DOCSLIB.ORG

Prediction of Enophthalmos by Computed

Total Page：16

File Type：pdf, Size：1020Kb

Download full-text PDF Read full-text

Load more

618 Britishjournal ofOphthalmology 1994; 78: 618-620 Prediction of enophthalmos by computed tomography after 'blow out' orbital fracture Br J Ophthalmol: first published as 10.1136/bjo.78.8.618 on 1 August 1994. Downloaded from R W Whitehouse, M Batterbury, A Jackson, J L Noble Abstract In 11 patients with blow out fracture of the orbit, measurement of orbital volume using computed tomography (CT) more than 20 days after injury correlated weli with enophthalmos measured from the same scans (r=0.87, p<0-001, SEE 0-63 mm), with a 1 cm3 increase in orbital volume causing 0*8 mm ofenophthal- mos. This confirms the cause of enophthalmos after blow out fracture to be increase in orbital volume rather than fat atrophy or fibrosis. In a further 25 patients scanned within 20 days of injury the degree of enophthalmos was less marked than would be predicted from the orbital volume measurement. This was prob- ably because of the presence of oedema, haemorrhage, or both behind the globe which would prevent immediate development of CT measurement of enophthalmos. orbital Figure I Transaxial low dose CT image ofthe orbits. A volume within 20 days ofinjury may predict the medial wall blow outfracture ofthe right orbit is present. The final degree ofenophthalmos and identify those cross sectional area ofeach orbit has been measuredfor patients at risk of late enophthalmos, allowing orbital volume calculation. appropriate early surgical intervention. (BrJ Ophthalmol 1994; 78: 618-620) medial wall and floor fractures, the latter associ- ated with malar fractures in two cases. All scans were performed on a GE9800 general purpose Enophthalmos is a common result of 'blow out' CT scanner (General Electric, Milwaukee, USA) fracture of the orbit. It is now widely accepted using a low radiation dose dynamic transaxial that enophthalmos results from an increase in scan technique as previously described"' http://bjo.bmj.com/ volume of the bony orbit with consequent pos- (120 kVp, 40 mA, 2 s scan time, 3 mm con- terior displacement of the globe as the retro- tiguous sections). Each orbital volume was mea- bulbar fat and muscles are displaced into the sured on the CT console: On each image the bony blow out defect.' 2 Other possible factors such as outline of each orbit was traced out, the anterior fat atrophy or fibrosis3 have not been demon- limit being closed with a line joining the strated to contribute to a significant degree. zygomaticofrontal processes (Fig 1). The areas of Enophthalmos may be both cosmetically un- these outlines were then measured and summed on October 1, 2021 by guest. Protected copyright. acceptable and may cause diplopia by interfering for each orbit. Orbital volume was calculated with extraocular muscle function.4 Enoph- from the summed area multiplied by the section thalmos may not develop immediately after trauma and its onset may occasionally be delayed for 2 months or more."6 Early surgical interven- tion can improve the outcome67 but identifying those patients at risk of late enophthalmos is problematic. Computed tomography (CT) has been used to measure both orbital volume89 and enoph- Department of Clinical thalmos' in blow out fractures but a specific in Radiology, Manchester vivo relation between these measurements has Royal Infirmary, Oxford not been previously defined. The current study Road, Manchester M13 9WL aims to evaluate the relation between orbital R W Whitehouse volume and enophthalmos following blow out A Jackson fracture. Manchester Royal Eye Hospital, Oxford Road, Manchester M13 9WH Patients and methods M Batterbury J The CT examinations of 36 consecutive patients L Noble attending the department ofdiagnostic radiology Correspondence to: Dr R W Whitehouse. for assessment of orbital blow out fractures were Figure 2 The position ofeach globe is measuredfrom the Accepted for publication reviewed. There were 13 orbital floor fractures, zygomaticofrontal process baseline to the back ofthe lens II April 1994 three medial wall fractures, and 21 combined (distances I and 2). Prediction ofenophthalmos by computed tomography after 'blow out' orbitalfracture 619 4 Figure 3 also demonstrates that a large propor- tion of patients (14/36), particularly those with 3 smaller blow out fracture volumes had negative enophthalmos (exophthalmos) at the time of Br J Ophthalmol: first published as 10.1136/bjo.78.8.618 on 1 August 1994. Downloaded from E 2 scanning. When only scans performed more than 20 days E0 1 0 after injury were included, the correlation -cE 0 improved with a similar slope but less negative intercept value to the regression formula (Fig 3, regression line 2): CL -1 c UJ E=0-77V - 0-68 (2) -2 r=0-87, p<0001, SEE 0-63 mm, n=ll In order to demonstrate the dependence on time -3 after injury of the relation between orbital -4 volume and enophthalmos, formula (2) was used -1 0 2 3 4 5 to calculate the expected degree ofenophthalmos from the measured blow out fracture volume for Blow out fracture volume (cm3) all 36 subjects. Unexplained enophthalmos was Figure 3 Relation between blow outfracture volume calculated as measured enophthalmos minus and enophthalmos. Line I is thickness. Both the precision and accurac:y ofthis expected enophthalmos. Unexplained enoph- the regressionfor all 36 data measurement have been previously demon- thalmos was then plotted against time since points, line 2 isfor points strated to be better than 2%." The bllow out injury (Fig 4), using a logarithmic time axis to marked (0), scanned more the than 20 days after injury. fracture size was assumed to be discirepancy clarify the distribution of data points within 20 in volume between the traumatised and normal days ofinjury. orbit volumes, as right and left orbital vol[umes in an individual normally lie within 0 65 cm'3 ofeach other. " 12 Globe position was measured firom the Discussion same zygomaticofrontal process baselin(e to the The results of this study confirm that enoph- posterior surface ofthe lens in each orbit (Fig 2), thalmos after blow out fracture of the, orbit is enophthalmos being the normal eye edistance linearly related to the increase in volume of the minus the traumatised eye distance. blown out orbit. This relation is consistent in injuries between 20 days and 2 years old, with each cm3 increase in volume causing approxi- STATISTICAL TESTS mately 0-77 mm of enophthalmos. Injuries less The relation between enophthalmos andI orbital than 20 days old have a similar regression line volume discrepancy was assessed using.Pearson slope (approximately 0-8 mm per cm') but an correlation coefficients. intercept of -1-67, indicating a coexisting ten- dency for relative exophthalmos in the trau- matised orbit. This should be expected; it http://bjo.bmj.com/ Results presumably represents the effect of soft tissue A highly significant correlation between b)low out swelling in the retrobulbar soft tissues counter- fracture volume and enophthalmos was demon- acting the increase in orbital volume caused by strated in the entire group of 36 subjects (Fig 3, the blow out. Calculating unexplained enoph- regression line 1): thalmos quantifies the effect of this soft tissue E=0-82V- 1-67 (1) swelling, which can then be plotted against time Figure 4 Relation between r=0-74, p<0001, SEE 1-1 mm, n==36 since injury (Fig 4). This graph demonstrates on October 1, 2021 by guest. Protected copyright. unexplained enophthalmos that enophthalmos may be less marked than and time since injury. A log,, where expected by up to 3 mm within 20 days of injury. time scale has been used to E = enophthalmos (mm) Thus up to 3 mm of enophthalmos may be clarify the distribution of = data points. The broken line V blow out fracture volume (cm'3) obscured by retrobulbar soft tissue swelling after indicates 20 days post- SEE = standard error of estimate blow out fracture and this effect may still be injury. apparent 20 days after injury. The volume change of 1 cm' required to produce 0-8 mm of globe movement is greater than that predicted by E a an in vitro model" where a 2 8% volume change E * (approximately 0 56 cm') caused 1 mm of globe Un . 0 a movement. This discrepancy may reflect some E I 0 - resistance to movement of the globe in vivo, * perhaps because of its suspensory ligament - * ~ IE attachments to the bony orbit.'4 Alternatively, it 0 -1 c may be due to the compartmentalisation of 0 'am I.| orbital fat, some of which may be displaced by a . -2 * m blow out fracture, without causing globe dis- placement."S The cause of this discrepancy is of x * * U less practical importance than the recognition of c -3 - ~~~~~* the in vivo relation between orbital volume I change and globe position; this may be used to -4 assess the need for surgery or to estimate the 1 10 100 1000 volume of replacement material required to Time since injury (days log1o scale) reduce enophthalmos. 620 Whitehouse, Batterbury, _Jackson, Noble In a previous retrospective analysis we have influence of time or repair and fracture size. Ophthalmology 1983; 90: 1066-70. demonstrated that surgery is more likely to be 7 Converse JM, Smith B. On the treatment of blow out fractures performed in patients with larger volume blow of the orbit. Plast Reconst Surg 1978; 62: 100-4. 8 Forbes G, Gehring DG, Gorman CA, Brennan MD, Jackson out fractures,6 confirming previous findings.'7 IT. Volume measurements of normal orbital structures by Br J Ophthalmol: first published as 10.1136/bjo.78.8.618 on 1 August 1994. Downloaded from The current study demonstrates that measure- computed tomographic analysis. Amj Roentgenol 1985; 145: 149-54. ment of orbital volume within 20 days of injury 9 Whitehouse RW, Jackson A.
Recommended publications
  • Multipurpose Conical Orbital Implant in Evisceration

    Multipurpose Conical Orbital Implant in Evisceration

    Ophthalmic Plastic and Reconstructive Surgery Vol. 21, No. 5, pp 376–378 ©2005 The American Society of Ophthalmic Plastic and Reconstructive Surgery, Inc. Multipurpose Conical Orbital Implant in Evisceration Harry Marshak, M.D., and Steven C. Dresner, M.D. Doheny Eye Institute, Keck School of Medicine, University of Southern California, Los Angeles, California, U.S.A. Purpose: To evaluate the safety and efficacy of the porous polyethylene multipurpose conical orbital implant for use in evisceration. Methods: A retrospective review of 31 eyes that underwent evisceration and received the multipurpose conical orbital implant. The orbits were evaluated at 1 week, 1 month, and 6 months after final prosthetic fitting for implant exposure, superior sulcus deformity, and prosthetic motility. Results: There were no cases of extrusion, migration, or infection. All patients had a good cosmetic result after final prosthetic fitting. Prosthetic motility was good in all patients. Exposure developed in one eye (3%) and a superior sulcus deformity developed in one eye (3%). Conclusions: Placement of an multipurpose conical orbital implant in conjunction with evisceration is a safe and effective treatment for blind painful eye that achieves good motility and a good cosmetic result. visceration has proved to be effective for the treat- forms anteriorly to the sclera to be closed over it, without Ement of blind painful eye from phthisis bulbi or crowding the fornices, and extends posteriorly through endophthalmitis. By retaining the sclera in its anatomic the posterior sclerotomies, providing needed volume to natural position, evisceration has the advantage of allow- the posterior orbit. ing the insertions of the extraocular muscles to remain intact, promoting better motility.
  • Treatment of Congenital Ptosis

    Treatment of Congenital Ptosis

    13 Review Article Page 1 of 13 Treatment of congenital ptosis Vladimir Kratky1,2^ 1Department of Ophthalmology, Queen’s University, Kingston, Canada; 21st Medical Faculty, Charles University, Prague, Czech Republic Correspondence to: Vladimir Kratky, BSc, MD, FRCSC, DABO. Associate Professor of Ophthalmology, Director of Ophthalmic Plastic and Orbital Surgery, Oculoplastics Fellowship Director, Queen’s University, Kingston, Canada; 1st Medical Faculty, Charles University, Prague, Czech Republic. Email: [email protected]. Abstract: Congenital ptosis is an abnormally low position of the upper eyelid, with respect to the visual axis in the primary gaze. It can be present at birth or manifest itself during the first year of life and can be bilateral or unilateral. Additionally, it may be an isolated finding or part of a constellation of signs of a specific syndrome or systemic associations. Depending on how much it interferes with the visual axis, it may be considered as a functional or a cosmetic condition. In childhood, functional ptosis can lead to deprivation amblyopia and astigmatism and needs to be treated. However, even mild ptosis with normal vision can lead to psychosocial problems and correction is also advised, albeit on a less urgent basis. Although, patching and glasses can be prescribed to treat the amblyopia, the mainstay of management is surgical. There are several types of surgical procedure available depending on the severity and etiology of the droopy eyelid. The first part of this paper will review the different categories of congenital ptosis, including more common associated syndromes. The latter part will briefly cover the different surgical approaches, with emphasis on how to choose the correct condition.
  • The Management of Congenital Malpositions of Eyelids, Eyes and Orbits

    The Management of Congenital Malpositions of Eyelids, Eyes and Orbits

    Eye (\988) 2, 207-219 The Management of Congenital Malpositions of Eyelids, Eyes and Orbits S. MORAX AND T. HURBLl Paris Summary Congenital malformations of the eye and its adnexa which are multiple and varied can affect the whole eyeball or any part of it, as well as the orbit, eyelids, lacrimal ducts, extra-ocular muscles and conjunctiva. A classification of these malformations is presented together with the general principles of treatment, age of operating and surgical tactics. The authors give some examples of the anatomo-clinical forms, eyelid malpositions such as entropion, ectropion, ptosis, levator eyelid retraction, medial canthus malposition, congenital eyelid colobomas, and congenital orbital abnormalities (Craniofacial stenosis, orbi­ tal plagiocephalies, hypertelorism, anophthalmos, microphthalmos and cryptophthalmos) . Congenital malformations of the eye and its as echography, CT-scan and NMR, enzymatic adnexa are multiple and varied. They can work-up or genetic studies (Table I). affect the whole eyeball or any part of it, as Surgical treatment when feasible will well as the orbit, eyelids, lacrimal ducts extra­ encounter numerous problems; age will play a ocular muscles and conjunctiva. role, choice of a surgical protocol directly From the anatomical point of view, the fol­ related to the existing complaints, and coop­ lowing can be considered. eration between several surgical teams Position abnormalities (malpositions) of (ophthalmologic, plastic, cranio-maxillo-fac­ one or more elements and formation abnor­ ial and neurosurgical), the ideal being to treat malities (malformations) of the same organs. Some of these abnormalities are limited to Table I The manag ement of cong enital rna/positions one organ and can be subjected to a relatively of eyelid s, eyes and orbits simple and well recognised surgical treat­ Ocular Findings: ment.
  • Infantile Glaucoma in Rubinstein–Taybi Syndrome J Dacosta and J Brookes 1271

    Infantile Glaucoma in Rubinstein–Taybi Syndrome J Dacosta and J Brookes 1271

    Eye (2012) 26, 1270–1271 & 2012 Macmillan Publishers Limited All rights reserved 0950-222X/12 www.nature.com/eye CASE SERIES Infantile glaucoma J DaCosta and J Brookes in Rubinstein–Taybi syndrome Abstract Taybi syndrome. Nystagmus, enophthalmos, right exotropia, unilateral axial myopia, Purpose Long-term follow-up of patients increased horizontal corneal diameters, and with Rubinstein–Taybi-associated infantile corneal oedema were present. Intraocular glaucoma. pressures were 45 mm Hg on the right and Methods Case series. 28 mm Hg on the left with advanced optic disc Results Three cases of infantile glaucoma in cupping. Bilateral goniotomies were performed association with Rubinstein–Taybi syndrome and this controlled intraocular pressure in are presented. combination with topical treatment. Vision was Discussion This report highlights the 6/96 on the right and 6/19 on the left at the importance of measuring intraocular pressure age of 3 years. in this condition, as glaucoma is one of the major preventable causes of blindness in childhood. Case 3 Eye (2012) 26, 1270–1271; doi:10.1038/eye.2012.123; published online 22 June 2012 A 5-month-old boy with micrognathia and broad thumbs. The left corneal diameter was Keywords: glaucoma; infantile; Rubinstein– increased with corneal oedema. Previously, Taybi syndrome goniotomy had been attempted. Intraocular pressure was not controlled with topical therapy, and Baerveldt tube surgery was Introduction performed. Eighteen months after surgery, intraocular pressure was controlled and Multiple ocular abnormalities have been described in Rubinstein Taybi syndrome. This vision was 6/76 on the right and 6/96 on case series describes long-term follow-up of the left.
  • Clinical and Molecular Genetic Aspects of Leber's Congenital

    Clinical and Molecular Genetic Aspects of Leber's Congenital

    Clinical and Molecular Genetic Aspects 10 of Leber’s Congenital Amaurosis Robert Henderson, Birgit Lorenz, Anthony T. Moore | Core Messages of about 2–3 per 100,000 live births [119, 50]. ∑ Leber’s congenital amaurosis (LCA) is a It occurs more frequently in communities severe generalized retinal dystrophy which where consanguineous marriages are common presents at birth or soon after with nystag- [128]. mus and poor vision and is accompanied by a nonrecordable or severely attenuated ERG 10.1.1 ∑ As some forms are associated with better Clinical Findings vision during childhood and nystagmus may be absent, a wider definition is early LCA is characterized clinically by severe visual onset severe retinal dystrophy (EOSRD) impairment and nystagmus from early infancy with LCA being the most severe form associated with a nonrecordable or substantial- ∑ It is nearly always a recessive condition but ly abnormal rod and cone electroretinogram there is considerable genetic heterogeneity (ERG) [32, 31, 118]. The pupils react sluggishly ∑ There are eight known causative genes and and, although the fundus appearance is often three further loci that have been implicated normal in the early stages,a variety of abnormal in LCA/EOSRD retinal changes may be seen. These include pe- ∑ The phenotype varies with the genes ripheral white dots at the level of the retinal pig- involved; not all are progressive. At present, ment epithelium, and the typical bone-spicule a distinct phenotype has been elaborated pigmentation seen in retinitis pigmentosa. for patients with mutations in RPE65 Other associated findings include the ocu- ∑ Although LCA is currently not amenable to lodigital sign, microphthalmos, enophthalmos, treatment, gene therapy appears to be a ptosis, strabismus, keratoconus [28], high re- promising therapeutic option, especially fractive error [143],cataract,macular coloboma, for those children with mutations in RPE65 optic disc swelling, and attenuated retinal vas- culature.
  • Entropion Ectropion

    Entropion Ectropion

    1 Involutional Entropion vs Q Involutional Ectropion What does the term Entropion mean? Ectropion 2 Involutional Entropion vs A Involutional Ectropion What does the term Entropion mean? Ectropion It means the eyelid margin is turning inward 3 Involutional Entropion vs Q Involutional Ectropion What does the term What does the term Entropion mean? Ectropion mean? It means the eyelid margin is turning inward 4 Involutional Entropion vs A Involutional Ectropion What does the term What does the term Entropion mean? Ectropion mean? It means the eyelid margin is It means the eyelid margin is turning inward turning outward 5 Involutional Entropion vs Q Involutional Ectropion The Plastics book identifies six general causes of entropion and/or ectropion. What are they? (Note that while most apply to both entropion and ectropion, a few apply only to one or the other.) Entropion Categories Ectropion ? ? ? ? ? ? 6 Involutional Entropion vs A Involutional Ectropion The Plastics book identifies six general causes of entropion and/or ectropion. What are they? (Note that while most apply to both entropion and ectropion, a few apply only to one or the other.) Entropion Categories Ectropion Congenital Involutional Paralytic Cicatricial Mechanical Acute Spastic 7 Involutional Entropion vs Q Involutional Ectropion Of the six, which can result in entropion? Entropion Categories Ectropion ? Congenital ? Involutional ? Paralytic ? Cicatricial ? Mechanical ? Acute Spastic 8 Involutional Entropion vs A Involutional Ectropion Of the six, which can result in entropion?
  • Delayed Cranial Nerve Palsies and Chiari Type I Malformation After Epidural Anesthesia in the Setting of Childbirth

    Delayed Cranial Nerve Palsies and Chiari Type I Malformation After Epidural Anesthesia in the Setting of Childbirth

    Open Access Case Report DOI: 10.7759/cureus.12871 Delayed Cranial Nerve Palsies and Chiari Type I Malformation After Epidural Anesthesia in the Setting of Childbirth James P. Caruso 1 , Salah G. Aoun 1 , Jean-Luc K. Kabangu 1 , Olutoyosi Ogunkua 2 , Carlos A. Bagley 1 1. Neurosurgery, University of Texas Southwestern Medical Center, Dallas, USA 2. Anesthesiology, University of Texas Southwestern Medical Center, Dallas, USA Corresponding author: James P. Caruso, [email protected] Abstract Epidural analgesia is an efficient method of controlling pain and has a wide spectrum of therapeutic and diagnostic applications. Potential complications may occur in a delayed fashion, can remain undiagnosed, and can be a source of significant morbidity. We present a 37-year-old woman presented with severe spontaneous occipital headaches, diplopia, and dizziness that occurred spontaneously six weeks after giving birth. Her primary method of pain control during labor was epidural analgesia. Her neurologic exam revealed a cranial nerve six palsy with ptosis, and her brain MRI demonstrated a Chiari I malformation which had not been previously diagnosed. CT myelography of the lumbar spine revealed extradural contrast extravasation within the interspinous soft tissue at L1-L2, which was the site of her prior epidural procedure. She underwent epidural blood patch administration, and her cranial nerve palsy resolved along with all of her other symptoms. The development of concurrent Chiari I malformation and cranial nerve palsy after epidural anesthesia is an exceptionally rare occurrence. Neurologic complications after epidural anesthesia are likely under-reported, since patients are often lost to follow-up or have subtle neurologic signs which can easily be missed.
  • Marfan Syndrome: Jeffrey Welder MSIII, Erik L

    Marfan Syndrome: Jeffrey Welder MSIII, Erik L

    Marfan Syndrome: Jeffrey Welder MSIII, Erik L. Nylen MSE, Thomas Oetting MS MD May 6, 2010 Chief Complaint: Decreased vision and glare in both eyes. History of Present Illness: A 28 year old woman with a history of Marfan syndrome presented to the comprehensive ophthalmology clinic reporting a progressive decrease in vision and worsening glare in both eyes. She had been seen by ophthalmologists in the past, and had been told that her crystalline lenses were subluxed in both eyes. She had not had problems with her vision until recent months. Past Medical History: Marfan syndrome with aortic stenosis followed by cardiology Medications: Oral beta blocker Family History: No known family members with Marfan Syndrome. Grandmother with glaucoma. Social History: The patient is a graduate student. Ocular Exam: External Exam normal. VA (with correction): OD 20/40 OS 20/50 Current glasses: OD: 6.75+ 5.00 x 135 OS: -5.25 + 4.25 x 60 Pupils: No anisocoria and no relative afferent pupillary defect Motility: Ocular motility full OU. Anterior segment exam: Inferiorly subluxed lenses OU (figure 1 and 2). The angle was deep OU and there was no lens apposition to the cornea in either eye. Dilated funduscopic exam: Posterior segment was normal OU with no peripheral retinal degeneration . Course: The patient’s subluxed lenses led to poor vision from peripheral lenticular irregular astigmatism and glare. She was taken to the operating room where her relatively clear lenses were removed and iris sutured intraocular lenses were placed. The surgical video for one of the eyes may be viewed at http://www.facebook.com/video/video.php?v=153379281140 Page | 1 Figure One: Note the inferiorly subluxed lens.
  • Surgical Cure of Senile Entropion* by Wallace S

    Surgical Cure of Senile Entropion* by Wallace S

    Br J Ophthalmol: first published as 10.1136/bjo.45.10.678 on 1 October 1961. Downloaded from Brit. J. Ophthal. (1961) 45, 678. SURGICAL CURE OF SENILE ENTROPION* BY WALLACE S. FOULDS Addenbrooke's Hospital, Cambridge THE large number of surgical procedures which has been devised for the treatment of senile entropion suggests that none is universally successful. In this paper a new operation which has been successfully employed for over 3 years in the treatment of entropion is described. Not only has this procedure resulted in a cure of the condition in every case in which it has been used, but in addition the cosmetic appearance of the eyelid post- operatively has been excellent. It is thought that the operation offers advantages over other procedures currently used in the correction of this deformity. History Entropion of the lower lid was recognized, and surgical treatment of the condition described nearly 2,000 years ago by Celsus (Arruga, 1956). For many years the condition was believed to be due to spasm of the orbicularis muscle, but more recently it has been attributed to laxity of one or more of copyright. the lid structures together with a loss of lid support due to enophthalmos resulting from atrophy of orbital fat. Fox (1959) has shown that the condition is not relieved by local anaesthesia of the eye nor by facial nerve block, and many writers (Butler, 1948; Fox, 1951; Kirby, 1953) have stressed that it occurs where the tissues of the lid are atonic and there is no evidence of orbicularis spasm.
  • A Matter of Millimeters

    A Matter of Millimeters

    3/28/2016 PATIENT INFORMATION A MATTER OF • 57 WM • POH • (+) trauma OS MILLIMETERS • PMH • Hyponatremia • Hyperglycemia • Chronic low back pain ALANNA O’KEEFE – MARCH 29TH, 2016 PATIENT INFORMATION EXAM INFORMATION • CC – “my eye sticks out”, “friends were worried” • BCVA: 20/20 OU • (-) pain, diplopia, pulsatile exophthalmos, bruit • Refraction • No thyroid problems • OD: -4.75 -1.25 x 040 • (+) occasional FBS, redness • OS: -2.75 -2.25 X 150 • EOMS: Limitation OS • VF: clear OU • Exophthalmometry • Base: 100 mm • OD: 14 mm • OS: 21 mm http://www.jcytol.org/showbackIssue.asp?issn=0970-9371;year=2008;volume=25;issue=2 1 3/28/2016 EXAM INFORMATION PLAN • Observation • Exophthalmos • Incomplete lid closure OS • CT • IOP • CTA • 16 mmHg OU • Lens • AMD • 1+ NS • Preservision and Amsler grid • Optic Nerve • Cataracts • OD: .15 • Monitor yearly • OS: .25 • CMA with Presbyopia • Vitreous • Asteroid hyalosis OD • SRx •Macula • Soft coalesced drusen OU CLINICAL MANIFESTATIONS EXOPHTHALMOS OVERVIEW • Exophthalmos • Proptosis • Bruit • Exophthalmos • Chemosis • Specific • Arterialized corkscrew conjunctival vessels • Thyroid-Associated Ophthalmopathy • Diplopia • Glaucoma 2 3/28/2016 MEASUREMENT HERTEL EXOPHTHALMOMETER • Exophthalmometry • Hertel exophthalmometer • Base measurement • Used for future measurements • Exophthalmos measurement • Accuracy • Patient fixation • Positioning • Worm’s-eye view https://www2.aofoundation.org/wps/portal/!ut/p/a0/04_Sj9CPykssy0xPLMnMz0vMAfGjzOKN_A0M3D2DDbz9_UMMDRyDXQ3dw9wMDA x8jfULsh0VAdAsNSU!/?ActiveNumber=1&ActivePopup=1&StepPos=510&contentUrl=%2Fsrg%2Fpopup%2Fadditional_material%2F92%2F
  • Leber Congenital Amaurosis

    Leber Congenital Amaurosis

    Leber congenital amaurosis Authors: Doctors Bart P Leroy1 and Sharola Dharmaraj2 Creation date: November 2003 Scientific Editor: Professor Jean-Jacques de Laey 1Dept of Ophthalmology & Ctr for Medical Genetics, Ghent University Hospital, Ghent, Belgium 2Johns Hopkins Center for Hereditary Eye Diseases, Wilmer Eye Institute, Baltimore, MD, USA Abstract Key words Disease name /synonyms Definition / Diagnostic criteria Differential diagnosis Etiology Clinical description Diagnostic methods Epidemiology Genetic counseling Prenatal diagnosis Management including treatment Unresolved questions References Abstract Leber congenital amaurosis (LCA) is a retinal dystrophy and/or dysplasia of prenatal onset. About 10 to 20% of blind children are thought to suffer from LCA, which makes it one of the frequent causes of childhood blindness. It is thought to account for 5% of inherited retinal disease. Affected children fail to fix and follow due to little or no retinal sensitivity to visual stimuli. Electroretinography shows either no or very reduced retinal function. Fundus examination in the first months of life is frequently normal, but later chorioretinal atrophy with intraretinal pigment migration becomes apparent. In some patients, a macular puched-out lesion is present. Patients have nystagmus and frequently poke their eyes. LCA is inherited as an autosomal recessive trait in the large majority of patients, with only a limited number of cases with autosomal dominant inheritance described. LCA is genetically heterogeneous, and, to date, mutations have been identified in six different genes known to be associated with LCA: AIPL1, CRB1, CRX, GUCY2D, RPE65 and RPGRIP1. At least another three additional loci have been linked to the condition. Although therapy is not currently available, encouraging results have been obtained with gene therapy in a dog model for this disease.
  • Late Congenital Leber's Amaurosis At

    Late Congenital Leber's Amaurosis At

    sease Di s a e n y d E f D i o s l o a r d n r e u r s o J ISSN: 2684-1622 Journal of Eye Diseases and Disorders Case Report Late Congenital Leber’s Amaurosis at Lubumbashi in the Democratic Republic of Congo: Case Report Yogolelo Asani Bienvenu1*, Kasamba Ilunga Eric2, Luembe Kasongo Daudet1, Iye Ombamba Kayimba Bruno1, Omewatu Mungomba Jacques3, Alfani Binti Lungwe Marie-Ange4, Ngoy Kilangalanga Janvier5. 1Ophthalmology Department of University Clinics of Lubumbashi, Lubumbashi, Democratic Republic of the Congo 2Laboratory service of University Clinics of Lubumbashi, Lubumbashi, Democratic Republic of the Congo 3Anatomopathology Department of University Clinics of Lubumbashi, Lubumbashi, Democratic Republic of the Congo 4Department of English, Higher Pedagogical Institute, Lubumbashi, Democratic Republic of the Congo 5Ophthalmological Center Saint Joseph of Kinshasa, Lubumbashi, Democratic Republic of the Congo ABSTRACT The authors report a case of congenital Leber's amaurosis on convergent strabismus alternates lefteye fixatordiscoveredat a late stage in an 8-year-old male child at University Clinics of Lubumbashi. This observation draws the attention of clinical researchers to the need of good early clinical examination in the presence of retinal dystrophy in children. Keywords: Congenital Leber’s amaurosis, retinal disease, early onset, retinal dystrophy, DR Congo. INTRODUCTION shows a small congenital central cortical opacity in the right eye and in the left eye the anterior and posterior segment are Described in 1869 by Leber, the congenital Leber's amaurosis is without any particularity. On examination of the fundus of both the earliest and most severe form of all hereditary retinal eyes, we noticed a macula with fine whitish punctuations having dystrophies.