The Journal of the Louisiana State Medical Society : official organ of the Louisiana State Medical Society最新文献

Introduction: A 71 year old Peruvian woman presented with blurry vision and bilateral eye pain and had evidence of bilateral endophthalmitis on eye examination. Her past medical history was notable for multiple abdominal surgeries within the preceding months, including an incarcerated inguinal hernia repair which required an ileostomy placement, and cholecystitis requiring cholecystostomy tube placement. Over her multiple long hospitalizations, she developed bacteremia and fungemia on several occasions, with organisms including Enterobacter cloacae, Klebsiella pneumoniae, Bacteroides fragilis, Pseudomonas aeruginosa, Enterococcus faecium, and Candida albicans. On exam, she was febrile and tachycardic. She had bilateral conjunctival injection, hypopyon, and severely decreased visual acuity. She also had a III/VI harsh holosystolic murmur at the apex. Her lungs were clear to auscultation. Transesophageal echocardiogram revealed severe mitral regurgitation and a 16 x 15 mm mitral valve vegetation and a perforated aneurysmal posterior mitral valve leaflet. The patient underwent bilateral vitrectomy and was treated with flucytosine and ambisome. Blood and vitreous humor cultures were obtained, but remained negative, likely due to being drawn after the patient had been empirically treated with antifungals and antibiotics. She continued to spike fevers and developed heart failure, but refused valve replacement surgery as she wished to return to her home country.

Discussion: A systemic source of infection should be sought in the presence of bilateral endophthalmitis. In our case, we suspected seeding from endocarditis or an intra-abdominal infection, given the patient history of multiple complicated abdominal surgeries and recurrent bacteremia and candidemia. Based on the vegetation's large size and bilateral endophthalmitis, we believed Candida was the most likely culprit. Additionally, eye lesions observed during surgery appeared consistent with fungal endophthalmitis. Candida is a rare but very morbid cause of infective endocarditis. Arterial embolization and metastatic infections such as endophthalmitis are more frequent in candidal endocarditis, likely due to the generally larger vegetation size. Valve replacement should be strongly considered regardless of other factors, as some studies have shown a mortality benefit for antifungals and surgery compared to antifungals alone. In cases in which valve replacement is not feasible, the patient should be kept on lifelong suppressive antifungal therapy.

Case: A 44 year old woman with a history of stroke s/p aneurysm clipping, seizure, and substance abuse was brought to the hospital after a family member received a call from her friend saying she was acting unusual and may have had a seizure while hanging out on the street. At her baseline, she needed modest assistance in daily activities after her CVA and conversed without issue. Her mother died of a stroke. On exam the patient was afebrile, normotensive with mild tachycardia to 110. The patient moved all her extremities but was lethargic, agitated, responded to pain but would not follow commands and moaned nonsensical speech. Labs were unrevealing with mild leukocytosis (WBC: 11.7 × 109/L);, normal metabolic panel, ammonia, glucose, and a negative urine toxicology. Initial computed tomography (CT); of her head demonstrated atrophy with large area of encephalomalacia in Left middle cerebral artery (MCA); area. Repeat CT, 1 day later showed edema and sulcal effacement in the right occipital, posterior, temporal, and posterior parietal lobes with evolving infarct in right posterior cerebral artery (PCA); and right MCA territories. CT angiogram showed occlusion of the clinoid segments of both internal carotid arteries, consistent with Moyamoya pattern of collateral flow. Neurosurgery was consulted and recommended cerebral bypass. The patient was unable to consent for surgery and her closest relative refused surgery. The patient received supportive therapy with minimal improvements in word findings/ communication and no improvement in inability to perform daily activities. She was accepted to inpatient stroke rehab upon discharge.

Discussion: Moyamoya disease is a rare vascular condition which leads to progressive stenosis of the internal carotid arteries through wall thickening of the associated arteries which leads to progressive strokes and the development of collateral vessels. Moyamoya is a Japanese term for a "puff of smoke" which describes the appearance on imaging of the small collateral vessels that develop around the progressively blocked arteries. There is a hereditary association and our patient's mother likely had the disease as well. The prognosis is poor and the disease will lead to a cognitive decline with associated CVAs. Treatment includes cerebral revascularization or bypass. The case highlights the need for early diagnosis, as our patient was too debilitated to make medical decisions for treatment at the time of her diagnosis.

Introduction: Dehiscence of a mitral valve annuloplasty ring is a rare occurrence that often manifests as mitral regurgitation and heart failure. We present a case of mitral ring dehiscence which was initially unrecognized by standard 2-dimensional transthoracic echocardiography (2D TTE); and 2-dimensional transesophageal echocardiography (2D TEE);.

Case: A 65-year-old woman was referred to Cardiology clinic for evaluation of dyspnea. Her history included tobacco abuse, atrial fibrillation status post pulmonary vein isolation, nonischemic cardiomyopathy, and prior mitral valve repair with annuloplasty ring for rheumatic valvular disease. She had been asymptomatic post-surgery. Physical examination, cardiac rhythm and initial ischemic workup were unremarkable. Pulmonary function tests revealed moderate emphysematous type obstructive lung disease. A 2D TTE demonstrated moderate mitral regurgitation with normal left ventricular function. In right heart catheterization, large v waves were noted and 2D TEE also revealed severe mitral regurgitation. On 2D TEE, the mitral valve annuloplasty ring was visible above the native anterior mitral valve leaflet. Color Doppler flow estimated the effective regurgitation orifice area of 0.4cm2 using the proximal isovelocity surface area method and regurgitant volume of 58 cc, consistent with severe mitral regurgitation. A "floating mitral ring" and dehiscence measuring 1 cm in diameter were seen on high resolution three-dimensional reconstruction resulting from the detachment of the ring from the weakened posterior annulus. Based on these findings patient was referred to cardiothoracic surgeon for re-do mitral valve surgery.

Discussion: This was a perplexing case as the patient's dyspnea could be explained by many disease processes including atrial fibrillation, mitral regurgitation and chronic obstructive lung disease. The standard imaging modalities did not help us to formulate a diagnosis. 3D TEE provided invaluable and unparalleled information of mitral valve pathology. Annuloplasty ring dehiscence is a well described complication of mitral valve repair and should always be considered in symptomatic patients.

Differential diagnoses of mediastinal masses are often based on the anatomic locations. Traditionally the posterior mediastinum has been home to esophageal and neurogenic cysts, but a new entity has been found to be prevalent since its initial report in 2005: the Müllerian cyst. We present a 49-year-old with history of cough who was found to have such a mass. We will discuss the surgical outcome and the details of this interesting entity. The literature pertinent to this type of cyst will be evaluated. In all cases reported, the literature demonstrates that surgical removal results in uneventful follow-up with no evidence of malignancy or cyst recurrence.

Introduction: The prevalence of sarcoidosis varies as much as 1-40 cases per 100,000 depending on region and population. Sarcoid typically occurs in people younger than 50 years old, with a peak incidence with ages between 20 and 40 years old. African Americans are 3 times more likely to develop sarcoidosis than Caucasian Americans, and woman are more likely than men to develop sarcoidosis in any ethnic group; nonetheless, it remains a valid differential across any population.

Case: A 32 year old Hispanic man presented to the Emergency Department with night sweats, fatigue, and 35 pound unintentional weight loss over the last several weeks. He also reported a sore throat, occasional cough w/ yellowish sputum, and new onset dyspnea with exertion. He moved from Mexico to the U.S. 20 years prior and last visited the country 7 yrs ago. He never smoked and denied any TB exposure. His vitals at admit demonstrated tachycardia (pulse 108); and temperature of 99.4 ᴼF. He had coarse bilateral breath sounds on exam. Serum chemistries were unremarkable. Chest radiograph demonstrated perihilar fullness. Chest CT revealed enlarged mediastinal and perihilar LAD and airspace consolidation in right middle and lower lobes bilaterally. The patient was admitted to a negative pressure room w/ airborne precautions and RIPE therapy was initiated. PPD and AFB's were negative. He underwent bronchoscopy and was discharged on RIPE. Lung biopsy showed non-caseating granulomas. RIPE therapy was stopped, and he was referred to ophthalmology to rule out uveitis. Cultures from the procedure were negative for fungal growth, and he was started on prednisone 40 mg daily with taper 6 weeks later as his weight returned, night sweats subsided, and dyspnea on exertion improved.

Discussion: Sarcoidosis should be considered as a diagnosis in any gender of any racial or ethnic group. Sarcoid is a great mimicker of many serious illnesses including malignancies such as lymphomas, TB and atypical mycoplasma, fungal infections, and other granulomatous diseases, and other autoimmune disorders such a hypersensitivity pneumonitis. Diagnosis requires patient investigation and careful analysis of these differentials.

Introduction: Gonorrhea is the 2nd most common sexually transmitted disease in the US with 800,000 cases of gonorrhea each year. Disseminated gonorrhea infection occurs in 0.5 percent - 3 percent of these patients and is more frequent in woman younger than 40 years of age.

Case: A 36 year old woman with a history of polysubstance abuse presented with 10 day history of feeling generally unwell. At presentation, vitals were remarkable for tachycardia and hypotension. Physical exam was remarkable for conjunctival pallor, bibasilar crackles, and tachycardia with grade III/VI systolic murmur loudest over the 2nd inter-costal space and loudest with expiration. No skin lesions were noted. Labs demonstrated leukocytosis (WBC 20,200 with 84 percent neutrophils);, anemia (Hb 6.7);, thrombocytosis (platelets 423 k/uL);, abnormal liver function tests (alkaline phosphatase 239 IU, AST 151 IU ALT 71 IU, albumin 2.5g/dL);, PT/INR 17.1/1.5. Troponin 0.42, BNP 823, D-dimer 619, and a urine drug screen that was positive for benzodiazepines, opiates, barbiturates, amphetamine, and THC. Hep panel and HIV were negative. Chest radiograph showed mild cardiomegaly and early interstitial edema. The patient was placed on broad spectrum antibiotics and given adequate fluid resuscitation and blood products. Blood cultures grew Neisseria gonorrhoeae. 2D ECHO showed a large pedunculated/mobile echo density adherent to the non-coronary and lefts cusps of the aortic valve. Proximal aortic root and aorto-mitral continuity were thickened, consistent with aortitis and/or abscess formation. Initial EKG on arrival showed junctional tachycardia which progressed into complete heart block. Cardiology was consulted and a pacemaker was placed emergently. However despite all aggressive measures the patient died of cardiac complications.

Discussion: Endocarditis is a rare complication of disseminated gonorrhea, occurring in only 1-2 percent of patients with gonoccocemia. The aortic valve is most commonly affected. Valve replacement is warranted in cases with severe dysfunction. Mortality remains around 19-20. Neisseria gonorrhoeae endocarditis should be included in the differential diagnosis in sexually active patients with endocarditis.

Introduction: Neurosyphilis is an infection of the central nervous system by Treponema pallidum, which can occur after the initial syphilis infection. Although commonly associated with late stage disease, patients with early neurosyphilis may present with acute syphilitic meningitis, meningovascular syphilis, or uveitis.

Case: A 28 year old man with a past medical history of HIV (CD4 364);, and recent diagnosis of uveitis presented to the Emergency Department with a positive RPR result. His visual acuity had been gradually declining over the past few months. He denied painless or painful ulcerating lesions on his penis, or scrotum, difficulty concentrating, dermatitis on the soles/palms, or difficulty with proprioception. Physical exam was notable for atrophic hyperpigmented polycyclic, annular plaques and patches along the hairline as well as several areas of confluent hyperpigmented polycyclic plaques and nodules on the patient's face, back, left arm, and right posterior leg. Fundoscopic exam revealed bilateral posterior uveitis and chorioretinitis. Evaluation of cerebrospinal fluid revealed a lymphocytic pleocytosis with a positive VDRL and FTA-ABS. Aqueous crystalline penicillin G was initiated for treatment of early neurosyphilis. Within six hours of beginning the infusion, the patient had a documented temperature of 101.8°F, heart rate of 128 beats per minute, blood pressure 142/84, with generalized malaise and headache. Fever and tachycardia resolved over the next 12 hours, with weakness and headache resolving within 1-2 days. His symptom complex was consistent with the Jarisch-Herxheimer reaction. Histopathology of skin biopsy of the back showed perivascular inflammation and rare spirochetes, consistent with secondary syphilis. The patient completed 14 days of aqueous crystalline penicillin G and was discharged after receiving the first of three benzathine penicillin injections.

Discussion: The initial manifestations of syphilis in this patient were posterior uveitis and pruritic skin plaques. His diagnosis should be appropriately classified as secondary syphilis with concomitant symptomatic early neurosyphilis, requiring 14 days of aqueous crystalline penicillin G. This type of presentation is not specific to immunocompromised populations and must be considered even in the general population. Making the diagnosis of early neurosyphilis, regardless of stage, is critical, as it necessitates a longer duration of treatment. Furthermore, clinicians should be reminded of the profound immunologic reaction, Jarisch-Herxheimer, which may occur when treating any treponemal disease.

Introduction: Cor triatriatum is a congenital cardiac anomaly in which the left (sinister) or right (dexter) atrium is divided into two compartments by residual embryonic tissue, resulting in a tri-atrial heart. As cor triatriatum dextrum can present clinically in various ways and have multiple associated cardiac anomalies, this report attempts to contribute to what is known about this exceedingly rare disorder.

Case: A 40 year old Hispanic man with a medical history of gastritis presented with complaints of palpitations, dizziness and bilateral lower extremity edema. He was found to have atrial fibrillation and new onset heart failure. The patient was admitted for rate control and further evaluation, which revealed several cardiac anomalies. Initial 2D echocardiography demonstrated severe right atrial enlargement, right ventricular hypertrophy and an engorged coronary sinus, which prompted further assessment of the patient's cardiovascular anatomy. Transesophageal echocardiography (TEE) revealed a severely enlarged, septated right atrium with a possible unroofed coronary sinus and a small patent foramen ovale (PFO). Left- and right-heart catheterization established a coronary-cameral fistula between the right coronary artery (RCA) and right atrium, as well as left-to-right shunt. The patient improved clinically with conservative management including diet modification, furosemide and digoxin for fluid and rate control, and was referred to cardiothoracic surgery for further evaluation.

Discussion: Cor triatriatum dextrum is an extremely rare cardiac condition: In high-volume echocardiographic laboratories, prevalence is less than 0.01 percent. This case highlights the association between cor triatriatum and other congenital cardiac anomalies, including persistent left superior vena cava with an unroofed coronary sinus, PFO and left-to-right shunt; all of which were found in this patient. While cases of cor-triatriatum sinistrum often require correction in infancy (due to left sided heart failure, pulmonary edema and cyanosis), cor-triatriatum dextrum is sometimes diagnosed in adulthood due to the lack of left heart and pulmonary involvement.

Case: A 39 year-old man presented to the emergency department complaining of a cough of 2 months in duration. He also complained of weight loss of 20 pounds in two months and night sweats over the same period. An x-ray of the chest was performed and revealed innumerable lung nodules and masses. Computed tomography of the chest and abdomen were subsequently performed which confirmed the masses, but imaging did not reveal an obvious primary source. A testicular exam was performed but no obvious abnormalities were noted. Because of his age as well as the appearance of the lesions, a testicular ultrasound was then performed. The ultrasound found evidence of a 1.5 cm hypoechoic mass on the right testicle with dystrophic calcification in the interpolar region. Urology performed a right radical inguinal orchiectomy. Pathology demonstrated a malignant mixed germ cell tumor with seminoma and yolk sac components being prominent. He underwent four cycles of etoposide, ifosfamide, and cisplatin. His last positron emission tomography scan did not demonstrate active disease.

Discussion: Each year about 8700 men are diagnosed with testicular cancer. 75 percent of these occur between the ages of 20 to 44, and the median age of diagnosis is 33. Testicular cancer should be suspected when a young male presents with metastatic disease such as in this case. In this patient, the genital exam was normal due to the small size of the testicular mass, but he had significant metastatic lesions. When cannonball metastases are seen on imaging, germ cell tumors and renal cell carcinoma should be high in the differential diagnosis.

Introduction: We present a case of disseminated Cryptococcus in a non-HIV host, where the primary manifestation was pulmonary with diffuse pulmonary infiltrates. This patient was on high dose corticosteroids for autoimmune hemolytic anemia.

Case: A 79 year old Caucasian man with a history of autoimmune hemolytic anemia on 100 mg of prednisone daily, coronary artery disease s/p bypass surgery, ischemic cardiomyopathy, chronic obstructive pulmonary disease, sleep apnea, chronic kidney disease, and history of bilateral pulmonary emboli presented to Hematology/Oncology clinic with symptoms of productive cough, worsening shortness of breath, hemoptysis. Anticoagulation had been stopped due to symptoms. The patient was referred to the emergency department from clinic where a chest CT demonstrated numerous calcified lymph nodes and diffuses grand glass opacities worse on the right and new compared to imaging from 6 months prior. The patient was placed on empiric antibiotics for treatment of pneumonia after blood and sputum cultures were obtained. Initial blood cultures grew Cryptococcus neoformans in both sets. CSF obtained by Lumbar puncture was negative for Cryptococcal. Serum Cryptococcal antigen titer was 1:2560. Infectious disease was consulted and the patient was started on induction therapy with liposomal Amphotericin B, followed by Fluconazole consolidation therapy. Hematology/ Oncology reduced the patient's prednisone dose gradually but further complications attributed to corticosteroids eventually necessitated the need to transition to Rituximab therapy. Follow up imaging on return to pulmonary clinic demonstrated marked improvement in the bilateral infiltrates.

Discussion: This patient was unique in that he demonstrated disseminated Cryptococcus but lacked neurologic complications, which is often how disseminated disease is clinically suspected. Blood cultures resulted positive for Cryptococcus and appropriate antifungal therapy was initiated before other sites were affected. The patient was HIV negative and not a post-transplant patient but was on high dose chronic prednisone for his AIHA, and therefore immunosuppressed. Opportunistic and atypical infections should be considered in all immunosuppressed patients to aid in earlier diagnosis and prevention of further dissemination of disease and further complications.