Fronto-orbital Remodeling Research Articles

Metopic craniosynostosis: Dynamic cranioplasty for trigonocephaly versus fronto-orbital remodeling and advancement — A retrospective study

The aim of this study was to compare a traditional fronto-orbital remodeling and advancement (FORA) with the dynamic cranioplasty for trigonocephaly (DCT).The authors analyzed patients who underwent surgery for trigonocephaly. Perioperative data were compared. Parents were asked to use a visual analog scale to evaluate the pre- and postoperative distance between the eyes, the forehead shape, and the global appearance of the face. A panel of observers was asked to grade pre- and postoperative photographs using a similar visual analog scale. Pre- and postoperative anthropometric data were collected and analyzed in a subset of the study population aged 9 years or older.The total sample size was 51 patients (DCT n = 39; FORA n = 12). Durations of surgery and anesthesia were shorter in the DCT group (115 vs 194 min, p = 0.001; 226 vs 289 min, p = 0.001). Patients in the DCT group received similar preoperative ratings to those in the FORA group, but significantly higher postoperative ratings by parents for all three questions. There were no significant differences in postoperative ratings by the panel or postoperative anthropometric data.DCT is safe and effective. It is preferred over FORA because it is associated with shorter durations of surgery and anesthesia, while providing higher degrees of parental satisfaction and similar aesthetic and anthropometric outcomes.

Preclinical Application of Augmented Reality in Pediatric Craniofacial Surgery: An Accuracy Study

Augmented reality (AR) allows the overlapping and integration of virtual information with the real environment. The camera of the AR device reads the object and integrates the virtual data. It has been widely applied to medical and surgical sciences in recent years and has the potential to enhance intraoperative navigation. In this study, the authors aim to assess the accuracy of AR guidance when using the commercial HoloLens 2 head-mounted display (HMD) in pediatric craniofacial surgery. The Authors selected fronto-orbital remodeling (FOR) as the procedure to test (specifically, frontal osteotomy and nasal osteotomy were considered). Six people (three surgeons and three engineers) were recruited to perform the osteotomies on a 3D printed stereolithographic model under the guidance of AR. By means of calibrated CAD/CAM cutting guides with different grooves, the authors measured the accuracy of the osteotomies that were performed. We tested accuracy levels of ±1.5 mm, ±1 mm, and ±0.5 mm. With the HoloLens 2, the majority of the individuals involved were able to successfully trace the trajectories of the frontal and nasal osteotomies with an accuracy level of ±1.5 mm. Additionally, 80% were able to achieve an accuracy level of ±1 mm when performing a nasal osteotomy, and 52% were able to achieve an accuracy level of ±1 mm when performing a frontal osteotomy, while 61% were able to achieve an accuracy level of ±0.5 mm when performing a nasal osteotomy, and 33% were able to achieve an accuracy level of ±0.5 mm when performing a frontal osteotomy. despite this being an in vitro study, the authors reported encouraging results for the prospective use of AR on actual patients.

Infants With Trigonocephaly Treated With Fronto-Orbital Remodelling and Endocortical Positioning of Resorbable Plates-A Retrospective Cohort Study Including 101 Patients.

Fronto-orbital remodeling is the gold standard for surgical treatment of metopic synostosis in many craniofacial centers. For fixation, resorbable materials are widely used. Because of swelling during resorption, the positioning of the plates can be relevant. Therefore, the goal of this study was to investigate whether there are limitations to endocortical positioning of resorbable plates. In this retrospective study, all patients with metopic craniosynostosis resulting in trigonocephaly, who were treated with a standardized fronto-orbital remodeling and endocortical positioning of resorbable plates, between February 2012 and December 2019 were included. The endpoint of this study was the postoperative complications that can be attributed to the material used. A total of 101 patients, who were treated at our unit, could be included with a male predominance, 81 (80.2%) boys versus 20 (19.8%) girls. Surgery was performed at a median age of 9 months (±2.56). No complications were seen related to resorbable plates. All patients of our study population were categorized as Whitaker classification I and II. This study shows that the risk level in using endocortical resorbable plates positioned on the inner surface of the bone is low. From an aesthetic point of view, we could not detect any disadvantage.

Persistent discrepancies in orbital morphology after surgical treatment of unicoronal craniosynostosis: a critical image-based analysis.

Unicoronal craniosynostosis (UCS) is characterized by complex orbital deformity and is typically treated by asymmetrical fronto-orbital remodeling (FOR) during the 1st year of life. The aim of this study was to elucidate to what extent orbital morphology is corrected by surgical treatment. The extent to which orbital morphology was corrected by surgical treatment was tested by analysis of differences in volume and shape between synostotic, nonsynostotic, and control orbits at two time points. In total, 147 orbits were analyzed from patient CT images obtained preoperatively (mean age 9.3 months), at follow-up (mean age 3.0 years), and in matched controls. Semiautomatic segmentation software was used to determine orbital volume. For analysis of orbital shape and asymmetry, geometrical models, signed distance maps, principal modes of variation, and three objective parameters (mean absolute distance, Hausdorff distance, and dice similarity coefficient) were generated by statistical shape modeling. Orbital volumes on both the synostotic and nonsynostotic sides were significantly smaller at follow-up than volumes in controls and significantly smaller both preoperatively and at follow-up than orbital volumes on the nonsynostotic side. Significant differences in shape were identified globally and locally, both preoperatively and at 3 years of age. Compared with controls, deviations were mostly found on the synostotic side at both time points. Asymmetry between synostotic and nonsynostotic sides was significantly decreased at follow-up, but not compared with the inherent asymmetry of controls. On a group level, the preoperative synostotic orbit was mainly expanded in the anterosuperior and anteroinferior regions and smallest on the temporal side. At follow-up, the mean synostotic orbit was still larger superiorly but also expanded in the anteroinferior temporal region. Overall, the morphology of nonsynostotic orbits was more similar to that of controls than to synostotic orbits. However, the individual variation in orbital shape was greatest for nonsynostotic orbits at follow-up. In this study, the authors presented what is, to their knowledge, the first objective automatic 3D bony evaluation of orbital shape in UCS, defining in greater detail than has been done previously how synostotic orbits differ from nonsynostotic and control orbits, and how orbital shape changes from 9.3 months of age preoperatively to 3 years of age at the postoperative follow-up. Despite surgical treatment, both local and global deviations in shape persist. These findings may have implications for future directions in the development of surgical treatment. Future studies connecting orbital morphology to ophthalmic disorders, aesthetics, and genetics could provide further insight to enable better outcomes in UCS.

3-Dimensional Morphometric Outcomes After Endoscopic Strip Craniectomy for Unicoronal Synostosis.

Endoscopic strip craniectomy with postoperative helmeting (ESCH) for unicoronal synostosis has shown to be a less morbid procedure when compared with fronto-orbital remodeling (FOR). We aim in this pilot study to report objective methods and quantitative morphologic outcomes of endoscopically treated unicoronal synostosis using 3-dimensional surface scans. Our electronic records were reviewed for ophthalmological, neurodevelopmental outcomes, and helmet-related complications. For morphologic outcomes, the following parameters were used: Cranial Index, Cranial Vault Asymmetry Index, Anterior Symmetry Ratio (ASR), and Root Mean Square between the normal and synostotic sides of the head. Three-dimensional stereophotogrammetry scans were evaluated at 3 time points preoperative, 6 months post-op, and at the end of the treatment, which was compared with age-matched scans of normal controls and FOR patients. Nonparametric tests were used for statistical analysis. None of the ESCH cases developed strabismus, major neurodevelopmental delay, or helmet complications. All morphologic parameters improved significantly at 6 months post-op except for the Cranial Vault Asymmetry Index. The ASR was the only parameter to change significantly between 6 months post-op and final scans. At end of helmet treatment, ASR and Root Mean Square differed significantly between the ESCH and both FOR and control groups. Endoscopic strip craniectomy with postoperative helmeting for single unicoronal synostosis had excellent clinical outcomes. Most of the improvement in head morphology occurred in the first 6 months of treatment. Despite the normalization of the overall head shape, there was residual asymmetry in the frontal and temporal regions of the head.

Correction of trigonocephaly after endoscopic strip craniectomy with postoperative helmet orthosis therapy: a 3D stereophotogrammetric study.

Endoscopic strip craniectomy with postoperative helmet orthosis therapy (ESCH) has emerged as a less invasive alternative to fronto-orbital remodeling for correction of trigonocephaly. However, there is no standardized objective method for monitoring morphological changes following ESCH. Such a method should be reproducible and avoid the use of ionizing radiation and general anesthesia for diagnostic imaging. The authors analyzed a number of metrics measured using 3D stereophotogrammetry (3DSPG) following ESCH, an imaging alternative that is free of ionizing radiation and can be performed on awake children. 3DSPG images obtained at two time points (perisurgical and 1-year follow-up [FU]) of children with metopic synostosis who had undergone ESCH were analyzed and compared to 3DSPG images of age-matched control children without craniofacial anomalies. In total, 9 parameters were measured, the frontal angle and anteroposterior volume in addition to 7 novel parameters: anteroposterior area ratio, anteroposterior width ratios 1 and 2, and right and left anteroposterior diagonal ratios 30 and 60. Six eligible patients were identified in the operated group, and 15 children were in the control group. All 9 parameters differed significantly between perisurgical and age-matched controls, as well as from perisurgical to FU scans. Comparison of FU scans of metopic synostosis patients who underwent surgery to scans of age-matched controls without metopic synostosis revealed that all parameters were statistically identical, with the exception of the right anteroposterior diagonal ratio 30, which was not fully corrected in the treated patients. The left anterior part of the head showed the most change in surface area maps. In this pilot study, ESCH showed satisfactory results at 1 year, with improvements in all measured parameters compared to perisurgical results and normalization of 8 of 9 parameters compared to an age-matched control group. The results indicate that these parameters may be useful for craniofacial units for monitoring changes in head shape after ESCH for trigonocephaly and that 3DSPG, which avoids the use of anesthesia and ionizing radiation, is a satisfactory monitoring method.

Establishing Standards of Care in Craniosynostosis: Results from a Survey of ERN CRANIO Member Institutions

European Reference Networks (ERNs) are networks involving hospitals with particular expertise in rare conditions. ERN-CRANIO focuses on rare disorders of the skull and face including craniosynostosis. We undertook a pilot study in the form of an electronic survey to understand current practice in craniosynostosis management across ERN-CRANIO, which at the time consisted of 29 member institutions across 11 countries. Most (19 of 29) units replied; however, some answered the survey only partially. The majority (87.5%) of units have specific management protocols. For single-suture cases, 8 of 15 units see >50 new cases per year, 4 of 15 see 21-50 cases, and 3 of 15 see fewer than 20 cases. Duration of follow-up ranges from age 10 years or less (3 of 6) to indefinite (2 of 6). A variety of surgical techniques are used. For sagittal synostosis, the endoscopic and helmet technique is the most common (5 of 14), and for metopic and unicoronal synostosis, preferred by 9 of 14 centers was the frontoorbital remodeling (bandeau) technique. For multisutural syndromic craniosynostosis, 2 of 16 centers see >20 new cases per year, 4 of 16 see 11-20 cases, 5 of 16 see 6-10 cases, and 4 of 16 see fewer than 5 cases. Most centers (12 of 15) either never discharge syndromic patients or follow until adulthood. Eleven of 14 units perform prophylactic vault expansion, while 3 of 14 wait for clinical indication. Nine of 13 units operate at 6-12 months. Again, a wide variety of techniques are used, most commonly frontal advancement (4 of 13) initially. This study provides a useful snapshot of current standards of care in craniosynostosis across the high-volume centers of the ERN. Going forward, these results can be used to direct more detailed analysis of current practice, which will then be useful for constructing a management guideline for patients presenting with both single-suture and multisutural craniosynostosis.

Intravenous Tranexamic Acid is Associated With a Clinically Significant Reduction in Blood Loss in Craniosynostosis Surgery.

Blood loss is a potential cause of morbidity and mortality in craniosynostosis surgery. Recent reports have suggested that the use of tranexamic acid (TXA), an antifibrinolytic agent, mitigates this blood loss. A retrospective cohort study of patients undergoing craniosynostosis surgery at a tertiary craniofacial hospital in Sydney was undertaken. Primary outcomes were blood loss and transfusion requirements. Two groups were compared: those who received intravenous prophylactic TXA and those who underwent surgery without TXA. Statistical analysis was performed with Student t test and the Mann-Whitney U test for nonparametric results. We identified 206 patients who underwent craniosynostosis surgery over an 8 year period; 78 control patients and 128 patients that received TXA. Tranexamic acid was found to result in a weight-adjusted calculated blood loss mean difference of 9.6 ml/kg across all procedures (P = 0.0332 95% confidence interval 0.7734-18.4266). The actual blood loss reduction achieved with TXA was 6.7 ml/kg in spring cranioplasties, compared to 15.2 ml/kg in fronto-orbital remodeling procedures. There was a statistically and clinically significant reduction in postoperative transfusion incidence, with transfusions required in 27% of controls and 6% of TXA patients (P < 0.0001). The number needed to treat to prevent giving a unit of blood postoperatively was 4.8. There were no incidences of TXA-specific complications. This study found that TXA is a safe and effective method of decreasing blood loss and transfusion requirements in patients undergoing craniosynostosis surgery. The clinical benefit of TXA is particularly evident in the more invasive craniosynostosis surgeries.

Photogrammetric evaluation of corrective surgery for trigonocephaly

The aim of this study was to capture preoperative, postoperative, and follow-up head shapes of male trigonocephaly patients who underwent fronto-orbital remodelling (FOR), using three-dimensional (3D) photography. Fifty-seven male infants with metopic synostosis operated on using standardized FOR during a 5-year period were included. All measurements were compared with those of an age-matched healthy control cohort (n = 253 for early postoperative comparison, n = 43 for the 1-year follow-up comparison) to determine the effect of FOR at 14 days and at 1 year post-surgery. Intracranial volume, frontal angle, nasofrontal angle, interfrontoparietal–interparietal ratio, and inter-orbital distances were measured 1 day preoperatively, 14 days and 1 year postoperatively. Mean age at surgery was 9.7 ± 1.1 months. Prior to surgery, boys with metopic synostosis showed a reduced intracranial volume (−7.0%, P < 0.001), frontal angle (−10.2%, P < 0.001), interfrontoparietal–interparietal ratio (−4.9%, P < 0.01), and orbital distances (−6.5%, P < 0.001) compared to the reference group, but values did not differ significantly from the specific control group after surgery (all P> 0.05). This was consistent by the time of the follow-up examination. Corrective surgery should therefore aim to achieve volume expansion and correction of the deformity. Furthermore, 3D photogrammetry provides a valuable alternative to computed tomography scans in the diagnosis of metopic synostosis, significantly reducing the amount of radiation exposure to the brain.

Emissary veins and pericerebral cerebrospinal fluid in trigonocephaly: do they define a specific subtype?

The premature fusion of the metopic suture may be associated with the presence of emissary veins (EV) and abnormally large pericerebral cerebrospinal fluid (CSF) spaces which suggest an associated focal disturbance in CSF dynamics. The incidence of such findings and their potential significance in terms of management of the disease have not been fully elucidated. The aim of this study is to investigate whether these phenomena identify specific subtypes of trigonocephaly. In such a direction, we evaluated the volume of the pericerebral CSF spaces and their relationship to the morphology ("Ω," "V," or flat type) of the prematurely fused metopic suture and to the value of the interfrontal angle value on the grounds of computed tomographic (CT) scan examinations. The preoperative brain CT scans of 74 children (52 boys, 22 girls) with trigonocephaly who had undergone fronto-orbital remodeling were evaluated. The volume of the pericerebral CSF spaces and the value of the interfrontal angle were calculated. The type of intracranial notch was studied and classified according to its shape on the preoperative CT scan: a groove "Ω," a ridge/"V" ridge or absent when flat and evidence of emissary veins related to the abnormally fused suture. Preoperatively, an endocranial metopic groove or ridge was seen in 70% of the children. Emissary veins were identified in 34 of 74 patients (45%), at a mean distance of 2.04 cm (1.18-2.94 cm) from the nasion. The presence of large pericerebral CSF spaces significantly correlated with the presence of EV (p < 0.05), with the "Ω" type (p < 0.05) and with interfrontal angles under 134° (p < 0.005). Metopic suture early fusion shows an association between EV, pericerebral CSF spaces, and the "Ω" groove appearance of the suture. This association identifies a specific subgroup in which the presence of emissary veins and large pericerebral CSF spaces is an indicator of local venous hypertension due to the sagittal sinus constriction within an osseous groove created by the abnormal suture fusion process. The implications for the surgical management and long-term results as compared to trigonocephalic children with small or absent normal peripheral spaces and EV are still to be determined.

Sporting activity after craniosynostosis surgery in children: a source of parental anxiety

PurposeCraniosynostosis correction involves major skull surgery in infancy—a potential source of worry for parents when their treated children begin involvement in sports.MethodsElectronic multiple choice survey of parents of children who had undergone craniosynostosis surgery in infancy using 5-point Likert scales.ResultsFifty-nine completed surveys were obtained from parents of children who had undergone previous craniosynostosis surgery. Mean age of children was 7.8 years (range 3 months to 22 years), with 36 non-syndromic and 23 syndromic cases. The most common surgery was fronto-orbital remodelling (18). Fifty-two of 59 were involved in athletic activity. The most intense sport type was non-contact in 23, light contact in 20, heavy contact in 4 and combat in 5. Participation level was school mandatory in 12, school club in 17, non-school sport club in 21 and regional representative in 2. One child had been advised to avoid sport by an external physician. Mean anxiety (1–5 Likert) increased with sport intensity: non-contact 1.7, light contact 2.2, heavy contact 3.5 and combat 3.6. Twenty-nine of 59 parents had been given specific advice by the Craniofacial Team regarding athletic activity, 28 of which found useful. Three sport-related head injuries were reported, none of which required hospitalisation.ConclusionLittle information exists regarding sports for children after craniosynostosis surgery. This study suggests that parental anxiety remains high, particularly for high impact/combat sports, and that parents would like more information from clinicians about the safety of post-operative sporting activities.

Minimising blood loss in Fronto-orbital Advancement Remodelling (FOAR) - the development of an improved technique over 10 years

Minimising blood loss in Fronto-orbital Advancement Remodelling (FOAR) - the development of an improved technique over 10 years

Partial hybrid cranial vault remodeling in late correction of scaphocephaly and revision surgery. A monocentric retrospective study of nine consecutive cases

There is currently no consensus on the surgical attitude to be adopted for late management of sagittal synostosis or for revision scaphocephaly surgeries without prolapse of the cerebellar tonsils. We present here a monocentric retrospective study of nine patients operated consecutively by a fixed expansion method of the cranial vault which may be associated with a fronto-orbital remodeling. The procedure consists in the realization of multiple parietal tongue-in-groove osteotomies fixed by resorbable plates. Simultaneous fronto-orbital remodeling have been performed when needed. No intraoperative complication was noted. The average operating time was 141min. Six patients (66.7%) had a blood transfusion during the perioperative period. The average hospital stay was 4.8days. With a mean follow-up of 26,7months, no surgical revision was noted. In all patients with clinical or ophthalmologic signs of intracranial hypertension, we highlighted a disappearance of signs within 4months. No protective helmet has been used. The craniofacial remodeling was judged very good by the family and the surgical team. Multiple tongue-in-groove tenons remolding cranioplasty associated or not with a fronto-orbital advancement is a safe technique. It seems to us to be a good alternative to floating or fixed bi-parietal or bi-parieto-frontal cranial flaps because of the very large increase in endocranial volume and the possibility of physiologically and aesthetically remodeling the parietal and fronto-orbital regions.

Controversy on the Age of Hypertelorism Correction.

Orbital hypertelorism (HTO) is a challenging craniofacial problem seen in association with some congenital deformities. The age of HTO correction is a matter of debate. to evaluate the outcome of HTO correction and determine the optimal timing for intervention, striving for the earliest possible intervention with the lowest relapse. A standard craniofacial approach with medial bone resection, 4 walls orbital box osteotomy and orbital medialization were done for all patients. Skeletal and soft tissue procedures were done as indicated. there were 10 patients aging 6 to 19 years. Seven were associated with craniofacial clefts, and 3 with craniosynostosis syndromes. HTO was severe in 8 cases and moderate in 2 cases. It was asymmetric in 2 cases. Frontoorbital remodeling was done in 3 cases with craniosynostosis. Failed surgery was reported in 2 cases. A redo surgery was done for one of them with an excellent outcome, while refused by the other. Nine patients had an excellent outcome. The mean level of satisfaction was 93.37%. Three patients had ugly facial scars. No major complications were recorded. The time for surgical treatment of HTO is determined by the severity of the associated deformity. If there is an urgent factor indicating intervention, early correction can be performed exceptionally; otherwise, HTO correction should be performed after the age of 6 years.

S1-04 SESSION 1: NEUROSURGERY PREDICTIVE FACTORS OF FRONTAL LOBE CHANGES FOLLOWING FRONTOFACIAL SURGERY

Introduction: Children with syndromic faciocraniosynostosis may require frontofacial surgery (FFS) to treat exophthalmos, obstructive sleep apnoea, raised ICP and facial deformity. FFS procedures include monobloc, midfacial bipartition, Le Fort III with fronto-orbital remodelling and orbital box osteotomy. These techniques can be static or use external distraction via RED-frame. A previous study from our unit reported post-FFS frontal lobe changes (FLC) in 2/3 of the patients, but risk factors were not identified.1The aim of this study is to identify predictive factors for FLC following FFS. Methods: Pre- and postoperative CT scans (when both available and excluding those with artefacts) of patients who had FFS were analysed. Demographics, diagnosis, history of previous craniofacial surgery, operation notes and complications were collected. Two paediatric craniofacial neurosurgeons independently assessed all CT scans on: abnormal bony structures (i.e.copper beating, deep sphenoid and/or midline ridge, high cristae galli, clover leaf skull), metalwork (i.e. wires, plates and screws), ventriculoperitoneal (VP) shunt, and frontal lobe changes. Results: The inter-observer agreement for CT assessments between the 2 examiners was 0.92. Of 51 consecutive patients (M:F, 29:22) who had FFS (at average age 8.3 years, range 3 months to 19.1 years old) met the inclusion criteria, 20 had had previous FFS. (Increased) FLC following FFS was seen in patients who had: 1) previous FFS with pre-existing damage (5/7) and without pre-existing damage (5/13); 2) intraoperative dural tears: single (6/14) and multiple dural tears (5/10); 3) FFS under the age of 1 year old (3/3); 4) bony abnormalities (overall 15/39 and 3/31 as first-time FFS); 5) Monobloc RED-frame distraction (14/36); and 6) metalwork in situ (3/6), 3/3 with metalwork in situ and normal brain parenchyma prior to FFS had FLC. No association was found between FLC and syndromic diagnosis, age above one year old and presence of VP shunt. After first-time FFS, 77,4% showed normal brain parenchyma on post-operative CT-scan. Conclusion: While the majority of single time FFS results in no CT- visable FLC, such changes are seen in 12.6% of the first-time FFS cases – a significant lower number than our previous study. This study suggests the following predictive factors for post-FFS FLC: frontal surgery at young age (<1-year-old), previous FFS, use of RED-frame, intra-cranial metal work and abnormal bony structures.

A 24-month cost and outcome analysis comparing traditional fronto-orbital advancment and remodeling with endoscopic strip craniectomy and molding helmet in the management of unicoronal craniosynostosis: A retrospective bi-institutional review

IntroductionEndoscopic strip craniectomy with helmeting (ESCH) has been shown to be a safe and efficacious alternative to fronto-orbital remodeling (FOR) for selected children with craniosynostosis. In addition to clinical factors, there may be economic benefits from the use of ESCH instead of FOR. MethodsA retrospective review of 23 patients with nonsyndromic unicoronal craniosynostosis (UCS) treated with FOR was carried out at Great Ormond Street Hospital (GOSH) for Children in London, UK. Secondary data were used for the ESCH cohort from a paper published by Jimenez and Barone (2013). Data were collected on surgical time, transfusion rates, length of hospital stay, adverse event rates, reintervention rates, and overall costs. Costs were categorized and then assigned to the appropriate data sets. ResultsThe mean age of patients undergoing FOR (vs. ESCH) was 17.4 mo (vs. 3.1 mo) with a mean surgical time of 234 min (vs. 55 min), mean transfusion volume of 221.6 mL (vs. 80.0 mL), mean transfusion rate of 14/23 (vs. 2/115), and a total immediate overnight stay of 3.13 days (vs. 97% next-day discharge). The FOR group had a higher adverse event rate (5/23 vs. 4/115, p=<0.005) and a higher number requiring extraocular muscle surgery (4/23 vs. 7/109, p=0.16). There was a substantial difference in overall costs between the two groups. Total variance cost for the FOR group was £7436.5 vs. £4951.35, representing a cost difference of £2485.15 over the 24-month study period. ConclusionESCH, in comparison to FOR, appears as a more economical method in the management of USC patients, as well as having clinical benefits including reduced adverse event rate and improved ophthalmic outcomes.

Management of median and paramedian craniofacial clefts

Median and paramedian craniofacial clefts are associated with hypertelorism, anterior encephalocele, positional abnormalities of the maxilla, and nasal deformity. Cleft lip and palate, eyelid coloboma, and widow's peak are frequently present. The authors collected data from 30 patients (mean age, 5.8 years; range, 4 months to 18 years) operated between 1986 and 2017 with median or paramedian craniofacial clefts of differing degrees of severity. Malformations of the different anatomic units and their surgical treatment were assessed, as well as complication rates. All patients presented nasal malformations and either telecanthus (n = 16) or hypertelorism (n = 14). Most patients (n = 23) had anterior encephalocele. All patients underwent nasal corrections, and most of them had medial canthopexy (n = 24). Excision of encephalocele was associated with fronto-orbital remodeling. Medialization of the orbits was performed in 11 patients, mainly by box shift (n = 9). Patients from outside Switzerland (n = 23) were operated at an older age than those in the native patient group. Because of staged reconstruction, 13 patients had more than one operation. Surgical complications included three infections and one expander exposition. One patient had bone resorption of a frontal bone flap. Nasal correction needed more than one procedure in 5 patients, and medial canthopexy had to be repeated in 7 patients. Esthetic results were satisfactory, permitting social integration. Median and paramedian craniofacial clefts need adapted and carefully planned corrections respecting the growth of anatomic units. The quality of the medial canthal and nasal reconstruction is to a large extent responsible for the overall result.

Investigating the cause of late deformity following fronto-orbital remodelling for metopic synostosis using 3D CT imaging

PurposeLate deformity/indentation is well-recognised following fronto-orbital remodelling (FOR) for metopic synostosis. We hypothesise that if damage to temporalis muscle were a contributor, the thickness of soft tissue and bone in the affected area would be reduced. Materials and methodsSoft tissues and bone were separately segmented and reconstructed three-dimensionally from computed tomograms of 8 patients 1.5–18 years post-FOR performed at 16 ± 2 months for metopic synostosis and from 8 age-matched controls. Soft tissue (taken as proxy for temporalis muscle) and bone thickness overall and in the indented areas were computed. ResultsPost-FOR, three-dimensional soft tissue thickness maps demonstrated temporalis extending upwards but falling short of the indented area. Overall skull thickness increased with age post-FOR (logarithmic fit R2 = 0.71) and for controls (R2 = 0.90). Although immediately post-FOR the future indented area had a thickness of 98% of control, it decreased linearly to 64% 16 years later (Pearson's r = 0.84). ConclusionThese findings suggest that late post-FOR deformity/indentation is enhanced by limited upward extension (or retraction downwards) of temporalis muscle, while bone thickness in the affected area gradually decreases. This supports the hypothesis that aberrant re-attachment of the temporalis muscle makes a material contribution to late deformity following FOR for metopic synostosis.

Results Following Adoption of a Modified Melbourne Technique of Total Scaphocephaly Correction.

The Melbourne technique was described in 2008 as a novel method for complete correction of scaphocephaly. Since 2015, it has become our operation of choice for children with sagittal synostosis who are too old at presentation for minimally invasive techniques. Our modifications were 2-position (initially supine then prone) technique and undertaking a formal fronto-orbital remodeling to correct forehead contour. Retrospective chart review was used to record demographics, blood transfusion frequency and volumes, operating time, length of stay, clinical outcome, and complications. Eleven underwent modified Melbourne procedure between July 2015 and March 2017; 9 of 11 were male. All had a diagnosis of nonsyndromic sagittal synostosis. Mean age at surgery was 29 months. Mean surgical time was 6 hours. All patients required blood transfusion with a mean volume transfused of 29 mL/kg (range 13-83 mL/kg). For those 5 patients where preoperative and postoperative measurements were available, there was an increase in mean cephalic index (CI) from 0.64 to 0.75. All postoperative patients had a CI of over 0.70. Three-dimensional shape analysis indicated head shape change addressing all phenotypic aspects of scaphocephaly. In the 5 patients in which analysis could be undertaken, the mean intracranial volume increased from 1481 cm preoperatively to 1671 cm postoperatively, a mean increase in intracranial volume of 14%. The postoperative intracranial volume was higher than preoperative in all 5 patients. There were 4 minor and no major complications. Modified Melbourne procedure is safe and effective for the treatment of severe scaphocephaly in sagittal synostosis.

How I do it. 3D endoscopic treatment of metopic craniosynostosis through a single incision.

Endoscopic approaches for craniosynostosis are a growing field in pediatric neurosurgery. In metopic synostosis, previous reports for complete fronto-orbital remodeling have proposed an intervention with multiple incisions (bregmatic, tarsal, and preauricular) to open frontonasal and frontoethmoidal synostotic sutures, and orbital roof. We propose a technique to complete all these osteotomies with a unique incision anterior to the bregmatic fontanel under 3D endoscopic vision, and review possible complications, limits, and pitfalls. Under endoscopic assistance, a complete fronto-orbital remodeling could be completed with a unique incision without mayor drawbacks.