What does the CT show?
hint: there is some air where it does not belong.
The patient originally had pancreatitis secondary to hypertriglyceridemia from his diabetes with triglycerides of 4,000 . He presented with sepsis and biliary obstruction.
CT shows severe pancreatitis with biliary duct dilation and thick walled gallbladder. There is pneumatosis of the loops of transverse colon and a large amount of air in the peripancreatic fluid collection concerning for fistulization of the collection to the adjacent colon. His original biliary stent had migrated and because of duodenal stenosis, ERCP could not be performed and a bilary stent was placed percutaneously by IR. Portal, splenic and mesenteric vein occlusions were noted.
Pancreatitis results in arterial and venous complications as well as localized necrosis and inflammation of the bowel. Thrombosis of the splenic vein, portal vein or SMA can occur. This is thought to be due to venous congestion/stasis and mass effect of the inflamed pancreas with damage to the vessel wall by pancreatic enzymes. Severe pancreatitis can be associated with multiple organ failure and death.
The most common cause of pancreatitis is gallstones (40%) with alcohol consumption at 24-27%
Alpha Oumar Toure, Sosmane K, Ibrhima K et al. Isolated superior mesenteric vein thrombosis in acute pancreatitis: a case report. Case Rep Clin Med. 2015;4.
Chua T, Walsh M, Baker M, Stevens T. Necrotizing pancreatitis: diagnose, treat ,consult. Cleveland clinic Journal of Medicines. 2017 Aug ;84(8):639-648.
Boumitri C, Brown E, Kahaleh M. Necrotizing Pancreatitis: Current Mangement and Therapies. Clin Endosc. 2017 Jul 50(4):357-365.
What is the diagnosis?
Our patient was transferred in with the diagnosis of sigmoid volvulus on CT. GI spent 2.5 hours trying to decompress the sigmoid before it was recognized that she had a cecal volvulus and needed surgery. What is the difference between the two?
A cecal volvulus is caused by rotation of the cecum usually to the LUQ with 1-3 percent of large bowel obstructions caused by a cecal volvulus. The rotation can also occur above the cecal valve causing a torsion in the ascending colon. There is a defective peritoneal fixation of the ascending colon in 10% of the population putting them at risk for malrotation. People with obstructive lesions in the L colon are also at risk for cecal volvulus. The “ whirl sign” can be seen in this patient; the rotated mesentery in the center of the patients xray.
Sigmoid volvulus accounts for 2-50% of large bowel obstruction with the higher number found in Eastern countries. It usually affects adults and is more common in males. The sigmoid rotates on itself and the loop is seen in the mid abdomen or the RUQ forming the “coffee bean” sign. People with chronic constipation may be more at risk. Sigmoid volvulus can often be detorsed but surgical resection is most commonly recommended at a later date. A barium study can differentiate the two types of volvulus.
Our patient was taken to the OR and had a dead right colon from her cecal volvulus. This was resected and she did well.
Frank AJk, Goffner LB, Fruauff AA. Cecal volvulus: the CT whirl sign. Abdom Imaging 1993.18(3):288-9.
Rosenblat J, Rozenblit AM, Wolf E, DuBrowRA, et al.indings of cecal volvulus at CT. Radiology. 2010 Jul. 256:169-75.
Yoo SJ, Park KW, Cho SU. Definitive diagnosis of intestinal volvulus in utero. Ultrasound Obstet Gynecol. 1999 Mar.13(3).
Atamanalp, S Sigmoid Volvulus 2010 Eurasian J ed 42(3):142-147
What is wrong?
Our patient sustained a uterine rupture after a motor vehicle collision.
While uterine rupture is a rare cause of mortality in the US, it is frequent in Africa, presumably because of the limited access to C sections (it accounts for 36% of maternal deaths in the last 10 years in Ethiopia). Uterine rupture occurs in 0.6-1.6% of patients with blunt abdominal trauma. Fetal mortality approaches 100% in these cases, with maternal mortality at 10%. It is estimated that only 10-37 minutes of viability remain for the fetus after uterine rupture if the fetus is expelled from the uterus. If the fetus remains in the uterus even though it is ruptured, a viable infant may be delivered.
Trauma affects 6-7 % of preganancies in the US. While uterine rupture causing fetal demise is not common, abruption of the placenta is seen more frequently. Ultrasound detects only 40-50% of post-traumatic abruptions, so clinical suspicion must remain high and tocometer monitoring should be performed in cases of trauma.
There is no universal treatment for uterine rupture, but total hysterectomy, subtotal hysterectomy, or repair of the uterus are options.
In the case of this patient, the infant had no heartbeat on arrival, and the mother was resuscitated and taken to the OR for uterine repair. During the ex-lap, a small liver laceration was repaired along with the uterus, and the patient recovered. Thanks to Dr. Creel for this case.
Woldeyes W, Amenu D, Segni H. Uterine rupture in pregnancy following fall from a motorcycle: a horrid accident in pregnancy—a case report and review of the literature. 2015. Case Reports in Obstetrics and Gynecology vol 2015, article ID 715180. http://dx.doi.org/10.1155/2015/715180.
Smith JF, Wax JR. Rupture of the unscarred uterus, Up to Dat 21.2, March 2012. http://uptordate.com/contents/rupture-of-the -unscarred-uterus.
Njoku O, Joannes U, Christian M, Azubike O. Trauma during pregnancy in a Nigerian setting: patterns of presentation and pregnancy outcome. International Journal of Critical Illness and Injury Science,2013 vol 3., no 4, pp269-273.
What needs to be done?
Globally there are millions of snake bites annually with 2,000 deaths. The poisonous snakes of North America include cottonmouths, rattlesnakes, coral snakes and copperheads. The tiger rattlesnake near the Arizona-Mexico border has the most potent bite, and the copperhead has the least. Rattlesnakes are known for their hemotoxic properties. The sea snake, Mohave rattler, coral snake (including Mambas) and cobra family of snakes have venom with predominantly neurotoxic characteristics.
While rattlesnakes have been thought of as containing mainly hemotoxic venom components, there has been a shift to a more neurotoxic venom requiring more antivenom to treat. Only rattlesnakes with more potent neurotoxin will be able to feed on hemotoxic resistant squirrels, mice, and small animals, which gives them an evolutionary advantage.
The snake which bit our patient was a Protobothrops elegans, which is from the Ryukyu Islands in Japan, and was the patient’s own snake. The patient was a snake breeder and was accidentally bitten. At the outside hospital, the patient was given Crofab antivenin, which has no effect on Protobothrops elegans. The effects of its venom were mainly local and he was managed with multiple fasciotomies of the hand. He continues to recover.
The most toxic venom of any snake is the Fierce Snake or inland Taipan. Its bite contains enough venom to kill 100 people. World wide the saw-scaled viper (carpet viper), cobra, common krait, and Russel’s viper in India are responsible for most of the world’s snakebite fatalities.
Laustsen A, Lomnte B, Lohse B, Fernandez J, et al. Unveiling the nature of black mamba venom through venomics and antivenom immunoprofiling Identification of key toxin targets for antivenom development. Journal of Proteomic 2015. 119:126-142.
Spawls S, Branch B. The dangerous snakes of Africa; natural history, species directory, venoms, an dsnakebite. 1995 Dubai:Oriental Press: Ralph Curtis-Books. Pp 49-51. ISBN 978-0-88359-029.
So S, Murakami T, Ikeda N, Chijwa T. Identification and evolution of venom phospholipase A2 ingibitors from Protobothrops elegans serum. 2011. Biochemistry and Molecular Biology Regular papers. 2011. https://doi.org/10,1271.bbb.100676
What is wrong?
Our patient had lymphatic fluid draining from the R chest wound. While thoracic duct injury is most often noted in the L chest; there is significant lymphatic drainage on the R as well which can be injured by thoracic surgery or neck dissection. The typical L sided thoracic duct occurs in only 65% of the population due to variations which include multiple thoracic duct branches.
Another cause of chylous effusion is central line placement. Cases have been reported of chylous effusions after IJ placement. In addition to trauma; chylothorax can occur after childbirth, in association with lymphoma (accounts for 70% of non traumatic cases) , sarcoid, thoracic goiters, amyloidosis, filariasis, SVC thrombosis and yellow nail syndrome which is a congenital abnormality of the lymph system.
Injury to the thoracic duct can result in cervical chylous fistula, chylothorax or chylopericardium. Since 2.4 L of chyle is transported through the lymp system every day; a large effusion can rapidly accumulate causing dyspnea. .
The diagnosis is made by sending triglycerides on the fluid and the cell count should be lymphocyte predominant. Both of our patients were diagnosed in the ED by sending triglycerides on the fluid
Conservative management of a lymphatic injury includes low-fat diet, TPN and drainage of the chylothorax. Failing conservative management results in either lymphangiogram with embolization of the leak or thoracic duct ligation surgically.
Our first pt had a lymphangiogram demonstrating leakage of lymph and underwent repair of his subclavian stenosis. The drainage stopped. The second patient was presumed to have chylothorax from malignancy and was referred to oncology.
Kumar S, Kumar P. Thoracoscopic management of thoracic duct injury: Is there a place for conservatism. J Postgrad Med. 2004 Jan-Mar ;50(1): 57-9.
Saxene P, Shankar S, Kumar V, Naithani,N. Bilateral chylothorax as a complication of internal jugular vein cannulation. Lung India. 2015 v 32(4) July-Aug . 370-74.
McGrath E, Blades X, Anderson P. Chylothorax: Aetiology, diagnosis and therapeutic opaions. Respiratory Medicine. V 104 (1) Jan 2010:1-8.
Nair SK, Petko M, Hayward MP. Aetiology and management of chylothorax in adults. Eur J Cardiothorac Surg. 32(2). 362-369.c
What do you notice on the abdominal CT?.
Our patient had pneumobilia. This can be a benign condition or it may be a serious condition. In the case of our patient he had a bile duct injury in the past and underwent a bepatico-duodenostomy which explains the air in the biliary tree. Other common conditions associated with pneumobilia include: an incompetent sphincter of Oddi or a spontaneous biliary enteric fistula. Serious conditions include gas forming organisms or an emphysematous gall bladder.
Pneumobilia is to be distinguished from gas in the portal venous system. This can indicate severe abdominal pathology requiring urgent operative intervention as in cases of ischemic bowel. . Gas in the portal venous system was first described by Wolfe in infants with necrotizing enterocolitis. Portal venous gas tends to flow to the periphery of the liver( within 2 cm of the liver edge) while biliary gas is more central.
Our patient was admitted with fever and found to have pneumonia. He was successfully treated and discharged. Incidentally, large a large amount of stool was seen in the colon compatible with his known chronic constipation.
Sherman SC, Tran J. Pneumobilia: benign or life-threatening. J Emerg Med 2006 Feb;30(2):147-53.
Marshall SF, Polk RC. Spontaneous internal biliary fistula .Surg Clin Normth AM 1958;38:679.
Abboud B, Hachem J, Yazbeck T Doumit C. Hepatic portal venous gas: Physiopathyology, etiology, prognosis and treatment. World J Gastroenterol. 2009 Aug 7;15(29): 3585-3590.
What do you notice and what should be done?
Our patient was injected with water under high pressure. The air in the leg was a result of that high pressure injection. The extent of damage depends on a number of factors, but the most important determinant the chemical composition of the injected material. Paint and paint thinner cause a significant inflammatory response and result in a high percentage of amputations. Grease injuries case a small inflammatory response but are associated with scarring and loss of function when they occur in the hand. The amount of material injected determines contributes to the compartment pressure, so as more material is injected, the possibility of compartment syndrome increases.
Initially, a high pressure injury may not look bad. There can be either an innocuous entrance or no visible break in the skin. Several hours later, the involved area may become extremely painful because of vascular compromise and tissue necrosis. Our patient had been seen in an ED the day prior to coming here and was given Bactrim and told to elevate the leg. He continued to have pain, and this is what brought him to our ED. The leg was quite swollen when he arrived. He was given antibiotics, managed without surgery, and discharged in three days.
Another case of a high pressure water hose injury was seen at Children’s Hospital. A four year old tried to drink out of a power washer hose and injected water into the R tonsillar pillar. He had significant air in the mediastinum and neck but did not require surgery.
FOR PHYSICS NERDS
Medium duty power washers range in pressure from 1,300-2,000 psi and are used for cleaning driveways. Heavy duty washers have 2,700-4,000 psi and can strip paint. Paint guns can generate 3,600 psi, and a garden hose has 40 psi of pressure.
Duplechain JK, Espinola T, Miller RH. Water spout injection into the neck. Arch Otolaryngol Head Neck Surg. 1993;119:237-38.
Sampson C, High-pressure water injection injury. Int J Emerg Med 2008. Jun 1 (2) 151-154.
Verhoeven N, Hiemer R. High-pressure injection injury of the hand: an often underestimated trauma: case report with study of the literature. Strategies Trauma Limb Reconstr 2008 Apr.3(!):27-33.ps
what is wrong?
Our patient had holoprosencephaly. This is a developmental abnormality where the embryo fails to develop into two hemispheres. The prosencephalon refers to the forebrain of the embryo and develops in the fifth week of pregnancy along with the face. Most cases are not compatible with life and result in death in utero In less severe cases, babies are born with facial deformities affecting the eyes ,nose and upper lip. Some cases result in normal facies with an absent falx and/or corpus callosum. The cause is not known but mutations in the gene encoding the Sonic Hedgehog protein which directs the development of the central nervous system can cause holoprosencephaly.
Our patient had abnormal brain development with normal facies. He had cervical spondylosis with myelopathy and was tetraplegic. He had congenital hydrocephalus requiring a VP shunt at birth, an occipital encephalocele and seizures. He was 36 years old and cared for by his mother at home.
He came in with a complaint of SOB and tachycardia, PE protocol was negative and his tachycardia was attributed to cholecystitis with a sludge filled gallbladder.
1. Totori-Donali, P, Ross A. 2006 Brain Malformations . In Totori-Donati, Paolo, Raybaud C. Pediatric Neuroradiology: Brain, Head, Neck and Spine. 1. Springer pp92-95. ISBN 3-540-41077-5.
2. Rash BG, Grove EA Patterning the dorsal telencephalon: a role for sonic hedgehog? The Journal of Neuroscience , 27(43): 11595-603. Do1:10.1523/neurosci.3204-07 PMID 17959802.
3. Muenke, M, Beachy P. june 2000 Genetics of ventral forebrain development and holoprosencephaly. Current Opinon in Genetics & Development 10(3) 262-9. Doi 10.1016/s0959-437x(00)00084-8.
What is the circular finding on her MRI at L4-5 ? she has no weakness or sensory loss.
Our patient had a synovial cyst. Once again Dr. Docherty is correct) A synovial cyst in the spine is a fluid filled sac resulting from degeneration of a facet joint. They were first described in 1885 by Baker( who also described the one behind the knee). On MRI they appear well demarcated and are extradural. They are more common in women. The cysts are most often found at the L4,5 level because that is the area of maximum mobility. They grow very slowly and often are asymptomatic but they can cause radicular symptoms, spinal stenosis or even cauda equina.
Surgery is indicated for intractable symptoms. The complications of surgery include cerebrospinal fluid fistula, discitis, epidural hematoma, seroma, deep vein thrombosis and death. Our patient is scheduled for surgery because of intractable pain.
Khan A, Girardi F. spinal lumbar synovial cysts. Diagnosis and management challenge. Eur Spine J v 15(8) 2006:1176-1182.
Baker W. formation of synovial cysts in connection with joints. St. Batholomews Hospital Reports. 1885;21:177-190.
Baker W. On the formation of synovial cysts in the leg in connection with disease of the knee-joint. 1887. Clin Orthop. 1994;299:2-10.
Lyons M, et al. Surgical evaluation and management of lumbar synovial cysts: the Mayo Clinic experience. J Neurosurg. 2000;93.1(supple):53-57.
What could this be?
Our patient had a sternoclavicular joint infection unrelated to her car accident. She is an IV heroin user. With the increase in IV heroin use this type of infection is increasing in incidence. This used to be a rare occurrence with 225 cases reported in the past 45 years but recently it has become more common.
The sternoclavicular joint is composed of the medial head of the clavicle, the outer aspect of the manubrium and the cartilage of the first rib with great vessels lying immediately beneath. The complications of sternoclavicular joint infection include: mediastinitis, osteomyelitis or chest wall abscess.
Of all sternoclavicular joint infections. 17% are found in IV drug users. Diabetics, patients with chronic renal failure and patients with previous arthritis at the joint are more likely to get infections. It is postulated that bacteria may enter the sternoclavicular joint from the adjacent valves of the subclavian vein or the joint may be infected by injecting between the heads of the sternocleidomastoid muscle. En-bloc resectin of the sternoclavicular joint is indicated with a pectoralis muscle flap used to close the defect.
Our patient underwent surgical debridement and is on long term antibiotics. Her culture grew MSSA.
Muesse J, Blackmon S, Wllsworth W, Kim M. Treatment of sternoclavicular joint osteomyelitis with debridement and delayed resection with muscle flap coverage improved outcomes. volume 2014. Surgery Research and Practice. Article ID 747315 http://dx.doi.org/10.1155.2014/747315.
Murga A, Copeland H, Hargrove R, et al. Treatment for sternoclavicular joint infections: a multi Our patient underwent surgical debridement and is on long term antibiotics. Her culture grew MSSA.
-institutional study. 2017. Journal of Thoracic Disease. Jun; 9(6):1503-1508. Doi:10.21037/jtd2017.05.76
Ross J, Shamsuddin H. Sternoclavicular septic arthritis: review of 180 cases. 2004. Medicine May;84(3):139-48.
What do you notice?
Our patient had tonsillar abscesses caused by mononucleosis. These are to be differentiated from peritonsillar abscesses because they do not require drainage.
Infectious mononucleosis is a viral infection caused by Ep[stein-Barr virus which presents with sore throat, lymphadenopathy and atypical lymphocytes in the peripheral blood. Spontaneous resolution often takes 4-6 weeks. Tonsillar abscesses may develop when infection from acute tonsillitis spreads into the the peritonsillar space or causes an abscess of Weber’s glands, which are minor salivary gland in the supratonsillar space. 1% of patients with mono develop peritonsillar abscesses. Most pertonsillar abscesses are bacterial often Strep.
Clinically a peritonsillar abscess presents with trismus, soft palate deviation away from the infected side with uvular deviation and lymphadenopathy. The differential includes lymphoma, carcinoma of the soft palate and minor salivary gland tumors. Quinsy was the original term to describe a peritonsillar abscess. It comes from the French word esquinacie.
If peritonsillar abscesses are untreated they can lead to jugular vein thrombosis, cavernous sinus thrombosis, and dissection into the internal carotid artery.
Our patient did not require drainage but did get antibiotics as prophylaxis for peritonsillar abscess. He was discharged on augmentin after three days.
Fusano C, Chundnofsky C, Vanearbeek P. Bilateral peritonsillar abscess: not your usual sore throat. J Emerg Med. 2009 (29) 45-47.
Wadhera R, Kalra V, Gulati S, Ghai A. Infectious mononucleosis presenting with spontaneous rupture of bilateral quinsies. 2013 Egyptian Journal of Ear , Nose Throat and Allied Sciences. 14;45-46.
Flores C, Matthews M, Caruso D, Foster K, Stroschein M. A rare case of peritonsillar abscess resulting in cervical necrotizing fasciitis. 2017 Otolaryngology Case Resports Volume 5; 1-5.
Mohamad I, Yroko A. Peritonsillar swelling is not always quinsy. 2013 Malaya Fam Physician 8(2): 53-55.
What could these painful lesions be?
Our patient had cellulitis from a cat bite.
In a Mayo clinic study 1/3 of people presenting with cat bites to the hand had to be hospitalized with 85% of bites occur on the hand or wrist where they may pierce a joint or tendon sheath. Antibiotics are unable to penetrate a tendon sheath and surgery is required.
In wounds that were cultured in Dr. Talan’s study, aerobes alone were isolated from 36 percent and anaerobes alone from 1 percent so most were mixed infections. Pasteurella multocida was the most common isolate of cat bites involving 75% of cat bites. Other common aerobes included strep, staph, moraexella and Neisseria. Anaerobes included fusobacterium, bacteroides, porphyromona and prevotella. Patients were most often treated with a beta lactam antibiotic and a beta lactamase inhibitor. Usually amp/sulbactam. Clindamycin does not treat Pasteurella. Pasteurella is also susceptible to amp, pcn, doxy, sulfa, fluroquinolones and azithromycin.
Our patient was pcn allergic and treated with clinda and ceftriaxone for cellulitis.
It was felt that she did not have cat scratch fever since it usually present with swollen lymph nodes near the site of the bite which had occurred on the heel and her adenopathy was in the inguinal region. Cat scratch fever, caused by Bartonella henselae , occurs one to three weeks after the bite( sometimes as long as two months later) It is associated with chills and joint pains. Rarely, encephalitis, endocarditis, optic neuritis or oculoglandular syndrome can occur. Immunocompromised patients are most susceptible to these infections. Cat scratch disease can be treated with azithromycin, sulfa or Cipro. There is a PCR test available with a sensitivity of 43-76% but the diagnosis can also be made on lymph node biopsy.
Babovic N, Cayci C, Carlsen B. Cat bite infections of the hand: assessment of morbidity and predictors of severe infection. J Hand Surg Am 2014 Feb 39(2) 286-290. DOI 10.1016/JHSA2013.11.003
Talan D, Citron D, Frederick M, Abrahamian , D, Moran G, Goldstein E. Bacteriologic analysis of infected dog and cat bites. NEJM 1999, Jan 14.
Goldstein E, Citron D, Hudspeth M, Gerardo S, Merriam C. Trovafloxacin compared with levofloxacin, ofloxacin, Cipro, azithro and clarithro againt unusual aerobic and anaerobic human and animal bite-wound pathogens. J Antimicrob Chemother 1998;41: 391-396.
Rolain J, Brouqui P, Koehler J, Maguina C, et al. Recommendations for treatment of human infections cased by bartonella species. 2004 Antimicrobial Agents and chemotherapy 48(6) 1921-1933.
Our patient had leukocytoclastic vasculitis which is a hypersensitivity vasculitis. This is a small vessel vasculitis and leukocytoclasis refers to the vascular damage caused by infiltrating neutrophils. It may be caused by by underlying collagen vascular disease, medications, malignancy or infections. In our case the patient had a previous group B strep infection as well as herpes. Both have been shown to cause leukocytoclastic vasculitis.
While leukocytoclastic vasculitis often involves the skin; it can also involve joints, the GI tract and the kidneys. Pathologically, autoantibodies including ANCA and tumor necrosis factor attract neutrophils. One type of leukocytoclastic vasculitis involves IgA deposition in the skin and joints. This is called Henoch Schonlein vasculitis. It is common in children under age 10 but also occurs in adults.
The skin rash often lasts for weeks and if it involves only skin no treatment is needed. In cases of severe joint pain colchicine can be used because it has an antineutrophil effect. Steroids and biologic immune suppression are used in serious cases of organ involvement. Our patient was treated for herpes and his rash resolved although he still had joint aches at two weeks post treatment.
Lotti T, Ghersetich I, Comachi C, Jorizzo J. Cutaneous small vessel vasculitis. J Am Acad Derm. 1998. Nov 39(5) 667-87.
Jeannett J, Falk R. Small vessel vasculitis NEJM 1997 Nov 20 337 (21)1512-23.
Our patient had photodermatitis. This is a form of allergic contact dermatitis is which the allergen is activated by sunlight.
Causes of photodermatitis
Sulfa, psoralens, tetracyclines, benzoylperoxide, retinoids, amiodarone, ibuprofen , naprosyn
Lemon oil, PABA (found in sunscreens). Hexachlorophene( found in antibacterial soaps)
Plant related photodermatitis
Citrus fruits including limes and grapefruit of the Rutaceae family , psoralens found in celery, carrot or parsley( the family Apiaceae)
Pellagra- lack of B3 either by lack of the vitamin in the diet or inability to use the niacin consumed.
Pellegra is common in people who eat a corn based diet when the corn is not processed with alkali which makes niacin available for absorption.The name comes from Pelle agra, Italian for rough skin. It was first described in 1735 in Spain by Gaspar Casal. Pellegra was widespread in the American south in the 1900s with more than 3 million people affected. Dr. Joseph Goldberger traced the disease to corn by feeding prisoners cereal diets until they developed the classic skin lesions. Now corn is processed with alkali to make the niacin bioavailable.
Pellegra is often seen in alcoholics, end stage HIV, malabsorption and carcinoid syndrome (due to excess turnover of tryptophan, precursor of niacin, to serotonin)
Our patient was treated with nicotinomide and all her herbal supplements were stopped. She had also been on high doses of grapefruit powder and shitake mushroom powder. Her rash resolved after a week in the hospital. Below is a chart of the timeline of vitamin deficiencies.
Rodriguez E. Valbuena M, Rey M Porras de Quintana L. 2006 Causal agents of photoalleric contact dermatitis diagnosed in the national institute of dermatology of Colombia. Photodermatol Photoimmunol Photomed 22(4) 189-192.
Ngan V. 2003 Pellegra DermNet New Zealand, April 9 2017.
Hegyi J, Schwartz, R, Hegyi V. Pellegra: Dermatitis, dementia, and diarrhea. International Journal of Dermatology 43(1) 1-5. doi 10.111/j 1365-4632. 2004.01959 PMID 14693013.
Savvvoula S, Pellegra: A non-eradicated old disease, Clin Pract 2014 March 27, 4(1). 637
what do you notice on CT?
Our patient had heterotaxy syndrome with polysplenia. The word is derived from hetero meaning “different” and taxy meaning “arrangement”.Heterotaxy syndrome is an embryologic abnormality where organs and vessels do not follow the standard developmental pattern. It occurs in 1.4 per 10,000 infants. This is also known as situs ambiguous and is categorized based on the number of spleens. Asplenic patients have a higher incidence of congenital heart disease which leads to earlier detection of the syndrome.
Even though there are many spleens in some patients, the spleens may not be functional and these patients are at risk for sepsis with encapsulated organisms.
Applegate KE, Goske MJ, Pierce G, Murphy D. Situs revisited. Imaging of the heterotaxy syndrome. Radiographics. 1999, 19:837-852
Peoples WM, Moller JH, Edwars JE. Polysplenia: A review of 146 cases. Pediatr Cardiol. 1983;4:129-137.
Lin AE, Ticho BS, Houde K, et al. Heterotaxy. Associated conditions and hospital-based prevalence in newborns. Genet Med 2000;2:157-172.
Cupers S, Va Linthout C, Desprechins B, et al. Heterotaxy syndrome with intestinal malrotation, polysplenia and azygos continuity. Clin Pract 2018. Jan8;8(1), 1004.
What is the metallic object in her pelvis?
Our patient had an endoscopy capsule camera lodged in the terminal ileum. Capsules are traditionally used to detect sites of GI bleeding, diagnose cancer in the small bowel, diagnose celiac disease and screening for polyps. In the case of our patient she had a stricture of the terminal ileum and the camera lodged there.
The camera captures two pictures per second which are sent by a radio transmitter to a recorder that the patient wears on a belt. A number of electrodes are attached to the patients abdomen similar to an EKG. After 8 hours the batteries are depleted and the 55,000 images are “glued" into a video which is then downloaded on to a computer. The first capsule, for the small bowel, was FDA approved in 2001. There are two other type of capsules; esophageal and colonic. The indications for the esophageal capsules are Barrett’s esophagus and esophageal varices. These capsules have not become popular because of the prevalence of UGI endoscopy so 90% of the capsules are small bowel type.
Capsule endoscopy of the small bowel is more sensitive than small-bowel follow through xrays which is still the only way to image the small bowel. In patients with possible strictures a dissolvable “patency capsule” can be ingested first to see if it will pass. The pill camera contains metallic batteries and can cause a perforation so removal is recommended. It can also interfere with pacemakers.
Our patient underwent ileocolic resection with removal of the capsule camera because of recurrent pain, She has done well post op
Capsule endoscopy costs $919-$2,379. This is compared to $1,500 to $10,000 for upper GI endoscopy.
Mergener K, Update on the use of capsule endoscopy. 2008 Gastroenterol Hepatol. Feb;4(2): 107-110.
Marmo R, Rotondano G, Rondonotti C, et al. Casule endoscopy vs other diagnostic procedures in diagnosing obscure gastrointestinal bleeding: a cost-effectiveness study. Eur J Gastroenterol Hepatol. 2007;19:535-542.
Flemming J, Cameron S. Small bowel capsule endoscopy: indications, results, and clinical benefit in a university environment. 2018. Medicine Vol 97(14). P e0148.
Her MRI is shown below and shows an epidural fluid collection anterior to spinal cord .
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.
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.
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.
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
What is in the differential for her R eye pain and blurry vision?
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.
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.
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.
What do you think she has?
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.
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.
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.