A 37y.o. male comes in with altered mental status

what is wrong?

holoprocecephaly.JPG

Our patient had holoprosencephaly.   This is a developmental abnormality where the embryo fails to develop into two hemispheres. The prosencephalon refers to the forebrain of the embryo and develops in the fifth week of pregnancy along with the face.  Most cases are not compatible with life and result in death in utero  In less severe cases, babies are born with facial deformities affecting the eyes ,nose and upper lip. Some cases result in normal facies with an absent falx and/or corpus callosum. The cause is not known but mutations in the gene encoding the Sonic Hedgehog protein which directs the development of the central nervous system can cause holoprosencephaly. 

holoprosencephaly.JPG

Our patient had abnormal brain development with normal facies.  He had cervical spondylosis with myelopathy and was tetraplegic.  He had congenital hydrocephalus requiring a VP shunt at birth, an occipital encephalocele and seizures.  He was 36 years old and cared for by his mother at home.

holoprosencephaly1.JPG

He came in with a complaint of SOB and tachycardia,  PE protocol was negative and his tachycardia was attributed to cholecystitis with a sludge filled gallbladder.

 

1.       Totori-Donali, P, Ross A. 2006 Brain Malformations . In Totori-Donati, Paolo,  Raybaud C. Pediatric Neuroradiology: Brain, Head, Neck and Spine. 1. Springer pp92-95. ISBN 3-540-41077-5.

2.       Rash BG, Grove EA Patterning the dorsal telencephalon: a role for sonic hedgehog? The Journal of Neuroscience , 27(43): 11595-603. Do1:10.1523/neurosci.3204-07 PMID 17959802.

3.       Muenke, M, Beachy P. june 2000 Genetics of ventral forebrain development and holoprosencephaly. Current Opinon in Genetics & Development 10(3) 262-9.  Doi 10.1016/s0959-437x(00)00084-8.

A 43 y.o. woman with previous back pain comes in with R leg pain.

What is the circular finding on her MRI at L4-5 ? she has no weakness or sensory loss.

synovial cyst.JPG

Our patient had a synovial cyst. Once again Dr. Docherty is correct) A synovial cyst  in the spine is a fluid filled sac resulting from degeneration of a facet joint. They were first described in 1885 by Baker( who also described the one behind the knee).  On MRI they appear well demarcated and are extradural.  They are more common in women.  The cysts are most often found at the L4,5 level because that is the area of maximum mobility. They grow very slowly and often are asymptomatic but they can cause radicular symptoms,  spinal stenosis or even cauda equina.

 synovial cyst

synovial cyst

Surgery is indicated for intractable symptoms.  The complications of surgery include cerebrospinal fluid fistula, discitis, epidural hematoma, seroma, deep vein thrombosis and death.   Our patient is scheduled for surgery because of intractable pain.

 Baker’s cyst behind the knee

Baker’s cyst behind the knee

Khan A, Girardi F. spinal lumbar synovial cysts. Diagnosis and management challenge. Eur Spine J v 15(8) 2006:1176-1182.

Baker W. formation of synovial cysts in connection with joints. St. Batholomews Hospital Reports.  1885;21:177-190.

Baker W. On the formation of synovial cysts in the leg in connection with disease of the knee-joint. 1887. Clin Orthop. 1994;299:2-10.

Lyons M, et al. Surgical evaluation and management of lumbar synovial cysts: the Mayo Clinic experience. J Neurosurg. 2000;93.1(supple):53-57.

A 36 y.o. woman complained of a painful nodule on the L chest 2 weeks after an MVC

What could this be?

Our patient had a sternoclavicular joint infection unrelated to her car accident. She is an IV heroin user. With the increase in IV heroin use this type of infection is increasing in incidence.  This used to be a rare occurrence with 225 cases reported in the past 45 years but recently it has become more common.

 inflammation around the L sternoclavicular joint with distal clavicle osteo.

inflammation around the L sternoclavicular joint with distal clavicle osteo.

The sternoclavicular joint is composed of the medial head of the clavicle, the outer aspect of the manubrium and the cartilage of the first rib with great vessels lying immediately beneath.  The complications of sternoclavicular joint infection include: mediastinitis, osteomyelitis or chest wall abscess.

Of all sternoclavicular joint infections. 17% are found in IV drug users. Diabetics, patients with chronic renal failure  and patients with previous arthritis at the joint are more likely to get infections. It is postulated that  bacteria may enter the sternoclavicular joint from the adjacent valves of the subclavian vein or the joint may be infected by injecting  between the heads of the sternocleidomastoid muscle. En-bloc resectin of the sternoclavicular joint is indicated with a pectoralis muscle flap used to close the defect.

sternoclav joint inf2.JPG

Our patient underwent surgical debridement and is on long term antibiotics.  Her culture grew MSSA.

 

Muesse J, Blackmon S, Wllsworth W, Kim M. Treatment of sternoclavicular joint osteomyelitis with debridement and delayed resection with muscle flap coverage improved outcomes.   volume 2014. Surgery Research and Practice.  Article ID 747315  http://dx.doi.org/10.1155.2014/747315

Murga A, Copeland H, Hargrove R, et al. Treatment for sternoclavicular joint infections: a multi Our patient underwent surgical debridement and is on long term antibiotics. Her culture grew MSSA.

-institutional study. 2017. Journal of Thoracic Disease. Jun; 9(6):1503-1508.  Doi:10.21037/jtd2017.05.76

Ross J, Shamsuddin H. Sternoclavicular septic arthritis: review of 180 cases. 2004. Medicine May;84(3):139-48.

A 28 y.o. with a sore throat was sent from an outside hospital for peritonsillar abscess

What do you notice?

tonsils, mono.JPG

Our patient had tonsillar abscesses caused by mononucleosis.  These are to be differentiated from peritonsillar abscesses because they do not require drainage.

 Infectious mononucleosis is a viral infection caused by Ep[stein-Barr virus which presents with sore throat, lymphadenopathy and atypical lymphocytes in the peripheral blood.  Spontaneous resolution often takes 4-6 weeks.  Tonsillar abscesses may develop when infection from acute tonsillitis spreads into the the peritonsillar space or causes an abscess of Weber’s glands, which are minor salivary gland in the supratonsillar space.  1% of patients with mono develop peritonsillar abscesses. Most pertonsillar abscesses are bacterial often Strep.

 bilateral spontaneous tonsillar rupture in mono

bilateral spontaneous tonsillar rupture in mono

Clinically a peritonsillar abscess presents with trismus, soft palate deviation away from the infected side with uvular deviation and lymphadenopathy. The differential includes lymphoma, carcinoma of the soft palate and minor salivary gland tumors. Quinsy was the original term to describe a peritonsillar abscess.  It comes from the French word esquinacie.  

If peritonsillar abscesses are untreated they can lead to jugular vein thrombosis, cavernous sinus thrombosis, and dissection into the internal carotid artery.

 Our patient did not require drainage but did get antibiotics as prophylaxis for peritonsillar abscess. He was discharged on augmentin after three days.

 

 typical PTA

typical PTA

Fusano C, Chundnofsky C, Vanearbeek P. Bilateral peritonsillar abscess: not your usual sore throat. J Emerg Med. 2009 (29) 45-47.

Wadhera R, Kalra V, Gulati S, Ghai A. Infectious mononucleosis presenting with spontaneous rupture of bilateral quinsies. 2013 Egyptian Journal of Ear , Nose Throat and Allied Sciences. 14;45-46.

Flores C, Matthews M, Caruso D, Foster K, Stroschein M. A rare case of peritonsillar abscess resulting in cervical necrotizing fasciitis. 2017 Otolaryngology Case Resports Volume 5; 1-5.

Mohamad I, Yroko A. Peritonsillar swelling is not always quinsy. 2013 Malaya Fam Physician 8(2): 53-55.

A 71y.o. woman with multiple sclerosis presents 3 wks after a feral cat bit her on the heel with a rash on her leg.

What could these painful lesions be?

 Contrary to popular belief, cat bites often cause severe injuries. Leave it to a cat to ruin even Christmas.

Contrary to popular belief, cat bites often cause severe injuries. Leave it to a cat to ruin even Christmas.

CatbiteinjuryPS4.jpg

 Our patient had cellulitis from a cat bite.

In a Mayo clinic study 1/3 of people presenting with cat bites to the hand  had to be hospitalized with 85% of bites occur on the hand or wrist where they may pierce a joint or tendon sheath. Antibiotics are unable to penetrate a tendon sheath and surgery is required.

In wounds that were cultured in Dr. Talan’s study,  aerobes alone were isolated from 36 percent and anaerobes alone from 1 percent so most were mixed infections. Pasteurella multocida was the most common isolate of cat bites involving 75% of cat bites.  Other common aerobes included strep, staph, moraexella  and Neisseria.  Anaerobes included fusobacterium, bacteroides, porphyromona and prevotella.  Patients were most often treated with a beta lactam antibiotic and a beta lactamase inhibitor. Usually amp/sulbactam.  Clindamycin does not treat Pasteurella.  Pasteurella is  also susceptible to amp, pcn, doxy, sulfa, fluroquinolones and azithromycin.

Our patient was pcn allergic and treated with clinda and ceftriaxone for cellulitis.

 biopsy of cat scratch showed necrotic areas with star shaped abscessed and granulomas

biopsy of cat scratch showed necrotic areas with star shaped abscessed and granulomas

  It was felt that she did not have cat scratch fever since it usually present with swollen lymph nodes near the site of the bite which had occurred on the heel and her adenopathy was in the inguinal region.  Cat scratch fever, caused by Bartonella henselae , occurs one to three weeks after the bite( sometimes as long as two months later) It is associated with chills and joint pains.  Rarely,  encephalitis, endocarditis, optic neuritis or oculoglandular syndrome can occur. Immunocompromised patients are most susceptible to these infections.  Cat scratch disease can be treated with azithromycin, sulfa or Cipro. There is a PCR test available with a sensitivity of 43-76% but the diagnosis can also be made on lymph node biopsy.

 

 

Babovic N, Cayci C, Carlsen B. Cat bite infections of the hand: assessment of morbidity and predictors of severe infection. J Hand Surg Am 2014 Feb 39(2) 286-290. DOI 10.1016/JHSA2013.11.003

Talan D, Citron D, Frederick M, Abrahamian , D, Moran G, Goldstein E. Bacteriologic analysis of infected dog and cat bites. NEJM 1999, Jan 14.

Goldstein E, Citron D, Hudspeth M, Gerardo S, Merriam C. Trovafloxacin compared with levofloxacin, ofloxacin, Cipro, azithro and clarithro againt unusual aerobic and anaerobic human and animal bite-wound pathogens. J Antimicrob Chemother 1998;41: 391-396.

Rolain J, Brouqui P, Koehler J, Maguina C, et al. Recommendations for treatment of human infections cased by bartonella species. 2004 Antimicrobial Agents and chemotherapy 48(6) 1921-1933.

A 29 y.o. with high risk sexual behavior comes in with two rashes

petechiae.JPG
 could these rashes by related?

could these rashes by related?

Our patient had leukocytoclastic vasculitis which is a hypersensitivity vasculitis. This is a small vessel vasculitis and leukocytoclasis refers to the vascular damage caused by infiltrating neutrophils. It may be caused by by underlying collagen vascular disease, medications, malignancy or infections. In our case the patient had a previous group B strep infection as well as herpes. Both have been shown to cause leukocytoclastic vasculitis.

 small vessel vasculitis with neutrophils lining vessels

small vessel vasculitis with neutrophils lining vessels

While leukocytoclastic vasculitis often involves the skin; it can also involve joints, the GI tract and the kidneys. Pathologically, autoantibodies including ANCA and tumor necrosis factor attract neutrophils. One type of leukocytoclastic vasculitis involves IgA deposition in the skin and joints. This is called Henoch Schonlein vasculitis. It is common in children under age 10 but also occurs in adults.

 henoch schonlein vasculitis ofter occurs on lower extremities

henoch schonlein vasculitis ofter occurs on lower extremities

The skin rash often lasts for weeks and if it involves only skin no treatment is needed. In cases of severe joint pain colchicine can be used because it has an antineutrophil effect. Steroids and biologic immune suppression are used in serious cases of organ involvement. Our patient was treated for herpes and his rash resolved although he still had joint aches at two weeks post treatment.

Lotti T, Ghersetich I, Comachi C, Jorizzo J. Cutaneous small vessel vasculitis. J Am Acad Derm. 1998. Nov 39(5) 667-87.

Jeannett J, Falk R. Small vessel vasculitis NEJM 1997 Nov 20 337 (21)1512-23.

A 45 y.o. transexual female with HIV and CD4 count of <5 presents with a rash

 What is the differential diagnosis?

What is the differential diagnosis?

Our patient had photodermatitis.  This is a form of  allergic contact dermatitis is which the allergen is activated by sunlight.

Causes of photodermatitis

Medications

                Sulfa, psoralens, tetracyclines, benzoylperoxide, retinoids, amiodarone, ibuprofen , naprosyn

                Lemon oil, PABA (found in sunscreens). Hexachlorophene( found in antibacterial soaps)

Plant related photodermatitis

Citrus fruits including limes and grapefruit of the Rutaceae family , psoralens found in celery, carrot or parsley( the family Apiaceae)

Vitamin deficiency

Pellagra- lack of B3 either by lack of the vitamin in the diet or  inability to use the niacin consumed.

 pellegra in an alcoholic

pellegra in an alcoholic

Pellegra is common in people who eat a corn based diet when the corn is not processed with alkali which makes niacin available for absorption.The name comes from Pelle agra, Italian for rough skin.  It was first described in 1735 in Spain by Gaspar Casal.  Pellegra  was widespread in the American south in the 1900s with more than 3 million people affected. Dr. Joseph Goldberger traced the disease to corn by feeding prisoners cereal diets until they developed the classic skin lesions.  Now corn is processed with alkali to make the niacin bioavailable.

Pellegra is often seen in alcoholics,  end stage HIV, malabsorption and carcinoid syndrome (due to excess turnover of tryptophan, precursor of niacin, to serotonin)

Our patient was treated with nicotinomide and all her herbal supplements were stopped.  She had also been on high doses of grapefruit powder and shitake mushroom powder.  Her rash resolved after a week in the hospital. Below is a chart of the timeline of vitamin deficiencies.

vit.JPG

Rodriguez E. Valbuena M, Rey M Porras de Quintana L. 2006 Causal agents of photoalleric contact dermatitis diagnosed in the national institute of dermatology of Colombia. Photodermatol Photoimmunol Photomed 22(4) 189-192.

Ngan V. 2003 Pellegra DermNet New Zealand, April 9 2017.

Hegyi J, Schwartz, R, Hegyi V. Pellegra: Dermatitis, dementia, and diarrhea. International Journal of Dermatology 43(1) 1-5. doi 10.111/j 1365-4632. 2004.01959 PMID 14693013.

Savvvoula S, Pellegra: A non-eradicated old disease, Clin Pract 2014 March 27, 4(1). 637

 

A 28 y.o. woman comes in with LUQ pain

what do you notice on CT?

polysplenia.JPG

Our patient had heterotaxy syndrome with polysplenia. The word is derived from hetero meaning “different” and taxy meaning “arrangement”.Heterotaxy  syndrome is an embryologic abnormality where organs and vessels  do not follow the standard developmental pattern. It occurs in 1.4 per 10,000 infants. This is also known as situs ambiguous and is categorized based on the number of spleens.  Asplenic patients have a higher incidence of congenital heart disease which leads to earlier detection of the syndrome.

 This is an US of a 35 wk fetus showing the azygous to the R of the aorta in heterotaxy polysplenia syndrome; this baby had malrotation as well

This is an US of a 35 wk fetus showing the azygous to the R of the aorta in heterotaxy polysplenia syndrome; this baby had malrotation as well

Even though there are many spleens in some patients, the spleens may not be functional and these patients are at risk for sepsis with encapsulated organisms.

heterotaxy with liver on the L.JPG

Applegate KE, Goske MJ, Pierce G, Murphy D. Situs revisited. Imaging of the heterotaxy syndrome. Radiographics. 1999, 19:837-852

Peoples WM, Moller JH, Edwars JE. Polysplenia: A review of 146 cases. Pediatr Cardiol. 1983;4:129-137.

Lin AE, Ticho BS, Houde K, et al. Heterotaxy. Associated conditions and hospital-based prevalence in newborns. Genet Med 2000;2:157-172.

Cupers S, Va  Linthout C, Desprechins B, et al. Heterotaxy syndrome with intestinal malrotation, polysplenia and azygos continuity. Clin Pract 2018. Jan8;8(1), 1004.

A 24 y.o. social worker with a hx of crohn's since 2010 comes in with RLQ pain

What is the metallic object in her pelvis?

capsule endos2.JPG

Our patient had an endoscopy capsule camera lodged in the terminal ileum.  Capsules are traditionally used to detect sites of GI bleeding, diagnose cancer in the small bowel, diagnose celiac disease and screening for polyps.  In the case of our patient she had a stricture of the terminal ileum and the camera lodged there.

capsule endos.JPG

The camera captures two pictures per second which are sent by a radio transmitter to a recorder that the patient wears on a belt. A number of electrodes are attached to the patients abdomen similar to an EKG.  After 8 hours the batteries are depleted  and the 55,000 images  are “glued" into a video which is then downloaded on to a computer. The first capsule, for the small bowel, was FDA approved in 2001. There are two other type of capsules; esophageal and colonic. The indications for the esophageal capsules are Barrett’s esophagus and esophageal varices. These capsules have not become popular because of the prevalence of UGI endoscopy so 90% of the capsules are small bowel type.

Capsule endoscopy of the small bowel  is more sensitive than small-bowel follow through xrays which is still the only way to image the small bowel.   In patients with possible strictures a dissolvable “patency capsule” can be ingested first to see if it will pass. The pill camera contains metallic batteries and can cause a perforation so removal is recommended. It can also interfere with pacemakers.

 1-4% of crohn's patients retain the endoscopy capsule

1-4% of crohn's patients retain the endoscopy capsule

Our patient underwent ileocolic resection with removal of the capsule camera because of recurrent pain,  She has done well post op

Capsule endoscopy costs  $919-$2,379. This is compared to $1,500 to $10,000 for upper GI endoscopy.

Mergener K, Update on the use of capsule endoscopy. 2008 Gastroenterol Hepatol. Feb;4(2): 107-110.

Marmo R, Rotondano G, Rondonotti C, et al. Casule endoscopy vs other diagnostic procedures in diagnosing obscure gastrointestinal bleeding: a cost-effectiveness study. Eur J Gastroenterol Hepatol. 2007;19:535-542.

Flemming J, Cameron S. Small bowel capsule endoscopy: indications, results, and clinical benefit in a university environment.  2018. Medicine Vol 97(14). P e0148.

A 27 y.o. woman presents with HA and difficulty letting go of objects when she grips them.

Her MRI is shown below and shows an epidural fluid collection anterior to spinal cord . 

 What is wrong?

What is wrong?

Our patient had a spontaneous CSF leak.  She was leaking CSF at T12 causing decreased volume in the head and intracranial hypotension.  The key to the diagnosis was that she had an orthostatic headache; much worse when she was upright.  Compression of the cord probably accounted for her arm findings. As the CSF continues to leak: the lower part of the brain descends causing tension on cranial nerves. This can result in blurred or double vision, taste distortion, facial weakness and balance problems.

csf leak cranial n.JPG

Spontaneous dural leak was first described by a German neurologist, Georg Schallenbrand  in 1938 when he discovered negative CSF pressure in patients with spinal taps.  It occurs in 5/100,000 people and is more common in women. 94% of individuals with a spontaneous CSF leak first present to an ED for evaluation and are misdiagnosed as migraines, meningitis, chiari malformations or psychiatric problems.

Spontaneous leaks are often familial and can be associated with Marfan’s, aortic aneursyms  or other connective tissue diseases.  It is estimated that 2/3 are associated with connective tissue disease involving the dura. When the leak is small, the headache produced is called a “second half of the day headache”  because it often appears in the afternoon.

 fluorescein injected in the lumbar area can show where the dural leak is after septoplasty

fluorescein injected in the lumbar area can show where the dural leak is after septoplasty

CSF leaks are also associated with trauma: lumbar punctures, brain surgery and ENT surgery, with rhinoplasty being a frequent culprit. They can occur in weight lifters because of increased pressure.  They can be difficult to diagnose with CT and MRI often being negative.  CT myelogram or T2 weighted MR myelography are often used to make the diagnosis.  Of course,  if fluid is leaking from the nose or ear, a beta-2-tranferrin assay can be performed to make the diagnosis.

Treatment involves sealing the leak either with a blood patch or fibrin glue. Our patient  was a weight lifter although it is not clear if this caused her CSF leak.  A blood patch was done and she became asymptomatic. 

csf leak ana.JPG

References

Leep–Hunderfund,  A, Mokri B, Second half of the day headache as a manifestation of spontaneous CSF leak. 2011. Journal of Neurology 259(2): 306-10.

Schievink W, Maya M, Moser F, Tourge J, Torbati S. Frequency of spontaneous intracranial hypotension in the ED. 2007. Journal of Headache and Pain 8(6) 325-328.

Schievink W, Deline C. Headache secondary to intracranial hypotension 2014. Current Pain and Headache Reports. 18(457):1-9.

 

And my favorite

                SPINAL CSF LEAK FOUNDATION/ BECAUSE YOUR DURA MATERS

A 54 y.o. woman with seronegative arthritis presents with eye pain.

What is in the differential for her R eye pain and blurry vision?

 Hint: she has no cell or flare, no iritis, no uveitis and no retinal abnormalities

Hint: she has no cell or flare, no iritis, no uveitis and no retinal abnormalities

Our patient had enlargement of the lacrimal gland thought to be ANCA negative Wegener’s granulomatosis ( now known as granulomatosis with polyangiitis).  She had four episodes of swelling of the lacrimal gland since 2015 all of which responded to steroids. The ct showed enlargement of the gland as well as stranding surrounding it.

 anatomy of the lacrimal system

anatomy of the lacrimal system

The diagnosis of Wegener’s is often made by biopsies of the upper respiratory tract but the yield of these biopsies for making the diagnosis is only 50%.   It often involves the lungs or kidneys and biopsies of those sites more often reveal the diagnosis.  Since 1982, when  ANCAs (anti-neutrophil cytoplasmic antibodies ) were first described this blood test has been used to diagnose Wegener’s.  The C-ANCA or cytoplasmic antibody directed against serine proteinase 3 is most specific. It is thought that tumor necrosis factor causes serine proteinase to be expressed on the surface of neutrophils and these are the targets for ANCA. When serine proteinase combines with ANCA oxygen radicals are created which damage blood vessels.

Eye manifestations occur in about 50% of patients with the disease.  Focal vasculitis will produce  conjunctivitis, episcleritis, uveitis and granulomatous vasculitis of the retina and optic nerve as well as lacrimal gland enlargement. Eye and upper respiratory tract disease, i.e. sinus disease, may occur years before lung and kidney involvement.

 lacrimal gland involvement in Wegener's

lacrimal gland involvement in Wegener's

DIFFERENTIAL OF LACRIMAL GLAND ENLARGEMENT

ACUTE- bacterial infection, mumps, Epstein barr, gonorrhea and staph, tuberculosis

CHRONIC- Sjogrens,  thyroid disease, orbital pseudotumor, Wegener’s, Behcet’s,sickle cell disease, amyloid

TUMORS- adenocystic CA, squamous CA, lymphoma

 

Our patient was treated with antibiotics in the ED which had no effect and was switched to steroids with resolution of her swelling.

Khu J, Freedman. Lacrimal gland enlargement as an early clinical or radiological sign in thyroid orbitopathy. American Journal of Ophthalmology Case Reports  2017. https://doi.org/10.1016/j.ajoc. 2016.10.005

Lanza JT, Ku Y, Lucente RE. Har-El. Wegener’s granulomatosis of the orbit: lacrimal gland involvement as a major sign. Am J Otolaryngol 1995 16(2):119-22.

Carrington CB, Liebow A. Limited forms of angiitis and granulomatosis of tWegener’s type. 1966. Amer J med 41:491-527.

Singer O, Mccune W. Update on maintaenance therapy for granulomatosis with poyangiitis and microscopic polyangiitis.  2017. Current Opinion in Rheumatology 29(3) 348-53.

 

A 31 y.o. woman presents with abdominal pain and a positive RPR at 1:16

What do you think she has?

 Arrow points to the uterus.

Arrow points to the uterus.

 View from lower in the pelvis showing the bladder.

View from lower in the pelvis showing the bladder.

Our patient had a mature teratoma with a tooth visible on the CT scan. An episode of torsion caused her pain.  Teratomas result form a germ cell retained in the ovarian tissue. The cell is totipotential and can give rise to hair bone, fat, neural tissue or even thyroid tissue. The teratoma is made up of ectoderm and mesoderm usually but when predominantly ectoderm it is called a dermoid.

Types of teratomas include:  mature (which is the predominant type), immature,  specialized teratomas i.e. thyroid tissue (struma ovarii), or fetiform teratomas. A fetiform teratoma appears to be a developing fetus without developed organs or axial skeleton.

Teratomas can be diagnosed on US when specific patterns occur but are best diagnosed on CT scan where fat can be identified within the tumor. An example of US being used to diagnose a teratoma is the dot and dash sign shown below where the ends of hair strands appear to be in dots and dashes. 

 Dot and dash sign of a teratoma.

Dot and dash sign of a teratoma.

The patient also had a positive test for syphilis.  She had numerous ED visits and several positive tests over four years but had never been treated.  Her RPR was positive  at 1:16.  As a review:

PRIMARY SYPHILIS- presents with a chancre and is present 3-90 days after exposure (average is 21 days).

SECONDARY SYPHILIS- appears 4-10 weeks after exposure and has many presentations including a rash on the palms and soles, sore throat, optic nerve involvement, liver disease, fever, and sore throat.  Symptoms resolve without treatment in 3-6 wks.

LATENT SYPHILIS- presents without symptoms and is defined as a positive serology. It is divided into early (< one year after secondary syphilis) and late (more than one year after secondary syphilis).  Our patient had latent syphilis. 

TERTIARY SYPHILIS- occurs 3-15 years after the initial infection.  One third of people develop tertiary disease without treatment. It can affect the heart (aortitis), brain (tabes dorsalis with poor balance and lightning pains in the extremities) or skin (gummas).

Our patient incidentally was found to have latent syphilis and was treated with penicillin.  An LP was done to rule out tertiary syphilis and was negative.  She had surgery and recovered without incident after her teratoma removal.

Useless trivia: The word Syphilis was coined in 1530  by an Italian poet who wrote a story about a shepherd who had the disease.  The shepherd was named Syphilis and his name came to represent the disease.

 Dark field microscopy of syphilis

Dark field microscopy of syphilis

Kent ME, Romanelli F. Reexaming syphilis: an update on epidemiology, clinical manifestations and management.2008  Annals of Pharmacotherapy 42(20 226-36.

Vinals-Iglesias H, Chimenos-Kustner E. the reappearance of a forgotten disease in the oral cavity: syphilis.2009   Medicina oral, patologia oral y cirugia buccal 14 (9): e416-20.

Dlewski J, Duong M. the rash of secondary syphilis. Canadian Medical Association Journal . 2007. 176(1):33-35.

Sahin H, Abdullazade S, Sanci M. Mature cystic teratoma of the ovary: a cutting edge overview on imaging  features.  Insights Imaging 2017 Apr;8(2):227-241.

Weiss J, Burgess J, Kaplan K. Fetiform teratoma(homunculus) Arch Pathol Lab Med. 2006;130:1552-1556.

A 44 y.o. male presents with a sore throat

What do you notice on imaging?

 hint: it's near the epiglottis

hint: it's near the epiglottis

Our patient had a thyroglossal duct cyst. 65% of the time they are a midline neck mass below the level of the hyoid bone and can be seen moving with swallowing.  Our patient had one at the base of the tongue;  unusual in that it was not below the hyoid. .  The persistent duct can promote oral secretions and the cysts can become infected.  The tract can lie dormant for decades and present in later life. The cyst can rupture creating a draining sinus known as a thyroglossal fistula. Another complication is malignancy; with remnants of thyroid tissue in the cyst becoming malignant. Carcinoma occurs in 1-2% of thyroglossal duct cysts.

 cysts can occur anywhere along the thyroglossal duct tract

cysts can occur anywhere along the thyroglossal duct tract

During embryonic development, the thyroid gland is formed at the base of the tongue  and moves caudally down into the neck through a canal known as the thyroglossal duct.  The duct normally disappears after the thyroid is formed but can leave behind remnants of the duct which cause cysts.

Since most of the cysts are below the hyoid; the classic treatment is resection of the center of the hyoid bone and removal of 1/8 inch of core of the tongue superior to the hyoid.  The hyoid is then reconnected. This is called the Sistrunk procedure and is 95% effect at removing the cyst.

 more common location of a thyroglossal duct cyst

more common location of a thyroglossal duct cyst

Our patient was treated with unasyn and steroids. He improved and was discharged for outpt direct laryngoscopy and biopsy.  He was quite upset that when he was initially seen in ent clinic the CT did not show a cyst and then it recurred several weeks later.

McNicoll MP, Hawkins DB, England K, Penny R, Maceri DR. Papillary carcinoma arising in a thyroglossal duct cyst. 1988 Otolaryngology-Head and Neck Surgery 99(1) 50-54.

Deaver MJ, Silman EF, Lotfipous S. Infected thyroglossal duct cyst. 2009 Western Journal of Emergency Medicine 10(3):205.

Sistrunk WE The surgical treatment of cyst of the thyroglossal tract. 2016. Annals of Surgery 71(2):121-122.

Maddalozzo J, Venkatesan TK, Gupta P. Complications associated with the Sistrunk procedure. 2001 The Laryngoscope 111(1):119-123.

 

A 73 y.o. woman with a hx of breast ca and osteoporosis presents with jaw pain.

 hint: she has had two teeth removed but her jaw won't heal.

hint: she has had two teeth removed but her jaw won't heal.

Our patient had phossy jaw. This is osteonecrosis of the jaw associated with phosphorus poisoning

 non healing fistula from dead bone.

non healing fistula from dead bone.

. It was first described in people who worked in match factories in the 1800s. Workers dipped treated wood into a white phosphorus solution, dried the sticks and cut them into matches. They worked 12-14 hours a day and the phosphorus was so pervasive in the factories that the walls of the factories had a blue green glow and workers’ clothes glowed in the dark.

 Match factory worker with phossy jaw

Match factory worker with phossy jaw

Today white phosphorus is not used in matches but phossy jaw persists in some patients who are given bisphosphonates for osteroporosis.  This was the case in our patient; she developed jaw pain and had teeth removed but the jaw never healed.  A sequestrum of dead bone became infected and developed a fistula into the mouth. The same condition can result from bisphosphonates used to treat cancer. 6-11% of patients with multiple myeloma who received bisphosphonate for metastatic bone lesions developed osteonecrosis of the jaw. The percentage is even higher in those with recent dental extractions.

Bisphophonate-related osteronecrosis of the jaw (BRONJ)is thought to be caused by trauma to the teeth that have limited capacity for healing because of the effects of the bisphosphonate therapy. Bisphosphonates bind to osteooclasts and decrease bone turnover. Other non resorptive treatments like denosumab or antiangiogenic treatments can cause jaw necrosis as well.

USELESS TRIVIA

In 1670 phosphorus was discovered by an alchemist who was boiling his own urine in search of gold.  After allowing 50 buckets of urine to putrify,  Hennig Brandt  boiled it and isolated  a white waxy substance which glowed in the dark. He named it phosphorus which in Greek means “light bearer”.

Since phosphorus is so flammable, the slightest heat from friction, as in striking a match, can cause it to burst into flame.

 

Hughes JP, Baron R, Buckland DH, et al. Phosphorus necrosis of the jaw: a present-day study with clinical and biochemical studies. British journal of industrial medicine. 19.83-99.

Hellstein JW, Marek C. Bis-phossy jaw, phossy jaw and the 21st century: Bisphosphate-associated complications of the jaws. Journal of oral and maxillofacial surgery. 62(12):1563-5.

Phosphorus poisoning in the match industry in the United States. JAMA 2010.  303(22)2303.

A 59 y.o. woman comes in the L chest pain

What do you notice on her CXR?

gastric band 2.JPG

Our patient has a lapband.  This device was used in the past for weight loss surgery but is no longer favored because of band migration and erosion. This is an important part of the history to obtain because of the complications associated with weight loss surgery.

A brief synopsis of the evolution of weight loss surgery follows. These surgeries are intended for those with a BMI of 30 or greater.

 the arrow points to the bypassed duodenum of the Roux-en-Y

the arrow points to the bypassed duodenum of the Roux-en-Y

1.       Roux-en-Y gastric bypass involves a combination of stomach reduction and connection of the small stomach to a portion of the intestine.  Food bypasses the stomach and part of the small bowel.  This is good for weight loss both from early satiety and dumping but if the pt presents with:

Vomiting-- be aware the anastomosis with the stomach can stenose causing the equivalent of an esophageal food impaction. Internal hernias, and edema of the anastomosis can result as well as anastomotic ulcers.

An acute abdomen—consider an anastomotic leak

RUQ pain—consider gallstones; 15% of Roux-en-Y patients get them

2.        Lap band- is much less invasive but done less often now because of migration of the band and erosion of the esophagus. This occurs in about 2% of patients. These patients present with vomiting.

3.       Gastric sleeve- removes 80% of the stomach.  Consider a ruptured staple line if the pt presents with a GI bleed.

4.       Duodenal switch- leaves the pyloris intact and is associated with fewer anastomotic ulcers. It is more effective at curing diabetes.  These patients are more likely to develop malnutrition and thiamine/vitamin deficiencies.

5.       Intragastric balloons- are having a resurgence but the Orbera Intragastric balloon and ReShape integrated dual balloon system  have recently been implicated in 12 deaths including perforation of the stomach, perforation of the esophagus , pancreatitis and spontaneous hyperinflation of the balloon.

 this procedure leads to weight loss with the possibility of malnutrition especially thiamine deficiency.

this procedure leads to weight loss with the possibility of malnutrition especially thiamine deficiency.

Our patient had esophagitis with thickening of the distal esophagus found on CT. The CT was done to ro rib fractures since she would be predisposed to malnutrition..  She was referred back to her bariatric surgeon. Remember, any patient with a gastric bypass can present with Wernicke’s.

 

DeMaria EJ, Pate V, Warthen M, Winegar DA Baseline data from American Society fo Metabolic and Bariatric surgery-designated Barietric Surgery Centers of Excellence using the Bariatric Outcomes Longitudinal Database Surg Obes Relat Dis. 2010;6:347.

Nelson DW, Blair KS, Martin MJ. Analysis of obesity-related outcomes and bariatric failure rates with the duodenal switch vs gastric bypass for morbid obesity. Arc Surg 2012;147:847.

Le Roux CW, Welbourn R, Weling M, et al. Gut hormones as mediators of appetite and weight loss after Roux-en-Y gastric bypass. Ann Surg 2007;246:780.

 

 

A 70 y.o. woman presents with chest pain and a positive troponin.

what do you see on her xray?

 What is the cause of her pain?

What is the cause of her pain?

Our patient had a large paraesophageal hernia and no coronary artery disease on cath.  Hiatal hernias are categorized as being either paraesophageal or sliding.  In a paraesophageal hernia the  GE junction is in place at the level of the diaphragm but part of the stomach pushes into the chest beside the esophagus. In a sliding hiatal hernia the GE junction protrudes into the chest.  Only 5% of hiatal hernias are paraesophageal.

 EKG of our patient unchanged from 2014

EKG of our patient unchanged from 2014

Patients with paraesophageal hernias often have chronic symptoms  of early satiety because a meal distends the thoracic stomach and causes chest pain.  The hernia may also cause gastric erosions and ulcer.   Occasionally acute ischemia related to torsion can cause intense pain, perforation and death. Skinner and Belsey reported a series of 31 patients with paraesophageal hernias that were treated medically because of minimal symptoms and six patients died of complications including strangulation, perforation, exsanguination or acute dilation of the intrathoracic stomach.  For this reason, generally, a surgical repair is warranted.

Sliding hernias where the GE junction slides into the chest with the stomach are more likely to cause  esophagitis or stricture associated with gastroesophageal reflux. 

 a paraesophageal hernia on CT

a paraesophageal hernia on CT

Esophagoscopy confirmed that the hernia was paraesophageal with the GE junction in place.  Erosions were seen in the thoracic stomach and the pt was treated with an NG to decrease distention and a proton pump inhibitor.  Her symptoms improved and she was scheduled for surgery. Her trop peaked at 0.09 presumably from demand ischemia since her cath was normal.

Shafii A, Agle S, Zervos E. Perforated gastric corpus in a strangulated paraesophageal hernia: A case report.2009. Journal of Medical Case Reports, 3(6507). DOI:10.1186/1752-1947-3-6507.

Dunn D, Quick G. Incarcerated paraexophageal hernia. American Journal of Emergency Medicine . 1990. Vol 8(1). 36-39.

Pearson F, Cooper JD, Ilves R et al Massive hiatal hernia with incarceration: A report of 53 cases. Ann Thorac Surg 1983;35:45-51.

Skinner DB, Belsey R. Surgical management of esophageal reflux with hiatus hernia: Long-term results with 1,030 patients. J Thorac Cardiovasc Surg 1967;53:33-54.

 

A 30 y.o. woman presents to the the psychiatric area of the ED with refusal to eat. In her evaluation for altered mental status she gets a head ct.

 What do you notice?

What do you notice?

Our patient had Proteus syndrome.  It is a rare somatic mutation that results in asymmetric  overgrowth of bone and soft tissue.It was first described in 1979 and four years later was given the name "Proteus syndrome" after the Greek god Proteus who was able to assume multiple forms. It is characterized by macroglossia, hemifacial overgrowth and hyperpigmentation. 

 aymmetric growth of the legs

aymmetric growth of the legs

Genetic sequencing has localized the gene responsible to AKT1.  This is not in a germ cell and so can occur later in life.  If it develops later in life the phenotype will be less severe.

 ear enlargement from Proteus syndrome

ear enlargement from Proteus syndrome

Joseph Merrich, the man studied in the 19th century by Treves who was thought to have neurofibromatosis is now thought to have had Proteus syndrome. Our patient had such severe bony overgrowth of the jaw she could barely open her mouth.  She was scheduled for reconstructive surgery.

Lindhurst MJ, Sapp JC, Teer JK, et al. Mosaic activating mutation in AKT1 associated with the Proteus syndrome. 2011 NEJM Aug 18, 365(7).

Cohen MM Jr, Proteus syndrome; an update. 2005 Am J Med Gen Seminars in Med. Aug 115 137C(1) 38-52.

Lacerda LS, Alves UD, Zanier et al. . 2014 Differential diagnosis of overgrowth syndromes; the most important clinical and radiologic manifestations.  Radiol Reg Prac :947451

A 57 y.o. male presents with abdominal distention

What do you notice on the CT of the abdomen?

 hint the wall of the bowel is abnormal

hint the wall of the bowel is abnormal

Our patient has pneumatosis cystoides intestinalis or gas filled cysts in the bowel wall. This was first described in 1730 by DuVernoy during a cadaver dissection.  Although the first thing we are taught to rule out is ischemic bowel;  the condition is often benign.

It is estimated that 15% of pneumatosis is primary; in 85% of cases there appear to be secondary causes. The causes of pneumatosis include:

traumatic and mechanical- this includes pyloric stenosis where the bowel contracts against an obstruction or bowel surgery which can disrupt the bowel wall layers.

Inflammatory and autoimmue- this includes crohn's disease, NEC, and conditions where steroids are used for treatment. Steroids are thought to cause atrophy of the mucosa and lead to spaces which can fill with gas

Infectious- c diff, HIV and CMV have all been shown to cause pneumatosis

Transplantation probably because of immune suppression, and neoplasm are also associated with pneumatosis. 

 in pneumatosis the ruptured cysts can cause free intraperitoneal air and yet the bowel wall is intact as shown above.

in pneumatosis the ruptured cysts can cause free intraperitoneal air and yet the bowel wall is intact as shown above.

Often the patient is treated with flagyl but oxygen therapy can be used. it is thought that 350 mm of oxygen increased the oxygen in the cyst to blood diffusion gradient and this will lead to absorption of the cysts.  The cysts themselves contain nitrogen and carbon monoxide.

 

 submucosal cysts

submucosal cysts

Pneumatosis is called the surgeons dilemma because no one wants to perform unnecessary surgery, yet missing ischemic bowel can be devastating.  The two things to look for are lactic acidosis and severe abd pain.  Our patient had neither and he was managed conservatively with antibiotics.  The pneumatosis resolved.

  1.  Brauman C, Menenahosc C, Jacobi C . Pneumatosis intestinalis- a pitfall for surgeons. 2005 Scand Journal of Surg 94(10 47-50.
  2. Zulke C, Ulbrich S, Graeb C, Hahn J. Pneumatosis cystoides intestinalis following allogenic transplantation. 2002;29(9) 795-798.

A 31 y.o male presents with abd pain

What do see on the CT?

Screen Shot 2018-06-11 at 8.56.12 AM.png

Our pt had an intussusception of the small bowel into the colon and the right colon intussuscepted into itself.  At surgery , it was not able to be reduced and a right hemicolectomy was performed. A  3 cm inflammatory polyp was found to be the lead point of the intussusception.

 intussuscepted bowel

intussuscepted bowel

The cause of intussusception is unknown in children.Iit can present with currant jelly stools and can often be reduced with an enema. Risk factors in children include  cystic fibrosis and intestinal polyps. The usual age of intussusception in children is 6-18 months.   In adults,  there is often a lead point is present and the most common causes are endometriosis, bowel adhesions and tumors.  Meckel’s diverticulum, duplication cysts and hyperplasia of the Peyer’s patches can also cause intussusception.

 In the most frequent type of intussusception, the ileum enters the cecum.The part that is contained within bowel is the proximal part of the bowel since peristalsis carried it forward.  The trapped bowel may become ischemia necessitating surgical intervention.

 Intussusception caused by a&nbsp; J tube

Intussusception caused by a  J tube

Our patient had a second bowel obstruction after surgery which was managed conservatively and thought to be secondary to an adhesion.  He was discharged after resolution of his symptoms and is doing well. The cause of his polyp was felt to be heavy NSAID use.

Marsicovetere T, Ivatury, et al. Intestinal intussusception: etiology, diagnosis and treatment. 2017 Clinics in colon and rectal surgery 30(1):30-39

Gluckman S, Karpelowsky J, et al. Management for intussusception in children. The Cochrone Database of Systematic Reviews.  6:CD006476.

Gayer G, Zissin R, Apter S, PapaM, Hertz M. Pictorial review: adult intussusception—a CT diagnosis. 2002  Br J Radiol. 75(890): 185-90

A 31 y.o. graduate student comes in with abdominal bloating. The CT results are shown

What is the differential for new onset ascites with peritoneal studding?

peritonitis.JPG

Our patient had peritoneal tuberculosis.  He was from India with no previous history of Tbc. Tuberculous peritonitis  is increasing in prevalence.  It is common in patients with immunocompromised states, chronic kidney disease, or cirrhosis of the liver  It is most often caused by spread from pulmonary foci but can be caused by direct invasion of the  bacillus through the bowel wall.

 

 peritoneal thickening from adenocarcinoma

peritoneal thickening from adenocarcinoma

The differential for ascites with peritoneal studding includes:

METASTATIC NEOPLASMS

-Carcinomas of the GI tract and ovary( stomach,colon, appendix, gallbladder and pancreas)

-pseudomyxoma peritonei-is a more benign condition where thick gelantinous materil covers the surface of the peritoneal cavity. It is thought to be a low grade appendiceal tumor which can be debulked since the tumor does not invade abdominal organs.

- Lymphoma generally associated with herpes virus:  human herpes virus 8 (also associated with Kaposi’s) and Epstein Barr virus

-sarcoma

INFECTIOUS AND INFLAMMATORY LESIONS

-granulomatous peritonitis includes tuberculosis,Histoplasmosis, or pneumocystis.  It also  includes sarcoid, and foreign material such as talc or barium. 

-Sclerosing encapsulating peritonitis may occur in a patient on peritoneal dialysis; the cause is not known.

MISCELLANEOUS TUMORS AND TUMORLIKE LESIONS

-Endometriosis occurs in 10% of women of childbearing age

-Melanosis can be associated with cystic teratomas

- splenosis is heterotopic splenic tissue often occurring after trauma to the spleen

 

 peritoneal pseudomyxoma

peritoneal pseudomyxoma

35 cases of bovine tuberculosis were reported in New York City  from 2001-4 and linked to fresh cheese (queso fresco) brought to NYC from Mexico where 17% of cattle being slaughtered are positive for M. bovis.  Pasteurized milk is free of the disease. Our patient had a positive omental biopsy showing tuberculosis.  His ascitic fluid  and pleural fluid cultures were negative.  He was treated with rifampin, INH, pyrazinamide and ethambutol.

 difficult to see red bacilli in the peritoneal biopsy

difficult to see red bacilli in the peritoneal biopsy

Levy A, Shaw J, Sobin L.  Secondary tumors and tumorlike lesions of the peritoneal cavity: imaging features with pathologic correlation. Radiographics. 2009. 29:347-373.

Srivastava U, Almusa O, Tung K, Heller M. Tuberculous peritonitis. Radiol Case Rep 2014;9(3):971.

MMWR Human Tuberculosis Casued by Mycobacterium bovis, New York City, 2001-2004 June 2005 54(24):605-608.