Surgical management of the anophthalmic orbit, part 1: Congenital

Congenital microphthalmos and anophthalmos are rare conditions in which orb ital growth is deficient. Hypoplasia of the globe affects the bony orbit (m icro-orbitism), the conjunctival sac, and eyelids (microblepharism), and it may be associated with abnormalities of the entire hemifacial skeleton (he mifacial microsomia). In the present article, the authors review a series o f 19 patients with microphthalmos (nine had right-sided, one had bilateral, and nine had left-sided microphthalmos) who were treated in the Orbitopalp ebral Unit at Hospital Foch over a period of 15 years (follow-up, 5 months to 18 years). Orbital expansion was achieved using spherical implants (n = 13), orbital o steotomies (n = 4), and orbital expanders (n = 2). Both expanders were remo ved within 6 months because of failure (one infection and one rupture). The current preferred method for orbital expansion is to use serial implants i n the growing orbit and osteotomies in cases of late referral or insufficie nt orbital volume in the older child. The target proportions of the reconst ructed orbit are not planned to mirror the healthy side exactly. The inferi or orbital rim is kept higher to support the orbital implant, and the orbit is kept shallow to avoid a sunken appearance. Cranial bone grafts were used to augment deficient orbital contours; they w ere assisted by anterior transposition of the temporalis muscle (n = 5) whe n additional orbital volume was required. Conjunctival sac reconstruction w as achieved by the use of serial conformers placed in the conjunctival sac during the neonatal period, followed by grafts of buccal mucosa and full-th ickness skin maintained in place with a tarsorrhaphy for 3 to 6 months. Eye lid reconstruction using local flaps and skin grafts proved to be necessary in cases treated by osteotomy expansion, although reconstruction was not r equired after expansion using serial solid shapes. The results illustrate a n evolution in approach and concepts of reconstruction of the microphthalmi c orbit and emphasize the need for an integrated craniofacial approach for this complex deformity.