Evisceration is the removal of the contents of the globe while leaving the sclera and extraocular muscles intact.
Enucleation is the removal of the eye from the orbit while preserving all other orbital structures.
Exenteration is the most radical of the three procedures and involves removal of the eye, adnexa, and part of the bony orbit.
1 in 50,000 births
complete absence of the globe through failure of formation of the optic vesicle, and it is an extremely rare condition.
Unilateral in 75%
presents most often as an isolated finding or within the spectrum of otomandibular dysostosis.
developmental-field defect located in Pfeifer’s diacephalic region
defined as the (bilateral) border zone between the frontonasal and the posterolateral regions of the face and stretches from the temple, crossing the orbit and the lateral parts of the nose, including the ala nasi, running along the philtral column of the upper lip, and ending between the premaxilla and the posterolateral part of the palate and the upper jaw at the nasopalatine duct
types of craniofacial malformations that occur here include isolated clefts of the lip (alveolus and palate) or of the nose, most of the Tessier-type clefts (numbers 2, 3, 4, 8, 9, 10, 11, and 12), as well as cryptophthalmia, microphthalmia, and anophthalmia.
Thus, microphthalmia and anophthalmia also may be found in conjunction with hypertelorism (a malformation of the frontonasal region) or with hemifacial microsomia (a disorder of the posterolateral region).
At the time of birth normal globes are said to have a volume of 2.5 ml.
eye and the orbit grow the fastest during the first year of life.
70% of the increase of the globe’s volume occurs by 4 years of age and 90 percent by age 7.
While growth of the eye ends at age 14, the growth of the orbit ends at age 11 in females and at age 15 in males
This is why enlarging the orbit in the first years of life should be attempted
globe plays a role of essential importance in orbital growth, and its diminished contribution in the congenitally anophthalmic orbit causes:
conjunctival sac atresia
short, phimotic lids (microblepharism)
In severe microphthalmos, the eye is grossly hypoplastic and blind.
Unilateral clinical anophthalmia always results in hypoplasia of the ipsilateral bony orbit, as there is no stimulus inducing growth of the orbital socket.
This, in turn, leads to facial asymmetry with hypoplasia of the ipsilateral midface, a canted occlusal plane, and a short ascending ramus of the mandible.
In contrast, bilateral anophthalmia may lead to symmetrical microsomia of the midface as a whole
Formerly, microphthalmia and anophthalmia were treated by osteotomy of the bony orbit followed by lengthening of the lashbearing lid and grafting of mucosa to the socket
Early management consists of eviscerated (removal of its contents, preserving the sclera with its extraocular muscle attachments and Tenon’s capsule) or enucleated (removal of the globe, preserving Tenon’s capsule) and replaced by a spherical implant
Asscess is usually through combined bicoronal and lid incisions
Serial expansion with fixed sized expanders
implant serves as a support for the eyelids and conjunctival sac anteriorly and maintains the volume of the orbit. Exchanged every 6 months for larger prostheses
In a second stage, a removable ocular prosthesis that mimics the eye is fitted to the contours of the conjunctival sac
dermis fat grafts
idea was that this would grow and exert some pressure onto the bony walls of the orbit.
three wall orbital expansion (Tessier)
lateral wall osteotomy and temporalis transfer for bulk and vascularised bed to support SSG (Lee PRS 1999)