The optic nerve is most endangered in the following:
Fibrous dysplasia. Craniofacial fibrous dysplasia ranges from mild to severe disease and is divided into 4 zones. Zone 1 is orbital, zone 2 is hairbearing cranium, zone 3 is the cranial base and zone 4 is the maxilla and mandible. There is a small risk of malignant transformation. Orbital disease can cause proptosis, optic nerve atrophy and blindness.
Isolated cleft palate:
Can occur as submucous clefts which often present only with speech abnormalities.
The facial bipartition technique is best suited to the correction of:
Apert’s syndrome. The main cranial feature of this syndrome is bicoronal synostosis. The aims of treatment include addressing form and function. Crisis management involves treatment of ocular exposure including tarsorrhaphy or early fronto-orbital advancement, treatment of raised intracranial pressure with ventriculoperitoneal (VP) shunts, and treatment of airway compromise. Midface hypoplasia can be treated by distraction or Le Fort 3 osteotomy/advancement or Monasterio’s technique of Monobloc advancement. The treatment of the cranium is often cranial vault remodelling and fronto-orbital advancement but facial bipartition has a number of advantages in selected cases.
The incidence of hydrocephalus in craniofacial dysostosis patients is:
10%.
The LeFort II osteotomy is limited in value but may be indicated in:
Binder’s syndrome. Binder’s syndrome is a sporadic congenital malformation with nasomaxillary hypoplasia. Le Fort II or III osteotomies may be indicated depending on presentation with simultaneous bone grafting. Rhinoplasty is usually undertaken at a later stage.
Click to expand