Anatomo-Clinical Aspects of Retinoblastoma: A Series of 144 Cases.

Retinoblastoma (RB) is the most common intraocular primary malignancy for infants and young children. The tumor is bilateral in 40% of cases and unilateral in 60% of cases. The hereditary form is due to a germinal mutation in the RB1 tumor suppressor gene. In developed countries, patients treated for RB have excellent survival, but unfortunately in developing countries delays in diagnosis and lack of human and financial resources are responsible for deaths. We conducted a retrospective study of 144 cases of RB in order to evaluate the clinico-pathological aspect of RB for the national reference center of RB in Morocco. Our study highlighted the indispensable collaboration between the clinician and the pathologist. Besides the diagnostic confirmation, the anatomopathological study gives us information on histopronostic risk factors to guide the treatment.

Bilateral cataract in a child with blepharophimosis-ptosis-epicanthus inversus syndrome: A surgical challenge.

INTRODUCTION: Blepharophimosis-ptosis-epicanthus inversus syndrome (BPES) is a rare autosomal dominant genetic disorder characterized by complex orbito-palpebral anomalies. We report a rare case of BPES associated with bilateral congenital cataract. OBSERVATION: This study reports the case of a 6-month-old infant with BPES in whom a bilateral congenital cataract was diagnosed, after the parents noticed leukocoria and signs of poor vision in their child. No other ophthalmologic manifestations commonly associated with this syndrome were found. The infant underwent cataract surgery first, with lens phacoaspiration and posterior capsulotomy coupled with anterior vitrectomy and placement of a 3-piece foldable hydrophobic posterior chamber lens in the capsular bag. The surgery was a real challenge due to the orbito-palpebral anomalies that limited a small surgical space, and the placement of the IOL was a matter of discussion. DISCUSSION: Publications on the association of congenital cataract with BPES are very rare. The link between these two anomalies is difficult to establish since different genes on different chromosomes code for the two diseases. A lateral canthotomy can be considered to overcome the surgical difficulties due to the reduced working space. The surgical management of pediatric cataract varies in the literature. CONCLUSION: This case highlights the difficulty of cataract surgery in children, even more so when associated with BPES, and the challenge of improving vision in these children given the high risk of amblyopia.

Spontaneous corneal perforation complicating ocular rosacea: Case report.

INTRODUCTION: Ocular rosacea is a multifactorial disease. Its pathophysiology remains unclear. The ocular manifestations of rosacea are not specific and can range from simple blepharoconjunctivitis to sight-threatening such as corneal perforation. CASE REPORT: We report the case of a 10-year-old child who presented with a red painful right eye. Based on the clinical findings, we concluded that she had a corneal perforation on ocular rosacea. She benefited from an ipsilateral lamellar autokeratoplasty by lamellar autograft. The evolution was marked by a good healing and a good visual recovery despite a corneal scar. DISCUSSION: Ocular rosacea is a multifactorial disease, with an unclear physiopathology. Corneal involvement remains the least common, but the most challenging since serious complications can occur. Corneal perforation is the most severe. Several techniques have been reported and used in the management of corneal perforations such as conjunctival flap, amniotic membrane grafting, and the use of a corneal patch. The later, corneal autografting, remains a simple and effective technique with satisfactory anatomical results. CONCLUSION: Ocular rosacea is a pathology that is still poorly understood and of delayed diagnosis. It can lead to serious vision-threatening complications such as corneal perforation. The corneal patch is a simple, effective and efficient technique that has given good results in our case.

[Unilateral exophthalmia revealing large aneurysm of the internal carotid: a case report]. / Exophtalmie unilatérale révélant un grand anévrysme de la carotide interne: à propos d´un cas.

Intracavernous carotid artery aneurysm is rare but it leads to major complications. Common clinical picture is characterized by ophthalmoplegia associated with intense headaches. Cerebral angiography is used to confirm clinical suspicion and it guides therapeutic management. Endovascular embolization is the standard treatment. We here report the case of a 80-year-old woman presenting to the emergency room with right-sided exophthalmos and severe headaches occurring 2 weeks before her visit. The diagnosis of fissurized intracavernous carotid artery aneurysm was retained.

[Late exophthalmos revealing post-traumatic carotid-cavernous fistula: a case report]. / Exophtalmie tardive révélant une fistule carotido-caverneuse post traumatique: à propos d'un cas.

Carotid-cavernous fistula is an abnormal communication between the carotid artery and the cavernous sinus. It is most commonly due to congenital arteriovenous malformations but it can also be caused by a trauma. It is a rare disease which can give rise to visual and life-threatening consequences. We here report the case of a young female patient aged 34 years who had been victim of road accident with craniofacial and lower limb trauma two years before her admission. Left exophthalmos with slowly progressive evolution occurred one year after road accident without other associated signs, revealing left carotid-cavernous fistula. What is peculiar about this case is the relatively long delay between the trauma, the onset of exophthalmos and the diagnosis of post-traumatic carotid-cavernous fistula.

[Eyelid molluscum contagiosum: a case report]. / Molluscum contagiosum palpébral: à propos d'un cas.

Molluscums contagiosum (MC) are benign skin lesions caused by Molluscipoxvirus, primarily affecting children and young adults. They manly involve the skin and rarely the mucous membranes. Clinical diagnosis is easy, confirmed by histological examination of the lesion. However there is no consensus regarding therapy. Eyelid molluscum contagiosum is rare, posing a problem of differential diagnosis especially when it is isolated as well as a therapeutic problem given the proximity of the eyeball. We report the case of a 7-year old girl with isolated eyelid lesion. The patient underwent lesion excision. Anatomopathological examination showed molluscum contagiosum. This study aims to describe the clinical, therapeutic and evolutionary features of this rare localization of molluscum contagiosum.