[Marcus Gunn Syndrome. Study of 3 rare cases]. / Syndrome de Marcus Gunn. Etude de 3 cas rares.

PURPOSE: reporting three rare cases of Marcus Gunn Syndrome and analyzing the clinical features of this entity. METHODS: Case reports of three patients with a Marcus Gunn Syndrome, treated at the Ophthalmology B CHU service Rabat - Morocco. RESULTS: Our patients are two boys aged 1 and 2 years old, and a 31 year old woman with a Marcus Gunn Syndrome. As special features, in the boys' cases this syndrome is bilateral, as for the other case it is associated with congenital fibrosis clinically predominant of the Ipsilateral inferior rectus muscle in an adult. Surgical treatment was not offered for children because of their young age. The adult patient has benefited from a recession of the inferior rectus muscle and a pleating in the superior rectus muscle. The forced duction test objectified a fibrosis of the inferior rectus muscle. Surgical correction of Ptosis and Synkinesis has not been indicated in this patient, given the residual oculomotor disorder and the risk of exposure keratitis. CONCLUSION: Marcus Gunn Syndrome is exceptional in adults. This can be explained by the tendency of the lid retraction to fade with age. Bilateral involvement in Marcus Gunn Syndrome is rare. Its association with congenital fibrosis of extraocular muscles has allowed a better etiopathogenic approach highlighting a supra nuclear origin of this Syndrome.

[Langerhans cell histiocytosis located in the orbit]. / L'histiocytose Langerhansienne a localisation orbitaire.

Langerhan's cell Histiocytosis of the orbit. Langerhan's cell Histiocytosis is a rare condition that affects children and less frequently young adults. It is characterized by a proliferation of histiocytes derived from Langerhan's cells. Orbital involvement is described in 20% of cases where orbital eosinophilic granuloma located in the frontal bone is the most frequent. The malignant nature of this disease is not established. Its evolution is unpredictable and spontaneous regression after simple biopsy were described. The authors report four cases of Langerhan's cell Histiocytosis and will discuss on the clinical and radiological aspects as well as on the evolution of orbital histiocytoses X.

[Pathomimia in ophthalmology: We must acknowledge it!]. / La pathomimie en ophtalmologie : il faut y croire !

Pathomimia is an unconscious falsification of physical manifestations. The eyelids and ocular surface are almost always affected because of the easy accessibility to these structures for the purpose of attracting attention from one's entourage as well as the medical community. We report four cases of lid and conjunctival pathomimia. The diagnosis was made after a long follow-up and numerous ancillary tests.

Gamma-D crystallin gene (CRYGD) mutation causes autosomal dominant congenital cerulean cataracts.

Congenital cataracts are a major cause of bilateral visual impairment in childhood. We mapped the gene responsible for autosomal congenital cerulean cataracts to chromosome 2q33-35 in a four generation family of Moroccan descent. The maximum lod score (7.19 at recombination fraction theta=0) was obtained for marker D2S2208 near the gamma-crystallin gene (CRYG) cluster. Sequencing of the coding regions of the CRYGA, B, C, and D genes showed the presence of a heterozygous C>A transversion in exon 2 of CRYGD that is associated with cataracts in this family. This mutation resulted in a proline to threonine substitution at amino acid 23 of the protein in the first of the four Greek key motifs that characterise this protein. We show that although the x ray crystallography modelling does not indicate any change of the backbone conformation, the mutation affects a region of the Greek key motif that is important for determining the topology of this protein fold. Our data suggest strongly that the proline to threonine substitution may alter the protein folding or decrease the thermodynamic stability or solubility of the protein. Furthermore, this is the first report of a mutation in this gene resulting in autosomal dominant congenital cerulean cataracts.

[Primary hepatic localization of the PPNET (primitive peripheral neuroectodermal tumors). Case report]. / Les tumeurs neuroectodermiques primitives périphériques PPNET à localisation hépatique primaire. A propos d'un cas.

Our purpose is to study, through the case of a patient operated for right hepatic tumour, the clinical, radiological, anatomopathologic and therapeutic aspects of the peripheral primitive neuroectodermal tumours (PPNET). This tumour (PPNET) is a neoplasm belonging to the Ewing's family tumours, whose histology is similar. Its diagnosis requires the contribution of histopathology, immunohistochimy and cytogenetic studies. The primary hepatic localization of this rare tumour (our case), has never been reported. The treatment, in theory copied on that of the Ewing's sarcoma, is complex and not yet codified, which makes it another disappointing aspect of this disease whose prognosis remains dark.

[Isolated hypertrophy of the superior oblique muscle in Basedow disease]. / Hypertrophie isoleé du muscle oblique supérieur d'origine basedowienne.

INTRODUCTION: Proptosis is the most common feature of Graves' ophthalmopathy. We report an exceptional case of proptosis in relation with an isolated enlargement of the superior oblique muscle in Graves' ophthalmopathy. OBSERVATION: After ocular contusion a 37 years old man presented a progressive right exophthalmos. On examination there was an unilateral proptosis with dilated conjunctival vessels, without any clinical signs of inflammation and no decreased vision. Ocular movements were full. On general examination signs of hyperthyroidism were present. CT-scan showed a superior oblique muscle enlargement with enlarged superior ophthalmic vein. Cerebral angiography excluded the diagnosis of a carotid-cavernous fistula. Dosage of thyroid hormone revealed hyperthyroidism. DISCUSSION: This case has a double interest: --Diagnostic: a CT-scan picture mimicking a carotido-cavernous fistula in this particular clinical context (history of ocular trauma, enlarged conjunctival vessels). --Isolated enlargement of superior oblique muscle in Graves' disease, which is exceptional in the literature.

[Diagnostic difficulties in pigmented tumor of the optic nerve head. A case report]. / Difficultés diagnostiques face à une tumeur papillaire pigmentée. A propos d'un cas.

Melanocytoma of the optic disc is a benign pigmented tumor located on the optic nerve head. Most cases are visually asymptomatic but in rare cases it can cause visual loss, which does not necessarily involve malignant transformation. We present a case of melanocytoma in a 49-year-old woman who presented with decreased vision in her left eye. The pigmented mass was in close continuity with the juxtapapillary adjacent choroid. Fluorescein angiography demonstrated hypofluorescence with adjacent disc edema. Ultrasonography disclosed a high internal reflectivity mass in the optic nerve head without retrobulbar extension. Magnetic resonance imaging (T1 weighted images) disclosed a hyperintense signal on the optic nerve head. The tumor was stationary for 20 months. This report emphasizes problems differentiating a melanocytoma from malignant melanoma of the optic nerve. Unusual features of melanocytoma (superior nasal location in the optic disc with an adjacent choroidal component, a decrease in visual acuity and disc edema surrounding the tumor) are discussed. Visual loss can be induced by optic neuropathy or retinal vascular obstruction. Melanocytomas grow very slowly over several years or remains stable, in contrast to malignant melanoma. In suspicious cases, close follow-up with serial fundus photographs is essential, although malignant transformation is exceptional.

Morphological representation of order-statistics filters.

We propose a comprehensive theory for the morphological bounds on order-statistics filters (and their repeated iterations). Conditions are derived for morphological openings and closings to serve as bounds (lower and upper, respectively) on order-statistics filters (and their repeated iterations). Under various assumptions, morphological open-closings and close-openings are also shown to serve as (tighter) bounds (lower and upper, respectively) on iterations of order-statistics filters. Simulations of the application of the results presented to image restoration are finally provided.

On the invertibility of the morphological representation of binary images.

We investigate the invertibility of the morphological representation of binary images. A criteria for the invertibility of the morphological representation of binary images is proposed. Necessary and sufficient conditions for the exact reconstruction of a binary image from its morphological representation are provided.

Comments on "On the invertibility of morphological representation of binary images" [and reply].

The authors comments that Charif-Chefchaouni and Schonfeld (see ibid., vol.3, no.6, p.847, 1994) investigated the invertibility of a morphological representation of binary images and determined the necessary and sufficient conditions for its inverse. The authors show that one of the derived necessary conditions is not valid. A counterexample is given to illustrate our observations. Charif-Chefchaouni and Schonfeld reply that the new sufficient condition is proposed for the invertibility of the morphological image representation. A modification of its inverse is subsequently used to derive a new necessary condition for the invertibility of the morphological image representation. A composition of these conditions is finally used to provide a new necessary and sufficient condition under some restrictions for the invertibility of the morphological image representation. These necessary and sufficient conditions form a revision of one of the necessary conditions for the invertibility of the morphological image representation stated in the original paper.

[Solitary retinal astrocytic hamartoma simulating retinoblastoma]. / Hamartome astrocytaire isolé simulant un rétinoblastome.

Retinal astrocytic hamartoma and retinoblastoma may be very similar clinically and their differentiation in atypical cases can be difficult, even with the use of ultrasonography and computed tomography. In such cases, a close follow-up is recommended before enucleation. This paper reviews the case of a 18 month old girl who presented with a solitary retinal astrocytoma of the right eye, without any other physical or ocular disorder. The initial presentation simulated a retinoblastoma; nevertheless atypical patterns as yellow calcifications and the lack of tortuous and dilated feeding blood vessels were present. Ophthalmoscopic and ultrasound regular evaluation did not reveal any change after one year follow-up. Additional investigations performed in order to exclude tuberous sclerosis (neurological and dermatological examination, CT-scans) showed no other organ involvement, which ruled out a phakomatosis. The clinical appearance and course of astrocytic hamartomas, its differential diagnosis from other retinal tumors, especially retinoblastoma, and its association with tuberous sclerosis are discussed.

[Ocular relapse of acute lymphoblastic leukemia]. / Rechute oculaire de leucemie aigue lymphoblastique.

A bilateral leukemic hypopyon can be inaugural in the child's leukemia or reveal a relapse. A five years old child with acute lymphoblastic leukemia presented after 30 months of treatment a bilateral hypopyon. Anterior chamber paracentesis with cytological survey revealed leukemic cells and confirmed the ocular relapse. The treatment included the association of topical corticosteroids, chemotherapy and radiotherapy. This child died unfortunately 16 months later following a medullar relapse. We remind the different clinical aspects of leukemic invasion of the anterior segment and the therapeutic methods for this relapse.

[Tuberculosis of the thyroid. Diagnosis and treatment]. / La tuberculose de la thyroïde. Diagnostic et traitement.

Thyroid tuberculosis is rare. We present the case of a 25 year-old woman with tuberculosis of the thyroid. Although seldom observed, tuberculosis should be considered in the differential diagnosis of nodular lesions of the thyroid. Diagnosis is made by histological examination and demonstration of the tubercle bacilli from biopsy or aspiration specimen. The efficacy of fine-needle aspiration cytology in diagnosis of tuberculosis of the thyroid is proved. Administration of antituberculous drugs is considered as the treatment of choice. Abscess drainage is sufficient. In rare cases surgery is necessary. The prognosis is good.

[Cutaneous leishmaniasis of the lid]. / Localisation palpébrale des leishmanioses cutanées.

We report 3 cases of patients presenting with lid cutaneous leishmaniasis in which clinical presentation was a true diagnostic problem. The patients were 3 women aged 46, 36, and 60 years. The first patient presented with an ulceration of the superior eyelid that had been treated as chalazion. The second patient had a chronic lesion of the eyelid, present for 1 year, fitting the criteria for tuberculosis, syphilis, or sarcoidosis. The last patient had an erosive lesion of the internal canthus, which was suspicious of basal cell carcinoma. The presence of other cutaneous lesions and the chronic progression led to a biopsy for anatomopathology and parasitology analysis. These analyses confirmed the diagnosis of cutaneous leishmaniasis. The patients were then treated with antimony derivatives. Progression of the disease was marked by the appearance of a pigmented nonretractile scar. The different clinical characteristics, epidemiology, and treatment of this affection are reviewed.

[Wolfram syndrome. Three case reports]. / Syndrome de Wolfram. A propos de 3 cas.

Wolfram syndrome is a rare disorder defined by the occurrence of diabete mellitus, diabete insipidus, optic atrophy and deafness; DIDMOAD is a commonly accepted acronym. We report 3 further cases: two girls and one boy. Their age ranged from 12 to 17 years. The diagnosis was based on the presence of juvenile diabete mellitus, bilateral optic atrophy, urologic signs, with urinary tract dilation; and deafness in two cases. The ophthalmic signs of Wolfram syndrome are progressive decrease in visual acuity, constriction of the peripheral visual field with or without central scotoma, color vision disturbances and bilateral optic disc atrophy. Diabetic retinopathy is a rare complication. The other clinical features are discussed so as to differentiate between Wolfram syndrome and other optic atrophies associated with diabete mellitus. We discuss of the pathogenic hypothesis including the mitochondrial dysfunction.

[Tumor of the lacrimal sac. Dangers of simple excision biopsy]. / Tumeur du sac lacrymal. Dangers de la biopsie exérèse simple.

Lacrimal sac tumors are relatively uncommon tumors which recur readily and have poor prognosis if adequate care is not provided early. We report here a case of lacrimal sac tumor in a 56-year-old patient with bilateral pseudophakia and an atypical chronic dacryocystisis aspect of the left eye. After clinical and paraclinical investigations, the patient underwent surgical excision of the tumor followed by external radiotherapy. During follow-up, the patient developed a local recurrence which required surgical removal and radiotherapy. Despite early and adapted management, the prognosis of this tumor remains poor.

[Tuberculous uveitis]. / Uvéite tuberculeuse.

Ocular tuberculosis is relatively rare; however, the most common ocular lesion during ocular tuberculosis is uveitis. The recrudescence of this disease is probably caused by human immunodeficiency virus or long-term corticotherapy. The authors report a of chronic unilateral tuberculosis-related uveitis complicated by panophthalmia with subconjunctival abcesess and scleral fistula. A histopathological study revealed a granulomatous inflammation with caseous material. An extensive work-up revealed no extraocular lesion. A systemic bactericidal treatement associated with topical steroids decreased the local inflammation with phthisis bulbi, and with no spread of the infection. The authors discuss the etiopathogenic, clinical, and therapeutic aspects of tuberculosis-related uveitis.

[Orbital lymphangioma. A case report]. / Lymphangiome orbitaire. A propos d'un cas.

The diffuse form of orbital lymphangioma is well known for its difficult surgical treatment. A diffuse orbital lymphangioma was diagnosed in a 6-year-old girl, revealed by unilateral recurrent proptosis. The imaging procedure discovered a mass presumed to be vascular in nature. The initial incompleted surgical removal was followed by 3 recurrences motivating 3 reoperations. Finally, the tumor was removed incompletely, with an acceptable reduction of the proptosis. The pathologic analyses indicated lymphangioma. Some vascular orbital tumors such as lymphangioma, may be very difficult to manage because of local spreading and frequent recurrence.

[Melanoma of the iris and pregnancy]. / Mélanome de l'iris et grossesse.

Iris melanoma is a malignant melanocytic tumor, making up 1.2%-6.6% of uveal malignant melanomas. The growth of choroidal melanoma during pregnancy is described and a hormonal influence is suggested. We present a case of iris melanoma occurring during pregnancy. A 32-year-old woman, in the 28(th) week of her fifth pregnancy, was referred to the ophthalmologist with a 2-month history of blurred vision and pain in her left eye. The visual symptoms became progressively worse with decreasing vision. Her visual acuity was 20/70 in her left eye, with a temporal, pigmented, prominent iris mass extending into the trabecular meshwork and the corneal endothelium. Elsewhere other pigmented localizations were found in the iris and in the trabecular meshwork. There was secondary glaucoma with intraocular pressure of 36mmHg and C/D=0.9. The right eye was normal. A general physical examination found no pigmented lesions. Chest radiography and hepatic ultrasonography revealed no metastasis. The diagnosis of iris malignant melanoma was made from the diffuse involvement extending into the trabecular meshwork and the advanced secondary glaucoma; an enucleation was performed and histopathological examination confirmed the diagnosis of iris melanoma with involvement of the trabecular meshwork. The patient had no further problems (follow-up of 11 months). From this case report, we discuss the differential diagnosis of iris melanoma and the hormonal influence on its growth.

[Medulloepithelioma of the ciliary body. A case report]. / Le médullo-épithéliome du corps ciliaire. A propos d'un cas.

Medulloepithelioma of the ciliary body is an uncommon intraocular tumor occurring during the first year of life. Malignant degeneration may occur. We report the case of a 4-year-old child who presented medulloepithelioma of the left eye disclosed by oesotropia at 2 years of age. Clinically, there was oesotropia, positive light perception and cataract with vascular membrane spreading to the nasal side of the irido-corneal angle. CT scan and ultrasound B revealed a ciliary body tumor involving the sclera and orbital fat. After exenteration, the pathology study reported malignant medulloepithelioma of the ciliary body with scleral extension. No local recurrence or metastasis has been observed at 8 months follow-up. We discuss the clinical, radiological and therapeutic features of this uncommon tumor.