Cardinal features of the Syndromic cranial synostosis are:
Most of these patients have bicoronal synostosis but multiple cranial sutures in different combinations may also be involved.
Most children therefore undergo frontal/superior orbital rim advancement in infancy with repeat advancement as needed. (link to frontal orbital advancement, same as the bicoronal)
For the midface deformity, a Le Fort III or monoblock osteotomy is required.
A monoblock osteotomy done transcranially is utilized when the forehead, orbits, and midface all require a similar magnitude of advancement.
The "standard" Le Fort III osteotomy is performed when the superior orbital rim and forehead are in a satisfactory position and only the midface requires advancement.
The Le Fort III osteotomy using conventional fixation and bone grafts is usually performed after craniofacial growth is complete, although some proponents do it during the period of growth.
More often the Le Fort III osteotomy is done via distraction osteogenesis in the younger patients in order to overcorrect the deformity, reduce complications, obviate the need for bone grafts, and hopefully reduces the total number of operations the child might need.
The conventional monoblock osteotomy can also be done as a single stage or…
…via distraction in children up to age 6-8 years. Single stage advancements are not often done after this age due to the excessive risk of complications (infections, CSF leak, etc.).
The patient is prepared for surgery with presurgical orthodontia and fabrication of an occlusal splint to establish a Class I relationship or, if overcorrection is desired, the desired occlusal relationship.
The patient is placed in a supine position on a well-padded headrest.
Nasal intubation is required as the patient will be placed in temporary MMF during the procedure.
The osteotomy is approached through a coronal incision with or without an upper buccal sulcus incision. Extensive dissection of the orbit (deep orbit, anterior and posterior to medial canthus which remains attached), zygoma, midface, and nose is required.
Some surgeons use a lower lid incision as well, but if this can be avoided fewer complications of lid malposition will result. Similarly the osteotomies can usually be done without a buccal sulcus incision.
The following pages provide general information regarding orbital anatomy and dissection
After the exposure of the forehead and the orbits, a bifrontal craniotomy beginning 1 cm above the superior orbital rim and extending to behind the coronal sutures is outlined.
Burr holes are first placed at the vertex, avoiding the sagittal sinus, and nasal frontal region as well as temporally. An epidural dissection between these points is made.
The neurosurgeon then completes the osteotomies using a craniotome.
After the bone flap is removed, the dura is freed from the anterior and middle fossae in the epidural plane.
The dura is protected with neurosurgical cottonoids.
Malleable retractors are used intracranially to retract and protect the dura and intraorbitally to protect the orbital contents when performing the osteotomies.
Vertical osteotomies are made just behind the lateral orbital rims and down through the lateral orbital wall with a saw.
A vertical osteotomy using a saw at the junction of the zygomatic arch and zygomatic body is completed.
With the brain carefully retracted, a right angle saw is turned intracranially and the orbital roof is osteotomized beginning at the lateral wall osteotomy and ending at the cribriform plate.
An osteotome is inserted from intracranially and the medial orbit is osteotomized behind to the canthus and on to the medial floor.
An osteotome is used to perform osteotomies across the orbital floor anterior to the inferior orbital fissure, connecting to the medial wall osteotomies.
The osteotomy through the pterygomaxillary junction is completed with a curved osteotome from the infratemporal fossa or transorally if an upper buccal sulcus incision has been made.
To assure that the osteotome does not hit vital structures, one usually guides the direction of the osteotome with one hand while a palpating finger is placed medially at the posterior edge of the palate.
The final osteotomy is made from intracranially and goes from the cribriform plate through the posterior septum to the level of the palate.
It is safest to place a finger in the mouth at the posterior palate to assure the osteotome does not go too deep or penetrate the mucosa.
Disimpaction forceps are then introduced and the midface is down- and out-fractured, mobilizing it completely.
The patient is then placed in MMF using the occlusal splint and stainless steel wire loops.
Once the desired vertical dimension is established, fixation with titanium plates and screws (1.5 or 2.0 mm) at the zygoma and temporal region secures the advancement.
If the monoblock procedure is being performed in a child above the age of 6-8 years as a single stage, there is a high risk of CSF leak and/or infection. Therefore the anterior cranial base in the midline is usually filled with a galea frontalis flap and tissue sealant (eg, Fibrinogen etc.).
This may require cutting a small notch out of the frontal bone to prevent constriction of the flap.
The bony gaps are then filled with bone grafts wedged into position and secured to the plate. Inlay bone grafts may also be placed along the orbital floor.
The frontal bone flap is then replaced and affixed to the supraorbital rim with titanium plates and screws.
The coronal bone gap created from the advancement is then filled with particulate bone shavings harvested with a manual hand-held burr-hole instrument or commercial harvester (eg. Safe scraper) from the frontal bone flap or the posterior skull.
Most patients with Syndromic synostosis have canthal malposition so a lateral canthopexy in an overcorrected superior position is completed prior to closure.
If the advancement is thought to be secure, the MMF may be removed, but most surgeons prefer a period of 3-6 weeks of MMF.
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.
Postoperative examination by an ophthalmologist may be requested, although sever periorbital edema may prevent useful assessment. The following signs and symptoms are usually evaluated:
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.
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.