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Blepharophimosis ptosis epicanthus inversus syndrome (BPES) is a rare congenital hereditary abnormality. It includes complex orbital-palpebral malformations, causing aesthetic and functional ramifications. Management of BPES requires two steps : diagnosis and treatment.We performed a retrospective descriptive study of 44 patients (88 eyelids) with blepharophimosis-ptosis-epicanthus inversus syndrome (BPES). In our series, we opted for two-stage surgery in 28 cases : epicanthus-telecanthus surgery followed by ptosis surgery. Simultaneous surgery was performed in 5 cases.The mean age at the first visit was 6 years (6.1±6.4). The mean age of our patients at the time of the first surgery was 6.6 years. Epicanthus surgery was performed in 35 cases. The two techniques used to correct epicanthus were Y-V plasty in 30 cases (85.7%, n=35) and Y-V+double Z plasty in 5 cases (14.3%, n=35). Correction of the telecanthus was performed at the same time by a medial canthal tendon plication in 31 cases (88.6%, n=35) or transnasal canthopexy in 4 cases (11.4%, n=35). The mean age at the time of ptosis surgery was 7.23 years (±6.25), ranging from 8 months to 27 years. Ptosis surgery was performed in 41 cases (79 eyelids), of which 3 patients underwent unilateral ptosis surgery due to asymmetrical ptosis. The techniques used were levator resection in 64 eyelids and frontal suspension in 15 eyelids.BPES is often clinically diagnosed. The difficulty in management lies in the complex surgery required. There is no established consensus regarding surgical techniques or the timing of the surgeries.

To assess the impact of glaucoma treatment and ocular surface disease (OSD) on the vision-specific quality-of-life (VS-QoL) of glaucoma patients attending Farhat Hached university hospital Sousse-Tunisia.This was a cross-sectional study enrolling one-hundred-twenty patients followed for primary open angle glaucoma. All patients successfully responded to the Arabic version of the National Eye Institute Visual Function Questionnaire 25 (NEI-VFQ 25). QoL was quantified in terms of scores (0-100) and correlated with the characteristics of glaucoma treatment and status of the ocular surface.One hundred and twenty patients were studied. The mean number of medications and instilled drops was 1.95 (1-4) and 2.69 (1-7) respectively. A total of 66.7% patients reported side effects of treatment. On examination, moderate or severe dry eye syndrome was identified in 90% of cases. A total of 16.7% of patients had superficial punctate keratopathy. The number of instilled drops per day, the use of brimonidine or oral carbonic anhydrase inhibitors, and the presence of OSD had a negative impact on the NEI-VFQ 25 scores.Glaucoma treatment and OSD are 2 factors potentially reducing the QoL of glaucoma patients, on which the ophthalmologist can act by optimizing treatment and regularly examining the ocular surface of glaucoma patients.

To assess the vision-specific quality-of-life (VS-QoL) of glaucoma patients followed at Farhat Hached university hospital, Sousse-Tunisia, and identify clinical and sociodemographic factors potentially affecting it.This was a cross-sectional study enrolling one-hundred twenty patients followed for primary open angle glaucoma (POAG). All the patients successfully responded to the Arabic version of the National Eye Institute Visual Function Questionnaire 25 (NEI-VFQ 25). Quality of life was quantified in terms of scores (0-100) and correlated with sociodemographic and clinical factors.One hundred and twenty patients were studied. The mean age was 62.38±10.68years. The global mean score (GMS) score was 70.11±18.45% ranging from 23.71 to 98%. Urban residence was associated with a better total score (P=0.01). Educational background was correlated with the subscale scores of distant activities (P=0.01), social functioning (P=0.03), physical activity limitations (P=0.01) and peripheral vision (P=0.01). The total NEI-VFQ 25 had a statistically significantly negative correlation with the duration of the glaucoma (P=0.002), the cup to disc ratio of the better and the worse eye (P10Vision-specific quality of life of glaucoma patients, as measured by the NEI-VFQ 25, is correlated to many sociodemographic and clinical elements. QoL evaluation is an essential factor to be considered in the management of glaucoma patients. In our context, research should be directed first to the validation of a questionnaire in the local dialect.

Metastasis to the central nervous system, either through a hematogenous route or through the cerebrospinal fluid, is extremely rare in nasopharynx cancer. We aim to expose clinical aspects, therapeutic features and prognosis of nasopharyngeal carcinoma with brain metastases. We retrospectively reviewed the medical history of about 420 patients with nasopharyngeal carcinoma treated during 17 years at the university hospital of Sfax (Tunisia). Among them, three patients had brain metastasis. We excluded patients with direct extension to the brain. Tumours of the nasopharynx were locally advanced. The first patient had brain metastases at the initial diagnosis. The two other patients had brain metastases at 10 and 16 months during the follow-up. Ocular signs were the symptoms. Lesions were unique in two patients. Synchronous bone metastases were recorded in the three cases. All patients had whole brain radiation therapy and palliative chemotherapy. All patients had a progression of the disease and died. Brain metastases in nasopharynx cancer represent a rare event. Prognosis is poor, depending on age, surgical excision and synchronous metastases. Survival does not exceed 6 months.

Four patients (two women aged 50 and 67 years, a 36-year-old man, and a 5-year-old child) presented with necrosis of the eyelid after local anesthesia using 2% lidocaine with epinephrine. In three cases, the anesthesia was administered prior to suturing an eyelid laceration, and the necrosis involved the upper lid; in one case, the anesthesia was administered prior to temporal artery biopsy. Surgical debridement of the necrotic tissue was performed in all cases. Three patients developed a bacterial superinfection treated with systemic antibiotics with good results. Necrosis of the eyelid is a rare complication occurring after local anesthesia using lidocaine with epinephrine. Most dermatologists refrain from use of epinephrine with local anesthetics for digital blocks. Necrosis is due to prolonged vasoconstriction of arteries. It is observed especially commonly in patients at increased risk (arteritis, Raynaud's phenomenon and severe microvascular diseases). In the eyelid, few cases have been reported.

Nasopharyngeal carcinoma (NPC) represents one of the most frequent epithelial tumours of the child in intermediate risk regions. In the Maghreb, it represents the first cancer of teenagers of 15-20 years old. The Epstein Barr virus (EBV) is the most important etiologic factor. Its role in the pathogeneses of NPC has been confirmed by several studies. Young NPCs are characterized by a low rate of EBV antibodies and a high level of LMP1 cell expression than in adult's NPC. The undifferentiated carcinoma nasopharyngeal type (UCNT) represents the most frequent histological type. Immunohistochemical analyses of North Africa early onset NPC is characterized by a weak expression of bcl-2 and p53 and a strong expression of LMP1 and c-kit what makes them different from the adult's NPC. Clinically, cervical node involvement is constantly present. Juvenile NPC is characterized by a very important locoregional extension as well as a high rate of distant metastases. More than 15% of patients had metastases at diagnosis. Radiotherapy is still the standard therapy of NPC. Only some retrospectives studies have been published to determine the benefit, the type and the timing of the chemotherapy in the treatment of juvenile NPC. Metastatic relapses constitute the main cause of death at these young patients. An improvement of the prognosis can be waited with concomitant chemotherapy and intensity modulated radiotherapy. However, randomized multi institutional studies are necessary to standardize the treatment of the NPC in childhood.

Ochronosis or alkaptonuria is a rare inherited disease. It is characterized by the deposition of dark pigments in collagen-rich tissues, which leads to clinical manifestations such as arthropathy. The ochronotic pigment can be found in the sclera, the conjunctiva, and the limbic cornea. Vision is usually not affected. We report the case of 47-year-old patient who complained of lower back pain. Ophthalmologic examination showed dark pigments in the conjunctiva. The increased levels of homogentisic acid in urine confirmed the diagnosis of ochronosis.

Nasofrontal fistulas, also called nasofrontal dermal sinuses, are very rare and found for the most part in children. This congenital malformation may be revealed by local infection or neuromeningitis, making this a serious disorder. We report one case of nasofrontal dermal sinus diagnosed in an 11-month-old girl, which was complicated by left fronto-orbital infection. Through this case, the authors stress the role of imaging methods in confirming the diagnosis and looking for associated cysts (dermoid and epidermoid).

The aim of our study was to search for the incidence, the responsible organisms and the favoring causes of death of post-traumatic meningitis (PTM).This retrospective study was conducted over a seven-year period (January 1st, 1996 - December 31, 2002) in the ICU and the neurosurgery department of the Habib-Bourguiba University Hospital, Sfax, Tunisia.Over the study period, 38 patients presented PTM (0.96% of patients hospitalized for head injury), 92% of them had received antibiotic prophylaxis on admission. Mean time between head injury and the diagnosis of PTM was 9+/- 8 days (range: 2-34 days). The most common isolated organisms were multidrug resistant A. baumanii, and K. pneumoniae and reduced susceptibility S. pneumoniae. Factors predictive of prognosis in the 14 days following the diagnosis of meningitis were Glasgow coma score (GCS) on the day of diagnosis of PTM, absence of nuchal rigidity, CSF protein, CSF/blood glucose ratio, and S. pneumoniae as the causal agent of PTM.Antibioprophylaxis in patients with head trauma must be avoided to prevent the emergence of multidrug resistant bacteria when PTM occurs. GCS on the day of diagnosis of PTM, CSF protein concentration, CSF/blood glucose ratio, and S. pneumoniae as the causal agent of PTM are predictive factors of mortality of patients with PTM.

Extradural arachnoid cysts are uncommon expanding lesions in the spinal canal, which may communicate with the subarachnoid space. Usually located in the lower thoracic spine, they may cause symptoms by compressing the spinal cord or nerve roots. We report a case of an extradural thoracic arachnoid cyst revealed by progressive spinal cord compression. CT myelography and MRI enabled diagnosis. Rapid neurological improvement was observed after surgical resection.

Costo-vertebral echinoccoccus is a rare and serious condition which often presents with neurological complications requiring urgent surgical intervention.We report the case of a seven year old girl admitted with a clinical picture suggesting medullary compression secondary to multifocal Hydatid disease. As well as a costo-vertebral cyst with extension into the spinal canal, lesions were also seen in the lower lobe of her right lung and throughout the liver, confirmed by CT scan and by magnetic resonance imaging. A resection of Hydatid material as well as the posterior part of the 4th rib, the transverse process of the 4th costal vertebra and adjacent bone was carried out via a posterolateral thoracotomy. The cyst in the right lung was successfully excised at the same operation. Three months later six hepatic lesions were removed via a subcostal approach again without complication. A scan 23 months later revealed no evidence of residual hydatid disease.This case illustrates the pathophysiological features of costovertebral Hydatid disease and the advantages of an anterior surgical approach.

Fibrous dysplasia is classified among benign bone tumors. It is a bone development anomaly characterized by hamartoma proliferation of fibrous tissue within the medullary bone, with secondary bony metaplasia, producing immature, newly formed and weakly calcified bone, without maturation of the osteoblast. It can be monostotic or polyostotic. The craniofacial localization occurs in 10%-25% of cases in monostotic forms and in 50% of cases in polyostotic forms. Fibrous dysplasia essentially affects children and young adults, with no sex preference. Its diagnosis is not always straightforward because the functional symptomology is often absent or not specific. Medical imagery is necessary and in difficult cases a bone biopsy. The major complication of the illness remains sarcomatous degeneration. Only symptomatic forms are treated. We report an observation of craniofacial fibrous dysplasia affecting a child and discovery of proptosis, with no other ocular or general functional signs.

1/ To evaluate the incidence of hypothyroidism following radiotherapy in the management of breast and nasopharyngeal carcinomas, 2/ to define the role of a systematic post therapeutic screening.From January 1996 through March 2001 a systematic evaluation of the clinical and the biological thyroid function was performed on a cohort of 84 patients that received supraclavicular irradiation. Selected patients had either a mammary (37 cases) or a nasopharyngeal (47 cases) carcinoma. Initial work up included thyroid inspection and palpation, and biological tests: serum FT4 and TSH levels, radioimmunochemistry, completed by dynamic thyroid stimulation, using TRH, in case of border line low T4 or isolated high TSH levels. Tests were repeated every three months the first year, and then every six months. Replacement therapy with L Thyroxin was administered in case of hypothyroidism.All selected patients had a normal function initially. With a mean two years follow up (1-5 years), 24 patients (29%) experienced hypothyroidism, half of whom (13 cases) being purely biological. Five patients (11%), with a nasopharyngeal carcinoma, presented also with associated pituitary failure. Clinical symptoms were minor or mild in all cases. Hypothyroidism was detected at a mean 21 months follow up. In 2 patients, hypothyroidism disappeared spontaneously within 6 months. Possible predictive factors were evaluated: age of the time of radiation, gender, percentage of irradiated thyroid, total dose, dose per fraction, tumour type and chemotherapy. Only age appeared significantly correlated with thyroid dysfunction (range: 10-30 years, P=0.002).Hypothyroidism is a frequent and certainly underestimated complication following radiotherapy of the neck. In such patients, a systematic clinical and biological evaluation every three months the first year, and then every six months until five years is recommended.

Aicardi syndrome is usually associated with a very poor outcome.We report a particular case of Aicardi syndrome characterised by a partial agenesis of the corpus callosum associated with an arachnoidal interhemispheric cystic formation, and by a complete control of epilepsy and a favorable outcome regarding visual and psychomotor performances.A better prognosis could be expected in Aicardi syndrome when spasms occur lately and are not preceded by partial seizures, when agenesis of corpus callosum is partial and especially when chorioretinal lacunae are small, isolated, few and spare the macula.

By definition, image-guided neuroendoscopy is a connection between a neuroendoscope and a neuronavigational system creating a computer-guided instrument. Our objective was to adapt our rigid endoscope with our neuronavigation system to perform computer-guided endoscopy.The rigid neuroendoscope, equipped with light emitting diodes, was connected to the work station. We report our early results with this device in 17 patients: 8 ventriculocisternostomies, 5 ventriculocisternostomies with biopsies of tumors of the posterior part of the third ventricule, 4 biopsies or tumor removal like colloid cysts. No technical complications were encountered.The development of image-guided neuroendoscopy has modified our approach to neuroendoscopy because the surgical procedure is facilitated. The use of fluoroscopy becomes unnecessary. The surgeon can choose, independent of the preestablish surgical technique, the entry point, the target and, of course, the optimal trajectory. The technique is adaptable to the individual anatomy of each patient, the location and the nature of the target. It is an undeniably useful teaching tool and represents a real progress in minimally invasive neurosurgery.

The direct transnasal approach is a less invasive approach to the sella turcica and represents an alternative to the sublabial transsphenoidal approach with minor oro-nasal complications. In this study, we describe this approach with emphasis on post-operative course.We studied retrospectively 60 patients operated on consecutively by a transnasal approach between January 1996 and December 2000.There were 47 macroadenomas, 9 microadenomas and 4 non-adenomatous pathologies. Despite its narrowness, the transnasal approach was possible in all cases and allowed tumor removal as satisfactory as with the sublabial approach. Eight patients had complications not directly related to the approach: 1 death (meningitis with brain stem ischemia), 3 pituitary fossa hematomas, 4 rhinorrheas and 2 meningitis. Seven patients had complications related to the approach: 2 nasal obstructions, 1 nasal dryness, 2 sinusitises and 2 hyposmias. All these local complications were transitory and disappeared within 3 months.In our experience, the direct transnasal approach is safe, quick and less invasive than the sublabial approach.

A chronic steroid-dependent hyalitis was the first sign of a transscleral erosion in three patients with severe myopia. This erosion was seen for more than 5 years after the procedure which consisted of a very tight scleral buckling with a 5 mm wide Lincoff sponge. Hyalitis disappeared in all three cases after removal of the material. In one case, the transscleral erosion was the cause of a recurrent retinal detachment. The sensitivity of hyalitis to oral steroid therapy suggests an immune process. Hyalitis in patients with a tight buckle must be cured by removal of the material.