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.

 

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.