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Trigonocephaly: Results after surgical correction of nonsyndromatic isolated metopic suture synostosis in 54 cases
Journal of Cranio-Maxillo-Facial Surgery 40 (2012) 347e353
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Trigonocephaly: Results after surgical correction of nonsyndromatic isolated metopic suture synostosis in 54 cases Michael Engel*, Oliver C. Thiele, Joachim Mühling, Jürgen Hoffmann, Kolja Freier, Gregor Castrillon-Oberndorfer, Robin Seeberger Department of Maxillofacial Surgery, University Hospital Heidelberg, Germany
a r t i c l e i n f o
a b s t r a c t
Article history: Paper received 18 October 2010 Accepted 25 May 2011
Children with nonsyndromatic isolated metopic suture synostosis suffer from a significant deformity of the supraorbital ridges, the temporal regions and hypotelorism. We retrospectively analyzed 54 consecutive cases of isolated nonsyndromatic metopic synostosis treated over a 14-year-period. The data were evaluated using patients’ clinical records, skull radiographs in two planes, CT-scans, MRI scans and pre-/post-operative photographs. Surgery with standardized fronto-orbital advancement was performed at a median age of 11.5 months. Follow-up ranged from 4.5 months to 177.4 months, with an average of 51.9 months. The average blood loss was less than 255 ml and the average post-operative length of stay was 5 days. Not a single major complication was observed except for uncomplicated dural tears in six cases. According to the classification of Whitaker, results were considered good to excellent (Category I and II) in all except one case (Category IV). As the current techniques have been standardized for routine use, surgical risks are reasonably low with no mortality or permanent morbidity. We think that the treatment of single metopic synostosis is safe with very low reoperation rates and short length of hospital stay. Overall, our results showed acceptable minor complication rates and generally satisfactory aesthetic outcomes. Ó 2011 European Association for Cranio-Maxillo-Facial Surgery.
Keywords: Metopic synostosis Craniosynostosis Trigonocephaly Craniofacial surgery
1. Introduction Isolated metopic suture synostosis occurs in 1 in 2,500e1 in 70,000 live births and accounts for less than 15% of all cases referred to craniofacial centres (Sadove et al., 1990; Genitori et al., 1991; Collmann et al., 1996; Bottero et al., 1998). The incidence of metopic synostosis has increased and has accounted for up to 30% in some series (Keshavarzi et al., 2009). The illustrative term trigonocephaly was coined by Welcker in 1862 (Welcker, 1862). The author realized that the deformity could occur either as an isolated anomaly or as part of a syndrome combined with malformations outside the skull. It is now estimated that 10e20% of trigonocephalic patients are affected by syndromes such as Opitz C syndrome, SayeMeyer syndrome or Frydman syndrome (Cohen, 1986; Schaap et al., 1992), although most metopic synostosis are nonsyndromic (Keshavarzi et al., 2009). There is a male predominance of 65e85% (Delhemmes et al., 1986; Genitori et al., 1991; * Corresponding author. Department of Maxillofacial Surgery, University Hospital Heidelberg, Im Neuenheimer Feld 400, 69120 Heidelberg, Germany. Tel.: þ49 6221 5638811; fax: þ49 6221 564222. E-mail address: [email protected] (M. Engel).
Aryan et al., 2005). Some data indicate that the fusion of the metopic suture might be secondary to the influence of regional dura growth factor mediated signals (Bradley et al., 1997). Despite this advance, the aetiology of isolated trigonocephaly is unknown, although 2e5% of cases are familial. It is believed that it may be inherited as an autosomal dominant trait with a very low penetrance (Furuya et al., 1984; Collmann et al., 1996). The most severe deformities have been reported in Japan so that some ethnic factors may also be involved (Collmann et al., 1996). Case reports of nonsyndromic metopic craniosynostosis as an autosomal dominant inherited disorder and as a partial monosomy of chromosome 11q have been published, but this does not account for most affected patients (Lewanda et al., 1995). The clinical hallmarks of metopic synostosis are trigonocephaly, with the keel-shaped forehead, hypotelorism and epicanthus, and temporal narrowing with an associated abnormality of the supraorbital rim. The premature closure of the metopic suture ranges from mild with slight prominence of the metopic ridge to severe with gross distortion of the forehead, supraorbital bar and orbits (Collmann et al., 1996; Shimoji et al., 2002; Aryan et al., 2005; Fearon et al., 2007). At the age of 2e3 years the metopic suture is normally patent and allows growth of the frontal bones. In 10% of adults, complete fusion of the metopic
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suture never occurs (Furuya et al., 1984; Genitori et al., 1991). Premature fusion of the metopic suture results in restriction of the normal growth of the frontal bones. The fusion is believed to occur during intrauterine development (Azimi et al., 2003; Aryan et al., 2005). Radiological findings in plain radiographs are ossification of metopic suture and anterior fontanel, hypotelorism and a broadening of the occipital region. CT demonstrates the following findings: orbital hypotelorism, possible ethmoidal hypoplasia, medial orbital rim protrusion, retruded lateral orbital rims and narrowed bitemporal width (Kotrikova et al., 2007). MRI shows the soft-tissue alterations, such as triangular brain deformation in the front and can be used to detect increased intracranial pressure. The clinical significance of trigonocephaly is still a matter of discussion. It has been suggested that normal brain growth may be restricted within the small anterior fossa, thereby causing mental retardation (Collmann et al., 1996). There is an increasing body of evidence to suggest that children with nonsyndromic trigonocephaly may have a higher risk of cerebral impairment (Becker et al., 1997; Shimoji et al., 2002; Speltz et al., 2004). It is estimated that isolated craniosynostosis is associated with a three- to five-fold increase in risk for cognitive deficits. Although the causal relation between this condition and neurodevelopment is uncertain, it would appear that these types of calvarial abnormalities are at the very least a visible and easily diagnosable marker for an increased risk of neurodevelopmental problems (Speltz et al., 2004). The intracranial pressure has been shown to be elevated in 8e17% of patients (Campbell et al., 1995; Cohen and Persing, 1998; Renier et al., 1987). The pressure does not reach high levels and gross papilloedema or even optic nerve atrophy has rarely been reported, from which it can be inferred that intracranial hypertension does not play a major part in trigonocephaly (Tuite et al., 1996). Surgery is performed predominantly on the basis of aesthetic, and thus psychosocial considerations (Collmann et al., 1996). 2. Material Fifty-four patients underwent a surgical procedure for isolated nonsyndromatic metopic craniosynostosis at the Department of cranio-maxillo-facial surgery of the University of Heidelberg between 1994 and 2008. Medical records of all patients were reviewed in their entirety. The following data were retrospectively collected, including name, date of birth, sex, age at point of operative correction, other medical diagnoses, dates, and operative records of all surgical procedures performed, hospital course, complications, average volume of transfused blood, pre- and postoperative haemoglobin, dates of follow-up visits, findings at followup and the most recent assessment of outcome. Any other relevant data were also noted. The pre-operative evaluation of all patients included a careful clinical and ophthalmological examination. Twenty of our patients had plain radiographs, six a CT scan and 23 an MRI prior to surgery. The operative exposure was the same in all cases. These procedures were undertaken in association with the neurosurgery team at our institution. Under general endotracheal anaesthesia, a large-bore intravenous line was inserted, and the patient was given dexamethasone, mannitol, and cefazolin. Blood was transfused in all cases. The objectives of the surgical techniques are to open the abnormally closed suture, increase the intracranial volume, correct the position of the bone segments and induce cranial growth in the desired direction. Basied Tessier’s groundbreaking “tongue in groove technique” and Marchac’s “early bilateral development” we use the standardized osteotomy developed especially to be applied to trigonocephaly (Fig. 1) (Tessier, 1967; Mühling et al., 1991; Mühling, 1995). The operation consists of removing, shaping and repositioning the fronto-orbital skull segments. The osteotomy lines are placed along the cranial sutures.
Fig. 1. Standardized osteotomy especially applied to trigonocephalus.
Table 1 Whitaker classification of surgical results. Category I Category II
Category III
Category IV
No refinements or surgical revisions considered advisable or necessary Soft-tissue or lesser bone-contouring revisions advisable apt to be performed on an outpatient basis or requiring a maximum of 2-day hospitalization Major alternative osteotomies or bonegrafting procedure advisable, i.e., orbital repositions, onlay bone grafts, being these procedures not so extensive as the original operations A major craniofacial procedure advisable, duplicating or exceeding the original operation
The inlay incision in the temporal area has a retention function. The osteotomy is carried out in two steps. First the frontal bone segment is removed, and then the orbital segment can be cut through after the dura is detached. After this, the orbital segment is bent up in the midline. The lateral region of the orbits is formed into a 90 curve onto the skull. In the area of the metopic suture the frontal bone segment is sectioned. After reinserting of the osseus fragments with a slight advancement and fixed with titanium plates (removed 6 months after surgery in a second operation) or resorbable mini- or microplates, the metopic suture is automatically opened. The detached temporalis muscle was carefully reinserted at its original site to avoid temporal depression. We employed the classification of Whitaker and associates to evaluate the surgical results (see Table 1), (Whitaker et al., 1987). 3. Results Fifty-four patients underwent surgical correction of metopic synostosis at the Department of cranio-maxillo-facial surgery of the University of Heidelberg over a 14-year period from 1994 to 2008. The median age at referral was 5 months. There were 40 boys and 14 girls. Follow-up ranged from 4.5 months to 177.4 months, with an average follow-up of 51.9 months. The surgery was performed between the ages of 6 and 52 months. The median age at surgery was 11.5 months. A family history of craniosynostosis was noted in two of the children. Seven of the 54 patients were found to have developmental delay. In two cases the ophthalmological examination showed mild papilloedema pre-operatively without any other clinical signs of elevated intracranial pressure as well as an unremarkable result in the CT scan or MRI. In the pre-operative evaluation, plain radiographs and the CT-scans confirmed the clinical diagnosis and ensured that no other suture was affected. The MRI in 23 cases showed no signs of elevated intracranial pressure or any
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other pathological result. All 54 patients had significant deformity of the supraorbital ridges and temporal regions, with hypotelorism (Fig. 2a, b). All these children underwent craniofacial reconstruction, according to the standardized fronto-orbital advancement especially applied to trigonocephaly (Fig. 3aed). The average weight of the child at time of surgery was 11.5 kg (5e17.5 kg). The median average amount of blood loss during the operation was less than 255 ml, ranging from 80 ml to 600 ml. Blood was transfused in all cases. The average pre-operative haemoglobin was 11.9 (9.2e15.2), and post-operatively 9.6 (6.2e11.6). The average hospital stay after operation was 5 days, with a planned ICU stay of only 1 day for all patients. In all cases in our series, no major complication was seen except for uncomplicated dural tears in six cases. According to the classification of Whitaker, 43 patients had a Class 1 outcome, with excellent cosmesis (Fig. 4a, b). Ten patients were Class 2, with a prominent wire, plate, or bony spicule of no aesthetic consequence. No further therapy was undertaken in this group. None of our patients were Class 3 and only one patient was Class 4 requiring a repeat operative procedure (see Table 2). The Class 4 patient had a recurrence of the bony ridge at the metopic suture. This patient underwent a second operation (fronto-orbital remodelling) with very good cosmesis at 9-year follow-up. A slight bilateral temporal depression was seen in majority of our cases.
Fig. 2. a, b: Typical craniofacial appearance of a child with metopic craniosynostosis. Photos taken just prior to surgery.
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4. Discussion Trigonocephaly affects the normal anatomic architecture of the fronto-orbital region. The metopic synostosis is characterized by premature closure of the metopic suture. This early closure results in a wide spectrum of deformity. Mild cases are marked by ridging of the metopic suture with minimal associated facial and cranial abnormalities. More severe cases are marked by varying degrees of trigonocephaly, hypotelorism and abnormalities of the orbital rim. The goal of treatment is directed towards achieving an excellent cosmetic result. The timing of surgery remains an issue. Age at first operation in Cohen’s series of 23 patients ranged from 2 months to 56 months (mean 8.2 months), with 15 patients being operated before 6 months and 8 after 7 months. The surgery in our series was performed between the ages of 6 and 52 months. The median age at operation in this series was 11.5 months. This is slightly older than reported in other series except in the studies of Kelleher et al. with a median age of 12 months and Keshavarzi with a median age of 16.6 months (Cohen and Persing, 1998; Cohen et al., 1994; Kelleher et al., 2007; Keshavarzi et al., 2009). Many surgeons favour operative intervention during the first 3e9 months of life (Delhemmes et al., 1986; Genitori et al., 1991; Aryan et al., 2005). Hilling et al’s patients were operated at a mean age of 13 months (range 5e51 months), with 80% undergoing surgery between the age of 6 and 15 months. Using the Spearman rank correlation, their data suggest that there is no significant correlation between the child’s appearance and the age in months at which the operation was performed within the given range of age at surgery (Hilling et al., 2006). Collmann reported a male predominance of 65e85%. In line with Collmann and other authors, our patients showed a male predominance of 80% (Genitori et al., 1991; Collmann et al., 1996). The average hospital stay in Aryans series was 3.6 days, with an ICU stay required for only six patients (Aryan et al., 2005). In Keshavarzi et al’s series, average stay was 2.76 days (Keshavarzi et al., 2009). In our series, the average hospital stay after operation was 5 days, with a planned ICU stay for all patients required for only 1 day. In our experience, maximal post-operative swelling took place on the second day. We preferred to observe patients until there was a clinical seduction of swelling before leaving the hospital. Kelleher et al. reported an average blood loss of 200 ml, with surgery being performed at 12 month of age on average (Kelleher et al., 2007). Aryan et al. reported an average blood loss of 400 ml, with the surgery being performed at 7.5 months of age on average (Aryan et al., 2005). Estimated mean blood loss in a study of Keshavarzi was 190 ml in a group of 17 patients (Keshavarzi et al., 2009). The average (mean) transfusion volume for patients treated with fronto-orbital advancement was 184 ml, with 5 (29%) patients requiring no blood transfusion. The surgery was performed at a median age of 16.6 months. The transfused blood for each of our patients was given to replace the amount of the estimated blood loss during the operation. Although the average amount of blood loss in our series was less than 255 ml, this still represents a considerable percentage of the baby’s blood volume and clearly has to be taken into account when considering timing. In 50 of our cases, titanium plates were used for fixation. We removed the material after 6 months. In the last four treated cases of our series, we changed our approach and used resorbable plates for fixation because there were some disadvantages using titanium plates. The plates either had to be removed at a second operation or there was growth-induced intracranial migration of the metal osteosynthesis plates and wires and there were potential complications related to this (Kosaka et al. 2003; Stelnicki and Hoffman, 1998). The development and bone apposition/resorptive pattern of skull growth create the potential for growth restriction and eventual metal device translocation to the endocranial surface may be seen (Fearon
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Fig. 3. a, b, c, d: Intra-operative photograph (a, b) showing metopic abnormality of the supraorbital rim and the brain, result after fronto-orbital remodelling (c). Post-operative result (d).
Fig. 4. a, b: Pre- and Post-operative photograph of the child seen in Figs. 2 and 3. Post-operative picture was taken 6 months after surgery.
M. Engel et al. / Journal of Cranio-Maxillo-Facial Surgery 40 (2012) 347e353 Table 2 Results according to Whitaker classification (total number of patients ¼ 54). Category
Number of patients
I II III IV
43 10 0 1
et al. 1995; Orringer et al. 1998). Currently we use resorbable osteosynthesis materials for all craniosynostosis procedures in infancy to avoid the known disadvantages (Becker et al. 1999; Imola et al. 2001; Obwegeser, 1998). Pre-operative imaging can be helpful to confirm the clinical diagnosis of trigonocephaly. Computed tomographic scans permit excellent visualization of the underlying bony architecture, helping surgeons appreciate bony anomalies and plan surgical correction. Plain radiographs ensure that the metopic suture is closed and no other suture is affected. MRI shows soft-tissue alterations, such as triangular brain deformation anteriorly and can be used to detect clinical evidence of increased intracranial pressure (Kotrikova et al., 2007). We believe that physical examination is a very sensitive tool in the diagnosis of the metopic sutural craniosynostoses. In our clinical experience, we are in agreement with Fearon et al., that craniofacial surgeons with various levels of experience were able to accurately diagnose single-sutural synostosis in most cases by physical examination alone. Considering the potential side effects from ionizing radiation, risks of sedation, and costs, surgeons may wish to reserve computed tomographic scans only for infants with suspected metopic suture craniosynostosis, in whom the physical examination is not clearly diagnostic (Fearon et al., 2007). Metopic synostosis affects not only the normal anatomic architecture of the fronto-orbital region, but it also results, as some authors have described, in concomitant intracranial abnormalities, such as cognitive deficits, and in elevated intracranial pressure. Some authors believe that the intracranial pathology is due to the congenital anomaly and not secondary to constriction caused by the synostosis. Treatment is directed towards achieving an excellent aesthetic result rather than addressing the intracranial pathology (Aryan et al., 2005). Seven of our 54 patients were found to have a mild developmental delay. In the series of Aryan, six of the 39 patients (15%) were found to have developmental delay (Aryan et al., 2005). Eight of Collmann et al.’s 73 patients had mental delay (Collmann et al., 1996). Anderson and Geiger’s series had an 18% rate of mental retardation. Oi and Matsumoto reported about five with cognitive deficits in a series of 13 patients (Oi and Matsumoto, 1987). They felt that the degree of angulation of the frontal bone correlated with the degree of disability. While intracranial pressure can be elevated in some patients, it is rarely elevated highly, and gross papilloedema or optic nerve changes have only been reported rarely (Tuite et al., 1996). We use noninvasive ophthalmological examination to eliminate papilloedema as a sign of high ICP. The presence of papilloedema with elevated ICP should be noted and CT-scans or MRI should be obtained (Cohen and Persing, 1998). The MRI or CT-scans of our patients did not reveal any signs of intracranial pressure. In two of all our cases, the ophthalmological examination showed a mild papilloedema preoperatively without any other clinical signs of elevated intracranial pressure as well as an unremarkable CT scan and MRI. The papilloedema of these patients resolved during the follow-up examinations after surgery. In our opinion, fundoscopic examination as a noninvasive technique to estimate ICP should be performed in all cases. The diagnosis of elevated ICP for children with single-suture craniosynostosis must be made from a constellation of clinical
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findings and can be supported by the measurement of ICP in selected cases (Thompson et al., 1995a, b). Other noninvasive techniques were reported by other authors. Rifkinson-Mann et al. recently found increased resistance to CSF absorption for 70% of patients with craniosynostosis when cerebral haemodynamics were analyzed by transcranial Doppler (Rifkinson-Mann et al., 1995). Gault et al. attempted to correlate ICP with the reduction in intracranial volumes as measured by CT-scans. Volume measurements alone did not serve as a reliable predictor of elevated ICP (Gault et al., 1992). Utilizing invasive techniques Thompson et al. reported their results with overnight, subdural ICP monitoring of 74 children with premature closure of a single cranial suture. A single coronal suture was involved in 37 patients, a sagittal suture was involved in 25 patients, and a metopic suture was involved in 12 patients. The ages of the patients ranged from 2 months to 10 years, with 35 children being under 1 year of age at the time of monitoring. ICP was raised in 13 (17%), borderline in 28 (38%), and normal in 33 (45%) patients. Thirty-three percent with metopic synostosis had borderline or elevated ICP (Thompson et al., 1995a, b). Marchac reported about 8% of elevated intracranial pressure for craniosynostosis (Marchac and Renier, 1986). We are in agreement with other authors, that the prevalence of ICP may be higher than previously reported and chronic and gradual increases in ICP may occur in the absence of papilloedema or an unremarkable CT or MRI (Rifkinson-Mann et al., 1995). We avoid invasive techniques to estimate ICP. We prefer fundoscopy as a noninvasive technique to estimate ICP. If there is any sign of papilloedema, we will use MRI or CT scan to clarify ICP. In children less than 1-yearold with isolated synostosis of the metopic suture, whose parents already wish to proceed to normalize skull shape, ICP monitoring is unlikely to contribute to clinical management. For children without signs and symptoms of elevated ICP, whose fundoscopy and CT or MRI do not suggest elevated ICP and whose parents do not elect for surgery, close follow-up by a paediatric neurosurgeon, a craniofacial surgeon, a neurodevelopmental specialist and a paediatric ophthalmologist for fundoscopic examination is recommended. Improvement in behaviour has been observed with resolution of papilloedema (Cohen et al., 1993; Campbell et al., 1995). In all cases in our series, no major complications or deaths were seen, similar to other series (Aryan et al., 2005; Fearon et al., 2007; Kelleher et al., 2007; Keshavarzi et al., 2009). Complications in Cohen’s series included minor wound dehiscence (n ¼ 1), seizures (n ¼ 1), and increased intracranial pressure (n ¼ 1) (Cohen et al., 1994). Dural tears common after surgical correction of craniosynostosis. The dural tears in six of our 54 cases were not thought to be major complications, and were closed immediately. No CSF fistula was seen in follow-up. Dural tears ranged from 5% to 60% in other series (Renier et al., 1985; Mühling, 1995; Esparza et al., 2008). To avoid CSF fistulas with their known complications and treatment, dural tears need be repaired immediately intra-operative (Mühling, 1995). Our patients presented with varying degrees of diffuse deformities of the frontal bones, with extension around the orbits. In our 54 cases the craniofacial reconstruction was performed to achieve an overall correction of the frontal bones as well as the periorbital region. Results in our series were satisfactory in 53 cases (Class I and II outcome) except one patient with a recurrence of the bony ridge at the metopic suture, needing further therapy (Class IV outcome). Our Class 4 patient had a recurrence of the bony ridge at the metopic suture. This patient underwent a second operation for burring of the metopic ridge, with a very good cosmetic result at 9year follow-up. Aryan et al. reported similar results. 31 patients in a series of 39 cases with metopic synostosis showed Class I and 5 with Class II outcome with three patients who needed further treatment (Aryan et al., 2005). In the study by Kerwin et al. of 39
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patients, only one required repeat surgery (Kerwin et al., 1997) Esparza et al. used frontal remodelling without fronto-orbital “bandeau” in 50 cases of trigonocephaly, which was performed at a mean age of 6.65 months. According to Whitaker’s classification, the results of Esparaza in 42 patients were classified into Category I, 7 into Category II and 1 into Category III (10.6%) (Esparza et al., 2008). Sloan et al. reported excellent to good surgical results in a group of 20 patients with metopic synostosis. They also had no major complications, performed no planned reoperations, and only one patient (5%) required unplanned reoperation (Sloan et al., 1997) Total reoperation (reoperative fronto-orbital remodelling and calvarial vault reshaping) was necessary in two patients (9%) of Cohen’s Collective (Cohen et al., 1994). Bitemporal hollowing is common after surgical correction of metopic synostosis (Persing et al., 1994; Oh et al., 2006; van der Meulen et al., 2009). Hollowing is usually located just lateral and slightly cranial to the lateral apex of the eyebrow. Theories on its aetiology focus either on bone or on soft-tissue. Aryan reported that the temporalis muscle should be carefully reinserted at its original site or even be transferred anteriorly in order to improve the postoperative temporal depression (Aryan et al., 2005). In contrast to Aryan, van der Meulen describes that the iatrogenic damage to the temporal bone for instance could result from the two osteotomies that are performed in that area in a parallel horizontal plane to mobilize the supraorbital bandeau. For him, temporal hollowing seems to be of bony origin and can be explained by skeletal growth inhibition in the affected area (van der Meulen et al., 2009). The surgical technique seems to be relevant in the origin of bitemporal hollowing. The choice of operative technique could result in an underestimation of the lateral expansion needed to correct the initial dysmorphology (van der Meulen et al., 2008; van der Meulen et al., 2009). The posterior edge of the forehead in the temporal region could end up not extending sufficiently backwards to support or fill the temporal area, leaving a temporal depression (Persing et al., 1994; Oh et al., 2006; van der Meulen et al., 2009). So, the posterior edge in the temporal area should be extended to enlarge the bitemporal deficiency. In our experience, a slight bilateral temporal depression was seen in majority of our cases although the temporalis muscles were carefully reinserted. In future cases of treatment for trigonocephaly, our interest will focus on the surgical technique with an adequate lateral expansion enlarging the bitemporal diameter to improve the phenomenon of post-operative temporal hollowing. We believe that temporal hollowing is based on a combination of surgical technique, bony origin with skeletal growth inhibition and softtissue.
5. Conclusion As the current techniques have been standardized for routine use, surgical risks are reasonably low with no mortality or permanent morbidity. In agreement with other authors, we think that the treatment of single metopic synostosis is safe with very low reoperation rates and short hospital stay. Overall, our results for fronto-orbital advancement in cases of isolated metopic synostosis showed acceptable minor complication rates and generally satisfactory aesthetic outcomes. Conflict of interest All authors disclose any financial and personal relationships with other people or organisations that could inappropriately influence or bias this work.
This study was not sponsored by any other person or organisation that was not affiliated to the Department of Oral and Maxillofacial Surgery.
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M. Engel et al. / Journal of Cranio-Maxillo-Facial Surgery 40 (2012) 347e353 Mühling J: Kraniofaziale Chirurgie. In: Hausamen JE, Machtens E, Reuther J (eds), Kirschnersche Operationslehre. Berlin Heidelberg New York: Springer, 403e427, 1995 Obwegeser JA: Absorbable and bioconvertible osteosynthesis materials in maxillofacial surgery. Mund Kiefer Gesichtschir 2: 288e308, 1998 Oh AK, Greene AK, Mulliken JB: Prevention of temporal depression that follows fronto-orbital advancement for craniosynostosis. J Craniofac Surg 17: 980e998, 2006 Oi S, Matsumoto S: Trigonocephaly (metopic synostosis). Child’s Nerv Syst 3: 259e265, 1987 Orringer JS, Barcelona V, Buchman SR: Reasons for removal of rigid internal fixation devices in craniofacial surgery. J Craniofac Surg 9: 40, 1998 Persing JA, Mayer PL, Spinelli HM: Prevention of bitemporal hollowing after frontoorbital advancement for craniosynostosis. J Craniofac Surg 5: 271e274, 1994 Renier D, Sainte-Rose C, Marchac D: Intracranial pressure in craniosynostosis. In: Marchac D (ed.), Craniofacial surgery. (Proceedings of the First International Congress of the International Society for Cranio-Maxillo-Facial Surgery, CannesLa Napoule, 1985). Berlin Heidelberg New York: Springer, 110e113, 1987 Rifkinson-Mann S, Goraj B, Leslie D, Visintainer PF: Padua hemangioma. Transcranial Doppler analysis of cerebral hemodynamics in primary craniosynostosis: study in progress. Surg Neurol 44: 333e337, 1995 Sadove AM, Kalsheck JE, Eppley BL, Javed I: Modifications in the surgical correction of trigonocephaly. Plast Reconstr Surg 85: 853e858, 1990 Schaap C, Schrander-Stumpel CT, Fryns JP: Opitz-C syndrome: on the nosology of mental retardation and trigonocephaly. Genet Couns 3: 209e215, 1992 Shimoji T, Shimabukuro S, Sugama S, Ochiai Y: Mild trigonocephaly with clinical symptoms: analysis of surgical results in 65 patients. Child’s Nerv Syst 18: 215e224, 2002
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Sloan GM, Wells KC, Raffel C, McComb JG: Surgical treatment of craniosynostosis: outcome analysis of 250 consecutive patients. Pediatrics 100(1): E2, 1997 Speltz ML, Kapp-Simon KA, Cunningham M, Marsh J, Dawson G: Single-suture craniosynostosis: a review of neurobehavioral research and theory. J Pediatr Psychol 29: 651e668, 2004 Stelnicki EJ, Hoffman W: Intracranial migration of microplates versus wires in neonatal pigs after frontal advancement. J Craniofac Surg 9: 60e64, 1998 Tessier P: Osteotomies totales de la face: syndrome de Crouzon, syndrome d’Apert; oxycéphalies, scaphocéphalies, turricéphalies. Ann Chir Plast 12: 273e279, 1967 Thompson DNP, Harkness W, Jones B, Gonsalez S, Andar U, Hayward R: Subdural intracranial pressure monitoring in craniosynostosis: its role in surgical management. Child’s Nerv Syst 11: 269e275, 1995a Thompson DNP, Malcolm GP, Jones BM, Harkness WJ, Hayward RD: Intracranial pressure in single-suture craniosynostosis. Pediatr Neurosurg 22: 235e240, 1995b Tuite GF, Chong WK, Evanson J, Narita A, Taylor D, Harkness WF, et al: The effectiveness of papilledema as an indicator of raised intracranial pressure in children with craniosynostosis. Neurosurgery 38: 272e280, 1996 van der Meulen J, Nazir P, Mathijssen I, et al: Bitemporal depressions after cranioplasty for trigonocephaly: a long-term evaluation of supraorbital growth in 92 patients. J Craniofac Surg 19: 72e79, 2008 van der Meulen JJ, Willemsen J, van der Vlugt J, Nazir PR, Hilling D, Mathijssen IM, et al: On the origin of bitemporal hollowing. J Craniofac Surg 20: 752e756, 2009 Welcker H: Untersuchungen über Wachstum und Bau des menschlichen Schädels. Leipzig: Engelmann, 1862 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: 195e212, 1987
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Trigonocephaly: Results after surgical correction of nonsyndromatic isolated metopic suture synostosis in 54 cases Michael Engel*, Oliver C. Thiele, Joachim Mühling, Jürgen Hoffmann, Kolja Freier, Gregor Castrillon-Oberndorfer, Robin Seeberger Department of Maxillofacial Surgery, University Hospital Heidelberg, Germany
a r t i c l e i n f o
a b s t r a c t
Article history: Paper received 18 October 2010 Accepted 25 May 2011
Children with nonsyndromatic isolated metopic suture synostosis suffer from a significant deformity of the supraorbital ridges, the temporal regions and hypotelorism. We retrospectively analyzed 54 consecutive cases of isolated nonsyndromatic metopic synostosis treated over a 14-year-period. The data were evaluated using patients’ clinical records, skull radiographs in two planes, CT-scans, MRI scans and pre-/post-operative photographs. Surgery with standardized fronto-orbital advancement was performed at a median age of 11.5 months. Follow-up ranged from 4.5 months to 177.4 months, with an average of 51.9 months. The average blood loss was less than 255 ml and the average post-operative length of stay was 5 days. Not a single major complication was observed except for uncomplicated dural tears in six cases. According to the classification of Whitaker, results were considered good to excellent (Category I and II) in all except one case (Category IV). As the current techniques have been standardized for routine use, surgical risks are reasonably low with no mortality or permanent morbidity. We think that the treatment of single metopic synostosis is safe with very low reoperation rates and short length of hospital stay. Overall, our results showed acceptable minor complication rates and generally satisfactory aesthetic outcomes. Ó 2011 European Association for Cranio-Maxillo-Facial Surgery.
Keywords: Metopic synostosis Craniosynostosis Trigonocephaly Craniofacial surgery
1. Introduction Isolated metopic suture synostosis occurs in 1 in 2,500e1 in 70,000 live births and accounts for less than 15% of all cases referred to craniofacial centres (Sadove et al., 1990; Genitori et al., 1991; Collmann et al., 1996; Bottero et al., 1998). The incidence of metopic synostosis has increased and has accounted for up to 30% in some series (Keshavarzi et al., 2009). The illustrative term trigonocephaly was coined by Welcker in 1862 (Welcker, 1862). The author realized that the deformity could occur either as an isolated anomaly or as part of a syndrome combined with malformations outside the skull. It is now estimated that 10e20% of trigonocephalic patients are affected by syndromes such as Opitz C syndrome, SayeMeyer syndrome or Frydman syndrome (Cohen, 1986; Schaap et al., 1992), although most metopic synostosis are nonsyndromic (Keshavarzi et al., 2009). There is a male predominance of 65e85% (Delhemmes et al., 1986; Genitori et al., 1991; * Corresponding author. Department of Maxillofacial Surgery, University Hospital Heidelberg, Im Neuenheimer Feld 400, 69120 Heidelberg, Germany. Tel.: þ49 6221 5638811; fax: þ49 6221 564222. E-mail address: [email protected] (M. Engel).
Aryan et al., 2005). Some data indicate that the fusion of the metopic suture might be secondary to the influence of regional dura growth factor mediated signals (Bradley et al., 1997). Despite this advance, the aetiology of isolated trigonocephaly is unknown, although 2e5% of cases are familial. It is believed that it may be inherited as an autosomal dominant trait with a very low penetrance (Furuya et al., 1984; Collmann et al., 1996). The most severe deformities have been reported in Japan so that some ethnic factors may also be involved (Collmann et al., 1996). Case reports of nonsyndromic metopic craniosynostosis as an autosomal dominant inherited disorder and as a partial monosomy of chromosome 11q have been published, but this does not account for most affected patients (Lewanda et al., 1995). The clinical hallmarks of metopic synostosis are trigonocephaly, with the keel-shaped forehead, hypotelorism and epicanthus, and temporal narrowing with an associated abnormality of the supraorbital rim. The premature closure of the metopic suture ranges from mild with slight prominence of the metopic ridge to severe with gross distortion of the forehead, supraorbital bar and orbits (Collmann et al., 1996; Shimoji et al., 2002; Aryan et al., 2005; Fearon et al., 2007). At the age of 2e3 years the metopic suture is normally patent and allows growth of the frontal bones. In 10% of adults, complete fusion of the metopic
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suture never occurs (Furuya et al., 1984; Genitori et al., 1991). Premature fusion of the metopic suture results in restriction of the normal growth of the frontal bones. The fusion is believed to occur during intrauterine development (Azimi et al., 2003; Aryan et al., 2005). Radiological findings in plain radiographs are ossification of metopic suture and anterior fontanel, hypotelorism and a broadening of the occipital region. CT demonstrates the following findings: orbital hypotelorism, possible ethmoidal hypoplasia, medial orbital rim protrusion, retruded lateral orbital rims and narrowed bitemporal width (Kotrikova et al., 2007). MRI shows the soft-tissue alterations, such as triangular brain deformation in the front and can be used to detect increased intracranial pressure. The clinical significance of trigonocephaly is still a matter of discussion. It has been suggested that normal brain growth may be restricted within the small anterior fossa, thereby causing mental retardation (Collmann et al., 1996). There is an increasing body of evidence to suggest that children with nonsyndromic trigonocephaly may have a higher risk of cerebral impairment (Becker et al., 1997; Shimoji et al., 2002; Speltz et al., 2004). It is estimated that isolated craniosynostosis is associated with a three- to five-fold increase in risk for cognitive deficits. Although the causal relation between this condition and neurodevelopment is uncertain, it would appear that these types of calvarial abnormalities are at the very least a visible and easily diagnosable marker for an increased risk of neurodevelopmental problems (Speltz et al., 2004). The intracranial pressure has been shown to be elevated in 8e17% of patients (Campbell et al., 1995; Cohen and Persing, 1998; Renier et al., 1987). The pressure does not reach high levels and gross papilloedema or even optic nerve atrophy has rarely been reported, from which it can be inferred that intracranial hypertension does not play a major part in trigonocephaly (Tuite et al., 1996). Surgery is performed predominantly on the basis of aesthetic, and thus psychosocial considerations (Collmann et al., 1996). 2. Material Fifty-four patients underwent a surgical procedure for isolated nonsyndromatic metopic craniosynostosis at the Department of cranio-maxillo-facial surgery of the University of Heidelberg between 1994 and 2008. Medical records of all patients were reviewed in their entirety. The following data were retrospectively collected, including name, date of birth, sex, age at point of operative correction, other medical diagnoses, dates, and operative records of all surgical procedures performed, hospital course, complications, average volume of transfused blood, pre- and postoperative haemoglobin, dates of follow-up visits, findings at followup and the most recent assessment of outcome. Any other relevant data were also noted. The pre-operative evaluation of all patients included a careful clinical and ophthalmological examination. Twenty of our patients had plain radiographs, six a CT scan and 23 an MRI prior to surgery. The operative exposure was the same in all cases. These procedures were undertaken in association with the neurosurgery team at our institution. Under general endotracheal anaesthesia, a large-bore intravenous line was inserted, and the patient was given dexamethasone, mannitol, and cefazolin. Blood was transfused in all cases. The objectives of the surgical techniques are to open the abnormally closed suture, increase the intracranial volume, correct the position of the bone segments and induce cranial growth in the desired direction. Basied Tessier’s groundbreaking “tongue in groove technique” and Marchac’s “early bilateral development” we use the standardized osteotomy developed especially to be applied to trigonocephaly (Fig. 1) (Tessier, 1967; Mühling et al., 1991; Mühling, 1995). The operation consists of removing, shaping and repositioning the fronto-orbital skull segments. The osteotomy lines are placed along the cranial sutures.
Fig. 1. Standardized osteotomy especially applied to trigonocephalus.
Table 1 Whitaker classification of surgical results. Category I Category II
Category III
Category IV
No refinements or surgical revisions considered advisable or necessary Soft-tissue or lesser bone-contouring revisions advisable apt to be performed on an outpatient basis or requiring a maximum of 2-day hospitalization Major alternative osteotomies or bonegrafting procedure advisable, i.e., orbital repositions, onlay bone grafts, being these procedures not so extensive as the original operations A major craniofacial procedure advisable, duplicating or exceeding the original operation
The inlay incision in the temporal area has a retention function. The osteotomy is carried out in two steps. First the frontal bone segment is removed, and then the orbital segment can be cut through after the dura is detached. After this, the orbital segment is bent up in the midline. The lateral region of the orbits is formed into a 90 curve onto the skull. In the area of the metopic suture the frontal bone segment is sectioned. After reinserting of the osseus fragments with a slight advancement and fixed with titanium plates (removed 6 months after surgery in a second operation) or resorbable mini- or microplates, the metopic suture is automatically opened. The detached temporalis muscle was carefully reinserted at its original site to avoid temporal depression. We employed the classification of Whitaker and associates to evaluate the surgical results (see Table 1), (Whitaker et al., 1987). 3. Results Fifty-four patients underwent surgical correction of metopic synostosis at the Department of cranio-maxillo-facial surgery of the University of Heidelberg over a 14-year period from 1994 to 2008. The median age at referral was 5 months. There were 40 boys and 14 girls. Follow-up ranged from 4.5 months to 177.4 months, with an average follow-up of 51.9 months. The surgery was performed between the ages of 6 and 52 months. The median age at surgery was 11.5 months. A family history of craniosynostosis was noted in two of the children. Seven of the 54 patients were found to have developmental delay. In two cases the ophthalmological examination showed mild papilloedema pre-operatively without any other clinical signs of elevated intracranial pressure as well as an unremarkable result in the CT scan or MRI. In the pre-operative evaluation, plain radiographs and the CT-scans confirmed the clinical diagnosis and ensured that no other suture was affected. The MRI in 23 cases showed no signs of elevated intracranial pressure or any
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other pathological result. All 54 patients had significant deformity of the supraorbital ridges and temporal regions, with hypotelorism (Fig. 2a, b). All these children underwent craniofacial reconstruction, according to the standardized fronto-orbital advancement especially applied to trigonocephaly (Fig. 3aed). The average weight of the child at time of surgery was 11.5 kg (5e17.5 kg). The median average amount of blood loss during the operation was less than 255 ml, ranging from 80 ml to 600 ml. Blood was transfused in all cases. The average pre-operative haemoglobin was 11.9 (9.2e15.2), and post-operatively 9.6 (6.2e11.6). The average hospital stay after operation was 5 days, with a planned ICU stay of only 1 day for all patients. In all cases in our series, no major complication was seen except for uncomplicated dural tears in six cases. According to the classification of Whitaker, 43 patients had a Class 1 outcome, with excellent cosmesis (Fig. 4a, b). Ten patients were Class 2, with a prominent wire, plate, or bony spicule of no aesthetic consequence. No further therapy was undertaken in this group. None of our patients were Class 3 and only one patient was Class 4 requiring a repeat operative procedure (see Table 2). The Class 4 patient had a recurrence of the bony ridge at the metopic suture. This patient underwent a second operation (fronto-orbital remodelling) with very good cosmesis at 9-year follow-up. A slight bilateral temporal depression was seen in majority of our cases.
Fig. 2. a, b: Typical craniofacial appearance of a child with metopic craniosynostosis. Photos taken just prior to surgery.
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4. Discussion Trigonocephaly affects the normal anatomic architecture of the fronto-orbital region. The metopic synostosis is characterized by premature closure of the metopic suture. This early closure results in a wide spectrum of deformity. Mild cases are marked by ridging of the metopic suture with minimal associated facial and cranial abnormalities. More severe cases are marked by varying degrees of trigonocephaly, hypotelorism and abnormalities of the orbital rim. The goal of treatment is directed towards achieving an excellent cosmetic result. The timing of surgery remains an issue. Age at first operation in Cohen’s series of 23 patients ranged from 2 months to 56 months (mean 8.2 months), with 15 patients being operated before 6 months and 8 after 7 months. The surgery in our series was performed between the ages of 6 and 52 months. The median age at operation in this series was 11.5 months. This is slightly older than reported in other series except in the studies of Kelleher et al. with a median age of 12 months and Keshavarzi with a median age of 16.6 months (Cohen and Persing, 1998; Cohen et al., 1994; Kelleher et al., 2007; Keshavarzi et al., 2009). Many surgeons favour operative intervention during the first 3e9 months of life (Delhemmes et al., 1986; Genitori et al., 1991; Aryan et al., 2005). Hilling et al’s patients were operated at a mean age of 13 months (range 5e51 months), with 80% undergoing surgery between the age of 6 and 15 months. Using the Spearman rank correlation, their data suggest that there is no significant correlation between the child’s appearance and the age in months at which the operation was performed within the given range of age at surgery (Hilling et al., 2006). Collmann reported a male predominance of 65e85%. In line with Collmann and other authors, our patients showed a male predominance of 80% (Genitori et al., 1991; Collmann et al., 1996). The average hospital stay in Aryans series was 3.6 days, with an ICU stay required for only six patients (Aryan et al., 2005). In Keshavarzi et al’s series, average stay was 2.76 days (Keshavarzi et al., 2009). In our series, the average hospital stay after operation was 5 days, with a planned ICU stay for all patients required for only 1 day. In our experience, maximal post-operative swelling took place on the second day. We preferred to observe patients until there was a clinical seduction of swelling before leaving the hospital. Kelleher et al. reported an average blood loss of 200 ml, with surgery being performed at 12 month of age on average (Kelleher et al., 2007). Aryan et al. reported an average blood loss of 400 ml, with the surgery being performed at 7.5 months of age on average (Aryan et al., 2005). Estimated mean blood loss in a study of Keshavarzi was 190 ml in a group of 17 patients (Keshavarzi et al., 2009). The average (mean) transfusion volume for patients treated with fronto-orbital advancement was 184 ml, with 5 (29%) patients requiring no blood transfusion. The surgery was performed at a median age of 16.6 months. The transfused blood for each of our patients was given to replace the amount of the estimated blood loss during the operation. Although the average amount of blood loss in our series was less than 255 ml, this still represents a considerable percentage of the baby’s blood volume and clearly has to be taken into account when considering timing. In 50 of our cases, titanium plates were used for fixation. We removed the material after 6 months. In the last four treated cases of our series, we changed our approach and used resorbable plates for fixation because there were some disadvantages using titanium plates. The plates either had to be removed at a second operation or there was growth-induced intracranial migration of the metal osteosynthesis plates and wires and there were potential complications related to this (Kosaka et al. 2003; Stelnicki and Hoffman, 1998). The development and bone apposition/resorptive pattern of skull growth create the potential for growth restriction and eventual metal device translocation to the endocranial surface may be seen (Fearon
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Fig. 3. a, b, c, d: Intra-operative photograph (a, b) showing metopic abnormality of the supraorbital rim and the brain, result after fronto-orbital remodelling (c). Post-operative result (d).
Fig. 4. a, b: Pre- and Post-operative photograph of the child seen in Figs. 2 and 3. Post-operative picture was taken 6 months after surgery.
M. Engel et al. / Journal of Cranio-Maxillo-Facial Surgery 40 (2012) 347e353 Table 2 Results according to Whitaker classification (total number of patients ¼ 54). Category
Number of patients
I II III IV
43 10 0 1
et al. 1995; Orringer et al. 1998). Currently we use resorbable osteosynthesis materials for all craniosynostosis procedures in infancy to avoid the known disadvantages (Becker et al. 1999; Imola et al. 2001; Obwegeser, 1998). Pre-operative imaging can be helpful to confirm the clinical diagnosis of trigonocephaly. Computed tomographic scans permit excellent visualization of the underlying bony architecture, helping surgeons appreciate bony anomalies and plan surgical correction. Plain radiographs ensure that the metopic suture is closed and no other suture is affected. MRI shows soft-tissue alterations, such as triangular brain deformation anteriorly and can be used to detect clinical evidence of increased intracranial pressure (Kotrikova et al., 2007). We believe that physical examination is a very sensitive tool in the diagnosis of the metopic sutural craniosynostoses. In our clinical experience, we are in agreement with Fearon et al., that craniofacial surgeons with various levels of experience were able to accurately diagnose single-sutural synostosis in most cases by physical examination alone. Considering the potential side effects from ionizing radiation, risks of sedation, and costs, surgeons may wish to reserve computed tomographic scans only for infants with suspected metopic suture craniosynostosis, in whom the physical examination is not clearly diagnostic (Fearon et al., 2007). Metopic synostosis affects not only the normal anatomic architecture of the fronto-orbital region, but it also results, as some authors have described, in concomitant intracranial abnormalities, such as cognitive deficits, and in elevated intracranial pressure. Some authors believe that the intracranial pathology is due to the congenital anomaly and not secondary to constriction caused by the synostosis. Treatment is directed towards achieving an excellent aesthetic result rather than addressing the intracranial pathology (Aryan et al., 2005). Seven of our 54 patients were found to have a mild developmental delay. In the series of Aryan, six of the 39 patients (15%) were found to have developmental delay (Aryan et al., 2005). Eight of Collmann et al.’s 73 patients had mental delay (Collmann et al., 1996). Anderson and Geiger’s series had an 18% rate of mental retardation. Oi and Matsumoto reported about five with cognitive deficits in a series of 13 patients (Oi and Matsumoto, 1987). They felt that the degree of angulation of the frontal bone correlated with the degree of disability. While intracranial pressure can be elevated in some patients, it is rarely elevated highly, and gross papilloedema or optic nerve changes have only been reported rarely (Tuite et al., 1996). We use noninvasive ophthalmological examination to eliminate papilloedema as a sign of high ICP. The presence of papilloedema with elevated ICP should be noted and CT-scans or MRI should be obtained (Cohen and Persing, 1998). The MRI or CT-scans of our patients did not reveal any signs of intracranial pressure. In two of all our cases, the ophthalmological examination showed a mild papilloedema preoperatively without any other clinical signs of elevated intracranial pressure as well as an unremarkable CT scan and MRI. The papilloedema of these patients resolved during the follow-up examinations after surgery. In our opinion, fundoscopic examination as a noninvasive technique to estimate ICP should be performed in all cases. The diagnosis of elevated ICP for children with single-suture craniosynostosis must be made from a constellation of clinical
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findings and can be supported by the measurement of ICP in selected cases (Thompson et al., 1995a, b). Other noninvasive techniques were reported by other authors. Rifkinson-Mann et al. recently found increased resistance to CSF absorption for 70% of patients with craniosynostosis when cerebral haemodynamics were analyzed by transcranial Doppler (Rifkinson-Mann et al., 1995). Gault et al. attempted to correlate ICP with the reduction in intracranial volumes as measured by CT-scans. Volume measurements alone did not serve as a reliable predictor of elevated ICP (Gault et al., 1992). Utilizing invasive techniques Thompson et al. reported their results with overnight, subdural ICP monitoring of 74 children with premature closure of a single cranial suture. A single coronal suture was involved in 37 patients, a sagittal suture was involved in 25 patients, and a metopic suture was involved in 12 patients. The ages of the patients ranged from 2 months to 10 years, with 35 children being under 1 year of age at the time of monitoring. ICP was raised in 13 (17%), borderline in 28 (38%), and normal in 33 (45%) patients. Thirty-three percent with metopic synostosis had borderline or elevated ICP (Thompson et al., 1995a, b). Marchac reported about 8% of elevated intracranial pressure for craniosynostosis (Marchac and Renier, 1986). We are in agreement with other authors, that the prevalence of ICP may be higher than previously reported and chronic and gradual increases in ICP may occur in the absence of papilloedema or an unremarkable CT or MRI (Rifkinson-Mann et al., 1995). We avoid invasive techniques to estimate ICP. We prefer fundoscopy as a noninvasive technique to estimate ICP. If there is any sign of papilloedema, we will use MRI or CT scan to clarify ICP. In children less than 1-yearold with isolated synostosis of the metopic suture, whose parents already wish to proceed to normalize skull shape, ICP monitoring is unlikely to contribute to clinical management. For children without signs and symptoms of elevated ICP, whose fundoscopy and CT or MRI do not suggest elevated ICP and whose parents do not elect for surgery, close follow-up by a paediatric neurosurgeon, a craniofacial surgeon, a neurodevelopmental specialist and a paediatric ophthalmologist for fundoscopic examination is recommended. Improvement in behaviour has been observed with resolution of papilloedema (Cohen et al., 1993; Campbell et al., 1995). In all cases in our series, no major complications or deaths were seen, similar to other series (Aryan et al., 2005; Fearon et al., 2007; Kelleher et al., 2007; Keshavarzi et al., 2009). Complications in Cohen’s series included minor wound dehiscence (n ¼ 1), seizures (n ¼ 1), and increased intracranial pressure (n ¼ 1) (Cohen et al., 1994). Dural tears common after surgical correction of craniosynostosis. The dural tears in six of our 54 cases were not thought to be major complications, and were closed immediately. No CSF fistula was seen in follow-up. Dural tears ranged from 5% to 60% in other series (Renier et al., 1985; Mühling, 1995; Esparza et al., 2008). To avoid CSF fistulas with their known complications and treatment, dural tears need be repaired immediately intra-operative (Mühling, 1995). Our patients presented with varying degrees of diffuse deformities of the frontal bones, with extension around the orbits. In our 54 cases the craniofacial reconstruction was performed to achieve an overall correction of the frontal bones as well as the periorbital region. Results in our series were satisfactory in 53 cases (Class I and II outcome) except one patient with a recurrence of the bony ridge at the metopic suture, needing further therapy (Class IV outcome). Our Class 4 patient had a recurrence of the bony ridge at the metopic suture. This patient underwent a second operation for burring of the metopic ridge, with a very good cosmetic result at 9year follow-up. Aryan et al. reported similar results. 31 patients in a series of 39 cases with metopic synostosis showed Class I and 5 with Class II outcome with three patients who needed further treatment (Aryan et al., 2005). In the study by Kerwin et al. of 39
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patients, only one required repeat surgery (Kerwin et al., 1997) Esparza et al. used frontal remodelling without fronto-orbital “bandeau” in 50 cases of trigonocephaly, which was performed at a mean age of 6.65 months. According to Whitaker’s classification, the results of Esparaza in 42 patients were classified into Category I, 7 into Category II and 1 into Category III (10.6%) (Esparza et al., 2008). Sloan et al. reported excellent to good surgical results in a group of 20 patients with metopic synostosis. They also had no major complications, performed no planned reoperations, and only one patient (5%) required unplanned reoperation (Sloan et al., 1997) Total reoperation (reoperative fronto-orbital remodelling and calvarial vault reshaping) was necessary in two patients (9%) of Cohen’s Collective (Cohen et al., 1994). Bitemporal hollowing is common after surgical correction of metopic synostosis (Persing et al., 1994; Oh et al., 2006; van der Meulen et al., 2009). Hollowing is usually located just lateral and slightly cranial to the lateral apex of the eyebrow. Theories on its aetiology focus either on bone or on soft-tissue. Aryan reported that the temporalis muscle should be carefully reinserted at its original site or even be transferred anteriorly in order to improve the postoperative temporal depression (Aryan et al., 2005). In contrast to Aryan, van der Meulen describes that the iatrogenic damage to the temporal bone for instance could result from the two osteotomies that are performed in that area in a parallel horizontal plane to mobilize the supraorbital bandeau. For him, temporal hollowing seems to be of bony origin and can be explained by skeletal growth inhibition in the affected area (van der Meulen et al., 2009). The surgical technique seems to be relevant in the origin of bitemporal hollowing. The choice of operative technique could result in an underestimation of the lateral expansion needed to correct the initial dysmorphology (van der Meulen et al., 2008; van der Meulen et al., 2009). The posterior edge of the forehead in the temporal region could end up not extending sufficiently backwards to support or fill the temporal area, leaving a temporal depression (Persing et al., 1994; Oh et al., 2006; van der Meulen et al., 2009). So, the posterior edge in the temporal area should be extended to enlarge the bitemporal deficiency. In our experience, a slight bilateral temporal depression was seen in majority of our cases although the temporalis muscles were carefully reinserted. In future cases of treatment for trigonocephaly, our interest will focus on the surgical technique with an adequate lateral expansion enlarging the bitemporal diameter to improve the phenomenon of post-operative temporal hollowing. We believe that temporal hollowing is based on a combination of surgical technique, bony origin with skeletal growth inhibition and softtissue.
5. Conclusion As the current techniques have been standardized for routine use, surgical risks are reasonably low with no mortality or permanent morbidity. In agreement with other authors, we think that the treatment of single metopic synostosis is safe with very low reoperation rates and short hospital stay. Overall, our results for fronto-orbital advancement in cases of isolated metopic synostosis showed acceptable minor complication rates and generally satisfactory aesthetic outcomes. Conflict of interest All authors disclose any financial and personal relationships with other people or organisations that could inappropriately influence or bias this work.
This study was not sponsored by any other person or organisation that was not affiliated to the Department of Oral and Maxillofacial Surgery.
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