British Journal of Plastic Surgery (1979 , 32, gb-105

CRANIOFACIAL SURGERY-INDICATIONS, ASSESSMENT AND COMPLICATIONS

By DAVID WTTHEWS, C.B.E., M.D., F.R.C.S. Hospital for Sick Children, Great Ormond Street, London

MAJOR craniofacial operations for the treatment of congenital malformations are now firmly established following Tessier’s (1967) pioneering work and the unavoidable risks which they carry, are fairly generally agreed. It is, therefore, timely to consider the factors which are important in the preoperative assessment of patients for surgery, the indications for recommending it and the complications which may be encountered. This review is based on 46 cases operated upon by the craniofacial team at the Hospital for Sick Children over a 6-year period. It represents about 500 hours of operating time. The series consists of 31 cases of hypertelorism, of which 23 were corrected trans- cranially and 8 extracranially, and 15 cases of craniosynostosis, of which g were corrected transcranially (Apert 5, Crouzon 4) and 6 extracranially (Apert 2, Crouzon 4).

INDICATIONS

Cosmetic. The need to improve the patient’s appearance is the most obvious indication for operation. Many of these cases are monsters, although in infancy and early childhood they may be intellectually and psychologically normal. But until there was a possibility of effecting a major cosmetic improvement, they were hidden away, often finishing in institutions, irretrievably damaged in personality and intellect. Older patients may already be set on this downhill path by the time treatment is offered, but it is most important to recognise the possibility of salvaging the victims of such circum- stances, even when the outlook initially seems discouraging. This has been a very happy experience in my association with this work. For example, one girl was changed from an unhappy, introverted recluse into an outgoing happy person, who sought and held a job and subsequently married, after seeking counsel regarding the hereditary incidence of Apert’s syndrome and insisting on sterilisation. Many similar transforma- tions occurred.

Some patients have been intellectually and psychologically handicapped from birth, the degree varying from minor subnormality to imbecility, with gross behavioural problems. This is particularly so in Apert’s syndrome and I have tended to regard it as unjustifiable to expose such a child or adolescent to so major an operation unless I felt the patient had sufficient intelligence to benefit. This attitude is not shared by some psychiatrists who insist that these patients are capable of benefiting within the ambit of their own comprehension. A more diflicult question is whether a cosmetic improvement on such a child is worth attempting to lighten the burden of parents and siblings taking care of a monster. Operation on these grounds is certainly sometimes justified.

It can be stated unequivocally that a cosmetic improvement in a patient of average intellect and behavioural normality confers benefits which are infinitely worth while socially, psychologically and economically. It greatly improves the chances of a normal life and employment.

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Ophthalmic. Craniosynostosis occurring alone or in conjunction with hyper- telorism leads to gross exophthalmos and cornea1 exposure which can cause blindness from infection and cornea1 ulceration. The need to increase the depth of the orbits by advancing the facial skeleton in order to avoid this disaster is an urgent indication for operation, and there is no other way in which it can be achieved. Blindness can occur, although rarely, from optic atrophy. This is unlikely to be due to stretching of the optic nerve which is, in fact, capable of gradual distension without atrophy. When atrophy does occur, it is more likely to be due to stenosis of the optic canals; although, theoretically, this should be prevented by decompression, in practice it is difficult to recognise and correct before irreparable damage is done.

In hypertelorism there is also the vexed question as to whether binocular vision can be achieved by operation. The answer is not known. Tessier has written that if it is to succeed, operation needs to be undertaken by the time the child is 4 years old. In my opinion, if operation is undertaken with this objective, it should be performed when the child is very much younger, but I am not convinced that it can ever be achieved. To have any possibility of success, the globes must travel with the orbital framework which means that section of the orbital walls must be behind the equator of the globes; this is what Tessier (1976) has termed the “useful orbit”.

Respiratory. Nasal narrowing in craniosynostosis is well known to all who have studied these children and may be severe enough to lead to complete obstruction of the nasal airway and even constitute a hazard to life in young infants. The airway is also reduced by an antero-posterior reduction of the diameter of the oro-nasal sphincter, and this can only be relieved by advancing the face, which brings the palate forward. If there is a superadded element of micrognathia, as exemplified in the Treacher- Collins syndrome, this danger is increased.

Neurological. The devastating effects of rising intracranial pressure on the developing brain in craniosynostosis have for long engaged the skills of the neuro- surgeons, who have experienced much difficulty in achieving permanent relief from craniotomy. A much better prospect of lasting relief is offered by combining vault osteotomies with advancement of the face and the anterior cranial fossa. Cerebral compression may well be an indication for the early performance of a major recon- structive procedure.

Dental. In craniosynostosis there is a gross degree of malocclusion which, unless corrected, leads to caries and early loss of teeth. The malocclusion may be so severe that mastication is impossible, leading to restriction of diet, stunting of growth and even failure to thrive in childhood.

ASSESSMENT

This involves the accurate identification of the anatomical abnormality and of the effect this has had on neighbouring structures. An equally important aspect is assessment of the mental state of the patient and the pyschological effects on parents and family. For both these reasons, it is essential to admit the patient for a few days for a preoperative work-up. The specialists involved include the paediatrician, the neurologist and neurosurgeon, the radiologist, the plastic surgeon, the ophthalmologist, the otorhinolaryngologist, the dental surgeon, the anaesthetist, the psychiatrist, the social worker and nursing staff. It is impossible to achieve a study in depth by all the departments concerned on an out-patient basis or to stage the joint discussions amongst

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several members of this team which are vital to proper decision making. The value of such an opportunity for the nursing staff and social worker to get to know the whole family cannot be overestimated.

When study of the malformations in depth is made in this way, it is immediately apparent that they are complicated and complex.

Hypertelorism. This is not just abnormally spaced eyes. Indeed, hypertelorism is often the result of other deformities, and in assessing the likelihood of curing the hyper- telorism one has to assess the possibility and risk of correcting the primary cause. Most commonly, this is concerned with abnormalities of the base of the skull in general, and of the ethmoid bone in particular. The cribriform plate may either be partially depressed or totally thrust down by a primary encephalocoele. Or the ethmoid may be foreshortened and widened, becoming more square than rectangular, with widening of the lateral ethmoid masses which push the orbits apart.

There are always other deformities associated with hypertelorism which require correction, quite apart from reduction of the intercanthal distance: e.g. a short nose, a wide nose, a bifid nose tip, an alar cleft, frontal meningocoele, abnormal rotation of the orbits, abnormality of the hairline, wide separation of the eyebrows, coloboma or gross abnormality of the eyelids, a missing eyeball, a small eyeball, unequal deviation of the bony masses from the midplane on the 2 sides, strabismus, ptosis and antimongo- loid slant of the canthi. One also has to distinguish the apparent canthal widening of telecanthus from the true separation of hypertelorism (Fig. I).

Assessment of the surgical possiblity of correcting a patient labelled as hyper- telorism must take account of the need also to correct any of these associated deformities. Secondary operations, including camouflage procedures, will be needed in many severe

FIG. I. Hyperrelorism. There is a short bifid noglph an absent bridge, strabismus and an epicanthic

FIG. 2. Apert’s Syndrome. Associated with the hypertelorism are ptosis, strabismus and lateral canthal dystrophia.

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cases, although with experience the number which can be dealt with at the primary operation greatly increases.

The principle of correction of hypertelorism is to bring the eyes together without compromising vision and ocular movements, or respiratory function. The decision as to whether this is undertaken transcranially or extracranially depends upon exact identification of the anatomical malformation. Success or failure depends upon this, as does also the maximum reduction of risk.

Craniosynostosis. This also presents complex anatomical malformations which have to be analysed in assessment of operability and the likelihood of success. There is an element of both coronal and facial stenosis in both Crouzon’s and Apert’s syndromes but the degree varies considerably. In plagiocephaly, coronal stenosis predominates without significant involvement of the face, in contrast to facial atresia with hypoplasia of the maxilla in all planes, so that even if advanced, normal occlusion cannot result; or stenosis and malformation of the cranial base may widen the angle of the normal baseline so much that a gross anterior open bite results, producing what Tessier (1976) has described as a “facial lordosis”.

In Apert’s syndrome the ethmoid may be enlarged to give an associated hyper- telorism, and encephalocoeles are common, in contrast to Crouzon’s disease, where the ethmoid is not widened; enlargement of the frontal sinuses and their prolapse into the ethmoidal area in Apert’s syndrome is also a contributary cause of hypertelorism. In Crouzon’s disease, where there is no hypertelorism, there is sometimes, instead, an associated telecanthus caused by skin surplus at the medial canthi.

Orbital stenosis causes shallowness of the orbital cavities leading to a reduction in the useful orbit, i.e. the depth to just behind the ocular equator, leading to exoph- thalmos, exorbitism and strabismus. Ptosis is always present in some degree and may be severe. Abnormalities of the shape of the orbits also causes lateral canthal dystrophia (Fig. 2).

The exact nature and degree of all these anatomical failures varies in each case, together with the severity of the hypoplasia of the nasal passages, determining not only whether correction is made intracranially, but also the angles of the osteotomies and the size and shape of the bone grafts needed to restore normality.

In both hypertelorism and craniosynostosis, assessment involves the whole deformity rather than restriction of concentration just to the wide set of the eyes, or to the fusion of the bony sutures. Only by so doing is it possible to choose the right case for surgery, to forestall unexpected difficulties and hazards at operation, and to get the best possible results.

Timing of surgery. No review of assessment for craniofacial surgery can be comprehensive without reference to the unsolved question of determining the optimum time for operation. It is by no means certain that there is an optimum time. The obvious fears regarding early operation are of postoperative growth failure of facial bones and grafts, or of relapse from resorption of grafts. There is a growing amount of evidence in postoperative cases, that retardation of growth of facial bones is not as serious a possiblity as was feared, but nothing is known as yet about the long-term fate of the grafts. There is much to be said for early operation on the grounds of psycho- logical benefit to the patient and the family, and because young children tend to with- stand the surgical trauma better than older patients. A Le Fort I procedure in adol- escence can do much to retrieve a relapse occlusal position and early operation should not be withheld to evade this possibility. The vexed question of the need to attempt

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to achieve binocular vision can also be argued in favour of early operation, although it is uncertain whether this can be obtained.

Against all this, however, is the reasonable attitude that a long-lasting good result is more likely to follow an operation postponed until much of the facial growth has had time to take place; until the second dentition is well advanced; until the child is about 12. The author’s opinion, based on personal experience and on the recorded opinions of others, is that it is best to wait until about this age unless there is an urgent need for early intervention, such as the risk of deterioration of vision from correctable causes, progressive rise in intracranial pressure, a dangerous degree of respiratory obstruction and, possibly, in rare circumstances, the chance of achieving binocular vision.

EARLY COMPLICATIONS

Deaths. Two patients died; the 2nd and 4th in the series. Both followed trans- cranial operations for hypertelorism. The 1st followed a cardiac arrest shortly after completion of the operation and was probably due to hypovolaemia precipitating gross electrolyte imbalance. The 2nd was due to rapid irreversible cerebral oedema occurring 36 hours after a trouble-free operation; it may have been due to water intoxication induced by pituitary disturbance from retracting the brain which was abnormal. The child had a frontal encephalocoele and there was no corpus callosum. Or it may have been due to the length of time the brain was exposed; subsequently the order of the steps of the operation was changed so that the brain would be exposed for the minimum length of time. Since then, no similar occurrence or anxiety has been experienced.

Immediate postoperative period. The patient should be stirring by the time the bandages are applied; there should be no prolonged period of unconsciousness. Occasionally there are transient spasms of one of more limbs as consciousness returns, but these have never led to any paresis or lasting abnormality of tone. Electrolyte imbalance can occur and its prevention needs repeated monitoring. Persistent bleeding is also potentially serious in so far as it may require what amounts to a total replacement transfusion which can precipitate a haemorrhagic diathesis lasting several days and needing treatment with special blood fractions, packed cells and platelets. Bleeding of this magnitude comes from the cut bony surfaces of donor sites on ribs and ilium; it can be forestalled by using bone wax at operation. Haemorrhage from the facial area is not large enough to be serious, and no extradural haematoma has developed in our cases.

Pneumothorax can occur from rib resection, despite a satisfactory X-ray of the chest at the time the rib grafts are cut. It develops slowly during the subsequent hours of surgery; another chest X-ray is therefore always taken at the end of the operation. Three patients have had an unexpected pneumothorax discovered in this way. All responded to intercostal drainage without causing anxiety.

Diabetes insipidus developed in 2 cases, I of which took a week to resolve and required the administration of pitressin. The cause was almost certainly traction on the frontal lobe of the brain in the midplane to expose the cribriform plate in the presence of an abnormality of the cerebrum and skull base. Both patients had primary encephalocoeles anterior to the pituitary gland.

A transient leak of cerebrospinal fluid into the nose has occurred after almost every transcranial operation and has taken up to IO days to stop. Rarely there has also been a transient leak through the scalp drains. There have been no cases of menin- gitis during this early period of leakage, nor has there been any major infection of blood clot or of bone grafts at this time. Orbital haematomas have, however, led on occasion

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to chemosis and lid retraction with exposure of the globe. Failure to counter this by reapplication of the central tarsorrhaphy stitch has, on one occasion, led to cornea1 ulceration and scarring.

Exhaustion must also be added to the list of complications which can occur in the immediate postoperative period. To some extent this is unavoidable with constant monitoring, but it must be recognised and the patient adequately rested with sedatives. Progressive exhaustion is potentially lethal and its avoidance with drugs very simple. It is only too easy for anxious bystanders, including the surgeon, to mistake essential sleep for pathological reduction of consciousness.

LATE COMPLICATIONS

Neurological. In 2 cases, persistent cerebrospinal rhinorrhoea complicated convalescence and neurosurgical intervention was necessary to close the leak with a graft after a few weeks; neither gave subsequent trouble. A 3rd case in which no leak was discovered during convalescence had an attack of meningitis 15 months after surgery, followed by an obvious leak which had to be closed surgically. No patient in the series has sustained demonstrable brain damage or reduction in the intelligence quotient; in general, this has increased postoperatively at the average rate for age. The behaviour and mental outlook of many patients have been radically improved, with lasting benefit to the whole family.

Anosmia is a complication which would be expected to occur with removal, in many cases, of as much as two-thirds of the cribriform plate and much of the septal mucosa high up in the nose. It is very difficult to test for this accurately, particularly in young patients, and the results are subjective findings. Surprisingly, however, this complication appears to be much rarer than would have been supposed. Only I patient in the whole series volunteered the observation that she had lost the sense of smell.

Ophthalmic. The eyes are obviously very vulnerable in these operations, but no eye has suffered any injury in our series from operative manipulation; this is a preventable accident.

Movement of the globes has, however, caused oculomotor disturbance in 3 cases in which it was not found to be present preoperatively. The disturbance was complex involving the external rectus and the oblique muscles ; 2 have needed ophthalmic correction. The 3rd has improved spontaneously. Strabismus is frequently present in craniosynostosis and hypertelorism and it is often made transiently worse by cranio- facial operation, but all, except those mentioned, have regained their preoperative condition.

Ptosis is also a common finding with these congenital malformations. It is almost always made temporarily worse by advancing the face in craniosynostosis. This is probably due to the loss of support of the lids by the globes when the lids are moved forward with the bony orbits. After a few weeks, however, the levator muscle improves in tone sufficiently to restore the preoperative state. It then may, like any other con- genital ptosis, require adjustment by shortening the levator muscle (Fig. 3).

Postoperative oedema, in one case, led to serious cornea1 ulceration with scarring which, happily, is slowly resolving (Fig. 4). The only other case in the series in which vision has been affected is a child in whom there developed a massive infection of the bone grafts, leading to purulent discharge, resorption and surgical removal of almost all the grafts over a period of several months. In this patient, vision in one eye has been reduced to perception of light.

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FIG. 4. Hypertelorism. A, Preoperative showing wide nose, alar cleft, absent globe, abnormal hair lint and absent eyebrow. B, After correction there is cornea1 scarring due to chemosis, leading to exposure

keratitis.

Respiratory. Further reduction of an already inadequate airway can occur with medial movement of the orbital walls in all these cases, unless additional nasal width is gained by removing the septum from some distance behind the columella, leaving the patient with only one opening connecting the nose with the nasopharynx. If this is done routinely, this complication can be avoided.

Dental occlusion. An overjet has been reduced by 3 mm over a period of 5 years in a child operated on at the age of 12 years. Given sufficient initial overjet, this is of no consequence in a child of this age, but a 2nd case in which this has also happened after an operation at the age of 5 years is disquieting. Both these occurrences raise the questions of postoperative growth of facial bones and of the fate of bone grafts. The whole series will need to be carefully monitored over many years before informed comment can be made on these important matters.

Bone resorption. In one case-the 4th in the series-complete slow aseptic resorption of the frontal bone necessitated reconstruction of the forehead with split-rib grafts. In this case, the frontal bone had been completely detached and laid aside for several hours, whilst the face and skull were advanced, before being wired back into place. Since this complication, the frontal bone has not been detached in this way and no other case of resorption has occurred (Fig. 5).

Cosmetic. Aesthetic imperfections sometimes occur. These are unavoidable in so major an operation, aimed to treat so complex a deformity. But they are important since cosmetic improvement is the main indication for surgery. Perhaps the degree

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FIG. 5. Crouzon’s Disease. Aseptic resorption of the frontal bone after detachment at operation.

FIG. 6. Media2 Canthal Drift. A, Preoperatively. B, Postoperatively showing drift of the soft tissues despite correct bony narrowing.

CRANIOFACIAL SURGERY IO5

and frequency of imperfection is in inverse ratio to experience, but even with experience, secondary retouching operations may be needed.

The most tiresome aesthetic shortcoming is the tendency of the medial canthi to drift away from the nose, widening the intercanthal distance despite permanent bony narrowing. It is due to tightness of the soft tissues imposed by medial movement of the bony parts, Its occurrence is less likely to occur if the periosteal tissue is deliberately divided after it has been lifted from the lateral orbital walls ((Fig. 6).

The lateral camhi can also cause the eyelids to lie unnaturally in relation to the pupil, unless they are fixed to the orbital wall at exactly the right height.

Additional modelling with bone or a prosthesis may be required to give symmetry to the cheek bones, a pleasing contour to the forehead and supra-orbital ridges, or to improve the shape and length of the nose.

Residual ptosis and strabismus also constitute aesthetic blemishes, quite apart from the physical disabilities they create.

An occasional secondary intervention has been performed for all these reasons. They are comparatively trivial procedures, but are well worth doing since they greatly enhance the final appearance and so the contentment of the patient and family.

I wish to record that this review embraces the work of the whole team and all the back-up facilities provided by the service and nursing departments of the Hospital for Sick Children.

REFERENCES

TESSIER, P. (1967). Osteotomies totales de la face. Syndrome de Crouzon, syndrome d’Apert. Oxyckphalis. Scaphockphalis. Turrickphalis. Annals de Chirurgie Plastique, 12, 273.

TESSIER, P. (1976). Orbital Hypertelorism. In “Symposium on Plastic Surgery in the Orbital Region”, p. 255. Saint Louis: C. V. Mosby Co.

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