General fibrosis syndrome (congenital enophthalmos with ocular muscle fibrosis and ptosis; congenital fibrosis of the inferior rectus with ptosis; strabismus fixus; vertical retraction syndrome (congenital fibrosis syndrome )

General: Present from birth; familial history; apparent autosomal dominant transmission; sex-linked recessive transmission also reported.

Ocular: Ptosis; enophthalmos; disk hypoplasia; astigmatism; esotropia; exotropia; hypotropia; nystagmus; visual loss; positive forced duction test; may be associated with Marcus Gunn jaw-winking and synergistic divergence in attempted right gaze.

Clinical: None.

Brodsky MC, et al. Neural misdirection in congenital ocular fibrosis syndrome: implications and pathogenesis. J Pediatr Ophthalmol Strabismus 1989; 26:159.

Fraunfelder FT, Roy FH. Current Ocular Therapy. 5th ed. Philadelphia: WB Saunders, 2000.

Harley RD, et al. Congenital fibrosis of the extraocular muscles. J Pediatr Ophthalmol Strabismus 1978; 15:346-358.

Kim, JH, Hwang JM: Hypoplastic oculomotor nerve in congenital fibrosis syndrome and synergistic divergence with magnetic resonance imaging. Ophthalmology 2005; 112: 728-

Miller NR, ed. Walsh and Hoyt's Clinical Neuro-Ophthalmology. vol. 2. 4th ed. Baltimore: Williams & Wilkins, 1985.

Yazdani A, Traboulsi EI: Classification and surgical management of patients with familial and sporadic forms of congenital fibrosis of the extraocular muscles. Ophthalmology 2004; 111: 1035-l042.