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Clinical evaluation of non-syndromic scaphocephaly surgically corrected with the procedure of total vertex craniectomy
Journal of Cranio-Maxillo-Facial Surgery xxx (2018) 1e5
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Clinical evaluation of non-syndromic scaphocephaly surgically corrected with the procedure of total vertex craniectomy Matthias Kreppel*, Martin Kauke, Ali-Farid Safi, Andrea Grandoch, Nina Pocek-Behn, € ller Hans-Joachim Nickenig, Joachim Zo Department for Oral and Craniomaxillofacial Plastic Surgery, University of Cologne, Germany
a r t i c l e i n f o
a b s t r a c t
Article history: Paper received 3 October 2017 Accepted 30 May 2018 Available online xxx
The present investigation constitutes a retrospective evaluation of the outcome in children who received surgical correction of a scaphocephalic phenotype by median total vertex craniectomy. Between September 2009 and September 2015, a total of 35 infants with non-syndromic scaphocephaly were treated according to the same standardized operative technique of total vertex craniectomy by a single surgeon approach. At the time of surgery, the patients were between 3 and 12 months of age, with a median of 5 months. The mean duration of the procedure was 94 min. The duration of postoperative follow-up was a mean of 24 months (range 6e49 months). A total of 34 (97%) patients were successfully treated by total vertex craniectomy and were thus classified as category I according to the Whitaker score. Only one individual was assigned to category IV, necessitating secondary major craniofacial corrective surgery. Aesthetic outcomes were excellent in 34 cases and poor in one case. No major complication occurred. The reoperation rate was 3%. The surgical method we present herein is a wide median craniectomy which can be applied in young individuals with non-syndromic single-suture scaphocephaly. © 2018 European Association for Cranio-Maxillo-Facial Surgery. Published by Elsevier Ltd. All rights reserved.
Keywords: Craniofacial surgery Scaphocephaly Total vertex craniectomy Craniosynostosis
1. Introduction Craniosynostosis is defined by the premature closure of one or multiple cranial sutures, showing an annual incidence of approximately 1 in 2500 births (Persing, 2008; Safi et al., 2018a,b). An interplay of multiple causative factors (e.g., genetics, environment and teratogenics) are held responsible for the development of this disease (Wilkie et al., 2017). In approximately 15%, craniosynostotic deformities are associated with a genetic defect and a consecutive syndromic phenotype (termed syndromic craniosynostosis). However, nonsyndromic cases are much more frequent and constitute about 85% of all craniosynostosis cases (Abraham et al., 2018). Scaphocephaly, the premature closure of the sagittal suture, is the most frequent single suture nonsyndromic craniosynostosis, contributing 50e60% of all cases (Simpson et al., 2017). The incidence is about 1 in 5000 children, with a male to female ratio of 4 to
* Corresponding author. Department for Oral and Craniomaxillofacial and Plastic Surgery, University of Cologne, Kerpener Straße 62, 50931, Cologne, Germany. Fax: þ49 221 478 7360. E-mail address: [email protected] (M. Kreppel).
1 (Lajeunie et al., 1996; Kolar, 2011; Simpson et al., 2017). Considering the pathophysiology as propagated by Rudolph Virchow in 1851, sutural premature closure entails growth restriction perpendicular to the prematurely closed suture and compensatory growth at the remaining patent sutures (Virchow, 1851; Al-Shaqsi et al., 2018). Therefore in the case of sagittal synostosis, one can observe compensatory growth from the coronal and lambdoid sutural complex (Virchow, 1851; Simpson et al., 2017). This pathological growth pattern results in the typical scaphocephalic phenotype: a dysmorphic cranial vault, appearing boat-shaped due to an enlargement of the anterior-posterior diameter with a varying degree of temporal hollowing (narrowed transverse dimension) € ller, 2003; Simpson (Marsh and Vannier, 1986; Mühling, 1986; Zo et al., 2017). Consequently, the head is rather long and small, frequently showing frontal bossing and occipital coning (Persing, 2008; Simpson et al., 2017; Al-Shaqsi et al., 2018). As the sagittal suture is closed and osseous overgrowth occurs, an osseous prominence can frequently be palpated in the median plane (AlShaqsi et al., 2018). Craniofacial anomalies involving the sutural system are very complex entities of disease. Depending on the number and combination of premature suture obliterations, the repercussions are
https://doi.org/10.1016/j.jcms.2018.05.057 1010-5182/© 2018 European Association for Cranio-Maxillo-Facial Surgery. Published by Elsevier Ltd. All rights reserved.
Please cite this article in press as: Kreppel M, et al., Clinical evaluation of non-syndromic scaphocephaly surgically corrected with the procedure of total vertex craniectomy, Journal of Cranio-Maxillo-Facial Surgery (2018), https://doi.org/10.1016/j.jcms.2018.05.057
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M. Kreppel et al. / Journal of Cranio-Maxillo-Facial Surgery xxx (2018) 1e5
manifold. Most importantly, pathological restrictive growth patterns of the cranial vault may negatively impact the integrity of the brain. As the cranial vault represents an osseous cage specially designed to protect the brain, restricted expansion and elevated rigidity poses a hindrance to the brain's expansion, potentially resulting in an entrapment syndrome with neurological impair€ller, 2003; Safi et al., 2018a,b). Thus, in cases of clinical ment (Zo manifestation of intracranial hypertension, immediate surgical €ller, 2003; Safi intervention is warranted (Stein and Schut, 1977; Zo et al., 2018a,b). However, scaphocephaly is rarely associated with elevated intracranial pressure. Hence, surgical intervention generally aims to correct the cranial dysmorphia in an elective cosmetic surgical setting (Barritt et al., 1981; Greene and Winston, 1988; Al-Shaqsi et al., 2018). Due to our intrinsic sense of beauty, mainly governed by the fact that we judge aesthetics based on the absence or presence of specific proportions between craniofacial components, we are likely to negatively perceive individuals with clinically visible scaphocephaly. Hence, this malformation is likely to entail negative social pressure (Foltz and Loeser, 1975; Barritt et al., 1981). Here we evaluate a one-step surgical procedure that we routinely perform at our institution for correction of cranial dysmorphia due to premature sagittal synostosis, namely, the total vertex craniectomy. 2. Materials and methods Subjects in the retrospective cohort study presented herein were all patients who were diagnosed with a craniosynostosis and were consecutively treated at the Department of Oral and Craniomaxillofacial Plastic Surgery at the University of Cologne between September 2009 and September 2015. For consideration, patients had to meet the following criteria: nonsyndromic premature closure of the sagittal suture (scaphocephaly), treatment naive, total vertex craniectomy for correction of the dysmorphic skull, and follow-up care at our department. Predictor variables were age, sex, time point of surgical assessment, reason for initial presentation, postoperative complications, pre- and postoperative hemoglobin and duration of postsurgical follow-up. All patients who were referred to our clinic were clinically and radiographically evaluated by our head physician and surgeon. Accessory evaluation included standard preoperative hematologic diagnostics, ophthalmological examination and photographic documentation of the patient's dysmorphic craniofacial system. Postoperative evaluation included hematologic analysis and supervision by a pediatric specialist for 1 day in the intensive care unit (ICU) as well as for the remaining days of their postoperative hospitalization. In consideration of the as low as reasonably achievable (ALARA) principle, patients did not regularly receive pre- and/or postoperative x-raybased imaging. Imaging was applied only in difficult diagnostic situations and whenever postoperative imaging was necessary (e.g. exclusion of complications). Additionally, following the surgical procedure, each patient was re-evaluated by our head physician and surgeon. After their stay, patients were advised to regularly check in for follow-up. The follow-up standardly consisted of an evaluation of the surgical outcome (physical appearance, skull shape) and an evaluation of the overall aesthetics by both the surgeon and the parents of the individual. The comments on the aesthetic assessment were used to judge the success of the surgical procedure presented below.
department. Per standard procedure, every individual received general endotracheal anesthesia and perioperative care according to a standardized protocol. All individuals underwent to equal treatment for the correction of a single-suture sagittal craniosynostosis with scaphocephalic phenotype. We performed the procedure of total vertex craniectomy (approximately 9 cm wide) including the sagittal suture as well as the proximal coronal and lambdoid sutural complex. Figs. 1e6 present pre-, intra-, and postoperative pictures. With to this procedure, we intended to achieve unhindered brain expansion and consecutive autoinductive remodeling of the cranial vault, led by the expanding brain. Thus, this procedure does not involve active remodeling of the cranial vault. A detailed description follows. Prior to incision, a vasoconstrictive agent was administered intracutaneously following the course of the marked skin incision lines. The bicoronal incision was placed slightly posterior to the habitual bicoronal incision line. The superficial incision was followed by careful subgaleal dissection anteriorly and posteriorly, thereby exposing the cranial vertex (Figs. 4 and 5). In the median plane, the periost was divided. It is particularly important to visualize the coronal and lambdoid sutures. Multiple bilateral burr holes were made between the coronal and lambdoid sutures up to 4.5 cm lateral to the midline (Fig. 5). The osteotomy was carried out extending posteriorly of the lambdoid suture and anteriorly of the coronal suture (Fig. 5). A large portion of the occipital bone is removed. In cases in which the infant exceeded the fifth month of age, additional lateral osteotomies posterior to the lambdoid suture and at the coronal suture were performed. This is important, because the correction of the biparietal width becomes increasingly difficult as the malleability of the cranial bone decreases with the individual's age. Special care must be taken, as the additional osteotomies potentially increase the individual's blood loss. Importantly, an osseous fragment was left in situ for protection of the sagittal sinus (Fig. 5). In the end, after removal of the cranial vertex, tight wound closure was achieved. Two surgical drains were placed in order to allow for postoperative drainage (Fig. 6).
2.2. Evaluation of surgical outcome We evaluated the surgical outcome according to the classification of Whitaker et al. as recently modified and applied by Engel et al. and Safi et al. following fronto-orbital advancement, the subjective view of the patient's parents and the surgeon, as well as the rate of postoperative complications (Tables 1 and 2) (Whitaker et al., 1987; Engel et al., 2012; Safi et al., 2018a,b). If the surgeon as
2.1. Surgical technique All patients were treated by the same surgeon in cooperation with assisting surgeons from our local institutional neurosurgical
Fig. 1. Preoperative view of an individual affected by scaphocephaly.
Please cite this article in press as: Kreppel M, et al., Clinical evaluation of non-syndromic scaphocephaly surgically corrected with the procedure of total vertex craniectomy, Journal of Cranio-Maxillo-Facial Surgery (2018), https://doi.org/10.1016/j.jcms.2018.05.057
M. Kreppel et al. / Journal of Cranio-Maxillo-Facial Surgery xxx (2018) 1e5
Fig. 2. Preoperative view of an individual affected by scaphocephaly.
Fig. 3. Preoperative 3D-CT imagebased three-dimensional reconstruction of the skull of an individual with synostosis of the sagittal suture. The sutural prominence is clearly visible.
well as the parents were satisfied with the result, the aesthetic result was classified as excellent, and poor if either the parents or the surgeon were unsatisfied.
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Fig. 4. Intraoperative view of the cranial vertex and marked osteotomy lines. After a midline incision the vertex of the skull must be exposed with visualization of the sagittal, coronal and lambdoid sutures. The occipital prominence must be exposed as the procedure includes the resection of this portion.
occipital region, showing a diameter of approximately 5 cm. In 9 cases the surgical drains collected a great amount of blood, necessitating further transfusion of blood components. The evaluation at hand of the Whitaker Classification (Table 1) yielded the following results: 34 were categorized as Whitaker I (no refinements or surgical revisions considered advisable or necessary by the surgeon or the patient), and 1 individual was assigned to Whitaker IV (a major craniofacial procedure necessary, duplicating or exceeding the original operation). The individual assigned to Whitaker IV showed a persistent pathological growth pattern of the cranial vault despite undergoing total vertex craniectomy. Approximately 5 months following the initial treatment, the patient underwent a combination of fronto-orbital advancement and partial median craniectomy. The postoperative result was rated I according to the classification of Whitaker. Nine months following the second surgical intervention, the patient was re-evaluated and still showed an excellent result. The surgeon and parents were asked to rate the aesthetic outcome of the procedure. In only 1 case the surgeon and relatives agreed upon a poor aesthetic outcome (Whitaker IV, see above). However, after surgical correction, both the parents and the surgeon were fully satisfied.
3. Results 4. Discussion In total, 35 patients met our inclusion criteria. The diagnosis was validated by an experienced craniofacial surgeon. Mean age at the time of surgery was 5.1 months, ranging from 3 to 12 months. It is important that patients not exceed the first year of life in order to take advantage of the unique osteoinductive, osteoconductive and osteogenic potential of the dura mater (Mabbutt and Kokich, 1979; Hobar et al., 1993). This is an important factor and a preliminary theoretical consideration held responsible for successful osseous regeneration after the execution of a total vertex craniectomy. Only one patient had to be treated on an emergency basis due to elevated intracranial pressure; the other 34 patients were treated in an elective setting. The average preoperative weight was 7.5 kg, ranging from 5.4 to 10 kg. Preoperative and postoperative hemoglobin were 11.5 g/dl (range 9e13.5 g/dl) and 9.7 g/dl (range 7.4e13.5 g/dl), respectively. Mean duration of the surgical procedure was 95 min (range 49e281 min). Every individual received erythrocyte concentrates and fresh frozen plasma (FFP) with a mean amount of 265 ml (range 50e525 ml) and 240 ml (range 42.50e500 ml), respectively. No major postoperative complications occurred. One patient suffered from soft tissue necrosis in the
Patients with scaphocephalic deformities are an important patient cohort, necessitating special care and consideration. The deformity is rarely life-threatening; however, it frequently entails severe functional and aesthetic repercussions (Simpson et al., 2017; Al-Shaqsi et al., 2018). Functional impairment includes neurocognitive developmental delay and retardation of motor skills (Da Costa et al., 2012). In order to create sufficient space for the underlying brain to freely expand, and in order to create a wellshaped, aesthetically pleasant craniofacial complex, surgical intervention is recommended in the early years of an infant's life (Safi et al., 2018a,b). Most importantly, Al-Shaqsi et al. have just recently published an article about morphological and clinical changes in individuals affected by scaphocephaly whose parents opted against a surgical corrective intervention (Al-Shaqsi et al., 2018). The authors report that the scaphocephalic phenotype remained stable over the course of time and that the condition did not worsen or improve. In consideration of this investigation, surgical intervention seems necessary in order to correct the pathological cranial phenotype. In accordance with the literature, we
Please cite this article in press as: Kreppel M, et al., Clinical evaluation of non-syndromic scaphocephaly surgically corrected with the procedure of total vertex craniectomy, Journal of Cranio-Maxillo-Facial Surgery (2018), https://doi.org/10.1016/j.jcms.2018.05.057
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M. Kreppel et al. / Journal of Cranio-Maxillo-Facial Surgery xxx (2018) 1e5 Table 1 Classification in accordance with Whitaker et al., 1987. Category I Category II
Category III
Category IV
Fig. 5. Intraoperative view after reshaping and fixation. Repositioned bone fragment was fixated by a bioreabsorbable plating system.
No refinements or surgical revisions considered advisable or necessary by the surgeon or the patient Soft-tissue or lesser bone-contouring revisions desirable whether performed or not. In case of revision, these interventions were performed on an outpatient basis or with a hospital stay not exceeding 2 days. Major alternative osteotomies or bone grafting procedure desirable, i.e., orbital repositions, onlay bone grafts. These procedures were not as extensive as the original intervention. A major craniofacial procedure necessary, duplicating or exceeding the original operation.
Table 2 Clinical features. Median age (range), months Sex, n (%) Male Female Pre-operative weight Mean weight (range), kg Pre-operative HB concentration (range), g/dl Postoperative HB concentration (range), g/dl Perioperative blood products transfused - Erythrocyte concentration (range), ml - Fresh frozen plasma (range), ml Whitaker Score
Aesthetic outcome
Fig. 6. Postoperative view after wound closure and implementation of two surgical drains (upper view).
have experienced that many parents who have opted against a surgical correction have been unsatisfied with their decision afterward, mainly owing to the poor aesthetic outcome (Greene and Winston, 1988). Therefore an intervention that is mainly performed due to cosmetic reasons must be evaluated regarding its aesthetic outcome and its rate of complications (Greene and Winston, 1988; Simpson et al., 2017). However, the perception of a craniofacial system in terms of aesthetic consideration is very subjective in nature. Thus, all patients were carefully reviewed by our head physician who operated on all individuals himself and thereby was able to reliably compare pre- and postoperative cranial vault shape and aesthetic outcome. A widely used classification system for evaluation of the surgical outcome after craniofacial surgical procedures is the Whitaker score (Whitaker et al., 1987; Safi et al., 2018). We utilized this scoring system and thus assigned every patient to one of the groups ranging between I (no revision necessary) and IV (revision necessary, consisting of a surgical intervention that is either comparable or exceedingly serious in the surgical effort). In addition to that, we considered the surgeon as well as the individual's parents' opinion on the surgical outcome. Both were able to either classify the outcome as poor or excellent. In 34 out of 35 cases, the surgical procedure was evaluated as Whitaker I and aesthetically excellent. Taking these data into consideration, we can report on excellent postoperative results following the procedure of total vertex craniectomy for the correction of scaphocephaly. Other classification systems used to rate the success and feasibility of a surgical procedure for correction
Complications - Postoperative blood transfusion necessary - Skin necrosis - Without complications
5±2 29 (83%) 6 (17%) 7.5 ± 1.35 11.5 ± 1.1 9.7 ± 1.5 265 ± 122 240 ± 110 I ¼ 34 (97%) II ¼ 0 (0%) III ¼ 0 (0%) IV ¼ 1 (3%) Excellent n ¼ 34 (97%) Poor n ¼ 1 (3%) n¼9 n¼1 n ¼ 24
of the craniofacial complex is, for example, the image-based or clinically assessed cephalic index (CI, percentage of width to length) (van Lindert et al., 2013). The comparability to other surgical procedures is, however, limited when using the CI as a means for comparison. The results show high variability depending on the method of determining the CI (e.g. in images, or clinically with a caliper) (van Lindert et al., 2013). In order to provide an easy-tounderstand and easily reproducible method, we have opted to use the above-described classification system. Over the course of time, a number of different surgical approaches for the correction of scaphocephaly have been proposed: for example, the sagittal synostectomy, midline strip craniectomy, subtemporal craniectomy, total craniectomy, bilateral parasagittal strip craniectomy, the total vertex craniectomy, calvarial reconstruction techniques (H and Pi-procedure) and total cranial vault remodeling (TCVR) such as the Melbourne technique (Epstein €ller, 2003; Simpson et al., et al., 1982; Collmann et al., 1996; Zo 2017). Recently endoscopic techniques have been developed and are increasingly applied for the correction of scaphocephaly. However, techniques for total calvarial reshaping remain the most commonly applied method (Simpson et al., 2017). The application of restrictive procedures such as the strip craniectomy has been abandoned by many surgeons due to its insufficiency to fully correct the deformity by simply removing the closed suture (Simpson et al., 2017). Therefore, in order to achieve good longterm results, more extensive corrective procedures are generally preferred (Simpson et al., 2017). A more extensive surgical method
Please cite this article in press as: Kreppel M, et al., Clinical evaluation of non-syndromic scaphocephaly surgically corrected with the procedure of total vertex craniectomy, Journal of Cranio-Maxillo-Facial Surgery (2018), https://doi.org/10.1016/j.jcms.2018.05.057
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for correction of scaphocephaly is presented within this article (see Surgical technique section above). The procedure is essentially a large median craniectomy with additional resection of €ller and occipital and frontal bony segments as propagated by Zo €ller and Mühling, 2012). The Epstein et al (Epstein et al., 1982; Zo total vertex craniectomy shows excellent clinical results and is frequently preferred over the other methods in the treatment of € ller, 2003). Rapid clinical and radiological young infants (Zo amelioration of the preoperative clinical phenotype are visible only 2 weeks following the intervention (Epstein et al., 1982). Importantly, all of the above-mentioned procedures are performed within the first months of an individual's life in order to take advantage of the great plasticity that is unique to an infant's dura mater, which should always be left untouched (Epstein et al., €ller, 2003; Simpson et al., 2017; Safi et al., 2018a,b). The 1982; Zo dura bears a great osteoinductive, osteoconductive and osteogenic potential, providing great plasticity when it comes to bone regeneration and remodeling. Additionally, the aforementioned procedures (including the total vertex craniectomy) rely on the brain's expansion and consecutive passive cranial reshaping. Therefore the methods are less likely to sufficiently correct the malformed cranial vault in individuals exceeding the first year of € ller, 2003; Simpson et al., 2017). Additionally, methods that life (Zo cause vast osseous defects can entail large calvarial defects after surgery. We have not observed this complication following total vertex craniectomy, even after having performed the procedure on a 1-year-old individual; however, one must be aware of the possibility of this complication in older individuals. More extensive methods rely less on passive reshaping mechanisms and allow active resphaping of the cranium for calvarial reconstruction. They are rather suitable for older patients (>1 year of age) (Simpson et al., 2017). Due to the fact that extensive reshaping approaches resect smaller osseous fragments, the risk of malossification with large calvarial defects is reduced when compared to the abovementioned procedures such as our total vertex craniectomy. Excellent examples of such procedures are the H and Pi-Procedure as well as TCVR according to the Melbourne technique (Simpson et al., 2017). The Pi-Procedure (“squeeze procedure” according to Jane and Persing, 1986) consited of a pi (p)shaped resection of the cranial vault the resected coronal sutures represent the horizontal line and the flanks of the p-sign are represented by the occipitally extending osteotomy lines lateral to the sagittal suture €ller, 2003; Simpson et al., 2017). This method allows easy (Zo reshaping of the cranial vault by “squeezing” in an anteriorposterior direction, inducing a shortening of the anteriorposterior dimension of the individual's skull. In accordance with the results of Epstein et al., who regularly performed the total vertex craniectomy, we can report excellent results which are generally apparent within only 1 week to 10 days, both clinically and radiographically (Epstein et al., 1982). Additionally, we can report phenomenal long-term results and a low reoperation rate.
5. Conclusion Considering our data, one can reasonably conclude that the surgical technique of total vertex craniectomy is a procedure with a very low rate of clinical complications and a very high rate of excellent aesthetic outcomes. The procedure is suitable for young individuals less than 1 year of age. Consequently, young individuals with sagittal synostosis with scaphocephalic phenotype can be
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surgically treated with the help of the surgical procedure presented herein, a modified median craniectomy. Conflict of interest The authors report no financial disclosure and no conflicts of interest. Acknowledgements Figs. 1e6 presented are property of Univ.-Prof. Dr. Dr. Joachim € ller. Zo References Abraham P, Brandel MG, Dalle CO, Reid CM, Kpaduwa CS, Lance S, et al: Predictors of postoperative complications of craniosynostosis repair in the national inpatient sample. Ann Plast Surg, 2018 Al-Shaqsi S, Zellner E, Ching J, Forrest C, Phillips J: The natural history of cranial morphology in sagittal craniosynostosis. J Craniofac Surg, 2018 Barritt J, Brooksbank M, Simpson D: Scaphocephaly: aesthetic and psychosocial considerations. Dev Med Child Neurol 23(2): 183e191, 1981 € rensen N, Krauß J, Reinhart E, Pistner H: Kraniosynostosen-PrinziCollmann H, So pien und Risiken der chirurgischen Behandlung. Br J Oral Maxillofac Surg 34(5): 477, 1996 Da Costa AC, Anderson VA, Savarirayan R, Wrennall JA, Chong DK, Holmes AD, et al: Neurodevelopmental functioning of infants with untreated singlesuture craniosynostosis during early infancy. Childs Nerv Syst 28(6): 869e877, 2012 Engel M, Thiele OC, Mühling J, Hoffmann J, Freier K, Castrillon-Oberndorfer G, et al: Trigonocephaly: results after surgical correction of nonsyndromatic isolated metopic suture synostosis in 54 cases. J Craniomaxillofac Surg 40(4): 347e353, 2012 Epstein N, Epstein F, Newman G: Total vertex craniectomy for the treatment of scaphocephaly. Childs Brain 9(5): 309e316, 1982 Foltz EL, Loeser JD: Craniosynostosis. J Neurosurg 43(1): 48e57, 1975 Greene Jr CS, Winston KR: Treatment of scaphocephaly with sagittal craniectomy and biparietal morcellation. Neurosurgery 23(2): 196e202, 1988 Hobar PC, Schreiber JS, McCarthy JG, Thomas PA: The role of the dura in cranial bone regeneration in the immature animal. Plast Reconstr Surg 92(3): 405e410, 1993 Kolar JC: An epidemiological study of nonsyndromal craniosynostoses. J Craniofac Surg 22(1): 47e49, 2011 Lajeunie E, Le Merrer M, Bonaiti-Pellie C, Marchac D, Renier D: Genetic study of scaphocephaly. Am J Med Genet 62(3): 282e285, 1996 Mabbutt LW, Kokich VG: Calvarial and sutural re-development following craniectomy in the neonatal rabbit. J Anat 129(Pt 2): 413e422, 1979 Marsh JL, Vannier MW: Cranial base changes following surgical treatment of craniosynostosis. Cleft Palate J 23(Suppl. 1): 9e18, 1986 Mühling J: Surgical treatment of premature craniosynostosis; 1986 Persing JA: MOC-PS(SM) CME article: management considerations in the treatment of craniosynostosis. Plast Reconstr Surg 121(4 Suppl): 1e11, 2008 €ller J: Clinical evaluation Safi A-F, Kreppel M, Grandoch A, Kauke M, Nickenig H-J, Zo of standardized fronto-orbital advancement for correction of isolated trigonocephaly. J Craniofac Surg 29(1): 72e75, 2018a €ller J: Rigid external Safi A-F, Kreppel M, Kauke M, Grandoch A, Nickenig H-J, Zo distractor-aided advancement after simultaneously performed lefort-iii osteotomy and fronto-orbital advancement. J Craniofac Surg 29(1): 170e174, 2018b Simpson A, Wong AL, Bezuhly M: Surgical correction of nonsyndromic sagittal craniosynostosis: concepts and controversies. Ann Plast Surg 78(1): 103e110, 2017 Stein SC, Schut L: Management of scaphocephaly. Surg Neurol 7(3): 153e155, 1977 SJ, Maal TJ, et al: Validation of van Lindert EJ, Siepel FJ, Delye H, Ettema AM, Berge cephalic index measurements in scaphocephaly. Childs Nerv Syst 29(6): 1007e1014, 2013 Virchow R. Ueber den Cretinismus, Namentlich in Franken, und uber patholovische Schadedformen. (Quoted by numerous authors.) Verh Phus-Med Ges Warzburg, vol. 2; 1851, a30, 1851 Whitaker LA, Bartlett SP, Schut L, Bruce D: Craniosynostosis: an analysis of the timing, treatment, and complications in 164 consecutive patients. Plast Reconstr Surg 80(2): 195e206, 1987 Wilkie AO, Johnson D, Wall SA: Clinical genetics of craniosynostosis. Curr Opin Pediatr 29(6): 622e628, 2017 €ller J, Mühling J: Kraniofaziale Chirurgie. Mund- Kiefer-und Gesichtschirurgie: Zo 417e438, 2012 €ller JE: Kraniofaziale Chirurgie: Diagnostik und Therapie kraniofazialer FehlbilZo dungen; 16 Tabellen, Georg Thieme Verlag; 2003
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Clinical evaluation of non-syndromic scaphocephaly surgically corrected with the procedure of total vertex craniectomy Matthias Kreppel*, Martin Kauke, Ali-Farid Safi, Andrea Grandoch, Nina Pocek-Behn, € ller Hans-Joachim Nickenig, Joachim Zo Department for Oral and Craniomaxillofacial Plastic Surgery, University of Cologne, Germany
a r t i c l e i n f o
a b s t r a c t
Article history: Paper received 3 October 2017 Accepted 30 May 2018 Available online xxx
The present investigation constitutes a retrospective evaluation of the outcome in children who received surgical correction of a scaphocephalic phenotype by median total vertex craniectomy. Between September 2009 and September 2015, a total of 35 infants with non-syndromic scaphocephaly were treated according to the same standardized operative technique of total vertex craniectomy by a single surgeon approach. At the time of surgery, the patients were between 3 and 12 months of age, with a median of 5 months. The mean duration of the procedure was 94 min. The duration of postoperative follow-up was a mean of 24 months (range 6e49 months). A total of 34 (97%) patients were successfully treated by total vertex craniectomy and were thus classified as category I according to the Whitaker score. Only one individual was assigned to category IV, necessitating secondary major craniofacial corrective surgery. Aesthetic outcomes were excellent in 34 cases and poor in one case. No major complication occurred. The reoperation rate was 3%. The surgical method we present herein is a wide median craniectomy which can be applied in young individuals with non-syndromic single-suture scaphocephaly. © 2018 European Association for Cranio-Maxillo-Facial Surgery. Published by Elsevier Ltd. All rights reserved.
Keywords: Craniofacial surgery Scaphocephaly Total vertex craniectomy Craniosynostosis
1. Introduction Craniosynostosis is defined by the premature closure of one or multiple cranial sutures, showing an annual incidence of approximately 1 in 2500 births (Persing, 2008; Safi et al., 2018a,b). An interplay of multiple causative factors (e.g., genetics, environment and teratogenics) are held responsible for the development of this disease (Wilkie et al., 2017). In approximately 15%, craniosynostotic deformities are associated with a genetic defect and a consecutive syndromic phenotype (termed syndromic craniosynostosis). However, nonsyndromic cases are much more frequent and constitute about 85% of all craniosynostosis cases (Abraham et al., 2018). Scaphocephaly, the premature closure of the sagittal suture, is the most frequent single suture nonsyndromic craniosynostosis, contributing 50e60% of all cases (Simpson et al., 2017). The incidence is about 1 in 5000 children, with a male to female ratio of 4 to
* Corresponding author. Department for Oral and Craniomaxillofacial and Plastic Surgery, University of Cologne, Kerpener Straße 62, 50931, Cologne, Germany. Fax: þ49 221 478 7360. E-mail address: [email protected] (M. Kreppel).
1 (Lajeunie et al., 1996; Kolar, 2011; Simpson et al., 2017). Considering the pathophysiology as propagated by Rudolph Virchow in 1851, sutural premature closure entails growth restriction perpendicular to the prematurely closed suture and compensatory growth at the remaining patent sutures (Virchow, 1851; Al-Shaqsi et al., 2018). Therefore in the case of sagittal synostosis, one can observe compensatory growth from the coronal and lambdoid sutural complex (Virchow, 1851; Simpson et al., 2017). This pathological growth pattern results in the typical scaphocephalic phenotype: a dysmorphic cranial vault, appearing boat-shaped due to an enlargement of the anterior-posterior diameter with a varying degree of temporal hollowing (narrowed transverse dimension) € ller, 2003; Simpson (Marsh and Vannier, 1986; Mühling, 1986; Zo et al., 2017). Consequently, the head is rather long and small, frequently showing frontal bossing and occipital coning (Persing, 2008; Simpson et al., 2017; Al-Shaqsi et al., 2018). As the sagittal suture is closed and osseous overgrowth occurs, an osseous prominence can frequently be palpated in the median plane (AlShaqsi et al., 2018). Craniofacial anomalies involving the sutural system are very complex entities of disease. Depending on the number and combination of premature suture obliterations, the repercussions are
https://doi.org/10.1016/j.jcms.2018.05.057 1010-5182/© 2018 European Association for Cranio-Maxillo-Facial Surgery. Published by Elsevier Ltd. All rights reserved.
Please cite this article in press as: Kreppel M, et al., Clinical evaluation of non-syndromic scaphocephaly surgically corrected with the procedure of total vertex craniectomy, Journal of Cranio-Maxillo-Facial Surgery (2018), https://doi.org/10.1016/j.jcms.2018.05.057
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M. Kreppel et al. / Journal of Cranio-Maxillo-Facial Surgery xxx (2018) 1e5
manifold. Most importantly, pathological restrictive growth patterns of the cranial vault may negatively impact the integrity of the brain. As the cranial vault represents an osseous cage specially designed to protect the brain, restricted expansion and elevated rigidity poses a hindrance to the brain's expansion, potentially resulting in an entrapment syndrome with neurological impair€ller, 2003; Safi et al., 2018a,b). Thus, in cases of clinical ment (Zo manifestation of intracranial hypertension, immediate surgical €ller, 2003; Safi intervention is warranted (Stein and Schut, 1977; Zo et al., 2018a,b). However, scaphocephaly is rarely associated with elevated intracranial pressure. Hence, surgical intervention generally aims to correct the cranial dysmorphia in an elective cosmetic surgical setting (Barritt et al., 1981; Greene and Winston, 1988; Al-Shaqsi et al., 2018). Due to our intrinsic sense of beauty, mainly governed by the fact that we judge aesthetics based on the absence or presence of specific proportions between craniofacial components, we are likely to negatively perceive individuals with clinically visible scaphocephaly. Hence, this malformation is likely to entail negative social pressure (Foltz and Loeser, 1975; Barritt et al., 1981). Here we evaluate a one-step surgical procedure that we routinely perform at our institution for correction of cranial dysmorphia due to premature sagittal synostosis, namely, the total vertex craniectomy. 2. Materials and methods Subjects in the retrospective cohort study presented herein were all patients who were diagnosed with a craniosynostosis and were consecutively treated at the Department of Oral and Craniomaxillofacial Plastic Surgery at the University of Cologne between September 2009 and September 2015. For consideration, patients had to meet the following criteria: nonsyndromic premature closure of the sagittal suture (scaphocephaly), treatment naive, total vertex craniectomy for correction of the dysmorphic skull, and follow-up care at our department. Predictor variables were age, sex, time point of surgical assessment, reason for initial presentation, postoperative complications, pre- and postoperative hemoglobin and duration of postsurgical follow-up. All patients who were referred to our clinic were clinically and radiographically evaluated by our head physician and surgeon. Accessory evaluation included standard preoperative hematologic diagnostics, ophthalmological examination and photographic documentation of the patient's dysmorphic craniofacial system. Postoperative evaluation included hematologic analysis and supervision by a pediatric specialist for 1 day in the intensive care unit (ICU) as well as for the remaining days of their postoperative hospitalization. In consideration of the as low as reasonably achievable (ALARA) principle, patients did not regularly receive pre- and/or postoperative x-raybased imaging. Imaging was applied only in difficult diagnostic situations and whenever postoperative imaging was necessary (e.g. exclusion of complications). Additionally, following the surgical procedure, each patient was re-evaluated by our head physician and surgeon. After their stay, patients were advised to regularly check in for follow-up. The follow-up standardly consisted of an evaluation of the surgical outcome (physical appearance, skull shape) and an evaluation of the overall aesthetics by both the surgeon and the parents of the individual. The comments on the aesthetic assessment were used to judge the success of the surgical procedure presented below.
department. Per standard procedure, every individual received general endotracheal anesthesia and perioperative care according to a standardized protocol. All individuals underwent to equal treatment for the correction of a single-suture sagittal craniosynostosis with scaphocephalic phenotype. We performed the procedure of total vertex craniectomy (approximately 9 cm wide) including the sagittal suture as well as the proximal coronal and lambdoid sutural complex. Figs. 1e6 present pre-, intra-, and postoperative pictures. With to this procedure, we intended to achieve unhindered brain expansion and consecutive autoinductive remodeling of the cranial vault, led by the expanding brain. Thus, this procedure does not involve active remodeling of the cranial vault. A detailed description follows. Prior to incision, a vasoconstrictive agent was administered intracutaneously following the course of the marked skin incision lines. The bicoronal incision was placed slightly posterior to the habitual bicoronal incision line. The superficial incision was followed by careful subgaleal dissection anteriorly and posteriorly, thereby exposing the cranial vertex (Figs. 4 and 5). In the median plane, the periost was divided. It is particularly important to visualize the coronal and lambdoid sutures. Multiple bilateral burr holes were made between the coronal and lambdoid sutures up to 4.5 cm lateral to the midline (Fig. 5). The osteotomy was carried out extending posteriorly of the lambdoid suture and anteriorly of the coronal suture (Fig. 5). A large portion of the occipital bone is removed. In cases in which the infant exceeded the fifth month of age, additional lateral osteotomies posterior to the lambdoid suture and at the coronal suture were performed. This is important, because the correction of the biparietal width becomes increasingly difficult as the malleability of the cranial bone decreases with the individual's age. Special care must be taken, as the additional osteotomies potentially increase the individual's blood loss. Importantly, an osseous fragment was left in situ for protection of the sagittal sinus (Fig. 5). In the end, after removal of the cranial vertex, tight wound closure was achieved. Two surgical drains were placed in order to allow for postoperative drainage (Fig. 6).
2.2. Evaluation of surgical outcome We evaluated the surgical outcome according to the classification of Whitaker et al. as recently modified and applied by Engel et al. and Safi et al. following fronto-orbital advancement, the subjective view of the patient's parents and the surgeon, as well as the rate of postoperative complications (Tables 1 and 2) (Whitaker et al., 1987; Engel et al., 2012; Safi et al., 2018a,b). If the surgeon as
2.1. Surgical technique All patients were treated by the same surgeon in cooperation with assisting surgeons from our local institutional neurosurgical
Fig. 1. Preoperative view of an individual affected by scaphocephaly.
Please cite this article in press as: Kreppel M, et al., Clinical evaluation of non-syndromic scaphocephaly surgically corrected with the procedure of total vertex craniectomy, Journal of Cranio-Maxillo-Facial Surgery (2018), https://doi.org/10.1016/j.jcms.2018.05.057
M. Kreppel et al. / Journal of Cranio-Maxillo-Facial Surgery xxx (2018) 1e5
Fig. 2. Preoperative view of an individual affected by scaphocephaly.
Fig. 3. Preoperative 3D-CT imagebased three-dimensional reconstruction of the skull of an individual with synostosis of the sagittal suture. The sutural prominence is clearly visible.
well as the parents were satisfied with the result, the aesthetic result was classified as excellent, and poor if either the parents or the surgeon were unsatisfied.
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Fig. 4. Intraoperative view of the cranial vertex and marked osteotomy lines. After a midline incision the vertex of the skull must be exposed with visualization of the sagittal, coronal and lambdoid sutures. The occipital prominence must be exposed as the procedure includes the resection of this portion.
occipital region, showing a diameter of approximately 5 cm. In 9 cases the surgical drains collected a great amount of blood, necessitating further transfusion of blood components. The evaluation at hand of the Whitaker Classification (Table 1) yielded the following results: 34 were categorized as Whitaker I (no refinements or surgical revisions considered advisable or necessary by the surgeon or the patient), and 1 individual was assigned to Whitaker IV (a major craniofacial procedure necessary, duplicating or exceeding the original operation). The individual assigned to Whitaker IV showed a persistent pathological growth pattern of the cranial vault despite undergoing total vertex craniectomy. Approximately 5 months following the initial treatment, the patient underwent a combination of fronto-orbital advancement and partial median craniectomy. The postoperative result was rated I according to the classification of Whitaker. Nine months following the second surgical intervention, the patient was re-evaluated and still showed an excellent result. The surgeon and parents were asked to rate the aesthetic outcome of the procedure. In only 1 case the surgeon and relatives agreed upon a poor aesthetic outcome (Whitaker IV, see above). However, after surgical correction, both the parents and the surgeon were fully satisfied.
3. Results 4. Discussion In total, 35 patients met our inclusion criteria. The diagnosis was validated by an experienced craniofacial surgeon. Mean age at the time of surgery was 5.1 months, ranging from 3 to 12 months. It is important that patients not exceed the first year of life in order to take advantage of the unique osteoinductive, osteoconductive and osteogenic potential of the dura mater (Mabbutt and Kokich, 1979; Hobar et al., 1993). This is an important factor and a preliminary theoretical consideration held responsible for successful osseous regeneration after the execution of a total vertex craniectomy. Only one patient had to be treated on an emergency basis due to elevated intracranial pressure; the other 34 patients were treated in an elective setting. The average preoperative weight was 7.5 kg, ranging from 5.4 to 10 kg. Preoperative and postoperative hemoglobin were 11.5 g/dl (range 9e13.5 g/dl) and 9.7 g/dl (range 7.4e13.5 g/dl), respectively. Mean duration of the surgical procedure was 95 min (range 49e281 min). Every individual received erythrocyte concentrates and fresh frozen plasma (FFP) with a mean amount of 265 ml (range 50e525 ml) and 240 ml (range 42.50e500 ml), respectively. No major postoperative complications occurred. One patient suffered from soft tissue necrosis in the
Patients with scaphocephalic deformities are an important patient cohort, necessitating special care and consideration. The deformity is rarely life-threatening; however, it frequently entails severe functional and aesthetic repercussions (Simpson et al., 2017; Al-Shaqsi et al., 2018). Functional impairment includes neurocognitive developmental delay and retardation of motor skills (Da Costa et al., 2012). In order to create sufficient space for the underlying brain to freely expand, and in order to create a wellshaped, aesthetically pleasant craniofacial complex, surgical intervention is recommended in the early years of an infant's life (Safi et al., 2018a,b). Most importantly, Al-Shaqsi et al. have just recently published an article about morphological and clinical changes in individuals affected by scaphocephaly whose parents opted against a surgical corrective intervention (Al-Shaqsi et al., 2018). The authors report that the scaphocephalic phenotype remained stable over the course of time and that the condition did not worsen or improve. In consideration of this investigation, surgical intervention seems necessary in order to correct the pathological cranial phenotype. In accordance with the literature, we
Please cite this article in press as: Kreppel M, et al., Clinical evaluation of non-syndromic scaphocephaly surgically corrected with the procedure of total vertex craniectomy, Journal of Cranio-Maxillo-Facial Surgery (2018), https://doi.org/10.1016/j.jcms.2018.05.057
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M. Kreppel et al. / Journal of Cranio-Maxillo-Facial Surgery xxx (2018) 1e5 Table 1 Classification in accordance with Whitaker et al., 1987. Category I Category II
Category III
Category IV
Fig. 5. Intraoperative view after reshaping and fixation. Repositioned bone fragment was fixated by a bioreabsorbable plating system.
No refinements or surgical revisions considered advisable or necessary by the surgeon or the patient Soft-tissue or lesser bone-contouring revisions desirable whether performed or not. In case of revision, these interventions were performed on an outpatient basis or with a hospital stay not exceeding 2 days. Major alternative osteotomies or bone grafting procedure desirable, i.e., orbital repositions, onlay bone grafts. These procedures were not as extensive as the original intervention. A major craniofacial procedure necessary, duplicating or exceeding the original operation.
Table 2 Clinical features. Median age (range), months Sex, n (%) Male Female Pre-operative weight Mean weight (range), kg Pre-operative HB concentration (range), g/dl Postoperative HB concentration (range), g/dl Perioperative blood products transfused - Erythrocyte concentration (range), ml - Fresh frozen plasma (range), ml Whitaker Score
Aesthetic outcome
Fig. 6. Postoperative view after wound closure and implementation of two surgical drains (upper view).
have experienced that many parents who have opted against a surgical correction have been unsatisfied with their decision afterward, mainly owing to the poor aesthetic outcome (Greene and Winston, 1988). Therefore an intervention that is mainly performed due to cosmetic reasons must be evaluated regarding its aesthetic outcome and its rate of complications (Greene and Winston, 1988; Simpson et al., 2017). However, the perception of a craniofacial system in terms of aesthetic consideration is very subjective in nature. Thus, all patients were carefully reviewed by our head physician who operated on all individuals himself and thereby was able to reliably compare pre- and postoperative cranial vault shape and aesthetic outcome. A widely used classification system for evaluation of the surgical outcome after craniofacial surgical procedures is the Whitaker score (Whitaker et al., 1987; Safi et al., 2018). We utilized this scoring system and thus assigned every patient to one of the groups ranging between I (no revision necessary) and IV (revision necessary, consisting of a surgical intervention that is either comparable or exceedingly serious in the surgical effort). In addition to that, we considered the surgeon as well as the individual's parents' opinion on the surgical outcome. Both were able to either classify the outcome as poor or excellent. In 34 out of 35 cases, the surgical procedure was evaluated as Whitaker I and aesthetically excellent. Taking these data into consideration, we can report on excellent postoperative results following the procedure of total vertex craniectomy for the correction of scaphocephaly. Other classification systems used to rate the success and feasibility of a surgical procedure for correction
Complications - Postoperative blood transfusion necessary - Skin necrosis - Without complications
5±2 29 (83%) 6 (17%) 7.5 ± 1.35 11.5 ± 1.1 9.7 ± 1.5 265 ± 122 240 ± 110 I ¼ 34 (97%) II ¼ 0 (0%) III ¼ 0 (0%) IV ¼ 1 (3%) Excellent n ¼ 34 (97%) Poor n ¼ 1 (3%) n¼9 n¼1 n ¼ 24
of the craniofacial complex is, for example, the image-based or clinically assessed cephalic index (CI, percentage of width to length) (van Lindert et al., 2013). The comparability to other surgical procedures is, however, limited when using the CI as a means for comparison. The results show high variability depending on the method of determining the CI (e.g. in images, or clinically with a caliper) (van Lindert et al., 2013). In order to provide an easy-tounderstand and easily reproducible method, we have opted to use the above-described classification system. Over the course of time, a number of different surgical approaches for the correction of scaphocephaly have been proposed: for example, the sagittal synostectomy, midline strip craniectomy, subtemporal craniectomy, total craniectomy, bilateral parasagittal strip craniectomy, the total vertex craniectomy, calvarial reconstruction techniques (H and Pi-procedure) and total cranial vault remodeling (TCVR) such as the Melbourne technique (Epstein €ller, 2003; Simpson et al., et al., 1982; Collmann et al., 1996; Zo 2017). Recently endoscopic techniques have been developed and are increasingly applied for the correction of scaphocephaly. However, techniques for total calvarial reshaping remain the most commonly applied method (Simpson et al., 2017). The application of restrictive procedures such as the strip craniectomy has been abandoned by many surgeons due to its insufficiency to fully correct the deformity by simply removing the closed suture (Simpson et al., 2017). Therefore, in order to achieve good longterm results, more extensive corrective procedures are generally preferred (Simpson et al., 2017). A more extensive surgical method
Please cite this article in press as: Kreppel M, et al., Clinical evaluation of non-syndromic scaphocephaly surgically corrected with the procedure of total vertex craniectomy, Journal of Cranio-Maxillo-Facial Surgery (2018), https://doi.org/10.1016/j.jcms.2018.05.057
M. Kreppel et al. / Journal of Cranio-Maxillo-Facial Surgery xxx (2018) 1e5
for correction of scaphocephaly is presented within this article (see Surgical technique section above). The procedure is essentially a large median craniectomy with additional resection of €ller and occipital and frontal bony segments as propagated by Zo €ller and Mühling, 2012). The Epstein et al (Epstein et al., 1982; Zo total vertex craniectomy shows excellent clinical results and is frequently preferred over the other methods in the treatment of € ller, 2003). Rapid clinical and radiological young infants (Zo amelioration of the preoperative clinical phenotype are visible only 2 weeks following the intervention (Epstein et al., 1982). Importantly, all of the above-mentioned procedures are performed within the first months of an individual's life in order to take advantage of the great plasticity that is unique to an infant's dura mater, which should always be left untouched (Epstein et al., €ller, 2003; Simpson et al., 2017; Safi et al., 2018a,b). The 1982; Zo dura bears a great osteoinductive, osteoconductive and osteogenic potential, providing great plasticity when it comes to bone regeneration and remodeling. Additionally, the aforementioned procedures (including the total vertex craniectomy) rely on the brain's expansion and consecutive passive cranial reshaping. Therefore the methods are less likely to sufficiently correct the malformed cranial vault in individuals exceeding the first year of € ller, 2003; Simpson et al., 2017). Additionally, methods that life (Zo cause vast osseous defects can entail large calvarial defects after surgery. We have not observed this complication following total vertex craniectomy, even after having performed the procedure on a 1-year-old individual; however, one must be aware of the possibility of this complication in older individuals. More extensive methods rely less on passive reshaping mechanisms and allow active resphaping of the cranium for calvarial reconstruction. They are rather suitable for older patients (>1 year of age) (Simpson et al., 2017). Due to the fact that extensive reshaping approaches resect smaller osseous fragments, the risk of malossification with large calvarial defects is reduced when compared to the abovementioned procedures such as our total vertex craniectomy. Excellent examples of such procedures are the H and Pi-Procedure as well as TCVR according to the Melbourne technique (Simpson et al., 2017). The Pi-Procedure (“squeeze procedure” according to Jane and Persing, 1986) consited of a pi (p)shaped resection of the cranial vault the resected coronal sutures represent the horizontal line and the flanks of the p-sign are represented by the occipitally extending osteotomy lines lateral to the sagittal suture €ller, 2003; Simpson et al., 2017). This method allows easy (Zo reshaping of the cranial vault by “squeezing” in an anteriorposterior direction, inducing a shortening of the anteriorposterior dimension of the individual's skull. In accordance with the results of Epstein et al., who regularly performed the total vertex craniectomy, we can report excellent results which are generally apparent within only 1 week to 10 days, both clinically and radiographically (Epstein et al., 1982). Additionally, we can report phenomenal long-term results and a low reoperation rate.
5. Conclusion Considering our data, one can reasonably conclude that the surgical technique of total vertex craniectomy is a procedure with a very low rate of clinical complications and a very high rate of excellent aesthetic outcomes. The procedure is suitable for young individuals less than 1 year of age. Consequently, young individuals with sagittal synostosis with scaphocephalic phenotype can be
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surgically treated with the help of the surgical procedure presented herein, a modified median craniectomy. Conflict of interest The authors report no financial disclosure and no conflicts of interest. Acknowledgements Figs. 1e6 presented are property of Univ.-Prof. Dr. Dr. Joachim € ller. Zo References Abraham P, Brandel MG, Dalle CO, Reid CM, Kpaduwa CS, Lance S, et al: Predictors of postoperative complications of craniosynostosis repair in the national inpatient sample. Ann Plast Surg, 2018 Al-Shaqsi S, Zellner E, Ching J, Forrest C, Phillips J: The natural history of cranial morphology in sagittal craniosynostosis. J Craniofac Surg, 2018 Barritt J, Brooksbank M, Simpson D: Scaphocephaly: aesthetic and psychosocial considerations. Dev Med Child Neurol 23(2): 183e191, 1981 € rensen N, Krauß J, Reinhart E, Pistner H: Kraniosynostosen-PrinziCollmann H, So pien und Risiken der chirurgischen Behandlung. Br J Oral Maxillofac Surg 34(5): 477, 1996 Da Costa AC, Anderson VA, Savarirayan R, Wrennall JA, Chong DK, Holmes AD, et al: Neurodevelopmental functioning of infants with untreated singlesuture craniosynostosis during early infancy. Childs Nerv Syst 28(6): 869e877, 2012 Engel M, Thiele OC, Mühling J, Hoffmann J, Freier K, Castrillon-Oberndorfer G, et al: Trigonocephaly: results after surgical correction of nonsyndromatic isolated metopic suture synostosis in 54 cases. J Craniomaxillofac Surg 40(4): 347e353, 2012 Epstein N, Epstein F, Newman G: Total vertex craniectomy for the treatment of scaphocephaly. Childs Brain 9(5): 309e316, 1982 Foltz EL, Loeser JD: Craniosynostosis. J Neurosurg 43(1): 48e57, 1975 Greene Jr CS, Winston KR: Treatment of scaphocephaly with sagittal craniectomy and biparietal morcellation. Neurosurgery 23(2): 196e202, 1988 Hobar PC, Schreiber JS, McCarthy JG, Thomas PA: The role of the dura in cranial bone regeneration in the immature animal. Plast Reconstr Surg 92(3): 405e410, 1993 Kolar JC: An epidemiological study of nonsyndromal craniosynostoses. J Craniofac Surg 22(1): 47e49, 2011 Lajeunie E, Le Merrer M, Bonaiti-Pellie C, Marchac D, Renier D: Genetic study of scaphocephaly. Am J Med Genet 62(3): 282e285, 1996 Mabbutt LW, Kokich VG: Calvarial and sutural re-development following craniectomy in the neonatal rabbit. J Anat 129(Pt 2): 413e422, 1979 Marsh JL, Vannier MW: Cranial base changes following surgical treatment of craniosynostosis. Cleft Palate J 23(Suppl. 1): 9e18, 1986 Mühling J: Surgical treatment of premature craniosynostosis; 1986 Persing JA: MOC-PS(SM) CME article: management considerations in the treatment of craniosynostosis. Plast Reconstr Surg 121(4 Suppl): 1e11, 2008 €ller J: Clinical evaluation Safi A-F, Kreppel M, Grandoch A, Kauke M, Nickenig H-J, Zo of standardized fronto-orbital advancement for correction of isolated trigonocephaly. J Craniofac Surg 29(1): 72e75, 2018a €ller J: Rigid external Safi A-F, Kreppel M, Kauke M, Grandoch A, Nickenig H-J, Zo distractor-aided advancement after simultaneously performed lefort-iii osteotomy and fronto-orbital advancement. J Craniofac Surg 29(1): 170e174, 2018b Simpson A, Wong AL, Bezuhly M: Surgical correction of nonsyndromic sagittal craniosynostosis: concepts and controversies. Ann Plast Surg 78(1): 103e110, 2017 Stein SC, Schut L: Management of scaphocephaly. Surg Neurol 7(3): 153e155, 1977 SJ, Maal TJ, et al: Validation of van Lindert EJ, Siepel FJ, Delye H, Ettema AM, Berge cephalic index measurements in scaphocephaly. Childs Nerv Syst 29(6): 1007e1014, 2013 Virchow R. Ueber den Cretinismus, Namentlich in Franken, und uber patholovische Schadedformen. (Quoted by numerous authors.) Verh Phus-Med Ges Warzburg, vol. 2; 1851, a30, 1851 Whitaker LA, Bartlett SP, Schut L, Bruce D: Craniosynostosis: an analysis of the timing, treatment, and complications in 164 consecutive patients. Plast Reconstr Surg 80(2): 195e206, 1987 Wilkie AO, Johnson D, Wall SA: Clinical genetics of craniosynostosis. Curr Opin Pediatr 29(6): 622e628, 2017 €ller J, Mühling J: Kraniofaziale Chirurgie. Mund- Kiefer-und Gesichtschirurgie: Zo 417e438, 2012 €ller JE: Kraniofaziale Chirurgie: Diagnostik und Therapie kraniofazialer FehlbilZo dungen; 16 Tabellen, Georg Thieme Verlag; 2003
Please cite this article in press as: Kreppel M, et al., Clinical evaluation of non-syndromic scaphocephaly surgically corrected with the procedure of total vertex craniectomy, Journal of Cranio-Maxillo-Facial Surgery (2018), https://doi.org/10.1016/j.jcms.2018.05.057