Long-term results of minimally invasive strip craniectomy without helmet therapy for scaphocephaly - a single-centre experience.

Scaphocephaly is the most common type of craniosynostosis and various surgical techniques are used for treatment. Due to late postoperative changes of the head shape, long-term outcome data is important for evaluating any new surgical technique. At our institution, minimally invasive strip craniectomy without regular helmet therapy is the standard treatment in scaphocephalic patients. Between October 2021 and February 2023, we retrospectively examined the skull shape of patients who underwent minimally invasive strip craniectomy for scaphocephaly using a 3D surface scan technique. The cephalic index (CI), the need for helmet therapy and additional cosmetic outcome parameters were investigated. We included 70 patients (72.5% male). The mean follow-up time was 46 (10-125) months and the mean CI was 75.7 (66.7-85.2). In 58 patients, the final cosmetic result was rated as "excellent/good" (mean CI: 76.3; 70.4-85.0), in 11 as "intermediate" (mean CI: 73.3; 66.7-77.6), and in one case as "unsatisfactory" (CI 69.3). The presence of a suboccipital protrusion was associated with a "less than good" outcome. The CI correlated significantly with the overall outcome, the presence of frontal bossing, and the interval between scan and surgery (age at scan). Minimally invasive strip craniectomy is an elegant and safe method to correct scaphocephaly. Our data show good cosmetic results in the long term even without regular postoperative helmet therapy.

Skeletal expansion via craniofacial distraction osteogenesis technique in syndromic craniosynostosis: impact on ophthalmic parameters.

BACKGROUND: This study aims to compare the changes in ophthalmic parameters among syndromic craniosynostosis patients who underwent craniofacial skeletal expansion procedures via distraction osteogenesis (DO). METHOD: A retrospective study was conducted involving syndromic craniosynostosis patients who underwent surgical expansion via the DO technique from the year 2012 to March 2022. Changes in six parameters which consist of visual acuity, refractive error, optic disc health, intraocular pressure, degree of proptosis and orbital volume were measured objectively pre and post-surgery. For categorical parameters, the Chi-square cross-tab test was done. Paired sample T-test was used for normally distributed variables. Wilcoxon signed-rank test was used for non-normally distributed data. RESULTS: Visual impairment was present in 21.4% of eyes before surgery and increased to 28.5% post-surgery. Three patients had changes of refractive error post-surgery with one developed hypermetropia, another developed anisometropia and the last had improvement to no refractive error. Two patients had optic disc swelling which was resolved post-surgery. Intraocular pressure changes were inconsistent post-surgery. All patients achieved a significant reduction in the degree of proptosis post-surgery. Orbital volume calculation using computed tomography (CT) scans shows a significant increase in volume post-surgery for all patients. CONCLUSION: Our study shows a significant increase in orbital volume post-surgery with a reduction in the degree of proptosis. Optic disc and nerve health improved after the surgery. Changes in terms of visual acuity, refractive error and IOP were inconsistent after the surgical intervention.

A novel case of Horner syndrome as the presenting sign of craniosynostosis.

Craniosynostosis, the premature fusion of cranial sutures, can lead to distortion of skull shape and neurological dysfunction. We present a novel case of Horner syndrome as the presenting sign of craniosynostosis associated with elevated intracranial pressure. A 10-year-old boy presenting for strabismus follow-up was noted to have new-onset anisocoria, greater in the dark, and mild right upper eyelid ptosis. Apraclonidine testing was concerning for Horner syndrome. Neuroimaging demonstrated previously undiagnosed sagittal craniosynostosis with tortuous optic nerves and large cerebrospinal fluid spaces around both optic nerves. The patient was referred to neurosurgery and underwent a lumbar puncture with an opening pressure of 44 cm H2O. He underwent surgical cranial expansion. By six months postoperatively, his anisocoria had resolved.

[Prospective analysis of inflammatory markers and perioperative clinical data in children with craniosynostosis undergoing reconstructive surgery]. / Prospektivnyi analiz dinamiki laboratornykh markerov vospaleniya i klinicheskoi kartiny perioperatsionnogo perioda pri rekonstruktivnykh operatsiyakh u detei s kraniosinostozami.

BACKGROUND: Craniosynostosis (CS) is a group of skull malformations manifested by congenital absence or premature closure of cranial sutures. Reconstructive surgery in the second half of life is traditional approach for CS. The issues of surgical stress response after reconstructive surgery for CS in children are still unclear. OBJECTIVE: To evaluate clinical and laboratory parameters in children undergoing traumatic reconstructive surgery for CS. MATERIAL AND METHODS: Inclusion criteria were CS, reconstructive surgery, age <24 months, no comorbidities and available laboratory diagnostic protocol including complete blood count, biochemical blood test with analysis of C-reactive protein, procalcitonin, ferritin and presepsin. The study included 32 patients (24 (75%) boys and 8 (25%) girls) aged 10.29±4.99 months after surgery between October 2021 and June 2022. Non-syndromic and syndromic forms of CS were observed in 25 (78.1%) and 7 (21.9%) cases, respectively. RESULTS: There were no infectious complications. We analyzed postoperative clinical data, fever, clinical and biochemical markers of inflammation. CONCLUSION: Early postoperative period after reconstructive surgery for CS in children is accompanied by significant increase of inflammatory markers (C-reactive protein, procalcitonin, ferritin). However, these findings do not indicate infectious complications. This is a manifestation of nonspecific systemic reaction. Severity of systemic inflammatory response syndrome with increase in acute phase proteins indicates highly traumatic reconstructive surgery for CS in children. Analysis of serum presepsin allows for differential diagnosis between infectious complication and uncomplicated course of early postoperative period.

A retrospective observational cohort study on the postoperative respiratory complications and their risk factors in brachycephalic dogs undergoing BOAS surgery: 199 cases (2019-2021).

OBJECTIVES: To observe the occurrence of postanaesthetic respiratory complications and to determine their prevalence and risk factors in dogs undergoing brachycephalic obstructive airway syndrome surgery. MATERIALS AND METHODS: Data from 199 clinical records were retrospectively analysed. Univariable logistic regression followed by multivariable logistic regression was used to identify associations between the dependent variables (set as the postoperative respiratory complications observed in the study dogs) and various independent covariates. The quality of model-fit was assessed using the likelihood ratio test. P≤0.05 was considered statistically significant. RESULTS: Four postoperative respiratory complications were observed: hypoxaemia (n=10/199; 5%), dyspnoea requiring tracheal re-intubation (n=13/199, 7%), dyspnoea requiring tracheostomy (n=10/199, 5%) and aspiration pneumonia (n=12/199, 6%). Univariable logistic regression showed an association between postoperative aspiration pneumonia and increasing body condition score and American Society of Anaesthesiology classification; however, when these covariates were evaluated in the multivariable model significance was not maintained. Risk factors for tracheostomy were preoperative and postoperative aspiration pneumonia (odds ratio: 9.52, 95% confidence interval: 1.56 to 57.93) and increasing brachycephalic obstructive airway syndrome grade (odds ratio: 4.65, 95% confidence interval: 0.79 to 27.50). CLINICAL SIGNIFICANCE: High brachycephalic obstructive airway syndrome grade and aspiration pneumonia, either developing peri-operatively or as pre-existing condition, may represent risk factors for postoperative tracheostomy. Preoperative diagnosis of aspiration pneumonia may further increase the risk of postoperative complications.

Changes in mandibular position during midface distraction in patients with syndromic craniosynostosis.

The purpose of this study was to evaluate changes in mandibular position during midface distraction. Midface distraction was performed in patients with syndromic craniosynostosis to increase upper airway volume. Although this treatment resulted in changes in occlusion, the concomitant changes in mandibular position were poorly understood. In this retrospective study, three-dimensional (3D) cephalograms were obtained before and after midface distraction in 15 patients with syndromic craniosynostosis. Perioperative polysomnography scores and changes in maxillary and mandibular position, mandibular volume, and upper airway volume were analyzed. Results showed a significant improvement in apnea-hypopnea index (AHI) (from 20.6 ± 21.3 to 6.9 ± 5.1, p < 0.05) and upper airway volume (from 2951.65 ± 2286.38 to 5218.04 ± 3150.05 mm3, p < 0.001). When the lowest point of the sella turcica was set as the reference point, the mandible moved significantly in an anterior direction (from 47.9 ± 11.5 to 51.9 ± 9.8 mm, p < 0.05). Mandibular volume did not change significantly perioperatively (from 32530.19 ± 10726.01 to 35590.50 ± 14879.21 mm3, p = 0.10). There were positive correlations between the rates of improvement in AHI and the amount of mandibular movement in the anterior and inferior directions (both p < 0.05). Within the limitations of the study, it seems that the mandible moved in the anterior-inferior direction after midface distraction, and the amount of movement correlated with improvement in respiratory function. Therefore, it is important to consider the position of the mandible when determining the direction of midface distraction, as it may influence the therapeutic effect.

Association of cranial base suture/synchondrosis fusion with severity of increased intracranial pressure in Crouzon syndrome.

This study investigated how the fusion states of the cranial base is related to the degree of increased intracranial pressure (ICP) in patients with Crouzon syndrome. This retrospective cohort study enrolled patients who were diagnosed with Crouzon syndrome between May 2007 and April 2022. We categorized the patients into three groups: A, B, and C, according to the severity of increased ICP and the number of cranial vault remodeling procedures for corrective operation. The preoperative fusion states of the cranial base sutures/synchondroses were examined using facial bone computed tomography and compared between groups. Overall, 22 patients were included in Groups A, B, and C, including 8, 7, and 7 patients, respectively. The preoperative average grades of the total cranial base suture/synchondrosis fusion appeared to significantly increase with severity, except for the frontoethmoidal suture, which showed the opposite tendency. In the subgroup analysis, frontosphenoidal, sphenoparietal, sphenosquamosal, parietomastoid, and occipitomastoid suture and petro-occipital synchondrosis were associated with earlier fusion in the more severe group. Premature closure of the cranial base sutures/synchodroses seems to be associated with increased ICP severity in patients with Crouzon syndrome. Precise evaluation of minor sutures/synchondroses at the first visit might help build subsequent operative plans and predict disease prognosis.

Nasal monobloc osteotomy for correction of late nasal and orbital asymmetry of unicoronal synostosis: A morphometric and outcomes study.

BACKGROUND: Craniofacial asymmetry associated with unicoronal synostosis (UCS) may persist into the teenage years despite surgery in infancy. This study evaluated outcomes following a nasal monobloc procedure by mobilizing a united nasomaxillary and bilateral medial orbital segment of bone (nasal monobloc) to perform corrective translational and rotational movement for secondary correction of residual nasal-orbital asymmetry associated with UCS. METHODS: A retrospective review of all UCS patients treated with nasal monobloc at our institution was performed. Demographic information was recorded, and pre- and postoperative 2D imaging was used for morphometric outcome analysis. Outcomes and complications were tabulated. RESULTS: The study included 14 patients (5 males, 9 females; mean age 14.6 years; range 9.6 to 22.5 years; mean follow-up 70.6 months range 12 to 132 months). Ancillary procedures (scar revision, forehead/orbital contouring, MEDPOR® augmentation) were performed in all patients at the time of the nasal monobloc. One patient underwent a repeat procedure 6 years later following technique modification. Additionally, another patient experienced late overgrowth of the frontal sinus with forehead asymmetry. The morphometric analysis demonstrated significant (p < 0.05) pre-op to post-op improvements in naso-orbital asymmetry, as demonstrated by horizontal orbital aperture ratio (0.88 vs 0.99), midline to exocanthion ratio (0.91 vs 0.98), orbital index ratio (1.15 vs 1.01), and midline discrepancy (7.1 degrees vs 2.7 degrees). CONCLUSION: Nasal monobloc osteotomy provides a reasonable surgical treatment to improve both the nasal and orbital asymmetries associated with unicoronal synostosis, including frontal nasal deviation, basal nasal deviation, and orbital aperture asymmetry. It is important to note that confounding anatomic variables such as globe dystopia, strabismus, and scleral show may affect the perception of orbital symmetry.

Multiscale mechanical characterisation of the craniofacial system under external forces.

Premature fusion of craniofacial joints, i.e. sutures, is a major clinical condition. This condition affects children and often requires numerous invasive surgeries to correct. Minimally invasive external loading of the skull has shown some success in achieving therapeutic effects in a mouse model of this condition, promising a new non-invasive treatment approach. However, our fundamental understanding of the level of deformation that such loading has induced across the sutures, leading to the effects observed is severely limited, yet crucial for its scalability. We carried out a series of multiscale characterisations of the loading effects on normal and craniosynostotic mice, in a series of in vivo and ex vivo studies. This involved developing a custom loading setup as well as software for its control and a novel in situ CT strain estimation approach following the principles of digital volume correlation. Our findings highlight that this treatment may disrupt bone formation across the sutures through plastic deformation of the treated suture. The level of permanent deformations observed across the coronal suture after loading corresponded well with the apparent strain that was estimated. This work provides invaluable insight into the level of mechanical forces that may prevent early fusion of cranial joints during the minimally invasive treatment cycle and will help the clinical translation of the treatment approach to humans.

What can a morphometric study of unoperated children teach us about the natural history of metopic synostosis?

OBJECTIVE: Outcomes of surgical repair of trigonocephaly are well reported in the literature, but there is a paucity of information on the natural history of unoperated children. The authors evaluated a group of unoperated children with metopic synostosis to describe the natural change in head shape over time. METHODS: A database was screened for scans of children with unoperated trigonocephaly (2010-2021). Multisuture cases and those with a metopic ridge were excluded. Three-dimensional surface scans (3D stereophotogrammetry/CT) were used for morphological analysis. Nine previously published parameters were used: frontal angle (FA30°), anteroposterior (AP) volume ratio (APVR), AP area ratio (APAR), AP width ratios 1 and 2 (APWR1 and APWR2), and 4 AP diagonal ratios (30° right APDR [rAPDR30], 30° left APDR [lAPDR30], 60° right APDR [rAPDR60], and 60° left APDR [lAPDR60]). RESULTS: Ninety-seven scans were identified from a cohort of 316 patients with a single metopic suture, in which the male-to-female ratio was 2.7:1. Ages at the time of the scan ranged from 9 days to 11 years and were stratified into 4 groups: group 1, < 6 months; group 2, 6-12 months; group 3, 1-3 years; and group 4, > 3 years. Significant improvements were detected in 5 parameters (APVR, APAR, APWR1, rAPDR30, and lAPDR30) over time, whereas no significant differences were found in FA30, APWR2, rAPDR60, and lAPDR60 between age groups. CONCLUSIONS: Forehead shape (surface area and volume), as well as narrowing and anterolateral contour at the frontal points, differed significantly over time without surgery. However, forehead angulation, narrowing, and anterolateral contour at temporal points did not show significant differences. This knowledge will aid in surgical and parental decision-making.

A diffusion tensor imaging comparison of white matter development in nonsyndromic craniosynostosis to neurotypical infants.

PURPOSE: Nonsyndromic craniosynostosis (NSC) is associated with neurocognitive deficits, and intervention at infancy is standard of care to limit the negative effects of NSC on brain development. In this study, diffusion tensor imaging (DTI) was implemented to investigate white matter microstructure in infants with NSC undergoing cranial vault remodeling, and a comparison was made with white matter development in neurotypical controls. METHODS: Infants presenting with NSC (n = 12) underwent DTI scans before and after cranial vault remodeling. Neurotypical infants (n = 5), age matched to NSC patients at preoperative scans, were compared to preoperative DTI scans. Pre- and postoperative NSC scans were compared in aggregate, and the sagittal synostosis (n = 8) patients were evaluated separately. Finally, neurotypical infants from the University of North Carolina/University of New Mexico Baby Connectome Project (BCP), who underwent DTI scans at timepoints matching the NSC pre- and postoperative DTI scans, were analyzed (n = 9). Trends over the same time period were compared between NSC and BCP scans. RESULTS: No significant differences were found between preoperative NSC scans and controls. White matter development was more limited in NSC patients than in BCP patients, with microstructural parameters of the corpus body and genu and inferior and superior longitudinal fasciculi consistently lagging behind developmental changes observed in healthy patients. CONCLUSION: Infant white matter development appears more limited in NSC patients undergoing cranial vault remodeling relative to that in neurotypical controls. Further investigation is needed to explore these differences and the specific effects of early surgical intervention.

Evaluation of neurocognitive and social developments after craniosynostosis surgery.

OBJECTIVE: The study focused on assessing the potential neurocognitive and social developmental issues in children with non-syndromic craniosynostosis (NSC) who received optimal surgical treatment. The primary objective was to determine whether NSC, even after optimal surgical treatment, could have negative effects on brain development. METHODS: The study included a total of 73 pediatric patients aged between 2 and 6 years who had previously undergone surgery for NSC at the Gazi University Faculty of Medicine, Department of Neurosurgery. These patients were carefully matched with 107 healthy children who visited the outpatient clinic of the same department in terms of sociodemographic characteristics such as age, gender, and social status. To assess the neurocognitive and social development of the participants, the child psychologist administered a developmental scale to the child and his/her family via video conference. This scale was adapted from the Bayley-III Infant and Child Development Scale by the Gazi University Faculty of Medicine, Division of Pediatric Neurology. RESULTS: The study found no social or gross motor developmental issues in patients who had undergone optimal surgical treatment for NSC. However, the risk of fine motor developmental deficiencies was 4.79 times higher than that of the normal population, and the risk of language developmental deficiencies was 5.75 times higher than that of the normal population. CONCLUSIONS: Despite timely treatment of NSC, long-term neurocognitive and social development issues may arise in affected children. Therefore, it is crucial to monitor these patients after completing surgical treatment and thoroughly examine their development using a multidisciplinary approach.

QEEG findings in nonsyndromic sagittal craniosynostosis.

Despite the undertaken treatment, children with nonsyndromic sagittal craniosynostosis (NSC) are burdened with problems with speech development, visuospatial and other cognitive deficits. The electroencephalographic assessment has not influenced the diagnostics and treatment strategy of craniosynostosis so far but the introduction of quantitative EEG (QEEG) protocols renewed an interest in the functional aspect of this disease. In this study we retrospectively assessed the QEEG records of 25 children with NSC aged 1-18 months (mean age 9.62 months) before and after surgery. In each case, the amplitude, interhemispheric (ICoh) and intrahemispheric (HCoh) coherence indices were calculated. Obtained data were compared to age-matched control group of 25 normocephalic children. Children with NSC presented significantly lower values of amplitudes and intrahemispheric coherence in occipital, posterior parietal and posterior temporal regions than normocephalic children. The values of amplitudes, ICoh and HCoh in pre- and postoperative QEEG records mostly remained unchanged, with a slight improvement in HCoh in centro-parietal area. These findings suggest that NSC children present their own QEEG profile. The operative treatment improves an intrahemispheric connectivity, but there still exists a significant difference in the occipitotemporal, frontotemporal and centro-frontal areas, which may be considered as a functional substrate of reported speech and neurocognitive problems. QEEG findings in nonsyndromic sagittal craniosynostosis.

Hemi one-piece distraction osteogenesis for unilateral coronal craniosynostosis.

INTRODUCTION: It was the aim of the study to assess the efficacy of the hemi one-piece distraction osteogenesis and to compare it to the traditional one-piece distraction osteogenesis technique. METHODS: Two different surgical techniques were used; the one-piece distraction and the hemi one-piece distraction. The principal distinction between the two techniques is that in the hemi style approach, the intact sutures on the contralateral side were left undisturbed, with no osteotomy performed. RESULTS: The hemi one-piece group had a significantly lower median value of plastic surgery time, total operation time, and transfusion rate (plastic surgery time 69 min (range 65-120) vs. 20 min (range 17-32.5), p < 0.001; transfusion 80 mL (range 0-150) vs. 0 mL (0-60), p = 0.1. Nasofrontal advancement was successful with no major complications. Median endocranial angulation improved (one-piece: 166.1°-176.0°, hemi: 162.9°-173.0°, p = 0.023 & p = 0.012 respectively). CONCLUSION: This study reveals less invasive, highly effective techniques for craniosynostosis treatment, notably a unilateral osteotomy with distraction method. Nevertheless, to confirm their long-term efficacy and durability, more studies with longer follow-ups are essential.

The Craniofacial Collaboration UK: Developmental Outcomes in 7- and 10-Year-Old Children With Metopic Synostosis.

The Craniofacial Collaboration (CC-UK) is a shared initiative across the Psychology teams attached to 4 highly specialized craniofacial centers in the United Kingdom. The CC-UK aims to address key limitations in the existing craniofacial literature by analyzing data for homogenous samples of children with craniosynostosis. This article presents the fifth wave of CC-UK data collection, focused on 7- and 10-year olds who have undergone primary corrective surgery for metopic synostosis (MS). Data for children with sagittal synostosis and MS have previously been presented at 3 and 5 years. This paper continues to build on this with consideration to older age groups, presenting the first CC-UK analysis of cognitive assessment data using the Wechsler Abbreviated Scale of Intelligence-Second Edition. Results show that the majority of children with MS fall within the average ranges across behavioral and neurodevelopmental domains. However, several domains indicated a trend of heightened concern when compared with normative data, particularly for parent-reported outcomes, suggesting that there may be some subtle difficulties for children with MS. Consideration of how these findings compare with that of previous CC-UK analyses is explored. Further, implications for clinical practice and future research are considered, with the need for longitudinal analyses, as well as data from multiple perspectives (eg, school, parents, and self) at older age points to establish patterns over time. Through collaboration across the highly specialized craniofacial centers, the CC-UK hopes to work toward this goal moving forward.

Endoscopy-Assisted Craniosynostosis Surgery Versus Cranial Vault Remodeling for Non-Syndromic Craniosynostosis: Experience of a Single Center.

AIM: To evaluate and compare open cranial vault remodeling (OCVR) and endoscopy-assisted craniosynostosis surgery (EACS) in patients with non-syndromic craniosynostosis and to develop an algorithm to determine the most appropriate surgery for each patient. MATERIAL AND METHODS: Eighty-five children with craniosynostosis who underwent surgery between 2010 and 2022 were retrospectively analyzed. Demographic data, comorbidities, and peri-operative findings of the patients were recorded. Pre- and post-operative comparisons were made between predetermined measurement techniques for each deformation. In addition, measurements were obtained by computed tomography (CT) or 3D stereophotogrammetric (3DSPG) methods from eligible patients and compared with one another. RESULTS: In our study, 61 patients underwent EACS, whereas 24 underwent OCVR. The operating time of OCVR was approximately 54.4 minutes longer than that of EACS (p < 0.001). The intra-operative blood loss was around 139 ml higher in OCVR (p < 0.001). The length of hospital stay for patients who underwent EACS was shorter at 8.4 days on average (p < 0.001). Surprisingly, 5 complications were observed in OCVR compared with 7 in EACS. While the cosmetic outcome of EACS was superior in most of the pathologyspecific measurement techniques, the metopic index increased only in patients with metopic synostosis after both surgical operations. Still, this increase was lower in EACS than in OCVR. CONCLUSION: This study suggests that endoscopic craniosynostosis surgery has lower estimated blood loss and operation and hospitalization times, as well as comparable cosmetic results compared with open vault surgeries on long-term follow-up. CT and 3DSPG methods can help distinguish between different types of measurement techniques for synostoses. However, no significant differences were found in the comparisons since 3DSPG can also provide reliable measurements comparable to those on CT during follow-up.