Sinonasal Burkitt Lymphoma in a 2-Year-Old Child: An Uncommon Case Report.

Burkitt Lymphoma (BL) is a highly aggressive B-type non-Hodgkin lymphoma. It rarely develops at the level of the sinonasal cavities, especially in young children. We present the case of a 2-year-old boy who presented with a 2-month history of nasal obstruction, snoring, and epistaxis followed by a recent dyspnea. Examination revealed a protruding tissue mass in the left nasal cavity. Computed tomography scan showed a homogenous soft-tissue mass in the left nasal cavity extending toward the ipsilateral orbit, infratemporal fossa, nasopharynx, as well as the ethmoid and maxillary sinuses. Magnetic resonance imaging revealed a homogenous infiltrating mass with a hypo-intense signal on T2-weighted images reaching the infratemporal fossa and oropharynx. Biopsy confirmed the diagnosis of BL. The patient was referred to the clinical hematology department and received complex chemotherapy. Following chemotherapy, imaging revealed significant regression of the mass. We performed a nasopharyngeal biopsy which confirmed the absence of any persistent disease. This case report highlights a rare case of an extensive sinonasal BL emphasizing the importance of a high level of suspicion to ensure an early diagnosis and treatment.

An intramuscular hemangioma of the semispinalis muscle: A case report.

Intramuscular hemangiomas are uncommon benign endotheliomas that typically occur in the trunk and limbs. Head and neck involvement is relatively infrequent, with the masseter muscle being the most commonly affected site. We present a rare case of intramuscular hemangiomas arising from the semispinalis muscle. A 31-year-old male presented with a painless swelling in the left upper neck region, gradually increasing in size over the past year. Imaging studies revealed a well-defined mass originating from the semispinalis muscle. Surgical excision was performed successfully, and histological examination confirmed the diagnosis of a mixed intramuscular hemangioma. The patient remained recurrence-free during the 2-year follow-up period. Intramuscular hemangiomas in the posterior neck muscles are rare, with only a few reported cases. Wide surgical resection with control of feeding vessels is the optimal treatment, and follow-up is recommended to monitor for local recurrence. This case report highlights the clinical presentation, diagnostic challenges, and successful surgical management of intramuscular hemangiomas in a unique location, emphasizing the importance of accurate diagnosis and appropriate treatment of this rare tumor.

Tracheocele: A Rare Entity.

Introduction: Tracheocele or tracheal diverticulum is an uncommon benign entity that can be congenital or acquired. It is usually diagnosed incidentally on cervicothoracic imaging. Our aim is to describe the etiopathogenic, clinical and paraclinical features of the tracheocele as well as its therapeutic modalities. Case Report: We report 2 cases of asymptomatic congenital tracheocele occurred in a boy and a woman, incidentally found on cervical CT scan done for accidental ingestion of chicken bone and infected thyroid hematocele respectively. The tracheocele, in our 2 cases, was probably congenital: no risk factors were noted and the opening of the tracheocele was narrow. The tracheocele was located in the right posterolateral tracheal wall in the 2 cases. It communicated with the tracheal lumen in one case. The female patient underwent a right lobectomy and resection of the tracheocele. For the boy, our attitude was conservative. The evolution was uneventful in the 2 cases. Conclusions: The presence or absence of risk factors, CT scan, bronchoscopy and histologic exam may distinguish between congenital and acquired forms. Asymptomatic patients are managed conservatively. Surgical resection is the treatment of choice for symptomatic patients.

Evidence of Direct Toxicological Effects of Scorpion Venom on Central Nervous System in Tunisian Children.

BACKGROUND: Severe scorpion envenomation can lead to severe neurological manifestations, which are an indicator of the severity of the scorpion sting. The direct action of scorpion venom on the central nervous system can explain partly these neurological disorders. METHODS AND FINDINGS: We report a case of severe scorpion envenomation in 16-month-old boy with no pathological history admitted in ICU for severe scorpion envenomation. The result of cerebral MRI agrees with the hypothesis of direct action of scorpion venom on the central nervous system. Patient had improved; however, he has kept as neurological sequelae language disorders and blindness. The boy was discharged 21 days after ICU admission. CONCLUSION: Our observation confirms that severe scorpion envenomation can be complicated by severe neurological manifestations. Although one case report is not enough to conclude such important hypothesis regarding the direct effect of scorpion venom on central nervous system (especially that the age of patient is more than one year), our case agrees with this hypothesis.

[Role of magnetic resonance imaging in preoperative assessement of hilar cholangiocarcinoma]. / Rôle de l'imagerie par résonance magnétique dans l'exploration des cholangiocarcinomes hilaires.

Magnetic resonance cholangiopancreatography is a non-invasive imaging, highly performant in detecting and locating biliary stenosis and in predicting its malignancy. The combination of two and three-dimensional MRI sequences is recommended to assess a biliary obstacle. Cholangiopathies are the main differential diagnosis of cholangiocarcinomas. In addition to bile duct abnormalities beyond the stenosis, the predictive signs of malignancy are: asymmetric irregular luminal narrowing (longer than 3mm and thicker than 13mm), abrupt discontinuation of stenosis, and enhancement superior to that of the adjacent hepatic parenchyma during arterial and portal acquisitions. Diffusion sequences improve the sensitivity of biliary stenosis detection. Moreover, restriction of diffusion is useful for distinguishing malignant from benign stenosis. Combining Magnetic resonance cholangiopancreatography, late LAVA and diffusion sequences improves the sensitivity of detection of a possible biliary extension without significant specificity. MRI with gadolinium chelate injection is efficient in assessing vascular extension of cholangiocarcinomas. However its accuracy remains lower than that of Ct-scan. To assess hepatic, lymphatic and peritoneal extensions of cholangiocarcinomas, it is fundamental to combine diffusion and dynamic MRI sequences.

Intima-media thickness of carotid artery in patients with Behçet's disease.

BACKGROUND AND AIMS: In this study we investigated the carotid intima-media thickness (cIMT) in Tunisian patients with Behçet's disease (BD), and we evaluated the relationship between traditional and nontraditional cardiovascular risk factors, disease manifestations, disease severity, use of immunosuppressive agents and cIMT in patients with BD. METHODS: Fifty patients with BD were individually matched to 50 control subjects on the basis of age, gender and traditional cardiovascular risk factors. Subjects with diabetes mellitus, evidence of myocardial infarction or cerebrovascular disease were excluded from the study. Patients with arterial involvement were also excluded. We measured serum high-sensitivity C-reactive protein (hsCRP), fibrinogen, creatinine levels and lipid profile. We also measured serum vitamin B12, folate, total plasma homocysteine levels, and HOMA-IR values. We used B-mode ultrasonography to assess the cIMT. RESULTS: cIMT in the BD group was significantly higher than in the control group (0.658 ± 0.112 mm vs. 0.581 ± 0.087 mm, respectively, p <10(-3)). The frequency of plaques in the carotid arteries was similar between study groups. In univariate analyses, the cIMT of BD patients was correlated with age (r = 0.510, p <10(-3)), male gender (p = 0.032), and creatinine clearance (r = -0.421, p = 0.003). It was inversely correlated with HOMA-IR values and serum vitamin B12 level. cIMT values were independent of disease manifestations, disease duration, or corticoid therapy. In the multivariate analysis, only male gender and creatinine clearance remained significantly associated with cIMT. CONCLUSIONS: Our data indicate morphologic evidence of subclinical atherosclerosis in patients with BD. Increased arterial wall thickness was not associated with the disease duration, clinical manifestations and immunosuppressive therapy.

A novel dominant mutation in SIX1, affecting a highly conserved residue, result in only auditory defects in humans.

Branchio-oto-renal (BOR) and Branchio-otic (BO) syndromes are dominant disorders characterized by variable hearing impairment (HI) and branchial defects. BOR includes additional kidney malformations. BO/BOR syndromes are genetically heterogeneous and caused by mutations in EYA1 and SIX1 genes. Mutation in SIX1 is responsible also for DFNA23, a locus for non-syndromic HI. Strikingly, the severity of the phenotype did not seem to correlate with the type of SIX1 mutation. Herein, we identified a novel mutation in SIX1 (p.E125K) in a Tunisian family with variable HI and preauricular pits. This mutation is located at the same position as the mutation identified in the Catwhesel (Cwe) mouse. No renal and branchial defects were observed in our family nor in Cwe/+ mice. A homology model revealed that the replacement of the Glutamate by a Lysine alters the electrostatic potential surface propriety which may affect the DNA-binding activity.

Adenoid cystic carcinoma of the parotid with facial nerve invasion.

BACKGROUND: Adenoid cystic carcinoma comprises approximately 10% of all epithelial salivary neoplasms and most frequently involves the parotid. Perineural invasion is a common feature but intraneural invasion and spread is less common. AIM: The authors report a case of parotid adenoid cystic carcinoma with invasion and spread via the facial nerve with an emphasis on radiologic features. OBSERVATION: We report a case of a 47-year-old man presenting with a right submandibular mass evolving for 4 years. On physical examination, the mass was firm painless, well delineated. There was no facial paralysis. The magnetic resonance imaging (MRI) examination showed a right parotid gland solid mass located in the deep lobe of the parotid gland with mastoid bony involvement.The mass was heterogenously enhanced. High resolution non contrast computed tomographie (CT) scan of the temporal bone showed in the axial and coronal cuts, a widening of the mastoid segment of the facial canal with involvement of the facial nerve. Pathologic examination of the resected specimen revealed histological features of adenoid cystic carcinoma with intra neural invasion of the mastoid segment of the facial nerve. The surgical margins were free. The evolution was favourable 5 years later. CONCLUSION: This study confirms that multiplanar approach of MRI allows rapid detection of adenoid cystic carcinoma. The influence of intraneural invasion and spread on survival remains controversial.

[Traumatic head injury in children in south Tunisia epidemiology, clinical manifestations and evolution. 454 cases]. / Traumatismes crâniens chez l'enfant dans le sud Tunisien profil epidémiologique, manifestations cliniques et evolution. A propos de 454 cas.

AIM: To determine epidemiological, causes, clinical and Para clinical manifestations and outcome in children with traumatic head injury in south Tunisia. METHODS: A retrospective study over a 8 year period (1997-2004) of 454 children's with head injury admitted to the Intensive Care Unit (ICU) of a university hospital (Sfax-Tunisia). Basic demographic, clinical, biological and radiological data were recorded on admission and during the ICU stay. RESULTS: Mean age (+/- SD) was of 7.2 +/-3.8 years. The main cause of trauma was traffic accidents (69.4%). There were a predominance of male patient's with a sex-ratio of 2.21. The Score of coma of Glasgow adapted to the child, was on average from 8 +/- 3 points. Extra-cranial pathology was present were observed in 38.9%. The cerebral CT-Scan performed on admission for all patients was pathological in 86.3% of the cases. The most observed lesions were meningeal Haemorrhage (35.2%) and the cerebral contusion (34.5%). According to the "Traumatic Coma Dated Bank classification" we noted a high proportion of type 2 group (46%). Secondary systemic insults were observed in 377 children (83%). Evolution was marked by the death of 82 children (18%). Among survivors, a good recovery was observed in 229 children (50.4%). CONCLUSION: In Tunisia, head injury is a frequent cause of hospitalization. It is essentially involved in traffic accidents. The short term prognosis is poor with a high (18%) mortality. Prevention is highly advised.