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Journal Francais D Ophtalmologie, 2006
Fraser syndrome is a rare autosomal recessive disorder; the most consistent features are cryptoph... more Fraser syndrome is a rare autosomal recessive disorder; the most consistent features are cryptophthalmos, syndactyly of fingers and toes, laryngeal stenosis, and urogenital abnormalities. We report a newborn case at day 1 of life who had multiple abnormalities, born from a consanguineous marriage. Clinically, the newborn had an ankyloblepharon on the left side, a cryptophthalmos on the right side, a syndactyly, anorectal abnormalities with ambiguous genitalia, laryngeal stenosis, and ear malformations. TDM of the cranium and orbits and the transfontanel ultrasound were normal. The abdominal ultrasound showed renal abnormalities. Right eye surgery showed a reduced cornea to an opaque thin plate clinging to the iris without an anterior chamber and a nonindividualized eyeball. The authors discuss the morphological abnormalities, the clinical and paraclinical aspects of this syndrome, its multispecialized clinical management, and the importance of prenatal diagnosis.
Journal Francais D Ophtalmologie, 2005
Introduction : Le syndrome d'Alport est une affection héréditaire caractérisée par l'existence d'... more Introduction : Le syndrome d'Alport est une affection héréditaire caractérisée par l'existence d'une néphropathie hématurique progressive. Il s'associe souvent à des anomalies oculaires. Notre travail a pour but de déterminer les particularités cliniques, histologiques et génétiques et d'analyser les atteintes ophtalmologiques de ce syndrome. Matériels et Méthodes : L'étude a porté sur 5 familles du sud Tunisien. Tous les sujets ont bénéficié d'un examen des urines par bandelettes réactives à la recherche d'une hématurie microscopique que nous avons complété par une exploration de la fonction rénale, une ponction-biopsie rénale et un examen ORL avec audiogramme. Un examen ophtalmologique complet a été pratiqué avec une angiographie rétinienne en cas de lésions détectées au niveau du fond d'oeil. Discussion : Les anomalies oculaires varient de 11 à 45 %. Le lenticône antérieur et les taches maculaires sont caractéristiques de ce syndrome. Sur le plan génétique, le mode de transmission le plus fréquent est dominant lié à l'X dans 85 % des cas. La forme autosomique récessive est retrouvée dans 15 % des cas et la forme autosomique dominante est exceptionnelle. Conclusion : Le syndrome d'Alport associe une néphropathie hématurique progressive évoluant vers l'insuffisance rénale terminale, une surdité de perception bilatérale et des lésions oculaires, le tout évoluant dans un contexte familial.
Journal Francais D Ophtalmologie, 2005
Journal Francais D Ophtalmologie, 2005
Journal Francais D Ophtalmologie, 2005
Journal Francais D Ophtalmologie, 2005
1S290 multiples remaniements du gène SOX 2 , fréquemment impliqué lors d'anomalies du développeme... more 1S290 multiples remaniements du gène SOX 2 , fréquemment impliqué lors d'anomalies du développement oculaire. Discussion : La gestion d'une malformation grave embryologique ne peut se faire qu'au sein d'une équipe pluridisciplinaire, après avis de tous les protagonistes, permettant d'affiner le diagnostic, et permettant ainsi à la famille de prendre une décision en toute connaissance de cause. C'est dans cette optique que la famille a consulté au sein de quatre équipes médicales, et qu'une recherche génétique a pu être menée à bien rapidement. Conclusion : L'anophtalmie bilatérale est quasiment toujours associée à des anomalies neurologiques qui assombrissent le pronostic. L'équipe médicale doit donner les moyens à la famille de réaliser leur futur travail de deuil s'ils prennent la décision d'une interruption de grossesse, ou de se préparer à accueillir un enfant avec des handicaps, s'ils décident de garder cet enfant.
Journal Francais D Ophtalmologie, 2005
Journal Francais D Ophtalmologie, 2005
Journal Francais D Ophtalmologie, 2005
Avulsion of the optic nerve is a rare and serious injury. The authors report two cases of optic n... more Avulsion of the optic nerve is a rare and serious injury. The authors report two cases of optic nerve avulsion. The first one concerns a 5-year-old boy who presented ocular trauma after falling on the handlebars of a bicycle. His visual acuity was light perception in the right eye, and his right pupil was unresponsive to light. The anterior segment was normal. The ophthalmoscopic examination showed a total separation of the optic nerve head from the sclera with peripapillary hemorrhage. The second case concerns a 30-year-old man who was hit in the right eye with a stick. On admission, he had no light perception in the right eye, a right afferent pupil defect, a small laceration of the right lower eye lid and no abnormalities on the anterior segment. The fundus examination showed a mild vitreous hemorrhage. Ocular ultrasonography showed vitreous hemorrhage coming directly from the optic nerve head in a mushroom pattern. A CT scan of the orbit revealed a thickened optic nerve. Color Doppler ultrasonography documented slowing of blood flow in the central retinal artery. The two patients received 1 mg/kg/day of prednisone for 2 weeks. No improvement was noted. Optic nerve avulsion is often caused by sudden and forceful rotation of the eye with tearing of the optic nerve as it exits the globe. The nerve can be partially or totally avulsed. The prognosis is usually poor.
Archives De Pediatrie, 1996
MJ. PENNIELLO -J. GUARNIERt -F. FREYMUTII -A. DENIS -P. COUTHEOUX • B. GUILLOIS ,%~wke de p~dlatd... more MJ. PENNIELLO -J. GUARNIERt -F. FREYMUTII -A. DENIS -P. COUTHEOUX • B. GUILLOIS ,%~wke de p~dlatde B -CHU Cl~aene~u -Avenue G. Ck~men¢¢au -14033 CAEN C~dex.
Journal Francais D Ophtalmologie, 2005
Journal Francais D Ophtalmologie, 2005
Journal Francais D Ophtalmologie, 2005
Clinical Genetics, 2002
Primary congenital glaucoma (PCG) is a heterogeneous autosomal recessive disorder caused by unkno... more Primary congenital glaucoma (PCG) is a heterogeneous autosomal recessive disorder caused by unknown developmental defect(s) of the anterior chamber of the eye. A member of the cytochrome P450 gene family, CYP1B1, was found to be mutated in PCG patients in different populations, albeit to a variable extent. In this study, CYP1B1 mutations were searched for in 32 unrelated PCG patients from Morocco. Two mutations were detected in 11 (34%) patients. One, 4339delG, is novel and causes a frameshift at residue 179. The other, G61E, was previously found in patients from Turkey and Saudi Arabia. Seven patients were homozygous for 4339delG and two other patients for G61E, whereas the two remaining patients were compound heterozygotes. The close association of 4339delG with a rare allele of D2S177, a microsatellite marker located 270 kb upstream of CYP1B1, strongly suggested a founder effect for 4339delG. The occurrence of this mutation was tentatively dated at between 900 and 1700 years ago. Typing 4339delG and G61E mutations should help to prevent blindness resulting from a delayed diagnosis of PCG in Morocco.
Journal Francais D Ophtalmologie, 2004
Asteroid hyalosis is a rare degenerative condition of the vitreous. No causal relationship betwee... more Asteroid hyalosis is a rare degenerative condition of the vitreous. No causal relationship between retinitis pigmentosa and asteroid hyalosis has been established. We report a case of a 65-year-old male admitted for progressive hemeralopia. Visual acuities were 2/10 e in the right eye and 4/10 e in the left eye. Ophthalmic examination revealed a large number of asteroid and refringent bodies, a typical retinitis pigmentosa with a cystoid macular oedema confirmed by fluorescein angiography. Electrodiagnostic testing revealed an altered electroretinogram. Visual fields showed a typical generalized constriction. We discuss the clinical aspects and physiopathogenic mechanisms of this rare association through this case and other cases found in the literature.
Journal Francais D Ophtalmologie, 2005
Discussion: HRTII examination with cornea module revealed numerous 20 to 26 μm diameter high-cont... more Discussion: HRTII examination with cornea module revealed numerous 20 to 26 μm diameter high-contrast round particles within the corneal epithelium and anterior stroma, resembling acanthamoeba cysts. Morphologic details of these cyst-like particles suggested the presence of a double wall. Stellate cells as well as ovoid irregular objects, possibly inflammatory cells, trophozoites, altered cysts, or activated keratocytes, were also present in the area of stromal infiltrates. An irregularly thickened stromal nerve was seen, consistent with radial keratoneuritis. Confirmation of AK was obtained by cytological examination of corneal smears which revealed the presence of numerous acanthamoeba cysts. Conclusion: HRTII cornea module provides non-invasive, high-contrast, in vivo images of the cornea. It can be helpful in the diagnosis of AK by identifying acanthamoeba cyst-like structures in the cornea. This technique has also potential uses in monitoring the efficiency of anti-infective treatment.
Journal Francais D Ophtalmologie, 2005
Journal Francais D Ophtalmologie, 2005
Journal Francais D Ophtalmologie, 2005
Journal Francais D Ophtalmologie, 2006
Fraser syndrome is a rare autosomal recessive disorder; the most consistent features are cryptoph... more Fraser syndrome is a rare autosomal recessive disorder; the most consistent features are cryptophthalmos, syndactyly of fingers and toes, laryngeal stenosis, and urogenital abnormalities. We report a newborn case at day 1 of life who had multiple abnormalities, born from a consanguineous marriage. Clinically, the newborn had an ankyloblepharon on the left side, a cryptophthalmos on the right side, a syndactyly, anorectal abnormalities with ambiguous genitalia, laryngeal stenosis, and ear malformations. TDM of the cranium and orbits and the transfontanel ultrasound were normal. The abdominal ultrasound showed renal abnormalities. Right eye surgery showed a reduced cornea to an opaque thin plate clinging to the iris without an anterior chamber and a nonindividualized eyeball. The authors discuss the morphological abnormalities, the clinical and paraclinical aspects of this syndrome, its multispecialized clinical management, and the importance of prenatal diagnosis.
Journal Francais D Ophtalmologie, 2005
Introduction : Le syndrome d'Alport est une affection héréditaire caractérisée par l'existence d'... more Introduction : Le syndrome d'Alport est une affection héréditaire caractérisée par l'existence d'une néphropathie hématurique progressive. Il s'associe souvent à des anomalies oculaires. Notre travail a pour but de déterminer les particularités cliniques, histologiques et génétiques et d'analyser les atteintes ophtalmologiques de ce syndrome. Matériels et Méthodes : L'étude a porté sur 5 familles du sud Tunisien. Tous les sujets ont bénéficié d'un examen des urines par bandelettes réactives à la recherche d'une hématurie microscopique que nous avons complété par une exploration de la fonction rénale, une ponction-biopsie rénale et un examen ORL avec audiogramme. Un examen ophtalmologique complet a été pratiqué avec une angiographie rétinienne en cas de lésions détectées au niveau du fond d'oeil. Discussion : Les anomalies oculaires varient de 11 à 45 %. Le lenticône antérieur et les taches maculaires sont caractéristiques de ce syndrome. Sur le plan génétique, le mode de transmission le plus fréquent est dominant lié à l'X dans 85 % des cas. La forme autosomique récessive est retrouvée dans 15 % des cas et la forme autosomique dominante est exceptionnelle. Conclusion : Le syndrome d'Alport associe une néphropathie hématurique progressive évoluant vers l'insuffisance rénale terminale, une surdité de perception bilatérale et des lésions oculaires, le tout évoluant dans un contexte familial.
Journal Francais D Ophtalmologie, 2005
Journal Francais D Ophtalmologie, 2005
Journal Francais D Ophtalmologie, 2005
Journal Francais D Ophtalmologie, 2005
1S290 multiples remaniements du gène SOX 2 , fréquemment impliqué lors d'anomalies du développeme... more 1S290 multiples remaniements du gène SOX 2 , fréquemment impliqué lors d'anomalies du développement oculaire. Discussion : La gestion d'une malformation grave embryologique ne peut se faire qu'au sein d'une équipe pluridisciplinaire, après avis de tous les protagonistes, permettant d'affiner le diagnostic, et permettant ainsi à la famille de prendre une décision en toute connaissance de cause. C'est dans cette optique que la famille a consulté au sein de quatre équipes médicales, et qu'une recherche génétique a pu être menée à bien rapidement. Conclusion : L'anophtalmie bilatérale est quasiment toujours associée à des anomalies neurologiques qui assombrissent le pronostic. L'équipe médicale doit donner les moyens à la famille de réaliser leur futur travail de deuil s'ils prennent la décision d'une interruption de grossesse, ou de se préparer à accueillir un enfant avec des handicaps, s'ils décident de garder cet enfant.
Journal Francais D Ophtalmologie, 2005
Journal Francais D Ophtalmologie, 2005
Journal Francais D Ophtalmologie, 2005
Avulsion of the optic nerve is a rare and serious injury. The authors report two cases of optic n... more Avulsion of the optic nerve is a rare and serious injury. The authors report two cases of optic nerve avulsion. The first one concerns a 5-year-old boy who presented ocular trauma after falling on the handlebars of a bicycle. His visual acuity was light perception in the right eye, and his right pupil was unresponsive to light. The anterior segment was normal. The ophthalmoscopic examination showed a total separation of the optic nerve head from the sclera with peripapillary hemorrhage. The second case concerns a 30-year-old man who was hit in the right eye with a stick. On admission, he had no light perception in the right eye, a right afferent pupil defect, a small laceration of the right lower eye lid and no abnormalities on the anterior segment. The fundus examination showed a mild vitreous hemorrhage. Ocular ultrasonography showed vitreous hemorrhage coming directly from the optic nerve head in a mushroom pattern. A CT scan of the orbit revealed a thickened optic nerve. Color Doppler ultrasonography documented slowing of blood flow in the central retinal artery. The two patients received 1 mg/kg/day of prednisone for 2 weeks. No improvement was noted. Optic nerve avulsion is often caused by sudden and forceful rotation of the eye with tearing of the optic nerve as it exits the globe. The nerve can be partially or totally avulsed. The prognosis is usually poor.
Archives De Pediatrie, 1996
MJ. PENNIELLO -J. GUARNIERt -F. FREYMUTII -A. DENIS -P. COUTHEOUX • B. GUILLOIS ,%~wke de p~dlatd... more MJ. PENNIELLO -J. GUARNIERt -F. FREYMUTII -A. DENIS -P. COUTHEOUX • B. GUILLOIS ,%~wke de p~dlatde B -CHU Cl~aene~u -Avenue G. Ck~men¢¢au -14033 CAEN C~dex.
Journal Francais D Ophtalmologie, 2005
Journal Francais D Ophtalmologie, 2005
Journal Francais D Ophtalmologie, 2005
Clinical Genetics, 2002
Primary congenital glaucoma (PCG) is a heterogeneous autosomal recessive disorder caused by unkno... more Primary congenital glaucoma (PCG) is a heterogeneous autosomal recessive disorder caused by unknown developmental defect(s) of the anterior chamber of the eye. A member of the cytochrome P450 gene family, CYP1B1, was found to be mutated in PCG patients in different populations, albeit to a variable extent. In this study, CYP1B1 mutations were searched for in 32 unrelated PCG patients from Morocco. Two mutations were detected in 11 (34%) patients. One, 4339delG, is novel and causes a frameshift at residue 179. The other, G61E, was previously found in patients from Turkey and Saudi Arabia. Seven patients were homozygous for 4339delG and two other patients for G61E, whereas the two remaining patients were compound heterozygotes. The close association of 4339delG with a rare allele of D2S177, a microsatellite marker located 270 kb upstream of CYP1B1, strongly suggested a founder effect for 4339delG. The occurrence of this mutation was tentatively dated at between 900 and 1700 years ago. Typing 4339delG and G61E mutations should help to prevent blindness resulting from a delayed diagnosis of PCG in Morocco.
Journal Francais D Ophtalmologie, 2004
Asteroid hyalosis is a rare degenerative condition of the vitreous. No causal relationship betwee... more Asteroid hyalosis is a rare degenerative condition of the vitreous. No causal relationship between retinitis pigmentosa and asteroid hyalosis has been established. We report a case of a 65-year-old male admitted for progressive hemeralopia. Visual acuities were 2/10 e in the right eye and 4/10 e in the left eye. Ophthalmic examination revealed a large number of asteroid and refringent bodies, a typical retinitis pigmentosa with a cystoid macular oedema confirmed by fluorescein angiography. Electrodiagnostic testing revealed an altered electroretinogram. Visual fields showed a typical generalized constriction. We discuss the clinical aspects and physiopathogenic mechanisms of this rare association through this case and other cases found in the literature.
Journal Francais D Ophtalmologie, 2005
Discussion: HRTII examination with cornea module revealed numerous 20 to 26 μm diameter high-cont... more Discussion: HRTII examination with cornea module revealed numerous 20 to 26 μm diameter high-contrast round particles within the corneal epithelium and anterior stroma, resembling acanthamoeba cysts. Morphologic details of these cyst-like particles suggested the presence of a double wall. Stellate cells as well as ovoid irregular objects, possibly inflammatory cells, trophozoites, altered cysts, or activated keratocytes, were also present in the area of stromal infiltrates. An irregularly thickened stromal nerve was seen, consistent with radial keratoneuritis. Confirmation of AK was obtained by cytological examination of corneal smears which revealed the presence of numerous acanthamoeba cysts. Conclusion: HRTII cornea module provides non-invasive, high-contrast, in vivo images of the cornea. It can be helpful in the diagnosis of AK by identifying acanthamoeba cyst-like structures in the cornea. This technique has also potential uses in monitoring the efficiency of anti-infective treatment.
Journal Francais D Ophtalmologie, 2005
Journal Francais D Ophtalmologie, 2005
Journal Francais D Ophtalmologie, 2005