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  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.

 

A 66 y.o. woman presents with nasal congestion

 What is wrong?

What is wrong?

Our patient had a large mass in the nasal cavity.  She had a hx of squamous cancer ten years pta centered over the R forehead and involving the frontal sinus which had been resected. The mass extended into the anterior cranial fossa.  

Nasal masses were first described by Hippocrates in 460 BC.  He is considered the "Father of Rhinology"  because of his description of nasal polyps.  

CAUSES OF NASAL MASSES

 

BENIGN

The most common benign nasal masses are polyps ( often related to asa allergy) and hemangiomas

 encephalocele

encephalocele

CONGENITAL Congenital lesions occur in the first 12 weeks of development  when neural crest cells are migrating.  Lesions  in children include dermoids, hemangiomas, gliomas , encephaloceles, and rhabdomyosarcomas.

VASCULITIS-

Wegener’s is a granuomatosis inflammation  with necrosis occurring in  the respiratory tract, lung and kidney. Sinusitis is the presenting finding in  1/2 to 2/3 of patients.

 Rhinoscleroma

Rhinoscleroma

INFECTIOUS

Rhinoscleroma is  a chronic bacterial infection caused by Klebsiella rhinocleromatis.

Rhinosporidiosis is a chronic granulomatous disease  endemic in India, Sri Lanka and parts of Africa.  The responsible agent is an a protozoan belonging to a group of fish parasites  and found in water.It stains with fungal stains and  has endospores so for many years it was thought to be a fungus. It causes bony erosion. 

Leprosy- Nasal involvement is universal and occurs early in the disease. The disease is caused by infection with Mycobacterium Leprae.

Tuberculosis, leishmaniasis, blastomycosis, and syphilis have all been reported to cause destructive lesions of the  central face.

 leprosy

leprosy

MALIGNANT:

 Squamous cell cancer is the most common malignancy of the nose accounting for over 90% of tumors.

Lethal midline granuloma ( now known to be an aggressive lymphoma)  is less common. 

Giant cell tumors of the nasal cavity are rare and tend to recur locally. These tumosr have osteoclast-like giant cells and may arise from the nasal septum.

 lethal midline granuloma

lethal midline granuloma

 

Our patient had a squamous cell carcinoma of the nose. She underwent surgery. The mass was invading through the cribriform plate into the anterior cranial fossa and a debulking procedure was done endoscopically.

 

Lathi A, Syed M, Kalakoti P, Kishve S. Clinico-pathological profile of sinonasal masses: a study from a tertiary care hospital of India.  ACTA Otorhinolaryngologica Italica. 2011 Dec;31(6):372-377.

Humayun A, Zahurul H, Ahmed S, et al. Clinicopathological study of sinonasal masses. Bangladesh J Otohinolaryngol. 2010;16:15-22.

Gallo ES, Pehousheck JF, Crowson AN. An exophytic nasal nodule. Arch Dermatol 2010 Jul 146(7) 789-94

Swain S, Ray Ritam. Wegener’s Ganulomatosis of Nose: A case report. 2011 Indian J Otolaryngol Head Neck Surg. Oct 63(4):402-404.

A 42 y.o. woman co one month of intermittent shoulder pain; EMS called in for an acute MI and transmitted the first image.

 The EKG in the ED appears below

The EKG in the ED appears below

 What could account for the difference in the two EKGs?

What could account for the difference in the two EKGs?

Our patient had a spontaneous dissection of the L main, mid LAD and diagonal arteries without atherosclerotic disease. The flap intermittently closed.  The first case of coronary dissection without atherosclerotic disease was reported in 1931 involving a 42 y.o. woman who  presented with sudden cardiac death and a dissection was found on autopsy.. There is an association with fibromuscular dysplasia. Spontaneous coronary dissection accounts for 0.1-4% of all ACS cases.  Among younger women <50 it accounts for 10.8% of patients with ST elevation MI. In a case series 12% of individuals had simultaneous involvement of multiple coronary arteries.

While non-atherosclerotic coronary dissection was previously thought to be related to pregnancy  and the peripartum period, more recently other causes have been reported.  Fibromuscular dysplasia, connective tissue disease , systemic inflammation  and coronary artery spasm have all been implicated.  Isometric exercise increases cardiocirculatory stresses and shear forces against the coronary artery wall and may also be implicated. Cocaine has been associated with coronary dissection as well.

 notice the lucency in the circumflex characteristic of a dissection

notice the lucency in the circumflex characteristic of a dissection

Coronary artery dissection can be difficult to diagnose on cath where the image is 2-D. For patients with an intimal tear, multiple radiolucent lines separating the true and false lumens may appear with slow contrast clearing. Dissection may also appear as just compression of the lumen and is best seen on optical coherence tomography.

 dissection may also appear as narrowing on cath; which can be shown as a dissection on optical coherence tomography.

dissection may also appear as narrowing on cath; which can be shown as a dissection on optical coherence tomography.

The treatment includes antiplatelet therapy(asa and clopidogrel)  and beta blockade.  Revascularization can be challenging since PCI would often include long segments at risk for restenosis and the danger of extending the dissection. They are generally treated without stents as was our patient unless they have persistent pain. Thrombolytic therapy should be avoided in cases of dissection because there are reports of extension of the dissection;cath is preferred   Overall the frequency of thrombotic occlusion is higher that the risk of spontaneous dissection so in rural centers thrombolysis should not be withheld.

The natural history of  dissected segments is that they heal spontaneously if patients survive the initial event.   our patient was  initially was placed on a balloon pump  and treated with balloon angioplasty of the LAD and diagonal.  Her troponin peaked at 175.   Renal dopplers did not show fibromuscular dysplasia.  She was sent home with dual antiplatelet therapy and a betablocker. She returned one day after discharge with chest pain which was thought to be due to anxiety.  At that time her trop continued to trend down and she was discharged home.

 

Hill SF, Sheppard MN. Non-atheroclerotic coronary artery disease associated with sudden cardiac death. Heart 2010;96:119-1125.

Vanzett o G, Berger-Coz E, Barone-Rochette, G et al. Prevalence, therapeutic management and mediu-term prognosis of spontaneous coronary artery dissection: results from a database of 11,605 patients.  Eur J Cardiothorac Surg. 2009;35:250-254.

Saw J, Starovoytov A, ManciniJ, Buller CE. Non-atherosclerotic coronary artery disease in young women. J Am Coll Cardiol. 2011;58:B113. 

A 45 y.o. male presents with proptosis of the R eye his CT and MRI are shown below

What does he have?

muco4.jpg
muco3.JPG
 hint he had a previous craniotomy for trauma in 1996.&nbsp;

hint he had a previous craniotomy for trauma in 1996. 

Our patient had a mucocele which is an accumulation of mucoid secretions and desquamated epithelium within a sinus.  In this case it was cause by retained sinus lining after his frontal sinus fracture. He was sp cranialization of the R frontal sinus after his injury.  Over the course of many years he developed  a large mucocele intracranially but extradurally that displaced the globe and caused proptosis.  These may develop very slowly and can be reported 10 to 15 years after the fracture.  They can also occur without trauma in an obstructed sinus.

 proptosis with a R frontal sinus mucocele

proptosis with a R frontal sinus mucocele

 The frontal sinus is most commonly involved  in mucocele formation  with only rare mucoceles in the sphenoid, ethmoid and maxillary sinuses. The frontal sinus  is shared with the  superior orbital wall which explains the proptosis as the mucocele enlarges. 

Mucoceles were first described in 1820 by Langenbeck and called hydatides. The repair of mucoceles is complicated by the fact that they will recur if even a small amount of mucous producing tissue is left.

 open approach for mucocele removal

open approach for mucocele removal

The repair can be open or endoscopic with neurosurgeons generally preferring the open route to remove the entire cyst lining. If the posterior wall of the frontal sinus is eroded  a biopore sheet can be inserted to reconstruct the posterior frontal sinus wall.  It is placed between the orbit and the dura. Other mucoceles are treated endoscopically with or without stents.

Our patient’s course was complicated by the fact that he was psychotic and homeless.  He lacked capacity to consent for himself and two physicians were required to agree on the necessity of surgery. He underwent endoscopic endonasal drainage of the intracranial mucocele with prompt reduction in his proptosis.  His recovery was uneventful.

 

 

Alberti PW, Marshall HF, Munro B.  Fronto-ethmoidal mucocele as a cause of unilateral proptosis. Br J Ophthalmol.  1968;52:833.

Suri A, Mahapatra AK, et al. Giant mucoceles of the frontal sinus: a series and review. J Clin Neurosci. 2004;11:214-8.

Tan CS, Yong VK, Yip LW, AmritjS. An unusual presentation of a giant frontal sinus mucocele manifesting with a subcutaneous forehead mass. Ann Acad Med Singapore. 2005;34:397-8.

 

A 31 y.o. comes from prison with vision loss; he was hit in the L eye playing softball.

He has  a hyphema but also the fundus appears below.  What is the white patch?

berlins edema.JPG

Our patient had Berlin’s edema of the eye.  This whitish patch is caused by shock waves traversing the eye from the site of impact after blunt trauma.  It was first described by Berlin in 1873.  It occurs hours after injury and it thought to be caused by extracellular edema .  A cherry-red spot may also appear on the fovea because cells involved with the whitening are not present in the fovea.  Visual acuity may decrease to 20/200 but usually resolves in 3-4 weeks.

eye lens.JPG

traumatic lens dislocation

An estimated 30% of eye injuries presenting to the ED will have Berlin’s edema.  Blunt trauma to the eye also may result in hyphema, lens dislocation, vitreous hemorrhage, retinal breaks and detachment, and early and late glaucoma. Macrophages filled with hemoglobin or degenerated erythrocytes  raise the intraocular pressure by obstructing the trabecular meshwork after a vitreous hemorrhage. The increase in ocular pressure can occur immediately after an eye injury or years later. Two peaks of glaucoma have been reported , less than one year and greater than 10 years after trauma. 3.4% of patients with eye injuries develop glaucoma.

eye us.JPG

Our patient had both Berlin’s edema and a visible traumatic hyphema. He had normal eye pressure.  He was treated with  cyclopentolate 1% and prednisolone 1%.  His vision gradually improved.

 

Hart JC, Blight R. Commotio retinae. Arch Ophthalmol. 1979. 97(9): 1783.

Mendes S. Campos A, Beselga D, et al. Traumatic maculopathy 6 months after injury: a clinical case report. Case Reports in Ophthalmology. 2014;5(1): 78-82.

Fenton RH. Zimmerman LE. Hemolytic glaucoma, an unusual cause of open angle secondary glaucoma. Arch Ophthalmol. 1963; 70:236-267

Campbell DG. Simmons RJ. Grant WM. Ghost cell as a cause of glaucoma. Am J Ophthalmol. 1979;97:2141-46.

Sihota R, Kumar S et al Early predictors of traumatic glaucoma after closed globe injury. 2008. JAMAVol 126(7)921-926

 

A 49 y.o. woman with breast cancer and autoimmune hepatitis with cirrhosis and hepatocellular carcinoma presents with abd pain.

what do you notice  on her CT?

splenic infarct.JPG

Our patient had a splenic infarct. Splenic infarct occurs when one of the branches of the splenic artery is occluded  or there is venous  congestion with ischemia.  The complications include: a ruptured spleen , bleeding, pseudocyst or  abscess.

 gross pathology of a splenic infarct.

gross pathology of a splenic infarct.

CAUSES OF SPLENIC INFARCTION

Splenomegaly-  Splenic infarct is more common in hematological disorders associated with splenomegaly like myeloproliferative disorders. Splenomegaly caused by storage diseases like Gaucher’s can also result in infarct.  Probably the most common cause of splenic infarct is sickle cell disease where autosplenectomy occurs over time.

Splenic infections such as mono, cmv, malaria and babesiosis  are associated with splenic infarcts.  Hypercoagulable states such as malignancy , antiphospholipid syndrome  or factor V Leiden  may also result in splenic infarcts. The infarcts can also be embolic  associated with endocarditis or a fib.

Any compromise of the splenic artery can result in infarction including aortic dissection, external compression by a tumor or torsion of the splenic artery

Vasculitis and DIC are also associated with infarcts. In patients with cancer  a “vascular syndrome” has been described in patients receiving angiogenesis inhibitors like bevacizumab. Immediately following initiation of treatment; strokes, MIs , PEs and splenic infarcts have been reported.

Our patient had cancer which is known to cause a hypercoagulable state.  Decreased protein C, protein S, and antithrombin, activation of factor X, and increased platelet catabolism all  have been implicated in thrombogenesis in cancer patients. 

 splenic and liver infarcts in a patient with bevacizumab.

splenic and liver infarcts in a patient with bevacizumab.

In our patient, coagulation studies were normal, embolic disease was ruled out with a normal cardiac echo. Blood cultures showed no growth. It was assumed that her infarct was due to splenomegaly and her malignancies causing a hypercoagulable state.

 

Malka D, Van den Eynde M, Boige V et al. Splenic infarction and bevacizumab. 2006. Lancet Oncol 7(12)1038.

Rawla P, Vellipuram AR, Bandaru SS, Raj JP Splenic infarct and pulmonary embolism as a rare manifestation of CMV infection. 2017 Case Reports in Hematology  1850821.

Breuer C, Janssen G, et al. Splenic infarction in a patient  with hereditary spherocytosis, protein C deficiency and acute infectious mono. 2008 Eur J  Pediatr 167(12) 1449-52.

Frippiate F, Donckier J, Vandenbossche P , et al. Splenic infarctin report of three cases of atherosclerotic embolization originating in the aorta and retrospective study of 64 caes. 1996. Acta Clin Belg. 51(6) 395-402.

An 88 y.o. presents with reddened itchy skin. What is the problem?

 hint the peripheral smear has unusual cells pictured below.

hint the peripheral smear has unusual cells pictured below.

sezary1.JPG

Our patient had Sezary syndrome.  This is a type of cutaneous lymphoma occurring in 3% of skin lymphomas. It was first described by Albert Sezary in 1938.  It is an accumulation of T cells and therefore a T cell lymphoma where the T cells invade the skin causing intense pruritus.  The cells can also appear in the blood where they appear to be “cerebriform” lymphocytes. The disease can be associated with mycosis fungoides and is associated with lymphadenopathy in the later stages. Clinically,  it is associated with redness of the skin, hyperkeratosis of the palms and  and occasionally lagophthalmos.  ( inability to close the eyelids;referring to the myth that rabbits sleep with their eyes open)

 mycosis fungoides with typical plaques

mycosis fungoides with typical plaques

Mycosis fungoides  is the most common form of cutaneous T-cell lymphoma. It is an unusual expression of CD4-T cells. These lymphocytes form dense plaques in the skin with lymphoid cells lining  up along the epidermal basal layer.  Mycosis fungoides is considered a less virulent lymphoma than Sezary syndrome and often patches in the skin can be dormant for years. They may begin as simply hypopigmented dermal thickening.  In the final stages tumors may develop in the patches.

mf lymphoskinbx.JPG

Treatment consists of radiation of the skin with electron beam or UVB light and psoralen with UVA therapy.  Recently imiquimod has been used in treatment of skin lesions. Imiquimod increases cytokine release  and inflammatory reaction.

Our patient received a retinoic acid compound and light treatments.  Because of the overall poor prognosis of Sezary syndrome, he was made DNR/DNI.  

 

Sezary A, Bouvrain Y. Erhthrodermie avec presence de cellules monstrueuses dans le derme et le sang circulant. Bulletin dela Societe francaise de dermatologie et de syphiligraphie, Paris, 1938;45:254-260.

Thangavelu M, Finn WG, Yelavarthi KK,  et al. Recurring structureal chromosome abnormalities in peripheral blood lymphocytes of patients with mycosis fungoides/Sezary syndrome Blood 89(9)3371-7.

Jawed S. Hyskowski P, et al. Primnary cutaneous T-cell lymphoma ( mycosis fungoides and Sezary syndrome. 2014. Journal of the American Academy of Dermatology 70(2) 223. E1-223e17.

Yamashit T, Abbade L, Marques M, Marques S.  Mycosis fungoides and Sezary syndrome: clinical histopathological and immunohistochemical review and update. An Bras Dermatol 2012Nov-Dec 87(6 ) 817-830.

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

Hint; he has hilar adenopathy shown below

sarcoid1.JPG
 hilar adenopathy

hilar adenopathy

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

 

 involvement of sacral roots on MRI

involvement of sacral roots on MRI

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

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

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

 &nbsp;e nodosum

 e nodosum

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

 

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

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

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

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

What does he have?

branchial cleft abs.JPG

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

 common sites for branchial cleft cysts

common sites for branchial cleft cysts

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

 Other branchial cleft sites

Other branchial cleft sites

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

 MRI of our patients neck

MRI of our patients neck

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

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

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

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

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

What condition does he have?

melanosis.JPG

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

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

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

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

 carpopedal spasm

carpopedal spasm

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

 

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

 

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

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

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

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

 What is the differential?

What is the differential?

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

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

rbc.JPG

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

 causes of hemolysis

causes of hemolysis

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

If you see schistocytes you may have a thrombotic microangiopathy.

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

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

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

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

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

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

thrombocy.JPG

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

 what do you notice when compared to the normal image below

what do you notice when compared to the normal image below

kagers normal.JPG

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

kagers fat pad.JPG

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

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

calcaneus haglands..JPG

 

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

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

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

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

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

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

 you notice some gallstones ....and

you notice some gallstones ....and

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

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

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

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

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

 

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

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

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

 look for gall bladder perforation on US

look for gall bladder perforation on US

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

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

thanks to Dr Baumgartner

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

an apparently normal 7 wk gestation

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

and you also notice this mass;  what is it?

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

 molar twin pregnancy at 19 wks.

molar twin pregnancy at 19 wks.

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

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

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

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

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

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

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

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

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

What is wrong?

 what do you want to do?

what do you want to do?

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

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

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

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

sdlipohemarthrosisf.JPG
 from Magid

from Magid

 Us showing fluid in the joint&nbsp;

Us showing fluid in the joint 

 

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

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

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

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

shoulderdis.JPG

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

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

 What do you notice on her chest CT?

What do you notice on her chest CT?

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

saddlef.jpg

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

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

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

stroke ,blood flow.JPG

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

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

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

 The urethra is very dilated suggesting a stricture

The urethra is very dilated suggesting a stricture

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

What happened?

urethra, rupture with nec.JPG

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

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

 Gas in the soft tissues from necrotizing fasciitis.

Gas in the soft tissues from necrotizing fasciitis.

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

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

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

 The fasces appears on the left.

The fasces appears on the left.

Useless trivia:

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

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

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

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

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

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

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

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

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

goiter2f.jpg

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

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

 goiters in the Himalayas

goiters in the Himalayas

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

goiter belt.JPG

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

 cretinism(severe iodine deficiency in three children in the Congo

cretinism(severe iodine deficiency in three children in the Congo

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

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

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

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

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