Gyrate atrophy of the choroid and retina with hyper-ornithinemia responsive to vitamin B6: a case report

<p>Abstract</p> <p>Background</p> <p>Gyrate atrophy of the retina and choroid is a rare autosomal recessive inherited disease, characterized by progressive chorioretinal atrophy that results in progressive deterioration of peripheral and night vision and leading to blindness.</p> <p>Case presentation</p> <p>This report presents a case of a 28-year-old man consulting for a progressive fall of visual acuity with hemeralopia. Eye fundoscopy showed regions of confluent rounded chorioretinal atrophy. The visual field and retinal angiography were altered. A high level of plasma ornithine (629 nmol/mL) was detected and a diagnosis of gyrate atrophy of the retina and choroid was made. The patient was treated with high dose Pyridoxine supplement (300 mg/d for 6 months) and the ornithine level of his serum was successfully reduced.</p> <p>Conclusion</p> <p>The exact mechanism of chorioretinal atrophy in hyper-ornithinemia is not known and a small percentage of the affected people respond to Vitamin B6 supplementation.</p

Bilateral Medical Rectus Advancement versus Bilateral Lateral Rectus Recession for Consecutive Exotropia

&lt;!--[if gte mso 9]&gt;&lt;xml&gt; Normal 0 false false false MicrosoftInternetExplorer4 &lt;/xml&gt;&lt;![endif]--&gt;&lt;!--[if gte mso 9]&gt;&lt;xml&gt; &lt;/xml&gt;&lt;![endif]--&gt; &lt;!--[if gte mso 10]&gt; &lt;style&gt;&#13; /* Style Definitions */&#13; table.MsoNormalTable&#13; {mso-style-name:"Table Normal";&#13; mso-tstyle-rowband-size:0;&#13; mso-tstyle-colband-size:0;&#13; mso-style-noshow:yes;&#13; mso-style-parent:"";&#13; mso-padding-alt:0cm 5.4pt 0cm 5.4pt;&#13; mso-para-margin:0cm;&#13; mso-para-margin-bottom:.0001pt;&#13; mso-pagination:widow-orphan;&#13; font-size:10.0pt;&#13; font-family:"Times New Roman";&#13; mso-ansi-language:#0400;&#13; mso-fareast-language:#0400;&#13; mso-bidi-language:#0400;}&#13; &lt;/style&gt; &lt;![endif]--&gt; &lt;p&gt;PURPOSE: To compare bilateral medial rectus advancement (BMRA) and bilateral lateral rectus recession (BLRR) for the treatment of consecutive exotropia. METHOD: This randomized clinical trial was performed on 14 patients with consecutive exotropia. Inclusion criteria were history of bilateral medial rectus recession, exotropia of 20 PD or more with far-near discrepancy &lt; 10 PD. Exclusion criteria consisted of more than once medial rectus recession, restricted adduction, history of operation on the lateral rectus, positive forced duction test of the lateral rectus, concomitant neurologic disorders and follow-up less than 6 months' duration. RESULTS: Seven patients underwent BMRA and 7 patients underwent BLRR. Mean age was 11.4±6.9 (range 5 to 21) years in the BMRA group and 13.7±7.1 (range 5-22) years in the BLRR group (P=0.44). Two patients in the BMRA group and 3 subjects in the BLRR group were amblyopic. Mean preoperative exotropia was 27.8±6.3 PD and 39.2±14.8 PD (P=0.09) which was reduced to 4.2±2.3 PD and 3.4±2.2 PD (P=0.94) in the BMRA and BLRR groups respectively. Successful alignment was achieved in 71.4% and 85.7% of cases in the BMRA and BLRR groups respectively (P=0.94). All amblyopic patients achieved successful alignment postoperatively. CONCLUSION: Bilateral medial rectus advancement and bilateral lateral rectus recession are comparable in efficacy for treatment of consecutive exotropia.   &lt;/p&gt; &lt;!--[if gte mso 9]&gt;&lt;xml&gt; &lt;w:WordDocument&gt; &lt;w:View&gt;Normal&lt;/w:View&gt; &lt;w:Zoom&gt;0&lt;/w:Zoom&gt; &lt;w:PunctuationKerning /&gt; &lt;w:ValidateAgainstSchemas /&gt; &lt;w:SaveIfXMLInvalid&gt;false&lt;/w:SaveIfXMLInvalid&gt; &lt;w:IgnoreMixedContent&gt;false&lt;/w:IgnoreMixedContent&gt; &lt;w:AlwaysShowPlaceholderText&gt;false&lt;/w:AlwaysShowPlaceholderText&gt; &lt;w:Compatibility&gt; &lt;w:BreakWrappedTables /&gt; &lt;w:SnapToGridInCell /&gt; &lt;w:WrapTextWithPunct /&gt; &lt;w:UseAsianBreakRules /&gt; &lt;w:DontGrowAutofit /&gt; &lt;/w:Compatibility&gt; &lt;/w:WordDocument&gt; &lt;/xml&gt;&lt;![endif]--&gt;&lt;!--[if gte mso 9]&gt;&lt;xml&gt; &lt;w:LatentStyles DefLockedState="false" LatentStyleCount="156"&gt; &lt;/w:LatentStyles&gt; &lt;/xml&gt;&lt;![endif]--&gt; &lt;!-- /* Font Definitions */ @font-face {font-family:Palatino; mso-font-alt:"Book Antiqua"; mso-font-charset:0; mso-generic-font-family:roman; mso-font-pitch:variable; mso-font-signature:7 0 0 0 147 0;} @font-face {font-family:Calibri; mso-font-alt:"Century Gothic"; mso-font-charset:0; mso-generic-font-family:swiss; mso-font-pitch:variable; mso-font-signature:-1610611985 1073750139 0 0 159 0;} /* Style Definitions */ p.MsoNormal, li.MsoNormal, div.MsoNormal {mso-style-parent:""; margin-top:0cm; margin-right:0cm; margin-bottom:10.0pt; margin-left:0cm; text-align:right; line-height:115%; mso-pagination:widow-orphan; direction:rtl; unicode-bidi:embed; font-size:11.0pt; font-family:Calibri; mso-fareast-font-family:"Times New Roman"; mso-bidi-font-family:Arial; mso-bidi-language:FA;} @page Section1 {size:612.0pt 792.0pt; margin:72.0pt 90.0pt 72.0pt 90.0pt; mso-header-margin:36.0pt; mso-footer-margin:36.0pt; mso-paper-source:0;} div.Section1 {page:Section1;} --&gt; &lt;!--[if gte mso 10]&gt; &lt;style&gt;&#13; /* Style Definitions */&#13; table.MsoNormalTable&#13; {mso-style-name:"Table Normal";&#13; mso-tstyle-rowband-size:0;&#13; mso-tstyle-colband-size:0;&#13; mso-style-noshow:yes;&#13; mso-style-parent:"";&#13; mso-padding-alt:0cm 5.4pt 0cm 5.4pt;&#13; mso-para-margin:0cm;&#13; mso-para-margin-bottom:.0001pt;&#13; mso-pagination:widow-orphan;&#13; font-size:10.0pt;&#13; font-family:"Times New Roman";&#13; mso-ansi-language:#0400;&#13; mso-fareast-language:#0400;&#13; mso-bidi-language:#0400;}&#13; &lt;/style&gt; &lt;![endif]--&gt