Atlas Oral Maxillofacial Surg Clin N Am 10 (2002) 4356

Frontoorbital reconstruction
Steven R. Buchman, MDa,b,c,*, Karin Muraszko, MDd,e
Section of Plastic and Reconstructive Surgery, University of Michigan Medical School, 2130 Taubryn Health Care Center, 1500 East Medical Center Drive, Ann Arbor, MI 48109-0340, USA b Craniofacial Anomalies Program, University of Michigan Medical Center, 1500 East Medical Center Drive, F7859 Mott Hospital, Ann Arbor, MI 48109-0219, USA c Division of Pediatric Plastic Surgery, CS Mott Childrens Hospital, 1500 East Medical Center Drive, Ann Arbor, MI 48109, USA d Department of Neurosurgery, University of Michigan Medical School, Ann Arbor, MI 48109, USA e Division of Pediatric Neurosurgery, CS Mott Childrens Hospital, Ann Arbor, MI 48109, USA
a

Frontoorbital reconstruction is one of the mainstays of surgical management for craniofacial deformities. Variations on operative procedures using frontoorbital advancement (FOA) as a basis to achieve surgical correction of congenital deformities of the face and skull provide the foundation for addressing a large number of reconstructive needs in craniofacial surgery. Specically, FOA procedures entail removal of the bones of the forehead, supraorbital rims, and calvarium, reshaping and repositioning those bones, and stable xation of the reconstruction (Fig. 1B; see Figs. 4B, 6B). This article outlines the current management of craniofacial deformities using FOA as a treatment strategy. The article addresses patient selection, surgical indications and timing of treatment, perioperative care, and operative techniques. This article is in no way meant to be a primer for the undertaking of this complex multifaceted procedure but rather is a source of information to better understand the approach to the reconstructive the approach to the reconstructive dilemmas posed by a unique group of patients with challenging congenital deformities. Only trained craniofacial surgeons, in appropriate facilities, with dedicated assembled teams should take on the care and responsibility for these high-risk procedures. The specialized care and expertise required by the patients in need of FOA is not to be underestimated. Serious complications such as brain damage, blindness, and death are known risks of the FOA procedure and can become more likely when the surgical management of these patients is approached in a cavalier manner. To address various congenital deformities, craniofacial surgeons use the FOA procedure with success. Patients with syndromic and nonsydromic craniosynostosis make up the overwhelming majority of cases that require the procedure. As in most surgical procedures, patient selection is one key to obtaining an excellent outcome. The most common groups of patients who require FOA are those with plagiocephaly secondary to unicoronal craniosynostosis (Figs. 1A, 2, 3), brachycephaly secondary to bilateral coronal craniosynostosis (Figs. 4A, 5), and trigonocephaly secondary to metopic craniosynostosis (Figs. 6A, 7). A sound insight into the variables that inuence these craniofacial malformations may well be a key predictor of growth abnormalities, which could be invaluable in the development and application of surgical strategies tailored to each individual patient and to each individual diagnosis. Although many of these patients display a wide spectrum of phenotypes, the basic principles underlying the scope, timing, and approach to these complex craniofacial anomalies are based on the solid fundamental principles and tenets of craniofacial reconstruction. Patients with disparate craniosynostoses often undergo a similar pattern of reconstructive procedures to achieve improvements in the shape, symmetry, and balance of their face and cranial vault. The overall reconstructive goals of maximizing

* Corresponding author. E-mail address: sbuchman@umich.edu (S.R. Buchman). 1061-3315/02/$ - see front matter 2002, Elsevier Science (USA). All rights reserved. PII: S 1 0 6 1 - 3 3 1 5 ( 0 1 ) 0 0 0 0 2 - 6

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Fig. 1. (A) Skull viewed from above displaying plagiocephaly secondary to unicoronal craniosynostosis. (B) Frontal orbital advancement for plagiocephaly. The entire forehead and supraorbital bar is removed; the aected side is advanced to attain symmetry and the bones are xed in the advanced position.

results, minimizing the number of operations, and achieving a predictable outcome always should guide the appropriate management for this complex group of patients.

Diagnosis and initial clinical evaluation When rst confronted with a patient who may be aicted with craniosynostosis, an organized and thorough approach is mandatory. A comprehensive and systematic evaluation is necessary to arrive at the appropriate diagnosis, fully assess any associated medical concerns, and embark on the proper clinical management of the patient. A careful functional and esthetic assessment is vital to a successful outcome and is best ascertained through a focused but detailed history and physical examination. A trained craniofacial surgeon and an experienced pediatric neurosurgeon best address the initial evaluation of these dicult patients. The expertise of these two accomplished subspecialties complement each other by combining their individual talents and skills to treat the multifaceted concerns of children aicted with these complex deformities. The pediatric neurosurgeon is charged with discerning the best approach to care for the impact of the structural anomalies on the central nervous system. Associated issues such as hydrophalus and Chiari I malformation must be recognized. It is important that elevations in intracranial pressure be recognized early. Such assessment of intracranial pressure is best evaluated by a pediatric neurosurgeon. The imaginative craniofacial surgeon also relies on the prociency of a pediatric neurosurgical colleague to conne him or her to the realm of the possible, emphasize safety, and temper creativity by stressing practicality and discerning the limitations of the brain. Although the anatomic features of the craniofacial anomaly often may be immediately obvious in children with craniosynostosis, the detection of signicant associated problems that impact each patient may be much subtler. A targeted but thorough history is imperative to focus the physical examination, formulate a more specic diagnosis, and outline the correct clinical course of management. A family history or a family resemblance of the child to a close or distant relative connotes an inherited trait and

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Fig. 2. (A) Preoperative AP view of a patient with left unicoronal craniosynostosis. Note the anomalous left orbit and the C deformity of the face, opposite of the aected side. (B) Preoperative submental vertex view displaying the recessed left supraorbital rim. (C) Postoperative AP view. (D) Postoperative submental vertex view.

could be invaluable in making a more specic diagnosis and placing certain physical attributes into better perspective. A birth history can give clues as to possible complications around the time of delivery such as episodes of anoxia, infections, or trauma. Exposure to drugs, radiation, alcohol, or other harmful substances must be documented and may be an important factor in patient management. A history of headaches, lethargy, vomiting, or diculty seeing or following objects could be clues to underlying neurologic disorders. Seizure activity and developmental delay also indicate abnormal brain function. The physical examination of the patient with craniosynostosis is of particular importance in order to establish the diagnosis, identify the extent of the deformity, and form the basis of a treatment plan and surgical strategy for reconstruction. A comfortable familiarity of the associated physical ndings that distinguish the common craniofacial phenotypes should guide a vigilant examination of the patient. An initial skilled general survey of a child with craniosynostosis should detect and conrm readily apparent physical ndings, whereas procient powers of observation are needed to appreciate the subtler characteristics of abnormal facial form inherent in a comprehensive analysis of craniofacial dysmorphology. Attention to detail is rewarded with a clearer picture of the patients current status, an outline of the structural and functional decits,

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Fig. 3. (A,B) Three-dimensional computed tomographic scans of a patient with unilateral coronal craniosynostosis reveals a typical skull, orbital, and facial changes. Orbital dysmorphology is a consequence of the malpositioning of the lesser wing of the sphenoid bone posteriorly and of the supraorbital rim and brow prominence anterolaterally. Midfacial changes are the result of compensatory growth patterns stemming from cranial base suture fusion.

and a working classication of the deformity. The examiner should be alert to any growth disturbances, neurologic decits, and developmental delays displayed by the patient and assess basic functions such as the childs ability to see, eat, and breathe. Basic problems in any of these areas signicantly impact the appropriate medical and surgical management of the patient and guide appropriate consultation and further diagnostic evaluation.

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Fig. 4. (A) Skull viewed from above displaying brachycephaly secondary to bicoronal craniosynostosis. Inset displays that brachycephalic head is atter and taller than normal. (B) Frontal orbital advancement for brachycephaly. The entire forehead and supraorbital bar is removed, both sides are advanced to restore a normal shape, and the bones are xed in the advanced position.

A head circumference should be taken on all children in their evaluation for craniosynostosis. A small head could connote a primary growth abnormality of the brain or an uncompensated growth restriction of the skull secondary to craniosynostosis. A large head could indicate hydrocephalus or a compensated growth pattern of the skull secondary to craniosynostosis. Palpation of the cranium should include the fontanelles and the cranial sutures. An enlarged fontanelle is commonly seen in certain disorders, such as Apert syndrome, whereas bulging fontanelle could

Fig. 5. (A) Preoperative AP view of a patient with bicoronal craniosynostosis. Note the at, high forehead and the recessed supraorbital rims. (B) Preoperative lateral view. (C) Postoperative AP view. (D) Postoperative lateral view.

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Fig. 6. (A) Skull viewed from above displaying trigonocephaly secondary to metopic craniosynostosis. Frontal orbital advancement for trigonocephaly. (B) The entire forehead and supraorbital bar is removed, the lateral portions are advanced and reshaped to restore normal morphology, and the bones are xed in the advanced position.

indicate increased intracranial pressure and possible hydrocephalus. A palpable ridge can suggest synostosed sutures, as could premature closure of the fontanelles. The general shape of the head is an indication of the underlying pattern of craniosynostosis and is perhaps the most important factor in establishing a clinical diagnosis. Plagiocephaly (slant head) results from unilateral coronal craniosynostosis and is characterized by an ipsilateral recession of the supraorbital rim, a attening of the adjacent brow and forehead, and a trapezoidal shape to the cranial vault (Figs. 13). Patients with unilateral coronal craniosynostosis often display a classic C deformity of the face as a result of compensatory deformational forces (Fig 2A). Trigonocephaly (triangular head) results from metopic craniosynostosis and is characterized by a triangular shape of the forehead, supraorbital rims, and the cranial vault (Figs. 6 and 7). Bitemporal constriction and hypotelorism are also associated ndings of trigonocephaly. Brachycephaly (short and at head) results from bilateral coronal craniosynostosis, and although it can be found in cases of sporadic, nonsyndromic, and nonfamilial cases of craniosynostosis, it is the most common ndings in Apert, Crouzon, and Pfeier syndromes (Figs. 4 and 5). Intraocular assessment of the fundus and visualization of the optic disk should be sought to rule out pallor and papilledema; which are signs of increased intracranial pressure. Evidence of persistent torticollis could signal cervical spine deformities, an extraocular muscle problem, structural foreshortening of the sternocleidomastoid muscle, or simply an habitual inclination. The recognition, diagnosis, and treatment of this problem are of paramount importance because the muscle imbalance can result in signicant asymmetry that aects the growth and development of the face and cranium. A failure to recognize torticollis could have signicant clinical repercussions. Persistent torticollis is often associated with positional head deformity, which may lead to a spurious diagnosis of craniosynostosis. An uncorrected torticollis also can bring about abnormal positioning postoperatively, which leads to unrestrained deformational forces that can aect head shape during the healing process and result in the possible need for secondary surgery.

Tests, consultations, and clinical management Imaging studies are an invaluable aid in the evaluation of the patient with craniosynostosis. A skull series is helpful to scrutinize the cranial sutures and detect radiographic evidence of fusion.

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Fig. 7. (A) Preoperative AP view of a patient with metopic craniosynostosis. Note the triangular-shaped forehead and the bitemporal constriction. (B) Preoperative submental vertex view displaying the bilateral recession of the supraorbital rims. (C) Postoperative AP view. (D) Postoperative submental vertex view.

CT is an essential study for assessing the structural anatomy of the brain and surrounding cranial vault. A thorough evaluation of the scan is performed to look for evidence of hydrocephalus, signs of increased intracranial pressure (ICP), and indications of pathologic neuroanatomy. The scans also provide an exceptional examination of the bony anatomy of the craniofacial skeleton. Three-dimensional reformatting may convey a clearer picture of these complex deformities and can serve as an eective teaching tool (Fig. 3). MR imaging can be complementary to a CT scan by furnishing a distinct view of the brain and is particularly helpful in assessment of Chiari malformations. A neuroopthalmologic consultation for a careful dilated examination of the eyegrounds can be an important adjuvant to the initial cursory view of the fundus under suboptimal conditions. A dilated examination documents the baseline status of the optic disc and may impact the timing of surgical intervention. A genetics consultation can rene, conrm, or help establish the diagnosis, screen for associated problems, and suggest further evaluation or necessary testing. Genetic counseling can console and inform the patients family and generate a letter as a reference to document the inheritance patterns and subsequent risk to future ospring for the parents and the child. Rapid progress in the discovery of associated mutations for many of the

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craniofacial syndromes has resulted in blood tests that have been developed to assist geneticists in arriving at more accurate and specic diagnostic classications. Neuropsychologic evaluation is useful in determining baseline function and in detecting cognitive decits. The evaluation can serve as a comparison to the patterns of future development or, more specically, can lead to recommendations for interventional programs early on. Surgical indications and timing of treatment The surgeons goals are to provide a predictable and durable reconstruction that restores facial form, reestablishes symmetry, and attains the best results in the least number of operations. All of these objectives must be considered with regard to the safety of the patient and the nature of the growth in the child with specic types of craniosynostosis. Surgical intervention also must address any pressing health concerns, such as high ICP. These health concerns supplant any treatment protocol or timing philosophy for operative intervention in the child with craniosynostosis. Hydrocephalus can develop at any point along the course of treatment and must be recognized and treated quickly. It is imperative to be vigilant and maintain a high index of suspicion for any sign or symptom of high ICP. High ICP usually mandates acute surgical intervention targeted at the etiology, which could mean the placement of a shunt, the expansion of the cranial vault, or a combination of both. Close communication between the pediatric neurosurgeon and the craniofacial surgeon is essential in cases of high ICP so that an appropriate operation is performed and the timing of the operation is optimal. The timing of the release of the cranial sutures in a patient with craniosynostosis has been controversial. The benets of early release of the cranial sutures have more to do with the philosophy of the surgeons as to the impact of craniosynostosis on the brain and the benets and disadvantages of a more delayed approach to the durability of the overall reconstruction. There is no doubt that a documented problem with an increased ICP would mandate intervention; however, short of such an indication there is disagreement as to the surgical timing of reconstruction in the patient with craniosynostosis. The history of early reconstruction (neonatal to 3 months of age) was predicted on a belief that the synostotic suture was a principal participant in the expression of the abnormal phenotype. It was believed that by releasing the synostotic suture the calvarial vault and the frontoorbital bar could take on a normal growth pattern. The expanding brain, unfettered by the growth restriction derived from the fused suture, would thrust the facial skeleton forward, directing and guiding future development and inducing a normal growth pattern. This philosophy proved untrue as a simple suture release was remarkably unsuccessful in preventing the signicant sequelae and seeming inevitable disgurement associated with the growth and development of the craniofacial skeleton in patients with unicoronal, bicoronal, and metopic synostosis. The surgical philosophy then changed to a more comprehensive strategy aimed at releasing the synostotic sutures while concomitantly relocating the bones of the cranial vault into a more anatomically correct position. This strategy called for performing the operation early on, allowing the brain to expand and impel the well-reconstructed cranial vault and frontoorbital bar forward, inducing a normal growth pattern. These procedures also took advantage of the propensity for the expanding brain to promote normal growth and development of the craniofacial skeleton. The literature seems to document, however, that operations performed early on had a signicant incidence of revisional surgery. The incidence of revisional surgery is an important outcome measure because the number of operations should be minimized not only to subject the patient to less risk but also because the quality of the bone decreases each time it is subjected to a surgical procedure. The premise that the release of the brain allows a normal pattern of expansion and growth is most probably a faulty one. The entire cranial vault and facial skeleton seem to be aected by the fused sutures, especially in the case of syndromic craniosynostoses. Many of their pathologic growth disturbances may be manifestations of the nearly inaccessible sutures at the cranial base. Early release of the accessible craniosynostotic sutures without a specic medical indication seems doomed to failure. On the other hand, waiting too long to initiate reconstruction of the cranial vault and frontoorbital bar also could have a deleterious eect on the eventual outcome. Allowing the abnormal growth pattern of craniosynostoses to go unchecked could result

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in a craniofacial skeleton that is so skewed and misshapen as to make reconstruction even more dicult to get back to baseline. The transient osteogenic capability of the dura to heal bony defects should be taken advantage of because it precludes the need for extensive bone grafting. These bone grafts may be needed for future reconstruction and should be conserved whenever possible. Finally, the initial craniofacial reconstruction is also technically easier at a younger age, because the bones are more easily cut and shaped to allow a more accurate anatomic restoration. The surgical philosophy that drives the timing of the release of the cranial sutures in the patient with craniosynostosis must balance the desire to attain a normalization of morphology with the realization that the propensity of abnormal growth still persists. The authors developed a protocol of reconstruction at 9 to 12 months of age to achieve that balance. Exceptions to the protocol can be made on an individual basis with careful consideration of any pressing health concerns, such as high ICP, which would necessarily direct surgical intervention. It is important to reiterate, however, that the approach to the timing of surgical intervention for craniosynostosis is still varied and controversial. The syndromic craniosynostoses do not fall easily into one category and make up a number of varied conditions that result from multiple causes with a set of diverse factors that inuence their pathogenesis. The surgical approach to this challenging group of patients should be individualized with a surgical strategy formulated to address the overall craniofacial anomaly and the particular nuances in the deformation as manifested by each patient. The surgeon often chooses from a stable of established operative approaches that can be modied as desired to suit particular patient needs. The timing of intervention of the staged procedures required for comprehensive craniofacial reconstruction of syndromic craniosynostoses also should take into consideration the individual needs of the patients; however, these immediate needs always should be tempered by the experience and knowledge of a trained craniofacial surgeon and pediatric neurosurgeon. Specically, the impact of continued abnormal growth of the craniofacial skeleton cannot be underestimated because the surgical approach only deals with the structural aspect of the current abnormality and does not remedy the underlying growth disorder. FOA is usually the rst staged procedure performed on syndromic craniofacial patients, such as patients with Apert, Crouzon, Pfeier, and Saethre-Chotzen syndromes, which usually present with bicoronal craniosynostosis. Preoperative preparation The patients preoperative assessment and evaluation include a PT and PTT, a CBC, and an electrolyte panel. Blood is made available for type and cross of at least two units. There is great potential for signicant blood loss and uid shifts during the operation, and the expertise of the anesthesia team is vital to a smooth and successful outcome. The patient usually can be admitted the day of the surgery and undergoes placement of extensive monitoring equipment in preparation for the procedure. At least two large bore intravenous lines are started, and a central venous catheter may be required to help assess intraoperative uid status. A precordial Doppler is placed, as are EKG leads, a pulse oximeter, an arterial line, and a Foley catheter. Prophylactic antibiotics are used, and only a strip of the scalp is shaved to allow for a serpiginous coronal incision. Frontoorbital advancement and cranial vault reshaping The patient is placed in the supine position with the endotracheal tube centered out of the mouth. The operative table is set at 90 to 180 away from the anesthesiologist. A serpentine bicoronal incision is made from the back of the ear on one side to the other (Fig. 8). A supraperiosteal dissection is performed, reecting the ap forward, until approximately 1 cm above the superior orbital rims. The dissection is then carried subperiosteally to the orbits and the periorbita is degloved from the globes medially just above the medial canthus and laterally just below the zygomaticofrontal suture. Depending on the degree of the deformity, the temporalis muscle is taken down to expose the temporal fossa and allow repositioning of the muscle. In cases such as metopic synostosis in which there is an obvious bitemporal constriction with a malposition of the temporalis muscle and the bone, reposition of the temporalis muscle in an

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Fig. 8. A scallop-patterned coronal incision results in a better-hidden scar. With a traditional straight-line incision, the hair can part along the straight-line scar, especially when wet, rendering the scar quite noticeable. The scalloped pattern incision allows hair to better cover the resultant scar, rendering it less obvious.

anterior and medial direction can be an important operative strategy to address the decient temporal region (Fig. 9B). An arching incision in the periosteum is made with a cautery well above and behind the temporal line. A subperiosteal dissection is made rotating the temporalis anteriorly and medially. A back cut of the muscle at the level of the temporal portion of the zygoma may be necessary to gain the mobilization for medial advancement. A bifrontal craniotomy is preformed by the neurosurgeon, with care being taken to avoid damage to the dura and particularly the underlying sagittal sinus. The bifrontal craniotomy should extend down to within a centimeter of the orbit (Fig. 10). If the craniotomy extends down directly to the orbital roof there may be too small an orbital bar left. If the craniotomy is too high, there can be too much residual bone to be able to safely elevate the frontal lobes extradurally, without excessive retraction and damage. Mannitol (1 g/kg) is generally given at the beginning of the craniotomy and the pC02 is carefully monitored. Each of these maneuvers relaxes the brain and makes retraction more gentle. Careful extradural dissection of the subfrontal region allows for safe creation of the orbital bar. Dural tears can occur during craniofacial procedures and usually require just a primary closure of the tear. Tears in the sagittal sinus can be life threatening, particularly in young children, and require aggressive management. Care must be taken during reconstruction to avoid kinking of the sinus. Sagittal sinus thrombosis must be avoided as attempts are made to alter the overall calvarial shape. An osteotomy is performed at a level just below the zygomaticofrontal suture and carried along the orbital roof to the midline, which is met by a similar osteotomy coming from the opposite side (Figs. 9 and 10). The supraorbital bar is then removed. The osteotomy is performed while protecting the globes and the brain with malleable retractors. The cranial bone from the craniotomy is split with an osteotome, if possible, to secure additional bone for grafting. If temporal constriction is an element of the deformity, the temporal fossa is then approached and a barrel-stave osteotomy is performed. Moving posteriorly, barrel-stave osteotomies are also performed along the parietal skull, depending on the degree of deformity of the cranial vault (see Fig. 9). The barrel-stave osteotomies are then out-fractured and molded with a bone-bending forceps. Tactical cuts are made in the out-fractured bone to allow the parietal and temporal region to take on a more normal anatomic appearance. If there is a signicant element of proptosis, the periorbita can be incised to allow some of the fat to herniate. The frontoorbital bar is bent into an anatomic shape, (Figs. 9 and 10). The reconstruction of the frontoorbital bar varies depending on the nature of the patients original deformity. In unicoronal craniosynostosis, the recessed side is bent outward and brought forward. A normal contour is

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Fig. 9. (A) On the left is schematic depiction of a brachyturricephalic deformity in a syndromic patient with craniosynostosis. Note the retruded, at and high forehead; the towering cranial vault; and the foreshortened cranial base. On the right is a schematic depiction of a frontoorbital advancement in the same patient. The frontoorbital bar is advanced and bent into a convex shape. When possible, split cranial bone is used as an autogenous plate to hold the advancement forward. (B) The technique for addressing a turricephaly is presented. The cranial vault is lowered, the frontal bone is advanced, and the occipital cranial vault is outfractured and displaced posteriorly. The barrel staves maintain the integrity of the outfracture by locking in bone grafts as cross supports. As the high head is brought back to a more normal position, the cranial base is expanded and the brain further supports both the advanced frontal segment and the occipital outfractured segments of the skull.

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sought and then placed so that the aected side is placed in an advanced position. An advancement of at least 1 to 1.5 cm is preferred because overcorrection is often necessary to counter the recoil forces of the surrounding soft tissue and the predisposition for recession of the orbital bar over time. It may be necessary to place interpositional grafts if the orbit is constricted, and small or onlay grafts may be required for decient areas refractory to simple remolding (Fig. 11). Bicoronal synostosis demands that the entire frontoorbital bar be placed in the advanced position, whereas metopic synostosis mandates reshaping and bilateral advancement of the lateral orbital rims. The soft tissue in the coronal ap can be scored and released to lessen the resistance to advancement. The entire frontoorbital bar is then xed at the new position (Figs. 911). Metal microscrews are used for xation or, alternatively, resorbable systems can be substituted, but the advancement calls for structural integrity, so strength of the xation is paramount. Interpositional bone grafts are then placed to ll the gaps left at the sites of advancement which results

Fig. 10. (A) Sites of the osteotomies used in the described technique. These extend laterally from the bifrontal craniotomies at the parasagittal vertex and run posterior to the fused coronal sutures into the parietal bones, where they then turn medially 1 cm above the superior orbital rims to meet in the midline. (B) After advancement and reshaping of the orbital rims and caudal frontal bones (bandeau), and recontouring of the frontal bone, biodegradable plates and screws are used to x the bones at the nasion and the pterions and also where needed in the frontal bone.

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Fig. 11. Once properly shaped, the entire ipsilateral half of the supraorbital unit is then advanced by sliding the tenon extension forward the desired amount, where it is then rigidly xed to the adjoining temporal bone with absorbable plates and screws. When a large advancement is necessary, an interpositional bone graft can contribute additional stability and prevent relapse of the advanced segment.

in a continuous, stable, and structurally sound reconstruction. If a thin piece of bone graft can be split, it can be used as a plate itself and xed to the lateral orbital rim and the lateral portion of the supraorbital bar (Figs. 9 and 10). The bones of the forehead are cut, bent, and formed into a normal anatomic shape (see Fig. 10B). Certain techniques imply a particular order and osteotomy scheme that can lead to a cookbook reconstruction; however, each deformity is unique and does not lend itself to such a specic approach. The bones are then xed to the supraorbital bar. A gap of at least 2 cm is maintained between the posterior portion of the frontal bone and the anterior border of the remaining margin of the craniotomy to prevent a recurrent synostosis (see Figs. 9B and 10B). The decision to perform a lateral canthopexy varies from surgeon to surgeon. Some surgeons perform a lateral canthopexy in all frontoorbital advancements; others are more selective. The authors determine the need for a canthopexy clinically. Usually if the lateral canthus looks good preoperatively it will reattach in a normal position; however, if there is a poor position of the lateral canthus preoperatively or an intraoperative question arises as to malposition, the canthopexy is performed. The technique uses a 3.0 clear nylon suture and requires the careful isolation of the lateral canthal mass by grasping the lateral canthus externally with a forceps. The needle is driven through the skin, from inside to out, through the area that was being grasped. The needle is then introduced back through the same hole at a dierent trajectory and pulled back and forth to x the lateral canthal mass but release any skin attachment to the suture. The suture is pulled and any attachment to the lateral canthus is checked; the lower lid should move freely when the suture is pulled. The suture is xed behind the advanced lateral orbital rim or through a separate set of drill holes and pulls the canthus laterally and posteriorly. Attention is then directed to the temporal fossa, which is shaped with a combination of bony remolding and muscular reposition. The temporalis is xed with 3.0 absorbable suture. The frontalis is scored to release any memory of the abnormal forehead and allow a more natural drape of the soft tissue. The coronal ap is closed securely with interrupted absorbable galeal sutures, and the scalp is closed with closely placed 3.0 plain running sutures. A headwrap is applied and every attempt is made to extubate the child in the operating room.

Postoperative Postoperatively, the patient is sent to the intensive care unit, where vital signs and urine output are monitored closely. The authors prefer to use normal saline as the maintenance intravenous uid to help guard against hyponatremia. Sodium levels and hematocrit are followed on a regular basis. For challenging cases in which extensive dissection around the brain is required, phenobarbital may be used to prevent the possibility of seizures. Narcotics are used sparingly and judiciously so that an accurate neurologic examination can be followed. Antibiotics

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are continued throughout the postoperative course. The patients head is kept elevated to help minimize edema that often leads to the eyes swelling shut. Postoperative low-grade fevers are the rule rather than the exception and are rarely caused by infection; they usually resolve spontaneously over time. The child is usually transferred to the oor on the second postoperative day and is discharged within 5 days after the extensive periorbital swelling has decreased. Children with craniosynostosis require extensive follow-up for various issues. The growing child is in a dynamic state. There can be a mismatch of brain growth versus skull growth, which can lead to intracranial crowding and may promote the development of a Chiari I malformation or cerebellar tonsillar ectopia. Regular neurologic assessment is important to ensure that such problems are addressed quickly. The development of headaches in such children may indicate intracranial crowding. Other symptoms may be subtler, such as a decrease in school performance or coordination, change in vision, or loss of milestones. Careful radiological evaluation and detailed neurologic assessment are important. The authors advise that all children with craniofacial anomalies undergo regular ophthalmologic evaluation, including dilated fundoscopic examination. Unmonitored increases in intracranial pressure can be devastating and lead to permanent neurologic decits such as blindness. In cases in which intracranial crowding is not definite but is suspected, the use of an intracranial pressure monitor sometimes can be useful. It can help sort out whether there is any evidence of increased intracranial pressure or whether symptoms such as headache are better managed medically.

Summary The care of the patient with craniosynostosis is perhaps the most challenging problem in craniofacial surgery. The syndromes vary in their presentation and severity; however, many of the structural and functional sequelae have common characteristics. The multifaceted medical problems of these children are particularly well suited to a multidisciplinary team approach to their care. The surgeons must be aware of the most pressing health concerns of these patients and incorporate those concerns into a well-formulated phased plan of comprehensive reconstruction. The overall goals of treatment of syndromic and nonsyndromic craniosynostosis continually should guide the care of these exceptional children. Those goals are to provide a predictable and durable reconstruction that restores facial form, reestablishes symmetry, attains the best results in the least number of operations, and infuses the patient with a sense of emotional and physical health.

References
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