Successful treatment with cladribine of Erdheim-Chester disease with orbital and central nervous system involvement developing after treatment of Langerhans cell histiocytosis.

INTRODUCTION: Erdheim-Chester disease (ECD) is a rare, systemic form of non-Langerhans cell histiocytosis of the juvenile xanthogranuloma family with characteristic bilateral symmetrical long bone osteosclerosis, associated with xanthogranulomatous extra skeletal organ involvement. In ECD, central nervous system (CNS) and orbital lesions are frequent, and more than half of ECD pa tients carry the V600E mutation of the protooncogene BRAF. The synchronous or metachronous development of ECD and Langerhans cell histiocytosis (LCH) in the same patients is rare, and the possible connection between them is still obscure. Cladribine is a purine substrate analogue that is toxic to lymphocytes and monocytes with good hematoencephalic penetration. CASE REPORT: We presented a 23-year-old man successfully treated with cladribine due to BRAF V600E-mutation-negative ECD with bilateral orbital and CNS involvement ECD developed metachronously, 6 years after chemotherapy for multisystem LCH with complete disease remission and remaining central diabetes insipidus. During ECD treatment, the patient received 5 single-agent chemotherapy courses of cladribine (5 mg/m2 for 5 consecutive days every 4 weeks), with a reduction in dose to 4 mg/m2 in a fifth course, delayed due to severe neutropenia and thoracic dermatomal herpes zoster infection following the fourth course. Radiologic signs of systemic and CNS disease started to resolve 3 months after the end of chemotherapy, and CNS lesions completely resolved within 2 years after the treatment After 12-year follow-up, there was no recurrence or appearance of new systemic or CNS xanthogranulomatous lesions or second malignancies. CONCLUSION: In accordance with our findings and recommendations provided by other authors, cladribine can be considered an effective alternative treatment for ECD, especially with CNS involvement and BRAF V600E-mutation-negative status, when interferon-alpha as the first-line therapy fails.

Posterior reversible encephalopathy syndrome--A case report.

INTRODUCTION: Posterior reversible encephalopathy syndrome (PRES) is characterized by the following symptoms: seizures, impaired consciousness and/or vision, vomiting, nausea, and focal neurological signs. Diagnostic imaging includes examination by magnetic resonance (MR) and computed tomography (CT), where brain edema is visualized bilaterally and symmetrically, predominantly posteriorly, parietally, and occipitally. CASE REPORT: We presented a 73-year-old patient with the years-long medical history of hypertension and renal insufficiency, who developed PRES with the symptomatology of the rear cranium. CT and MR verified changes in the white matter involving all lobes on both sides of the brain. After a two-week treatment (antihypertensive, hypolipemic and rehydration therapy) clinical improvement with no complications occurred, with complete resolution of changes in the white matter observed on CT and MR. CONCLUSION: PRES is a reversible syndrome in which the symptoms withdraw after several days to several weeks if early diagnosis is made and appropriate treatment started without delay.

Visceral hybrid reconstruction of thoracoabdominal aortic aneurysm after open repair of type A aortic dissection by the Bentall procedure with the elephant trunk technique--a case report.

INTRODUCTION: Reconstruction of chronic type B dissection and thoracoabdominal aortic aneurysm (TAAA) remaining after the emergency reconstruction of the ascending thoracic aorta and aortic arch for acute type A dissection represents one of the major surgical challenges. Complications of chronic type B dissection are aneurysmal formation and rupture of an aortic aneurysm with a high mortality rate. We presented a case of visceral hybrid reconstruction of TAAA secondary to chronic dissection type B after the Bentall procedure with the 'elephant trunk' technique due to acute type A aortic dissection in a high-risk patient. CASE REPORT: A 62 year-old woman was admitted to our institution for reconstruction of Crawford type I TAAA secondary to chronic dissection. The patient had had an acute type A aortic dissection 3 years before and undergone reconstruction by the Bentall procedure with the 'elephant trunk' technique with valve replacement. On admission the patient had coronary artery disease (myocardial infarction, two times in the past 3 years), congestive heart disease with ejection fraction of 25% and chronic obstructive pulmonary disease. On computed tomography (CT) of the aorta TAAA was revealed with a maximum diameter of 93 mm in the descending thoracic aorta secondary to chronic dissection. All the visceral arteries originated from the true lumen with exception of the celiac artery (CA), and the end of chronic dissection was below the origin of the superior mesenteric artery (SMA). The patient was operated on using surgical visceral reconstruction of the SMA, CA and the right renal artery (RRA) as the first procedure. Postoperative course was without complications. Endovascular TAAA reconstruction was performed as the second procedure one month later, when the 'elephant trunk' was used as the proximal landing zone for the endograft, and distal landing zone was the level of origin of the RRA. Postoperatively, the patient had no neurological deficit and renal, liver function and functions of the other abdominal organs were normal. Control CT after 6 months showed full exclusion of the aneurysm from the systemic circulation without endoleak and good flow through visceral anastomosis. CONCLUSION: In patients with comorbidities, like in the presented case, visceral hybrid reconstruction of chronic dissection type B with TAAA could be the treatment of choice.

Traumatic mesenteric pseudocyst.

INTRODUCTION: Mesenteric pseudocysts have rarely been described in literature. They belong to a group of mesenteric cysts that are very rare intra-abdominal pathology regardless of the origin. The diagnosis is often difficult to make, because of the diversity of clinical symptoms. The definitive histopathological diagnosis determines the origin and further course of treatment. CASE REPORT: We reported a patient with post-traumatic mesenteric pseudocyst. It was localised on the mesenteric side, in the direct contact with the small intestine. We surgically removed the pseudocyst along with a part of the small intestine with success. The patient's recovery was eventless, with no complications. CONCLUSION: Only by complete cyst removal, the definitive, accurate histopathological diagnosis and classification can be made.

A 60-year expirience in the treatment of pancreatic insulinoma in the Military Medical Academy, Belgrade, Serbia.

BACKGROUND/AIM: Insulinomas are rare benign tumors in the most cases and the most frequent endocrine tumors of the pancreas. A wide spectrum of clinical manifestations in patients with insulinoma is the reason for difficult recognition of the disease with a long period of time between the onset of symptoms and the diagnosis. Diagnostic procedures include Whipple's triad, 72-hour fast test and topographic assessment. The only currative therapy for patients with insulinoma is operative treatment. METHODS: This retrospective study included 42 patients with diagnosis of insulinoma treated in our institution in a 60-year period. In all the patients a demographic and clinical data, types of biochemical methods for diagnosis, and diagnostic procedures for insulinoma localization were analyzed. Tumor size and localization, surgical procedures, postoperative complications and outcome were assessed. RESULTS: A study included 42 patients, 29 women and 13 men. The median age at diagnosis was 43 years. Median time between the onset of symptoms and diagnosis was 3 years. The most common clinical symptoms and signs were disturbance of consciousness and abnormal behavior in 73%, confusion and convulsions in 61% of patients. The diagnosis of insulinoma was estimated by Whipple's triad and 72-hour fast test in 14 patients. Determination of insulinoma localization was assessed by angiography in 16 (36%) of the patients, by ultrasound (US) in 3 of 16 (18.8%) patients, by abdominal computed tomography (CT) in 8 of 18 (44.5%) patients, and magnetic resonance imaging (MRI) in 2 of 8 (25%) patients. Insulinoma was found in 13 of 13 (100%) patients by arterial stimulation with venous sampling (ASVS) and in 13 of 14 (93%) patients by endoscopic ultrasound (EUS). Of the 42 patients, 38 (90.5%) underwent operative procedure. Minimal resection was performed in 28 (73.6%) of the patients [tumor enucleation in 27 (71%) and central pancreatectomy in one (2.6%) of the patients], and the major resection was performed in 9 (23.6%) of the operated patients [distal splenopancreatectomy in 8 (21%) and pancreaticoduodenectomy in one (2.6%) patient]. The overall mortality rate in postoperative period was 2.6% (one patient). CONCLUSION: A combination of ASVS and EUS as diagnostic procedures ensures high accuracy for preoperative determination of insulinoma localization. Minimal resection such as enucleation shoud be performed whenever it is possible.

Disseminated Rhodococcus equi infection in a patient with Hodgkin lymphoma.

INTRODUCTION: Rhodococcus (R) equi is an opportunistic, uncommon human pathogen that causes mainly infection in immunocompromised hosts. The disease is usually presented as subacute pneumonia that is mostly cavitary and sometimes bacteremic. CASE REPORT: We reported the extremly rare case of a 43-year-old woman with Hodgkin lymphoma, who developed R equi pulmonary infection after recieving multiple courses of chemotherapy. Secondary, the patient developed bacteremia, leading to sepsis and dissemination of R equi infection in many extrapulmonary sites. At addmission the patient was febrile, tachypnoic, tachycardic, hypotensive, with fa cial edema, splenomegaly, positive meningeal signs, left hemiparesis and paraparesis. Laboratory data included erythrocyte sedimentation rate (ESR) > 140 mm/h, C-reactive protein (CRP) 143.0 mg/L, red blood cells (RBC) 2.14 x 10(12)/L, whyite blood cells (WBC) 2.8 x 10(9)/L, lactate dehydrogenase (LDH) 706 U/L, serum albumin 26 g/L, sodium 127 mmol/L and potassium 2.7 mmol/L. Blood culture and culture of sputum and empyema were positive for R equi. Imaging studies demonstrated a large right cavitary pneumonia and abscess, empyema, pericarditis, mediastinal and intra-abdominal lymphadenopathy, brain and psoas abscesses, osteomyelitis and spondylodiscitis. The patient recovered completely after a 12-month treatment with combinations of parenteral and oral antibiotics (meropenem, vancomycin, teicoplanin, ciprofloxacin, rifampicin, macrolides etc), including drainage of abscesses and empyema. Eight years after completition of the treatment the patient was without recurrence of R equi infection and lymphoma. CONCLUSION: Since the eradication od R equi is very difficult, it is very important to make the diagnosis and initiate appropriate antibiotic therapy as soon as possible.

Experimental pleural empyema model in rabbits: Why, how and what are the next steps.

BACKGROUND/AIM: The use of new therapeutic methods to prevent development of fibrothorax as the final complication of the human pleural infections requires research with experimental animals. The aim of this study was to standardize the procedures for the establishment of our own experimental model of empyema in rabbits, since it should be able to offer similar conditions found in human pleural infections. METHODS: This experiment included 15 chinchilla rabbits, weighing from 2.3 to 2.8 kg. There were 12 rabbits in the experimental group, while 3 rabbits formed the control group. On the first day, we administered 0.4-0.5 mL of turpentine in the right pleural space of the rabbits from the experimental group in order to provoke sterile exudative pleurisy. After 24 h we injected 1 mL of Staphylococcus aureus and 1 mL of Escherichia coli bacteria in the same concentration of 4.5 x 10(8) bacteria/mL. Thoracocentesis for the pleural fluid analysis was performed 24, 48, 72, and 96 h after bacteria instillation. In these pleural samples we estimated the number of leucocytes and the values of lactate dehydrogenase (LDH), glucose and pH in pleural fluid, as well as the presence of bacteria. We did not protect the animals with antibiotics, and on the day 7 of the experiment they were sacrificed with the lethal dose of barbiturate (iv). The lung from the empyemic side of all experimental animals and the lung of one control animal were histopathologically examined. RESULTS: A total of 4 animals had a small amount of clear pleural fluids or there was no fluid obtained with thoracocentesis 24 and 48 h after the bacteria instillation. after the bacteria instillation. In the remaining 8 rabbits 24 h after bacteria administration the mean values (± SD) of the parameters monitored were as follows: Le 34.75 ± 6.13 x 109/L, LDH 17,000 ± 4,69 U/L, glucose 1.23 ± 0.45 mmol/L, and pH 6.975 ± 0.15. The obtained values met the criteria for the evaluation of effusion as pleural empyema or complex and complicated pleural effusion (LDH > 1000 U/L, glucose < 2.31 mmol/L and pH < 7.20). Bacterial cultures were positive in 5 out of 8 first pleural samples and in only 2 samples after 48 h of bacteria administration. There was a positive correlation between the number of leukocytes and the LDH value (r = 0.071, p < 0.001), and a negative correlation between the number of leukocytes and the glucose level (r = 0.864, p < 0.001), and the leukocytes number and pH of the pleural fluid (r = 0.894, p < 0.001). The mean glucose value increased after 48 h (3.23 ± 0.44 mmol/L), and the pH value rose after 72 h (7.22 ± 0.03) which was beyond the empyema level. CONCLUSION: The creation of the experimental empyema model is a very delicate work with uncertain success. Its value and importance are crucial for pleural pathology research. With the intention to obtain a more empyemic pleural reaction we created a model with two different human pathogen bacteria. We generated the satisfactory results, but not as good as those contained in some of the reference literature data.

Orbital lymphoma associated with Graves' disease: A case report.

INTRODUCTION: The presence of bilateral exophthalmos and palpebral, periorbital edema associated with hyperthyroidism is most often considered as an initial sign of Graves' ophthalmopathy. However, in up to 20% of cases, Graves' ophthalmopathy might precede the occurence of hyperthyroidism, which is very important to be considered in the differential diagnosis, especially if it is stated as unilateral. Among other less common causes of non-thyroid-related orbitopathy, orbital lymphoma represents rare conditions. We presented of a patient with Graves' disease, initially manifested as bilateral orbitopathy and progressive unilateral exophthalmos caused by the marginal zone B-cell non-Hodgkin lymphoma of the orbit. CASE REPORT: A 64-year-old man with the 3-year history of bilateral Graves' orbitopathy and hyperthyroidism underwent the left orbital decompression surgery due to the predominantly left, unilateral worsening of exophthalmos resistant to the previously applied glucocorticoid therapy. A year after the surgical treatment, a substantial exophthalmos of the left eye was again observed, signifying that other non-thyroid pathology could be involved. Orbital ultrasound was suggestive of primary orbital lymphoma, what was confirmed by orbital CT scan and the biopsy of the tumor tissue. Detailed examinations indicated that the marginal zone B-cell non-Hodgkin lymphoma extended to IV - B-b CS, IPI 3 (bone marrow infiltration: m+ orbit+). Upon the completion of the polychemiotherapy and the radiation treatment, a complete remission of the disease was achieved. CONCLUSION: Even when elements clearly indicate the presence of thyroid-related ophthalmopathy, disease deteriorating should raise a suspicion and always lead to imaging procedures to exclude malignancy.

[Significance of ligature of early detected insufficient perforated lower extremities veins as a cause of the varicose syndrome].

BACKGROUND/AIM: Perforated veins (PV) connect surface and deep veins net. Insufficient perforated veins (IPV) are considered to be one of the causes of the venous stasis syndrome. Ligating IPV removes increased pressure transmission from the deep veins to the surface veins system and prevents the occurrence of varicosis, as well. The aim of the study was to determine surface veins diameters prior and after the surgical operation on IPV, and to confirm good results of the treatment with this method in removing causes of the surface veins varicosis. METHODS: The study included 30 patients of both sexes (25 males and 5 females), mean age 30.10 +/- 10.24 years. The patients were classified in accordance with CEAP (clinical severity, ethiology or cause, anatomy, pathophysiology) classification as those with the initial varicous syndrome based on IPV. Any of the patients were submitted to color Doppler echophlebography. In case of diameter > or = 3.5 mm PV were marked as IPV. All of the IPV were divided into two groups, the group I being with ostium in the stem of Vena Saphena Magna (VSM) or Vena Saphena Parva (VSP), while the group II was with ostium in the venous tributors. VSM and VSP diameters at the level of IPV ostium, bellow and above (1 cm) the ostium were measured prior to IPV ligature, and 30 days after the surgical intervention. RESULTS: Comparing the results the highest ligature effects were shown at the level of IPV ostium in the stem of VSM and VSP of both groups (p < 0.0001) with the highest diameter reduction. There was a smaller reduction of diameter in the proximal and distal segment of ostium in the group I, while there was no diameter change in the distal segment of ostium in the group II. CONCLUSION: Saphenous stem diameter reduction at the level of ostium, proximally and distally of the confluence, confirms the significance of IPV ligature before pronounced varicosis appears.