Frontonasal encephalocoele

Redundant brain tissue is removed and the cranial base reconstructed. The eye sockets are moved closer together and the soft tissue reconstructed.

Typically the nose is long and this may require later correction.

Basal encephalocoele

In this case the brain was present in the roof of the mouth.

Craniofacial principles were used to repair the cranial base and brain herniation.

Hemifacial microsomia

We have a protocol of management at various stages of growth depending on the degree of deformity. Any tissue derived from the first and second branchial arch may be affected including the ear, the lower jaw, facial nerve and parotid gland.

We reserve distraction for cases of airway compromise. Microtia reconstruction and orthognathic surgery at the completion of growth along with soft tissue augmentation is sometimes required.

Rombergs disease

Rombergs disease is an atrophy of facial tissue that progresses over a decade or more.

It can affect the skin surface and all layers right through to the bone. Although many advocate delaying treatment until the disease has burnt out, I prefer to intervene in severe cases to minimise psychological distress.

Thyroid opthalmopathy (Graves disease)

This autoimmune disease causes an increased volume of muscle and fat tissue within the eye socket. This causes a proptosis of the globe with aesthetic disturbance and pain due to corneal exposure. The orbital volume can be enlarged to accommodate the increased fat and muscle tissue. In extreme cases the brow may be advanced forward to give better eyelid coverage over the globe. I operate on several cases each year.

Reference:Modified transconjunctival osteotomies for exorbitism in Graves opthalmopathy. Davis C. In Wolfe. A (Ed) Craniofacial Surgery 12; 259-61, 2007

Also previously known as Cohen syndrome. A coronal craniosynostosis is present and this is treated in the first year of life. The eyes are widely spaced (hypertelorism) and this is corrected using a facial bipartition at about age 8.

The maxillary occlusal plane is often convex and this is corrected concurrently. Frequently the nasal tip is very broad or bifid and this is also corrected.

Craniofacial interventions in Treacher Collins syndrome include bilateral ear reconstruction, shortening the vertical maxillary height and nasal length, advancing the mandible and point of the chin, and augmenting the cheek bone and lateral orbital regions.

Treatment is individualized according to individual needs.

The eye reaches adult size at about age 3. If the eye does not grow, the eye socket remains small.

We start expanding the eye socket at a very early age so as to produce a normal looking eyelid. The eye is replaced with a prosthesis.

There are a wide variety of ways the craniofacial region may be affected by the various types of neurofibromatosis. The example shown is a mild variant of cranioorbital neurofibromatosis.

In more extreme variants the eyesight is lost and the bone at the rear of the eye socket is missing producing a bulging and pulsatile eye (pulsatile exopthalmos).

During normal development brain tissue is present at the nasal tip and sometimes remnants may remain. It is very important to ensure there is no intracranial communication before removing certain lesions on the nose as CSF leak and meningitis may occur.

The example shown here shows a child with a heminasal hypoplasia and a dermoid connecting through the skull base with the brain. I removed this through a transcranial approach.
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