The standard treatment is either bilateral or unilateral supraorbital bar advancement and reshaping within the first year of life. The bilateral advancement is used more frequently and is the procedure of choice when the deformity is more severe, when it extends across the midline, and there is contralateral frontal bossing.
For this procedure the coronal approach is used.
After the exposure of the forehead and the orbits, a bifrontal craniotomy is outlined. It starts 1 cm above the superior orbital rim and extends to behind the coronal suture on the affected side and to just behind the lateral orbital rim on the contralateral side (insert)
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.
The supraorbital bar is then osteotomized.
A vertical osteotomy near the pterion (1) on the affected side is followed by a horizontal osteotomy to the lateral orbital rim (2). An oblique osteotomy is then made through the orbital rim (3) and a transverse osteotomy is completed at the nasal frontal junction (4).
Alternatively, a tongue-in-groove or step osteotomy can be used in the temporal region of the affected side as shown.
On the non-affected side, the osteotomy typically begins just behind the lateral orbital rim as a vertical osteotomy and is made similarly through the rim.
With the brain carefully retracted, a right angle saw is then turned intracranially and the orbital roof is osteotomized beginning at pterion laterally on the affected side and ending at the nasal frontal region medially, joining the osteotomy made extra cranially across the nasal frontal region. On the non-affected side the orbital roof osteotomy begins anterior to the pterion and ends medially as above.
An osteotome is then inserted at the pterion and the lateral orbital wall osteotomy completed on both sides, releasing the bandeau.
The bandeau will typically need to be reshaped on the affected side by making a closing wedge ostectomy either in the middle segment of the superior orbital rim or at the junction of the superior orbital rim and the temporal bone, or both. The sites of the osteotomies are chosen in order to get symmetric reshaping of the bandeau.
These are stabilized with sutures, wires, or resorbable plates (preferably), which can be placed intracranially or extra cranially. In adults titanium fixation is an alternative.
The supraorbital bar is then advanced 10-15 mm on the side of the fusion, hinging at the point just behind the orbital rim on the uninvolved side. In infants an overcorrected advancement is usually made, as there will be some recurrence of the deformity with growth.
In adults who have finished craniofacial growth, advancement is done so that the superior orbital rim is approximately 12 mm anterior to the cornea, and equal or symmetric with the uninvolved opposite side.
The key is to try to straighten the bandeau so that both sides are equal and symmetric, employing osteotomies and fixation wherever required to make this possible.
The advanced bar is then stabilized. A wire or suture is placed between the stable lateral orbital rim and the bandeau.
Pearl: To improve the stability of the advanced segment, a cranial bone graft may also be wedged and placed in the orbital roof between the stable posterior segment and the advanced anterior segment on the affected side.
Bone graft harvested from the frontal bone flap is then inserted in the temporal gap and held in position with a resorbable plate (infants and children) or metallic plate (adults).
If metallic or resorbable plate fixation is unavailable, self-retaining osteotomies may be designed and utilized in conjunction with wire or suture fixation.
The frontal bone flap is then typically split down the midline and reshaped with a bone bending forceps (children) or with a partial osteotomies and plate fixation (adults).
Usually the right and left flaps are rotated, bent contoured and switched to get the optimal symmetry.
The bone flaps are affixed to the supraorbital bar with resorbable plates or resorbable sutures (infants), or titanium plates (adults).
The coronal bone gap created from the advancement and harvesting of bone 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.
This picture demonstrates the large volume of particulate bone that can be harvested from the inner surface of a bone flap using a hand-held burr-hole instrument.
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.