A 48 y.o. complains of inability to urinate and lower abdominal pain

What is wrong?

stercoral colitis f.JPG

Our patient had stercoral colitis.This is an inflammatory colitis caused by increased intraluminal pressure from impacted fecal material in the colon.  The rectosigmoid is the most frequently involved. The most important complication is perforation and mortality for the condition is 32-57%.

The condition is almost always found in cases of chronic constipation, advanced age and medical cormorbidities. Colonic wall thickening is > 3 mm, and air migrates from the colon lumen into the wall of the colon. In addition to constipation; diseases which cause obstruction of transit such as Hirschprung’s, hypercalcemia, and Chagas disease can cause stercoral colitis.

stercoral colitis causing perforation with free abd fluid, and discontinuity of the sigmoid in an opiod user

stercoral colitis causing perforation with free abd fluid, and discontinuity of the sigmoid in an opiod user

Our patient presented with abdominal distention and no urine output.  She was found to have urinary retention with a creatinine of 15  and a K of 5.0. She had bladder obstruction with hydronephrosis bilaterally.  A Foley was placed and 4L of urine was obtained. She was disimpacted at the bedside by general surgery. On the second day after disimpaction her Cr was 1.0 and she was discharged.

toxic megacolon occurs in 1% of patients with c diff as in this patient.

toxic megacolon occurs in 1% of patients with c diff as in this patient.

Unal E, Onur M, Baci S, et al. Stercoral colitis: diagnostic value of CT findings. 2017 Diagn Interv Radiol. Jan;23 (1):5-9.  

Serpell J, Nicholls R. Stercoral perforation of the colon Br J Surg 1990;77:1325-1329.

Core Topics in General and Emergency Surgery (6 ed.). Elsevier. 2019. Pp216-233.

A 33 y.o. male presents with leg swelling(and Oh, by the way, a rash on the penis)

Why are his legs swelling?

Our patient had nephrotic syndrome from membranous glomerulonephropathy caused  by syphilis.

 

Membranous nephropathy can be caused by hep B, HIV,  hep C, NSAIDS, Li,  and gold salts.  It is also caused by autoimmune diseases like lupus and occasionally can be caused by solid cancers.  In the case of our patient, all other causes were ruled out and he improved with penicillin alone. 

pathologic findings of membranous glomerulonephropathy

pathologic findings of membranous glomerulonephropathy

Renal disease is also caused by heroin and cocaine.  7 of 8 heroin addicts that present with nephrotic syndrome have membronoproliferative glomerulonephritis in one study.  Blacks are 3-7 times more likely to suffer renal disease. 

Cocaine releases endothelins which induce endogenous angiotensin converting enzyme causing vasoconstriction.   This can result in renal infarct, endothelial injury  or acute rhabdo from muscle breakdown.  These effects can be  partially blocked by ACE inhibitors. The final common pathway of endothelins is to cause fibrosis and renal failure.

endothelins produce fibrosis.JPG

Crowe A, Howse M, Bell G, Henry J. Substance abuse and the kidney  2000 QJM 93:147-152.

Van Assen S, Bakker S. Did syphilis truly strike the kidneys this times. 2005. Nephrology, dialysis transplantation Vol. 20 Issue 6 1029-1031.

Zhang Z, Hever A, Bhasin N, et al. Secondary syphilis associated with membranous nephropathy. 2018 Perin J. 22:17-062.

 

A 39 y.o. woman with lupus presents with swollen breasts and no other swelling

What could be wrong?

svc.JPG

Our patient had classic peau d’orange, but had superior vena caval syndrome and not inflammatory breast cancer. She did not have swelling of her face because of extensive chest wall collaterals. 

collaterals on the chest wall with svc thrombosis.

collaterals on the chest wall with svc thrombosis.

It was first described in 1757 by William Hunter in a patient with syphilitic aortitis compressing the SVC. The syndrome occurs in 15,000 people in the US a year.  The most common cause is malignancy with 85% reported to be caused by malignancy in 2008 but now 40% are reported in non malignant cases due to the prevalence of ports.   Other causes of SVC syndrome include: fibrosing mediastinitis, tuberculosis, histoplasmosis , actinomycosis  and thrombosis caused by pacemaker leads.

fibrosing mediastinitis causing svc obstruction

fibrosing mediastinitis causing svc obstruction

Shaikh I, Berg K, Kman N. Thrombogenic Catherter-Associated Superior vena cava syndrome. 2013, Case Resports in Emergency Medicine Volume 2013, article ID 79054, 3 pages.

Higdon M, Higdon J. The treatment of oncologic emergencies. Am Fam Physician 2006;74:1873-80.

Wilson L, Dettebach F, Yahalom. Superior vena caval syndrome. 2007 NEJM 356:1862-1869.

Cohen R, Mena D, Carbajal-Mendoza A, et al. Superior vena caval syndrome: A medical emergency? Int J. Angio 2008 spring 17(1):43-46

 

A 62 y.o. male from St. Louis presents with fever, hypoxic respiratory failure and tender inguinal nodes.

Is there a unifying diagnosis?

tu.JPG
tu2f.jpg

Our patient had tularemia or “rabbit fever”, blood cultures were positive for Francisella tularensis. He lives in south St. Louis county and was scraping a dead rabbit from his driveway and presumably inhaled aerosolized bacteria.  Ten days later he presented with fever and hypoxia.  He had painful nodes in the R groin and they were biopsied. In addition to rabbits, voles and feral hogs in Texas have been reported as reservoirs of disease.

tular2.JPG

 Tularemia is also transmitted by tick bites and deer fly bites. The disease is named after Tulare county in California where the disease was first discovered in 1911. Although generally rare, the disease is present in sporatic outbreaks in Finland and Sweden with   4,830 cases reported in Sweden  from 1984-2012. 

Cases in the US in 2016

Cases in the US in 2016

Types of tularemia include:

Ulceroglandular- the most common type where an ulcer appears at the site of the infection.

Oculoglandular- when the bacteria enter through the eye

Oropharyngeal- from eating contaminated food where the bacteria causes mouth ulcers

Pneumonic- results either from breathing the organism or bacteremia  from ulceroglandular with dissemination.

Treatment is usually with gentamycin although doxycycline can also be used. Interest in tularemia increased in modern times since it is a potential biologic weapon with only 10 to 50 bacteria being sufficient to infect a person.

Ulceroglandular tularemia

Ulceroglandular tularemia

Desvars A, Furberg M, Hjertquist M, et al. Epidemiology and ecology of tularemia in Sweden. 1984-2012. 2015 emerg Infect Dis 21(1) 32-39.

Feldman K, Enscore R, Lathrop S., et al. An outbreak of primary pneumonic tularemia on Martha’s Vineyard.2001.  NEJM 345(22): 1601-6.

Dennis D, Inglesby T, Henderson D, et al. Tularemia as a biological weapon: medical and public health management. 2001 JAMA 285(21)2763-73.

Random rabbit facts:

In the wild rabbits don’t eat carrots or any root vegetables.

Their warrens can extend 10 feet underground.

Rabbits can see nearly 360 degrees.

Ovulation is triggered by intercourse and after a thirty day gestation 4-12 kits are born.

A 69 y.o. presents with shortness of breath

What do you see on the CT of the chest?

leio.JPG

Our patient had lymphangioleiomyomatosis or LAM.  It is a kind of cystic lung disease which can be associated with tuberous sclerosis and affects women in their childbearing years. In 82% of patients the initial presentation is with pneumothorax.  There are multiple complications of lymphatic obstruction including chylothorax, chylous ascites and chylopericardium. These complications are caused by smooth muscle cells that invade vessels and airways.

Kidney tumors, angiomyolipomas are present in 30% of the patients with LAM not associated with tuberous sclerosis and int 90 % of patients with tuberous sclerosis.

skin manifestations of tuberous sclerosis include adenoma sebaceum

skin manifestations of tuberous sclerosis include adenoma sebaceum

The other three types of cystic lung disease are Langerhan’s cell histiocytosis (eosinophilic granuloma), Birt Hogg Dube (which presents with lung cysts and renal CA caused by an abnormality of folliculin) and lymphoid interstitial pneumonia.  The latter is found in autoimmune diseases like Sjogrens and is caused by an overgrowth of lymphoid tissue.

more skin manifestations of cystic lung disease

more skin manifestations of cystic lung disease

Cystic lung disease does not include: cystic fibrosis(where the cysts are found in the pancreas), emphysema, metastatic cystic lesions  or bronchiectasis where air is trapped in spaces without true walls.  Any cystic lung disease can present with pneumothorax.

 

McCormack FX. Lymphangioleiomyomatosis: a clinical update. 2008 Chest 133(2):507-16.

Lampert S, Schmid A, Wiest O, Hahn E, Ficker J. Fire-eater’s lung. Two cases and review of the literature. 2006. Disch Med Wochenschr  131(7):319-22.

Koo H, Yoo C.  Multiple cystic lung disease. 2013 Tuberc Respir Dis 74(3) 97-103

A bust in the Musee l'Homme in Paris depicts a disease. What is it?

Screen Shot 2019-03-11 at 3.21.46 AM.png

This bust depicts gumma associated with tertiary syphilis. The gumma are granulomas attempting to wall off the treponema pallidum. Gummas have are a proteinaceous mass with a necrotic center. They eventaully become fibrotic.

Electron micrograph of spirochetes; similar spirochetes cause leptospirosis.

Electron micrograph of spirochetes; similar spirochetes cause leptospirosis.

Tertiary syphilis occurs either as gummas, neurosyphilis or cardiovascular syphilis with aortitis being the most serious feature. There are two main tests for tertiary syphilis: nontreponemal and treponemal. The non treponemal are the VDRL and RPR. These vary can be used to assess treatment status since they decrease with treatment. They are also prone to false positive results and can be positive in measles, lymphoma, tuberculosis and even pregnancy. For this reason the treponemal tests are also done. The FTA-Abs(fluorescent treponemal antibody absorption test) is the most sensitive test for detection of syphilis but is always positive after infection and cannot be used to follow the course of treatment.

There are several non-venereal types of treponema pallidum including yaws shown above.

There are several non-venereal types of treponema pallidum including yaws shown above.

The good news is that treponema pallidum pertenue and nonvenereal endemic syphilis are easily treated with penicillin. If untreated yaws can cause bony destruction and disfigurement.

Kent ME, Romanelli F. Reexaming syphilis: an update on epidemiology, clinical manifestations and management. 2008. Anneals of Pharmacology 42(2):226-36.

Eccleston K, Collins L, Higgins SP, Primary syphilis: the classical triad of skin rash, mucosal ulceration and lymphadenopathy. 2010. Int J of STD &AIDS 21(8) 537-45.

Bhatti MT. Optic neuropathy from viruses and spirochetes. 2007. Int Ophthalmol Clin 47(4) 37-66.

A 67 y.o. male arrests in front of EMS and his EKG is shown below

Would you call the cath lab?

lane first ekg.JPG

Our patient had hyperkalemia.  His second EKG is shown below. 

ekg2.JPG

The pt had a hx of seizures and was found in an empty bath tub.  He initially had agonal respirations and a weak pulse but arrested in front of EMS.   K was 7.9 and lactate was 9.7  He was found to have a SDH on CT. The cath lab was called but felt the changes were due to electrolyte abnormalities and did not cath the pt.

Although this case was hyperkalemia AV blocks do exist with MIs.

High degree AV block occurs in 9.8% with inferior MI and 3.2% with anterior MI in large studies.  High degree AV block associated with inferior MIs is located proximal to the bundle of His and the QRS is usually narrow.  High degree AV associated with anterior MI is usually distal to the AV node and is associated with high mortality and a widened QRS.

Inferior MI  can be associated with sinus brady, Mobitz I, and complete heart block since the SA node, AV node and his bundle are most often supplied by the RCA. Anterior MI is associated most often with a Mobitz II block and can cause complete heart block.

Pacing is recommended for symptomatic complete heart block, alternating right and left bundle branch block, symptomatic bradycardia and Mobitz I second-degree AV block.

 

blood supply.JPG

Meine TJ, Al-Khatib SM, Alexander JH, et al. Incidence, predictors and outcomes of high-degree atrioventricular block complicating acute myocardial infarction treated with thrombolytic therapy. Am Heart J 2005;149;670.

Hunt D, Sloman G, Bundle-branch block in acute myocardial infarction. 1969. Brit. Med J. 1(5636):85-8.

Sgarbossa EB, Pinski SL, Barbageleta A, et al. Electrocardiographic diagnosis of evolving acute myocardial infarction in the presence of left bundle branch block. GUSTO-1

N Engl J Med 1996;334:481-7.

Wilner B, deLemos, J, Neeland I. LBBB in patients with suspected MI: an evolving paradigm. 2017. American College of Cardiology.

 

A 22 y.o. woman presents with vomiting and weight loss

What does her upper GI show?

the arrows point at the problem

the arrows point at the problem

Our patient had a duodenal obstruction from superior mesenteric artery syndrome. She had compression of the third portion of the duodenum between the superior mesenteric artery and the aorta. The syndrome was first described in 1861 by Von Rokitansky.  The incidence is reported to be 0.1-.3%
It can occur after scoliosis surgery, anorexia with weight loss, rapid growth in adolescence,  or trauma.

an angle of 20 degrees between the sma and aorta is considered likely to cause sma syndrome

an angle of 20 degrees between the sma and aorta is considered likely to cause sma syndrome

The greatest barrier to making the diagnosis is not to think of it.  Delay in diagnosis can result in malnutrition, electrolyte abnormalities, a duodenal bezoar or even gastric perforation. The symptoms are typically relieved when the patient is in the L lateral decubitus, prone or knee to chest position.

surgical correction of sma syndrome with a duodenojejunostomy

surgical correction of sma syndrome with a duodenojejunostomy

Surgical correction is recommended if conservative measures fail. The standard procedure is a duodenojejunostomy  where an anastomosis is created between the duodenum and jejunum anterior to the superior mesenteric artery.

sma syndrome can be diagnosed on Ct as well. the large white arrow is the dilated duodenum, the small black area is the compressed distal duodenum and the two small black arrows show the closeness of the sma and aorta. with the duodenum compressed between.

sma syndrome can be diagnosed on Ct as well. the large white arrow is the dilated duodenum, the small black area is the compressed distal duodenum and the two small black arrows show the closeness of the sma and aorta. with the duodenum compressed between.

Our patient underwent a duodenojejunostomy which was complicated by a wound infection and contained duodenal leak. She recovered and is now able to eat whatever she wants without vomiting.

Gerasimidis T, George F. superior Mesenteric Artery Syndrom.  Dig Surg 2009. May 20. 26(3):213-14.

Saraya T, Kurai D, Ariga M, Nakamoto K, Koide T, Tamura M. Superior mesenteric artery syndrome caused by huge mycotic abdominal aortic aneursym. Intern Med. 2009. 48(12):1065-8.

Pottorf BJ, Husain FA, Hollis HW Jr, Lin E. Laparoscopic management of duodenal obstruction resulting from superior mesenteric artery syndrome.  JAMA Surg. 2014 Dec. 149(12):1319-22.

Rokitansky C. Handbuch der Pathologischen Anotomie. Vienna: Branmiller and Siedel. 1842.

 

A 36 y.o. woman presents with shortness of breath. What is the most likely cause?

what causes cannonball lesions of the lung?

what causes cannonball lesions of the lung?

Our patient had metastatic melanoma in the lungs. The differential of “cannon ball” lesions in the lungs includes metastatic disease, infection, vasculitis, autoimmune disorders  and sarcoid.  

METASTATIC DISEASE

The most common malignancies associated with cannon ball lesions are: renal cell ca, choriocarcinoma, endometrial carcinoma, prostate cancer, colon cancer,  and synovial sarcoma. While these are the most common; any cancer can produce lung nodules.

renal cell ca

renal cell ca

INFECTIOUS DISEASE

While tuberculosis classically presents in the upper lobes, in immunosuppressed patients including diabetics it can present as nodules.  Histoplasmosis, cryptococcosis , nocardia , and echinococcus can all present as nodules.

pulmonary tbc in a diabetic pt

pulmonary tbc in a diabetic pt

VASCULITIS/AUTOIMMUNE/SARCOID

Wegener’s, is often diagnosed by c-ANCA . These are antibodies to proteinase 3 and myeloperoxidase which occur in 90% of patients with Wegener’s. Necrotic nodules can be found in the lungs. Sarcoid and rheumatoid nodules can also appear as lung nodules.

rheumatoid nodules are composed of B lymphocytes and often occur after methotrexate is started.

rheumatoid nodules are composed of B lymphocytes and often occur after methotrexate is started.

Kshatriya R, Patel V, Chaudhari S, Patel P, et al.  Cannon ball appearance on radiology in a middle-aged diabetic female.  2016. Lung India. Sep-Oct 33(5):562-568.

Wheat LJ, Kaufmann C. Histoplasmosis. Infect Dis Clin N Am 2003;17:1-19.

Vohra P, Sharma M, Yadav A, Chaudhary U. Nocardiosis: A review of clinic-microbiological features, Int J Life Sci Biotechnol Pharma Res. 2013;2:20-29.

A 27 y.o. with a hx of sinusitis for two months presents with a seizure

What do you see?

IGG4sinusitis.JPG
and what is going on the in the L frontal lobe?

and what is going on the in the L frontal lobe?

Our patient had IgG4-related disease.   This is not a brain abscess but rather a systemic inflammatory condition where tissue is infiltrated by plasma cells that secrete IgG4.  IgG4 related disease can involve multiple sites in the body. It can be found in the heart, lung, GI tract, prostate and brain. There can be considerable organ destruction related to the disease.

IgG4 in the prostate with a dense lymphocytic infiltrate

IgG4 in the prostate with a dense lymphocytic infiltrate

The pathologic process was first described in 1977 when histologic research was done on 349 cases of Kuttner’s tumor( now known as IgG4-related sialadenitis).  Diffuse infiltration of lymphocytes was noted with plasma cell infiltration and eventual fibrosis. 

IgG4 related disease in the parotid on the R, Mikulicz disease

IgG4 related disease in the parotid on the R, Mikulicz disease

 Several diseases that have been thought to be unrelated are now linked by the fact that they contain IgG4 secreting plasma cells.  Some of these diseases include:  fibrosing mediastinitis, interstitial nephritis, autoimmune pancreatitis, retroperitoneal fibrosis, sensorineural hearing loss,  and inflammatory pseudotumors.

 

Our patient received rituximab.  Research is underway to evaluate plamablast-directed therapy with a monoclonal antibody(XmAb5871) which inhibits B-cell function.

 

Perugino C, Stone J. Treatment of igG4-related disease: Current and future approaches.2016.  Zeitschrift fur Rheumatologies. 75(7):681-686. PMID.27431746(https://www.ncbi.nlm.nih.gov/pubmed/27431746)

Seifert G, Donath K. Zur Pathogenese des Kuttner-Tumors der Submandibularis-Analyse von 4347 Fallen mit chronischer Sialadenitis der Submandibularis  ) on the pathogenesis of the Kuttner tumor of the submandibular gland-analysis of 349 cases with chronic sialadenitis of the submandibular . 24(3):81-92.  PMID 856776( https://www.ncbi.nlm.nih.gov/pubmed /856776

 

Wallace Z, Deshpande V, et al.  IgG4-related disease: Clinical and Laboratory Features in 125 Patients. 2015. Arthritis and Rheumatology 67(9): 2466-75. PMID 25988916(https://www.ncbi.nlm.nih.gov/pubmed/25988916)

 

A 41 y.o. woman fell while playing in the snow and injured her ankle

how would you treat her minimally displaced , non articular fibular fracture?

fib fx 2.jpg

Our patient had a “bimalleolar equivalent” fracture of the ankle.  Because of the spiral nature of the fracture shown on the lateral below, a stress view of the ankle must be done.

spiral fibular fx seen on lateral view

spiral fibular fx seen on lateral view

In this case the stress view showed widening of the ankle mortice and required a surgical repair.

widening of the ankle mortice on stress view

widening of the ankle mortice on stress view

Injuries to the fibula when there is an isolated fracture of the distal tip can often be treated like an ankle sprain. This is the most common injury to the ankle. Bimalleolar fractures on the other hand,  involve the fibula and medial malleolus and almost always require a surgical repair.  The third type of ankle fracture involves the syndesmosis between the tibia and fibula.  Referred to as a Maisonneuve fracture, it is a spiral fracture of the proximal third of the fibula near the head with tearing of the interosseous membrane and tibiofibular syndesmosis often with a deltoid ligament tear or medial malleolus fracture. There will be a widening in the ankle mortice. 

A fourth type of fibular fracture is common in long distance runners.  This is a stress fracture of the mid fibula which  comes on gradually and can be seen on ultrasound.

A similar disruption of the interosseous membrane occurs in the forearm with the Galeazzi fracture, a fracture of the distal radius with dislocation of the distal radioulnar joint. Because of the tear in the interosseous membranes both the Maisonneuve and Galeazzi fractures may cause compartment syndrome.

galeazzif.JPG

Hoglund L, Sibernagel K, Taweel N. Distal fibular stress fracture in a female recreational runner: a case report with musculoskeletal ultrasound imaging findings. Int J Sports Phys Ther. 2015 10(7):1050-58.

Pankovich AM. Maisonneuve fracture of the fibula. J Bone Joint Surg Am 1976. Apr;58(3):337-42.

Wilson FC. Fractures of the ankle: pathogenesis and treatment. South Orthop Assoc. Summer 9(2):105-15.

A 24 y.o. with spina bifida presents with shortness of breath

do you see a possible reason for her shortness of breath?

do you see a possible reason for her shortness of breath?

Our patient had a clot in the inferior vena cave and May Thurner syndrome.  This refers to compression of the iliac vein by the iliac artery causing low flow and a clot.  It can occur on the R or L but usually occurs on the L since the vein has to cut to the R to join the IVC.   While the R common iliac artery overlies the L iliac vein in 20% of people it is responsible for 2-3% of lower extremity DVTs.  It can be diagnosed on ultrasound in only 20% of cases since the veins are so deep. It should be considered especially in women between 20 and 40 with recurrent DVTs,  phlegmasia  alba dolens or phlegmasia cerulea dolens.

R iliac artery compressing the L iliac vein

R iliac artery compressing the L iliac vein

It was first described in 1851 but was more widely recognized in 1957 when an autopsy series found compression of the L iliac vein against the lumbar spine by the R common iliac artery on 22% of cadavers.

phlegmasia cerulea dolens

phlegmasia cerulea dolens

Our patient  had multiple pulmonary emboli as well as the IVC clot. She was anticoagulated and placed on Xarelto. She had chronic anemia and her hemoglobin trended downward on several occasions but was transfused and mild esophagitis was seen on endoscopy. She was eventually discharged.

 In other series, thrombectomy and stent placement were used to treat the syndrome.

phlegmasia alba.JPG

Peters M, Syed R, Katz M, et al May-Thurner syndrome: a not so uncommon cause of a common condition. 2012 Pro(Bayl Univ Med Center) Jul;25(3):231-233.

May R, Thurner J. The cause of predominantly sinistral occurrence of thrombosis of the pelvic veins. Angiology 1957;8(5):419-427.

Kivve MR, Ujiki M, et al.  Iliac vein compression in an asymptomatic patient population. J Vasc Surg 2004;39(5):937-943.

A 56 y.o. male presents with painful arm swelling and a rash

What is wrong?

What is wrong?

Our patient had Kaposi’s sarcoma.  The epidemic type (which he had) was more common among the HIV positive patients prior to good antiretroviral therapy.

It is also found in other immunosuppressed patients often after a transplant and in all cases is related to Herpesvirus 8.  As in our patient, the disease can be widely disseminated. Our patient had Kaposi’s of the lung as well causing left lower lobe collapse. He had tumor of the L chest wall and marked lymphadenopathy in posterior mediastinum and inguinal region.

Involvement of the lung caused LLL collapse

Involvement of the lung caused LLL collapse

Although it is called a sarcoma, it does not arise from mesenchymal tissue and is thought to be a cancer of the lymphatic endothelium.  It forms vascular channels that fill with blood and cause the purplish lesions visible on the skin. The diagnosis is made by biopsy and the presence of KSHV protein LANA.

Kaposi’s can be nodular or flat as appears on this image

Kaposi’s can be nodular or flat as appears on this image

Treatment is based on the sub-type, and the person’s immune function.  HAART suppresses Kaposi’s in 40% of AIDS associated Kaposi’s, but a number of people will have recurrence of the lesions after years on HAART.

Our patient remains on HAART and is free of the skin lesions, although the LLL collapse never improved. 

 

Untreated Kaposi’s becomes nodular

Untreated Kaposi’s becomes nodular

Sand M, Sand D, Thrandorf C, et al. Cutaneous lesions of the nose. Head and face medicine 6:7 doi;10.1186/1746-160X-6-7 pMIS 20525327.

HoffmannC, Sabranski M, Esser S 2017. HIV-Associated Kaposi’s Sarcoma.  Oncology Research and Treatment 40(3) 94-98.

Qunibi W, Al-Furayh O, Almeshari K, et al. Serologic association of humnan herpesvirus eight with posttransplant Kaposi’s sarcoma in Saudi Arabia. Transplantation 65(4):583-5.

Radu O, Pantanowitz L. Kaposi sarcoma. 2013 Archives of Pathology and Laboratory Medicine. Feb, Vol 137 No 2;289-294

A 79 y.o. woman presents with RUQ pain

What do you notice on CT?

polycystic liver.JPG

Our patient had polycystic liver disease which is defined as ten or more cysts in the liver.  Her pain was probably from bleeding into a cyst.

Polycystic liver disease is an inherited disorder affecting 1 in 100,000 people. The cysts enlarge with age and usually become symptomatic by age 50.  Three gene mutations have been linked to polycystic liver disease PRKCSH,LRP5 and SEC63 but more than 50% of individuals with the disease do not have these.  As the cysts enlarge they can produce bile duct compression, IVC thrombosis and PE, or portal hypertension. They commonly enlarge under the influence of estrogen and therefore are more common in women.

hydatid cysts.JPG

The differential for liver cysts includes hydatid disease, neoplastic cysts (cystadenoma, cystic sarcoma, metastatic ca from the ovaries, colon, kidneys or pancreas) and bile duct related cysts (Caroli’s disease, bile duct duplication)

World-wide, the most common liver cyst is caused by infection with the larval stage of a tapeworm,  Echinococcus granulosus. Dogs are the definitive host and sheep, cattle, goats and pigs are the intermediate hosts. In northern areas wolf and deer can be hosts, especially where reindeer are domesticated. In the European type,  the definitive host is the dog but even camels can be intermediate hosts.

hydatid cysts can occur in many organs including uterus and bone.

hydatid cysts can occur in many organs including uterus and bone.

The differential for liver cysts includes hydatid disease, neoplastic cysts (cystadenoma, cystic sarcoma, metastatic ca from the ovaries, colon, kidneys or pancreas) and bile duct related cysts (Caroli’s disease, bile duct duplication).  Our patients pain resolved and she was discharged without treatment.  In severe cases of polycystic liver a transplant can be performed.

Gevers TJ, Drenth JP. Diagnosis and management of polycystic liver disease. Nat Rev Gastroenterol Hepatol. 2013. 10(2):101-8

Abu-Wasel B, et al. Pathophysiology, epidemiology, classification and treatment options for polycystic liver disease. World J Gastroenterol. 2013. 19(35):5775-86.

Miliadis L, Giannakopoulos, et al. Spontaneous rupture of a large non-parasitic liver cyst: a case report J Med Case Reports 2010;4:2 doi  10.2286/1752-1947-4-2.

A 57 y.o. woman presents with jaundice and a Hct of 16. Her peripheral smear is shown below.

What is wrong?

What is wrong?

Our patient had spur cell hemolytic anemia. The other name for these speculated cells is acanthocytes. ( from the Greek word acanthi meaning thorn) This can occur in about 5% of patients end stage liver disease.  Liver dysfunction causes accumulation of abnormal apolipoprotein A-II-deficient lipoprotein in the plasma.  Red cells are loaded with cholesterol by this lipoprotein and acquire too much cholesterol in the outer layer of the membrane.  This causes the outer layer of the membrane to bulge out in spicules which are trapped in the spleen and destroyed.

While similar cells can be seen in other diseases like myxedema, and inherited disorders like abetalipoproteinemia the causes there are different with congenital membrane abnormalities being common in abetalipoproteinemia and lipid abnormalities in hypothyroidism.

echinocytes

echinocytes

Spur cells are not to be confused with echinocytes( from the Greek echinos meaning sea urchin) which may appear in uremia or  caused by excess  EDTA in an underfilled collection tube  or slow drying of the cells on a slide. The projections are smaller and more uniform in echinocytes.

JUST TO REVIEW HEMOLYTIC ANEMIA

A. CONGENITAL

Sickle cell

Thalassemia

G6PD deficiency

Hereditary spherocytosis

 abetalipoproteinemia

B. ACQUIRED

Infections- malaria, mycoplasma, mono, cmv, HIV, clostridia

Medications (sulfa, penicillin, antimalarial meds)

Autoimmune-SLE, lymphoproliferative disorders

Ulcerative colitis

Hypersplenism

Mechanical heart valves

Snake venoms

Liver disease

Microangiopathic hemolytic anemia- TTP

The only cure for spur cell hemolytic anemia is a liver transplant and our patient was being prepared for a transplant.

Doll DC, Doll NJ. Spur cell anemia. South Med J. 1982 Oct 75(10):1205-10.

Gerber B, Stussi G. Reversibility of spur cell anemia. Blood 2011. 118:4304 https: //doi.org/10.2282/blood-2010-11-321034.

L’Acqua C, Hod E. New perspectives on the thrombotic complications of haemolysisBr J Haematol 2015;168-175.

A 29 y.o. male comes in with L flank pain

What does the CT show?

CT was correctly read by the junior in EM1. Great work Scott!

CT was correctly read by the junior in EM1. Great work Scott!

Our patient had diverticulitis. Diverticular disease is one of the most prevalent conditions in western society.  While it primarily affects the elderly with 50% of individuals over 60 being affected, there is an increasing incidence in individuals under 40. 10-25% of individuals over 60 with diverticular disease will develop diverticulitis. It is a disease of western society with Europe, Australia and the US having the highest prevalence. In Africa and Asia the prevalence is less than 0.5% presumably because of the increased fiber in the diet and therefore shorter transit times and less intraluminal pressure.

our patient with a L sided diverticulum

our patient with a L sided diverticulum

It is important to recognize diverticular disease because of its complications: pain, peridiverticular abscess, bleeding, perforation, fistulae, strictures and obstruction.  A clinical entity of segmental colitis associated with diverticula (SCAD) has been more recently recognized where there is friable mucosa in the region of diverticula.  Case reports have suggested that these patients should be treated with aminosalicylates because some progress to inflammatory bowel disease.

diverticulae on colonoscopy

diverticulae on colonoscopy

For diverticulitis without significant complications which includes about 75% of cases, the mainstay of treatment includes bowel rest or clear fluids, antibiotics and pain control. Gram negative and anaerobic coverage is given with Cipro and Flagyl. Four to six weeks after resolution of the first attack of diverticulitis patients should undergo colonoscopy to rule out cancer. One third of patients will have a second attack.

The decision to admit is based on clinical status at presentation.  Patients who are immunosuppressed, are toxic looking with high fever, have no home support, and are unable to tolerate po fluids should be admitted. Our patient was discharged on Cipro and flagyl and did well. 

 

 

an 86 y.o with sigmoid diverticulae causing a stricture

an 86 y.o with sigmoid diverticulae causing a stricture

Weizman A, Nguyen G. Diverticular disease: epidemiology and management 2011 Can J Gastroenterol. July;25(7):385-389.

Pereira MC. Diverticular disease-associated colitis: Progression to severe chroni ulcerative colitis after sigmoid surgery. Gastrointest Endosc. 1998;48:520-3.

Rafferty J, Shellito P, Hyman NH, et al. Practice parameters for sigmoid diverticulitis. Dis Colon Rectum. 2006;49:939-44.

A 77 y.o. woman with pancreatic cancer has an IVC filter placed for iliac DVT.

Why is she still at risk of a pulmonary embolus?

ivc dup1.JPG

Our patient had a duplication of the IVC and the L IVC did not have a filter so she was still at risk of a PE. The IVC forms between six and ten weeks gestation. The infrarenal portion of the IVC is formed from the R supracardinal vein while the L supracardinal vein regresses. If the L supracardinal vein fails to regress, two IVCs are formed.  This occurs in 0.2-3% of the population. 

The significance of this anomaly is demonstrated in this case where a single IVC filter was not protective against PE. The anomaly is also associated with horseshoe kidney and malrotation of the gut.

Arrow showing the IVC on the L

Arrow showing the IVC on the L

Duplication occurs in other vascular structures as well The aortic arch can be duplicated causing a complete vascular ring that can compress the trachea or esophagus. The treatment is surgical if patients are symptomatic. Partial duplication can mimic dissection on ultrasound.

double aortic arch on cxr

double aortic arch on cxr

double aortic arch

double aortic arch

Our patient had bilateral DVTs but radiology was unable to place a second filter . Her course was complicated by a GI bleed where her pancreatic tumor was eroding into the duodenum.  The bleeding stopped spontaneously and she was discharged on lovenox.

 

Royal SA, Callen PW. CT evaluation of anomalies of the inferior vena cava and L renal vein. AJR AmJ Roentgenol. 1979;132(5):759-63

Smith TR, Frost A. Anomalous inferior vena cava associated with horseshoe kidneys.Clin Imaging 20(4):276-8.

Shaha P, Garg A, et al. Duplication of inferior vena cava with associated anomalies: a rare case report. J Clin Diagn Res 2016Mar;10(3) doi:10.7860/JCDR/2016/18240.7406.

A 54 y.o. with metastatic squamous cell ca of the tongue presents with RLQ pain.

What is in the RLQ?

What is in the RLQ?

Our patient had typhlitis or neutropenic colitis with a wbc of 200. 

The name comes from the Greek word typhlon or cecum and it is an inflammatory condition involving the cecum in neutropenic patients.  It was first described in children with leukemia but can occur in any immunocompromised patients including lymphoma, aplastic anemia, organ transplant patients and patients with AIDS.

It is diagnosed on the CT when the colonic wall Is greater than 3 mm or there is fat stranding around the cecum.

Cecal wall thickening seen more easily with contrast in typhlitis

Cecal wall thickening seen more easily with contrast in typhlitis

The cause of this neutropenic enterocolitis is not known but it occurs in 5% of  adult neutropenic patients. The cecum is often affected perhaps because of its poor blood supply and its ability to distend.  Microorganisms such as clostridium, pseudomonas, E coli.and   Klebsiella have been found in areas of necrotic bowel suggesting it is an infectious process in an immunocompromised host. It has also been reported in nonneutropenic adults following ingestion of food containing C. Perfingens type A.

Thrombosed R ovarian vein, shown non enhancing with arrows and open arrow showing fat stranding; another cause of RLQ pain.

Thrombosed R ovarian vein, shown non enhancing with arrows and open arrow showing fat stranding; another cause of RLQ pain.

The differential for RLQ pain is broad and includes hydrosalpinx, ovarian cyst, renal stones, diverticulitis, Crohns disease, epiploic appendagitis, ischemic bowel, cancer and  Yersinia, the classic boards question.  Ileocolitis has other infectious causes including salmonella, c diff and Campylobacter.

 

Our pt was treated with filgrastim( a white cell stimulator), and cefepime with flagyl.  He was discharged in 4 days. The mortality in the literature for the condition is 50%.

 

Yu J, fulcher A, Turner M, Halvorsen R. Helical CT Evaluation of acute right lower quadrant pain: part I, common Mimics of appendicitis.Am J roentgenol 2005;184(4):1136-1142.

Sobel J, Mixter CG, Kolhe P. et al. Necrotizing enterocolitis associated with clostridium perfringens illness at a state psychiatric hospital—Louisiana, 2010. MMWR Morb Mortal WKly Rp 2012;61:605.

Mullassery D, Bader A, Battersby A, et al. Diagnosis, incidence, and outcomes of suspected typhlitis in oncology patients—experince in a tertiary pediatric surgical center in the UK. 2009 Joural of Pediatric Surgery 44:381-385.

A 47 y.o. pt with bipolar disorder presents with R eye drainage

What is wrong with her eye?

What is wrong with her eye?

Our patient had an ocular implant after being hit in the eye with brass knuckles and requiring an evisceration for severe pain. The sclera is sewn together over an acrylic implant and a plastic contact lens covers it. The acrylic implant is porous and allows vessels to incorporate into the implant and allows the secure attachment of extraocular muscles improving mobility of the prosthesis. An artificial eye will be added at a later date.

eye implantdia.JPG

Surgical removal of the eye was first reported in the 1500’s. the first report of an implant after enucleation was in 1886.  In the past glass balls were used to replace the eye but more recently porous substances are use. Hydroxyapatite which is created from coral is often used.

ocular implant made from coral

ocular implant made from coral

Rarely,  following eye injury, sympathetic ophthalmia occurs which is thought to be due to an autoimmune inflammatory response toward ocular antigens; a delayed hypersensitivity to melanin in the photoreceptors in the retina. Although rare, this condition results in blindness so high dose immunosuppression is needed.

Treatment is changing for sympathetic ophthalmia since nanoparticles are being use to penetrate the cornea.The corneal epithelium  has multiple layers of epithelial cells which interconnect by tight junctions limiting the penetration of drugs in the past. Recent research has associated active molecules with naonocarriers to overcome ocular barriers and may be important in treating retinal diseases in the future.

Weng Y, Liu J, Jin S, Guo M, Liang X, Hu Z.  Nanotechnology-based strategies for treatment of ocular disease. Acta Pharm Sin B. 2017 May;7(3):281-291.

Phan LT, Hwang TN, McCulley TJ. Evisceration in the modern age. Middle East Afr J Ophthalmol. 2013 Jan:19(1):24-33.

Jordan DR.  Problems after evisceration surgery with porous orbital impalnts: experience with 86 patients. Ophthalmic Plast Reconstr Surg 2004 Sep 20(5):374-80.

Sami D, Young S, Petersen R. Perspective on orbital enucleation implants. Surv Ophthalmol 2007;52(3):244-65.