Onlay Bone Graft Augmentation for Rened Correction of Coronal Synostosis

JOHN H. GRANT III, THEODORE S. ROBERTS, JOHN D. LOESER, JOSEPH S. GRUSS,

Objective: The primary purpose of this study was to evaluate the long-term result of an onlay bone graft augmentation of the supraorbital ridge at the time of primary correction of coronal suture synostosis. Design: The study is a retrospective review of 62 consecutive patients treated for coronal synostosis from June 1991 through February 1997. The surgical technique utilized involved a standard bilateral fronto-orbital advancement and calvarial reshaping with the addition of an onlay bone graft in the supraorbital region. Setting: All patients were treated at a tertiary care craniofacial center. Results and Conclusion: A total of 62 patients were treated by this technique. Fifty patients underwent primary correction as infants (mean age 9.8 months). An additional 12 patients were older (mean age 8.2 years) and were treated for residual deformity having previously undergone correction by another technique. Results with follow-up as long as 7 years demonstrate stable forehead and orbital symmetry. Complications identied by chart review were minimal and not directly attributable to this modication in surgical technique.
KEY WORDS: bone graft, coronal synostosis, fronto-orbital advancement

M.D. M.D. M.D. M.D.

Since the rst attempts in the 1890s at strip craniectomy for correction of coronal synostosis by Lannelongue (1890) and later by Lane (1892), a variety of innovations in surgical technique have provided surgeons with an opportunity to improve upon and further rene their results. The early surgical techniques of strip craniectomy yielded to the craniofacial techniques after Tessier introduced them in 1967 (Posnick, 1996). Hoffman and Mohr (1976) popularized the technique of lateral canthal advancement. Whittaker described a unilateral correction of the deformed forehead and orbit. Later renements grew from the realization that unicoronal synostosis is a bilateral deformity (Posnick, 1996). Contemporary craniofacial techniques, practiced by experienced teams, have resulted in reproducibly good correction with very low morbidity (Mohr et al., 1978; Anderson, 1981; Munro, 1981; Tulasne and Tessier, 1981; Marsh and Schwartz, 1983; Jane et al., 1984; Laurent el al., 1990; Cohen et al., 1991; Elisevich et al., 1991;
Dr. Grant is an assistant professor with the Division of Plastic Surgery, Department of Surgery, University of Alabama at Birmingham, Birmingham, Alabama. Dr. Gruss is a professor with the Division of Plastic Surgery, Department of Surgery, and Dr. Roberts and Dr. Loeser are professors with the Department of Neurosurgery, University of Washington, Seattle, Washington. Presented at the Annual Meeting of the American Cleft PalateCraniofacial Association; 1997. Submitted November 2000; Accepted November 2001. Address correspondence to: John H. Grant III, M.D., Division of Plastic Surgery, University of Alabama, Birmingham, 1600 7th Avenue South, ACC 322, Birmingham, AL 35233. E-mail john.grant@ccc.uab.edu. 546

Kaiser and Bittel, 1991; Machado and Hoffman, 1992; McCarthy et al., 1995; Hoffman, 1996; Posnick, 1996). The morphologic abnormality seen in unicoronal synostosis is best understood if broken down into its individual components. The anterior plagiocephaly ipsilateral to the synostotic suture consists of a retrusion of the brow. In addition, the forehead, composed of the frontal bone, is attened or even slightly concave. The orbit and greater wing of the sphenoid are distorted in shape, characterized by the well-described Harlequin or Mephistophelean appearance on radiograph. Lastly, there is absence of the supraorbital ridge (Fig. 1). The contralateral deformity is marked by a frontal bossing and, in more severe cases, a downward displacement of the superior orbital rim. The characteristic facial twist, away from the side of synostosis, is thought by most authors to correct once primary surgery releases the fused suture. This paper describes an additional renement in surgical technique, which specically addresses the deformity of the supraorbital region on the affected side. Fronto-orbital advancement alone cannot be expected to correct for the absent supraorbital ridge. Anterior advancement of the abnormally shaped bone does not correct its shape. Advancement of the orbital rim without correction of the deformity results only in repositioning of an abnormal bone. Untwisting of the bandeau improves the conformation of the abnormal bone making up the forehead but does not fully compensate for absent anatomy. Additional orbital osteotomies with independent repositioning of the bone segments is helpful but may result in increasing

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complexity as multiple bony components are mobilized independently (Raimondi and Gutierrez, 1977; Lo et al., 1996b). In addition to an untwisting and forward repositioning of the bone, a correction of the abnormal shape should be accomplished by the addition of an onlay bone graft (Fig. 2). The onlay bone graft is a simple and reliable technique, which addresses the relative deciency of projection in the supraorbital region. It has the added benet of providing stability to the newly reshaped frontal bandeau. By utilizing this technique, one may correct the concavity of the forehead and add a supraorbital ridge. The reshaped and augmented bone may then be repositioned for optimal correction of the deformity METHODS A retrospective chart review was conducted of all patients undergoing correction for coronal synostosis at the Childrens Hospital and Medical Center in Seattle between June 1991 and February 1997. A total of 62 consecutive patients were identied. All patients were operated on by one craniofacial surgeon (J.S.G.) and one of two neurosurgeons (T.S.R. and J.D.L.). Operative notes and photographs taken intraoperatively were reviewed to verify that the operative technique utilized placement of the onlay bone graft. Follow-up has been through the Seattle Childrens Craniofacial Center. Clinic notes as well as hospital admissions records were reviewed to document complications after the operative course and duration of postoperative follow-up. The chart review identied 62 patients undergoing correction for coronal synostosis. Of these, 50 were infants, all of whom underwent primary surgical correction by the technique described in this paper. Seventeen were males and 33 were females. The mean age at the time of surgery was 9.8 months. Twenty-nine children had unicoronal synostosis (18 right and 11 left coronal sutures). Four infants had isolated bicoronal synostosis. Five infants had Saethre-Chotzen syndrome. Nine infants had Crouzon, Apert, or Pfeiffer syndrome. Three infants had not yet had denitive diagnosis at the time of this writing but are thought to represent point mutations of broblast growth factor receptors (Cohen, 1995). In this subgroup there were 48 bilateral fronto-orbital advancements and two unilateral fronto-orbital advancements with a mean follow-up time of 46 months (SD 21 months). Twelve older children (mean age 8.2 years) underwent secondary correction of residual deformity. These children had all undergone previous correction by other surgeons. This group consisted of three male and nine female patients. Seven had Saethre-Chotzen syndrome, three had Crouzon syndrome, one had Apert, and one had a unicoronal synostosis having undergone lateral canthal advancement at age 4 months. This small patient group is included to demonstrate the utility of this technique in secondary correction as well as primary correction. They are included in the data analysis for operative complications. The mean follow-up for this group is 40.2 20.6 months. We, like most contemporary authors, advocate a bilateral

FIGURE 1 A: Three-dimensional computed tomography reconstruction demonstrating the typical unicoronal synostosis deformity, with attening of the left brow and contralateral bossing. B: Anteroposterior view emphasizing the concave supraorbital region.

correction with removal of the supraorbital bandeau bilaterally. Our technique is as follows: through a zigzag bicoronal incision, the temporalis muscle is turned with the scalp ap en bloc. Inclusion of the temporalis muscle with the scalp ap eliminates the need to resuspend the muscle at the end of the procedure. In addition, it helps prevent bitemporal hollowing by leaving the muscle rmly attached to the pericranium with which its deep temporal fascia is continuous. The bandeau is designed with wide bilateral temporal tongue in groove extensions to allow for access to, and correction of, bulging in the squamous portion of the temporal bone (especially on the side of the synostosis). Osteotomies around these temporal extensions are designed in such a fashion that they leave bilateral, posteriorly based, parietal bone struts, to which the bandeau is rigidly xed after reshaping (Fig. 3). On the side of the syn-

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FIGURE 2 A: Anteroposterior view of onlay grafts in place on the reshaped bandeau. B: Note the improved projection afforded by the grafts in the region of the superior and lateral orbit rims as seen from a lateral view.

FIGURE 3 Planned osteotomies with the shaded area representing the posteriorly based strut of bone to which the temporal extension of the bandeau will be overlapped and secured.

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FIGURE 4 Demonstration of the untwisting of the bandeau that brings the superior rim of the orbit anteriorly and inferiorly. Curved arrow shows superior and anterior rotation of temporal extension of bandeau. Straight arrow demonstrates angle of brow ridge displacement.

ostosis, the entire lateral rim of the orbit is included with the frontal bandeau. The osteotomy includes the lateral most portion of the inferior orbital rim then continues toward the inferior oblique ssure. The osteotomy is then carried along the lateral orbital wall at the junction of the sphenoid and zygomatic bones. An osteosynthesis wire is sometimes required to stabilize the frontozygomatic suture. On the back table, an ex vivo reshaping of the bandeau involves untwisting it about a transverse axis parallel to the forehead (Fig. 4). The untwisting results in anterior and inferior rotation of the retruded supraorbital region on the side of the synostosis, thereby bringing it into better position. Untwisting also helps correct the contralateral bossing by rotating it posteriorly. The temporal extensions of the bandeau are shaped using the Tessier bone bender to produce a 90-degree angle from the plane of the forehead beginning at the lateral orbital rim. This bending is facilitated by osteotomizing the thickened sphenoid wing intracranially to weaken the bone. Bone plates (plates made of bone) held in place with osteosynthesis wires may be required to stabilize this portion of the reconstruction in the desired conformation. Once the bandeau has been untwisted and reshaped, an onlay bone graft of full-thickness calvarial bone, harvested from the frontal bone (removed at the initial bifrontal craniotomy), is secured to the supraorbital region with osteosynthesis wires. Care must be taken during this phase of the operation so that the onlay graft does not secondarily deform the reshaped bandeau. Properly positioned, it will actually afford added stability to the reshaped bandeau. Next, in situ contouring of the

orbits is carried out so that a symmetrical rounded shape is achieved for each. The bandeau is repositioned with three osteosynthesis wires providing initial points of xation. An initial wire is placed at the inferior orbital rim on the affected side. The second wire is placed at the nasal osteotomy, on the side opposite of the synostosis. Lastly, an osteosynthesis wire is placed across the osteotomy of the contralateral frontozygomatic suture. These three points of xation allow for an anterior inferior rotation (or tilting) prior to denitive xation. It should be emphasized that this technique differs from the uniplanar sliding advancement of the tongue-in-groove techniques. Rotation of the bandeau improves the nasoglabellar angle and further emphasizes the reconstructed supraorbital region on the affected side. Additionally, a large region of bonebone contact is achieved through this rotation that brings the temporal extensions of the reshaped bandeau superiorly so that they overlap the previously described posteriorly based parietal bone struts. This large overlap of bone-bone contact affords rigid xation to the repositioned bandeau. It is because of this rigid xation that the technique is particularly useful in severe brachycephaly, where soft tissue is limiting and the potential for relapse is higher. Lastly, the forehead reconstruction is then continued from the top of the bandeau sloping posteriorly at an angle of approximately 5060 degrees. Care must be taken to not make the forehead too high. RESULTS In this series, the mean age for primary correction was 9.8 months. Mean follow-up was 46 months (SD 21 months).

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FIGURE 5 Representative pre- and postoperative view of a patients brow region. Top picture demonstrates preoperative birds-eye view. Bottom picture demonstrates worms-eye view at 10 months postoperatively.

There were no deaths. One infant (1.6%) developed an infection consisting of a subgaleal abscess. This was treated by intraoperative drainage, irrigation, and placement of closed suction drains. The child then had 6 weeks of intravenous home antibiotic therapy and is now healed. There has been no visible loss of bone graft at 1 year of follow-up. Four children (6.4%) required secondary surgery for skull expansion. Of this group, two had Apert syndrome, one had Jackson Weiss syndrome, and one had a bicoronal synostosis. No patient with unicoronal synostosis required secondary surgery for skull expansion, incomplete correction, or relapse. One child (1.6%) had overcorrection requiring a secondary operation for contour burring of the healed bone graft. Seven children (11%) underwent removal of palpable hardware (osteosynthesis wires). This was performed through limited incisions as a day surgery procedure. With a mean follow-up of almost 4 years, calvarial

growth is complete and orbital growth is nearly complete. These results are therefore viewed as representative of nal. DISCUSSION It is generally agreed by most craniofacial surgeons that coronal suture synostosis results in a bilateral deformity. Marsh et al. (1986) and Lo et al. (1996a) provided detailed quantitative studies of computed tomography images, pointing out the asymmetry of the orbits as well as the anterior and middle cranial fossae. In the case of unicoronal synostosis, the forehead and orbital deformity consists of a at or concave supraorbital region on the affected side with a attened forehead. There is forehead bossing on the contralateral side. There may also be inferior displacement of the supraorbital rim. This bi-

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FIGURE 6 Top gure: Three-dimensional computed tomography scan of a patient treated by lateral canthal advancement. The small arrow shows the operative site at the right coronal suture. The large arrow shows the attened supraorbital region, despite anteroposterior advancement. Bottom gure: Clinical picture of the same patient with large arrow pointing to area of atness.

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FIGURE 7 A: Preoperative three-dimensional birds-eye view computed tomography scan of a patient with bicoronal synostosis. B: Postoperative threedimensional birds-eye view computed tomography of the same patient showing the onlay bone grafts. C: Preoperative anteroposterior view of the same patient. D: Postoperative worms eye view of the same patient.

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lateral deformity leaves the surgeon with no normal side for comparison. The technique described in this paper addresses the multiple components of the deformity associated with coronal synostosis (unicoronal and bicoronal). Modications of many existing techniques utilized by other authors have been combined to yield a logical, anatomically directed correction of the deformity. Particular emphasis is placed on reconstructing the forehead and orbital rim in a component fashion. A bilateral fronto-orbital advancement is performed. The design of the frontal bandeau is similar to that described by Posnick (1996). A three-quarter orbit osteotomy is performed on the affected side to avoid a step-off along the lateral orbital rim. McCarthy et al. (1995) also advocated this technique in their 1995 review of a 20-year experience describing the evolution of surgical techniques at New York University. We included a wide temporal extension of the bandeau in the region of the squamous portion of the temporal bone. This temporal wing of the bandeau may then be reshaped to allow for the correction of excessive bitemporal width or bulging in the temporal region on the synostotic side. Rather than the sliding advancement of the bandeau, we tilted the bandeau forward and inferiorly at the time of replacement (Hoffman and Mohr, 1976; Plese et al., 1981; Persing, 1990; Persing et al., 1990; Pollack et al., 1996). Stricker rst described this technique in 1972 (Marsh and Schwartz, 1983). We feel that this tilting is critical for several reasons: rst, it improves the nasofrontal angle; second, it emphasizes the reconstructed brow ridge on the synostotic side; and thirdly and probably most importantly, it allows for a large bilateral bone-bone contact area to stabilize the advanced bandeau. Without requiring metallic microplates or additional bone grafts, this repositioned bandeau is rigidly xed in place, perhaps contributing to our clinical relapse rate of zero in nonsyndromic patients. Current practice has substituted resorbable plates or lag screws for xation of the bone grafts and repositioned bandeau. Figure 5 demonstrates a representative patient pre- and postoperatively with a corrected contour in the supraorbital region. The original aspect of the technique presented in this paper consists of the addition of an onlay bone graft to the brow region on the side of the synostosis. The need for this bone graft may be realized by analyzing the shape of the forehead and orbital rim on the side of the synostosis. There is a attening or, in some cases, even a concavity, noted in the ipsilateral forehead. To simply advance this abnormal bone forward, would not change the fact that the bone itself is lacking in critical anatomic features (Fig. 6). The supporting base of the eyebrow and forehead must be reconstructed to provide a foundation for the soft tissues of the upper face. It is an added benet that the onlay bone graft helps to add structural support to the reshaped bandeau. It should be noted that this onlay bone graft is different from the advancement-onlay technique described by Cohen et al. (1991). Although these authors recognized the need to augment the decient bone of the brow region, their technique brought the entire frontal bone segment

from the bifrontal craniotomy forward to do so. A disadvantage of repositioning this large bony segment is a loss of exibility in reshaping the forehead, especially in brachycephalic patients who are prone to postoperative turricephaly. This series, like many other retrospective series, lacks a method of concise quantitative assessment of outcome. Reoperation for calvarial expansion in the subpopulation of patients with syndromic synostosis was performed for failure of the skull to grow proportionate to the requirements of the brain, resulting in increased intracranial pressure as determined by serial ophthalmologic exam and patient complaint of increasing severity of headaches. At the time of reoperation, the bone grafts were noted to have incorporated on the bandeau. At this second operation, the skull was expanded by advancing the bandeau along with the previously placed bone grafts. In summary, we feel that the addition of an onlay bone graft of calvarial bone in the region of the supraorbital ridge aids in the correction of the deformity associated with coronal synostosis. This technique has been performed in 50 consecutive primary corrections as well as 12 secondary reconstructions. There has been no mortality. Morbidity includes one infection requiring operative drainage, one reoperation for recontouring because of overcorrection, and seven reoperations for removal of palpable hardware. There have been four reoperations in patients who demonstrated poor skull growth. In each case, these relapse patients carry the diagnosis of a craniofacial syndrome rather than an isolated suture synostosis. It has been suggested that children with these syndromes demonstrate a diminished potential for calvarial growth (Wall et al., 1994). It should be noted that in our cases of reoperation, the onlay graft was present at the reoperation and the redo surgery was performed for skull expansion and not for forehead reshaping (Fig. 7). This technique is applicable to unicoronal synostosis as well as bicoronal synostosis. It has proven useful in SaethreChotzen, Apert, Crouzon, and Pfeiffer syndromes. Stability of the bone graft has persisted throughout the follow-up period of up to 7 years. REFERENCES
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