Ординатура / Офтальмология / Английские материалы / Oculoplasty and Reconstructive Surgery Made Easy_Garg,Touky, Nasralla_2009
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Figrue 9: Lateral orbitotomy for hemangioma (intraconal)
Figure 10: Complete endoscopic management of orbital tumor— hemangioma (Courtesy: Dr Milind Navlakhe)
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Capillary hemangiomas are the most common orbital tumors of childhood and can cause amblyopia secondary to occlusion of the pupil, anisometropia, or strabismus. Hemangiomas were classified based on size. Presence of aniosometropic astigmatism, ptosis, pupillary occlusion, lid margin change, proptosis, globe displacement, and strabismus need to be recorded.
Schwartz et al42 reported that size greater than 1 cm in largest diameter is an important predictor of amblyogenic factors and approximately half of these patients require treatment. Diffuse hemangiomas and hemangiomas in patients with PHACES syndrome cause amblyopia in the majority of cases. Periorbital hemangiomas can cause amblyopia secondary to anisometropia, induced astigmatism, strabismus or occlusion of the visual axis. Oral and intralesional steroids are considered to be the most accepted form of primary treatment. Surgery is a safe, effective treatment for selected lesions, provides a definitive early treatment, and prevents astigmatism and occlusion-related amblyopia and so some authors43 favor early surgery.
Figure 11: Orbital cellulitis (Courtesy: Dr Milind Kirtane)
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Preseptal cellulitis is the most common orbital disease in children older than 3 years.44 Awareness of orbital diseases in childhood, as a rare entity, is crucial for timely diagnosis and appropriate treatment, to save the vision and lives of affected children.
Endoscopic Management of Orbital Diseases
Both hemangiomas and orbital cellulites are amenable to treatment by endoscopy and have excellent results in the authors experience.
Nd: YAG Laser and Corticosteroids
Some authors45 have tried local Neodymium: YAG-laser therapy and in patients with large subcutaneous eyelid hemangiomas and involvement of the orbita tried with interstitial Neodymium: YAG-laser therapy in combination with systemic corticosteroids with good results.
Orbital Lymphangioma and AV Malformations
Orbital lymphangioma and AV malformations are uncommon, benign cystic appearing lesions, generally manifest in childhood. They usually present with a slowly progressive proptosis, displacement of the globe, ptosis and restriction of eye movements. Occasionally, focal lesions may remain asymptomatic. Spontaneous intraorbital hemorrhage may cause acute proptosis, compressive optic neuropathy and
Figure 12: Preand post-decompression
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loss of vision. Surgical decompression of the optic nerve may be performed if there is progressive decrease in visual acuity.46
CONGENITAL EYELID RETRACTION
AND CHILDHOOD THYROID
Congenital eyelid retractions may affect either the lower or the upper eyelid. They make up a rare condition and most cases are associated with craniofacial syndromes such as Crouzon or Apert syndromes or childhood thyroid. Burroughs et al 47 have proposed that thyroid eye disease may be present in children and even neonates with both mother and child being euthyroid. Upper eyelid retractions may spontaneously improve in some cases, but lower eyelid retractions do not. Treatment requires surgery, depending on the corneal consequences.
Figure 13: Congenital eyelid retraction
EYELID MALPOSITIONS
Ruban’s Classification (Ruban JM, Baggio E). Surgical treatment of congenital eyelid malpositions in children .J Fr Ophtalmol. 2004 Mar;27(3):304-26.)
It is important to separate eyelid malpositions, which are topographical disorders, from eyelid malformations, which are constitutional morphological disorders.
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Eyelid malpositions can be as follows:
A.Static–including
i.Epiblepharon—Epiblepharon is characterized by the absence of adhesion between the lower eyelid retractors and the orbicularis-skin layer, which allows the anterior lamella to roll over
ii.Congenital ectropion and entropion,
iii.Epicanthus—Epicanthus involves a semi-lunar fold of skin extending from the upper eyelid across the medial canthal area to the margin of the lower eyelid. Four types
a.Supra-ciliaris,
b.Palpebralis,
c.Tarsalis and
d.Inversus of epicanthus are described. Treatment requires surgery.
iv.Telecanthus—defined as an increased distance between canthi.
a.Primary telecanthus results from attenuation of the medial canthal tendons and is usually associated with other soft tIssue abnormalities such as epicanthus or blepharophimosis, or is seen after trauma.
b.Secondary telecanthus is caused by underlying bony malpositions with an abnormal separation between the orbits because of an increased thickness of the interorbital bones such as that seen in hypertelorism or in other complex craniofacial syndromes
v.Centurion syndrome.
B.Dynamic disorders: Ptosis and congenital eyelid retractions. Euryblepharon is distinguished by an enlargement of the horizontal palpebral fissure associated with enlarged eyelids.
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Congenital Entropion
When the eyelid margin is rolled inward against the globe,at birth, the condition is referred to as congenital entropion. Upper eyelid involvement is commonly associated with a tarsal abnormality, while lower eyelid entropion is often associated with epiblepharon. Entropion does not resolve spontaneously, and may cause corneal pathology if untreated. Serafino et al48 studied patients with lower bilateral congenital entropion to compare the results of incisional versus rotational surgery. The authors consider both techniques satisfactory, but the procedure of choice, considering the age of the patients and previous studies, remains rotational sutures because of its simplicity, quickness, and low risk of complication. Some authors49 have tried the pretarsal orbicularis muscle injection with 5 units of botulinum toxin with resolution of entropion four days after treatment and no recurrence of the entropion 7 months after botulinum toxin injection suggesting that injection of botulinum toxin may effectively treat certain cases of congenital entropion.
Surgical Correction of Entropion in Congenital Cutis Laxa50
Congenital cutis laxa is a rare generalized inherited elastosis, characterized by the appearance of premature aging and skin laxity with mild to severe systemic anomalies. Ocular manifestations include excess skin in the eyelids, ptosis and lower lid ectropion. Of the hyperelasticity syndromes—Ehlers Danlos, pseudopxanthoma elasticum and cutis laxa—only cutis laxa has normal skin wound healing. The diagnosis must therefore be established before surgical options for treatment are considered.
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Congenital Ectropion
Congenital eversion51 of the upper eyelids is a rare condition, the exact cause of which remains unknown. It is more frequently associated with Down’s syndrome and black babies. If diagnosed early and treated properly, the condition can be managed without surgery conservatively by lubricants, antibiotics and eyelid patching.
Congenital ectropion in congenital ichthyosis52 which is a generalized hyperkeratinization of the skin at birth. Depending on clinical aspects and severity, three forms of congenital ichthyosis have been defined: mitis, tarda, and gravis. Desquamation of the parchment-like hyperkeratinized skin begins shortly after birth and may require several weeks to complete. Skin alterations in the eyelid cause shortening of the anterior lamella, subsequently resulting in ectropion. This affects the upper eyelid more often than the lower and can lead to complications such as chronic palpebral or bulbar conjunctivitis and keratinization or exposure keratopathy. In mild forms of congenital ichthyosis surgical treatment of eyelid ectropion is not required. In more severe cases a skin graft may become necessary.
CRYPTOPHTHALMOS
Cryptophthalmos, a very rare congenital anomaly of the eye, is characterised by skin passing continuously from the forehead to the cheek over a malformed eye.
It is genetic disorder with systemic involvement (sometimes life threatening) and if not leaves the family devastated and unsatisfied with treatment and fearful of one more such occurrence in the family. Thoughtful and methodical approach can alleviate some aspects.
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Figure 14: Cryptophthalmos
Parental Age
The average paternal age is 27 years of age and the average maternal age is 24 years of age.
Known Risk Factors
Oligohydramnios is the most frequent complication during pregnancy.
Pathogenesis
Fras,1 that is specifically detected in a linear fashion underlying the epidermis and the basal surface of other epithelia in embryos. Loss of Fras1 function results in the formation of subepidermal hemorrhagic blisters as well as unilateral or bilateral renal agenesis. Homozygous Fras1 mutants have fusion of the eyelids and digits and unilateral renal agenesis or dysplasia. Modifier genes are important determinants of phenotypic variation. (The pathogenesis of
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phenotypic modules could include disruption to a morphogenetic field or a developmental field, mutation specific effects, or malfunction of temporally distinct genes).
•Triallelic inheritance
•Autosomal recessive inheritance
•Dominant mode of inheritance
The loss of a cytoplasmic multi-PDZ scaffolding protein, glutamate receptor interacting protein 1 (GRIP1), leads to the formation of subepidermal hemorrhagic blisters, renal agenesis, syndactyly or polydactyly and permanent fusion of eyelids (cryptophthalmos). GRIP1 can physically interact with Fras1 and is required for the localization of Fras1 to the basal side of cells. Grip1 is disrupted by a deletion of two coding exons .GRIP1 is required for normal cell-matrix interactions during early embryonic development and that inactivation of Grip1 causes cryptophthalmos.
Classification
A.Nouby’s classification of congenital upper eyelid coloboma and cryptophthalmos.
•Grade 1: Coloboma without cryptophthalmos.
•Grade 2: Coloboma with abortive cryptophthalmos.
•Grade 3: Coloboma with complete cryptophthalmos.
•Grade 4: Classic cryptophthalmos (absence of all eyelid structures and the eye is completely covered with skin).
•Grade 5: Severe cryptophthalmos (with severe deformity of the nose and ectropion of the upper lip).
B.Moreker-Agashe classification:
•Grade 1-Frasers syndrome
•Grade 2-Congenital orbito-palpebral cyst
•Grade 3-Bilateral cryptophthalmos
•Grade 4-Unilateral partial crytophthalmos
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•Grade 5-Symmetrical partial lateral ‘cryptophthalmos‘
•Grade 6-Symmetrical medial cryptophthamos
•Grade 7-Asymmetrical hemi cryptophthalmos
•Grade 8-Congenital symblepharon associated with meningoencephalocele.
•Grade 9-Bilateral abortive cryptophthalmos associated with oculocutaneous albinism
•Grade 10-Congenital symblepharon (abortive cryptophthalmos)
•Grade 11-Cryptophthalmos solum corneae
•Grade 12-Ablepheron macrostomia syndrome.
Treatment
Complete Cryptophthalmos
Weng et al53 described a three-stage reconstructive procedure in which a conchal cartilage ‘sandwich’ graft is first placed between the skin flap and the globe, mucous membrane subsequently grafted onto the inner surface of the cartilage graft, and lid level adjusted as the third stage. Ferri et al 54 have discussed the steps in more details.
Incomplete Cryptophthalmos
Dibben et al55 described that incomplete cryptophthalmos can be corrected by dissection of the eyelids from the cornea, reconstruction of the conjunctival fornices with buccal mucosa, and repairing the upper lid coloboma if present in a flap reconstruction using the inferior eyelid margin is the usual procedure.
Role of Amniotic Membrane
Stewart et al56 reported successful creation of a superior fornix in a case of partial cryptophthalmos after an amniotic