Incision Placement and Soft-Tissue Management

For the exposure of the craniofacial skeleton above the Le Fort I level, the approach used is the coronal skin incision. This allows for relatively camouflaged access to the anterior and posterior cranial vault, the orbits, the nasal dorsum, the zygomas, the upper maxilla, the pterygoid fossa, and the temporomandibular joints. For added cosmetic advantage, the placement of the coronal incision more posteriorly on the scalp and with postauricular rather than preauricular extensions is useful.²¹⁴ When the exposure of the maxilla at the Le Fort I level is required, a circumvestibular maxillary intraoral incision is used. Unless complications occur that warrant unusual exposure, no other incisions are required to manage any aspect of reconstruction in patients with a craniosynostosis syndrome. These incisions (i.e., coronal [scalp] and maxillary [circumvestibular]) may be reopened as needed to further complete the individual’s staged reconstruction.

A layered closure of the coronal incision (i.e., the galea and the skin) optimizes healing and limits scar widening. Resuspension of the midface periosteum to the temporalis fascia may facilitate the redraping of the soft tissues. Each lateral canthus should be reattached in a superoposterior direction to the newly repositioned lateral orbital rim. In children, the use of chromic gut on the skin may obviate the need for postoperative suture or staple removal.

Management of the Ventricular System

In the patient with a craniosynostosis syndrome who is to undergo intracranial volume expansion with the concurrent management of hydrocephalus as part of the reconstruction, the potential for morbidity increases.25,77,91,108,170,174 Complications may arise from either excessive cerebrospinal fluid drainage (overshunting) or inadequate shunting that leads to increased intracranial pressure. With overshunting there is decreased central nervous system mass to fill any surgically created retrofrontal dead space. Uncontrolled hydrocephalus may result in raised intracranial pressure, which leads to its own set of problems. In either situation, fronto–facial advancement via an intracranial approach to the midface or isolated cranial vault expansion procedures should be thoughtfully staged with ventriculoperitoneal shunt management. We believe that the presence or absence of a ventriculoperitoneal shunt is not in itself a major factor in the success of a fronto–facial advancement procedure. An important aspect is the satisfactory physiologic function of the ventricular system, with or without the placement of a shunt. Ultimately, the decision regarding the need for and sequencing of shunting is based on the patient’s neurologic findings and the neurosurgeon’s judgment. In a patient with a ventriculoperitoneal shunt in place before the surgery, experienced neurosurgical evaluation, including imaging studies of the ventricular system, is carried out to confirm satisfactory physiologic function.

Upper Midface Reconstruction Options

The osteotomy selected (i.e., extra cranial Le Fort III or intracranial, monobloc or facial bipartition) to manage the upper midface dysmorphology in the individual with a craniosynostosis syndrome should reflect the presenting skeletal deformities and provide a realistic opportunity for long term upper midface (naso-orbito-malar) region aesthetic enhancement.

A main objective of this phase of reconstruction is to “normalize” the orbits, zygomas, and cranial vault. Correction of the maxillo-mandibular deformity will require orthognathic surgery including at a minimum a separate Le Fort I osteotomy. The patient specific dysmorphology will depend on the original malformation, the previous procedures carried out, and the effects of ongoing growth.

When evaluating the upper and midface morphology in the mixed dentition child or young adult born with Crouzon syndrome, one should note: 1) if the supraorbital ridge is in good position when viewed from the sagittal plane (are the depth of the upper orbits adequate?); 2) if the midface and forehead have an acceptable arc of rotation in the transverse plane (is the midface arc concave?); and 3) if the root of the nose and orbits are of normal width (is there orbital hypertelorism?). If these structures are confirmed to be with acceptable morphology, then there is no need to further reconstruct the forehead and upper orbits. In those few, craniosynostosis syndrome patients, in whom the residual deformity is only in the lower half of the orbits, the zygomatic buttress, and the maxilla an extracranial Le Fort III osteotomy is likely to be effective treatment.

If the supraorbital region, anterior cranial base, zygomas, root of the nose as well as the lower orbits, and maxilla all remain deficient in the sagittal plane (horizontal retrusion), then an MB is indicated. In these patients, the forehead is also generally flat and retruded and will require reshaping and advancement. If upper midface hypertelorism (increased transverse width) with midface flattening (horizontal retrusion) and a concave facial curvature (reverse facial arc) is also present, then the MB unit is split vertically in the midline (facial bipartition). A wedge of interorbital (nasal and ethmoidal) bone is removed, and the orbits and zygomas are repositioned medially while the maxillary arch is widened. A facial bipartition is rarely required in Crouzon syndrome but the MB is. When a monobloc or facial bipartition osteotomy is carried out as the “upper midface” procedure, additional segmentation of the upper and lateral orbits may also be required to normalize the morphology of the orbital aesthetic units.

For almost all Apert syndrome patients, facial bipartition osteotomies combined with further cranial vault reshaping permits a better correction of the dysmorphology than can be achieved through any other upper midface procedure (i.e., MB or Le Fort III). When using facial bipartition osteotomies, correction of the concave midface arc of rotation is also possible. This further reduces the stigmata of the Apert “flat, wide and retrusive” facial appearance. The facial bipartition allows the orbits and zygomas to shift to the midline (correction of hypertelorism) as units while the maxilla is simultaneously widened (i.e., relief of the V-shaped face). Horizontal advancement of the reassembled upper midface complex is then possible to improve orbital depth and zygomatic length. The forehead is generally flat, tall, and retruded, with a constricting band just above the supraorbital ridge. Reshaping of the anterior cranial vault is also simultaneously carried out. A Le Fort III osteotomy is virtually never adequate for an ideal correction of the residual upper midface anomalies documented in Apert syndrome.

The study by McCarthy confirms that the Le Fort III osteotomy is not effective as an aesthetic option to manage the upper midface deformity in the majority of craniosynostosis syndrome patients (see section later in this chapter concerning Le Fort III option).²⁷⁶ By anatomic design, the Le Fort III prevents management of the whole orbital aesthetic unit during one operative setting. Therefore a major aesthetic shortcoming of the Le Fort III osteotomy, when its indications do not fit the presenting dysmorphology, is the creation of irregular step-offs in the lateral orbital rims. This will occur even when only a moderate Le Fort III advancement is carried out. These lateral orbital step-offs will be visible to the casual observer as unattractive at conversational distance and surgical attempts at modification performed later will be suboptimal. Another problem with the Le Fort III osteotomy is the difficulty in judging an ideal orbital depth. This frequently results in either residual proptosis or enophthalmos. Simultaneous correction of upper face (orbital) hypertelorism and the concave midface arc-of-rotation typical in Apert syndrome is also not possible with the Le Fort III procedure. Excessive lengthening of the nose, accompanied by flattening of the nasofrontal angle, will also occur if the Le Fort III osteotomy is selected when the skeletal dysmorphology favors an MB or facial bipartition. Unfortunately, it is not possible to later correct the elongated nose or the flattened naso-frontal angle. To avoid these short comings, it is not a matter of becoming more proficient at the Le Fort III osteotomy or simply “managing the overlying soft tissues” in a different way (i.e., canthopeties or midface lift). The Le Fort III osteotomy is not consistent with the presenting dysmorphology in most craniosynostosis syndrome patients and, therefore, will not provide the opportunity to achieve the desired aesthetic result. Nevertheless, the Le Fort III is often considererd the “go to” approach by surgeons as it is: 1) an extracranial procedure; 2) requires less surgeon skill/experience; 3) is less likely to result in significant blood loss; and 4) is less likely to result in perioperative complications (i.e., cranionasal fistula, intracranial abscess, bone resorption).

In most syndromal craniosynostosis patients, a suboptimal aesthetic result will occur if the surgeon attempts to simultaneously adjust the orbits and idealize the occlusion using the Le Fort III, MB or facial bipartition osteotomy without completing a separate Le Fort I osteotomy. The degree of horizontal deficiency observed at the orbits and at the maxillary dentition are rarely uniform. If a Le Fort I osteotomy to separate the “lower midface” from the “upper midface” complex is not carried out, excess advancement at the orbits with enophthalmos is likely to occur as the surgeon attempts to achieve a positive overjet at the incisors. The Le Fort I osteotomy is generally not performed at the time of the “upper midface” procedure. This will await skeletal maturity and is combined with orthodontic treatment. Until then, a degree of an Angle class III anterior open bite negative overjet malocclusion will remain. When the mature teenage or adult patient presents for surgical correction and requires both “upper midface” (i.e., naso-orbito-zygomatic) and “lower midface” (i.e., maxilla) management the procedures may be carried out simultaneously.

Monobloc and Facial Bipartition Osteotomies (Intracranial Approach to the Orbits and the Midface)

Cranial vault reconstruction in the child with a craniosynostosis syndrome should provide space for the compressed brain to expand. Immediately after completing cranial vault and monobloc (MB) or facial bipartition (FB) osteotomies with advancement, extradural (retrofrontal) dead space remains in the anterior cranial fossa above the skull base gap created by the osteotomy.²⁰⁸ The skull base gap is in direct communication with the nasal cavity. Therefore, the postoperative recovery may be complicated by cerebrospinal fluid leakage across the skull base gap followed by infection, fistula formation, and subsequent bone resorption in the glabella region. After fronto–facial advancement by either MB or FB, the communication between the nasal cavity and the cranial fossa must be managed to limit these potential complications. The most effective method to do so remains unclear, but all agree that it is a critical aspect for successful reconstruction. Technical aspects of management include the following: 1) gentle tissue handling; 2) achieving good hemostasis; 3) effective repair of dural tears; 4) avoidance of over expansion of the cranial vault; 5) maximum separation of dura and nasal mucosal tissue planes (i.e., interposing tissue such as bone grafts, tissue sealants, and flaps); 6) rigid plate and screw fixation of the osteotomies and bone segments; 7) avoidance of pressure gradients across the opening to facilitate nasal mucosa healing; and 8) prevention of too much or too little ventriculoperitoneal shunting.

After cranio–orbital reconstruction in the infant with a craniosynostosis syndrome, rapid filling of the expanded intracranial volume by the previously compressed frontal lobes of the brain has been documented.²⁰⁸ This has also been shown to occur after MB and FB advancement in children and young adults.²⁴⁹ More gradual filling of the space is thought to occur in older adults. At the time of the MB or FB osteotomies, the nasal cavity can be sealed from the cranial fossa by doing the following: 1) inserting pericranial tissue; 2) placing bone grafts to bridge the osteotomy gaps; and 3) using tissue sealants. This provides time for the re-epithelialization (i.e., healing) of the nasal mucosa. Until the torn nasal mucosa heals, communication between the nasal cavity and the anterior cranial fossa may result in the transfer of air, fluid, and bacteria followed by infection and naso–cranial fistula formation. The postoperative continuation of nasotracheal intubation for several days and nasopharyngeal tube placement after extubation have also proven useful to limit pressure gradients across the communication. The avoidance of positive pressure ventilation, the enforcement of sinus precautions, and the restriction of nose blowing further limits the reflux of air, fluid, and bacteria early after surgery.

Four clinical studies clarify issues of morbidity related to MB or FB osteotomies for the reconstruction of an individual with a craniosynostosis syndrome.

Posnick and colleagues studied the issues of retrofrontal dead space; communication across the skull base osteotomy gap; and associated morbidity in a consecutive series of children in the mixed dentition and young adults in the permanent dentition (n = 23) who were undergoing either MB or FB osteotomies in combination with cranial vault expansion.²⁰⁸ The procedures were carried out by a single craniofacial surgeon (Posnick) and one of three neurosurgeons at a single tertiary care hospital from 1987 to 1991. The extradural (retrofrontal) dead space was measured from consistent CT scan images at specific postoperative intervals (immediate, 6 to 8 weeks, and 1 year). The study confirmed the presence of an immediate retrofrontal dead space that generally filled in with the expanding brain and dura by 6 to 8 weeks after surgery. Specific intraoperative maneuvers were undertaken by the surgeons to close the nasofrontal communication, including flaps, fibrin glue, bone grafts, and Gelfoam. After surgery, care was taken to limit a pressure gradient across the communication (via the repair of dural tears, sinus precautions, and nasal stenting), with the objective of providing time for nasal mucosa healing. The infection rate in this study group was limited to 2 of 23 patients (9%). In both patients who developed an infection, a retrofrontal (extradural) fluid collection was noted, with drainage across the residual nasofrontal communication into the nose. Both patients healed without significant comorbidity (i.e., brain or eye injury) but did require further reconstruction of the resorbed portions of the anterior cranial vault and the supraorbital ridges (see Fig. 30-15).

Wolfe completed a critical analysis of 81 MB advancements carried out over a 27-year period.²⁸¹ This was a retrospective chart analysis of a series of patients who underwent either MB (frontofacial) advancement (MFFA) or FB (MFFA plus FB). The procedures were carried out at seven different craniofacial centers and included 49 MFFA and 32 MFFA plus FB. The MFFA and MFFA plus FB osteotomies were either placed in their preferred location in the operating room (standard approach) or gradually distracted (DO technique) with internal or external devices. Complications included two deaths (cardiac arrest in one patient and complications arising from hypovolemia in the other). One case was aborted as a result of a large-volume blood loss; there were three infections or sequestrations and one persistent cerebrospinal fluid leak (no meningitis). There were significant complications documented in the DO group and fewer in the non-DO group. Blood loss and operative time were equivalent for both standard and DO techniques. Interestingly, the incidence of infection and cerebrospinal fluid leaks was not diminished with the use of the alternative distraction DO approach. The author also concluded that, for the majority of patients, the standard approach offered improved morphologic results. The author then compared the morphologic results of the MMFA and MMFA plus FB with those of the extracranial Le Fort III option and concluded that the Le Fort III was less favorable. Regardless of the technique used, all patients required orthognathic surgery (at the Le Fort I level) to complete the reconstruction.

Bradley and colleagues completed a single-center retrospective study that compared differences in morbidity in a series of patients who were born with a craniosynostosis syndrome and who then underwent MB osteotomy for the correction of upper and midface anomalies or hypoplasia.¹⁹ The authors describe three different sequential treatment groups that were followed during a period of 23 years. Group I patients (1979 to 1989; n = 12) underwent MB osteotomies without any special attention paid to the retrofrontal dead space or the communication through the skull base between the anterior cranial fossa and the nasal cavity. Group II patients (1989 to 1995; n = 11) underwent MB osteotomies with various attempts at closure of the skull base gap with pericranial flaps and fibrin glue. Group III (1995 to 2002; n = 24) patients underwent MB osteotomies without immediate advancement. An internal distraction device was placed across the osteotomized zygomatic arch on each side. After a 7-day latency period, the advancement of the MB and forehead unit was initiated at 1 mm per day for approximately 2 to 4 weeks. The infection rate for Group III patients was significantly lower (2 of 24 [8%]) than it was for those in Groups I and II. Neither of the two infections in Group III resulted in bone loss. Group I patients had the greatest morbidity likely as a result of the limited fixation performed during the 1970s and 1980s.

As described by Bradley and colleagues, the DO technique does allow more time for the brain to expand into the retrofrontal dead space after the completion of an MB osteotomy (i.e. delayed for 7 days) before advancing the upper midface.¹⁹ In theory, this should facilitate early nasal mucosa healing and thereby limit the communication of fluid, air, and bacteria across the surgically created skull base gap. This likely explains the drop in infection rate in the Group III patients as compared with the Group I and II patients. The rate of infection in Bradley’s Group III patients, who were treated with a DO approach, essentially matches that of the patients described by Posnick and colleagues who were treated with a standard approach (8% and 9%, respectively).

Bradley and colleagues also described greater advancement in Group III (DO approach) patients as compared with Group I and II patients.¹⁹ Confounding variables may explain these differences, including increased surgical experience during the later years of the study (i.e., group III patients) and the impact of complications during the earlier years of the study (i.e., extremely high rates of infection in Groups I and II), which likely resulted in relapse and limited the long-term midface advancement. More importantly, there was no correlation between the number of millimeters of MB advancement and either greater functional gains or enhanced facial aesthetics.

A fourth clinical study sheds further light on this subject. Ahmad and colleagues described a series of 12 children who were born with a craniosynostosis syndrome who also had multiple functional problems, including the following: 1) raised intracranial pressure as a result of a diminished cranial vault volume; 2) exposure of the eyes (i.e., corneal irritation) as a result of shallow orbits; 3) airway obstruction from a reduced upper airway space (i.e. OSA); and 4) feeding difficulties.³ Each of the study patients underwent fronto–facial (MB) advancement with the use of DO techniques. The mean age at operation was 18 months (range, 4 to 30 months). The mean advancement was 16.6 mm at the forehead level and 17 mm at the midface level. Ocular protection and the reduction of intracranial pressure (when raised) was achieved in all children. At least a degree of airway improvement was achieved in all but one child. The authors subjectively state that there was marked improvement in every patient’s appearance. Complications included cerebrospinal fluid leaks (2 of 12 patients [16.6%]); pin-site infections (3 of 12 patients [25%]); external DO device frame slippage that required replacement (2 of 12 patients [16.6%]); and overadvancement with resulting enophthalmos (1 of 12 patients [8.4%]). In addition, one patient died 9 months after the procedure in conjunction with tracheal reconstruction. The published article was discussed by Hopper,¹¹¹ who recommends that clinicians consider a less invasive approach and accept limited aesthetics objectives in an attempt to lower perioperative morbidity. The approach that he suggests includes the following: 1) either anterior cranial or posterior cranial vault expansion without simultaneous midface advancement to relieve intracranial pressure; 2) extracranial Le Fort III advancement without simultaneous cranial vault expansion to open the upper airway; 3) tarsorrhaphies to protect the eyes from exposure rather than orbital expansion; and 4) continued tracheostomy for airway management rather than midface advancement.

For the individual with a craniosynostosis syndrome, to achieve the most favorable facial harmony and head and neck function, the surgeon needs both an aesthetic sense and the technical expertise to execute effective upper/midface surgical procedures. Several key aspects include:

In current clinical practice when either a monobloc or facial bipartition osteotomy is selected, the technique of “distraction” to gradually advance the upper/midface, after closure of the wounds is frequently used. Any potential for reduced infection across the skull base using the DO technique should be considered in light of limitations to achieving the aforementioned aesthetic objectives (points 1 to 4 mentioned previously). At the same time, clinicians must take into account the procedure specific morbidity associated with the DO technique including: 1) pin tract infection; 2) soft tissue scarring; 3) hardware loosening requiring re-application; 4) brain trauma from the screw penetration; and 5) the need for device removal. 6) In addition, effective DO technique depends on the patient’s, the patient’s family, and the clinician’s continued commitment to staying the course after surgery (approximately 4 months). It is hoped that in the future, clinicians will both master the known surgical techniques and also find novel solutions to manage the potential for morbidity associated with MB and facial bipartition osteotomies (i.e., CSF leak, intracranial abscess, and bone resorption) while focusing to the aesthetic details so that individuals with a craniosynostosis syndrome can more commonly benefit from the procedures inherent advantages.

Le Fort III Osteotomy (Extracranial Approach to the Midface)

Shetye and colleagues set out to examine long-term (10-year) midface skeletal stability and the potential for maxillary growth after extracranial Le Fort III advancements in children with craniosynostosis syndromes.²⁴² Their study group included 25 children (10 had Crouzon syndrome, 9 had Apert syndrome, and 6 had Pfeiffer syndrome) who underwent extracranial Le Fort III advancement before they were 11 years old (mean, 5.8 years; range, 3.8 to 10.9 years). Two different forms of fixation were used: Group 1’s treatment included intraosseous wires, bone grafts, suspension wires, dental splint, and 8 weeks of intermaxillary fixation; Group 2’s treatment involved titanium plates and screws, bone grafts, dental splints, and elastics. Good stability of the midface was documented in both groups at 1 year, 5 years, and 10 years. As has been found with previously published studies, no significant horizontal growth occurred in the midface after surgery. The form of fixation used was not a factor (i.e., plate and screw fixation did not increase incidence of growth restriction). The mandible was measured at the pogonion and found to advance 5.72 mm and 7.32 mm at the 5- and 10-year time points, respectively. This study confirms that, when the Le Fort III advancement is carried out at a young age, the occurrence of jaw disharmony with ongoing growth is inevitable and will require further staged reconstruction.

In a second publication, Shetye and colleagues evaluated a larger group of syndromal craniosynostosis patients who also underwent extracranial Le Fort III advancement between the years of 1973 and 2006.²³⁸ Three different stabilization techniques were sequentially used over the study period: Group 1 (n = 20) was treated with intraosseous wires, suspension wires, bone grafts, dental splinting, and 8 weeks of intermaxillary fixation; Group 2 (n = 20) was treated with titanium plates and screws, bone grafts, dental splinting, and elastics; and Group 3 (n = 20) was treated with the extended use of an external DO device during 3 months of healing. The stability of midface advancement was assessed with the use of measurements from 1- and 5-year postoperative lateral cephalometric radiographs. Patients in Groups 1 and 3 showed equal levels of skeletal stability, whereas Group 2 showed slightly less stability at the A point. At the orbitale, all three groups showed similar levels of stability. Interestingly, all three methods of fixation for the extracranial Le Fort III advancements showed good levels of stability. The study also confirmed that all study subjects who were in the mixed dentition demonstrated progressive facial disharmony as a result of ongoing mandibular growth, thereby necessitating further skeletal reconstruction by the late teenage years (i.e., different forms of fixation had no bearing on the incidence of growth restriction).

In the discussion, McCarthy, who was the senior investigator, stated his personal preference for the management of the midface deformity in syndromal craniosynostosis with an extracranial Le Fort III advancement followed by orthognathic surgery. When early Le Fort III advancement is indicated in a growing child, he prefers distraction to standard techniques. According to McCarthy, it is easier to overcorrect the advancement with the use of DO, which is one of his stated objectives for young children (i.e., those who are <11 years old). Standard extracranial Le Fort III advancements with plate and screw fixation and bone grafts are preferred by McCarthy for older children and adults (i.e., those who are >11 years old), because this allows for reduced treatment time and less patient psychosocial issues (i.e., there is no need to wear an external DO device for 3 months after surgery).

McCarthy and colleagues recently reviewed the long-term midface growth and periorbital/malar (upper midface) aesthetic results in individuals who were born with a craniosynostosis syndrome and who had undergone Le Fort III advancement.²⁷⁶ The study represented a follow-up period of more than 20 years for four identified patients (one with Apert syndrome and three with Crouzon syndrome) who were 4, 6, 14, and 20 years old at the time of the Le Fort III advancement. Thus, this study represented two young children and two skeletally mature individuals who underwent Le Fort III midface advancement. The authors’ review confirmed several points: 1) a Le Fort III advancement carried out during early childhood results in no further horizontal growth of the midface; 2) a Le Fort III advancement carried out during early childhood results in further vertical growth of the midface with clockwise rotation of the mandible; and 3) a Le Fort III advancement carried out in individuals with either Crouzon or Apert syndrome—whether during childhood or adulthood—does not effectively correct the presenting zygomatic, orbital, and nasal malformations. The upper midface aesthetic results as viewed through the overlying soft-tissue envelope (i.e., the eyelid–cheek–malar region) were suboptimal after Le Fort III advancement. Furthermore, with age, the upper midface aesthetic results became even more unfavorable.

McCarthy’s findings of continued vertical growth of the midface after Le Fort III osteotomy carried out during early childhood is consistent with the inability to achieve adequate nasal airflow in the operated patient. In addition, even if adequate nasal airflow is accomplished, at least some individuals will have limited masticatory muscle tone. In either case, when a child continues with an open-mouth protrusive tongue posture, hypereruption (i.e., vertical growth) of the maxillary dentition through the alveolar bone with clockwise rotation of the mandible is expected (see Chapter 4). A lack of horizontal growth of the maxilla after either a Le Fort I, Le Fort III, or MB osteotomy is carried out during childhood has been documented by a number of independent investigators.^{31,50,75,76,102,103,112,192} This results from both intrinsic growth limitations of the malformation and injury to the sutural growth centers from the osteotomies that are carried out.

Step 1. Satisfactory airway management in a patient who is undergoing an MB/FB osteotomy is essential. A method that I have used effectively involves an orotracheal tube that is secured adjacent to the cusp edge of the mandibular incisors with a circummandibular wire (Fig. 30-16, A). After the MB/FB osteotomies and disimpaction are completed, a nasotracheal tube is placed, and the orotracheal tube is removed (Fig. 30-16, B). Through this controlled approach, endotracheal tube injury at the completion of the osteotomies and dislodgement during disimpaction are prevented. In addition, direct contact between the maxillary and mandibular teeth can be achieved for the improved control of the occlusion. The nasotracheal tube remains in place at the end of the procedure to allow for nasal mucosal stenting during the initial postoperative phase. Other approaches to managing the airway have been described and used effectively (e.g., tracheostomy, submental intubation, orotracheal intubation without exchange).

Step 2. When feasible, I recommend the placement of Crawford tubes to protect the nasolacrimal apparatus during surgery (Fig. 30-17). The puncta are dilated, and a probe is inserted through each punctum to confirm entrance into the nose. The Crawford tube is inserted through each punctum and pulled through the nose. Within the nose, the Silastic sheeting is stripped; the tubing is tied in the nose, and the excess is cut in the nose with scissors.

    Temporary tarsorrhaphies are placed with 6-0 nylon suture from gray line to gray line just lateral to the pupil of each eye. As an alternative, corneal shields may be used to protect the cornea, but this prevents the direct examination of the pupils during surgery.

Step 3. Surgical arch wires are generally applied (Fig. 30-18). A throat pack is placed, and the mouth is cleansed. Erich arch bars are applied to the maxillary and mandibular teeth. Circum-mandibular wires are placed to further stabilize the mandibular arch bar, and the orotracheal tube is secured with a wire to the symphyseal region of the surgical arch bar.

Step 4. The patient is prepared and draped. The patient’s head is placed in a Mayfield (horseshoe-shaped) head holder in the neutral neck position. Cleansing of the entire scalp is performed with povidone–iodine (Betadine) soap; the soap is then washed out with sterile water. Next, the application of povidone–iodine solution to the scalp, face, and neck is carried out. The surgical field is draped to expose the neck down to the clavicles; the full face, including the external ears; and the anterior scalp back to the planned incision. The separation of the mouth/nose from the eyes/forehead is achieved with an additional sterile towel drape; this is done to limit the contamination of the intracranial cavity with oral or nasal flora.

Step 5. A coronal (scalp) skin incision is completed (Fig. 30-19). The incision site is postauricular and posterior in the scalp. Other incision modifications have been described (e.g., Z-plasty in the temporal regions). Lidocaine with epinephrine is injected to facilitate hemostasis.

Step 6. The anterior scalp flap is then elevated by remaining deep to the superficial layer of the deep temporal fascia over the temporalis muscles; subperiosteal over the mid-forehead region; subperiosteal down the lateral orbital rims; subperiosteal with exposure of the anterior maxilla; subperiosteal for exposure of the zygomatic aches; and subperiosteal over the dorsum of the nose. Elevation of the temporalis muscles off of the squamous temporal bones is then carried out (Fig. 30-20).

Step 7. Bifrontal craniotomy is completed (Fig. 30-21, A). The craniotomy lines are drawn with a sterile pencil, and bur holes are completed with a perforator as needed. The craniotomies are completed with a craniotome. The frontal bones are separated from the underlying dura and then removed. The frontal and temporal lobes of the brain are adequately retracted to safely accomplish skull base osteotomies. The brain is protected with cottonoid pledgets (Fig. 30-21, B).

Step 8. Zygomatic arch osteotomies are performed (Fig. 30-22). Retractors are placed, and an osteotomy is completed through the midzygomatic arch on each side with a sagittal (reciprocating) saw.

Step 9. With the use of the sagittal saw, the lateral orbital wall osteotomy is initiated into the inferior orbital fissure. The osteotomy is extended superiorly through the lateral orbital wall (Fig. 30-23).

Step 10. With the continued use of the sagittal saw, the lateral tenon extension of the osteotomy through the squamous temporal bone and skull base is carried out (Fig. 30-24).

Step 11. The orbital roof osteotomy is completed with the sagittal saw through the anterior skull base (Fig. 30-25).

Step 12. The orbital roof osteotomy continues laterally through the sphenoid wing. This osteotomy will join up with the previous tenon extension on each side (Fig. 30-26).

Step 13. A thin chisel placed through the anterior skull base is used to confirm the completion of the sphenoid wing osteotomy and continuity with the tenon extension (Fig. 30-27).

Step 14. With a thin chisel and working through the skull base, the medial orbital wall osteotomy is completed posterior to the medial canthus and nasolacrimal apparatus and inferiorly into the inferior orbital fissure (Fig. 30-28).

Step 15. The anterior aspect of the nasal septum is separated from the cranial base. A straight 15-mm wide chisel is placed through the cranial base just anterior to the crista galli and used to complete this osteotomy and to further separate the midface from the base of the skull (Fig. 30-29).

Step 16. The separation of the pterygomaxillary sutures is completed. A long 10-mm wide chisel is placed through the coronal incision and the infratemporal fossa to the pterygomaxillary suture. One double-gloved hand is placed in the patient’s mouth, and the other hand is used to place the chisel through the coronal incision and into the infratemporal fossa (Fig. 30-30, A). A mallet is then used to separate the pterygomaxillary suture with the chisel. The success of the separation is confirmed with the pterygomaxillary spreader forceps (Fig. 30-30, B).

Step 17. The midface (MB) is disimpacted with the use of two nasomaxillary forceps that are placed in the nose and the mouth. Pterygomaxillary spreader forceps are simultaneously placed through the coronal incision on each side. The midface is then disimpacted and stretched forward to confirm adequate advancement at the occlusal level (Fig. 30-31). Next, the endotracheal airway exchange is completed (see Fig. 30-16, B). Additional sterile drapes are placed over the scalp and the face and neck regions, and the throat pack is removed. The surgeon places the nasotracheal tube through the nose and into the oropharynx. The anesthesiologist then removes the orotracheal tube and completes the insertion of the nasotracheal tube through the larynx using the direct or GlideScope laryngoscopic technique.

Step 18. For FB, a midnasal osteotomy (i.e., an ostectomy) is completed. Working through the coronal incision, the surgeon marks out the proposed midnasal osteotomy with calipers and a pencil and then completes it with a sagittal saw. When the ostectomy is completed, the removal of portions of the underlying cartilaginous nasal septum is also accomplished (Fig. 30-32).

Step 19. For FB, separate sterile oral instruments are used to split the maxilla into two segments. An intraoral maxillary vestibular incision is made, and this is followed by the subperiosteal exposure of the anterior maxilla, the anterior nasal spine, and the nasal floor (Fig. 30-33, A). With a sagittal saw, a midline osteotomy is completed between the central incisors and then parasagittally down the hard palate; the palatal mucosa is left undisturbed (Fig. 30-33, B). The segmental separation is completed with a thin 5-mm wide chisel placed between the central incisors (Fig. 30-33, C). The Erich arch bar is also cut between the incisors. Further separation of the posterior maxilla is completed with a spreader forceps as needed (Fig. 30-33, D). The oral wound is closed, the intraoral instruments are discarded, and fresh gloves are put on the hands.

Step 20. For FB, stabilization of the upper orbits and the nasal bones is completed next (Fig. 30-34, A, and B). Repositioning of the facial halves medially with correction of the midface arc of rotation is completed; this will require refinement with a rotary drill (Fig. 30-34, A). The upper orbits and the nasal bones are fixed with a titanium plate and screws (Fig. 30-34, B). A rotary drill is also used to cranialize the frontal sinus, if needed (Fig. 30-34, C).

Step 21. Midface advancement at the level of the maxillary dentition is accomplished (Fig. 30-35). Working through the coronal scalp incision, the primary surgeon advances the midface. With the use of separate sterile oral instruments, the assistant to the surgeon wires the jaws together into the planned occlusion.

Step 22. Upper midface advancement is established at the zygomatic arches (Fig. 30-36). The amount of advancement at each zygomatic arch is measured with a caliper. A titanium plate is conformed to extend from the anterior maxilla across the arch and surgical gap to the posterior zygoma on each side. The plate is secured with titanium screws.

Step 23. Additional segmental osteotomies of the orbits are often required. Occasionally, the lateral superior orbits have further dysplasia and segmental osteotomies with reshaping for reconstruction is necessary (Fig. 30-37, A). If so, the lateral orbital segments are removed with a reciprocating saw. Additional segmental orbital osteotomies are then completed with a reciprocating saw (Fig. 30-37, B). The lateral orbital rim and superior orbital rim segments are further reshaped with a rotary drill (Fig. 30-37, C). The segments are fixed with titanium plates and screws (Fig. 30-37, D).

Step 24. Stabilization of the midface advancement at the upper orbital tenon extension is accomplished (Fig. 30-38). The desired advancement is measured with a caliper; a miniplate is adapted to bridge the gap between the tenon extension and the posterior cranial vault. Titanium screws are used for plate stabilization.

Step 25. Hyperplastic ethmoidal air cells are debrided as needed in patients with orbital hypertelorism (Fig. 30-39). With visualization through the anterior cranial base, rongeurs are used to debride hyperplastic ethmoidal air cells and to reduce midorbital hypertelorism.

Step 26. The opening between the anterior cranial fossa and the nasal cavity is managed (Fig. 30-40, A and B). The surgeon irrigates and suctions the intranasal cavity through the anterior cranial base exposure. My usual approach is to then apply a sheet of Gelfoam to separate the opening between the two cavities (Fig. 30-40, A). I then inject fibrin glue over the Gelfoam to seal the separation (Fig. 30-40, B). Other methods of managing the separation between the nasal cavity and the anterior cranial fossa may be used, depending on the clinical circumstances (e.g., cranial bone grafts, soft-tissue flaps).

Step 27. The anterior cranial vault is reshaped, advanced, and secured in place (Fig. 30-41, A, B, and C). With the use of a reciprocating saw, osteotomies of the removed anterior cranial vault are completed (Fig. 30-41, A). Rotary drill recontouring is also accomplished to achieve the desired shape (Fig. 30-41, B). Split- or full-thickness cranial bone graft is harvested and used as needed to fill in defects, and fixation is accomplished with plates and screws. Split-thickness cranial bone graft is also placed and fixed in the midzygomatic arch segmental defects (Fig. 30-41, C).

Step 28. Lateral canthopexies are completed (Fig. 30-42). Two holes are drilled at each new frontozygomatic suture region. The lateral canthi are identified with a skin hook through the coronal incision. A figure-of-eight wire suture is placed through each lateral canthus through the coronal incision. Each lateral canthus is fixed by passing the wire through the drill holes in the frontozygomatic suture.

Step 29. Each temporalis muscle is resecured to bone (Fig. 30-43). The temporalis muscles are repositioned anteriorly and secured to the lateral orbital rims and the temporal bones with interrupted sutures.

Step 30. The scalp wound is closed (Fig. 30-44). Suction drains are placed through the posterior scalp flap; one is placed on each side. One drain is placed under the anterior flap, and the other is placed below the posterior flap. The galea closure is completed with interrupted sutures, and the skin layer closure is completed with staples or resorbable sutures in accordance with surgeon preference.

Quantitative Assessment of Presenting Crouzon Deformity and Surgical Results on the Basis of Computed Tomography Scan Analysis after First-Stage Cranio–Orbital Reconstruction

Waitzman and colleagues developed a method of analysis based on CT scan measurements and that allows for a quantitative assessment of the upper and midface skeleton in both the horizontal and transverse planes.272,273 This method of quantitative CT scan analysis was used to document the differences in the cranio–orbito–zygomatic region between children with Crouzon syndrome who had not yet undergone reconstruction and age-matched controls.²⁹ Morphologic results that were achieved in those children 1 year after undergoing standard suture release and anterior cranial vault and upper orbital procedures to reshape these regions were also evaluated.²¹⁶

The preoperative CT scan measurements of the children with untreated Crouzon syndrome confirmed a widened anterior cranial vault at 108% of normal and a cranial length that averaged only 92% of normal.²⁹ In comparison with age-matched controls, orbital measurements revealed a widened anterior interorbital width at 122% of normal, an increased intertemporal width at 121% of normal, globe protrusion at 119% of normal, and a short medial orbital wall length at only 86% of normal. The distance between the zygomatic buttresses and the interarch widths were found to be increased at 106% and 103% of normal, respectively. The zygomatic arch lengths were substantially shortened at only 87% of the values of age-matched controls. These findings confirmed clinical observations of brachycephalic anterior cranial vaults with shallow and frequently hyperteloric orbits and globe proptosis. In general, the upper midface in patients with Crouzon syndrome is horizontally retrusive and transversely wide, which is reflected in wide and shortened zygomas. The same quantitative CT scan assessment was carried out in the children with Crouzon syndrome more than 1 year after they underwent anterior cranial vault and upper orbital osteotomies with reshaping to compare their values to the new age-matched control values.²¹⁶ No significant improvement in the cranio–orbito–zygomatic measurements was demonstrated.

Quantitative Intracranial Volume Measurements Before and After Cranio–Orbital Reshaping in Children with Crouzon Syndrome

In a previous study, the intracranial volumes in children with Crouzon syndrome before and after standard cranio–orbital reshaping procedures was documents.²⁰⁹ The intracranial volumes were also compared with those of an age- and gender-matched cohort, and the rate of cranial expansion with growth was reviewed. The study included 13 children who presented sequentially with Crouzon syndrome and who subsequently underwent a standard first-stage cranio–orbital reconstruction by the author (Posnick) in conjunction with a pediatric neurosurgeon. The average age at the time of operation was 13 months (range, 6 to 46 months). Postoperative clinical follow up ranged from 12 to 60 months at the time of the study’s completion. Of the children with Crouzon syndrome who were evaluated preoperatively, 12 out of 13 had intracranial volume values that were greater than the mean. When comparing postoperative volumes with the normative data, all 13 maintained volumes that were at or greater than the mean. When reviewing the cranial capacity of each patient with Crouzon syndrome over time, 5 of the 13 patients approximated the normal growth curve, whereas 6 of the 13 patients exceeded it. According to the findings of the study, for the majority of children who were born with Crouzon syndrome, the cranial capacity will exceed the mean early during life and expand at a rapid rate in conjunction with cranio–orbital decompression and reconstruction. The biologic explanation for these unexpected findings remains unclear. It should be noted that other researchers have studied the intracranial volume of children who were born with craniosynostosis.^{18,20,21,24,60,64,67,73,80,85–87,93,137,140,141,210,246,247}

Quantitative Assessment of Presenting Deformity during the Mixed Dentition in Children with Crouzon Syndrome and Surgical Results after Monobloc Osteotomy on the Basis of Computed Tomography Scan Analysis

During their middle childhood years, a group of children with Crouzon syndrome were assessed via quantitative CT scan measurements. They were found to have cranial vault lengths that averaged only 87% of the values of the age-matched norms. The medial orbital walls were horizontally short at 87% of normal, whereas the extent of globe protrusion was excessive at 134% of age-matched norms. The zygomatic arch lengths averaged only 84% of normal. These findings confirmed horizontal anteroposterior deficiency of the upper face and the midface.

After they underwent MB osteotomies in combination with anterior cranial vault reshaping and advancement carried out through an intracranial approach, the children’s cranio–orbito–zygomatic measurements were re-evaluated. The mean cranial length that was initially achieved after MB osteotomy was 98%, and at 1 year it was 92% of the control value. As compared with age-matched controls, the orbital measurements reflected improvements in both the midorbital hypertelorism (the mid-interorbital width was 97% initially after operation and 102% at 1 year), and the orbital proptosis (early after surgery, their values were 86% of the values for age-matched normal children; they were 92% at 1 year). The medial orbital wall length initially normalized at 101% and later at 97% of normal values. The zygomatic arch length initially corrected at 106% and later to 101% of normal.

Quantitative Assessment of Presenting Apert Deformity and Surgical Results on the Basis of Computed Tomography Scan Analysis after First-Stage Cranio–Orbital Reconstruction

In a previously published study, a method of quantitative CT scan analysis was applied to document the differences in the cranio–orbito–zygomatic region between children with Apert syndrome who had not been operated on and age-matched controls.²⁹ Eight consecutive infants and young children with Apert syndrome who underwent a standard cranio–orbital procedure by a single craniofacial surgeon (Posnick) in conjunction with one of three pediatric neurosurgeons over a 4-year period were reviewed 1 year after surgery.²¹⁷ The series included seven girls and one boy, with an average age at surgery of 12 months (range, 9 to 23 months). The average postoperative follow-up period was 34 months (range, 12 to 48 months) at the close of the study. Preoperative and postoperative (>1 year) CT scans were compared with those of age-matched controls, and percentages of normal were then compared for significant differences. Two of the children had clear evidence of hydrocephalus and required ventriculoperitoneal shunting before the cranio–orbital reconstruction at 3 and 5 months of age. A third child had mildly increased ventricular size, but clinical correlation did not suggest the need for shunting.

Significant preoperative morphologic findings included a wide anterior cranial vault at 110% of normal, a maximum cranial length that averaged only 90% of normal, a substantially widened anterior interorbital width at 117% of normal, an increased lateral interorbital distance at 112% of normal, and a widened bitemporal width at 122% of normal.²¹⁷ Globe protrusion was significant at 121% of normal, and the medial orbital wall length was less than normal at 92%. In the upper midface zygomatic region, both the width between the zygomatic buttresses and the interarch width were found to be increased at 109% of normal, whereas the zygomatic arch lengths were substantially shortened at 79% of normal. The measurements confirmed the clinical observations of brachycephalic and hyperteloric anterior cranial vaults, orbits, and zygomas accompanied by globe proptosis and midface deficiency. Results of surgical reconstruction as documented by CT scan measurements demonstrated that, at more than 1 year after surgery, none of the craniofacial measurements had significantly improved (P < .05) as compared with those of the new age-matched controls.

Quantitative CT scan measurements of the cranio–orbito–zygomatic region confirmed the clinical findings in the unoperated children to be brachycephalic anterior cranial vaults and upper face hypertelorism of the orbits and the zygomas,) with ocular proptosis and a flat midface.²¹⁷ It was found that cranial vault and upper orbital reshaping before 1 year of age does not achieve or maintain a corrected shape in the craniofacial skeleton in children with Apert syndrome. Although the cranio–orbito–zygomatic dysmorphology did not worsen when analyzed at least 1 year after surgery, values remained far from those of the new age-matched controls, thereby confirming the need for a staged reconstructive approach.

Quantitative Intracranial Volume Measurements Before and After Cranio–Orbital Reshaping in Children with Apert Syndrome

In published studies, a proven method for obtaining intracranial volumes with the use of CT scan measurement was applied to a consecutive series of children with Apert syndrome before any craniofacial procedures were performed.²⁰⁹ A standard cranio–orbital operation was performed for each child, and this was followed by longitudinal follow up and the remeasurement of the intracranial volume at least 1 year later. The patients’ intracranial volumes were also compared with those of an age-matched cohort, and each patient’s cranial growth velocity was reviewed. The study included six girls and two boys with an average age at operation of 12 months (range, 9 to 23 months). The average postoperative follow-up duration at the close of the study was 34 months (range, 12 to 48 months).²⁰⁹

Preoperative intracranial volume in the patients with Apert syndrome ranged from 393 mL in a 2-month-old girl to 1715 mL in a 28-month-old girl.²⁰⁹ A comparison of the preoperative intracranial volume in patients with Apert syndrome with those of the age- and gender-matched cohort group showed that six of the eight patients had values of at least two standard deviations above the mean. Interestingly, the other two were infants who underwent ventriculoperitoneal shunting for hydrocephalus earlier in life. Their measured preoperative intracranial volumes were two standard deviations below the mean. When the patients’ postoperative intracranial volumes were compared with those of the cohort group, all eight achieved values of at least two standard deviations above the mean. The majority of the measured preoperative and postoperative intracranial volume values of the patients with Apert syndrome followed a growth curve that greatly exceeded the rate expected for normal children. In three of the patients, cranial vault growth velocity seemed to match closely that expected for a normal child but with a starting point determined by their preoperative values.

The findings confirmed that untreated patients with Apert syndrome are generally macrocephalic early during their lives, that standard cranio–orbital procedures carried out during childhood do not alter this trend, and that continued cranial volume expansion often exceeds the mean. The ability to develop “normal” intracranial volume standards and to identify variations from normal in specific syndromes and in individual patients before and after surgery continues to be elusive.18,20,21,24,60,73,80,85,86,93,96,140,141,210,246,247

Quantitative Assessment of Presenting Deformity during the Mixed Dentition in Children with Apert Syndrome and Surgical Results after Facial Bipartition Osteotomy on the Basis of Computed Tomography Scan Analysis

In published studies, children with Apert syndrome were assessed during their middle childhood years with the use of quantitative CT scan measurements.²²⁰ Many of these children’s measurements varied from normal as compared with those of age-matched controls. The orbital measurements showed a substantially increased anterior interorbital width (123% of normal), an increased mid-interorbital width (122% of normal), and an increased intertemporal width (126% of normal). The globe protrusion beyond the sagittal plane of the lateral orbital walls was excessive (142% of normal). There was also a short medial orbital wall length (85% of normal). The width between the lateral orbital walls was excessive at 111% of normal. Zygomatic arch lengths were substantially shortened at 83% of normal.

After undergoing FB osteotomies with three-dimensional repositioning in combination with cranial vault reshaping, CT scan measurements were again taken both early after the operation and 1 year later.²²⁰ An analysis of the measurements showed an improvement toward the normal range. As compared with the values of age-matched controls, the orbital measurements reflected the correction of the hypertelorism; the anterior interorbital width early after operation was 106% of normal and later 105% of normal. The mid-interorbital width initially improved to 106% of normal and later to 100% of normal. The width between the lateral orbital walls stabilized at 108% of normal, and the intertemporal width was 115% of normal, which was an improvement over the preoperative value of 126% of normal. The zygomatic arch length was initially overcorrected at 110% of normal and then stabilized at 103% of normal.

Further studies were carried out to evaluate the presence of extradural (retrofrontal) dead space after the FB osteotomy to reconstruct the upper midface deformity associated with Apert syndrome.²⁰⁸ Seven patients with Apert syndrome (mean age, 8 years) underwent FB osteotomies with advancement. Extradural (retrofrontal) dead space was measured from a reproducible axial CT scan slice for each patient at standard postoperative intervals (1 to 2 weeks, 6 to 8 weeks, and 1 year). An initial extradural (retrofrontal) dead space was identified early after surgery in each patient. There was good resolution of the dead space by the 6- to 8-week postoperative interval through expansion of the dura and the frontal lobes of the brain. The dead space was confirmed to be closed in all patients at the 1-year postoperative interval. The morbidity of the same consecutive series of patients was then reviewed. For the seven children, there were no deaths, cardiopulmonary sequelae, injuries to the brain or eyes, new seizure activity, or central nervous system problems.