Craniostenosis.

Craniostenosis is a common problem in the pediatric neurosurgery departments. The management of this problem is still evolving. Some misconceptions exist regarding this condition particularly regarding the indications for surgery. The author started performing this surgery nearly two decades ago. The experience gained over time as well as the problems encountered are discussed.

Introduction

Craniostenosis is being increasingly recognized in clinical practice - both in the pediatric and neurosurgical clinics. The patients are seeking attention earlier - some as early as the neonatal period for surgical correction of the deformity. Although it may appear that the incidence has increased – after evaluation-, it may only be relative and not absolute. This is so because of increased awareness among all medical practitioners – mainly pediatric, and general practitioners. The days when these patients came in with blindness, delayed development, gross proptosis and advanced neurologic deficit are past. The pendulum has swung right across – and early recognition and referral seems to be the norm. Several centres in India are operating on such cases.[1-5]

Incidence

In the last 10 years, the senior author has operated on 70 cases of craniostenosis. The distribution is as follows: anterior plagiocephaly 41 (unilateral 24; bilateral 17); plagiocephaly and scaphocephaly 2; Apert syndrome 4; Cruzon syndrome 5; trigonocephaly 1; oxycephaly 5; scaphocephaly 9; Clover leaf deformity 1; Pfeiffer syndrome 2.

Timing of surgery

The practice was to operate on these children as soon as they were fit for surgery- even as early as one or two months old. But this practice has now changed. Although in the beginning, surgery was being performed on children at the age of 2-3 months, the senior author′s policy is to operate on children around 6 months of age. This is so for the following reasons:

Children withstand surgery better

Anesthesia – both induction and maintenance during – surgery- is smoother

The blood volume is sufficient to reduce the volume transfused

The bones are soft enough to allow bending and remodeling and at the same time permit rigid fixation

Recovery is smoother

However, there are some instances where surgery at an earlier age is warranted, especially in children with advanced disease and raised intracranial pressure. Here the surgery was done more as a “salvage” or “preventive” procedure.

Investigations

The following investigations were done in all children:

Complete blood counts

Basic blood biochemical tests

Coagulation parameters

Blood grouping

ECHO and cardiac evaluation

Plain skiagrams (sometimes omitted if CT scans have been done – particularly with 3D reconstruction- before referral)

CT scan (unenhanced study) with brain, bone windows and 3D reconstruction. The latter includes the face - in case facial correction is needed at a later date.

MRI in some syndromic (or advanced cases) to look for cerebral and hindbrain anomalies. MR venogram is also included.

Anesthesia

The anesthetic technique has evolved and changed over the years as our experience grew. We have been able to reduce blood loss over the years and the volume of blood transfused too has diminished. The main challenges are airway management, blood loss, hypothermia, intracranial pressure and air embolism. The appearance of the child alone, we have observed, is not a harbinger of the difficulty in intubation. In one child with Pfeiffer syndrome, we had to perform a tracheostomy before starting the surgery. In the early days, blood loss was a major problem and some of our children have had their blood volumes replaced! But now we have been able to reduce the loss to about 200 ml. This is because of two reasons a) increasing experience 2) use of tranexamic acid infusion before commencing the surgery. The insertion of a central venous catheter (tip placed in the right atrium) became the routine after we lost a child of air embolism early in our experience. Nowadays, by and large, we are able to manage with two large bore peripheral lines, the central line is inserted only in complex cases and syndromic variety. Continuous infusion of propofol has also resulted in smoother induction, recovery and maintenance of anesthesia.

All children are extubated and sent to the ICU at the end of the procedure.

Duration of surgery

When the first time this surgery was performed in our hospital (it was the first time for all except the senior author), it took around 14 h! Now the operating time is around 3 to 3.5 h.

Technique

Orbitofrontal advancement with cranial remodelling is the procedure of choice in all – except the sagittal synostosis cases and the case of clover leaf deformity. In cases of sagittal synostosis, the sagittal suture is excised (with extensions into the coronal and lambdoid sutures), parietal bone barrel stave osteotomies are done and the occipital bossing is corrected. The child with clover leaf deformity needed bilateral decompressions - before orbito frontal advancement to manage the raised ICP.

Endoscopic surgery has not been done in any case.

For fixation, stainless steel wires (26 or 28 gauge) were used exclusively. However, we are using wires less and are using nonabsorbable and absorbable synthetic sutures more. The wires are used only at the key fixation points. Plates of any kind have not been used. The reasons are two fold: We feel fixation with wires is sufficiently rigid and the other reason is cost. Since insurance has not emerged in a big way in our country, the patients′ family have to pay for the treatment. The cost of wires is considerably less than a set of mini plates and the cost of a set of absorbable plates is more than the cost of the entire treatment using wires and sutures. There have been no problems in using wires – except a few instances where the wire had to be removed several months or years later because of protrusion through the skin.

Postoperatively, the children are nursed in the ICU at least overnight. Feeds are started as soon as the child is fully awake and alert. Postoperative blood transfusion is given only if the hemoglobin is below 8 g/l and or the PCV is below 24.

The children are discharged home as soon as the facial edema and swelling have subsided.

Complications

Our complication rates have been decreasing over the years.

Three children died and of whom two were during surgery. One child with advanced Cruzon syndrome died of hemorrhage and another with nonsyndromic synostosis died of air embolism. One may expect the intracranial pressure to be high and the risk of air embolism, therefore, to be insignificant but strangely the incidence of air embolism in craniofacial surgery is around 67%! This includes clinically significant and insignificant instances. One child with Cruzon syndrome died several months after surgery - after making an uneventful recovery from the surgery- of obstructive sleep apnoea.

Only one child had major infection. This responded to antibiotic therapy and daily irrigation of the wound with antibiotic and poviodine – iodine solution.

In a few, the wire penetrated through the skin and had to be removed.

Second surgery had to be done in three – one each for growing fracture, unacceptable correction and pseudomeningocele.

Discussion

The past two decades of surgery for craniostenosis have been a great learning – and humbling - experience. (“After having done many of these procedures as “a member of a team” during my tenure as a fellow in Pediatric Neurosurgery – with the boss always present to bail me out during times of crisis and trouble- doing these alone was scary in the beginning. More so because this was a completely new field for the rest of the doctors and staffs in the OR.”- CB) High speed drills, craniotomes, various kinds of saws with experienced helpful OR staff and doctors to help were sorely missed in the first few years. Surgery in the initial years was performed with the very dependable Hudson′s brace, Gigli saw and a motor (used by the dentists). Every one had to be taught every stage and the “next step” had to be explained well in advance.

Contrary to the – even now – widely held view that this is merely a cosmetic surgery, we no longer consider this to be a surgery to make a child look pretty. This is explained clearly and in no uncertain terms to the parents. The parents are made to understand the surgery is done for the following reasons:

manage raised ICP

restore normal brain growth

restore normal facial growth (if facial deformities are present)

manage proptosis (and poor vision) if present

improve the self image and esteem of the child as he/she grows and attends school

The parents understand the above well – thanks to consultations with other doctors and the Internet!

But the war has not been won – the battle still rages. The misconception that this is merely a cosmetic surgery still persists even among the medical fraternity. This is one of main issues that we faced in the early days. Thankfully, this is not so prevalent now. The expectations of some of the parents are high; some expect their children to look like movie stars! But some patients′ explanation resolves this issue in many.

We have noticed that the cranial and facial changes evolve over a period of one year after surgery and the appearance at the end of the first year is more or less what one can expect to be the final result.

Matson recommended the child be operated upon as soon as he/she is fit for surgery. Although the bone is very malleable in the first 2-3 months, rigid fixation may not be possible. The ideal age is around six months, since the bone is quite malleable and rigid fixation is possible. So we operate around that age.

We have climbed a steep learning curve. Every case is a new learning and teaching experience. The technique has evolved over the years and modifications to the correction technique are made on a case-by-case basis.

As far as the parents are concerned, the overall satisfaction rate is “good to excellent”. The difference after surgery is significant and on long-term follow up the results have been excellent. What the parents report very often is that the child has become more “naughty and mischievous” after the surgery! No reasonable explanation can be given for this apart from postulating that the child has become more active after the normalization of the ICP. Another interesting observation – again no explanation is available – is that the parents say, “Doctor, the child looks fine when I look at him(her) but when I look at the reflection in the mirror the deformity seems to have returned”.!

Conclusion

The surgery for craniostenosis has taught us a lot. We have come a long way. The future will be in a greater team effort to ensure a better outcome.

The team has risen up to the challenges and now every one looks forward to this procedure. No longer do we say, “Oh no not another marathon surgery” but “we are ready”.

Same child [Figure 1] with growing fracture

Figure 1

Apert syndrome

Oxycephaly (pre-op)

Oxycephaly (3 years post-op)

Clover leaf deformity

Cruzon syndrome (The mother has Cruzon syndrome too. She has had bitemporal decompressions in childhood)

Pfeiffer syndrome