2005 Associates Clinical Vignette Abstract Winners
First Place
Hypotension, Tachycardia, Pulmonary Edema & "Zellballen's," Tony Hericks, DO, R2, Des Moines Internal Medicine Residency Program, Iowa Methodist Medical Center, Des Moines, IA, Jon Fudge, MD, Mentor
Case: We report a 47 year old, previously healthy, WF with irregular menses who underwent a work up that revealed a 9.1 x 7.5 cm necrotic perirenal mass on CT presumed to be renal cell carcinoma. Elective lap nephrectomy was performed during which she developed pulmonary edema. After conversion to open laparotomy a perirenal mass and left kidney were removed. She was transferred to the CCU post-op in cardiogenic shock.
Pertinent Exam Findings: BP 74/45, HR 112, P.O. 100% on 90% Fi02; Edema fluid in her ventilator circuit. Lungs with bilateral crackles. Heart tones were tachycardic and without murmurs. No peripheral edema. Cardiac index 2.7. PCWP 22.
Lab/Dx Findings: Pulmonary edema by chest x-ray. Sodium 134. Potassium 5.9. Creatinine 1.2. Random Cortisol 22.9. CK 316. Troponin I 8.7. Path report revealed a pheochromocytoma and normal left kidney.
Treatment: Included an intra-aortic balloon pump, dopamine, dobutamine, norepinephrine, and hydrocortisone. All supportive therapy was stopped at 48 hours with improvement and at almost one month resolution of her cardiomyopathy.
Discussion: Pheochromocytomas are rare chromaffin cell tumors that secrete excessive catecholamines. Prevalence is < 0.1% of the general population. About 10% of these cases are "incidentilomas". The usual symptoms include headache, sweating, tachycardia and sustained or paroxysms of hypertension. A rare complication is catecholamine induced cardiomyopathy and pulmonary edema. Occasionally surgery, trauma, or possibly epinephrine secreting tumors result in peripheral vasodilatation, hypotension, and congestive heart failure in patients with catecholamine induced cardiomyopathy. This case, as well as others reviewed, demonstrates the need for acute recognition and aggressive management of pulmonary edema and cardiogenic shock related to catecholamine induced cardiomyopathy, as it can be a reversible condition.
Second Place
Sea Anemones: An Uncommon Etiology for Embolic Events, Matt Driewer, MD, R3, Des Moines Internal Medicine Residency Program, Iowa Methodist Medical Center, Des Moines, IA, Steven Craig, MD, Mentor
Case: We report an 86 y/o woman with longstanding history of atrial fibrillation and a recent diagnosis of Coumadin sensitivity. She was systematically taken off all medications with rash resolution, Coumadin was stopped 12 days prior to admission. She is referred after experiencing severe bilateral lower extremity pain of two days duration. At admission, she denied all other review-of-systems questioning except for palpitations. Prior work-up included negative LE venous Doppler.
Pertinent exam findings were atrial fibrillation, Grade III/IV systolic murmur and cyanotic feet bilaterally with no pulses in the dorsalis pedis or popliteal arteries.
Laboratory and imaging data showed CBC was normal. BUN=22, Cr=1.2, INR=1.03, Troponin I < 0.3. EKG showed atrial fibrillation with LBBB.
The patient was admitted for further work up and underwent bilateral vascular duplex imaging, which showed obstruction of the proximal left SFA and distal right SFA. Heparin drip was initiated after bilateral arterial thrombectomy. TEE was negative for left atrial appendage mass but showed a 1.0cm mass on the aortic valve leaflet. She experienced VT arrest X2 and subsequently died after prolonged resuscitative efforts. Autopsy verified aortic valve tumor and coronary artery disease with thrombus. Microscopic pathology confirmed papillary fibroelastoma.
Discussion: Intra-cardiac tumor is an unlikely but real etiology for cardiogenic embolic events. TEE is the most sensitive test to diagnose an aortic valve mass. Papillary fibroelastomas are most commonly found on the aortic valve and are histologically benign. They are most commonly found only incidentally at autopsy. While myxoma is the most common intracardiac tumor, the frond-like projections of a papillary fibroelastoma make an ideal source for thrombi formation. These "fronds" may break off and become embolic as well. Simple excision can cure 81% of symptomatic papillary fibroelastomas while 10% require excision and aortic valve replacement or repair.
Third Place (tie)
An Unusual Tonsillitis, Ahmad Halwani, MD, R1, University of Iowa Internal Medicine Residency Program, Iowa City, IA, Roger Gingerich, MD PhD, Mentor.
DB is a 22/w/f, who was into her 8th month of maintenance therapy for ALL in 1st remission when she presented with several day history of persistent odynophagia and fever. She saw her PCP 2 days prior; Quick step and monospot tests were negative; PO quinolone was started. She continued to have fever to 39oC with occasional rigors and orthostatic lightheadedness as she was hardly able to eat or drink.
PMH was positive for precursor B-cell ALL diagnosed 14 months ago. Induction inluded cyclophosphamide, dauncrubicin, vincristine, prednisione and L-asparaginase. A complete remission was attained, and she had received consolidation followed by maintenance therapy with prednisone for the first five days of each month, weekly methotrexate, and daily 6-mercaptopurine. Exam was positive for fever to 38.8oC, orthostatic hypoension, 1.5-2 cm matted, tender nodes in the right anterior cervical lymph node chain with smaller lymphadenopathy on the left. The oral cavity was dry with a swollen erythematous right tonsil that was touching the uvula. A white exudate was noted on the medial aspect of the tonsil.
The patient was admitted for hydration, pain control and IV antibiotics. The patient improved some and was able to eat and drink. She still had fatigue and almost daily nocturnal fevers to 39oC. Blood cultures remained negative. With continued fevers and increasing cervical adenopathy, tonsillectomy and lymph node biopsy was performed on the 6th hospital day. The patient recuperated uneventfully but continued to complain of fatigue and fever. Meanwhile, the pathology of both excised tonsils and lymph node revealed EBV positive diffuse large B cell lymphoma, strikingly reminiscent of that seen in PTLD with effacement of normal architecture with atypical large lymphoid cells containing oval nuclei, and scant cytoplasm. Numerous mitolic figures were seen and most cells stained strongly for EBV RNA. Further workup included had negative HIV serology, a low CD4 count of 160, a normocellular BM biopsy, and a PET-CT with intense FDG uptake within the cervical lymph nodes, tonsils, and nasopharynx. Maintenance therapy was stopped and patient received two doses of anti-CD20 monoclonal antibody (Ritaxan™).
EBV-related LPD/lymphomas are rare complications of ALL treatment. They are usually associated profound immunosuppression as in congenital immunodeficiency, AIDS or in the setting of solid or BM transplant. The pathogenesis is thought to involve an unchecked growth of certain EBV infected B cells due to an impaired cytotoxic T cell response. E reported cases-7 in children-of this complication in ALL occurred during maintenance therapy involving MTX. The latter has also been implicated in EBV-related LPD as a complication of RA treatment with MTX. None presented clinically as our patient with tonsillitis. Patients treated with immunosuppressive therapy, especially those on MTX, need to be monitored closely for infectious complications, including EBV, which can cause potentially fatal LPD. The development of risk factor profiles will be important in identifying patients who require immunosuppressive therapy and who may be at risk for developing EBV LPD.
Third Place (tie)
Atypical Depression and a Medical Mystery, Thad Abrams, MD, R5, University of Iowa Internal Medicine Residency Program, Iowa City, IA, Anjan Bhattacharyya, MD, Mentor
A 62 y.o. male with a past history of depression, low testosterone and osteoporosis, presented with several months of anorexia, a 40# weight loss, fatigue, apathy and poor concentration. He denied prominent anhedonia or depressed mood. Prior work-up had included a negative evaluation for dysphagia including an EGD, colonoscopy, and CT scan of chest and abdomen. Recurrent depression was suspected but he was admitted for evaluation due to unusual features of low grade fevers, pancytopenia, hypoalbuminemia, and cognitive changes.
Exam revealed low grade fever, cachexia, dullness to percussion at the right lung base and hepatosplenomegaly. The rest of the physical exam was unremarkable. Mental status exam showed disheveled appearance and decreased psychomotor activity. Deficits were noted in concentration, orientation, frontal lobe functioning and verbal fluency.
Initial impression was of occult malignancy and detailed medical work-up and aggressive nutritional support were pursued. Testing showed a polyclonal gammopathy, elevated LDH, hypoalbuminemia, and mild pancytopenia. Further work up included CSFx2, pleural fluid, MRI head, bone marrow aspiration, liver biopsy, gastric, and colon biopsies. Serologies for Whipple's disease, Coxiella, Brucellosis, Legionella, Mycobacterim, ANA, and ANCA were negative.
In the face of a negative work up,his ongoing depressive symptoms were treated with antidepressant medication and 4 ECT treatments. He did not respond. He developed intermittent delirium thought to be secondary to sedatives. He expired in the ICU after a 90 day hospital stay. Autopsy revealed large B-cell intravascular lymphoma with multi-organ involvement.
IVL presents with elevated LDH and focal CNS findings consistent with either focal or generalized ischemia. Encephalopathy is one of the presenting findings. The lack of tissue diagnosis with evidence of organ injury on lab exam should raise the clinical suspicion. Treatment often consists of CHOP and sometimes Methotrexate.
This case illustrates the need for internists to be familiar with typical features of depression. Unusual features such as disproportionate anorexia, weight loss and focal cognitive deficits should prompt further medical evaluation. Susceptibility to delirium strongly suggests organic brain disease.
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