Secondary vault reconstruction after open or minimal invasive correction for unisutural, multisutural or syndromic craniosynostosis: A cohort study on the impact of diagnosis and type of initial surgical technique.

BACKGROUND: The aim of this study is to identify if certain types of craniosynostosis and/or surgical procedures are more likely to require a secondary intracranial procedure because of insufficient correction of skull shape or raised ICP after initial surgery. METHODS: All consecutive cases with craniosynostosis that were operated at our center between January 2010 and January 2019 were included and the number of secondary operations of the vault were determined, as well as diagnosis, the indication and type and timing of initial surgery. Monobloc and facial bipartition procedures were excluded. RESULTS: Over a nine year period, 790 vault corrections were performed in 780 patients of which 38 procedures were reoperations in 35 patient because of raised intracranial pressure or an insufficient esthetic result of the skull shape. Particularly patients with a multisutural or syndromic craniosynostosis are represented in this group, as well as three surgical procedures: 1. biparietal outfracturing for sagittal synostosis; 2. endoscopic stripcraniectomy with helmet therapy for unicoronal, multisutural or syndromic craniosynostosis; 3. conventional occipital expansion for syndromic craniosynostosis. CONCLUSIONS: The risk of a second intracranial correction for insufficient outcome of skull shape or for raised ICP is related to type of synostosis and type of initial surgical technique. Particularly multisutural and syndromic craniosynostosis are more likely to require repeat surgery for these indications. Concerning initial technique, biparietal outfracturing does not correct sagittal synostosis sufficiently, stripcraniectomy with helmet therapy appears to undercorrect unicoronal, multisutural and syndromic synostosis, and conventional occipital expansion for multisutural and syndromic synostosis has poorer outcome than occipital expansion with distraction.

Identifying the Misshapen Head: Craniosynostosis and Related Disorders.

Pediatric care providers, pediatricians, pediatric subspecialty physicians, and other health care providers should be able to recognize children with abnormal head shapes that occur as a result of both synostotic and deformational processes. The purpose of this clinical report is to review the characteristic head shape changes, as well as secondary craniofacial characteristics, that occur in the setting of the various primary craniosynostoses and deformations. As an introduction, the physiology and genetics of skull growth as well as the pathophysiology underlying craniosynostosis are reviewed. This is followed by a description of each type of primary craniosynostosis (metopic, unicoronal, bicoronal, sagittal, lambdoid, and frontosphenoidal) and their resultant head shape changes, with an emphasis on differentiating conditions that require surgical correction from those (bathrocephaly, deformational plagiocephaly/brachycephaly, and neonatal intensive care unit-associated skill deformation, known as NICUcephaly) that do not. The report ends with a brief discussion of microcephaly as it relates to craniosynostosis as well as fontanelle closure. The intent is to improve pediatric care providers' recognition and timely referral for craniosynostosis and their differentiation of synostotic from deformational and other nonoperative head shape changes.

Craniofacial Sutural Pattern and Surgical Management in Patients With Different Degrees of Trigonocephaly Severity.

The aim of this study was to identify quantitative tools to classify the severity of trigonocephaly to guide surgical management and predict outcome. METHODS: We reviewed high-resolution computed tomography images of 59 patients with metopic synostosis. We assessed the craniofacial sutural pattern as well as interfrontal and metopic angles, and we related the frontal angulation degree with the sutural pattern, the surgical management, and clinical outcome. RESULTS: We identified 3 groups according to the severity of trigonocephaly. No difference was found between the sutural pattern of nasion complex and severity, whereas the closure of zygomatic maxillary sutures increased with the severity degree (P < 0.05). The operative management was related to the severity degree (P < 0.001) and to the reduced age (P = 0.009). CONCLUSIONS: Interfrontal and metopic angles are complementary measurements to evaluate with high accuracy the degree of frontal angulation. In preoperative assessment, they may guide surgery decision in particular when the choice is not straightforward.

The unseen third dimension: a novel approach for assessing head shape severity in infants with isolated sagittal synostosis.

PURPOSE: This study aimed to develop a novel approach to assess the severity of skull dysmorphology in infants with isolated sagittal synostosis (ISS) and its relationship with the surgical results. METHODS: We divided 66 infants with ISS into three groups by combining the scaphocephalic (SSI-A) and platycephalic (VLI) indices as descriptors of the relation between length, width, and height. We evaluated each skull for morphology as hyperdolichocephalic (< 66%) versus dolichocephalic (66-77%) and as hyperplatycephalic (< 78%) versus platycephalic skull (78-85%). A score system was developed as follows: 2 points for values < 66% and < 78% and 1 point for values between 66 and 77% and 78 and 85% in SSI-A and VLI, respectively. The overall score was calculated and it was used to classify our patients on a 4-point ordinal scale, according to the severity of head shape (2 = mild, 3 = moderate, 4 = severe). RESULTS: Thirty-two infants resulted in mild group, 17 in moderate group, and 17 in severe group. SSI-A and VLI were reduced according to the severity of ISS. We demonstrated a positive correlation between SSA-A and VLI in mild subgroup of patients while we found a negative correlation between SSA-A and VLI in moderate and in severe subgroups. Moreover, a positive correlation was found between severe subgroup and Sloan III class of surgical results. CONCLUSION: This study describes a simple tool to better classify infants with ISS, considering the three-dimensional morphology of the skull, because it evaluates both the dolichocephalic and platycephalic component.

Early Surgical Treatment in Anterior Synostotic Plagiocephaly: Is This the Best Choice?

At the end of craniofacial growth, in anterior synostotic plagiocephaly, some aesthetical deficiencies may still be evident. This can depend on an inadequate initial correction or on altered postoperative growth or even on the combination of the 2 factors.Aesthetic alterations can result from various factors that could potentially affect the skeleton, the skin, subcutaneous, and muscular tissues.The pathological changes in the cutaneous and subcutaneous tissues are greater in patients who have undergone multiple surgical treatments of the frontoorbital area. The aim of this observational cohort study is to assess the residual aesthetic and functional impairment at the end of skeletal growth, in patients affected by anterior synostotic plagiocephaly who have undergone surgery at an early age. The purpose is to investigate whether early surgery can still be considered unavoidable in patients with this malformation.Between July 2012 and February 2015, patient's data were retrieved from our archives among the patients referred to our department from 2003 to 2012 for Anterior Synostotic Plagiocephaly at an early age.The authors studied this patient with CT scans and photographic documentation. On CT scans, the authors have assessed skeletal alterations, soft tissues alterations, and muscular tissue alterations. With photographic documentation, the authors have studied the perception of the malformation among external subjects.From this study it was possible to demonstrate that is many esthetical alterations are to still to be found in patients treated with an early surgical approach; for this reason in children without early complications, the authors suggest that surgical treatment should be delayed after the end of craniofacial growth.

Determining the fate of cranial sutures after surgical correction of non-syndromic craniosynostosis.

PURPOSE: "Secondary craniosynostosis" (SCS) refers to a loss of sutures after corrective vault reconstruction. There are no prior studies that comprehensively review SCS in various types of non-syndromic craniosynostosis. We assessed idiopathic and iatrogenic SCS using 3-dimensional computed tomography (3D CT). We also performed a systematic review to estimate the overall incidence of SCS in each craniosynostosis type, and to characterize its clinical features. MATERIALS AND METHODS: We retrospectively reviewed the CT images of patients who underwent surgical correction of craniosynostosis for all types of craniosynostosis between August 1999 and December 2015. A literature search of the Medline and Ovid databases was conducted in October 2016 using the search term "secondary craniosynostosis." RESULTS: In our series, iatrogenic SCS was observed in all patients who had manipulated normal patent sutures to variable extents. Three (17.6%) cases of idiopathic SCS developed on sagittal sutures, and were confirmed with a 12-month follow-up CT. In a pooled analysis of 10 articles, overall SCS developed in 123 of 1205 patients (10.2%). Iatrogenic SCS cases made up 87 of 1205 cases (7.2%), whereas 38 (3.1%) were idiopathic. Idiopathic SCS most commonly developed at the bi-coronal suture (n = 32, 84.2%), followed by the sagittal suture (n = 4, 10.5%) and uni-coronal suture (n = 1, 2.6%). CONCLUSION: This is the first review not only to describe SCS in all types of non-syndromic craniosynostosis, but also to classify SCS into iatrogenic and idiopathic types based on the underlying pathogenesis.

The Whitaker Classification of Craniosynostosis Outcomes: An Assessment of Interrater Reliability.

BACKGROUND: The Whitaker classification is a simple and widely used system for describing aesthetic outcomes after craniosynostosis surgery. The purpose of this study is to evaluate its interrater reliability for patients who have undergone fronto-orbital surgery. METHODS: A retrospective review of patients with craniosynostosis who underwent surgical intervention at a tertiary referral center was conducted. Inclusion criteria were as follows: single-suture craniosynostosis, surgical intervention before age 2 years, and photographs taken before revisions between 5 and 20 years of age. Thirteen craniofacial surgeons independently reviewed the subjects' photographs and assigned Whitaker classifications. Interrater reliability was assessed with the Cohen kappa statistic. RESULTS: Twenty-nine subjects were included. Average ages at surgery and at the time of postoperative photography were 0.8 year and 12.8 years, respectively. The κ value for all 13 raters was 0.1567 (p < 0.0001), indicating "slight agreement." Pairwise comparisons demonstrated κ values ranging from 0.0384 to 0.5492. The average rating for the set of 29 photographs differed significantly across the 13 raters (p = 0.0020) and ranged from 1.79 ± 0.68 to 2.79 ± 0.77. Finally, we found that average Whitaker classification did not differ significantly between subjects who subsequently underwent cranioplasty and/or fronto-orbital advancement and those who did not (subsequent procedures, 2.45 ± 0.55; no subsequent procedures, 1.88 ± 0.78; p = 0.1087). CONCLUSIONS: The Whitaker classification exhibits low interrater reliability and does not predict future treatment. It may benefit craniofacial surgeons to create new evaluation tools with greater precision, to improve the quality of patient care and craniofacial outcomes research.

Nonsyndromic Craniosynostosis and Deformational Head Shape Disorders.

This article provides an overview of etiology, epidemiology, pathology, diagnosis, and treatment of nonsyndromic craniosynostosis, including sagittal, metopic, coronal, lambdoid, and complex synostosis. Detailed discussion is presented regarding indications for surgical intervention and management options, including frontoorbital advancement, cranial vault reconstruction, endoscopic strip craniectomy, spring-assisted strip craniectomy, and cranial vault distraction osteogenesis. Deformational plagiocephaly is also presented with treatment options including repositioning, physical therapy, and helmet therapy.

Anthropometric Outcomes following Fronto-Orbital Advancement for Metopic Synostosis.

BACKGROUND: The authors' purpose is to present changes in anthropometric fronto-orbital dimensions after surgical correction of metopic synostosis. METHODS: The authors retrospectively analyzed craniometric dimensions in older patients with metopic synostosis corrected by fronto-orbital advancement performed by the senior author (J.B.M.). Preoperative and postoperative linear measures (frontal breadth, cranial width, and intercanthal distance) were taken by direct anthropometry. Interdacryon distance and width of the bandeau were also recorded intraoperatively, before and after widening. Follow-up anthropometric values were compared to age- and sex-matched normative data and standard (z) scores were calculated. RESULTS: Sixteen patients met the inclusion criteria. Syndromic diagnosis was documented in five of 16 patients. Average age at the last postoperative evaluation was 8.9 ± 3.8 years (range, 4 to 16 years). Mean frontal width z-scores decreased postoperatively from 0.82 to -0.32 (p = 0.007), indicating diminished growth in this dimension. The last measured frontal width strongly correlated with the breadth of the bandeau after surgical correction but not with preoperative values. Postoperative mean cranial width diminished significantly to a more normal value. Mean intercanthal distance was normal preoperatively and remained so but was significantly greater in syndromic than in nonsyndromic cases. CONCLUSIONS: Frontal growth rate is diminished in the coronal plane after fronto-orbital advancement. The authors recommend primary techniques to overcorrect the width of the bandeau and frontal region, including zygomaticosphenoid osteotomies and interpositional cranial bone grafts to advance/widen the lateral orbital rim. Continued evaluation is required to assess whether overcorrection results in normal frontotemporal shape and breadth at skeletal maturity.

Management of Craniosynostosis.

Many procedures exist for treatment of craniosynostosis. The goal of all surgical interventions is to correct the skull deformities associated with the synostosis and to prevent the sequela of elevated intracranial pressure. Open cranial vault reconstructions address these issues at the time of surgery, but have the potential for increased blood loss and longer hospital stays. Minimally invasive procedures have shorter operative times and decreased blood loss, but rely on the cranial abnormality to improve over time with helmets or distraction devices. Prolonged follow-up is necessary to monitor long-term cosmetic outcomes and evaluate for signs of increasing intracranial pressure.

Classification and Management of Metopic Craniosynostosis.

BACKGROUND: In the craniofacial surgery literature, there is a wide disparity of opinions regarding the management of nonsyndromic metopic synostosis. With the lack of level I evidence to support a particular regimen, we aimed to elucidate the current state of practice among craniofacial surgeons with the hope of establishing a standard of care. METHODS: A survey was sent to 102 craniofacial surgeons. The survey featured 2 parts: clinical scenarios and questions regarding the following: primary indication for surgery, preference of timing, and choice of operative intervention for patients presenting with nonsyndromic isolated metopic synostosis. Surgeons were also queried regarding preoperative, intraoperative, and postoperative protocols. RESULTS: The total response rate was 72% (73/102) for the clinical scenarios and 63% (64/102) for the complete survey. There was a large discrepancy when classifying and managing mild metopic synostosis, with between 16% and 35% of surgeons electing to operate on a mild case. All surgeons agreed to operate on moderate and severe cases. For 95% of respondents, skull deformity was the primary indication for treatment of craniosynostosis. Open surgical management was most commonly performed at 6 months (29%) of age. Open frontal orbital advancement was the most commonly performed procedure in mild (27%), moderate (77%), and severe (89%) cases. Endoscopic approaches were more likely to be used in milder cases by 19% of surgeons. CONCLUSION: Our survey demonstrates that there is a wide disparity of opinion among craniofacial surgeons regarding the diagnosis and management of mild nonsyndromic metopic synostosis. LEVEL OF EVIDENCE: Diagnostic, level 5.

Y-craniosynostosis by premature fusion of the metopic and coronal sutures: a new nosological entity or a variety of Saethre-Chotzen syndrome?

BACKGROUND: New forms and varieties of craniosynostoses are continuously identified due to the current increased interest of clinicians and genetists especially since the introduction of microarray-based comparative genomic hybridization (Array-CGH) techniques in the diagnostic setting of patients with craniofacial anomalies. METHODS: In this report, we describe the case of an infant who associated the early fusion of the metopic and both the coronal sutures. The interaction of the early fusion of the anterior group of the main cranial sutures gave the infant a particular clinical phenotypes with a Y configuration of the frontal bone and a globally reduced size of the skull. Such a deformity was observed in utero and was subsequently confirmed by the postnatal imaging of the head. RESULTS: This phenotype was never described previously in antenatal period to our knowledge. The array-CGH showed a heterozygous 9.0 Mb deletion in the chromosomal region 7p21.1p21.3 encompassing approximately 25 other genes, spanning from THSD7A to TWIST1/FERD3L. CONCLUSION: This case further illustrates the variability of the clinical spectrum of craniofacial disorders associated with TWIST1 abnormalities. It is important to note that the Saethre-Chotzen syndrome caused by microdeletion is generally characterized by a mental disability. However, of interest, the postoperative psychomotor development of the child considered hereby was within the normal limits.

Craniostenosis: a neurosurgeon's perspective.

In pediatric neurosurgery departments in India, craniosynostosis is being increasingly identified and dealt with during the past several years. The management of this problem is well established in units that have a strong pediatric bias, whereas it is still in infancy in certain departments. Some misconceptions exist regarding this condition with reference to clinical, genetic aspects and management-in particular, the surgical indications. The experience gained for more than 2 decades of treating this condition as well as the problems faced in the management of this condition will be discussed. Although the terms craniostenosis and craniosynostosis do not mean quite the same thing, the terms are used interchangeably and will be done so in this communication.

Surgical correction of craniosynostosis. A review of 100 cases.

PURPOSE: Provide outcome data for open cranial vault reshaping at a single institution by a single craniofacial surgeon treating 100 patients. METHODS AND SUBJECTS: A total of 100 patient records were reviewed. Criteria for selection included patients less than three years of age undergoing primary surgery with open cranial vault reshaping and a minimum follow up time of 2 years. RESULTS: Of the 100 patients (27 female, 73 male) treated 6 were syndromic and 94 nonsyndromic. Average age and weight were 8.9 months and 9.51 kg, respectively. The oldest child was 30 months and the youngest 5 months at the time of surgery. The estimated blood volume lost was 42.7% of total calculated blood volume ranging from 16.6% to 336%. Average surgical time was 216.7 min. Complications included 2 hematomas, 2 wound infections, 1 subgaleal abscess, 6 dural tears, 3 patients requiring reoperation for residual deformity, 4 cases requiring coronal scar revision, 1 sagittal sinus bleed, and 1 intraoperative death. CONCLUSIONS: Our review of 100 open repairs of patients with craniosynostosis demonstrates good long-term results with an overall low complication rate. The outcome data will assist in developing future prospective studies aimed at improving the multidisciplinary care of these patients.

Cerebral oxygenation and hemodynamic measurements during craniosynostosis surgery with near-infrared spectroscopy.

INTRODUCTION: Focal pressure-related changes in brain perfusion and metabolism are discussed in single-suture craniosynostosis and brachycephalic cases (bicoronal synostosis). Raised intracranial pressure levels could be measured in some cases. In order to find possible loco-regional brain tissue changes during plastic surgery, we investigated oxygenation and perfusion parameters using non-invasive near-infrared spectroscopy (NIRS) probes. METHODS: Twenty-two consecutively operated cases (mean age 7 months) with single-suture craniosynostosis were prospectively investigated using a NIRS probe (LEA(©), O2C, white light 500-800 nm, laser NIR). Measurements for oxygen saturation (SO(2)), relative quantity of hemoglobin (rHb), blood flow, and blood flow velocity of the bilateral frontal, temporal, and parietal cortices were taken transosseously (prior to decompression) and epidurally directly after decompression as well as 15 and 30 min after decompression and before closure. RESULTS: Twenty-two patients with scaphocephaly (11), trigonocephaly (6), anterior plagiocephaly (3), and brachycephaly (2) were investigated. SO(2) was improving in all patient subgroups, showing the highest levels in the fronto-temporal region; rHb improved in scaphocephalic, trigonocephalic, and brachycephalic children. Again, the highest values were found not only in the temporal but also in the frontal region and in brachycephalic patients also in the parietal cortex. CONCLUSION: These preliminary results of a new technology for brain tissue oxygenation and blood flow measurements suggest a regional compromise of cortical metabolism and circulation in patients with craniosynostosis.

An evaluation of complications, revisions, and long-term aesthetic outcomes in nonsyndromic metopic craniosynostosis.

BACKGROUND: The authors evaluated the complications, revisions, and long-term aesthetic outcomes of patients with isolated metopic synostosis. METHODS: A retrospective chart review was performed on consecutive metopic craniosynostosis patients treated from June of 1987 to June of 2012 at The Children's Hospital of Philadelphia. Patient demographics, operative details, and postoperative data were collected. Outcomes were reported as Whitaker classification and postoperative clinical characteristics assessed before additional interventions. Reoperation in patients with greater than 5 years of follow-up was noted. Appropriate statistical analyses were applied. RESULTS: From 1987 to 2012, 178 patients underwent surgical correction of isolated metopic craniosynostosis, and 147 met inclusion criteria. Average age at surgery was 0.83 year (range, 0.3 to 4.7 years); average follow-up was 5.8 years (range, 1.0 to 17.8 years). There were 13 surgical complications (8.8 percent), three major (2.0 percent), and 10 minor (6.8 percent). At follow-up, 67 patients (56.8 percent) were classified as Whitaker class I, six (5.1 percent) as class II, 43 (36.4 percent) as class III, and two (1.7 percent) as class IV. Patients with greater than 5 years' follow-up (n = 57) were more likely to have temporal hollowing (OR, 2.9; 95 percent CI, 1.2 to 7.3; p = 0.021), lateral orbital retrusion (OR, 4.9; 95 percent CI, 1.9 to 12.7; p = 0.001), and Whitaker class III or IV classification (OR, 4.0; 95 percent CI, 1.5 to 10.6; p = 0.006) compared with those with less than 5 years' follow-up. CONCLUSION: This study reports low complication and reoperation rates in the treatment of isolated metopic craniosynostosis, but demonstrates a clear trend toward worsening aesthetic outcomes over time. CLINICAL QUESTION/LEVEL OF EVIDENCE: Therapeutic, IV.

Percentile-based assessment of craniosynostosis.

Perioperative assessment of craniosynostosis is based mostly on subjective scores. In this study, we sought to find an objective method to assess cranial deformation based on normative craniofacial percentiles. Anthropometric datasets from 104 (79 males, 25 females) patients with craniosynostoses were included. Anthropometric data were compared with normative age-dependent percentiles. Deviations above the 90th or below the 10th percentile were defined as significant cranial deformation. The cohort comprised 69 children with sagittal, 22 metopic, nine coronal, two bicoronal, one lambdoid, and one with coronal + lambdoid craniosynostosis. Most children with sagittal synostosis were above the 90th percentile for cranial circumference and length, whereas only 27.9% were below the 10th percentile for cranial width. Most (83%) children with scaphocephaly had cranial indices below the 10th percentile. For trigonocephaly, we found normal cranial circumference values in most patients (10th-90th percentile), 40.9% were above the 90th percentile for cranial length, and 63.1% and 57.9% were above the 90th percentiles for sagittal and transverse circumferences. For unicoronal synostosis transverse circumference was above the 90th percentile in 83.3% of children. Matching of anthropometric data of craniosynostosis patients with craniofacial norms could be useful in grading the clinical picture and potentially adapting the operative procedure.

Spectrum of positional deformities - is there a real difference between plagiocephaly and brachycephaly?

AIM: This study analyses pathomorphological and physiological head shapes and classifies the pathomorphology in positional plagiocephaly and brachycephaly. PATIENTS AND METHODS: 78 infants with a positional plagiocephaly (5.99 months) and 32 infants with a positional brachycephaly (6.53 months) with a Cephalic index > 94% were investigated in this study and compared to a matched control group of 35 infants. The head shapes were analysed by stereophotogrammetry 3D data. RESULTS: The cephalic index, the total width, and coronal circumference were the highest values in patients with brachycephaly and the lowest values in the control group. The asymmetry of the head showed that the diagonal difference in brachycephalic patients more than doubled, and in patients with plagiocephaly almost tripled compared to the controls. A significantly higher total volume and vertex height was found for the patients with plagiocephaly and the patients with brachycephaly compared to the controls. CONCLUSION: The cephalic index is a valuable and reliable parameter in order to differentiate positional deformities from unaffected skulls. Pathomorphology of a plagiocephaly is associated with the most severe asymmetry of the head. Plagiocephaly and brachycephaly overlap in several criteria. Therefore it seems justified to speak of a continuum rather than to differentiate between plagiocephaly and brachycephaly.

Surgical timing of craniosynostosis: what to do and when.

Craniosynostosis, both isolated and syndromic, are challenging malformations for the craniofacial team. They present the team with an articulated cascade of choices, which need to be addressed early in life and in the growing age to intercept, remove, or correct the direct and indirect consequences of the malformation. Timing of treatment is thus critical and it stands on the experience of a multi-specialty trained craniofacial team. In this paper the authors discuss the timing of treatment of the major craniosynostosis, isolated and syndromic, reviewing the options for treatment and their experience in this complex field.