Hydatid cysts of the brain stem. Two cases

Cerebral hydatid cysts represent 2-3% of all intracranial masses in endemic countries. Its incidence in posterior fossa is very rare. We report two cases of brainstem location. Clinically, the lesion exhibited signs of brainstem tumor. In two patients, CT scan showed a hypodense lesion. There was no enhancement after contrast administration. One patient was explored by MRI; on precontrast images, the lesion appeared homogeneous with hyposignal intensity and smooth limits. T2 weighted MRI and post contrast examination confirmed the cyst nature of the lesion. Surgery was performed in the two patients. The cyst was first aspirated and its membrane was then removed. Post operatively, one patient died, the other one is still alive but severely affected two years later. CT scan showed total disappearance of the cyst. The clinical presentation, radiological findings and surgical procedures are discussed.     

Primary sporadic Burkitt lymphoma of the orbit,clinical characteristics,management, and outcomes: a case study

Background

Involvement of the orbit with Burkitt’s lymphoma is a very rare presentation of extra-nodal lymphoma.

Illustrative case

We report a case of a 2-year-old female presented an unusual location of sporadic Burkitt’s lymphoma arising in the orbital region. Diagnostic magnetic resonance imagining identified an oval-shaped mass on the lateral rectus of the right orbit that caused dislocation of eyeball, for which she underwent a biopsy from the periorbital swellings.The mass was histologically confirmed as Burkitt’s lymphoma, and postoperative aggressive chemotherapy was initiated.We describe clinical diagnosis, histological aspects, radiological features, and current management of this rapidly growing malignant tumor.

Conclusion

Because of the rapid progression of Burkitt lymphoma, and considering that it responds well to treatment, early recognition and appropriate management are important factors for survival and to preserve visual function.

     

Management of intramedullary spinal cord tumors: surgical considerations and results in 45 cases

Background and purpose

Intramedullary spinal cord tumors (IMSCT) are relatively rare neoplasms, accounting for less than 5% of all central nervous system tumors. The optimum management of these tumors still remains controversial. Many decades ago, partial surgical resection followed by radiotherapy was the conventional management for IMSCT. Nowadays, maximal surgical resection of IMSCT without adjuvant therapy is the rule. We discuss the management of our cohort of 45 patients and review retrospectively the surgical outcome and survival.

Methods and results

We reviewed the charts of 45 patients who underwent surgery for IMSCT in our institution since 1990. The study included 23 female and 22 male with a mean age of 28.7 years (range: 18 months-64 years). In 40 patients, the final diagnosis was based on the results of MR imaging. The cervical location of the tumor was the most common (20 cases). Surgical procedures included a gross-total resection in 31 cases, subtotal resection in six cases, partial resection in five cases and a biopsy in three cases. The large majority of patients had histologically-proven low-grade tumors composed essentially of astrocytomas (44,4%) and ependymomas (28,8%). There was no mortality related to surgery. Concerning the functional outcome at six months, we noted that 22.2% of our patients deteriorated, 47.3% stayed the same and 30.5% improved. We found that patients with mild or no preoperative deficits were exceptionally damaged by the surgical procedure.

Conclusions

The gold-standard treatment of IMSCT remains maximal microsurgical resection without adjuvant therapy. For malignant or rapidly recurrent IMSCT, the optimum management is still controversial. Determinant predictors for a good outcome after surgery of IMSCT are histological type of lesion, total removal of the tumor and a satisfactory neurological status before surgery.     

Squamous cell carcinoma arising in a papillary carcinoma of the thyroid: a case report

BACKGROUND: primary squamous cell carcinoma (scc) of the thyroid is a rare malignant tumor that needs to be distinguished from other neoplasms. Cases of papillary carcinoma merging in association with squamous cell carcinoma are extremely rare. AIM: the characteristics of such association will be studied herein through a review of literature. CASE REPORT: we report a case of an 87-year-old patient presented with an enlarged right lobe of the thyroid. Thyroid scintigraphy revealed a cold nodule. Clinically, malignancy was suspected. Histopathologic diagnosis was a well-differentiated scc associated with areas of papillary carcinoma. Exhaustive clinical, endoscopic and radiological examinations did not reveal any primary site of scc or any contiguous spread from neighboring structures. Surgery was followed by radiotherapy, but the patient died 6 months later. CONCLUSION: the coexistence of epidermoide and papillary carcinomas in the thyroid gland must be known because of its prognostic impact.     

Decompressive craniectomy in the management of sylvian infarction

Space-occupying middle cerebral artery infarction represents about 10 to 15% of supratentorial ischemic strokes. This syndrome carries a high rate of mortality and requires aggressive surgical decompression. The authors present 6 patients with signs of trans-tentorial herniation operated on between February 2001 and August 2003. Neurological preoperative status was evaluated with Glasgow coma scale score and postoperatively with Barthel index. Three patients had excellent recovery (Barthel Index up to 70), one remained dependant and two died. Younger patients had better prognosis. Decompressive surgery, when done early, should improve mortality rate and even functional outcome. Optimal selection of patients, with the help of Diffusion-Weighted imaging, could vouch good results.     

Aortic biological valve thrombosis in an HIV positive patient

Biological aortic valve thrombosis is an exceptional complication. A 64-year-old patient positive for human immunodeficiency virus presented for syncope on exertion, 2 years after an aortic bioprosthetic valve replacement and double coronary artery bypass. Transvalvular aortic mean gradient was approximately 50 mm Hg on echocardiogram and catheterization. Cardiac computed tomography scan showed a limited opening of the bioprosthesis cusps. Surgical exploration revealed thrombosis of the three cusps on the aortic side, limiting the opening of the valve. No relation could be established between the patient's human immunodeficiency virus status and valve thrombosis.     

DFNB66 and DFNB67 loci are non allelic and rarely contribute to autosomal recessive nonsyndromic hearing loss

We previously mapped the DFNB66 locus to an interval overlapping the DFNB67 region. Mutations in the LHFPL5 gene were identified as a cause of DFNB67 hearing loss (HL). However, screening of the coding exons of LHFPL5 did not reveal any mutation in the DFNB66 family. The objective of this study was to check whether DFNB66 and DFNB67 are distinctive loci and determining their contribution to HL. In the DFNB66 family, sequencing showed absence of mutations in the untranslated regions and the predicted promoter sequence of LHFPL5. Analysis of five microsatellites in the 6p21.31–22.3 region and screening of the LHFPL5 gene by DNA heteroduplex analysis in DHPLC revealed a novel mutation (c.89dup) in one out of 129 unrelated Tunisian families with autosomal recessive nonsyndromic (ARNS) HL. Our findings suggest that two distinct genes are responsible for DFNB66 and DFNB67 HL. These loci are likely to be a rare cause of ARNSHL.     

VEP contrast sensitivity responses reveal reduced functional segregation of mid and high filters of visual channels in autism

Despite the vast amount of behavioral data showing a pronounced tendency in individuals with autism spectrum disorder (ASD) to process fine visual details, much less is known about the neurophysiological characteristics of spatial vision in ASD. Here, we address this issue by assessing the contrast sensitivity response properties of the early visual-evoked potentials (VEPs) to sine-wave gratings of low, medium and high spatial frequencies in adults with ASD and in an age- and IQ-matched control group. Our results show that while VEP contrast responses to low and high spatial frequency gratings did not differ between ASD and controls, early VEPs to mid spatial frequency gratings exhibited similar response characteristics as those to high spatial frequency gratings in ASD. Our findings show evidence for an altered functional segregation of early visual channels, especially those responsible for processing mid- and high-frequency spatial scales.     

Isolated frontal sinus fungus ball: Diagnostic and therapeutic features and the role of endonasal endoscopic approach

Fungus ball (FB) is a non invasive form of fungal sinusitis that generally affects immunocompetent subjects. Isolated involvement of the frontal sinus is extremely rare. The treatment is surgical. Previously, it was based on the external approach. Recently, the endoscopic approach has been increasingly employed.We report three cases of frontal sinus fungus ball. Two patients underwent endoscopic endonasal frontal Draf type IIb sinusotomy with complete removal of the pathologic material. The third patient had an external approach due to the extensive pneumatisation of the frontal sinus, the defect in its floor and the orbital involvement. There were no intraoperative or postoperative complications. No recurrence of the disease was observed during the three, two and twelve months’ follow up period, respectively.Correct clinical and radiological diagnosis of isolated frontal sinus FB still remains a challenge. Endoscopic sinus surgery with endonasal Draf type IIb or type III frontal sinusotomy is effective for the treatment of frontal sinus FB. It is the treatment of choice and replaces the traditional external approaches.