What do you see?
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.
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.
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)
how would you treat her minimally displaced , non articular fibular fracture?
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.
In this case the stress view showed widening of the ankle mortice and required a surgical repair.
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.
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.
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.
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.
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.
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.
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.
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.
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.
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
What do you notice on CT?
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.
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.
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.
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.
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
Infections- malaria, mycoplasma, mono, cmv, HIV, clostridia
Medications (sulfa, penicillin, antimalarial meds)
Autoimmune-SLE, lymphoproliferative disorders
Mechanical heart valves
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.
What does the CT show?
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.
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.
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.
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.
Why is she still at risk of a pulmonary embolus?
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.
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.
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.
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.
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.
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.
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.
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.
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.
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.