Total vault reshaping

Premature closure of the sagittal suture results in scaphocephaly (dolichocephaly) or a boat shaped head. There may be a great deal of variability in the head shape, depending upon whether the closure started posteriorly or anteriorly and at what age. Consequently the occiput may be more affected than the frontal region, or vice versa. Commonly both are affected. The general goal of surgery is to shorten the skull in a sagittal plane and widen it in a coronal plane.

When the occipital region is significantly affected and the anterior is not, posterior vault reshaping alone may be all that is required.

Similarly if the deformity is mostly frontal, correction may be performed here only.

Most often both the anterior and the posterior skull need reshaping, and this can be done as a single stage or two separate stages.

Single stage total calvarial reshaping is usually confined to younger patients with less severe deformities.

When the deformity is more severe and one has to access the orbits anteriorly or the base of the occiput posteriorly, staged procedures are preferred.

With the patient in the sphinx or "sniffing" position anterior and posterior exposure of the total calvarial vault is made via a coronal incision.

After dissecting as far posteriorly as possible and into the superior orbits anteriorly, the skull is systematically disassembled through a series of craniotomies.

Barrel-stave or similar osteotomies are performed laterally and the bone flaps are bent shaped and replaced to give shortening in a sagittal plane and widening in the coronal plane. The supraorbital bandeau might also be widened.

Numerous techniques have been described to accomplish this. A comprehensive description of all these techniques is beyond the scope of this reference, but examples are illustrated.

More recently springs, endoscopic techniques and cranial distraction have been used. These are described in a separate procedure called " Early surgical treatment".

Most surgeons favors placement of a bulb suction drain under the scalp for 3-5 days. Resorbable skin sutures are often used.

A circumferential head dressing is utilized for 48 hours. The neurosurgeon may request placement of a lumbar drain if significant dural tears have occurred during surgery. Patients should spend at least 1-2 days in an intensive care unit for neurological monitoring.

Keeping the patient’s head in an upright position postoperatively may significantly improve periorbital edema and pain. Some surgeons use injectable corticosteroids during surgery to reduce periorbital swelling.

To prevent orbital emphysema, nose-blowing should be avoided for at least 10 days.

The following perioperative medications are controversial. There is little evidence to make strong recommendations for postoperative care.

• Avoidance of aspirin or nonsteroidal antiinflammatory drugs (NSAIDs) for 7 days.
• Analgesia as necessary.
• Antibiotics (many surgeons use perioperative antibiotics. There is no clear advantage of any one antibiotic, and the recommended duration of treatment is debatable.).
• Nasal decongestant may be helpful for symptomatic improvement in some patients.
• Steroids may help with postoperative edema. Some surgeons have noted increased complications with perioperative steroids.
• Ophthalmic ointment should follow local and approved hospital protocol. This is not generally required in case of periorbital edema. Some surgeons prefer it. Some ointments have been found to cause significant conjunctival irritation.
• Regular perioral and oral wound care has to include disinfectant mouth rinse, lip care if intraoral incision has been used.

Postoperative examination by an ophthalmologist may be requested, although sever periorbital edema may prevent useful assessment. The following signs and symptoms are usually evaluated:

• Vision
• Extraocular motion
• Diplopia
• Globe position
• Lid position

Postoperative imaging has to be performed within the first days after surgery to verify accuracy of surgery. 3-D imaging (CT, cone beam) is recommended.

Remove sutures from skin after approximately 7-10 days if nonresorbable sutures have been used.
Apply ice packs for the first 12 postoperative hours as able although infants and young children do not tolerate this well (may be effective in a short term to minimize edema).
Avoid sun exposure and tanning to skin incisions for several months.

Soft diet can be taken as tolerated until there has been adequate healing of any maxillary vestibular incision. In children and infants age appropriate diets are then prescribed.
Patients in MMF will remain on a liquid diet until such time the MMF is released.

Clinical follow-up depends on the complexity of the surgery, and whether the patient has any postoperative problems. Most patients are discharged at postoperative day 3-5 and seen again in 2-3 weeks.

• In patients undergoing monoblock or Le Fort III distraction, distraction typically begins at day five at 1 mm/day and is assessed weekly with plane radiographs and clinical examination until the desired position is reached. After advancement a period of consolidation of 1-3 months is recommended before the retractors are removed.
• In patients undergoing conventional advancement with intermaxillary fixation, MMF is kept in place for 4-6 weeks. Routine oral hygiene is prescribed. Patients with arch bars and/or intraoral incisions and/or wounds must be instructed in appropriate oral hygiene procedures. The presence of the arch bars or elastics makes this a more difficult procedure. A soft toothbrush (dipped in warm water to make it softer) should be used to clean the surfaces of the teeth and arch bars. Elastics are removed for oral hygiene procedures. Chlorhexidine oral rinses should be prescribed and used at least 3 times a day to help sanitize the mouth.
  For larger debris, a 1:1 mixture of hydrogen peroxide/chlorhexidine can be used. The bubbling action of the hydrogen peroxide helps remove debris. A Waterpik® is a very useful tool to help remove debris from the wires. If a Waterpik is used, care should be taken not to direct the jet stream directly over intraoral incisions as this may lead to wound dehiscence.

The patient needs to be examined and reassessed regularly and often. Additionally, ophthalmological, ENT, and neurological/neurosurgical examination may be necessary. If any clinical signs for meningitis or mental disturbances develop, professional help has to be sought. Due to the young age of many patients, routing CT-scans are performed only if clinically indicated to avoid excessive radiation exposure.