[Management of blepharophimosis, ptosis, epicanthus inversus syndrome at a referral center in Tunisia]. / Prise en charge du syndrome de blépharophimosis ptôsis épicanthus inversus (SBPE) dans un centre de référence en Tunisie.

INTRODUCTION: 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. PATIENTS AND METHODS: 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. RESULTS: 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. CONCLUSION: 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.

[Clinical study and risk factors for recurrence of basal cell carcinoma of the eyelid: results of a Tunisian series and review of the literature]. / Étude clinique et facteurs de risque de récidive des carcinomes basocellulaires des paupières : résultats d'une série tunisienne et revue de la littérature.

PURPOSE: To report our results of treatment of eyelid basal cell carcinomas and evaluate risk factors for recurrence. PATIENTS AND METHODS: We carried out a retrospective study of 172 basal cell carcinomas in 168 patients, treated in the Ophthalmology department of Sousse University Medical Center (Tunisia), from January 1987 to July 2012. Initial treatment was surgical excision with a standard margin of 4mm or a single radiation treatment. When excision was incomplete, we had the choice between further excision, radiotherapy or a "wait and see" approach. Mean follow-up in our study was 11.5 months with range from 1 month to 14 years. RESULTS: We treated 169 tumors in 165 patients, with three patients out of 168 having refused the proposed treatment. Primary radiotherapy was performed in 4 cases in patients presenting with large tumors and refusing disfiguring surgery. For the 165 other tumors (95.9%), surgical excision was performed. Surgical margins were clear in 106 cases (64.2%) and positive in 37 cases (22.4%). No residual tumor was noted in 2 cases for which biopsy was initially performed, and margins could not be visualized in 20 cases (12.1%) due to the small size or fragmentation of the specimen. For the incompletely excised tumors, we performed a second excision in three cases (8%), radiotherapy in 11 cases (29.7%) and a "wait and see" approach in 22 cases (59.4%). The recurrence rate was 6.9%, with a mean time of 32.8 months until recurrence. CONCLUSION: Basal cell carcinoma represents the most frequent malignant tumor of the eyelids. Surgery remains the standard treatment. The "wait and see" approach offers an interesting option for the management of incompletely excised basal cell carcinoma, especially with low risk lesions.

[Senile scleral plaque associated with scleritis in a patient with rheumatoid arthritis]. / Plaque sclérale sénile associée à une sclérite chez un patient atteint d'une polyarthrite rhumatoïde.

Senile scleral plaque is an age-related, commonly asymptomatic hyaline degeneration of the sclera. We report a case of calcific scleral plaque associated with anterior scleritis in an elderly patient with rheumatoid arthritis. Response to anti-inflammatory treatment was favorable.

[Eyelid necrosis after injection of lidocaine with epinephrine]. / Nécrose cutanée palpébrale secondaire à une injection de xylocaïne adrénalinée.

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.

[Unilateral congenital ptosis with ipsilateral superior rectus muscle overaction]. / Ptosis congénital associé à une hyperaction du muscle droit supérieur.

Congenital ptosis may be frequently accompanied by weakness of the ipsilateral superior rectus muscle. We report a case of ptosis with ipsilateral superior rectus muscle overaction. The 45-year-old patient presented with unilateral congenital ptosis. Ipsilateral superior rectus muscle overaction was found in the upgaze, with an inferior sclera show. This can be found in 40% of congenital ptosis. The authors give two possible causes: either an exaggerated Bell reflex or misdirection innervations of the superior rectus muscle by the superior division of the oculomotor nerve, initially innervating the upper eyelid elevator muscle. Histological or electrophysiological studies can be useful to determine the physiopathology of this association.

[Oculopalpebral and facial synkinesis associated with ptosis: epidemiological, clinical, and therapeutic features]. / Les syncinésies oculo-facio-palpébrales associées au ptosis : aspects épidémiologiques, cliniques et thérapeutiques.

Synkinetic movements of the upper eyelids may be noted in association with movements of either extraocular muscles or other muscles of the face. Patients with oculopalpebral or facial-palpebral synkinesis may also have ptosis of the involved eyelid. The clinical and therapeutic features of this association are specific. We mainly distinguish two forms of synkinetic movements, characterized either by the elevation of the ptotic eyelid such as in Marcus Gunn phenomenon and in the Fuchs sign or by the falling of the upper lid such as in Marin Amat syndrome. Many surgical techniques have been used to correct the blepharoptosis and the synkinetic movement. Levator resection is often advocated for correction of blepharoptosis with a mild degree of synkinesis. However, when the ptosis is major, eyelid lag is a possible outcome. For moderate or major synkinesis, bilateral frontalis suspension with disinsertion of the levator has been suggested. This procedure generally provides satisfactory cosmetic results.

[Orbital metastasis from cutaneous melanoma]. / Métastase orbitaire d'un mélanome malin cutané.

PURPOSE: We report the case of a patient presenting with a one-sided orbital metastasis from a cutaneous melanoma, and analyze clinical features, diagnostic difficulties and prognosis of such metastasis. CASE REPORT: We report the case of a woman aged 70 years, who presented with a right one-sided proptosis associated to a decrease of the vision. The clinical features were: visual acuity at 2/10, an axile proptosis and a papillary sectorial temporal atrophy. The computed tomography (CT) disclosed an intraorbital mass with double tonality, rounded and well limited driving back the optic nerve and erasing the limits of the lateral rectus muscle. The orbital magnetic resonance imaging (MRI) showed an intra-conal fusiform mass with an heterogeneous signal in T1 and T2, heightening intensely and heterogeneously after the injection of Gadolinium. Otherwise, the patient presented with an associated respiratory failure, and thoracic CT has disclosed diffuse metastases. It was then noted that a cutaneous melanoma of the left foot had been operated ten years before. The patient died during the following month. DISCUSSION: The cutaneous melanoma is a rare cause of orbital metastasis. The diagnosis is often easy, when a primitive tumor is known, but it remains uncertain for a long time. CONCLUSION: Orbital metastases from cutaneous melanoma are rare, generally occurring at the late stage of the disease with a life expectancy not passing one year.

[Ocular ochronosis. A case report]. / Manifestations oculaires de l'ochronose. A propos d'un cas.

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.

[Craniofacial fibrous dysplasia: a case report]. / La dysplasie fibreuse cranio-faciale. A propos d'un cas.

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.