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Mike > Peds/Strab > Flashcards

Peds/Strab Flashcards

1
Q

<p>What is the size of the eye at birth?</p>

A

<p>66% of that of the adult eye</p>

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2
Q

<p>What is the common refractive error in children?</p>

A

<p>Hyperopia which increases upto 7 years of age and then decreases</p>

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3
Q

<p>Which internal eye muscle is poorly developed at birth?</p>

A

<p>Dilator pupillae</p>

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4
Q

<p>What are the tools to check for VA in children?</p>

A

<p>VEP in infancyPreferential looking in first months of life</p>

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5
Q

<p>What is the normal axial length of an eye at birth?</p>

A

<p>17 mm, In adults it is 24 mm</p>

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6
Q

<p>What is the normal corneal diameter of a newborn?</p>

A

<p>Newborn: 9.51 year of life: 10.5Adult: 12 mm</p>

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7
Q

<p>What is the Ddx of an INFANT with poor vision but NORMAL ocular structures?</p>

A

<p>LCA, optic nerve hypoplasia, delay in maturation, optic atrophy, blue cone monochromatism, achromatopsia, CSNB, TORCH Infections, cortical visual impairment</p>

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8
Q

<p>Define the following terms:1. Anophthalmos2. Microphthalmos3. Nanophthalmos4. Buphthalmos5. Cryptophthalmos</p>

A

<p>1. Anophthalmos: B/l absence of eyes, hypoplastic orbits2. Microphthalmos: Small disorganized eye3. Nanophthalmos: Small eye with normal anatomy4. Buphthalmos: Large eye with elevated IOP5. Cryptophthalmos: Failure of differentiation of eyelid and anterior eye structures</p>

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9
Q

<p>What is the most common organism that causes preseptal cellulitis in children?</p>

A

<p>S. Aureus</p>

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10
Q

<p>What is the most common organism that causes postseptal cellulitis in children?</p>

A

<p>S. Aureus, S. pneumo</p>

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11
Q

<p>What are the treatment guidelines for emergent drainage in orbital cellulitis?</p>

A

<p>Age> 9 yearsFrontal sinusitisNon medial locationLarge sizeAnaerobic infection (gas on the CT)Recurrence after prior drainageChronic sinusitis (polyps)Optic neuropathyDental origin</p>

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12
Q

<p>What is a dermoid cyst?</p>

A

<p>It is a choristoma.The most common benign orbital lesion in childhood</p>

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13
Q

<p>How does a dermoid cyst appear on CT scan?</p>

A

<p>"Well circumscribed mass with bony remodeling"</p>

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14
Q

<p>What is the treatment for dermoid cyst?</p>

A

<p>Complete excision W/O rupture as it may cause granulomatous inflammation</p>

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15
Q

<p>What is epidermoid cyst?</p>

A

<p>Another choristoma with epidermal elements. Usually filled with keratin. Complete excision is requires as acute rupture may cause inflammation</p>

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16
Q

<p>What is a teratoma?</p>

A

<p>Rare cystic tumor arising from two or more germinal layers (ectoderm+endoderm+/-mesoderm)</p>

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17
Q

<p>What is the most common benign tumor in children?</p>

A

<p>Capillary hemangioma</p>

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18
Q

<p>What are the characteristics of capillary hemangioma?</p>

A

<p>Often manifests in the first few weeks of life and enlarges over for the first 6-12 months of life with complete regression by age 5-8 years in 80% of cases</p>

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19
Q

<p>What is the most common location for capillary hemangioma?</p>

A

<p>SN quadrant of the ORBIT and medial upper eyelid</p>

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20
Q

<p>How to distinguish a port wine stain from hemangioma?</p>

A

<p>Port wine stain DO NOT blanch with pressure</p>

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21
Q

<p>What is the pathology of capillary hemangioma?</p>

A

<p>Unencapsulated mass with numerous blood-filled channels lined by endothelium.</p>

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22
Q

<p>What is the most common treatment for capillary hemangioma?</p>

A

<p>Topical timolol. Treatment only if tumor causes ptosis, amblyopia, astigmatism with anisometropia or strabimus</p>

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23
Q

<p>Name this syndrome:High output congestive heart failure with multiple visceral capillary hemangioma</p>

A

<p>Kassabach-Merritt syndrome: Consumptive coagulopathy with platelet trapping-resulting in thrombocytopenia and cardiac failure</p>

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24
Q

<p>Name two conditions that cause intermittent proptosis?</p>

A

<p>Lymphangioma and orbital varix</p>

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25
Q

<p>What is the most common location of lymphagioma?</p>

A

<p>Superonasal quadrant of the ORBIT. Acute flares with URI. </p>

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26
Q

<p>What is the pathology of lymphangioma?</p>

A

<p>Unencapsulated mass with numerous lymph-filled channels lined by endothelium.</p>

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27
Q

<p>Name the proptosis that worsens with crying or straining?</p>

A

<p>Orbital varix</p>

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28
Q

<p>Name few choristomas?</p>

A

<p>Dermoid, epidermoid, lymphangioma</p>

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29
Q

<p>Is Neurofibroma, a hamartoma or choristoma?</p>

A

<p>Hamartoma</p>

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30
Q

<p>What is the difference between hamartoma and choristoma?</p>

A

<p>Hamartoma: abnormal tissue in normal locationChoristoma: Normal tissue in wrong location</p>

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31
Q

<p>What does "S-shaped eyelid" indicates?</p>

A

<p>Plexiform neurofibroma</p>

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32
Q

<p>What are the pathology of neurofibroma?</p>

A

<p>Well circumscribed, unencapsulated proliferation of schwann cells, perineural cells and axonsStains with S-100</p>

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33
Q

<p>What systemic disease is associated with optic nerve glioma?</p>

A

<p>NF 1</p>

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34
Q

<p>What is the most common primary orbital malignancy in children?</p>

A

<p>Rhabdomyosarcoma</p>

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35
Q

<p>What is the average age of diagnosis of Rhabdomyosarcoma?</p>

A

<p>8 years. (90% before age 16)</p>

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36
Q

<p>What is the most common location of rhabdomyosarcoma?</p>

A

<p>Superonasal orbit. Usually unilateral, acute presentation</p>

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37
Q

<p>What is the most common site of metastases of Rhabdo?</p>

A

<p>Chest</p>

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38
Q

<p>What are the types of Rhabdo?</p>

A

<p>Embryonal: Most commonPleomorphic: Least common, best prognosisAlveolar: Poorest prognosis(very high mortality)Botryoid: Subtype of embryonal</p>

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39
Q

<p>What are the treatment options for Rhabdo?</p>

A

<p>Urgent bx, XRT or chemo for microscopic metastases</p>

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40
Q

<p>Child with acute proptosis and periorbital ecchymosis (raccoon eyes). What is the diagnosis?</p>

A

<p>Neuroblastoma</p>

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41
Q

<p>What is the most common site of metastases for neuroblastoma?</p>

A

<p>In children it is boneIn adults it is uveal tract</p>

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42
Q

<p>Name the group of disorders that result from abnormal proliferation of histiocytes (Langerhans cells)?</p>

A

<p>Histiocytosis X (Langerhans cell histiocytosis)</p>

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43
Q

<p>Name the diagnosis:CT finding of "lytic lesions of orbital roof with progressive proptosis"</p>

A

<p>Eosinophic granuloma</p>

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44
Q

<p>What is the triad of langerhans cell histiocytosis (LCH) or (Hand-Schuller-Christian disease)</p>

A

<p>ProptosisLytic skull defectsDiabetes INSIPIDUS</p>

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45
Q

<p>What is the most common location of LCH?</p>

A

<p>Superolateral orbit</p>

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46
Q

<p>What is the most common viral infection associated with Burkitt's lymphoma?</p>

A

<p>EBV</p>

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47
Q

<p>What sinus is usually affected by Burkitt's lymphoma?</p>

A

<p>Maxillary sinus in black children</p>

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48
Q

<p>Name the diagnosis:"Path with "malignant B cells in "starry sky" appearance of histiocytes"</p>

A

<p>Burkitt's lymphoma</p>

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49
Q

<p>What is the most common week of gestation for failure of facial fissures to close?</p>

A

<p>6th-7th week of gestation</p>

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50
Q

<p>What craniofacial syndrome is associated with lower lid colobomas?</p>

A

<p>Goldenhar's syndrome</p>

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51
Q

<p>Name the syndromeOther name: Oculoauriculovertebral dysplasiaAbnormalities of 1st and 2nd brachial archesAssociated with Duane's syndrome</p>

A

<p>Goldenhar's syndrome</p>

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52
Q

<p>Name the syndrome.Proptosis, V-pattern XT, nystagmus, hypertelorism, shallow orbits, optic atrophy-due to kinking or stretching of optic nerve or narrowing of optic canal</p>

A

<p>Crouzon's syndrome. AD or sporadic</p>

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53
Q

<p>Name the syndrome. "Crouzon's + syndactyly"</p>

A

<p>Apert's syndrome (AD). Associated with increase in paternal age</p>

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54
Q

<p>Name the syndrome.White forelock, telecanthus, heterchromia iridis, fundus hypopigmentation and sensorineural hearing loss</p>

A

<p>Waardenburg's syndrome (AD)</p>

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55
Q

<p>What is the difference between telecanthus and hypertelorism?</p>

A

<p>Telecanthus: Increased inner canthal distance(ICD) and puncta and normal IPD, smaller palpebral fissure length (PFL)Hypertelorism: Increased IPD, ICD and normal PFL</p>

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56
Q

<p>Name the syndrome:RD, cataracts and or glaucoma, high myopia. micrognathia, glossoptosis. Close association with Stickler's syndrome</p>

A

<p>Pierre Robin sequence</p>

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57
Q

<p>Define the following terms:AblepharonAnkyloblepharonEuryblepharonBlepharophimosis</p>

A

<p>Ablepharon: Absence of eyelidsAnkyloblepharon: Partial or complete fusion of lid marginsEuryblepharon: Horizontal shortening of palpebral fissure due to inferior insertion of lateral canthal tendonBlepharophimosis: Horizontally and vertically shortened palpebral fissures with poor levator function</p>

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58
Q

<p>What is the triad of blepharophimosis?</p>

A

<p>Ptosis, Telecanthus, Epicanthus inversus</p>

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59
Q

<p>Name few causes of Ankyloblepharon?</p>

A

<p>SJS, OCP, thermal or chemical burns or inflammation</p>

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60
Q

<p>What is the triad of Horner's syndrome?</p>

A

<p>Ptosis, miosis and anhydrosis</p>

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61
Q

<p>What is the most common cause of congenital eCtropion?</p>

A

<p>Vertical shortening of Anterior lamella</p>

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62
Q

<p>What is the most common cause of congenital eNtropion?</p>

A

<p>Vertical shoterning of posterior lamella, tarsal plate defects, lid retractor dysgenesis</p>

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63
Q

<p>What is Distichiasis?</p>

A

<p>Partial or complete accessory row of eyelashes growing from posterior to meibomian orifices</p>

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64
Q

<p>Define the following terms:Epicanthus tarsalisEpicanthus inversusEpicanthus PalpebralisEpicanthus supraciliaris</p>

A

<p>Epicanthus tarsalis: Prominent fold in upper eyelidEpicanthus inversus: Prominent fold in LLEpicanthus Palpebralis: Fold equally distributed in UL &amp;amp; LLEpicanthus supraciliaris: Fold arises from eyebrow and extends to lacrimal sac</p>

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65
Q

<p>What is the diagnosis? Bluish swelling inferior and nasal to medial canthus present at birth</p>

A

<p>Dacryocystocele. Usually due to amniotic fluid or mucus </p>

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66
Q

<p>What are the most common organisms that cause dacryocystitis?</p>

A

<p>S. Pneumo, S.aureus, H.flu, Klebsiella, pseudomonas</p>

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67
Q

<p>Which valve was being covered by membrane in NLD?</p>

A

<p>Valve of Hasner</p>

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68
Q

<p>What are the treatment options for NLD in children?</p>

A

<p>Crigler's massage, probing and irrigation by age 13 months (95% cure rate), Silicone intubation if probing is unsuccessful, DCR after multiple failures</p>

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69
Q

<p>What is ocular melanocytosis?</p>

A

<p>Unilateral excessive pigment in uvea, sclera and episclera. </p>

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70
Q

<p>What is Nevus of Ota?</p>

A

<p>Ocular melanocytosis associated with pigmentation of eyelid skin</p>

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71
Q

<p>Define ophthalmia neonatorum?</p>

A

<p>Conjunctivitis within first month of life. Papillary conjunctivitis with no follicular reaction in neonate due to immaturity if immune system.</p>

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72
Q

<p>Name the types of ophthalmia neonatorum?</p>

A

<p>Chemical: first 24 hours due to silver nitrateN. Gonorrhea: 1-2 days. can occur with PROMChlamydia: 2-4 daysHSV: 5-14 days </p>

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73
Q

<p>What are the findings of N. Gonorrhea conjunctivitis?</p>

A

<p>Severe purulent d/c, chemosis, eyelid edema, hemorrhagic, may develop corneal ulceration or perforation.</p>

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74
Q

<p>What is the treatment for N. Gonorrhea conjunctivitis?</p>

A

<p>IV ceftriaxone and topical bacitracin. Treat for possible Chlamydia conjunctivitis.</p>

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75
Q

<p>What is the treatment for chlamydial conjunctivitis?</p>

A

<p>Topical and PO erythromycin (syrup) to prevent pneumonitis, treat mother and sexual partners with doxycycline</p>

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76
Q

<p>What are the signs of neonatal conjunctivitis? (Chlamydia)</p>

A

<p>Acute mucopurulent d/c, papillary rxn, pseudomembranes. Associated with pneumonitis, otitis, nasopharyngitis, gastritis</p>

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77
Q

<p>What is the most common cause of pediatric conjunctivitis compared to adults?</p>

A

<p>Bacterial (50-80%) in childrenViral in adults</p>

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78
Q

<p>What are the demographics of vernal keratoconjunctivitis?</p>

A

<p>Seasonal (spring) allergic conjunctivitis. M>F, onset by age 10 and resolves by puberty.</p>

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79
Q

<p>What factors are associated with VK?</p>

A

<p>Atopic dermatitis (85%), fam hx of atopy (66%)</p>

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80
Q

<p>What are the findings of VK?</p>

A

<p>Horner-trantus dots, shield ulcers, limbal follicles, copious ropy mucus, pseudomembrane, keratitis, micropannus, </p>

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81
Q

<p>What are Horner-trantas dots?</p>

A

<p>Elevated white accumulations of eosinophils at limbus</p>

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82
Q

<p>Name the diagnosis?Bilateral, thick pseudomembranous (woody appearance) conjunctivitis in children, common among young girls</p>

A

<p>Ligneous conjunctivitis</p>

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83
Q

<p>What is the pathology of VK?</p>

A

<p>High levels of histamine and IgE in tears, mast cells, eosinophils, basophils</p>

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84
Q

<p>What is the pathology of ligneous?</p>

A

<p>Acelluar eosinophilic hyaline material, IgG, T-cells, mast cells and eosinophils</p>

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85
Q

<p>What is the most common descent affected by Kawasaki?</p>

A

<p>Japanese descent</p>

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86
Q

<p>What is the diagnostic criteria for Kawasaki disease?</p>

A

<p>Need 5 of 6:Fever, bilateral conjunctivitis (90%), strawberry tongue, b/l nongranulomatous uveitis(80%), rash, cervical LAD, lesions of extremties (edema, erythema, desquamation)</p>

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87
Q

<p>What is the treatment for Kawasaki dz?</p>

A

<p>Aspirin. </p>

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88
Q

<p>What is the life threatening feature of Kawasaki disease?</p>

A

<p>Coronary artery aneurysm or MI</p>

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89
Q

<p>What diseases are associated with anterior megalophthalmos?</p>

A

<p>Marfan's, mucolipidosis type II, Apert's syndrome</p>

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90
Q

<p>What is congenital corneal staphyloma?</p>

A

<p>Protuberant corneal opacity due to intrauterine keratitis</p>

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91
Q

<p>What diseases are associated with cornea plana?</p>

A

<p>Scleroplana, microcornea and angle closure glacoma</p>

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92
Q

<p>Define megalocornea?</p>

A

<p>Horz diameter of cornea > 12 mm in newborns and > 13 mm in adults</p>

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93
Q

<p>What diseases are associated with megalocornea?</p>

A

<p>Marfan's, Alport's, Down, dwarfism, craniosynostosis and facial hemiatrophy</p>

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94
Q

<p>What are the complications of megalocornea?</p>

A

<p>Ectopia lentis, glaucoma, cataract-PSC</p>

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95
Q

<p>What are the findings of megalocornea?</p>

A

<p>large cornea, zonular dehiscence, phacodenesis, hypoplastic iris, ectopic pupil</p>

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96
Q

<p>What are the characteristics of megalocornea?</p>

A

<p>X-linked, b/l, 90% males, some AR, non progressive</p>

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97
Q

<p>Define microcornea?</p>

A

<p>Corneal diameter < 9 mm in newborns and < 10 mm in adults. AD or sporadic</p>

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98
Q

<p>What diseases are associated with microcornea?</p>

A

<p>Dwarfism, Ehlers-Danlos sx</p>

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99
Q

<p>What are the findings of microcornea?</p>

A

<p>small cornea, hyperopia, PHPV, microphakia, ACG or POAG</p>

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100
Q

<p>Define posterior keratoconus?</p>

A

<p>Discrete posterior corneal indentation with stromal haze and thinning</p>

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101
Q

<p>What are the characteristics of posterior keratoconus?</p>

A

<p>F>M, non progressive, usually central and unilateral, anterior corneal surface is normal, causes irregular astigmatism, VA is good.</p>

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102
Q

<p>What are the characteristics of anterior segment dysgenesis?</p>

A

<p>B/l, congenital, hereditary d/o affecting anterior segment structures</p>

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103
Q

<p>What are the findings of Axenfeld's anomaly?</p>

A

<p>Posterior embryotoxon, anteriorly displaced Schwalbe's line, 50% develop glaucoma, AD.</p>

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104
Q

<p>What are the findings of Alagille's syndrome?</p>

A

<p>Axenfeld's plus pigmentary retinopathy, corectopia, esotropia, biliary hypoplasia. ERG and EOG are abnl</p>

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105
Q

<p>What are the findings of Rieger's anomaly?</p>

A

<p>Axenfeld's plus iris hypoplasia with holes, glaucoma</p>

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106
Q

<p>What are the findings of Rieger's syndrome?</p>

A

<p>Reiger's anomaly plus MR and systemic abnl</p>

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107
Q

<p>What are the findings of Peter's anomaly?</p>

A

<p>Central corneal leukoma, iris adhesions, phacodenesis, 50% develop glaucoma. Usually sparadic, b/l (80%), AR or AD</p>

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108
Q

<p>What is the mnemonic for anterior segment dysgenesis?</p>

A

<p>STUMPED. (Sclerocornea, trauma, ulcer, metabolic dz, Peter's, Edema(CHED), Dermoids</p>

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109
Q

<p>What are the ocular findings of syphilis?</p>

A

<p>Interstitial keratitis, ectopia lentis, Argyll Roberson pupil, optic atrophy, salt and pepper retinopathy</p>

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110
Q

<p>What is Hutchinson's triad?</p>

A

<p>Interstitial keratitis, hutchinson's teeth and deafness</p>

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111
Q

<p>What is the most common location of coloboma?</p>

A

<p>Inferonasal</p>

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112
Q

<p>What ocular conditions are associated with Corectopia?</p>

A

<p>Ectopia lentis et pupillae, Axenfeld's Reiger syndrome, ICE, uveitis or trauma</p>

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113
Q

<p>What ocular conditions are associated with Dyscoria?</p>

A

<p>Posterior synechiae, Axenfeld Reiger syndrome, ectopia lentis et pupillae</p>

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114
Q

<p>What forms the persistent pupillary membrane?</p>

A

<p>Remnants of anterior tunica vasculosa lentisType I: iris to iris bridgeType II: iris to lens, may have associated anterior polar cataract</p>

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115
Q

<p>What are Brushfield's spots? Where are they usually seen?</p>

A

<p>Focal areas of iris stromal hyperplasia surrounded by relative hypoplasia. 85% in down syndrome.</p>

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116
Q

<p>What are lisch nodules and which disease are they associated with?</p>

A

<p>Neural crest hamartomas, associated with NF 1</p>

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117
Q

<p>Name the disease with the following pathology? Diffuse non necrotizing proliferation of histiocytes with scattered touton giant cells</p>

A

<p>Juvenile xanthogranuloma (JXG)</p>

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118
Q

<p>What is mittendorf's dot?</p>

A

<p>Small white opacity on posterior lens capsule. Remnant of the posterior tunica vasculosa lentis</p>

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119
Q

<p>What is microphakia associated with?</p>

A

<p>Lowe's syndrome</p>

120
Q

<p>What diseases are associated with microspherophakia?</p>

A

<p>Weil-Marchesani, Lowe's, and Alport's syndromes, congenital rubella, Peter's.</p>

121
Q

<p>What is the treatment for microspherophakia?</p>

A

<p>Cyclopegia (to tighten zonules, flatten lens and pull it posteriorly)</p>

122
Q

<p>What diseases are associated with bilateral congenital cataracts?</p>

A

<p>Usually AD, DM, galactosemia, Lowe's.</p>

123
Q

<p>What are the complications of congenital cataracts?</p>

A

<p>Irreversible nystagmus. Need treatment by age 3 months</p>

124
Q

<p>What causes unilateral cataracts?</p>

A

<p>PHPV, anterior polar or posterior lenticonus. Requires treatment by 6-8 weeks of life.</p>

125
Q

<p>What are sutural cataracts?</p>

A

<p>AR, bilateral opacities of Y sutures. Occurs during development of fetal lens nucleus.</p>

126
Q

<p>What are crystalline cataracts?</p>

A

<p>B/l, refractile rhomboid crystals (containing tyrosine and cysteine) radiating outward from the center of the lens into the juvenile nucleus</p>

127
Q

<p>What are coronary cataracts? what are they associated with?</p>

A

<p>Small punctate bluish opacities, wreath like cortical opacities encircling the nucleus in radial fashion. Associated with down syndrome</p>

128
Q

<p>What is the etiology of bilateral cataracts?</p>

A

<p>30% Hereditary, 30% systemic syndromes, 30% idiopathic</p>

129
Q

<p>What is the genetic predisposition of bilateral cataracts?</p>

A

<p>AD, AR X-linked</p>

130
Q

<p>What chromosomal abnormalities are associated with congenital cataracts?</p>

A

<p>TRisomy 13 (Patau sx), Trisomy 18, Trisomy 21 (Downs), Turner's syndrome, Cri-du-chat sx</p>

131
Q

<p>Name the enzyme deficiency that leads to galactosemia?</p>

A

<p>Galactose-1-P-uridyl transferase (most common)GalactokinaseUridine diphosphate (UDP) galactose-4-epimerase </p>

132
Q

<p>What is the triad of Alport's syndrome?</p>

A

<p>Anterior lenticonus, deafness, hemorrhagic nephropathy /renal failure. (XR)</p>

133
Q

<p>Name the etiology depending on the type of cataracts:1. Oil droplet cataract2. Cornea verticillata w/spoke like cataracts3. Punctate iridescent opacities in anterior and posterior cortex4. Snow flake or coronary cataracts5. Posterior spoke like opacity cataracts6. Pearly white nuclear opacities</p>

A

<p>1. Galactosemia2. Fabry's disease3. Hypocalcemia4. Down syndrome5. Hurler's6. Rubella cataracts</p>

134
Q

<p>What is the etiology of unilateral cataracts?</p>

A

<p>1. Idiopathic (80%)2. Ocular abnormalities (10%): Posterior lenticonus, PHPV, tumors as (RB, medulloepithelioma), anterior segment dysgenesis3. Trauma (10%)4. Intrauterine infections: Rubella</p>

135
Q

<p>What is the ddx for leukocoria?</p>

A

<p>Cataract, RB, Toxoplasmosis, toxocariasis, RD, ROP, PHPV, Coats, coloboma, Norrie's, myelinated nerve fibers, retinal dysplasia, retinoschisis, medulloepithelioma</p>

136
Q

<p>What is the ddx of congenital cataracts and glaucoma?</p>

A

<p>Lowe's, Hallermann-Streiff syndrome and rubella</p>

137
Q

<p>Which trimester is critical for rubella infection for worse prognosis?</p>

A

<p>Maternal infection in the 1st trimester</p>

138
Q

<p>What are the findings of neonate from maternal rubella infection?</p>

A

<p>B/l cataracts (white pearly nuclear opacities with retention virus in the lens nuclei which can disseminate into persistent AC inflammation following cataract sx), salt and pepper fundus, (+/- glaucoma), microphthalmos, necrotizing iridocyclitis and corneal clouding.Other systemic findings: PDA, deafness, and MR</p>

139
Q

<p>Define congenital, infantile and juvenile glaucoma?</p>

A

<p>Congenital: < 3 months of age Infantile: 3 months to 3 years of ageJuvenile: 3 years to 35 years of age</p>

140
Q

<p>What is the triad of congenital glaucoma?</p>

A

<p>Epiphora, photophobia, and blepharospasm</p>

141
Q

<p>What are the findings of congenital glaucoma?</p>

A

<p>IOP > 21, C/D >0.3, (cupping is reversible), buphthalmos (corneal diameter>13 mm, Haab's striae (circumferential or horizontal DM), corneal edema, scarring, astigmatism and myopia</p>

142
Q

<p>What are the causes of oblique or vertical striae?</p>

A

<p>trauma or forceps delivery</p>

143
Q

<p>Name the syndromes associated with congenital glaucoma?</p>

A

<p>Lowe's, Sturge-weber (50% if nevus flammeus involves upper eyelid), NF (25% if plexiform neurofibroma involves UL), congenital rubella, marfan's, weil-marchesani, stickler's, Hurler's and hunter</p>

144
Q

<p>What is the best time to measure IOP in a patient under anesthesia?</p>

A

<p>As soon as anesthesia was administered</p>

145
Q

<p>Name the anesthetic agetns that raise the IOP?</p>

A

<p>Ketamine and succynylcholine</p>

146
Q

<p>What are the treatment options for congenital glaucoma?</p>

A

<p>Medical therapy is a temporizing measure. Surgery is definitive treatment. Age< 18 months, goniotomyAge> 18 months, trabeculectomyIF either of them fails escalate the therapy to trabeculectomy with mitomycin C</p>

147
Q

<p>What is the most common cause of anterior uveitis in children?</p>

A

<p>RF-, ANA+, pauciarticular (<5 joints) arthritis in girls < 16 years of age</p>

148
Q

<p>What is the ddx of posterior uveitis?</p>

A

<p>Toxoplasmosis, toxocariasis, POHS, HSV, syphilis, SO, masquerade sx (RB,JXG, leukemia, lymphoma, melanoma, IOFB, RRD, RP, MS)</p>

149
Q

<p>What is the most common infectious cause of posterior uveitis?</p>

A

<p>Toxoplasmosis (98% congenital)Tachyzoite form is responsible for inflammation</p>

150
Q

<p>What are the findings of congenital toxo from first trimester infection?</p>

A

<p>neonatal convulsions, intracerebral calcifications, retinitis</p>

151
Q

<p>What are the findings of congenital toxo from second trimester infection?</p>

A

<p>Retinitis</p>

152
Q

<p>What are the ocular findings with congenital toxo?</p>

A

<p>INACTIVE chorioretinal scar in posterior pole, often in macula, ACTIVE white fluffy lesion (headlight in fog) adjacent to the scar.</p>

153
Q

<p>What is the treatment regimen for toxoplasmosis?</p>

A

<p>4 drug regimen:Abx: Clindamycin SulfadiazinePyrimethamine with folinic acidOR bactrim. Steroids after 2-4 days of starting abx. (NEVER be given w/o abx)</p>

154
Q

<p>What causes ocular toxocariasis?</p>

A

<p>Secondary infection from larval form of dog roundworm, toxocara canis. Acquired by ingestion of soil.</p>

155
Q

<p>How to diagnosis toxocariasis?</p>

A

<p>AC tap for eosinophils, ELISA and stool for ova/parasites</p>

156
Q

<p>What is the treatment modality for toxocariasis?</p>

A

<p>Topical steroids, cyclopegia for uveitis, PPV and surgical repair of RD</p>

157
Q

<p>What is the cause of PHPV or PFV?</p>

A

<p>Due to incomplete regression of tunica vasculosa lentis and primary vitreous</p>

158
Q

<p>Name the ocular abnormality?Microphthalmia, retrolental plaque, elongated ciliary processes, shallow AC, NVI, cataract, angle closure glaucoma, VH or RD</p>

A

<p>Persistent fetal vasculature PFV</p>

159
Q

<p>Name the enzyme deficiencies?Taysach'sSandhoff'sGM1 gnagliosidosesHurler'sHunter'sFabry'sMetachromic leukodystrophyKrabbe's diseaseGaucher's diseaseNiemann pick diseaseCystinosisGlaactosemiaRefsum'sAdreno leukodystrophyHomocystinuria</p>

A

<p>Taysach's - Hexosaminidase A - ARSandhoff's - Hexosaminidase B- ARGM1 gangliosidoses - (b-galactosidase A,B and C) - ARHurler's - (a-iduronidase)- ARHunter's- Iduronate sulfatase - XRFabry's - (a-galactosidase) - XRMetachromic leukodystrophy - Arylsulfatase (AR)Krabbe's disease - Galactocerebrosidase (AR)Gaucher's disease - (b-galactosidase) - ARNiemann pick disease - Sphingomyelinase - ARCystinosis - Defective transport of cysteine - ARGlaactosemia - (Gal-1-UDP transferase) - ARRefsum's - Phytanic acid oxidase- (AR)Adreno leukodystrophy - Peroxismal d/o (XR, AR)Homocystinuria - Cystathionine synthase - AR</p>

160
Q

<p>What are the risk factors for ROP?</p>

A

<p>Low birth weight (< 1.5 Kg), prematurity and coexisting illness</p>

161
Q

<p>By what gestational age retina finishes vascularization?</p>

A

<p>36 weeks for nasal retina and 40 weeks for temporal retina</p>

162
Q

<p>What is the zone classification of ROP?</p>

A

<p>Zone 1: centered on ON with radius of (2 x the distance from ON to fovea)Zone 2: centered on ON with radius of (4x the distance from ON to fovea)Zone 3: Centered on ON to the aura temporally with the remaining crescent anterior to zone 2</p>

163
Q

<p>What are the stages of ROP?</p>

A

<p>Stage 1: Demarcation lie between vascular and avascular retinaStage 2: Elevated ridgeStage 3: Ridge with tufts of extraretinal blood vessels (popcorn)Stage 4: subtotal RD (Extrafoveal-4A, Foveal-4B)Stage 5: Total RD with funnel</p>

164
Q

<p>What are the risk factors for ROP?</p>

A

<p>Low birth weight (< 1.5 Kg), prematurity (<30 weeks of gestation) and coexisting illness</p>

165
Q

<p>What are the indications for laser to peripheral retina in ROP?</p>

A

<p>Any zone with plus diseaseZone 1, Stage 3 regardless of plus disease</p>

166
Q

<p>What are the conclusions from ETROP study?</p>

A

<p>Early laser therapy for any zone with plus diseaseZone 1, Stage 3 regardless of plus disease</p>

167
Q

<p>What are the conclusions from Cryo-ROP study?</p>

A

<p>Cryo therapy preserves VA in eyes with threshold disease. Entire avascular retina in zone 2 w/o ridge</p>

168
Q

<p>Define plus disease in ROP?</p>

A

<p>Engorged tortuous vessels around the disc, vitreous haze, iris vascular congestion, progressive vascular incompetence- poor prognostic sign</p>

169
Q

<p>What are the conclusions from ETROP study?</p>

A

<p>Any zone with plus diseaseZone 1, Stage 3 regardless of plus disease</p>

170
Q

<p>What are the conclusions from Cryo-ROP study?</p>

A

<p>PRP to the entire avascular retina in zone 2 w/o ridge</p>

171
Q

<p>What are the conclusions of BEAT-ROP study?</p>

A

<p>Avastin of 0.025 ml in zone 1, stage 3 disease</p>

172
Q

<p>What is the genetic predisposition of FEVR?</p>

A

<p>AD, AR(most severe form), x-linked</p>

173
Q

<p>What are the stages of FEVR?</p>

A

<p>Stage 1: Avascular periphery Stage 2: NV (2A: w/o exudates, 2B: w/ exudates)Stage 3: Extramacular RD (3A: w/o exudates, 3B: w/ exudates)Stage 4: Macular involving RD (4A: w/o exudates, 4B: w/ exudates)Stage 5: TRD</p>

174
Q

<p>What are the findings of Leber's miliary aneurysms?</p>

A

<p>Also called Coats disease.Leucokoria, strabismus, telengiectatic blood vessels, non calcified yellow lesions, exudative RD, MAs, capillary non perfusion in the periphery</p>

175
Q

<p>Name the disease from the below FA findings?FA: blood fluid levels, saccular aneurysms (light bulb aneurysms) of retinal arterioles and venules.</p>

A

<p>Coats disease</p>

176
Q

<p>What is the treatment for Coats disease?</p>

A

<p>Cryo or Laser to stop the leaking blood vessels</p>

177
Q

<p>Name the disease?XL recessive, b/l leukocoria, retinal dysplasia, peripheral NV, hemorrhagic RD and retinal necrosis, deafness and MR</p>

A

<p>Norrie disease</p>

178
Q

<p>What is the most common hereditary macular dystrophy?</p>

A

<p>StargardtSecond most common: BEST disease</p>

179
Q

<p>What is the genetic makeup of Stargardt?</p>

A

<p>Chr 1, ABCA4, AR, less commonly AD</p>

180
Q

<p>What are the most common fundus findings of Stargardts?</p>

A

<p>B/l pisciform lesions, yellow-white flecks, bulls eye maculopathy, beaten metal appearance fundus, salt and pepper pigmentary changes</p>

181
Q

<p>What is the pathognomonic sign of stargardt on FA?</p>

A

<p>Silent choroid (due to accumulation of lipofuscin in RPE)</p>

182
Q

<p>What is the genetic make up of Best disease?</p>

A

<p>Chr 11, VMD (BEST 1) encodes bestrophin 1 located in basolateral aspect of RPE </p>

183
Q

<p>What are the stages of BEST or vitelliform disease?</p>

A

<p>S1: Previtelliform: Submacular yellow dotS2: Vitelliform stage: Yellow-orange egg yolk appearanceS3: Pseudohypopyon stage: Layering of lipofusin, RPE atrophyS4: Scrambled egg: irregular subretinal spotsS5: Round chorioretinal atrophy: atrophic scarS6: CNV stage</p>

184
Q

<p>What is the age of onset of Stargardt and Best diseases?</p>

A

<p>Stargardt: Progression with onset in the first 2 decadesBest: Progression with onset in the first decade</p>

185
Q

<p>Name the disease.Disease onset in first decade with drusen progressing to chorioretinal atrophy with staphyloma of macula. Progression to CNV eventually</p>

A

<p>North Carolina macular dystrophy</p>

186
Q

<p>What are the associations of RP?</p>

A

<p>Keratoconus, CME, Coats disease, optic disc drusen, myopia</p>

187
Q

<p>What is the triad of RP?</p>

A

<p>Waxy pallor to the ON, bone spicules, vascular attenuation</p>

188
Q

<p>What is the ddx of nyctalopia?</p>

A

<p>Uncorrected myopia, Vitamin A deficiency, Zinc def, choroideremia, PRP, CSNB, gyrate atrophy and Goldman-Favre disease</p>

189
Q

<p>What is the pathology of "bone spicules"?</p>

A

<p>RPE cells invade retina and surround retinal vessels</p>

190
Q

<p>Name the syndrome?Kidney failure, anterior lenticonus with anterior polar cataract, deafness.</p>

A

<p>Alport's syndrome</p>

191
Q

<p>Name the phenomenon?Golden-brown fundus (yellow/green sheen) in light adapted state, normal colored fundus in dark adapted state </p>

A

<p>Mizou-Nakamura phenomenon</p>

192
Q

<p>What are the ERG findings of Oguchi's disease?</p>

A

<p>Absent B-wave, only scotopic a-waveOguchi's is mapped to chr 2</p>

193
Q

<p>Name the disease?Onset during late childhood with nyctalopia, photophobia, constricted VF in affected males</p>

A

<p>Choroideremia (XL-recessive). Mutation in CHM gene that encodes for Geranylgeranyl transferase Rab escort protein</p>

194
Q

<p>Name the disease with deficiency of Ornithine aminotransferase and elevated ornithine levels and low lysine levels?</p>

A

<p>Gyrate atrophy (mapped to chr 10), AR disease with mutation in gene OAT.</p>

195
Q

<p>What are the fundus findings of Gyrate atrophy?</p>

A

<p>Scalloped areas of absent choriocapillaries with abrupt transition between normal and atrophic RPE. Myopia (90%), cataracts, vitreous degeneration and CME</p>

196
Q

<p>What is the treatment for gyrate atrophy?</p>

A

<p>Restrict Arginine, supplement Vitamin B6 (Pyridoxine).</p>

197
Q

<p>What is sectoral RP? What is the most common quadrant affected?</p>

A

<p>B/l RP retinal changes limited to 1 or 2 quadrants with most commonly involved inferonasal quadrant. Abnl ERG, VF but good prognosis</p>

198
Q

<p>What is tapetoretinal degeneration?</p>

A

<p>Process that involves the outer half of the retina (photoreceptor/RPE). RPE = tapeum nigran (black carpet)</p>

199
Q

<p>What are Leber Congenital Amaurosis ?</p>

A

<p>Early onset retinal dystrophies are collectively termed as LCA</p>

200
Q

<p>Name the disease?Reduced vision from birth, nystagmus, round, black pigmented flecks in the fundus, oculodigital reflex. possible cataracts and keratoconus</p>

A

<p>LCA</p>

201
Q

<p>What is oculodigital reflex?</p>

A

<p>The tendency to rub or poke into the eyes. Usually seen in LCA diseases</p>

202
Q

<p>What is the LCA mutation that has been treated with gene therapy?</p>

A

<p>RPE65. Treated with gene therapy by using AAV</p>

203
Q

<p>What is the ddx of Bull's-Eye maculopathy?</p>

A

<p>Stargardt dz, cone and cone-rod dystrophies, Hydroxychloroquine or chleorquine, ARMD, chronic macular hole, central areolar choroidal atrophy, Olivopontocerebellar atrophy, ceroid lipofuscinosis</p>

204
Q

<p>Name the disease:Nyctalopia, increased sensitivity to blue light, deep nummular pigment deposition in retina, optically empty vitreous. Related to Goldmann-Favre syndrome.</p>

A

<p>Enhanced S-cone syndrome.</p>

205
Q

<p>What is the genetic make up of Enhanced S-cone syndrome?</p>

A

<p>AR, mutations in NR2E3</p>

206
Q

<p>Name the syndrome:RP with DEAFNESS, ataxia, MR, rickets</p>

A

<p>Usher syndrome</p>

207
Q

<p>Name the syndrome:RP with DEAFNESS, ANOSMIA, ataxia, ichthyosis, cardiac adnormalities</p>

A

<p>Refsum's disease</p>

208
Q

<p>What is the genetic makeup of Refsum's disease?</p>

A

<p>Deficiency of Phytanic acid oxidase, AR</p>

209
Q

<p>What other ocular syndromes are associated with hearing loss?</p>

A

<p>Cogan's (IK + hearing loss)Sticklers (optically empty vitreous+ hearing loss)Waardenburg-Klein (iris heterochromia+white forelock+hearing loss)Duane's (15% has hearing loss)</p>

210
Q

<p>What is the difference in treatment in stargardt Vs RP?</p>

A

<p>Stargardt: Limit or avoid Vitamin ARP: Supplement Vitamin A</p>

211
Q

<p>Name the syndrome: <strong>Pigmentary retinopathy</strong> with flat ERG, <strong>polydactly</strong>, MR, short stature, obesity</p>

A

<p>Bardet-Biedl syndrome</p>

212
Q

<p>Name the disease:Ptosis, ophthalmoplegia, strabismus, retinal degeneration, cardiac conduction defects, only males are affected</p>

A

<p>CPEO, mitochondrial inheritance, 50% +Fam Hx, </p>

213
Q

<p>Name the gene involved in Juvenile retinoschisis?</p>

A

<p>X-linked recessive, (XLRS1-retinoschisin)</p>

<p><strong>Characteristics:</strong> Males, bilateral, present at birth, progresses rapidlytill age 5 and stable by age 20.</p>

<p>Cleavage at NFL (usually inferotemporal location)</p>

<p></p>

214
Q

<p>What type of scotoma is noted with retinoschisis?</p>

A

<p>Absolute scotoma</p>

215
Q

<p>Name the syndrome?</p>

<p>Optically empty vitreous, Lattice, RD, optic atrophy, high degree of myopia</p>

A

<p>Stickler's syndrome</p>

<p>Genes involved: COL11A1, COL11A2, COL2A1</p>

216
Q

<p>Name the syndrome with charateristics similar to stickler's but <strong>short stature, stubby hands</strong> and feet with <strong>ectopia lentis</strong>?</p>

A

<p>Weill-Marchesani syndrome</p>

217
Q

<p>What is the name of the lethal condition with Albinism and severe clotting disorder?</p>

A

<p>Hermansky-Pudlak syndrome.</p>

<p>Commonly seen in Peurto-ricans</p>

218
Q

<p>What is the name of the lethal condition with Albinism and recurrent infections, pancytopenia and malignancies?</p>

A

<p>Chediak-Higashi syndrome</p>

219
Q

<p>What are the 3 types of cones in the retina?</p>

A

<p>L- long wavelength- Red</p>

<p>M- Medium wavelngth-Green</p>

<p>S-short wavelength-Blue</p>

220
Q

<p>What are the typical abnormal colors in CONGENITAL dyschromatopsia?</p>

<p>What are the typical abnormal colors in ACQUIREDdyschromatopsia?</p>

A

<p>Red-green</p>

<p>Blue-yellow</p>

221
Q

<p>What is the pathology of CHRPE?</p>

A

<p>Densely packed melanocytes with larger melanosomes leading to increased thickness of RPE. RPE atrophy at places lead to lacunae with choroidal show</p>

222
Q

<p>What systemic disease is CHRPE ("bear tracks") associated with?</p>

A

<p>FAP- AD-mapped to chr 5</p>

<p>Gardner's syndrome (variant of FAP) -colonic polyps</p>

<p>Order colonoscopy</p>

223
Q

<p>What is the ddx of CHRPE?</p>

A

<p>Choroidal nevus or melanoma</p>

<p>Metastases</p>

<p>Reactive hyperplasia of RPE</p>

<p>BDUMP</p>

<p>Adenoma of retina</p>

<p>Combined hamartoma</p>

<p>Massive gliosis of retina</p>

<p></p>

<p></p>

224
Q

<p>What is the most common INTRAOCULAR malignancy in children?</p>

A

<p>RB</p>

<p>90% dx by 5 years of age. M=F, mapped to chr 13</p>

<p>94% sporadic, 6% AD</p>

<p>Bilateral cases are familial</p>

225
Q

<p>What is the chance of having a child with RB in the following conditions?</p>

<p>+ fam Hx:</p>

<ul> <li>Parents with 1 affected child</li> <li>Affected parent</li></ul>

<p>- Fam Hx:</p>

<ul> <li>Parents with 1 affected child</li></ul>

<p></p>

A

<p>+ fam Hx:</p>

<ul> <li>Parents with 1 affected child - 40%</li> <li>Affected parent-40%</li></ul>

<p>- Fam Hx:</p>

<ul> <li>Parents with 1 affected child-6%</li></ul>

226
Q

<p>What are the complications of endophytic RB?</p>

A

<p>Arises from inner retina and grows into the vitreous, simulate endophthalmitis with pseudohypopyon</p>

227
Q

<p>What are the complications of exophytic RB?</p>

A

<p>Arises from outer retina and grows toward choroid causing RD, extend through sclera, and simulate Coats disease or truamatic RD</p>

228
Q

<p>What are the poor prognostic signs of RB?</p>

A

<p>Bilateral tumor DOES NOT worsen the prognosis. Itis the status of the tumor in the worse eye.</p>

<p>ON invasion, extrascleral extension, vitreous seeding, uveal invasion.</p>

229
Q

<p>What is trilateral retinoblastoma?</p>

A

<ul> <li>Bilateral RB with pineolablastoma or parasellar neuroblastoma.</li> <li>Occurs in 3% of children in unilateral RB</li> <li>8% of children in bilateral RB</li></ul>

<p>​</p>

230
Q

<p>What histologic markers help with tumor differentiation in RB?</p>

A

<ul> <li>Homer-Wright Rosettes</li> <li>Flexner-Wintersteiner rosette</li> <li>Fleurettes</li> <li>Pseudorosettes</li></ul>

231
Q

<p>What are the diagnostic tools of RB?</p>

A

<ul> <li>Blood: Elevated LDH</li> <li>US: Acoustic solidity with high internal reflectivity</li> <li>FA: early hyperflourescence with late leakage</li> <li>MRI: ON involvement and pineal tumor</li> <li>CT: calcifications(less preferred due to radiation)</li> <li>Metastatic w/u: bone scan, LP, bone marrow bx</li></ul>

232
Q

<p>Name the chromosomes involved in the following phacomatoses?</p>

<ul> <li>NF 1</li> <li>NF2</li> <li>VHL</li> <li>TS</li> <li>Ataxia-Telengiectasia</li> <li>Sturge-Weber</li> <li>Wyburn-Mason</li></ul>

A

<ul> <li>NF 1 -17 - AD</li> <li>NF2 - 22 - AD</li> <li>VHL - 3 - AD</li> <li>TS - 9 - AD</li> <li>Ataxia-Telengiectasia - 11 - AR</li> <li>Sturge-Weber - None - sporadic</li> <li>Wyburn-Mason - none - sporadic</li></ul>

233
Q

<p>What is the criteria for dx of NF1?</p>

A

<ul> <li>6 or more cafe-au-lait spots</li> <li>2 or more neurofibromas</li> <li>or 1 plexiform neurofibroma</li> <li>Freckling of intertriginous areas</li> <li><strong>Optic nerve glioma</strong></li> <li><strong>2 or more Lisch nodules</strong></li> <li>First degree relative with NF1</li></ul>

234
Q

<p>What are the criteria for dx of NF2?</p>

A

<ul> <li>B/l cerebellar-pontine angle tumors (<strong>acoustic neuromas</strong>, hearing loss, ataxia, HA)</li> <li>First degree relative with NF2</li> <li>Fam hx of either unilateral acoustic neuroma or 2 of the following meningioma, schwannoma, neurofibroma, glioma, PSC</li> <li>May have pheochromocytoma</li> <li><strong>NO lisch nodules</strong></li></ul>

235
Q

<p>What is the mechansim of glaucoma in sturge-weber syndrome?</p>

A

<p>NV, increased episcelral venous pressure, and immature angle structures</p>

236
Q

<p>What is the triad of Tubersous sclerosis?</p>

A

<p>Adenoma sebaceum, MR, seizures (epilepsy)</p>

237
Q

<p>What are the ocular findings of tuberous sclerosis?</p>

A

<ul> <li>Astrocytic hamartoma of the retina (flat or mulberry shaped in the posterior pole)</li> <li>astrocytic hamartoma of the optic nerve</li></ul>

238
Q

<p>What are the ocular findings of Ataxia-telengiectasia?</p>

A

<p><strong>Prominent conjunctival vessels,</strong> impaired convergence, nystagmus and <strong>oculomotor apraxia</strong></p>

239
Q

<p>What are the ocular findings of incontinentia pigmenti?</p>

A

<p>X-linked dominant (only in females, lethal in males0</p>

<p>Proliferative retinal vasculopathy (resembles ROP), may have RD and retrolental membrane</p>

240
Q

<p>What are the ocular findings of optic nerve hypopliasia and complications?</p>

A

<ul> <li>Double ring sign, +RAPD</li> <li>strabismus, nystagus, amblyopia, , VF defects</li></ul>

241
Q

<p>What are the ocular findings of De-Morsier's syndrome?</p>

A

<ul> <li>B/l ON hypoplasia</li> <li>Septum pellucidum abnormality</li> <li>Pituitary and hypothalmus deficiency</li> <li>Agenesis of corpus callosum</li> <li>May have chaismal abnormalities with VF defects</li></ul>

242
Q

<p>What is the most important work up in De-Morsier syndrome?</p>

A

<p>Endocrine consult</p>

243
Q

<p>What is the most common location for optic pit?</p>

A

<p>Inferotemporal area</p>

244
Q

<p>What is the most common complication of optic pit?</p>

A

<p>Serous RD extending from the pit</p>

245
Q

<p>What are the associations of optic disc drusen?</p>

A

<ul> <li>Angioid streaks</li> <li>RP</li> <li>Alagille's syndrome</li></ul>

246
Q

<p>What is the pathology of optic disc drusen?</p>

A

<p>Clacified hyaline bodies</p>

<p>Stain + for Ca2+, aminoacids, hemosiderin and mucopolysaccharides</p>

<p>Stain <strong>NEG</strong>for amyloids</p>

247
Q

<p>What is the most common mutation in Leber's hereditary optic neuropathy (LHON)?</p>

A

<p>Mitochondrial mutationin motochondrial gene NADH.</p>

<p>Most common 11778</p>

<p>Greatest penetrance 3460</p>

<p><strong>Best prognosis 14484</strong></p>

<p><strong>M:F 9:1,</strong></p>

<p></p>

248
Q

<p>What is the age of onset of LHON?</p>

A

<p>15 and 30 with subacute sequential bilateral vision loss (=20/200).</p>

<p>Triggered by tobacco or alcohol</p>

249
Q

<p>What are the most commonly seen mitochondrial diseases?</p>

A

<p>Maternal inherited diabetes and deafness (MIDD)</p>

<p>LHON</p>

<p>Kearns-sayre syndrome</p>

250
Q

<p>What are the ocular findings of mitochondrial diseases?</p>

A

<p>CPEO, ptosis, pigmentary retinopathy, retrochiasmal vision loss, optic atrophy</p>

251
Q

<p>What is the pathology of mitochondrial diseases?</p>

A

<p>"ragged red" fibers on muscle bx</p>

252
Q

<p>What is the origin of insertion of oblique muscles? rectus muscles and levator papebrae?</p>

A

<ul> <li>All rectusmuscles: Annulus of zinn</li> <li>SO and LP: Orbital apex above the annulus of zinn</li> <li>IO: periosteum of maxillary bone</li></ul>

253
Q

<p>Damage to which muscle during strabismus surgery results in mydriasis?</p>

A

<p>Inferior oblique.</p>

<p>Inferior division of CN III upto the IO carries parasympathetic supply to iris constrictor</p>

254
Q

<p>Describe the course of visual development in children?</p>

A

<ul> <li>At birth: blinking response to light</li> <li>7 days: vestibulo-ocular response</li> <li>2 months: Fixation well developed</li> <li>6 months: VER acuity at adult level</li> <li>2 years: snellen at adult level</li> <li>7 years: stereoacuity at adult level</li></ul>

255
Q

<p>What is the purpose of worth 4 dot test?</p>

A

<p>To determine bonicularity and to determine the size and location of suppression scotoma in strabismus patients.</p>

256
Q

<p>What is the purpose of 4D baseout prism test?</p>

A

<p>To detect small suppression scotomas</p>

257
Q

<p>Define amblyopia?</p>

A

<p>Unilateral or bilateral reduction in VA that cannot be attributed to any structural abnormality of the eye or visual system.</p>

258
Q

<p>What are the types of amblyopia?</p>

A

<p>Strabismic</p>

<p>Refractive</p>

<p>Deprivational amblyopia</p>

259
Q

<p>How much of refractive errorcause refractive amblyopia?</p>

A

<p>Ametropia:</p>

<ul> <li>5D of hyperopia</li> <li>-8D of myopia</li> <li>2.5D of astigmatism</li></ul>

<p>Anisometropia:</p>

<ul> <li>1D of Hyperopia</li> <li>-3D of myopia</li> <li>1.5D of astigmatism</li></ul>

260
Q

<p>What is the Hering's law?</p>

A

<p>Equal and simultaneous innervation to synergistics muscles</p>

261
Q

<p>What is primary deviation in paralytic strabimus?</p>

<p>What is secondarydeviation in paralytic strabimus?</p>

A

<p>Deviation measured with normal fixating eye</p>

<p>Deviation measured with paretic eye fixation. It is always larger than primary deviation.</p>

262
Q

<p>What is Sherrington's law?</p>

A

<p>law of <strong>SAME</strong>eye muscles. Innervation to the ipsilateral antagonist decreases while innervation to the agonist inreases.</p>

<p>Eg: lateral rectus and medial rectus in the same eye</p>

<p></p>

263
Q

<p>What is Hering's law?</p>

A

<p>Law of BOTH eyes: Equal and simultaneous innervation tosynergistic muscles.</p>

264
Q

<p>Define optical axis and visual axis?</p>

A

<p>Optical axis: A line drawn through the center of cornea, lens and fovea</p>

<p>Visual axis: A line drawn through the fixating point and fovea</p>

<p>Pupillary axis: A line drawn through the center of the pupil to the fovea</p>

<p></p>

265
Q

<p>Define angle alpha?</p>

<p>Define angle Kappa?</p>

A

<p>Angle between visual axis and optical axis. Important in pseudophakic IOL centration.</p>

<p>Angle between visual axis and pupillary axis. Important in corneal refractive procedures.</p>

266
Q

<p>What are the characteristics of positive angle kappa and negative angle kappa?</p>

A

<p>Positive angle kappa:</p>

<ul> <li>Temporal postion of fovea</li> <li>Light reflex appears nasal</li> <li>pseudoXT or mask ET</li></ul>

<p>Negative angle kappa:</p>

<ul> <li>Nasalpostion of fovea</li> <li>Light reflex appears temporal</li> <li>pseudoET or mask XT</li></ul>

267
Q

<p>What tests are used for DETECTION of deviations?</p>

<p>What tests are used for MEASURMENTof deviations?</p>

A

<p>Detection:</p>

<ul> <li>Corneal light reflexes</li> <li>Monocular cover-uncover tests</li></ul>

<p>Measurements:</p>

<ul> <li>Modified Krimsky's</li> <li>Hirschberg's</li> <li>Simultaneous prism cover-uncover tests</li> <li>Alternate cover testing</li></ul>

268
Q

<p>What does double maddox rod measures?</p>

A

<p>Torsions</p>

269
Q

<p>What are the 3 steps in Parks-Beilchowsky three step?</p>

A

<p>1. Which eye has hyperdeviation</p>

<p>2. Is hyperdeviation greater in right or left gaze?</p>

<p>3. Is hyperdeviation worse with right or left head tilt?</p>

270
Q

<p>What are the characteristics of congenital nystagmus?</p>

A

<ul> <li>Horizontal and long-standing</li> <li><strong>Null point is present</strong></li> <li><strong>Dampened by convergence</strong></li> <li><strong>Absent while sleeping</strong></li> <li>OKN reversal in 60%</li> <li>No oscillopsia</li> <li>May develop a head posture</li></ul>

271
Q

<p>What diseases are associated with congenital nystagmus?</p>

A

<p>Albinism, aniridia, LCA, ON hypoplasia, congenital cataracts, achromatopsia</p>

272
Q

<p>How to distinguish latent vs congenital nystagmus?</p>

A

<p>Latent nystagmus:</p>

<ul> <li>worsens towards the viewing eye with covering one eye</li> <li>slow phase velocity decreases</li></ul>

<p>Congenital nystagmus:</p>

<ul> <li>Reversal of OKN drum</li> <li>dampens with convergence</li> <li>slow-phase velocityworsens</li></ul>

273
Q

<p>What are the characteristics of latent nystagmus?</p>

A

<ul> <li>Jerk nystagmus towards the viewing eye on monocular viewing</li> <li>Normal OKN reponse</li> <li>Decreasing slow phase velocity exponentially</li> <li>Associated with infantile esotropia and DVD</li> <li>Normal VA when both eyes are open</li></ul>

274
Q

<p>What is Alexander's rule of nystagmus?</p>

A

<p>Nystagmus intensity increases when looking toward fast phase and decreases when looking toward slow phase (ie, ADduction nulls, therefore no head posture)</p>

275
Q

<p>What is sensory nystagmus? What diseases are associated with sensory nystagmus?</p>

A

<p>A form of pendular nystagmus due to vision loss, morecommon than congenital nystagmus</p>

<p>Associated diseases:</p>

<ul> <li>Aniridia, albinism, CSNB, ONcoloboma, ON hypoplasia, cataracts, LCA, b/l macular coloboma</li></ul>

276
Q

<p>What is the triad of spasmus nutans?</p>

A

<ul> <li>Simmering nystagmus</li> <li>Head bobbing</li> <li>Torticolis</li></ul>

277
Q

<p>What needs to be ruled out with spasmus nutans?</p>

A

<p>Chiasmal glioma.</p>

<p>Spasmus nutans usual onset is during the first year of life with spontaneous resolution by age 3 years</p>

278
Q

<p>What are the characteristics of congenital esotropia?</p>

A

<ul> <li>M=F, present by 6 months of age</li> <li>+fam hx of strabismus</li> <li>Associated with cerebral palsy or hydrocephalus</li> <li>deviation >/= 30PD</li></ul>

<p>Treatment:</p>

<ul> <li>Treat amblyopia first</li> <li>Early sx: bilateral MR recession, or unilateral LR resection and MR recession</li></ul>

279
Q

<p>Define convergence and divergence insufficiency?</p>

A

<p>Convergence insufficiency: XT greater at near than distance</p>

<p>Diveregence insufficiency: ET greater at distance than near</p>

280
Q

<p>How to distinguish congenital superior oblique palsy from acquired SO palsy?</p>

A

<p>Congenital:</p>

<ul> <li>Due to long or floppy or absent SO tendon</li> <li>Fuse large amplitudes (15PD or more)</li> <li>Head tilt</li> <li>Facial asymmetry</li></ul>

<p>Acquired:</p>

<ul> <li>Due to trauma</li></ul>

281
Q

<p>What muscle is involved in Brown's syndrome? and what causes Brown's?</p>

A

<p>SO tendon sheath syndrome. Inability to ELEVATE in ADDuction.</p>

<p>Congenital or acquired</p>

<p>Causes: Trauma, inflammation, iatrogenic (following SO tuck, glaucoma drainage implant, scleral buckle)</p>

282
Q

<p>What measurement is clinically significant in A pattern stabismus?</p>

A

<p>>10 PD of divergence from upgaze to downgaze</p>

283
Q

<p>What measurement is clinically significant in Vpattern stabismus?</p>

A

<p>>15PD of covergence from upgaze to downgaze</p>

284
Q

<p>What is the mnemonic for A and V pattern strabismus?</p>

A

<p>MALE (<strong>M</strong>edial rectus to the <strong>A</strong>pex and<strong> L</strong>ateral rectus to the <strong>E</strong>mpty space</p>

285
Q

<p>What are the findings of Duane's retraction syndrome?</p>

A

<ul> <li>Cocontraction of MR and LRmuscles causing retraction of the globe with secondary narrowing of palpebral fissure</li> <li>Vertical deviations with charactersitics of upshoot and downshoot</li> <li>Head turn is common</li></ul>

286
Q

<p>What is the etiology of Duane's syndrome?</p>

A

<p>Abnormal innervation of LR by a branch of CN III.</p>

<p>Possible mechanisms:</p>

<ul> <li>Hypoplasia of CN6 nucleus</li> <li>Fibrosis of LR</li> <li>Midbrain pathology</li></ul>

287
Q

<p>What are the types of Duanes?</p>

A

<p>Type <strong>1</strong>:</p>

<ul> <li>Limitation of ab<strong>D</strong>uction, retraction of globe, narrowing of palpebral fissure</li></ul>

<p>Type <strong>2</strong>:</p>

<ul> <li>Limitation of A<strong>DD</strong>uction, appears exotropic</li></ul>

<p>Type <strong>3</strong>:</p>

<ul> <li>Limitation of ab<strong>D</strong>uction and a<strong>DD</strong>uction</li></ul>

<p></p>

288
Q

<p>What are the treatment modalities for Duane's?</p>

A

<p><strong>Treat</strong> for amblyopia and refrative error first, then surgery</p>

<p><strong>Indications for sx: </strong></p>

<ul> <li>abnormal head position or</li> <li>deviation in primary gaze</li></ul>

<p><strong>For globe retraction</strong>: simultaneous LR recession</p>

<p><strong>For upshoot or downshoot</strong>: splitting of LR or Faden procedure</p>

<p><strong>AVOID muscle resection</strong>: worsens globe retraction and upshoot and downshoots</p>

289
Q

<p>What cranial nerves are involved in Mobius syndrome?</p>

A

<p>CN6 and 7 palsies. Due to aplasia of involved brain sem nuclei.</p>

290
Q

<p>What are the ocular findings of Mobius syndrome?</p>

A

<p>Esotropia with limitation of ABduction (CN 6 palsy). Exposure keratitis with poor lid closure (Cn 7 palsy)</p>

291
Q

<p>What causes anterior segment ischemia, what are the findings and how to treat?</p>

A

<p>Cause: Sx on 3 or more eye muscles.</p>

<p>Findings: Anterior uveitis and corneal edema</p>

<p>Treat with topical steroids</p>

292
Q

<p>What is oculocardiac reflex?</p>

A

<p>Bradycardia with traction of EOMs, especially MR</p>

293
Q

<p>What medicine causes malignant hyperthermia?</p>

A

<p>Succinylcholine and inhalation agents</p>

<p></p>

294
Q

<p>What are the signs of malignant hyperthermia?</p>

A

<p>Tachycardia, unstable BP, arrhythmias, increased temp, muscle rigidity, cyanosis and dark urine</p>

295
Q

<p>What is the purpose of red-glass test?</p>

A

<p>To check for anomalous retinal correspondence (ARC) and to avoid post-operative diplopia surprises</p>

296
Q

<p>How to perform and interpret red-glass test?</p>

A

<p>1. Measure patient's deviation with a prism</p>

<p>2. Place the red-glass on non deviating eye and ask the patient to focus on the white light</p>

<p><strong>Interpretation:</strong></p>

<ul> <li>If they see "1light" - Suppression</li> <li>If they see "pink" - harmonious ARC</li> <li>If they see "2 lights":- Unharmonious ARC</li></ul>

Mike (6 decks)

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