Brachycephaly is the premature ossifications of cranial sutures. This condition can be treated with surgery using skull models and surgical simulation. The aim of this study was to emphasize the importance of preoperative surgical planning using 3D skull models in craniosynostosis surgery.
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Brachycephaly is the premature ossifications of cranial sutures. This condition can be treated with surgery using skull models and surgical simulation. The aim of this study was to emphasize the importance of preoperative surgical planning using 3D skull models in craniosynostosis surgery.
Brachycephaly is the premature ossifications of cranial sutures. This condition can be treated with surgery using skull models and surgical simulation. The aim of this study was to emphasize the importance of preoperative surgical planning using 3D skull models in craniosynostosis surgery.
Effectiveness of skull models and surgical simulation:
comparison of outcome between different surgical techniques in patients with isolated brachycephaly Hakan Emmez & smail Kkdk & Alp zgn Brcek & Aydemir Kale & Eren Seen & Gonca Erba & Reha Yavuzer & M. Kemali Baykaner Received: 13 May 2009 / Published online: 3 July 2009 # Springer-Verlag 2009 Abstract Purpose The aim of this study was to emphasize the impor- tance of preoperative surgical planning using 3D skull models in craniosynostosis surgery. Methods By using 3D polymethyl methacrylate skull models manufactured using 3D tomography images, the authors previously showed that after fronto-parietal osteotomy, instead of fixing the fronto-parietal bone flap without rotation, angled advancement with horizontal osteotomy provides maximum increase in intracranial volume, in a bilateral coronal craniosynostosis model. After changing the operation technique using data gathered from previous studies, we reviewed two bilateral craniosynostosis patients operated with the new technique and compared it with two patients that were operated with the old technique. Results Comparing cranial indexes (CI), significant improve- ment was detected in both groups. The decrease in CI in the second group was slightly better than the first group. In the comparison of intracranial volume (ICV), there was an increase in ICV values in both groups. The percentage of increase between two groups was similar. The morphological outcome was satisfactory in all patients. There were no major or minor complications and morbidity. Conclusions Current multislice tomography technology and stereolithographic procedures provide an excellent surgical simulation model to find new techniques and predict the outcome. These models should be used in all complex and syndromic craniosynostosis for both better results and reducing the operative time and associated blood loss. Keywords Brachycephaly . Craniosynostosis . Skull model . Surgical planning Introduction Craniosynostosis is the premature ossifications of cranial sutures that cause specific deformations of the skull. The incidence is reported to be 0.6/1,000 births [17]. The surgical correction of craniosynostosis is complex and cosmetic aspects present a major issue. An interdisciplinary approach is required for better outcome. Surgical time and blood loss are important in small children undergoing surgery. Brachy- cephaly can be isolated or present as a part of a syndrome. The length of the skull of these children is shortened, leading to a supraorbitonasal regression, whereas the breadth and the height are increased. In addition to a morphological alteration, premature synostosis prevents the adaptation of the cranial volume to the growing brain and causes intracranial hypertension which may lead to altered mental development and blindness [1, 14, 17]. The surgical goal in brachycephaly is not only to correct the dysmorphology but H. Emmez : A. . Brcek : A. Kale : E. Seen : M. K. Baykaner Division of Pediatric Neurosurgery, Department of Neurosurgery, Gazi University Faculty of Medicine, Ankara, Turkey . Kkdk : R. Yavuzer Department of Plastic and Reconstructive Surgery, Gazi University Faculty of Medicine, Ankara, Turkey G. Erba Department of Radiology, Gazi University Faculty of Medicine, Ankara, Turkey A. . Brcek (*) Beyin ve Sinir Cerrahisi AD, Gazi niversitesi Tp Fakltesi, Poliklinikler 1. Kat, 06500 Ankara, Turkey e-mail: alpob@yahoo.com Childs Nerv Syst (2009) 25:16051612 DOI 10.1007/s00381-009-0939-y also to enlarge the intracranial volume [1, 14]. Current computed tomography technology allows high-resolution imaging of craniosynostosis. Multiplane reconstructions can be performed for both diagnosis and surgical planning [12, 13]. By using 3D tomography images, a polymethyl methacrylate skull model can be manufactured. Virtual operative planning is possible by using these models (Fig. 1). By using these models, the authors previously showed that after fronto-parietal osteotomy, instead of fixing the fronto-parietal bone flap without rotation, angled advancement with horizontal osteotomy provides maximum increase in intracranial volume, in a bilateral coronal craniosynostosis model [4]. Depending on this finding, the authors change the operative technique in the last two cases and aim to analyze the effectiveness of two different surgical techniques in brachycephaly. As other malformations are present in syndromic cases, patients with isolated brachy- cephaly were selected to achieve better standardization. Fig. 1 A1A5 Left anterior plagiocephaly model in five projections; B1B5 scaphocephaly model in five projections; C1C5 Aperts syndrome model in five projections; D1D5 hypertelorism model in five projections; E1E5 trigonocephaly model in five projections 1606 Childs Nerv Syst (2009) 25:16051612 Patients and methods We found that instead of fixing the fronto-parietal bone flap without rotation, angled advancement with horizontal osteotomy provides maximum increase in intracranial volume (ICV) and maximum decrease in cephalic index (CI) in a bilateral coronal craniosynostosis model. After this finding, we changed our surgical technique. We reviewed the charts of two children with isolated brachycephaly who were operated with the old fronto-orbital advancement technique (180 rotation with horizontal osteotomy and angling) and the last two cases who were operated with new fronto-orbital advancement technique (only horizontal osteotomy and angling) in Gazi University Medical School between 2004 and 2008. Patients characteristics and outcome measures are shown in Table 1. Preoperative and postoperative computed tomography scans taken under similar conditions were compared in terms of ICV, CI, and morphological outcome. CI is the ratio of the maximum width of the head to its maximum length (i.e., in the horizontal plane, or front to back), sometimes multiplied by 100 for convenience. CI value is in close relation with morphology and has less effect on ICV. ICV measurement All patients underwent cranial computed tomography (CT) in both preoperative and postoperative period for assess- ment of intracranial volumes. CT examinations were performed with a 64-multidetector computed tomography (Lightspeed VCT, GE Healthcare, WI, USA). Scanning parameters were selected as follows: 100 kV, 180 mA, 0.625 mm slice thickness, and interval, field of view 22 cm. Patients were scanned from vertex to foramen magnum. After acquisition, images obtained were trans- ferred to a work station (GE Advantage Windows v.4.3). Axial images were used for volumetric reconstruction. The outline of each fifth section was traced manually. The computer stacked all data from each section together to build a virtual model. Volumetric values were obtained by the inherent software volume rendering algorithm. The calculated preoperative and postoperative intracranial volumes were compared. Morphological outcome is also evaluated. Surgical technique All of the operations were performed by the same surgeons in the craniofacial surgery team which includes neuro- surgeons and plastic and reconstructive surgeons. After injection of a local anesthetic, the operative procedure began with bicoronal skin incision behind the ears. The scalp was reflected in the subperiosteal layer up to the superior orbital rim. The temporalis muscle was also elevated and moved laterally in the subperiosteal plane to expose the pterion. Burr holes were placed according to the proposed craniotomy lines. The fronto-parietal bone flap involving the coronary sutures was elevated in one piece after the craniotomy which was performed 1 cm superior to the supraorbital bar. In the older technique, fronto-orbital bone flap was rotated 180 clockwise and divided into two pieces with horizontal osteotomy. Then, the pieces were fixed with angling using absorbable miniplates and screws. The new bone flap was fixed to supraorbital bar with absorbable miniplates and screws. The new bone flap was also fixed to temporal and parietal bones bilaterally with 1.2 cm advancement (Fig. 2). In the new technique after horizontal osteotomy to fronto-parietal bone flap, the bone flaps were fixed to each other with angling and then fixed to a supraorbital bar with absorbable miniplates and screws. The new bone flap was fixed to temporal and parietal bones bilaterally with 1.2 cm advancement (Fig. 3). We always use absorbable plating system since it allows the infants skull to grow once the system is absorbed, thus not inhibiting the necessary developmental growth seen with Table 1 Patients characteristics and operative techniques Patients Sex Age Operative technique 1 M 10 years Fronto-orbital advancement (180 rotation of fronto-parietal flap with horizontal osteotomy and angling) 2 F 36 months Fronto-orbital advancement (180 rotation of fronto-parietal flap with horizontal osteotomy and angling) 3 M 40 months Fronto-orbital advancement (only horizontal osteotomy and angling) 4 F 48 months Fronto-orbital advancement (only horizontal osteotomy and angling) Fig. 2 a, b Fronto-orbital advancement model with 180 rotation of fronto-parietal flap with horizontal osteotomy and angling Childs Nerv Syst (2009) 25:16051612 1607 the titanium system. After fixing with absorbable miniplates and screws, a silicone drain is placed under periosteal layer. Periosteum and scalp were closed. Results Forty-six patients with craniosynostosis were operated by the craniofacial surgery team in Gazi University Medical School between 2002 and 2008. This series includes hypertelorism (two patients), Aperts syndrome (three patients), Crouzon syndrome (two patients), plagiocephaly (13 patients), scaphocephaly (14 patients), and trigono- cephaly (eight patients). There were four isolated brachy- cephaly in this series. As the long-term follow-up period was not identical between patients, the authors have to analyze the short-term outcome. Fronto-orbital advance- ment with 180 rotation and angling was performed in two of the cases (patients 1 and 2), and fronto-orbital advance- ment with only horizontal osteotomy and angling of the bone flap was performed in the last two patients (patients 3 and 4). The CI, ICV, and morphological outcome in short term were compared. As the groups consist of two patients, no statistically significant data can be obtained. In the comparison of CI, significant improvement was detected in both groups. The decrease in CI in the second group was slightly better than the first group (Table 2). In the comparison of ICV, increase in ICV was found in both groups. The percentage of increase between the two groups was similar (Table 3). The morphological outcome was satisfactory in all patients (Fig. 4). There were no major or minor complications and morbidity. Discussion In 1851, Virchow [16] first suggested that premature fusion of cranial sutures is responsible for cranial dysmorphism. Since then, according to Virchows concept, to cure excision of the prematurely fused cranial suture had been tried by surgeons. Because of the refusion of the suture after the operation, this technique was abandoned. Today, we know that the local dura plays an important role. Secretion of transforming growth factor (TGF) and fibro- blast growth factor (FGF) from the local dura is responsible for inducing new bone formation and cranial suture fusion [10]. According to this theory, mere suture resection is followed by rapid osteogenesis and bone edge re-growth induced by TGF and FGF excreted from the dura. Single fused bone line excision cannot prevent the bone suture from refusion. In 1967, Tessier developed the fronto-orbital advance- ment method [15]. The main purpose of this method was to release any osseous constriction, correct the deformity, and allow for further normal growth. Marchac and Renier have utilized fronto-orbital advancement in treating craniosynos- tosis with encouraging results [8]. Brachycephaly is characterized by short anteroposterior cranial diameter, retropositioned supraorbital bar with abnormally wide cranium, and absence of frontal angle or even reversed. Usually, approximately 1-cm advancement is sufficient [2]. Brachycephaly most frequently occurs in syndromic craniosynostosis: Crouzon, Pfeiffer, and Apert syndromes. Fourteen percent of the cases are familial [1]. Bilateral Harlequin eye sign can be seen in plain skull radiographs (superior displacement of the lesser wings of both sphenoid bones). Brachycephaly causes functional and morphological problems that require fronto-orbital advance- ment in infancy to correct the brachycephalic deformity and Fig. 3 a, b Fronto-orbital advancement model with only horizontal osteotomy and angling Table 2 Cranial index values and changes Patients Preoperative CI Postoperative CI Decrease in CI (%) Interval between two CT (months) 1 95.2 86.7 9.2 3 2 93.6 84.2 10 2 3 93.7 84.9 11.3 1 4 99.2 86.3 13 3 CI cranial index, CT computed tomography Table 3 Intracranial volume measurements and changes Patients Preoperative ICV (cm 3 ) Postoperative ICV(cm 3 ) Increase in ICV (%) Interval between two CT (months) 1 1,407.9 1,462.5 3.6 3 2 993.3 1,052.7 5.9 2 3 1,207 1,259 4.1 1 4 1,267.1 1,373.9 7.7 3 ICV intracranial volume, CT computed tomography 1608 Childs Nerv Syst (2009) 25:16051612 to prevent mental impairment caused by the intracranial hypertension. The prevalence, natural history, and determi- nants of the condition are unclear. The mean CI by 2 years was 81.6% [1]. Postoperative mental status is better when surgery is performed before the patient reached 1 year of age. Although both morphological and functional outcomes are better in the subgroup that does not carry the FGFR3 P250R mutation, the differences were not statistically significant. Prominent bulging of the temporal fossa was frequently responsible for poor morphological outcome in carriers of this mutation [1]. Various surgical procedures have been devised to manage brachycephaly, but there is no standard surgical method in the treatment of brachycephaly. Suboptimal results may be related to the tendency of misdirected bone growth of the remaining cranium in brachycephaly. There- fore, the abnormal growth vectors should be corrected toward a more normal configuration [3]. In 1979, Marchac and Renier proposed the floating forehead concept. Elevation of supraorbital bar releases the fronto-ethmoidal and fronto-sphenoidal stenosis. Because the forehead is only attached to the face after advancement with no posterior fixation, this procedure enables the expanding brain to maintain and continue its advancement [3, 9]. Early surgery is recommended for all types of cranio- synostosis from both functional and technical points of view. Several studies showed that early intervention in other cases of craniosynostosis such as Apert syndrome and trigonocephaly prevents deterioration in mental function, which reinforces our conviction to operate early [11]. Regarding brachycephaly, surgical decompression may also be required in very young infants. A recent study conducted Fig. 4 Photograph showing the four patients and their 3D CT preoperatively and postoperatively (1a1d, 2a2d, 3a3d, 4a4d) Childs Nerv Syst (2009) 25:16051612 1609 among 99 children showed the necessity of treating them before the age of 1 year to prevent both morphological and functional impairment. The surgery may not improve a childs mental status but may prevent deterioration in mental function [1]. It had been suggested that early surgery might be deleterious to the fronto-glabellar morphology, with adverse effects on frontal sinus growth. However, it has been previously shown that the fronto-glabellar morphology was less dependent on frontal sinus growth after advance- ment and more on the morphology of the supraorbital bar itself. Brachiocephalic children who do not undergo surgery rarely develop a frontal sinus [1]. In this series, all the patients were older than 1 year, and increased intracranial pressure signs were observed in the cranial X-rays and bone flaps intraoperatively in all patients (Fig. 5). Cranioorbital normalization has become an objective of synostosis surgery with attention paid to the correction of forehead contour, browprojection, and the naso-frontal angle. Surgical reshaping, advancement, and stabilization of the caudal aspect of the frontal bones, the superior orbital rims, and the naso-frontal junction are performed in an attempt to achieve these goals. The standard operation includes bifrontal craniotomy with preservation of the caudal 1 cm of the frontal bones in continuity with the superior orbital rims, extended bilateral coronal suturectomies, re-contouring and anterior and caudal displacement of the supraorbital bar, and fixation of the advanced bar at the nasion (two different fixation methods) and at both pterions [6, 7]. The majority of published reports describe good result using postoperative photographs and images. These images provide objective outcome information, but they are not quantifiable and not available for comparison to any group. Recently, anthropometric measures are used to quantify the outcomes of different treatments for a given condition [17]. Although normative values often do not exist and there are no natural history studies that use such measurements as parameters, we evaluate the outcome in terms of cephalic index and intracranial volume. ICV is an important aspect of brachycephaly as mostly it is decreased and causes intracranial hypertension. CI can be used as an objective Fig. 6 1a, b Multiple osteotomy technique in brachycephaly in anteroposterior (a) and sagittal view (b); 2a, b fronto-orbital advancement technique with 180 rotation of fronto-parietal flap with horizontal osteotomy and angling in anteroposterior (a) and sagittal view (b); 3 fronto-orbital advancement technique with only horizontal osteotomy and angling in anteroposterior (a) and sagittal view (b); 4 only rotation technique in anteroposterior (a) and sagittal view (b); 5a only advancement technique Fig. 5 a Cranial X-ray showing the increased intracranial pressure sign; b fronto-parietal bone flap showing increased intracranial pressure 1610 Childs Nerv Syst (2009) 25:16051612 measurement of morphological outcome as it indicates the changes in the ratio of maximum width to maximum length of the cranium. We choose isolated brachycephaly to assess the effective- ness of using skull models since in syndromic cases associated malformations impair the standardization. Addi- tionally, in other non-syndromic craniosynostosis, procedures are well-defined with satisfactory outcomes. In brachy- cephaly, different advancement protocols are used, and increased intracranial pressure is an important aspect. We compared the changes in CI and ICV between five different surgical techniques including only fronto-orbital advance- ment, only rotation, advancement with 180 rotation and angling, only advancement with angling and multiple osteotomy technique, and only advancement technique (Fig. 6). We found that instead of fixing the fronto-parietal bone flap without rotation, angled advancement with horizontal osteotomy provided maximum increase in ICV and maximum decrease in CI in a bilateral coronal craniosynostosis model [13]. These findings encourage us to abandon our usual technique (advancement with 180 rotation, horizontal osteotomy, and angling) and perform only advancement with angling. After performing this technique in two children, we aimed to analyze the outcome in real patients. Since the number of patients is not enough, unfortunately, we could not have the chance to compare the patients with identical characteristics like age and follow-up period. The statistical analysis could not be performed also. In the comparison of two techniques, the decrease in CI was slightly better in the last technique; however, ICV increase was similar in both techniques. Morphological outcome was satisfactory in all patients. Since the number of patients is limited, statistical data could not be obtained. Also, patients characteristics like age and follow-up interval were not very similar. Larger series with similar patient characteristics can give us more objective information about the effectiveness of the surgical techniques. The surgical correction of craniosynostosis is a complex procedure and presents as an interdisciplinary challenge, as the procedure is comparatively rare even at specialized centers. As early surgery is beneficial, there is a demand for less drastic but effective techniques. Among other issues, surgical time and blood loss are of importance in small children undergoing surgery. Various surgical techniques have been described for brachycephaly. We think that to provide the best outcome with lower risks, virtual surgical planning is necessary for all patients with complex craniosynostosis. Current multislice computed tomography technology can be used for diagnosis and surgical planning with computer- assisted 3D visualization and surgical simulation. Three- dimensional models were created for the purpose of surgical simulation. These allow planning the course of the osteotomy and individual placing of the different bony fragments by an assigned matrix to simulate the surgical result and to assess the positioning of the individual bony fragments [57, 12]. Postprocessing time is the major disadvantage of 3D CT and limits the routine use of it. Stereolithography defines a 3D printing process that makes a solid object from a computer image by using a computer-controlled laser to draw the shape of the object onto the surface of a liquid plastic. We use skull models made of polymethyl methacrylate for operative simulation especially in patients with complex craniosynostosis and syndromic cases (Fig. 1). These models have important advantages like better evaluation than the 3D images, providing easy and virtual operative planning; shortening the operation time; and reducing the risks and complication, providing an excellent tool for parents cooperation and understanding, useful for training. Rotation and translation of the 3D model of the osteotomy segments was easy to handle. It provides better understanding of the pathology; operative simulation is easier and more real than the 3D CT models. Thus, the preoperatively planned surgery could be transferred directly to the operating table. Working on the model helps us to define the craniotomy borders, the bone flaps, and the new position of the flaps better. To know what to do and seeing the result of the procedure before the operation provides shorter operation time and more satis- factory outcome. Conclusion Although the expectation of genetic therapy for craniosy- nostosis will be possible in the future, today, as a surgeon, we have to find and try new techniques and procedures for better outcomes. Current multislice tomography technology and stereolithographic procedures provide excellent surgi- cal simulation models to find new techniques and predict the outcome. These models should be used in all complex and syndromic craniosynostosis. References 1. Arnaud E, Meneses P, Lajeunie E, Thorne JA, Marchac D, Renier D (2002) Postoperative mental and morphological outcome for nonsyndromic brachycephaly. Plast Reconstr Surg 110:612 discussion 13 2. Guo Z, Ding M, Mu X, Chen R, Suppl 2 (2007) Operative treatment of coronal craniosynostosis: 20 years of experience. Surg Neurol 68:S18S21 discussion S21 3. Koh KS, Kang MH, Yu SC, Park SH, Ra YS (2004) Treatment of nonsyndromic bilateral coronal synostosis using a multiple bone flap rotation-reposition technique. J Craniofac Surg 15: 603608 Childs Nerv Syst (2009) 25:16051612 1611 4. Kkdk , Demir HY, Kaya B (2008) zole bilateral koronal sinostoz modelinde farkl cerrahi tekniklerin sefalik indeks ve kafa ii hacim deiimine etkileri. 30th Turkish Plastic and Reconstructive Esthetic Surgery National Congress [oral presentation] 5. Levi D, Rampa F, Barbieri C, Pricca P, Franzini A, Pezzotta S (2002) True 3D reconstruction for planning of surgery on malformed skulls. Childs Nerv Syst 18:705706 6. Lo LJ, Marsh JL, Vannier MW, Patel VV (1994) Craniofacial computer-assisted surgical planning and simulation. Clin Plast Surg 21:501516 7. Lo LJ, Marsh JL, Yoon J, Vannier MW (1996) Stability of fronto- orbital advancement in nonsyndromic bilateral coronal synostosis: a quantitative three-dimensional computed tomographic study. Plast Reconstr Surg 98:393405 discussion 406399 8. Marchac D, Renier D (1979) The floating forehead. Early treatment of craniofacial stenosis. Ann Chir Plast 24:121126 9. Marchac D, Renier D, Jones BM (1988) Experience with the floating forehead. Br J Plast Surg 41:115 10. Morriss-Kay GM, Wilkie AO (2005) Growth of the normal skull vault and its alteration in craniosynostosis: insights from human genetics and experimental studies. J Anat 207:637653 11. Renier D, Brunet L, Marchac D (1987) IQ and craniostenosis: evolution in treated and untreated cases. In: Marchac D (ed) Craniofacial surgery. Springer, Berlin, pp 114117 12. Rodt T, Schlesinger A, Schramm A, Diensthuber M, Rittierodt M, Krauss JK (2007) 3D visualization and simulation of frontoorbital advancement in metopic synostosis. Childs Nerv Syst 23:13131317 13. Sannomiya EK, Reis SA, Asaumi J, Silva JV, Barbara AS, Kishi K (2006) Clinical and radiographic presentation and preparation of the prototyping model for pre-surgical planning in Apert's syndrome. Dentomaxillofac Radiol 35:119124 14. Schouman T, Vinchon M, Ruhin-Coupet B, Pellerin P, Dhellemmes P (2008) Isolated bilateral coronal synostosis: early treatment by peri-fronto-orbital craniectomy. J Craniofac Surg 19:4044 15. Tessier P (1967) Total facial osteotomy. Crouzon's syndrome, Aperts syndrome: oxycephaly, scaphocephaly, turricephaly. Ann Chir Plast 12:273286 16. Virchow R (1852) Uber den cretenismus, nametlich in Franken und uber pathologische Schadelformen. Verh Phys Med Ges Wurzburg 2:230 17. Walker ML, Collins JJ (2004) Nonsyndromic craniosynostosis and abnormalities of head shape. In: Winn ER (ed) Youmans neurological surgery. Saunders, Philedelphia, pp 33003314 1612 Childs Nerv Syst (2009) 25:16051612