Habiba Mizouni

Microscopic polyangiitis presenting with peripheral and central neurological manifestations

Although peripheral neuropathy is a common complication of microscopic angiitis, manifestations involving the muscle and the central nervous system have been rarely reported. We describe a 48-year-old man who rapidly developed a clinical picture of mononeuritis multiplex. A month after the appearance of the primary symptoms, he became comatose and had left hemiplegia in relation with a massive cerebral haematoma. Laboratory data revealed signs of inflammation, glomerular dysfunction with microhaematuria, and positive myeloperoxidase-antineutrophil cytoplasmic antibodies. The neuromuscular biopsy disclosed a small-vessel vasculitis, consisting with microscopic angiitis, associated with myositis and extensive axonal loss. The patient had surgical evacuation of the haematoma and received immunosuppressive therapy with good outcome. Thus, microscopic angiitis should be considered as a differential diagnosis in cases of myositis and intracerebral haemorrhage.

Adamantinoma of the tibia and fibula with pulmonary metastasis: an unusual presentation

Adamantinoma is a rare tumour of long bones, representing less than 1% of them. Adamantinoma commonly occurs in the tibia. It is locally aggressive and recurrences are uncommon after resection. Metastases have been reported in less than 10% of cases. The most common radiographic appearance is multiple sharply demarcated radiolucent lesions surrounded by areas of dense sclerotic bone. The authors report a patient who developed pulmonary metastasis 1 year after complete resection of primary neoplasm.

Tuberculosis tenosynovitis of the extensor tendons of the wrist

Mycobacterial tuberculous tenosynovitis of the extensor tendon sheath is an extremely rare manifestation of extrapulmonary tuberculosis. The diagnosis may be easily delayed because of its non-specific clinical signs. We report a new case of tuberculous tenosynovitis of the extensor without concomitant pulmonary tuberculosis or documented immunodeficiency.

Acute myocardial infarction in a patient with hypofibrinogenemia: a case report

IntroductionCongenital fibrinogen deficiency is a rare coagulation disorder usually responsible for hemorrhagic diathesis. However, it can be associated with thrombosis and there have been limited reports of arterial thrombotic complications in these patients.Case presentationA 42-year-old Tunisian man with congenital hypofibrinogenemia and no cardiovascular risk factors presented with new onset prolonged angina pectoris. An electrocardiogram showed features of inferior acute myocardial infarction. His troponin levels had reached 17 ng/L. Laboratory findings confirmed hypofibrinogenemia and ruled out thrombophilia. Echocardiography was not useful in providing diagnostic elements but did show preserved left ventricular function. Coronary angiography was not performed and our patient did not receive any anticoagulant treatment due to the major risk of bleeding. Magnetic resonance imaging confirmed myocardial necrosis. Our patient was managed with aspirin, a beta-blocker, an angiotensin-converting enzyme inhibitor and statin medication. The treatment was well tolerated and no ischemic recurrence was detected.ConclusionAlthough coronary thrombosis is a rare event in patients with fibrinogen deficiency, this condition is of major interest in view of the difficulties observed in managing these patients.

Infant-Type Anomalous Origin of the Left Coronary Artery From the Main Pulmonary Artery Diagnosed With Sixty-Four Multislice Computed Tomography

A 5-month-old girl was referred to the paediatric cardiology department for dyspnea, pallor, and failure to thrive. The onset of symptoms occurred about 8 weeks after birth. Her electrocardiogram showed ischemic change. Transthoracic echocardiography showed global hypokinesia with decreased left-ventricular ejection fraction (30%). The origin of the right coronary artery (RCA) was well visualized, but that of the left coronary artery (LCA) was not. Anomalous origin of the LCA was then suspected. A 64-multislice computed tomography (MSCT; VCT General Electric HealthCare, Milwaukee, WI) scan was performed using retrospective electrocardiographic (ECG)gating after intravenous administration of 12 ml iodinated contrast medium (Omnipaque 350; Amersham Health, Cork, Ireland) at a rate of 2.5 ml/s. The following imaging parameters were implemented: slice thickness 0.6 9 64 mm, speed rotation 0.33 s, tube voltage 80 KV, rotation time 330 ms, and ECG-modulated mA. Scanning was performed in the craniocaudal direction at a regular heart rate of 80 beats per minute after administration of a beta-blocker. Source images were reconstructed in both systolic and diastolic phases. Multiphase reconstruction data images were also used to assess left-ventricular ejection fraction. Coronary computed tomographic angiography scan clearly showed anomalous origin of the LCA from the pulmonary artery (ALCAPA). The LCA arose from the left inferolateral aspect of the main pulmonary artery (PA) just beyond the pulmonary valve. It then coursed toward the interventricular groove and branched into the left anterior descending (LAD) and circumflex arteries (Fig. 1a, b). The configuration of the RCA was typical (Fig. 1c). The RCA and LCA appeared normal in size with no coronary collateral development. MDCT scan confirmed the sonographic findings. LV enddiastolic and end-systolic volumes were increased (respectively, 120 and 86 ml), and left-ventricular ejection fraction was decreased (28%) (Fig. 1d). The diagnosis of ALCAPA syndrome was confirmed, and the patient was then referred for cardiac surgery. Surgical correction was performed with restoration of a dual coronary-artery system. ALCAPA is a rare congenital anomaly, but it is one of the most common causes of myocardial ischemia and infarction in children [1]. If left untreated, B90% of infants with ALCAPA syndrome die within the first year of life [2]. MSCT is fast and widely available technique that allows the detection of coronary artery anomalies and a clear three-dimensional view. The main disadvantages of multidetector CT angiography are its relatively high radiation dose and its inability to assess flow [1].

Back pain caused by a pseudo-tumorous vertebral collapse: atypical presentation of primary vertebral hydatidosis

Hydatidosis, also known as echinococcosis, is a rare but serious parasitic disease in endemic areas. Primary spinal location is extremely rare. This case report describes a rare instance of hydatid cyst that caused severe and progressive low-back pain and neurologic dysfunction. Spine MRI showed a unique vertebral collapse of Th12 body with multicystic lesions filling the spinal canal. In addition, hydatidosis serodiagnostic test was positive at 1/725. Treatment depended on the actual surgical removal of the cysts. Surgery consisted in excision and extirpation of the cysts, associated with decompressive laminectomy. The diagnosis was confirmed on the basis of histological results. No coincidental hydatid visceral involvement was found. Antihelminthic drugs (Albendazole) were promptly given before surgery for a long period. The outcome was satisfactorily marked by total regression of the motor deficit and sphincter disorders.

Imaging of Paranasal Sinus Mucoceles

Introduction: Mucoceles are cystic masses developing after obstruction of the sinus ostium. The symptoms are not specific. Computed Tomography scan (CT scan) and Magnetic Resonance Imaging (MRI) confirm the diagnosis. Objectives: We herein review the radiologic characteristics of mucoceles in CT scan and MRI. Materials and Methods: We report a retrospective study of 43 patients diagnosed with paranasal sinuses mucoceles. CT scans were performed for all patients, but MRI was carried out only in selected cases. Results: Our study was constituted of 27 males and 16 females with a mean age of 47 years. The CT scan appearence of mucoceles were in all cases as a well circumscribed expansile sinus mass with an effect on the neighbor bone structure. This mass was hypodense in 26 cases, isodense in 14 cases and hyperdense in 3 patients. The paranasal sinuses most frequently affected in our series were the fronto-ethmoidal sinuses. The most affected bone eroded was the lamina papiracea. Intracranial extension was seen in four cases. CT scan allowed to predict the cause of mucoceles in some cases and to provide information about anatomic variants. MRI was realized for 15 patients in addition to the CT scan. It allowed to study the extension of mucoceles to the neighboring organs especially orbital and endocranial ones. Conclusion: The presentations of mucoceles on imaging are quite variable. CT scan provides precious information about the location, bone erosion and extension of the mucoceles. MRI is indicated in some cases especially in cases of orbital or cranial extension.

Otosclerosis Surgery: Contribution of Imaging in Surgical Failures andLabyrinthine Complications Diagnosis

Objective: The purpose of this study was to review the role of post-operative imaging and to illustrate with a rich iconography the main causes of surgical failures and post-operative complications of otosclerosis. Subjects and methods: This a retrospective study of 49 ears having otosclerosis and undergoing middle ear surgery. CT scan was performed in the postoperative assessment when post-operative complication or unsatisfactory surgical results were suggested. Results: Surgical failures were noticed in 28 ears (57%): Hearing loss was the most frequent symptom (89%).Tinnitus was found in 21 cases and vertigo in three cases. Audiometric exploration found 21 cases of conductive hearing loss, six cases of mixed type and one case was a sensorineural type. The causes of surgical failure were erosion of the long process of the incus in four cases, uncudo-mallear dislocation in one case, displacement of the prosthesis in five cases and fibrosis of the oval window in one case. Progression of otosclerosis was noticed in 13 cases. Labyrinthine complication was retained in 21 ears of our study. The causes of labyrinthine complication were intravestibular protrusion of the prosthesis (15 cases), intravestibular footplate dislocation (1 case), perilymphatic fistula (1 case), ossificans labyrinthitis (1 case), infectious labyrinthitis (1 case) and intravestibular granulation tissue (1 case). In one case, no abnormities was retained in CT and MRI Conclusion: After this study, to ensure maximum efficiency in the postoperative monitoring and the management of complications and post-operative failures, the clinician is required to take into account both the functional findings, results audiometric and radiological data.

Role of CT and MRI in the Follow-Up of Operated Middle Ear Cholesteatoma

Background: Recurrence is the main risk that may occur during the follow-up of operated middle ear cholesteatoma. Imaging plays an important role in its diagnosis, leading to avoid surgical second look when it is not mandatory. The aim of our study was to evaluate postoperative CT and MRI in patients who had undergone middle ear cholesteatoma surgery. Methods: Retrospective study from June 2010 to June 2015 including operated patients for middle ear cholesteatoma whom follow-up was made in the ENT department of Rabta hospital and who had postoperative CT and/or MRI in the imaging department. Comparison of radiological and second look surgical findings was made with analysis of sensitivity, specificity, PNV, PPV for each type of imaging exam. Results: Forty ears included (36 patients, median age=38. 5, sex-ratio=1.1). Thirty four ears had CT showing well aerated middle ear cleft (n=1), total opacification (n=7), partial soft-tissue opacity with convex margins (n=11), pearl-shaped lesion (n=7) and concave margins opacity (n=8). CT was not able to further characterize these opacities (specificity 20%) but it was efficient in the evaluation of ossicular and bony walls lysis. Twenty five ears had MRI showing recurrent cholesteatoma (n=15), scar tissue (n=8) and aerated postoperative cavity with alteration of the labyrinth T2 signal (n=2). MRI specificity was about 25%. 100% PNV allowed excluding recurrence when MRI was showing no soft tissue mass. PPV of diffusion weighted imaging (DWI) and delayed post contrast T1 weighted imaging was respectively 83.3% and 71.4%. A hypersignal on DWI and no contrast uptake were highly in favor of cholesteatoma. Conclusion: CT is insufficient for the diagnosis of recurrent cholesteatoma. MRI contribution is hindered by false negatives due to too small lesions to be detected.