Epibulbar Lipodermoids

Epibulbar Lipodermoids

Figure 2.30. Capillary hemangiomas of the lids most frequently arise nasally from either the superior or the inferior palpebral fissure. They are poorly defined soft swellings of the eyelid with purple (red-blue) discoloration of the skin. They require treatment when rapid growth threatens the visual axis which can lead to irreversible deprivation amblyopia.

Figure 2.29. Ankyloblepharon filiforme adnatum is shown in this figure with fusion of the upper and lower eyelids by small filiform attachments which can be cut with scissors. Blepharophimosis is a narrow palpebral fissure which occurs as a congenital anomaly and should be included in the differential diagnosis of ankyloblepharon filiforme adnatum.

Figure 2.30. Capillary hemangiomas of the lids most frequently arise nasally from either the superior or the inferior palpebral fissure. They are poorly defined soft swellings of the eyelid with purple (red-blue) discoloration of the skin. They require treatment when rapid growth threatens the visual axis which can lead to irreversible deprivation amblyopia.

Figure 2.31. Treacher-Collins syndrome is characterized by malformations of the structures formed from the first branchial arch, groove, and pouch. Mandibulofacial dysostosis with antimongoloid slanting of the palpebral fissure and coloboma of the lower temporal lid is inherited as an autosomal dominant (5q11) disorder.

Figure 2.32. The coloboma of the lid in the Treacher-Collins syndrome involves the lateral third of the lower lid and may not affect the lid margin. Other lower lid anomalies such as absent lacrimal punctae and irregular lower lid lashes may also be present.

Figure 2.33. The most common findings of Goldenhar's syndrome, which is characterized by hemifacial macrosomia, are colobomas of the upper lids, solid epibulbar dermoids located at the inferotemporal border of the cornea, and solid lipodermoids located in the superotemporal sulcus near the lacrimal gland. Oculoauriculovertebral dysplasia is sporadic and the basic defect is unknown.

Epibulbar Lipodermoids

2.32

2.33

Ankyloblepharon Filiforme Adnatum

Figure 2.34. The coloboma of the lid in Goldenhar's syndrome involves the middle third of the upper lid which does affect the lid margin.

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Choristoma Dermoid

Figure 2.35. This typical dermoid of die Goldenhar's syndrome occurs as a solid mass of the conjunctiva at the inferotemporal border of the cornea. Flattening of the cornea occurs in die meridian of the dermoid, causing an associated astigmatism and occasionally a secondary amblyopia.

Figure 2.36. This complex choristoma located in the superotemporal sulcus of the left eye with the upper lid everted demonstrates die long lashes which may be present. This is different from the lipodermoid noted in the same position in some patients with Goldenhar's syndrome.

Figure 2.37. Fraser's syndrome is an autosomal recessive disorder which occurs when the maturation of die lids is interrupted and the lid folds fail to develop. This infant shows complete cryptophdialmos by the left lid (surface ectoderm) with complete coverage of the corneal epithelium.

Figure 2.38. This figure demonstrates Fraser's syndrome with partial cryptoph-thalmos. There is a continuation of the left superonasal lid (surface ectoderm) with the corneal epithelium.

Figure 2.39. The typical confluent eyebrows, long curly eyelashes, and tele-canthus associated with the Cornelia de Lange syndrome are present in this infant. Telecanthus is the lateral displacement of the inner canthi such that the medial portion of the eye is partially obscured, giving rise to the impression of strabismus and hypertelorism. In hypertelorism the eyes are widely spaced. Because a low nasal bridge may give rise to the impression of widely spaced eyes, the distance between the two eyes should always be measured.

Figure 2.37. Fraser's syndrome is an autosomal recessive disorder which occurs when the maturation of die lids is interrupted and the lid folds fail to develop. This infant shows complete cryptophdialmos by the left lid (surface ectoderm) with complete coverage of the corneal epithelium.

Epibulbar Lipodermoids
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