Background: Recent reports postulate that the concomitant vertical deviation f ound in congenital superior oblique palsy is due to mechanical abnormalities rat her than a congenitally paretic muscle, and is overcome i...Background: Recent reports postulate that the concomitant vertical deviation f ound in congenital superior oblique palsy is due to mechanical abnormalities rat her than a congenitally paretic muscle, and is overcome in most patients by fusi on. On the basis of the clinical characteristics alone a primary paresis is inde ed unlikely. Although intraoperatively a different elasticity of the superior ob lique tendon exists in congenital versus acquired cases of superior oblique pals y, preoperatively performed MR imaging shows that the clinical findings in conge nital superior oblique muscle malfunction could nevertheless be of paretic origi n. Materials andMethods: Seventeen consecutive patients (males: n=13; females: n =4) were examined. The vertical deviation in adduction was concomitant in vertic al versions, the excyclotropia was small and concomitant in all directions of ga ze and was less than 10°even after diagnostic occlusion. All patients showed a positive Bielschowsky head tilt phenomenon and large fusional ability. We perfor med preoperative MR imaging of both orbits in high resolution 3 mm sections in c oronal and axial orientationswith and without contrast enhancement. Results: In sixteen patients we found a significant reduction in muscle volume or even total aplasia of the superior oblique muscle of the affected side in comparison to th e sound muscle on the other side. In contrast, two patients had a full blown cli nical picture of a congenital superior oblique palsy but showed symmetrical musc le volumes on both sides in all coronal sections. Conclusions: Hypoplasia or apl asia of the superior oblique muscle on magnetic resonance imaging provides evide nce for a primary paretic cause for the vertical squint found with congenital su perior oblique dysfunction. It is not clear, however, whether this is caused by a primary hypoplasia or is of neurogenic origin. Our data together with the cons istent difference in tendon morphology of the congenital and acquired forms of s uperior oblique palsy seem to exclude a purely neurogenic cause for the affectio n.展开更多
Case report:Shortly after birth,a massive enlargement of the right eye was observed in an otherwise healthy male child.The cornea of the affected eye was vascularized and completely cloudy without a sharp border betwe...Case report:Shortly after birth,a massive enlargement of the right eye was observed in an otherwise healthy male child.The cornea of the affected eye was vascularized and completely cloudy without a sharp border between cornea and sclera.The diagnosis of a congenital glaucoma was made but an operation was not undertaken because of the difficult anatomical situation and the lost function.When the child was almost 3 years old enucleation was performed to prevent complications due to corneal exposure,and to improve the cosmetic aspect.Results:The morphological investigations of the enucleated eye disclosed findings typical of what is called in the literature “ congenital anterior staphyloma” or “ congenital corneal staphyloma”,namely a massively staphylomatous cornea with superficial neovascularization,destruction of Bowman’ s layer,and absence of Descemet’ s layer as well as corneal endothelium.Angle structures were completely absent,and the corneal back-side was lined by a pigment epithelial layer and focally by an additional inner layer of non-pigmented epithelium.There was no anterior chamber.The lens was markedly diminished in size(microphakia)and partly embedded in the corneal stroma.Pars muscularis and pars ciliaris of the ciliary body were separated.Elongated,thin ciliary processes were extended towards the small lens while the pars muscularis was fully covered by the retina.Conclusions:This rare,complex malformation syndrome which can be easily distinguished from primary congenital glaucoma should not be reduced conceptually to the corneal staphyloma because this staphyloma constitutes only a part of the whole.Taking the leading morphological aberrations into consideration we would rather propose the new term “ corneal staphyloma-anterior chamber agenesia-microphakia syndrome”(CSAMS).We hypothesize that CSAMS may be due to a pathological fusion of the early anterior optic cup.As the posterior eye segment is often normal in CSAMS,a staphyloma excision along with a sclero-keratoplasty might be an alternative therapeutic option to avoid enucleation and restore ambulatory vision.展开更多
文摘Background: Recent reports postulate that the concomitant vertical deviation f ound in congenital superior oblique palsy is due to mechanical abnormalities rat her than a congenitally paretic muscle, and is overcome in most patients by fusi on. On the basis of the clinical characteristics alone a primary paresis is inde ed unlikely. Although intraoperatively a different elasticity of the superior ob lique tendon exists in congenital versus acquired cases of superior oblique pals y, preoperatively performed MR imaging shows that the clinical findings in conge nital superior oblique muscle malfunction could nevertheless be of paretic origi n. Materials andMethods: Seventeen consecutive patients (males: n=13; females: n =4) were examined. The vertical deviation in adduction was concomitant in vertic al versions, the excyclotropia was small and concomitant in all directions of ga ze and was less than 10°even after diagnostic occlusion. All patients showed a positive Bielschowsky head tilt phenomenon and large fusional ability. We perfor med preoperative MR imaging of both orbits in high resolution 3 mm sections in c oronal and axial orientationswith and without contrast enhancement. Results: In sixteen patients we found a significant reduction in muscle volume or even total aplasia of the superior oblique muscle of the affected side in comparison to th e sound muscle on the other side. In contrast, two patients had a full blown cli nical picture of a congenital superior oblique palsy but showed symmetrical musc le volumes on both sides in all coronal sections. Conclusions: Hypoplasia or apl asia of the superior oblique muscle on magnetic resonance imaging provides evide nce for a primary paretic cause for the vertical squint found with congenital su perior oblique dysfunction. It is not clear, however, whether this is caused by a primary hypoplasia or is of neurogenic origin. Our data together with the cons istent difference in tendon morphology of the congenital and acquired forms of s uperior oblique palsy seem to exclude a purely neurogenic cause for the affectio n.
文摘Case report:Shortly after birth,a massive enlargement of the right eye was observed in an otherwise healthy male child.The cornea of the affected eye was vascularized and completely cloudy without a sharp border between cornea and sclera.The diagnosis of a congenital glaucoma was made but an operation was not undertaken because of the difficult anatomical situation and the lost function.When the child was almost 3 years old enucleation was performed to prevent complications due to corneal exposure,and to improve the cosmetic aspect.Results:The morphological investigations of the enucleated eye disclosed findings typical of what is called in the literature “ congenital anterior staphyloma” or “ congenital corneal staphyloma”,namely a massively staphylomatous cornea with superficial neovascularization,destruction of Bowman’ s layer,and absence of Descemet’ s layer as well as corneal endothelium.Angle structures were completely absent,and the corneal back-side was lined by a pigment epithelial layer and focally by an additional inner layer of non-pigmented epithelium.There was no anterior chamber.The lens was markedly diminished in size(microphakia)and partly embedded in the corneal stroma.Pars muscularis and pars ciliaris of the ciliary body were separated.Elongated,thin ciliary processes were extended towards the small lens while the pars muscularis was fully covered by the retina.Conclusions:This rare,complex malformation syndrome which can be easily distinguished from primary congenital glaucoma should not be reduced conceptually to the corneal staphyloma because this staphyloma constitutes only a part of the whole.Taking the leading morphological aberrations into consideration we would rather propose the new term “ corneal staphyloma-anterior chamber agenesia-microphakia syndrome”(CSAMS).We hypothesize that CSAMS may be due to a pathological fusion of the early anterior optic cup.As the posterior eye segment is often normal in CSAMS,a staphyloma excision along with a sclero-keratoplasty might be an alternative therapeutic option to avoid enucleation and restore ambulatory vision.
