Efficacy of tocilizumab in refractory giant cell arteritis

Giant cell arteritis is the most frequent form of vasculitis characterized by a high risk of vascular thrombosis. Major complications are blindness and other vascular ischemia but bowel ischemic involvement is rare. Treatment is based on long-term steroid therapy with numerous side effects. The efficacy of immunosuppressive drugs like azathioprine methotrexate or anti-tumor necrosis factor antibodies appears to be too low to reduce the use of steroids. Th17 lymphocytes and interleukin-6 play an important role in pathogenesis of giant cell arteritis. We report here a case of effective interleukin-6 blocker in the treatment of refractory giant cell arteritis with ileitis and high-dose steroid dependence despite 2 years of treatment with steroids and methotrexate. After infusions of tocilizumab, no relapse at 6 months was found despite the decrease in corticosteroids.     

Carotid free-floating thrombus causing stroke in a young woman with lupus anticoagulant: A case report and review of the literature

IntroductionIn young adults Stroke is a challenging condition and various tests are needed to diagnose and manage its underlying problems. Free floating thrombosis of internal carotid artery (FFT-ICA) is one of the rare problems among carotid artery diseases which can lead to stroke in adults. Owing to limited cases of FFT-ICA there is not a worldwide accepted consensus on management of FFT-ICA, but some recurrences after medical management have been reported in the literature.Presentation of caseA-25-year old woman was referred to hospital with sudden onset aphasia and right sided hemiparesis. Carotid duplex ultrasonography showed an iso-echogenic thrombus ranged about 5 × 10 mm partially attached to arterial wall of the right internal carotid artery. It was floating in accordance with the heartbeat. Anticoagulation therapy was initiated and subsequently she underwent an urgent operation. To explore the etiology of her problem, various lab tests were carried out; the results showed markedly elevated levels of lupus anticoagulant. She was discharged from hospital after an uneventful recovery showing gradual improvement of symptoms in follow-up visits.DiscussionFFT-ICA is one of the scarce disorders of carotid artery. Less than 150 cases have been reported in the literature indicating its common occurrence in old men. Hence the etiology of this case (FFT in a young patient with high levels of lupus anticoagulant antibody) may be the first of its type.ConclusionIn this case we chose surgical treatment resulting in complete resolution of symptoms and non-recurrence within 6 month follow-up.     

Ectomesenchymal chondromyxoid neoplasm. An unusual presentation. A case report

Ectomesenchymal chondromyxoid tumor (ECMT) is a rare benign intraoral tumor. Clinically, it presents as a slow growing, painless, firm, submucosal swelling exclusively occurring on the anterior dorsum of the tongue. Till date not more than 45 cases have been reported in literature. Histopathologically the tumor is characterized by a well circumscribed, lobular proliferation of round, polygonal, ovoid or fusiform cells in a net-like pattern in a myxoid to chondromyxoid background. Here, we present a rare case of ECMT occurring in a 17 years old male who presented to us with chief complaint of slow growing painless mass arising from anterior Dorsum of the tongue measuring about 1.5 cms × 1.5 cms. Mass was completely resected as excisional biopsy, it was found un encapsulated with muscle entrapment.     

Vasculite testicular – uma manifestação rara de artrite reumatoide

Testicular vasculitis is a very rare extra-articular manifestation of rheumatoid arthritis (RA). We describe the case of a 53-year-old man diagnosed with RA for eight years, who was poorly controlled and developed rheumatoid vasculitis, which manifested as leg ulcers and peripheral polyneuropathy. The patient also had acute neutrophilic meningitis and was treated with antibiotics and intravenous pulse therapy with methylprednisolone (500 mg daily) for three days, followed by oral cyclophosphamide (2 mg/kg daily) and prednisone. Overall improvement was observed, and the patient was discharged. But 15 days later, the meningitis recurred, and the patient was readmitted and treated again with antibiotics. Three days later, he developed pain and enlargement of his left testicle with gangrene. Unilateral orchiectomy was performed, revealing lymphocytic vasculitis. The patient died two days later due to aspiration pneumonia. This case illustrates a rare and severe manifestation of rheumatoid vasculitis.     

Appendicitis as a rare cause of mechanical small-bowel obstruction: A literature review of case reports

IntroductionAlthough bowel paralysis accompanying acute appendicitis is well known, mechanical bowel obstruction as a direct consequence of appendicitis remains a rare, but potentially life-threatening, acute abdomen. The aim of our literature review was to find all documented cases of this particular complication and compare them with our own case study.MethodsWe searched the PubMed database for relevant articles published from 1963 to 2015. The study included patients for whom direct links between appendicitis and strangulation of the terminal ileum were found, and for which the disease course had been documented in detail. The study also included our own case report since it met the inclusion criteria. A total of 190 articles were examined with a final yield of 17 case reports from 13 articles.Results17 patients (11 men and 6 women), with a mean age of 48 ± 23.9 years, met the inclusion criteria. The average period between symptom onset and surgery was 3.4 ± 3.7 days. Symptoms of the disease were consistent with small-bowel obstruction. Treatment included simple appendectomy (n = 7), possibly supplemented by segmental resection (n = 5), followed by ileocecal resection (n = 4), and one case that required a right-sided hemicolectomy (n = 1).ConclusionWe found mechanical bowel obstruction directly related to appendiceal inflammation to be extremely rare, and relatively few individual cases involving this potentially life-threatening complication have been documented in the literature. Clinical signs of the disease are variable, non-uniform, and consistent with symptoms of small-bowel obstruction during their progression.     

Mega-aorta syndrome development in giant cell arteritis. A same entity?

Giant cell arteritis (GCA) is the most common form of large vessel arteritis. GCA typically involves the branches of the external carotid artery, but is the leading cause of inflammatory aortitis. However, involvement of the aorta often goes undetected. We present a case of an 81-year-old man, with headache and intense chest pain, who was previously given a diagnosis of GCA with a temporal artery biopsy 6 years ago. Owing to the suspicion of acute aortic syndrome, an emergent computed tomography (CT) was performed. CT showed the development of mega-aorta syndrome, with a diameter of 75.2 mm in the ascending aorta, 61.8 mm in the aortic arch, 76.1 mm in the descending thoracic aorta, and 45.1 mm in the abdominal aorta, presenting a chronic type B aortic dissection. Although there are reported cases secondary to Takayasu arteritis, this is the first case reported in the literature of mega-aorta syndrome associated with GCA in a patient previously diagnosed using temporal artery biopsy.     

Giant gallstone performed by emergency laparoscopic cholecystectomy

INTRODUCTIONGallstone disease is very common, but the gallstone bigger than 5 cm in diameter is very rare. It is very challenging to be removed by laparoscopic cholecystectomy (LC) and poses extra difficulty in emergency.PRESENTATION OF CASEA 70-year-old man complained of abdominal pain in the right upper quadrant with fever of 38 °C for two days. Abdominal ultrasound indicated acute cholecystitis and a single, extremely large gallstone (95 mm × 60 mm × 45 mm). Emergency laparoscopic cholecystectomy was performed successfully.DISCUSSIONGallstone over 5 cm in diameter is very rare. LC will be very difficult for these cases, especially for the emergency cases. Emergency laparoscopic cholecystectomy can be successfully performed with clear exposure of the anatomy of the Calot's triangle. To the best of our knowledge, such giant gallstone has been rarely reported.CONCLUSIONWe have proven that for the rare giant gallstone about 10 cm in size, LC is a feasible option if the anatomy of the Calot's triangle can be clearly exposed; otherwise, open cholecystectomy is a safe choice.     

Giant osteochondroma of the talar neck

Giant osteochondroma is an uncommon entity and it is rare in the foot and ankle region. It is extremely rare to originate from the talus. In this case report we present a case of giant osteochondroma arising from the talar neck measuring 100 mm × 90 mm × 30 mm It is unique because of the size, site and the age at presentation.     

CT evaluation of patent artery after percutaneous cryoablation of renal cell carcinoma

PurposeThe purpose of this retrospective study was to determine the incidence of persistent patent artery after percutaneous cryoablation of renal cell carcinoma (RCC) and the relationship between patent arteries one month after cryoablation and early tumor progression.Materials and methodsOne hundred and fifty-nine patients (112 men, 47 women; mean age, 63.6 ± 14.6 [SD] years; age range: 21–91 years) who underwent percutaneous cryoablation for 186 RCCs (mean diameter, 1.9 ± 0.6 [SD] cm; range: 0.7–4.0 cm) were retrospectively included. After cryoablation, patients underwent contrast-enhanced computed tomography (CT) with ≤ 2-mm slice thickness within one week from cryoablation, and at one, three, and six months. The time course of patent artery in the ablated renal parenchyma after cryoablation was the primary endpoint. The relationships between patent arteries one month after cryoablation and treatment effectiveness, tumor vascularity, tumor enhancement one month after cryoablation, tumor subtype, and renal function changes were evaluated as secondary endpoints.ResultsCT showed patent arteries in the ablated renal parenchyma within one week in 166 RCCs (89.2%), at one month in 54 RCCs (29.0%), at three months in 8 RCCs (4.3%), and at six months in 2 RCCs (1.1%). The presence of patent artery one month after cryoablation was significantly associated with tumor enhancement at the same time point (P = 0.015). There was no association between patent arteries one month after cryoablation and treatment effectiveness (P = 0.693).ConclusionPatent arteries in the ablated renal parenchyma are commonly observed on CT examination after percutaneous cryoablation of RCC. However, they gradually disappear and do not require specific treatment.     

Le prolapsus muqueux de l'urètre chez la fillette: A propos de 22 cas colligés en dix ans et une revue de la littérature

ObjectiveTo report our experience in managing 22 cases of urethral prolapse.Matérial/Patients and méthodsThis is a retrospective study conducted at Urology Department of Lamordé national hospital in a ten years period (2002-2011) and based on 22 cases of urethral prolapsed in young girls.ResultsIn the last ten years, 22 cases of urethral prolapse have been recorded. The mean age of patients was 6 ± 3,3 years (ranges 3-10 years). The main reason for consultation was mild genital haemorrhage in 18 cases and 4 cases related to suspicious sexual abuse raising medico legal problems. The treatment combined psychological and medico surgical approaches. In all the cases a surgical excision of the prolapsed mucosa was performed followed by muco-mucosal stitching around indwelling Foley catheter for 72 hours. Post operative period was uneventful except one case of acute urinary retention managed by drainage and anti-inflammatory drug.ConclusionUrethral prolapse is a rare disease that affects pre-menarcheal girls in low social and economic context. Surgical treatment gives good clinical and esthetic results.     

Contralateral ureteral metastasis 4 years after radical nephrectomy

IntroductionMetastasis of renal cell carcinoma to the contralateral ureter is extremely rare. To date, only 50 cases of metastatic RCC to the ureter have been reported, among whom 6 cases occur at the contralateral site. We herein report a rare case of metastatic RCC in the contralateral ureter 4 years after radical nephrectomy.Presentation of caseA 74-year-old man presented with gross, painless hematuria for one month. Computed tomography scan confirmed that a 1.5 cm × 0.5 cm tumor occurred in the contralateral distal ureter. A 3.5 cm segment of ureter was resected and a uretero-vesical anastomosis with psoas hitch was accomplished.DiscussionThe reappearance of hematuria after radical nephrectomy is the most common manifestation of the metastasis to the bladder or ureter. The mechanism of metastasis is not clear. In pathology, vimentin and cytokeratins might help to differentiate between metastatic clear cell renal cell carcinoma and clear cell transitional cell carcinoma.ConclusionMetastasis of renal cell carcinoma to the contralateral ureter is rare. Early recognition is extremely important in protecting the remaining renal function and prolonging life-expectancy for post-nephrectomy patients. Complete metastectomy suitable anastomosis have been shown to improve survival.     

Major complications due to transjugular liver biopsy: Incidence,management and outcome

PurposeThe purpose of this study was to retrospectively evaluate the incidence of intraperitoneal bleeding and other major complications of transjugular liver biopsy (TJLB) and analyze their outcome and management.Materials and methodsThe clinical files of 341 consecutive patients who had TJLB were retrospectively analyzed. There were 237 men and 104 women (mean age: 51.38 ± 12.8 years; range: 17–89 years). All patients had TJLB because standard percutaneous transhepatic biopsy was contraindicated. Patients’ files were reviewed to search for major and minor procedure-related complications during or immediately after TJLB.ResultsTJLBs were technically successful in 331/341 patients (97.07%; 95%CI: 94.67–98.58%). Major complications consisted exclusively of intraperitoneal bleeding due to liver capsule perforation and were observed in 2/341 patients (0.59%; 95%CI: 0.07–2.10%). They were treated using transcatheter arterial or venous embolization with a favorable outcome. The most frequent minor complications were abdominal pain (35/341; 10.26%; 95%CI: 7.25–13.99%) and supraventricular arrhythmia (15/341; 4.40%; 95%CI: 2.48–7.15%). No cases of inadvertent injury of the carotid artery were observed.ConclusionMajor complications during TJLB are extremely rare and can be managed using arterial or venous embolization with a favorable outcome. Our results reinforce the general assumption that TJLB is a safe and well-tolerated technique.     

Clinical efficacy of transcatheter embolization of visceral artery pseudoaneurysms using N-butyl cyanoacrylate (NBCA)

PurposeTranscatheter endovascular embolization within a reasonable time before rupture or deterioration of a patient's general condition is an important procedure for managing visceral pseudoaneurysms. N-butyl 2-cyanoacrylate (NBCA, enbucrilate) is an embolic material used in the blockade of visceral pseudoaneurysms. This study evaluated the clinical efficacy of transcatheter embolization of visceral artery pseudoaneurysms using NBCA.Patients and methodsBetween June 2004 and February 2014, 13 patients (9 males and 4 females; age range, 26–80 years; mean, 57.9 years) with 14 pseudoaneurysms were treated by transcatheter embolization using NBCA. NBCA was mixed with iodized oil at a 1:3 ratio to control its polymerization time and to render it radiopaque. Pseudoaneurysms were located on the gastroduodenal artery (n = 1), pancreaticoduodenal artery (n = 2), dorsal pancreatic artery (n = 1), proximal jejunal artery (n = 1), colic artery (n = 1), splenic artery (n = 3), renal artery (n = 4; two in one patient), and hepatic artery (n = 1).ResultsAll patients recovered immediately following the embolization procedure, and two patients showed minor complications that required only medical observation.ConclusionsTranscatheter embolization using NBCA for the treatment of visceral pseudoaneurysms is a safe, effective, and low-cost treatment method with a high success rate.     

Cancer du testicule: particularités cliniques et limites thérapeutiques en milieu hospitalier urologique au Sénégal

ObjectiveTo investigate the clinical features and limitations of therapeutic management of testicular cancer in Senegal.Patients and methodsThis is a retrospective study over 15 years period between January 1997 and January 2012. Twenty-two cases were collected but only 17 had complete data for analysis.ResultsThe average annual incidence was 1.13 cases per year, with a mean age of 27 ± 9.5 years. The most affected age group was between 21 and 40 years.Clinical diagnosis was suspected by the presence of a scrotal swelling in10 cases and by empty scrotum associated with abdominal or pelvic mass in the remaining 7 cases. Orchiectomy was the main treatment option, done either by high inguinal approach in 8 patients or by trans- peritoneal route in other 7 patients. Histologically, a predominance of embryonic non seminomatous germ cell carcinoma was noted in 10 cases including infantile type in one case. Seven cases developed disease progression:3 cases of peritoneal carcinmatosis, 3 other cases of locoregional invasion with retroperitoneal lymphadenopathy and one case of pulmonary metastasis. At a mean follow up of six months, nine patients died, four were lost to follow up while the remaining four cases were still alive.ConclusionTesticular cancer is a rare tumor in Senegal and usually involves young people. Clinical diagnosis is always done at very advanced stage with a very high mortality rate.     

Renal epithelioid angiomyolipoma presenting clinically as renal cell carcinoma – A case report

We describe a 22-year old female who presented with a 5-year history of a palpable, painless mass in the right flank. Computerized tomography demonstrated a solid renal mass measuring 18 cm × 13 cm with peripheral calcification, areas of vascularity and necrosis. The appearance suggested renal cell carcinoma or nephroblastoma, but percutaneous renal biopsy suggested an adrenal origin. At right radical nephrectomy, the adrenal gland was completely normal. Histology showed sheets and nests of epithelioid cells with abundant eosinophilic to clear cytoplasm, confirming a diagnosis of epithelioid angiomyolipoma (EAML), a rare mesenchymal tumor belonging to the perivascular epithelioid cell tumor family (PEComas). At 33 months followup, there was no evidence of recurrence or metastases.     

Progressive multifocal leukoencephalopathy in autoimmune diseases

Progressive multifocal leukoencephalopathy (PML) is a subacute central nervous system infection due to reactivation of the JC virus. Most reported cases occurred in HIV-infected patients (80% of cases), patients with lymphoid malignancies (13%) and transplant recipients taking immunosuppressants (5%). Less often, PML has been described in patients with chronic inflammatory joint diseases associated with autoimmune disorders (lupus, rheumatoid arthritis [RA] and vasculitis) (2%). Magnetic resonance imaging of the brain shows suggestive changes and confirmation of the diagnosis is obtained by performing PCR tests to identify the JC virus in the cerebrospinal fluid or, when necessary, a brain biopsy. No treatments have been proven effective. Most patients experience progressive disease that is fatal within a few months or induces incapacitating neurological impairments. The risk of PML is 0.4/100 000 in patients with RA. In the few case-reports of PML in RA patients, the treatments used included methotrexate (five cases) combined with a biological agent such as infliximab (one case), rituximab (four cases), or leflunomide (two cases including one with concomitant rituximab). PML is an extremely rare but devastating complication. Rituximab therapy is associated with an increased prevalence of PML in RA patients (4/100 000), who should be informed of this risk. In patients with lupus, the risk of PML is higher than in RA (4/100 000) and 40% of cases of PML occur during low-dose glucocorticoid therapy without immunosuppressive therapy.     

Early Endothelial Dysfunction in Young Type 1 Diabetics

ObjectivesEndothelial dysfunction is a known precursor of atherosclerosis and can be assessed by measuring the brachial artery flow-mediated dilatation (FMD) via ultrasonography. This study investigated endothelial function in young type 1 diabetics without cardiovascular morbidity or diabetes-related pathology.MethodsYoung diabetics and healthy controls were recruited, both meeting strict inclusion and exclusion criteria. To prove absence of subclinical atherosclerosis, intima-media thickness (IMT) measurements at the carotid bifurcation were done in all of them. FMD was measured at the brachial artery. The results were compared using the t-test and the influences of different variables on FMD were assessed using multiple linear regression.ResultsTwenty-six diabetics (23.4 ± 5.8 years) and 36 healthy volunteers (23.1 ± 2.8 years) were recruited. The duration of diabetes was 9.2 ± 5.3 years; metabolic control was moderate (HbA1c 7.6 ± 1.0%) and IMT was normal in both groups.FMD was significantly impaired in type 1 diabetics (7.13 ± 0.43 vs. 8.77 ± 0.43%; p = 0.002). The FMD grade was associated with diabetes and age. Patients with a good metabolic control (HbA1c ≤ 7.0%) had a better FMD.ConclusionsIn type 1 diabetics, even without preclinical or clinical atherosclerosis, endothelial function is already disturbed and can be detected using ultrasonography.     

Right sided spleen laying retro-duodenal: A case report and review of the literature

IntroductionUnlike left sided accessory spleen that are seen in 10–30% of cases at autopsy, cases of right accessory spleens are extremely rare. This congenital body of healthy splenic tissue simulates tumors from neighboring organs and presents a challenge in formulating a differential diagnosis.Presentation of caseWe present the case of a patient whose CT scan of the abdomen showed a large mass, 11 × 8 cm, arising retro-duodenal and lying just anterior to the right kidney. To the best of our knowledge, this is the only case where the accessory spleen was found retro-duodenal, directly anterior to the kidney and completely separate from the supra-renal gland. The chief complaint of the patient was right upper quadrant pain, radiating to the back, and colicky in nature. The patient was diagnosed with duodenal gastro-intestinal stromal tumor and a retro-peritoneal sarcoma. The mass was removed via a Kocher’s incision and immunohistological examination showed that it was a right sided accessory spleen. The patient’s left sided spleen appeared normal.DiscussionEfforts to distinguish an accessory spleen from a retroperitoneal tumor with available scans, percutaneous biopsy or biochemical tests are inconclusive. Differential diagnosis between a retroperitoneal tumor and an accessory spleen can only be made after surgical exploration.ConclusionThis case highlights the fact that surgeons should consider the possibility of an accessory spleen when making a differential diagnosis of retroperitoneal tumors.     

Is surgical resection predict overall survival in frail patients with glioblastoma,IDH-wildtype?

PurposeWe assessed the impact of frailty on surgical outcomes, survival, and functional dependency in elderly patients harboring a glioblastoma, isocitrate dehydrogenase (IDH)-wildtype.MethodsWe retrospectively reviewed records of old and frail patients surgical treated at a single neurosurgical institution between January 2018 to May 2021. Inclusion criteria were: (1) neuropathological diagnosis of glioblastoma, IDH-wildtype; (2) patient ≥ 65 years at the time of surgery; (3) available data to assess the frailty index according to the 5-modified Frailty Index (5-mFI).ResultsA total of 47 patients were included. The 5-mFI was at 0 in 11 cases (23.4%), at 1 in 30 cases (63.8%), at 2 in two cases (4.2%), at 3 in two cases (4.2%), and at 4 in two cases (4.2%). A gross total resection was performed in 26 patients (55.3%), a subtotal resection was performed in 13 patients (27.6%), and a biopsy was performed in 8 patients (17.1%). The rate of 30-day postoperative complications was higher in the biopsy subgroup and in the 5-mFI = 4 subgroup. Gross total resection and age ≤ 70 years were independent predictors of a longer overall survival. Sex, 5-mFI, postoperative complications, and preoperative Karnofsky Performance Status score did not influence overall survival and functional dependency.ConclusionIn patients ≥ 65 years harboring a glioblastoma, IDH-wildtype, gross total resection remains an independent predictor of longer survival and good postoperative functional recovery. The frailty, assessed by the 5-mFI score, does not influence surgery and outcomes in this dataset. Further confirmatory analyses are required.     

Parasitic leiomyoma: A case report with literature review

IntroductionParasitic leiomyoma is an extremely rare variant of uterine leiomyoma occurring outside uterus. The aim of this study is to report a case of parasitic leiomyoma with brief literature report.Case reportA 46-yearo-old lady presented with upper abdominal heaviness and swelling of about 6 year duration. associated with nausea, shortness of breath and palpitation. There was large well defined, mobile, hard mass in epigastric area measuring about 12 × 10 cm. Abdominal ultrasound showed well defined, solid, 94 × 76 mm, mass in the epigastric region. Abdominal computed tomography scan showed round homogenous opacity at the epigastric region with features consistent with benign lesion. Laparotomy was done, histopathological examination confirmed the diagnosis of parasitic leiomyoma.ConclusionParasitic leiomyoma is an extremely rare subtype of uterine leiomyoma, presents with vague symptoms, diagnosed by ultrasound and managed by complete resection. Previous uterine procedures have been implicated in its etiology.