He returns 12 days later with one day of shoulder pain and flank pain. What happened?
this is her MRI. What does she have?
Our patient has a sacral insufficiency fracture. This is a stress fracture of the sacrum and is a common cause of low back pain in the elderly with osteoporosis who do not experience trauma. Bone scan and MRI are the most sensitive means of detection. Patients are managed conservatively with bed rest and rehabilitation. More recently, sacroplasty( a minimally invasive procedure similar to vertebroplasty) has emerged as an alternative therapy. The sacral insufficiency fracture is found in the sacrum parallel to the SI joint.
The sacral insufficiency fracture was first described by Lourie in 1982. Although they are found most commonly in osteoporosis they can be found in steroid-induced osteopenia, rheumatoid arthritis, paget's and renal osteodystrophy. The incidence may be as high as 89% in patients undergoing pelvic radiation for cervical cancer.
Patients present with low back pain and sacral radiculopathy mimicking cauda equina syndrome. Pain is often in the buttock , hip, or groin not necessarily over the sacrum. They are often associated with pubic rami fractures.
Elderly patients with trauma can also fracture in the same place as those without trauma, due to weakening of the bone. Below, is a patient sp an mvc with a negative pelvic xray and a sacral fracture on CT.
Our pt was managed conservatively.
Lyders EM, Whitlow CT, Baker MD, Morris PP. Imaging and treatment of sacral insufficiency fractures. 2020 AJNR 31 202-210.
Lourie H. Spontaneous osteoporotic fracture of the sacrum: an unrecognized syndrome in the elderly. JAMA 1982;248:715-717.
Blomlie V, rofstad E, Talle K, et al. Incidence of radiation-induced insufficiency fractures of the female pelvis: evaluation with MRI imaging AJR Am J Roentgenol 1996;167:1205-10.
Butler CL, Given CA, Michel SL, Tibbs PA. Percutanwous sacroplasty for the treatment of sacral insufficiency fractures. AJR 2005 184:10956-59.
Our patient had atlantoaxial rotatory subluxation as Dr. Noelker points out. The inferior facet of C1 is displaced completely anterior to the L facet of C2. The right inferior facet of C1 is also displaced anteriorly. This resulted in severe spinal canal stenosis at the level of C1-2 with severe cord impingement and impingement on the R vertebral artery. Our patient however, was neurologically intact. He had an xray prior to his corticosteroid injections showing this was not present.
Did the steroid injections contribute in anyway to the problem? It is known that steroids can cause destruction of joints and ligaments by preventing the inflammation which is necessary for healing. This is why steroid injections are given with three week intervals between them. Steroids also inactivate vitamin D, limit Ca absorption by the GI tract, and increase urinary excretion of calcium. This leads to a decreased uptake of Ca by the bone and ultimately weakness of the fibro-osseous junctions. In addition to delayed healing, the complications of cervical steroid injections as found in the malpractice literature include:
Quadriplegia, respiratory arrest, cardiac arrest, dural puncture, vertebrobasilar stroke, epidural hematoma, and cervical cord infarct.
Quadriplegia which was permanent has been reported with an intravascular injection. Transient quadriplegia has been reported with injection into a cervical radicular artery. In this report, the patient was quadriplegic for only 20 minutes . The authors state although injecting a local anesthetic may have only a temporary effect on spinal cord function, particulate steroids may act as an embolus and cause permanent impairment.
Respiratory arrest can be transient if lidocaine causes a high cervical block, so most physicians who give the injections just numb the skin and give steroids alone after that to avoid respiratory arrest.
Causes of cardiac arrest have been reported including blockade of the sympathetics leading to a bradycardic arrest and involvement of the phrenic nerve leading to apnea.
Our patient had surgery and is doing well. It is not known whether the steriods had any role in his condition. Thanks to Dr. Baumgartner for the case.
Sofat N, Kuttapitiya A. future directions for the management of pain in osteroarthritis. Rheumatol. Apri 2014;9(2):197-276.
Epstein NE. The risks of epidural and transforaminal steroid injections in the Spine: Commentary and a comprehensive review of the literature. Surg Neurol Int 22-Mar-2013;4:.
Abram SE, O’Connor TC.:: Complications associated with rpidural sterid injections. Reg Anesth 1996;21:149-62.
Karasek M, Bogduk N. Temporary neurologic deficit after cervical transforaminal injection of local anesthetic. Pain Med. 2004;5:202-5
This is a remnant of our reptilian ancestry called the vomeronasal organ(VNO) or Jacobson’s organ. It is auxillary olfactory sense organ useful for detecting pheromones. It was discovered in 1732 by Frederick Ruysch and later rediscovered by Ludwig Jacobson in 1813. In humans the organ, which is found along the nasal septum, regresses during fetal development but can be seen in about 25% of adults. Image below is from Chapter 41. Physiology of olfaction. DA Leopold and EH Holbrook. Cummings Otolaryngology. 5th edition.
The organ has been widely studied in animals. The vomeronasal organ connects to the olfactory bulb and eventually to the amygdala and hypothalamus. This is extremely important in reproduction and social behavior in animals since it triggers release of hormones needed for mating. The organ can detect both odorants and nonvolatile proteins such as urinary tract proteins. This explains why your dog is constantly sniffing urine from other dogs.
Snakes use this organ to sense prey, sticking out their tongue to gather scents and then touching it to the VNO. Elephants transfer scents from the tip of their trunks to the VNO connection which is located in the roof of their mouths. Cattle and pigs also have a connection to the VNO in the roof of their mouths behind their teeth and they often exhibit the Flehmen response in response to an odor. They lift their heads , wrinkle their noses and cease to breathe momentarily allowing the odor to go through the mouth into the VNO.
Our patient was being scoped for possible bleeding after sinus surgery. Her vomeronasal organ is just a vestigial remnant of evolution. It does not function in humans. Our patient had no nasal bleeding and was found to have an ulcer.
Jacobson L Anatomisk Beskrivelse over et nyt Organ I huusdyrenes naese veternaer-selskapets skrifter 1813. 2, 209-246.
Stoyanov G, Moneva K, sapundzhiev N, Tonchev AB. The vomeronasal organ incidence in a bulgarian population. 2016. The Journal of Laryngology & Otology. 130:1-4.
Trotier D. Vomeronasal organ and human pheromones. Annales francaises et de Pathologie Cervico-faciales. 2010. Vol 128 (4)222-28
What do you notice on her C-spine CT?
Our patient had an occipital condyle fracture seen in the figure below. Occipital condyle fractures are uncommon but can have fatal outcomes if missed. The first description of a death reported from a missed fracture was in a patient who was being discharged and turned his head suddenly. He arrested and autopsy showed a fracture of the occipital condyle which had been displaced into the brainstem. In large autopsy series of fatal car crashes, occipital condyle fractures have been reported at 4.2% .
Occipital condyle fractures are often seen in association with palsies of the 12th cranial nerve due to fracture through the hypoglossal canal. Because of the proximity of the jugular foramen, nerves IX,X, and XI can also be involved. Delayed nerve damage has been reported up to nine weeks after the injury because of dislocation of bony fragments.
Conservative management is the standard of care unless there is severe displacement of fragments. The duration of immobilization is three months. Unstable fractures may be treated with a Halo or occipito-cervical fixation. Interestingly, the MRI did not show the bony fragment but showed edema centered around the L occipital condyle and surrounding apical odontoid ligament and tectorial membrane consistent with an acute fracture. There was no cord signal abnormality. Our patient was treated with a hard collar.
Bell C. Surgical observations. Middlesex Hosp J. 1817;4:469-470.
Caroli E, Rocchi G, Orlando ER, Delfini R. Occipital condyle fractues: report of five cases and literature review 2005 Eur Spine J. Jun;14(5) 487-92.
Alker GJ, Oh US, Leslie EV. High cervical spine and cranio-cervical junction injuries in fatal traffic accidents: a radiological study. Orthop Clin North Am. 1978;0:1003-1010.
Bucholz RW, Burkhead WZ. The pathological anatomy of fatal atlanto-occipital dislocations. J Bone Joint Surg Am. 1979;61:248-250.
What caused this problem?
Our patient had corneal destruction from chronic methamphetamine use. This resulted in descemetoceles ( outpouching of the inner layer of the cornea). Drug induced ocular symptoms also include retinal vasculitis, episcleritis, panophthalmitis, endophthalmitis, scleritis, and globe rupture.
In the initial stages of chemical eye burns irrigation and correction of the pH to between 7.0 and 7.2 should be accomplished. Fluorescein staining may be absent with a complete epithelial defect. In mild burns a topical steroid is applied four times a day for one week to control inflammation. A cycloplegic is also given three times per day. In more severe burns prednisolone is given q 1 hr while awake and tapered rapidly between days 10 and 14 to minimize the risk of corneal melting. Amniotic membrane is often used to cover the cornea in severe burns. In some cases limbal stem cell transplants are performed from the contralateral eye.
Our patient will be receiving a corneal transplant.
Hazin R,Cadet JL, Kahook MY, Saed. Ocular manifestations of crystal methamphetamine use. 2009 Neurotox Res. 15(2):187-91.
Hemmati H, Colby K, edited by Scott I and Fekrat S. Treating acute chemical injuries of the cornea. 2017 Eyenet Magazine.
Our patient has wrist pain because of a scapholunate ligament tear. This is the most common ligament injury in the wrist. Normally the scaphoid and lunate move together but when a scapholunate tear occurs the scaphoid flexes and the lunate extends. This causes pain in the wrist. There are four classes of injury: 1 occult seen only on MRI, 2. Dynamic showing up on stress xrays or MRI, 3. Static visible on plain films as a gap between the scaphoid and lunate and 4. Advanced collapse where the ligament is completely torn and the injury has been there for years causing arthritis.
The pt may complain of pain while doing pushups or any activity that loads the wrist. A stress xray may be performed by making a tight fist for one xray and then bending the wrist towards the small finger for another xray and then bending toward the thumb for a final xray. A gap will occur at the scapholunate junction during activity if there is an injury. If there is a widening of 3-4 mm this is diagnostic and referred to as the Terry Thomas sign named for a British comedian who had a gap between his two front teeth.
World class tennis players are at risk for these wrist injuries . It is estimated that serves pack rotational velocities of 1,500 degrees per second which is nearing the limits of human physiology. Richard Berger of the Mayo clinic is currently operating on microscopic tears of the scapholunate ligament which are not even visible on MRI in these elite athletes so continued pain is a reason for referral even if there is no radiologic abnormality. These injuries also occur in the more common “fall on outstretched arm” injuries that we see associated with distal radius fractures.
Kuo CE, Wolfe SW Scapholunate instability current concepts in diagnosis and management J Hand Surg 2008;33A :998-1013.
Lew DM Osterman AL. Scapholunate instability in athletes. Clin sports Med 2001;10(1):131-40
Fufa D, Goldfarb C. Sports injuries of the wrist. Curr Rev Musculosketat Med 2013 m6(1) march 35-40
What do you see on the MRI?
Our patient had PRES( posterior reversible encephalopathy syndrome) which was first described in 1996. The image of the MRI shows edema posteriorly on the T2 weighted MRI. The reason for PRES in this case was autonomic dysreflexia secondary to spinal cord injury. In patients with spinal cord injury above the T6 level there is an imbalance of sympathetic discharge since sympathetic control of the splanchnic vascular bed is no longer functioning.
When there is a noxious stimulus, for example, an enema, there is unopposed activity of the spinal reflex mechanisms that remain intact in spite of the spinal cord injury. The splanchnic bed vasoconstricts and there is a sudden increase in blood pressure.
In our patient this resulted in extreme hypertension with enemas or urologic procedures leading to a seizure and PRES. This can be avoided by applying a topical anesthetic to the rectum prior to an enema or giving a dose of nifedipine.
PRES has also been reported with duloxetine, lupus, pregnancy, chemotherapeutic agents and anti-rejection drugs. In the case of chemotherapeutic agents or antirejection therapy this is thought to be a direct toxic effect on vascular endothelium in the brain.
Garg RK. Posterior leukoencephalopathy syndrome. 2001 Postgrad Med J. 77(903) 24-28.
Fugate J. Rabinstein A. Posterio reversible encephalopathy syndrom. Clinical and radiological manifectations, pathophysiology, and outstanding questions. 2015. The Lancet Neurology 14(9) 914-925.
Hinchey J, Chaves C, Appignani B, et al. A reversible posterior leukoencephalopathy syndrome. 1996. N Engl J Med 334(8) 494-500.
Kur JK, Esdaile JM. Posterior reversible encephalopathy syndrome—an underrecognized manifestation of systemic lupus erythematosus. 2006. The Journal of Rheumatolgy 33(11):2178-83.
Rajasekhar A, George TJ. Gemcitabine-induced reversible posterior leukoencephalopathy syndrome. A case resport and review of the literature. 2007 Oncologist 12(11)1332-1335.
Zappella N, Perier F, et al duloxetine-related posterior reversible encephalopathy syndrome. Medicine 2016 95:33(e4556)
What do you see in his bladder?
Our patient had a prostate abscess. Prostate abscesses are uncommon because of the use of broad-spectrum antibiotics in patients with lower urinary tract symptoms. When it does occur, it is difficult to distinguish from acute bacterial prostatitis but the distinction is important since the treatment differs.
Typical symptoms of abscess include dysuria, fever, and perineal and lower back pain which can mimic prostatitis. The prostatic abscess requires drainage, traditionally requiring an open perineal incision or transurethral resection. Currently minimally invasive treatment with TRUS(trans rectal ultrasound) or drainge via a transperineal aspiration is carried out.
Risk factors for prostate abscess include obstructive uropathy, straddle injury and urethral foreign bodies. HIV, immune suppression, diabetes and chronic indwelling foleys are also risk factors. The most common pathogens are gram negative with E coli and Klebsiella being involved in most cases. Pseudomonas, staph aureus and Strep agalactiea have also been implicated. More recently MRSA prostatic abscesses have been reported. In 2000, a new strain of community acquired MRSA, USA300 Panton-Valentine leucocidin (PVL) positive MRSA was reported. This PVL confers greater virulence increasing the likelihood of necrotizing pneumonia , endocarditis, osteo renal abscesses and prostate abscesses.
Our patient had obstructive uropathy and a MRSA abscess of the prostate. A transrectal ultrasound was used to localize the abscess but it could not be aspirated because of a high bladder neck. Using the rectal US as a guide an 18 gauge needle was used to aspirate the abscess trans perineally. Purulent material was obtained and cultured positive for MRSA. Blood cultures were also positive. The pt was treated with IV antibiotics and recovered without further incident.
Jang K, Lee D, Lee S, Chung B. Treatment of prostatic abscess: cass collection and comparison of treatment methods. 2012 Korean J Urology 53(12): 860-864.
Bhagat SK, Kekre NS, Gopaladrishnan G, et al. Changing profile of prostatic abscess. Int Braz J Urol. 2008;34:164-179.
Lachant D, Apostolakos, Pietropaoli A. Methicillin resistant staph aureus prostatic abscess with bacteremia. 2013 Case reports in Infectious Diseases. Article ID 613961.
What is the differential?
Our patient had bullous pemphigoid. Pemphigoid is an autoimmune blistering skin disease. It is different from pemphigus which is also autoimmune but the skin cleaves within the epidermis making the skin very fragile causing sloughing with minor trauma (Nikolsky’s sign) In pemphigoid the blisters are often firm and resist tearing .
It is the result of an attack on the basement membrane of the epidermis by immunoglobulins and activated T lymphocytes. The target is the protein BP180(also called Type XVII collagen) The protein is found in hemidesmosomes, structures the connect the dermis to the epidermis. The binding of antibodies to the hemidesmosomes destroys them and subepidermal blisters form.
The disease is more prevalent in elderly patients. It can be triggered by drugs or malignancy. It is also more common in people with underlying skin disease like psoriasis.
The differential for blistering diseases is broad.
AUTOIMMUNE- pemphigoid, and dermatitis herpetiformis (caused by gluten sensitivity), linear IgA dermatosis, eczema
INFECTIOUS -varicella, herpes, atypical enterovirus infection, necrotising fasciitis, erysipelas. Reactions to infection like erythema multiforme and staphylococcal scalded skin syndrome also cause blistering .
DRUGS-Stevens Johnson, fixed drug eruption , drug hypersensitivity syndrome.
CONTACT ALLERGIES- poison ivy, cold exposure
METABOLIC CAUSES- porphyria cutanea tarda (an abnormality in heme production)
GENETIC CAUSES- epidermolysis bullosa ( a genetic disease resulting in blistering. Gene therapy has been successfully used in treating this disease in a seven year old boy in Germany. In skin cells from a non blistered area stem cells were extracted, treated with a virus containing a correct copy of the LAMB3 gene , and grown into sheets which were used to cover his body. Blistering has not reoccurred in the grafted areas.
The treatment for bullous pemphigoid is generally steroids which our patient received.
In 2015 a seven year old boy was treated for epidermolysis bullosa with gene therapy. The patient’s cells contained a mutation in the LAMB3gene which affects a protein needed to strengthen the epidermi
Wojnarowska F ,Kitschig G, et al. Guidelines for the management of bullous pemphigoid. 2002 Br J Dermatol 147(2) 214-21.
Schmidt E, Zillikens D. The diagnosis and treatment of autoimmune blistering skin diseases. Dtsch Arztebl Int 2011 . 108(23):399-405
What would be in the differential?
Our patient had lichen planus. The term lichen planus was derived from the Greek word leichen meaning tree moss and the Latin word planus meaning flat. It was first used by Wilson in 1869. This is a skin disease caused by a cell-mediated response of unknown origin. It may be found in diseases of altered immunity , such as ulcerative colitis, vitiligo, dermatomyositis, myasthenia gravis and hepatitis C. It can be caused by medications including diuretics, ACE inhibitors, beta-blockers and NSAIDs. It is also associated with syphilis which is what our patient had.
Lichen planus affects one percent of the general population . It causes scaly raised areas that may occur on the genitals, skin or in the mouth where classically it forms Wichkham’s striae. It can cause ridged nails and is most common in patients in their 30s to 50s. The lesions are intensely pruritic. The differential in this case with penile and scalp lesions would have included lichen planus, psoriasis or lichen sclerosus. Lichen sclerosus presents with a hypopigmented glans with a cellophane-like texture and phimosis.
Pathologically, there is degeneration of the basal layer of the epidermis and a bandlike lymphocytic infiltrate obscuring the dermal epidermal junction. There is irregular epidermal hyperplasia with a saw tooth appearance. The basal layer of skin shows necrosis with keratinocyte damage.The pathology is what distinguishes it from an ID reaction where fungal infections in the feet can cause a diffuse eczematous rash.
It is treated with topical corticosteroids and may last up to a year. Our patient was treated for syphilis and given steroids for his scalp. His phone number has changed and he has been lost to follow up.
J Cutan Pathol 1993 apr;20(2):143-7.
Young SK, Kim MH, Kim CW, et al. A case of palmoplantar lichen planus mimicking secondary syphilis. Ann Dermatol 2009 21(4): 429-431.
Singh JP, Priyadarshi V, Goel H, Vijay M, et al. Penile lichen sclerosus: a n urologist’s nightmare! A singlecenter experience. Urol Ann 2015 7(3): 303-308.
Wilson E. On lichen planus. J Cutan Med Dis Skin. 1869;3:117-132.
Our patient had congenital lobar emphysema. It is a cause of respiratory distress in the neonate, but can also be found in young adults. The diagnosis is often made on cxr. Key findings include hyperlucency on the affected side with atelectasis of the normal lobes. The mediastinum may be displaced away from the affected side and may herniate into the contralateral hemithorax. Lung slide may be absent although there is no pneumothorax. The patient does not need a chest tube. This condition should be distinguished from Swyer- James, which is acquired hyperlucency after an adenovirus infection where the lung does not grow normally.
In two other conditions a chest tube is not needed although it may look like it on first glance. One condition is a large bleb which can mimic a pneumo. The danger of putting a chest tube into such a patient is that a persistent air leak can develop.
CXR with a large bleb below
Another condition that can mimic a pneumo/hemo is a collapsed lung. The image below is from a patient who presented one month ago to the ED. There was a call from the triage doctor stating, “patient is coming to TCC 2 needing an urgent chest tube. “ The image is below and represents a completely obstructed L mainstem bronchus with L lung collapse. A chest tube would not be indicated but rather a bronch.
Our patient underwent a resection of the emphysematous upper lobe and recovered without incident.It was easy to make the diagnosis on history in his case and it was confirmed on CT where lung markings could be seen on the empysematous side..
Stern EJ, Webb WR, Weinacker A, Muller NL. Idiopathic giant bullous emphysema (vanishing lung syndrome) imaging findings. In nine patients . AJR , 162(1994) 279-282.
Boushy SF, Kohen R, Billig DM, Heiman MJ. Bullous emphysema: clinical, roentgenologic and physiologic study of 49 patients. Dis Chest, 1968. 54:327-334.
Gelabert, C, Nelson M. Bleb Point: Mimicker of Pneumothorax in bullous lung disease. 201516(3).447-449.
There is no PE.
Our patient had dependent layering of contrast in the inferior vena cava which is a sign of cardiogenic shock. This also occurs if a patient arrests during a CT and involves not only the IVC but the major tributaries of the hepatic veins.
Indeed, our patient had cardiogenic shock along with an acute occlusion of the R common femoral vein. She had a heart rate of 40 with complete heart block n the ED and a transvenous pacemaker was floated. The patient was taken to the OR for her ischemic leg and then to the cath lab where she was noted to have a right coronary occlusion and RV failure. A right ventricular assist device was placed. Interestingly, the pacemaker placed correctly in the ED became completely entangled in the RV assist device.
In spite of milrinone, vasopressin , norepinephrine and a right ventricular assist device, the patient could not maintain her BP and expired.
An addendum was made to the original CT done in the ED stating the R coronary was occluded. Her initial trop had been normal and peaked at 30.
Roth, C, Sneider M, Bogot N, Todd M , Cronin. Dependent Venous Contrast Pooling and Layering: A sign of Imminent Cardiogenic Shock. American Journal of Radiology. 2006. 186:1116-19.
Tsai PP, chen JG, Huang JL, Shen WC. Dependent pooling : a contrast-enhanced sign of cardiac arrest during CT. AJR. 2002;178:1095-99.
Jana M, Gamnagatti SR, Kumar A. Case series: CT scan in cardiac arrest and imminent cardiogenic shock. Indian J Radiol Imaging. 2010;20:150-53.
Our patient had silicone buttock injections. Since her pain was upper abdomen and involved the R lower lungfield a silicone embolus was considered. How common is a silicone embolus?
Silicon was discovered in 1824 by Jons Berzelius. Silicones are synthetic polymers made by combining silicon and oxygen, which at high temperatures and pressures produce polydimethylsiloxone, injectable silicone.
Silicone is used to enhance body shape and contour; often by illegal practitioners. Specific groups are known to use silicone enhancement :exotic dancers, prostitutes and transgender individuals. Complications of silicone injections are in the range of 1-2% including silicone embolism to the lungs. In 1983 silicone was detected in cells obtained from bronchoalveolar lavage by use of spectrophotometry in patients presenting with acute pneumonia. Since then, many cases of silicone embolism have been reported.
In the case of illegal silicone injections, the proposed mechanisms of embolization include excessive local tissue pressure, local massage by the illegal practitioner, or direct intravascular injection. It is thought that large volumes of silicone result in local tissue damage and the silicone eventually gains access to the bloodstream and this results in pulmonary embolization. This can occur even weeks after an injection.
On CT scan this looks like peripherally distributed ground-glass opacities with interlobular septal thickening similar to eosinophilic lung disease and fat embolism. On plain film, silicone embolus can look like pneumonia and therefore a high index of suspicion is needed to make the diagnosis.
Steroids are the suggested treatment presumably because the silicone incites an immune response in the lung.
Our patient was an exotic dancer and had NO evidence of silicone embolization on CT. Her final diagnosis was gastritis and her pain resolved with proton pump inhibitors.
Teaching point: If you obtain a history of silicone injections and pneumonia; consider embolization. Other complications of silicone injections include renal failure, granulomas and cellultis.
Mendonca D, Leitao D, Friend R, Epelboim J , Eiger G. An unusual case of pulmonary embolism. Respiratory Care Aug 2012 Vol 57 (8) 1346-47.
2. Restrepo CS, Artunduaga M, Carrillo J, Rivera A, Ojeda P, Martinez-Jimenez S, et al. Silixone pulmonary embolism: report of 10 cases and review of literature. J Comput Assist Tomogr 2009. 33(2):233-237.
3. Chung KY, Kim SH, Kwon, et al. Clinicopathologic review of pulmonary silicone syndrome embolism with special emphasis on the resultant histologic diversity in the lung: a review of five cases. Yonsei Med J. 2002;43(2):152-159.
4. Zamorea AC, Collard HR, Barrera L, et al. silicone injection causing acute pneumonitis; a case series. Lung 2009;187(4): 241-244.
What is wrong?
Our patient had rickets with the classic “tie” sign ; increased uptake over the sternum on bone scan.
Rickets results from defective mineralization or calcification of bones due to deficiency or impaired metabolism of Vit D, Mg, phosphorus or calcium. If this occurs prior to epiphyseal closure in children bony abnormalities result. These include bowing of the femurs, scoliosis, and skull bossing.
Rickets was first described in Ephesus in the first century AD when Soranus described it in infants. In 1645, Whistler in England defined it as a specific medical condition. It wasn’t until 1923 thatSteenbock in the US showed that irradiation of food couldincrease the amount of Vitamin D and reverse the disease. Rickets was nearly eliminated from the US by 1945 due to the irradiation of milk.
There are several types of rickets.
1. The most common is Vit D deficient, which is caused mainly by malnutrition often in the mother who is then unable to provide Vit D in her breast milk.
Vit D responsive rickets persists in the rest of the world. The Middle East, despite high rates of sun-exposure has the highest rate of rickets worldwide. This is explained by limited sun exposure due to cultural practices and lack of Vit D supplementation in nursing women. 80% of girls in Iran and Saudi Arabia are Vit D deficient.
2. Our patient had a second type of rickets ; Vit D resistant rickets or X linked hypophosphatemia. This is a mutation in the X chromosome causing overactivityof fibroblast growth factor and decreased phosphate resorption by the kidneys.
3. A third type of rickets is caused by fibroblast tumors. In these individuals a tumor produces fibroblast growth factor and decreased absorption of phosphate in the kidneys. In these cases removal of the tumor cures the disease.
Our patient had scoliosis and multiple microfractures on bone scan. On her current ED visit plain films showed no new fracture in the back and she was given symptomatic treatment.
Imaging in all types of rickets shows pseudofractures which light up on bone scan where there is a stress fracture caused by insufficient mineralization. They tend to occur bilaterally in the mandible, at costochondral junctions ( causing the rachitic rosary) andin the epiphyses of the knees.
The Middle East & Africa Regional Audit Executive Summary, epidemiology, cost & burden of osteoporosis in 2011. The International Osteoporosis foundation. www.iofbonehealth.org.
Magnesium and vitamin D’s co-factors, by John Jacob Canell, MD. Citing The Lancet: The Vitamin D Council. Two cases of Mg dependent Vit D resistant rickets appeared in the Lancet in 1974.
Pettifor JM 2004. Nutritional rickets : deficiency of vit D, calcium, or both?. The American Journal of Clinical Nutrition (Review) 80(6 suppl): 1725S-9S.
Our patient had a uterine sarcoma causing a small bowel obstruction. While fibroids are present in 70% of women by age 50; uterine sarcomas represent only 4% of uterine malignancies. Most tumors occur after menopause and often they occur in the setting of pre existing fibroids. Sarcomas are more likely in women taking tamoxifen and African American women.
Sarcomas by definition have > 10 mitoses per high powered field. They are defined by the extent of their spread. Type 1 is confined to the uterus, Type 2 involves leiomyomatosis involving tumor growth within veins which can spread to the heart and type 3 involves tumor which has metastasized to distant organs like lung or lymph nodes. Five year survival for type 1 is 63%, type 2 is 35% and type 3 five year survival is 14%.
Our patient underwent a hysterectomy and resection of small bowel which was involved with tumor. She is doing well.
Galducci A, Cosio S, Romamini A, Genazzani AR. The Management of patients with uterine sarcoma: a debated clinical challenge. Crit Rev Oncol Hematol. 2008. 65(2) 129-42.
Zagouri F, Dimopoulos AM,Fotiou S, Kouloulias V, Papadimitriou CA. Treatment of early uterine sarcoma: disentangling adjuvant modalities. World J Surg oncol. 7:381477-7819-7-38.
Our patient had an enterovirus causing hand/foot/mouth disease. This was cultured from his throat swab.
This a common viral illness of infants and children less than five years of age but can also occur in adults. Typical symptoms include fever, mouth sores and a skin rash. It can be accompanied by viral meningitis. Rare complications include a polio-like paralysis or encephalitis which can be fatal. Coxsackievirus A 16 is the most common cause of hand,foot and mouth disease in the US.
From Aug 2014 to Jan 2015, 1,153 pople had confirmed enterovirus D68 infection.The virus was thought to be causing a polio-like paralysis in children. The first facility to report enterovirus D68 was Mercy Children’s Hospital in Kansas City , Missouri. They reported six children on one shift in the ED with severe respiratory symptoms. Several children in various sites across the country developed limb weakness or transverse myelitis prompting a CDC investigation.
The differential for rashes of the hands and feet includes:
1. Enterovirus- as in our patient usually benign and gone in 7 days
2. Syphilis- In secondary syphilis the reddish or brownish spots can occur on palms and soles. The lesions of disseminated GC tend to be pustules occurring on the distal extremities.
3. Rocky Mountain Spotted Fever- Rash begins as small macules on the wrists, forearms and ankles
On the sixth day petechiae deveop with a rash on the palms and soles in 80% of patients.
4. Janeway lesions of endocarditis- These are indications of septic emboli
5. Kawasaki disease-In Kawasaki erythema of palms and soles often occurs first accompanied by pain . Children often refuse to hold objects in their hands.
6. Measles-This rash start of the face and then progresses to the palms and soles.
7. Toxic Shock Syndrome- is more likely to desquamate than cause a papular rash
8. Reactive arthritis- Causes small hard nodules called keratoderma blennorrhagicum usually on the soles.
9. Meningococcemia- causes a petechial rash in 50-75% of cases. They do not blanch.
Our patient recovered without incident.
Schwartz, R. Enteroviruses treatment &Management May 2, 2014 http://emedicine. medscape.com/article217146-treatment
United States. Centers for disease Control and Prevention”Severe Respiratory Illness Associated with Enterovirus D68-Missouri and Illinois, 2014. MMWR 63:36 Sept 12, 2014. 798-99 http//www.cdc.gov/mmwr/preview/mmwrhtml/mm63e0908a1.htm
an abnormality was noted on cxr.
Our patient had a spinal epidural abscess which unfortunately had not been treated for some time.
He had a history in the past of an MVC in which he sustained an aortic injury and became paraplegic. He presented from a long term care facility with altered mental status and a widened mediastinum was noted on CXR. The CT showed the epidural abscess. In spite of antibiotics, he died of sepsis.
Epidural abscess is often not recognized on the first ED presentation. The “red flags” for the diagnosis include: unexplained fever, focal neurologic deficit , immunosuppression, IV drug use, prolonged steroid use, weight loss, enduring back pain and history of cancer. In 58% of the cases with errors, the “red flag” was documented but not acted upon.. The route of infection was identified in 52% of caseswith bacteremia as the most common cause (26%) followed by recent surgery(21%), and spinal injection. (6%). The most common risk factors were steroid use and diabetes. The most common organism was Staph Aureus, with MRSA in 25%. . The incidence is 5 cases for every 10,000 admissions in a study by Vakili however, our ED has seen at least three cases in the past month.
The second case (shown above) was a schizophrenic; well known for ingesting foreign objects and stabbing himself in the abdomen. He presented with a fever and paraplegia. His abscess wrapped around the aorta and esophagus and he was deemed “inoperable” by thoracic surgery. He remains paralyzed and in the hospital two months after presentation awaiting placement.
A third was a woman with diabetes who had previous back surgerywhich became infected one month pta. She presented to an outside hospital with nausea and vomiting and CT showed a psoas abscess. She was noted to have acute cholecystitis which was drained percutaneously and was discharged on long term IV antibiotics. Candida tropicalis grew from the gall bladder drain and nothing grew from the psoas abscess ( she had been previous treated with antibiotics.)
Bhise V et al. Errors in diagnosis of spinal epidural abscesses in the era of electronic health records. Am J Med 2017 Aug; 130:975.
Vakili M, Crum-Cianflone N. Spinal Epidural Abscess: A series of 101 Cases. American Journal of Medicine 2017, in press.
Davis DR, Wold RM, Patel RJ et al. The clinical presentation and impact of diagnostic delays on emergency department pateitns with spinal epidural abscess. J Emerg Med 2004;26(#):285-291.
Darouich RO. Spina epidual abscess. N engl J Med. 2006;355:2012-2020.
What do you notice on her PE protocol CT?
Our patient had tuberous sclerosis with cystic lung disease. There was no PE.
Tuberous sclerosis is a genetic disease that causes benign tumors to grow in the brain, lungs, kidneys, heart , skin, eyes and pancreas. It is caused by mutations in one of two genes: TSC1 or TSC2. These genes produce proteins that suppress tumors and when they are absent, tumors occur.
Tuberous sclerosis was first described by Bourneville in 1880 . He was a French surgeon who founded a day school for children with mental disability and noted a constellation of symptoms including seizures, mental disability and skin tumors which he named tuberous ( tuber from the Latin for swelling) and sclerosis (sklero from the Greek for hard).
The manifestations affect many organs. In the kidneys angiomyolipomas occur and polycystic kidneys in children may be the first manifestation of the disease. The disease in the lungs with multiple cysts is thought to be monoclonal metastasis from the renal angiomyolipomas which is why it has been reported after lung transplant.
Skin manifestations are found in 96% of individuals with tuberous sclerosis. Hypomelanic macules (ash leaf spots) , adenoma sebaceum( adenomas in a butterfly distribution on the face), Shagreen patches (thick skin patches dimpled like orange peel), and periungual fibromas are found in these patients.
Many of the findings of tuberous sclerosis are found in patients who do not have the disease. The lung cysts in isolation are called lymphangioleimyomatosis(LAM) . Tuberous sclerosis manifests differently in each patient with some patients like our patient with little symptomatology. She has mild obstructive lung disease and multiple kidney angiomyolipomas without renal failure and is followed periodically in clinic.
Henske EP. Metastasis of benign tumor cells in tuberous sclerosis complex. 2003 Genes Chromosomes and Cancer. 38(4):376-81.
Krueger D, Northrup H. International tuberous sclerosis complex consensus group (2013-10-01) Tuberous sclerosis complex surveillance and management: recommendations of the 2012 International Tuberous SclerosisComplex Consensus conference . (https://www.ncbi.nlm.nig.gov’pmc/articles/PMC4058297) Pediatric Neurology 49(4): 255-265.
Shepherd CW, Gomez MR, Lie JT, Crowson CS. Causes of death in patients with tuberous sclerosis. 1991 Mayo Clin Proc 66(8): 792-6.
Rakowski SK, Winterkorn EB, et al. Renal manifestations of tuberous sclerosis complex: Incidence, prognosis and predictive factors. 2006. Kidney Int 70(10):1777-82.
pictured below are the CT scan and the delayed image.
Why is she having pain?
Our patient had a calyceal rupture caused by a 2 mm stone. Calyceal or forniceal rupture is accompanied by urinary extravasation which can be seen on CT both in intraperitoneal and retroperitoneal areas. The majority of cases involve ureteral calculi but vascular compression from abdominal aortic aneursyms and malignancy have also been reported. Rarely, pregnancy can significantly obstruct the ureter, as well as a foley catherer balloon in one case.
75% of the stones causing rupture were distal, below the sacroiliac vessels. Calculi were small with a mean size of 4mm. Only 5% of patients in theGershman study showed signs of infection.
The physiologic reason for the rupture of the kidney is not known however it is presumed that pressure increases as the kidney increases in size, in accordance with Laplace’s law which states that the pressure tangent to the circumference of an object equals the product of the pressure across the wall and the radius of the object. It has been suggested that rupture protects the kidney by decreasing pressure in the collecting system.
Our patient was nauseated and given ondansetron and promptly developed the rhythm below.
She was treated with 4 gm of Mg ( goal of 2.5) and her K was raised to 4.0 . The polymorphic v tach resolved and she went to the OR where the stone was removed and a stent was placed in the R ureter.
She underwent a Left heart cath showing only 30% lesions so the final diagnosis was v tach secondary to QT prolongation and ondansetron. She had no further episodes of v tach and was discharged. And kudos to Phil who noticed the pelvic fracture. ( It had occurred earlier)
Gershman B, Kulkarni N, Sahani D, Eisner B. Causes of renal forniceal rupture, BJUI 2011. 108: 1909-1912.
Spurlock J, Burke T, Dunn N, et al. Calycealrupture with perirenal urinoma in a patient with cervical carcinoma.
Heffernan E, Skehan. Calyceal rupture secondary to ureteric obstruction by recurrent colorectal ca. Clin Nucl Med 2007;32:199-200.
Satoh S, Okuma A, Fuita Y, Tamaka M, Nakano H. spontaneous rupture of the renal pelvis during pregnancy: a case report and review of the literature. Am J Perinatology 2002;19:189-95.