Lateral rectus muscle disinsertion and reattachment to the lateral orbital wall in exotropic Duane syndrome: a case report

<p>Abstract</p> <p>Introduction</p> <p>The surgical correction of anomalous movement such as upshoot in Duane syndrome is challenging. Lateral rectus muscle disinsertion and reattachment to the lateral orbital wall is a new approach used to minimize or eliminate the effects of co-contraction including globe retraction, palpebral fissure narrowing and anomalous vertical movement.</p> <p>Case presentation</p> <p>We report a case of a 7-year-old boy who underwent this procedure for severe upshoot, globe retraction and exotropia in the left eye due to Duane syndrome. The patient achieved satisfactory ocular alignment following surgery. Upshoot and globe retraction were substantially improved.</p> <p>Conclusion</p> <p>Lateral rectus muscle disinsertion and reattachment to the lateral orbital wall is a safe and effective procedure for weakening of the anomalous lateral rectus muscle in Exotropic Duane Syndrome.</p

Neuroimaging Findings in First Unprovoked Seizures: A Multicentric Study in Tehran

How to Cite This Article: Molla Mohammadi M, Tonekaboni SH, Khatami AR, Azargashb E, Tavasoli A, Javadzadeh M, Zamani GR. Neuroimaging Findings in First Unprovoked Seizures: A Multicentric Study in Tehran. Iran J Child Neurol. 2013 Autumn; 7(4):24-31.ObjectiveSeizure is an emergency in pediatrics. It really matters to the parents of the involved child to have information about the causes, management and prognosis.First unprovoked seizures (FUS) are seizures that occur in patients without fever, trauma or infection. Due to the rapid improvement in diagnostic techniques in the last few decades, the etiology will be revealed and this term will no longer exist. This Study was designed to evaluate brain imaging findings in FUS patients. Materials &amp; MethodsNinety-six children with FUS, who were admitted in three major children’s hospitals in Tehran, underwent brain imaging and were enrolled into the study.The decision about the type of imaging (CT or MRI) was based on the patient’s medical and financial conditions. An expert radiologist in the field of pediatric neuroimaging interpreted the images. ResultsAltogether, 27.1% had abnormal findings of which 29.2% were in the brain MRI group and 14.3% were in the brain CT scan group.Abnormal results were gliosis (10.4%), hemorrhage (4.2%), dysgenesis (2.1%), dysmyelination (7.3%), encephalomalacy (1%), atrophy (5.2%) and infarction (2.1%). In some patients, the lesions were in 2 or 3 sites and some had more than one type of lesion.There was no association between the duration, age and type of seizure and imaging abnormlities. However, we found an association between the location of the lesion and the type of seizure. ConclusionWe recommend brain imaging in all patients with FUS and apart from some exceptions, brain MRI is superior to CT. ReferencesJohnston MV. Siezure in childhood. In: Kliegman RM, Behrman RE, editors. Nelson text book of pediatrics. 18th ed. Philadelphia: Saunders; 2010. p. 2457-70.Bluestein JS, Moshe SL. First unprovoked seizure. In: Maria BL, editor. Currents in management in child neurology. 3rd ed. Hamilton: BC Decker; 2005. p. 89-92.Khodapanahandeh F, Hadizadeh H. Neuroimaging in children with first afebrile seizures: to order or not to order? Arch Iran Med 2006 Apr;9(2):156-8.Alawaneh H, Bataineh HA. Urgent neuroimaging in children with first nonfebrile seizures. Middle East JFam Med 2008 Feb;6(1):24-6.Shinnar S, O’Dell C, Mitnick R, Berg AT, Moshe SL. Neuroimaging abnormalities in children with an apparent first unprovoked seizure. Epilepsy Res 2001 Mar;43(3):261-9.Kalnin AJ, Fastenau PS, deGrauw TJ, Musick BS, Perkins SM, Johnson CS, et al. Magnetic resonance imaging findings in children with a first recognized seizure. Pediatr Neurol 2008 Dec;39(6):404-14.King MA, Newton MR, Jackson GD, Fitt GJ, Mitchell LA, Silvapulle MJ et al. Epileptology of the first-seizure presentation: a clinical, electroencephalographic, and magnetic resonance imaging study of 300 consecutive patients. Lancet 1998 Sep 26;352(9133):1007-11.Pohlmann-Eden B, Beghi E, CarnfieldC, Carnfield P. The first seizure and its management in adults and children. BMJ 2006 Feb;332(11):339-34.Raman S, Susan K, Joyce W. Paroxysmal disorders.In: Menkes J, editor. Child neurology. 7th ed. Philadelphia: Lipincott; 2006. p. 857-942.Wical B. The first unprovoked seizure.Gillette Children’sSpecialty Healthcare. A PediatricPerspective 1999 Mar;8(3).Bano S, Yadav SN.Neuroimaging in epilepsy.Medi-Focus2010 Apr-Sep;9(3&amp;4):2-4.Rauch DA,Carr E, Harrington J.Inpatient brain MRI for new-onset seizures: utility and cost effectiveness.Clin Pediatr (Phila) 2008 Jun;47(5):457-60.Gaillard WD, Chiron C, Cross JH, Harvey AS, Kuzniecky R, Hertz-Pannier L et al. Guidelines for imaging infants and children with recent-onset epilepsy. Epilepsia 2009 Sep;50(9):2147-53.Barkovich AJ. Techniques and methods in pediatric neuroimaging. 4thed. Philadelphia: Lippincott Williams &amp;Wilkins; 2005. p. 4-7.Doescher JS, deGrauw TJ, Musick BS, Dunn DW, Kalnin AJ, Egelhoff JC et al. Magnetic resonance imaging and electroencephalic findings in a cohort of normal children with newly dignosed seizures. J Child Neurol 2006 Jun; 21(6):490-5. 

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

The effect of posterior subtenon methylprednisolone acetate in the refractory diabetic macular edema: a prospective nonrandomized interventional case series

BACKGROUND: To investigate the efficacy of posterior subtenon methylprednisolone acetate injection in treatment of refractory diffuse clinically significant diabetic macular edema (CSME). METHODS: In a prospective, nonrandomized, interventional case series, 52 eyes were diagnosed with CSME and treated with at least two sessions of laser photocoagulation according to Early Treatment Diabetic Retinopathy Study guidelines. At least 3 months after laser therapy, eyes with a residual central macular thickness were offered posterior subtenon injection of 40 mg methylprednisolone acetate. Main outcome measures were visual acuity, macular thickness and intraocular pressure. Potential complications were monitored, including intraocular pressure response, cataract progression and scleral perforation. RESULTS: Mean baseline visual acuity (in logMAR) improved significantly (p = 0.003) from 0.8 ± 0.36 to 0.6 ± 0.41 at 3 months. Mean foveal thickness decreased from 388 ± 78 μm at baseline to 231 ± 40 μm after 3 months (p < 0.0001). Visual acuity improvement in eyes with CSME with extrafoveal hard exudates was significant (p = 0.0001), but not significant in eyes with CSME with subfoveal hard exudates (p = 0.32). Intraocular pressure increased from 14.7 ± 2.0 mmHg (range, 12–18 mmHg) to a maximum value of 15.9 ± 2.1 mmHg (range, 12–20 mmHg) during the follow-up period. Complications in two eyes developed focal conjunctival necrosis at the site of injection. CONCLUSION: Posterior subtenon methylprednisolone acetate may improve early visual outcome in diffuse diabetic macular edema that fails to respond to conventional laser photocoagulation. Visual acuity improvement in eyes with CSME with extrafoveal hard exudates was significant; and this improvement is depends on location of hard exudates. Further study is needed to assess the long-term efficacy, safety, and retreatment