the shoots are tender and sap is likely to be exposed.  The name urushiol comes from the Japanese “urushi” meaning lacquer  because of the extensive work done on the Japanese lacquer tree in the 1920’s to identify urushiol.   Onethousandth of a milligram of urushiol sap will produce a typical dermatitis.  If poison ivy is burned the urushiol adsorbs to particles of soot and can be inhaled causing lung injury.  Poison ivy leaves have been stored for five years without losing their ability to cause dermatitis.  Even clothing exposed to poison ivy has retained it has  ability to cause dermatitis for one year after washing.

In short

“Leaves of three, let them be.”

Mohr C. The geographical  distribution in North America of poison-ivy and allies.  1925. Am Jour. Bot 12;338-350.

Walter Conrad Muenscher.  Poison Ivy and Poison Sumac. 1930. New York State College of Agriculture Extension Service.  NY State Coll of Agriculture.

Gladman AC. Toxicodendron dermatitis: poson ivy,oak, and sumac. 2006.  Wilderness &Environmental medicine. 17(2): 120-8.

A 64 y.o. woman presents with pain in the L heel.

What is the differential

This patient had cervical carcinoma metastatic to the calcaneus.

Only 1% of cervical cancer metastasizes to bone. The most common site is the spine and it is usually found at a median of 16 months after diagnosis. There are two types of metastases in cervical cancer; lymphatic and hematogenous.  The hematogenous metastases, as in our patient with a distant bone met, are associated with a higher risk of death.

 

Over the last 40 years the number of cases of cervical cancer has decreasedby 50%.  This is directly related to Pap smear testing which detects cervical cancer in the early stages. However cervical cancer still accounts for 10% of all cancers diagnosed in women.  The incidence is highest in African Americans, followed by Asians and Pacific Islanders and then Caucasians.  American Indians and native Alaskans have the lowest incidence of cervical cancer.  80% of the cases are in the developing world; with Malawi and Mozambique being the two countries with the highest incidence.  In addition to screening programs,  cytological examinations must be accurate to decrease the incidence of cervical cancer..  In Mexico for example, screening programs exist but the cytological evaluation is so poor that an estimated 54% of those screened are false negative.

Currently 15% of cervical cancer is diagnosed in women over 65.  This is thought to be due to a long latency period of human papilloma virus. 

 

Corrado G, Santaguida S, Zannoni G, et al. Femur metastasis in carcinoma of the uterine cervix: a rare entity. 2010 Arch Gynecol Obstet 281(5):963-965.

Thanapprapasr D, Nartthanarung A. et al. 2010.  Int J Gynecol Cancer.  April 20(3) 378-8.

Ratanatharathorm V, Powers WE, Steverson D, et al. 1994. Cancer May 73(9) 2372-9.

Li H, Wu X, Cheng X. Advances in diagnosis and treatment of metastatic cervical Ca. J Gynecol Oncol. 2016. July 29(4). E43.