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The Case Files is an anecdotal collection of emergency medicine cases to enable physicians and researchers to find clinically important information on unusual conditions.

Case reports should focus on:

    • Unusual side effects or adverse interactions.
    • Unusual presentations of a disease.
    • Presentations of new and emerging diseases, including new street drugs.
    • Findings that shed new light on a disease or an adverse effect.

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Wednesday, May 22, 2019

​BY HABIR MANDAIR; KEYUR KURANI; & KHALID MALIK, MD

A 62-year-old obese woman with end-stage renal disease (ESRD) who was on hemodialysis, hypertension, diabetes mellitus, and chronically elevated serum calcium levels presented to the ED with altered mental status. Nursing home staff stated that she was unable to answer questions coherently and seemed confused.

Her vitals were within normal limits except for a body temperature of 99.9°F. Several necrotic lesions of various sizes were found on her left and right buttocks and the medial aspects of both thighs. The patient also had severe necrosis of the third finger on her left hand and the second on her right hand. Calciphylaxis was suspected because of her history and the unique characteristics of the lesions.

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Lab results showed a serum calcium level of 13.6 mg/dL, a PTH of 102.2 pg/mL, and elevated vitamin D levels. The patient was admitted and treated by wound care, nephrology, and endocrinology. Medications and dialysis regimen were also adjusted to achieve adequate serum calcium control.

Recognition and Risk Factors

Calcific uremic arteriopathy (CUA), the more accurate name for calciphylaxis, is a rare disorder resulting from impaired calcium and phosphate homeostasis most often found in ESRD patients. Metastatic calcification of blood vessels isn't uncommon in ESRD patients, but CUA is distinctly different because it selectively targets dermal and subcutaneous arterioles in areas of high adiposity, as seen in the thighs and buttocks of our patient. This results in painful violaceous mottling and fixed livedo reticularis that progress to retiform purpura, followed by black eschar and ulceration.

The areas most commonly affected are the thighs, buttocks, and abdomen, but CUA can also affect the digits, which is a sign of more advanced disease. (UpToDate. May 18, 2018; https://bit.ly/2IF7PLg.) Early recognition of this disorder is extremely important because CUA has a high mortality rate with a six-month survival rate of less than 50 percent and even worse prognosis for patients on long-term hemodialysis. (UpToDate. May 18, 2018; https://bit.ly/2IF7PLg.)

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CUA lesions are the result of decreased blood flow in dermal and subcutaneous arterioles. Progression of the disease is related to factors that increase serum calcium X phosphate product such as hyperparathyroidism, ESRD, and mineral bone disease. The disease's predilection for areas of high adipose tissue suggests that adipocytes may also play a role in its pathogenesis. Our patient had chronically high calcium levels due to a multitude of co-morbidities, including obesity, which made achieving adequate serum calcium levels necessary for survival.

ESRD patients can have risk factors for CUA related to hyperphosphatemia; medications such as warfarin, calcium-based binders, vitamin D analogs, and systemic glucocorticoids; being female; obesity; hypercoagulable states such as protein C and S deficiency and antiphospholipid syndrome; hypoalbuminemia; diabetes; longer dialysis vintage; inflammatory and autoimmune conditions; and recurrent skin trauma. (Am J Nephrol. 2013;37[4]:325; http://bit.ly/2HYc6Y9.)

Diagnosing CUA

Physical findings of painful, non-healing, non-ulcerating lesions in areas of high adiposity, with or without necrosis, should be evaluated for CUA. Lab values that point to increased calcium and phosphate in the serum may increase the index of suspicion, as was the case with our patient, including hyperparathyroidism. Warfarin use prior to onset of lesions has been observed in studies as well. Histologic findings of skin lesions show dermal and pannicular arteriolar calcification, subintimal fibrosis, and thrombotic occlusion.

Calcification most commonly involves the medial layer of small arteries and arterioles, but it can also involve the intimal layer and interstitial subcutaneous adipose tissue. A skin biopsy may be performed to rule out other disorders that mimic CUA, though it is contraindicated in patients with infected lesions. Differential diagnosis of CUA includes atherosclerosis, cholesterol embolization, warfarin necrosis, endarteritis obliterans, vasculitis, cellulitis, purpura fulminans, oxalate vasculopathy, and antiphospholipid antibody syndrome. (UpToDate. May 18, 2018; https://bit.ly/2IF7PLg.)

Multidisciplinary Treatment

The optimal treatment for CUA has yet to be established, but it involves a multidisciplinary approach that addresses wound care as well as endocrine- and dialysis-related care. The administration of sodium thiosulfate has been shown to reduce pain in as little as two weeks after the first dose and completely resolve early lesions in many cases. (UpToDate. May 18, 2018; https://bit.ly/2IF7PLg.)

Our patient had advanced-stage lesions, making wound care the primary therapy and critical to recovery, as is pain management. This means selecting proper dressings, chemical debridement agents, and negative-pressure wound therapy. Surgical debridement is reserved for patients with infected wounds and chemical debridement for patients with wounds that are not infected. Opioids are often required for pain control, especially immediately following wound care debridement procedures. All patients suffering from CUA should avoid repetitive local tissue trauma. Most importantly, abnormalities of calcium, phosphorus, and PTH must be treated to reduce the calcium X phosphorus product levels. (UpToDate. May 18, 2018; https://bit.ly/2IF7PLg.)

CUA is a painful disorder with a poor prognosis. Patients with isolated digital ischemia has a somewhat better prognosis than those with proximal lesion disease, but both carry a high mortality rate. Infection is the primary cause of mortality. (UpToDate. May 18, 2018; https://bit.ly/2IF7PLg.) The incidence of CUA is rising in the United States and is thought to be the result of increased awareness and recognition. Early intervention can have a profound effect on patients' quality of life and survival. Treatment strategies are variable and dependent on the stage at which patients present.

Mr. Mandair is a fourth-year medical student at Xavier University School of Medicine who is currently rotating at Weiss Memorial Hospital ED. Mr. Kurani is a fourth-year medical student at the University of Medical and Health Sciences in St. Kitts, also rotating at Weiss ED. Dr. Malik is the chief of emergency medicine at Weiss Memorial Hospital in Chicago.

Tuesday, May 14, 2019

​BY STEVEN CRUZ; CLAUDIA BORNIA; & ERIC MIZUNO, MD

A 68-year-old male nursing home resident with a history of hypertension, cerebrovascular accident, COPD, and chronic respiratory failure with a tracheostomy presented to the ED with a 101°F fever of four days that didn't respond to Tylenol. The patient was alert and oriented x0. His Glasgow Coma Scale score was 5.

The patient was tachypneic at 30 bpm, and three systolic blood pressures fell below 100 mm Hg. An initial chest x-ray revealed bilateral pulmonary infiltrates and a left-sided pleural effusion, raising suspicion for pneumonia as the most probable cause of sepsis. His persistent hypotension did not respond to IV fluids, so a left internal jugular central line was placed in anticipation of the need for vasopressors. Ultrasonography was used to aid in line placement, and a non-pulsatile, compressible internal jugular vein was seen. An introducer needle was inserted, and dark venous blood was drawn on the first attempt. Central venous pressure tracings were consistent with venous waveforms.

A chest x-ray was ordered to confirm line placement. The line had extended to the left side of the heart, raising concern for arterial cannulation. A contrast CT scan was ordered to better characterize the intrathoracic anatomy and the extent of the pneumonia. It revealed a left-sided pleural effusion and a left-sided superior vena cava draining into the coronary sinus but not a right-sided superior vena cava. (Image 1.) The line was left in place due to correct venous cannulation.

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Image 1. CT scan of the chest with contrast visualizing the left-sided superior vena cava.

Ultrasound is used to guide the central line insertion. Confirmation of line placement is assessed by chest x-ray. The catheter tip should be visualized in the right heart at the junction between the superior vena cava and the right atrium. The x-ray of our patient showed the catheter tip projecting in the left thorax. (Image 2.) This initially raised concern for arterial catheter placement, but the pressure tracings obtained by manometry confirmed venous cannulation. A contrast CT scan supported the venous location of the catheter in an anomalous left-sided superior vena cava (LSVC) draining into the coronary sinus.

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Image 2. Drainage of the left-sided superior vena cava into the coronary sinus on CT with contrast.

Obliteration of the left posterior cardinal vein normally occurs in late embryonic life because of compression between the left atrium and the hilum of the left lung, which leads to the absence of an LSVC. Any disruption of this compression pattern will result in the persistence of an LSVC. This is one of the most common abnormalities of the venous circulation. Most case reports with a persistent LSVC have reported a coexisting RSVC. Our patient did not have a RSVC, however.

A dilation in the coronary sinus is often the first indication of an LSVC. When an LSVC is present, the drainage commonly falls into the coronary sinus. This leads to an enlargement of the coronary sinus, which can be seen on angiography. This drainage doesn't present with a shunt between the arterial and venous circulation. Any other patterns of drainage or congenital heart defects can lead to a right to left shunt.

Up to 0.5 percent of the population has an LSVC. Typically, this is an incidental finding with little functional importance. An ultrasound-guided central venous line placement may raise initial suspicion for the anomaly if unexpected resistance is felt. Resistance may indicate arterial placement of the line. Chest x-ray will show the line in the left thorax in a patient with an LSVC. A venous waveform on manometry and confirmatory contrast CT will show venous line placement in an anomalous LSVC.

The diagnoses of an LSVC is most commonly made incidentally in the occasion of a central line placement. LSVC should be considered in a patient with unusual central line placement. The post-procedural chest x-ray will show the catheter tip in the left heart raising suspicion for arterial involvement. Catheter tip manometry and careful review of waveform tracings should be used in these cases to confirm venous line placement. A contrast CT scan will definitively diagnose the presence of an LSVC.

References

    • Webb WR, Gamsu G, et al. Computed tomographic demonstration of mediastinal venous anomalies. AJR Am J Roentgenol. 1982;139[1]:157.
    • Goyal SK, Punnam SR, et al. Persistent left superior vena cava: A case report and review of literature. Cardiovasc Ultrasound. 2008;6:50; http://bit.ly/2HXMB9j.
    • Cambell M, Deuchar DC. The left-sided superior vena cava. Br Heart J. 1954;16[4]:423; http://bit.ly/2HZCp02.
    • Voci P, Luzi G, Agati L. Diagnosis of persistent left superior vena cava by multiplane transesophageal echocardiography. Cardiologica. 1995;40[4]:273.
Mr. Cruz is a third-year medical student from the University of Medicine and Health Sciences in St. Kitts. Ms. Bornia is a fourth-year medical student at the Windsor University School of Medicine in St. Kitts. Dr. Mizuno is a board-certified internist, an associate professor at the Northwestern Feinberg School of Medicine, and an attending at Weiss Memorial Hospital.

Tuesday, April 9, 2019

​BY KYLE NADEL & PAUL SILKA, MD

The older woman was short of breath and experiencing epigastric abdominal pain. She was an ex-smoker in her mid-70s with a past medical history of COPD, pulmonary fibrosis, breast cancer, and intermittent home oxygen. Her symptoms had started gradually a few hours earlier.

The patient's vital signs on arrival were a blood pressure of 122/80 mm Hg, heart rate of 101 bpm, respiratory rate of 42 bpm, and 91% SpO2 on 2 L/min via nasal cannula. She was tachypneic with accessory muscle use, and breath sounds were clear bilaterally but diminished on the left hemithorax.

The patient was promptly placed on continuous oxygen therapy via nonrebreather mask, and routine blood work was initiated. A chest x-ray revealed a left-sided pneumothorax, prompting the emergency physician to perform an anterior tube thoracostomy with a 19 French pigtail catheter placed in the fifth intercostal space at the midclavicular line. The pigtail catheter was attached to a Heimlich valve for air drainage without negative pressure or suction and secured with prefabricated adhesive dressing.

The patient was admitted to a post-procedure telemetry bed after a repeat chest x-ray demonstrated improvement of the left pneumothorax, but the patient developed worsening respiratory distress with oxygen saturation falling below 80% about seven hours after insertion of the catheter. (Figure 1.) Her heart rate also increased as she developed hypotension with absent breath sounds ipsilateral to the Heimlich valve and tracheal deviation contralaterally. Repeat chest x-ray revealed early left tension pneumothorax, and the next emergency physician on duty confirmed the x-ray findings of an acute left-sided tension pneumothorax because the pigtail catheter attached to the Heimlich valve had failed. A left lateral chest tube thoracostomy was performed using a 28French tube, which yielded immediate clinical improvement. The patient's vital signs stabilized, and a repeat chest x-ray demonstrated successful re-expansion of the left lung. (Figure 2.)


Figure 1: Chest x-ray showing left-sided tension pneumothorax seven hours after pigtail catheter and Heimlich valve insertion.

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Figure 2: Chest x-ray indicating resolution of tension pneumothorax after a large bore chest tube thoracostomy.

Why did the treatment fail and precipitate the need for an emergent thoracostomy with a larger traditional chest tube? Was this the appropriate initial treatment for a pneumothorax? Was the failure of the initial apparatus predictable and a known complication?

The current literature indicates that treatment of a pneumothorax using a Heimlich valve is safe and definitive. (Ann Transl Med 2015;3[4]:54; http://bit.ly/2GaSlMKBr J Surg 2014;101[2]:17; Ann Chir Gynaecol 1999;88[1]:36.) The procedure must be performed properly, and the patient and care team must have adequate instruction on postprocedural maintenance. Some reports even argue that the Heimlich valve is more advantageous than thoracostomy because its portability allows for patient ambulation, which is believed to aid in lung re-expansion. (Thorax 1973;28[3]:386, http://bit.ly/2WG7NWgAnn Transl Med 2015;3[4]:54, http://bit.ly/2GaSlMKBr J Surg 2014;101[2]:17.)

The efficacy of the treatment has been established, but a number of case studies reported a spontaneous tension pneumothorax following initial treatment with a pigtail catheter attached to a Heimlich valve. Existing reports implicate tension pneumothorax resulting from inadvertent dislodgement, patient tampering, malpositioned catheter, flutter occlusion due to exudation, and catheter kinking. (Thorax 1973;28[3]:386, http://bit.ly/2WG7NWgAJR Am J Roentgenol1992;158[4]:763, http://bit.ly/2MRpa22Chest 1988;94[1]:55, 1998;113[3]:838, 1990;97[3]:759.)

The Heimlich valve may also fail if the atmospheric pressure is lower than that of the intrapleural cavity. (Ann Transl Med2015;3[4]:54; http://bit.ly/2GaSlMK.)

The Heimlich valve was initially successful in partial lung re-expansion in this case, though a spontaneous tension pneumothorax ensued, requiring emergent chest tube thoracostomy. The chest x-rays revealed that the failure was caused not by the Heimlich valve itself, but the attached pigtail catheter, which evidently became kinked in the thoracic cavity. Given the narrow lumen of the pigtail catheter, even a small kink prevents air from passing, making it useless in lung re-expansion. Catheter kinking may have occurred because of poor placement, though was more likely induced by inadvertent patient movement or tampering.

Despite the adverse complication experienced in this case presentation, the literature along with our clinical experience support the use of a small-caliber pigtail catheter and a Heimlich valve. They are efficacious and relatively well tolerated in treating nontraumatic pneumothorax. Disposition of these patients, whether to the hospital or discharge, must include meticulous care instructions calling out the risk for developing a tension pneumothorax and instructions on how to proceed should it occur.

Mr. Nadel has a master's degree in nutrition from Columbia University and is currently a research associate, and Dr. Silka is an adjunct associate professor of emergency medicine, both at LAC+USC.

Wednesday, March 13, 2019

​BY RYAN LYNCH, MD, & MARK SUPINO, MD

A 2-year-old girl was playing outside when she spotted a furry caterpillar. Like her stuffed animals, it was fluffy and had long hair. She let the insect crawl on her legs, but then suddenly screamed in pain. Her parents brought her to the ED, though she was no longer in distress by the time she arrived.

The patient had a large urticarial lesion on her inner right thigh and a smaller one on her left thigh. (Photo.) There were no puncture wounds, bite marks, or barbs visible or palpable. Her vital signs and physical exam were within normal limits. The patient's father had captured the culprit in an empty pill bottle and brought it to the ED for identification. (Photo.)

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Urticarial lesions prompting ED presentation.

Megalopyge opercularis, known as the puss caterpillar, woolly slug, possum bug, or perrito (Spanish for little dog), is a moth found all over the United States. (United States Department of Agriculture. Department Circular 288. 1923; http://bit.ly/2Bdx6WG.) The larval caterpillar form has venomous long hair-like setae protruding from its body. (van Nieukerken EJ, et al. Order Lepidoptera Linnaeus, 1758. In: Animal Biodiversity: An Outline of Higher-Level Classification and Survey of Taxonomic Richness, Zhang ZQ (Ed), Magnolia Press: Auckland 2011. Vol 3148, p. 212.) Its fluffy, stuffed animal-like appearance is often confused, especially by children, for an innocuous caterpillar.

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Adverse reactions after contact with poisonous or barbed caterpillars, moths, or butterflies are classified as lepidopterism. (MMWR Morb Mortal Wkly Rep 1990;39[13]:219; http://bit.ly/2Tu5WlD.) This encompasses a group of symptoms varying in severity that includes sting reactions, hypersensitivity reactions, and lonomism, the most severe form. Most stinging and hypersensitivity reactions are self-limiting and require only minor supportive care with analgesics and antihistamines, but lonomism is a bleeding diathesis that has a much more serious presentation.

This bleeding diathesis activates prothrombin with simultaneous degradation of several important clotting factors, including fibrin, fibrinogen, and factor XIII. (Thromb Res 2004;113[2]:147; Thromb Res 2001;102[5]:437.) Patients with lonomism present with excruciating localized pain at the envenomation site, vague neurological symptoms such as dizziness or altered mental status, generalized abdominal pain, and evidence of bleeding. It can be lethal in up to four percent of patients and requires inpatient supportive care, hematology consultation, and possibly serum immunoglobulin and dialysis if symptoms of acute renal failure develop. (Thromb Res 2004;113[2]:147.)

Megalopyge opercularis, fortunately for our patient, is only capable of sting reactions and has not been reported to cause hypersensitivity reactions or lonomism. (JAMA 1961;175[13]:1155.) The identity of the organism responsible for a bite or sting can dictate expectations, outcome, and therapeutic management. The mild, self-resolving symptoms, relatively benign physical exam, and correct identification of the organism reassured the patient and her family. Minimal supportive care, such as removing any potentially retained barbs with adhesive tape and giving NSAIDs, is sufficient treatment. A final take-home point: It is best to stay away from unknown insects, even ones that may have a friendly appearance.

Dr. Lynch is a junior resident, and Dr. Supino is the associate program director of the emergency medicine residency at Jackson Health System/University of Miami.

Tuesday, February 26, 2019

​BY JEFFREY LOMBARDO, MD, & MARK SUPINO, MD

The progressive suprapubic pain was a cunning symptom.

The 38-year-old man had had five days of that pain and dysuria. By the time he presented to our ED, his pain had spread to the right lower quadrant as well. He had a history of diverticulitis after a laparoscopic left hemicolectomy four years earlier.

He reported no fevers, but complained of nausea and diarrhea. He was afebrile at 36.8°C with a pulse of 76 bpm. All other vital signs were normal. Physical exam was significant for suprapubic pain and right lower quadrant tenderness to palpation without rebound, guarding, or distention.

His workup revealed a normal white blood cell count of 4.2, as well as a normal basic chemistry and urinalysis. A CT of the abdomen and pelvis with intravenous contrast showed a dilated appendix tip measuring 12 mm with mucosal enhancement and fat stranding. The appendix was elongated and overlying the bladder.

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Computed tomography of the abdomen and pelvis with intravenous contrast. The yellow arrows show the appendix coursing through the lower abdomen and overlying the bladder.

Our patient was diagnosed with acute appendicitis. He was given antibiotics, intravenous fluids, kept NPO, and admitted to the surgical team. He underwent uncomplicated laparoscopic appendectomy and was discharged the next day.

Suprapubic pain and right lower quadrant pain are typical chief complaints in the emergency department, but it is important to keep a broad differential diagnosis when evaluating patients. In acute appendicitis, migrating right lower quadrant pain, fever, anorexia, nausea, and vomiting are unreliable, occurring consistently in only about half of cases. (Hong Kong Med J 2000; 6[3]:254; http://bit.ly/2UiKXCh.)

When considering additional diagnoses, it is important to recall anatomical structures near where the pain is reported and to be mindful of variations in appendiceal sizes and locations. The appendix in the retrocecal position can cause inflammation of the retroperitoneal organs, mimicking biliary colic or gastroenteritis. (Adv J Emerg Med 2018;2[2]:e21; http://bit.ly/2sL5uni.) A perforated appendix can cause fistula formation to the anterior abdominal wall and present as a palpable mass. (BMJ Case Rep 2016 Nov 10; http://bit.ly/2DwFa6h.) Our patient's appendix was elongated and overlying the bladder, resulting in suprapubic pain and dysuria.

Dr. Lombardo is a senior resident in emergency medicine, and Dr. Supino is the associate program director of the emergency medicine residency at Jackson Health System/University of Miami.