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68 Cards in this Set
- Front
- Back
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When will epicanthal folds persist?
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When the birth is pre-term or when the mother is an alcoholic
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What is epicanthal inversus?
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A medial skin fold sweeping upward from below
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What diseases are epicanthal folds associated with?
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Telecanthus, Down's, Fetal Alcohol Syndrome, Cri du Chat, Potter Syndrome, Zellweger Syndrome, Congenital Ptosis, Beckwith-Wiedmann Syndrome, De Lange Syndrome, Chromosome Deletion Syndromes, Klinefelter Syndrome, Turner Syndrome, Ehlers-Danlos Syndrome, Marinesco-Sjogren Syndrome, and Rubinstein-Taybi Syndrome
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Are the pupillary reflexes centered or decentered?
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Centered
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What is Telecanthus?
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An abnormally increased distance between the medial canthi of the eyelids
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In what syndrome is there lateral displacement of the punctum relative to the medial canthus?
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Type 1 Waardenburg's Syndrome
(AKA: Dystopia Canthorum) |
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How is Type 1 Waardenburg's Syndrome distinguished from Type 2?
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By the absence of Dystopia Canthorum
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Describe Telecanthus with Associated Abnormalities (TCAA).
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Rare genetic disorder affecting eyes and other body parts.
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What are the symptoms of TCAA?
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Very widely spaced eyes (hypertelorism), urinary tract abnormalities, and abnormalities in the development of the mouth and lips
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Describe Primary Telecanthus
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The inner canthi are far apart, although the outer canthi are normally spaced
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Describe a true ocular hypertelorism
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Both inner and outer canthi are abnormally far apart
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What are the other names for Blepharophimosis Syndrome?
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BPES, BPEI, and BPS
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What are the principal characteristics of BPS?
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Abnormal narrowing of the palpebral fissures with drooping and epicanthus inversus
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What are some disorders associated with BPS?
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Mental Retardation, Ovarian and Uterine Atrophy with Infertility, and Lacrimal Abnormalities
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Type 1 BPS is associated with what?
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Females and Infertility (premature ovarian failure)
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Type 2 BPS occurs in who?
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Both males and females
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What are the clinical signs of BPS?
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Inverted inner canthi fold between upper and lower lid, short palpebral fissures, lateral displacement of inner canthi, low nasal bridge and ptosis
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What is the palpebral fissure dimensions in BPS?
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20-22mm (Normal = 25-30mm)
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What is Epiblepharon?
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A developmental anomaly in which a horizontal fold of skin and underlying orbicularis muscle stretches across the border of the eyelid, pressing the eyelashes inward, against the eyelid
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What is entropion?
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a malposition resulting in the inversion of the yelid margin
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What are some ocular problems associated with Epiblepharon?
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Astigmatism and Keratopathy
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What is a coloboma?
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A notch or full-thickness defect of the eyelid
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What is the most common position of a coloboma?
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At the junction of the medial and middle third of the upper lid
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What are some disorders associated with Colobomas?
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Treacher Collins Syndrome,
Cryptophthalmos, Fraser Syndrome, Manitoba Oculotrichoanal Syndrome, Facial Deformities, Amniotic Band Syndrome, Oculoauricular Dysplasia (Goldenhar Syndrome), Franceschetti Syndrome |
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What is Microblepharon?
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A rare congenital anomaly manifested by a vertical shortening of the eyelids
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How do you characterize a Microblepharon?
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By a vertical shortage of upper and lower eyelid skin, causing nocturnal lagophthalmos, corneal exposure, and cosmetic deformity
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What are the three types of Ablepharon?
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Complete, incomplete, and Symblepharon
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What is the most common type of ablepharon?
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Complete
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Describe the complete variety of ablepharon.
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the eyelids do not form and the eyelid skin grows continuously from the forehead to the cheek, covering the underlying globe
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Describe the incomplete variety of ablepharon.
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Presents with facial skin fusing to the medial aspect of the globe with no eyelid structures in that area. The lateral eyelid is present and normal
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Describe the symblepharon variety of ablepharon.
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A congenital problem that presents with fusion of the upper eyelid skin to the superior portion of the globe
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What are some congenital anomalies associated with ablepharon?
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Mental Retardation,
Nasal Anomalies, Ear Anomalies, Cleft lip and palate, irregular dentition, genitourinary abnormalities, cardiac malformations, maningoencephalocele, abnormal hairline, umbilical hernia, anal atresia, ankyloglossia, laryngeal atresia, and Syndactyly |
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What are some syndromes associated with Cryptophthalmos?
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Fraser's Syndrome,
Cryptophthalmos-Syndactyly Syndrome, Malformative Syndrome with Cryptophthalmos, Cryptophthalmos Syndrome |
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Describe Cryptophthalmos.
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A form of ablepharon; Eyelids fail to form leaving K and conj exposed; Exposed K and Conj become more skin-like; Usually associated with other ocular and systemic developmental anomalies
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What is Pseudocryptophthalmos?
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A total ankyloblepharon where the eyelids form but fail to separate; there is excellent visual potential following surgery
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What is Ablepharon-Macrostomia Syndrome(AMS)?
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An extremely rare inherited disorder characterized by various physical abnormalities affecting the craniofacial area, the skin, the fingers, and the genitals
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What are some features of AMS?
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absence or severe underdevelopment of the upper and lower eyelids (ablepharon or microblepharon), absence of eyelashes and eyebrows, unusually wide, "fish-like" mouth(macrostomia), and/or incompletely developed, low set ears (pinnae)
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Describe a Grade 1 on the Nouby Grading Scale.
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Coloboma without Cryptophthalmos
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Describe a Grade 2 on the Nouby Grading Scale.
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Coloboma with abortive cryptophthalmos
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Describe a Grade 3 on the Nouby Grading Scale.
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Coloboma with complete cryptophthalmos
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Describe a Grade 4 on the Nouby Grading Scale.
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Classic Cyptophthalmos
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Describe a Grade 5 ont he Nouby Grading Scale.
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Severe Cryptophtlamos with severe deformity of the nose and ectropion of the upper lip
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What is Euryblepharon?
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It is a congenital eyelid anomaly characterized by a horizontal enlargement of the palpebral fissure.
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Describe the eyelid in Euryblepharon.
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It is shortened vertically compared with the horizontal dimension with associated lateral canthal malpositioning and lateral ectropion
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What can the shortening of the skin in Euryblepharon lead to?
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Lagophthalmos with a mild to moderate exposure keratopathy
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What can Euryblepharon be associated with?
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Can be isolated finding or ocular anomalies such as lateral displacement of the proximal lacrimal drainage system, a double row of meibomian gland orifices, telecanthus, and strabismus
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Describe the lateral canthal tendons in Euryblepharon?
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They are elongated and abnormally attached at a lower and more anterior position on the lateral orbit
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Describe a Grade 1 on the Nouby Grading Scale.
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Coloboma without Cryptophthalmos
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Describe a Grade 2 on the Nouby Grading Scale.
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Coloboma with abortive cryptophthalmos
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Describe a Grade 3 on the Nouby Grading Scale.
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Coloboma with complete cryptophthalmos
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Describe a Grade 4 on the Nouby Grading Scale.
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Classic Cyptophthalmos
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Describe a Grade 5 ont he Nouby Grading Scale.
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Severe Cryptophtlamos with severe deformity of the nose and ectropion of the upper lip
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What is Euryblepharon?
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It is a congenital eyelid anomaly characterized by a horizontal enlargement of the palpebral fissure.
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Describe the eyelid in Euryblepharon.
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It is shortened vertically compared with the horizontal dimension with associated lateral canthal malpositioning and lateral ectropion
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What can the shortening of the skin in Euryblepharon lead to?
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Lagophthalmos with a mild to moderate exposure keratopathy
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What can Euryblepharon be associated with?
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Can be isolated finding or ocular anomalies such as lateral displacement of the proximal lacrimal drainage system, a double row of meibomian gland orifices, telecanthus, and strabismus
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Describe the lateral canthal tendons in Euryblepharon?
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They are elongated and abnormally attached at a lower and more anterior position on the lateral orbit
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Describe the cosmetic Tx of Euryblepharon.
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Lateral canthal repositioning surgery corrects the canthal malpositioning, while the midface lift corrects the vertical eyelid shortening and supports the lateral canthus
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Describe Congenital Upper LId Eversion.
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Present at birth and typically bilateral
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What is the goal of management of Congenital Upper Lid Eversion?
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Prevent dessication of the xposed conj and allow spontaneous inversion of the lid
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In what people is Congenital Upper Lid Eversion most common?
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African-American and Down's
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What is a epicanthus?
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Vertical skin fold in the medial canthal area overlying the medial canthal tendon
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What are the different types of epicanthus?
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Epicathus Tarsalis, Epicanthus Palpebralis, and Superciliaris
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Where is Epicanthus Tarsalis located?
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Superior
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Where is Epicanthus Palpebralis?
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Equally distributed superior and inferior
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Describe superciliaris.
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Folds arise above the brow and extend downwards to the lateral part of the nose
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What are other names for Cri du Chat syndrome?
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Cat's Cry Syndrome and 5P Minus Syndrome
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What are some things associated with Cri du Chat Syndrome?
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Hypertelorism and Strabismus
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