Timing of Traitment

Craniofacial malformations are very diverse and optimal timing is different from case to case : the timing varies from operating in infancy – the first six months – for an isolated craniosynostosis to operating from 4 to 5 years of age for hypertelorism.

Therefore, it is essential to take advice from a craniofacial team during the first months of life to :

  • establish the diagnosis,
  • to evaluate the severity of the situation,
  • to determine the plan of treatment.


The ideal time for correction is between 4 months and 12 months of age, according to the anomaly.
We see an increasing number of positionnel deformities of skull, which are not craniosynostosis, and need a simple early nursing treatment (cf « conditions treated »)

Faciocraniosynostosis (Crouzon, Apert, etc.)

An operation on the frontal and cranial part of the malformation is usually also performed between 4 and 12 months of age. The facial retrusion is corrected later : in severe cases, before school age, around 5 years. In moderate cases, one waits until after final dentition, around 12 years of age.

This timing can be modified

  • in very severe cases with breathing problems,
  • with the use of gradual advancement by distraction in selected cases.

In most cases, a late advancement of the upper teeth through an oral approach (Le Fort I) will be necessary in adolescence, as well as different facial aesthetic touch-ups (eyelids, chin, forehead) to obtain the best possible final result.

This timing can be modified

  • in very severe cases with breathing problems,
  • with the use of gradual advancement by distraction in selected cases.

The principle of simultaneous correction of frontal and facial retrusion in faciocraniosynostosis is logical, but the inherent morbidity of a classical monobloc fronto-facial advancement is significant (50%). This morbidity limits the indications of the procedure and must be discussed at length prior to it being undertaken.
The techniques of distraction first used in the mandible have been progressively applied to the rest of the craniofacial skeleton, which has led to a simplification of the procedures involved. The addition of distraction to the numerous other craniofacial procedures has permitted the progression surgical interventions for morphological modification., while reducing their morbidity. This has been confirmed in the case of the monobloc advancement in various small series, as well as in our own experience. We initially saw occasional minor complications, but with increased experience, these allowed a more defined protocol to be devised, as follows:

  1. Distractor fixation with metallic screws as the period of their use has been increased to 6 months. A new type of resorbable fixation is being studied.
  2. Re-inforcement of bone with resorbable plates and screws, if required
  3. The use of Molina caps at the end of the temporo-malar distractor
  4. Obturation of nasal fossa
  5. Complete pterygo-maxillary disjunction, division of midline structures and intra-operative postional control
  6. At least 5mm of on table advancement, in order to augment the intra-cranial volume from the start
  7. Bone paste application in the coronal region to encourage eventual re-ossification
  8. Maintenance in ICU with ventilation for at least 24-48 hours.
  9. Distraction commencement is deferred until between day 7 and day 15 in case of a CSF leak.
  10. A distraction rate of 0.3 – 0.5 mm/day
  11. Maintenance of the distractors in place for at least 6 months after the end of the distraction process (which renders external distractors contra indicated).

The protocol for antibiotic prophylaxis remains unstandardised as the monobloc procedure, goes beyond the usual confines of surgery. The combination of the use of distractors with a complete fronto-facial osteotomy, poses the problem of a transitory implantation of constantly contaminated prostheses. We currently opt for preventative antibiotic therapy of short duration (48hrs) begun at induction, with the option of restarting therapy if signs of clinical infection are evident. It could be however, that broad spectrum prophylactic antibiotic therapy is in fact useful for the entire period of distraction, despite the risk of the development of resistant bacterial strains.
The absence of ossification at the frontal osteotomy sites seems to contradict the usual osteogenecity found at most sites of distraction. It does not however pose any problem, if the procedure is carried out early as secondary cranial osteogenesis, remains possible until 2 years of age. This reinforces our conviction of the benefits of early distraction, before the age of 12 months and allows the question of whether the one stage strategy should be adopted as routine, if morbidity is reduced and the stability of results is confirmed.
Since frontofacial monobloc advancement can be applied to the more severe neonatal deformities as well as the more minor, more slowly progressive deformities, much more frequent use of distraction for the technique is justified. This early series has allowed us to define a one stage strategy, while remaining aware that minor secondary interventions remain indispensable to correct the ultimate growth disturbances of faciocraniosynostosis.
In order to reduce the risks of the routine treatment of faciocraniosynostoses, a two stage strategy has remained widely practised for twenty years as it has allowed a dissociation of problems and a separation of risks.
The Monobloc frontofacial advancement allows a combination of these two stages into one, with diminished risk. It is probably the only way in which a patient can be weaned from his tracheotomy or an early tracheotomy can be avoided entirely.

Hypertelorism, Medial Facial Clefts, Encephalocele

When the eyes are positioned significantly apart, an early evaluation has to be done because there are cases where an early operation has to be done on the skull and forehead (associated craniosynostosis or encephalocele), on the eyelids, or the nose.

In most cases, one will not operate until the child is 4 or 5 years old. Then the orbits and eyes will be brought together and other anomalies corrected simultaneously.

Secondary limited operations on the nose and eyelids are often necessary later.

Lateral Clefts Involving Orbits, Eyelids, Ears (Tessier Clefts, Treacher-Collins, Hemifacial Dysplasia)

A deficiency of the eyelids is an emergency situation, and an eyelid repair has to be done in the immediate post-natal period to protect the eye.

A remodelling of the skull is sometimes done in infancy, but usually, one waits a few years before undertaking reconstruction. Distraction has some indications, especially for mandibles.

Neurofibromatosis : Orbito-palpebral and Facial

An evaluation has to be made when the anomaly is detected, but early operations are rare, with the exception of eyelid hypertrophy that can be corrected during childhood.
A protruding eye may be corrected by a craniofacial operation, but the timing varies for each individual case.

Fibrous Dysplasia, Osseous Hypertrophia

Often diagnosed late and slow growing, each individual case once again has to be evaluated.
The extent of the involvement will dictate the operative possibilities and the follow-up of visual acuity is very important. A medical treatment hoping to stabilize fibrous dysplasia is currently on clinical trial.

Post-traumatic Sequelae

Usually, one has to wait at least six months after the accident to allow the oedema disappear and the scars to diminish. An evaluation can then be made, with a good CT scan, and a plan of secondary treatment can be proposed if improvements are required. Some rare situations (bone or devices exposed, instability, infection, ectropion) may require a rapid intervention. Otherwise, patience is essential.