A Transfusion Regimen With Same-donor Packed Red Blood Cells Reduces Exposure to Multiple Blood Donors in Craniosynostosis Surgery.

In major craniosynostosis surgery with moderate to severe blood loss, patients may be exposed to multiple donors. We have previously reported a method for reducing donor exposure using mixed pediatric units including plasma. To further reduce donor exposure, we used plasma-free divided pediatric units. The study aimed to investigate the feasibility of the new strategy for reducing donor exposure. This prospective observational study recruited children younger than 1 year who were scheduled for nonsyndromic craniosynostosis surgery. One adult red blood cell unit was divided into 4 equal units on the day before the operation for use intra- or postoperatively. Number of donor exposures, estimated blood loss, crystalloid, colloid, and blood product volumes, and coagulation parameters were evaluated. Nineteen infants were included. The mean estimated blood loss was 19 (3) mL/kg and the transfusion volume was 17 (7) mL/kg. The median donor exposure per patient was 1 (range, 1-3). During surgery, all infants received at least one DPU. Two infants received transfusions from more than one donor during the intraoperative period. In the first 24 hours postoperatively, 14 infants received transfusion; 10 received only DPUs, whereas 4 received from multiple donors. In all, multiple donor exposure was prevented in 14 of 19 infants. Postoperative Pk-INR was 1.33 (0.16); no plasma or platelets were transfused. The plasma-free DPU transfusion protocol may be useful to reduce donor exposure in open craniosynostosis surgery in infants.

Parental satisfaction with hospital care for children with non-syndromic craniosynostosis: A mixed-method study.

PURPOSE: The study aims to investigate factors influencing parents' satisfaction with hospital care for children with craniosynostosis during hospitalization for surgery. DESIGN AND METHODS: A mixed-methods study with a convergent, parallel design was used. Ninety-five parents responded to the Swedish Pyramid Questionnaire for Treatment, a 25-item questionnaire with six quality domains. In addition, 20 parents were interviewed about their experiences. Frequencies were calculated, and content analysis was used to analyze free-text comments and transcribed interviews. RESULTS: Parents' assessment of the overall quality of care was high (mean 87%, range 10-100%). They were most satisfied in the domain staff attitudes and less satisfied with information routines and participation. Content analysis of the interviews gave two overarching themes: Factors that parents experienced as facilitating good quality of care and Factors that parents experienced as impeding good quality of care. CONCLUSIONS: Parents were generally satisfied with the care provided, and interviews captured parents´ views on important factors. Staff attitudes affected parents' perception of quality of care. PRACTICAL IMPLICATIONS: Clear information and dialogue as well as making parents feel they are part of their child's team can result in higher satisfaction, and allowing families to stay together in the hospital can ease the hospitalization experience. Using a theoretical model can help in suggesting relevant caring actions based on parents' reported care experiences.

Strabismus and refraction in non-syndromic craniosynostosis - A longitudinal study up to 5 years of age.

PURPOSE: To evaluate the refractive outcome and strabismus at 5 years of age, in children operated for various types of non-syndromic craniosynostosis, and further analyse the refractive and strabismic development over time. METHODS: Eighty-nine children, who had undergone operations for non-syndromic craniosynostosis, were examined at 5 years of age. These children also underwent ophthalmological examination preoperatively and up to 1 year after the operation. An age-matched control group including 32 healthy children was also recruited. Strabismus and eye motility were registered. Refraction was measured in cycloplegia. RESULTS: There was a difference regarding the refractive outcome between the different types of craniosynostosis. Higher values of hypermetropia were found in the metopic craniosynostosis group on both eyes. In the unicoronal craniosynostosis group, high values of hypermetropia and a higher degree of astigmatism were found on the side contralateral to the craniosynostosis. Strabismus was found in 11/88 children of whom 10/11 had unicoronal craniosynostosis. A vertical deviation on the side ipsilateral to the fused suture was highly prevalent (6/10 cases). Ophthalmological dysfunctions were rare in children operated for sagittal craniosynostosis. CONCLUSION: Ocular manifestations such as strabismus, astigmatism and anisometropia were highly prevalent in children operated for unilateral coronal craniosynostosis. Children operated for metopic craniosynostosis had higher rates of hypermetropia. The screening and follow-up protocols need to be tailored with regard to the type of craniosynostosis.

Springs Produce Favorable Morphological Outcomes Relative to H-craniectomy According to a Two-center Comparison of Matched Cases.

BACKGROUND: Sagittal synostosis is the most common type of premature suture closure, and many surgical techniques are used to correct scaphocephalic skull shape. Given the rarity of direct comparisons of different surgical techniques for correcting craniosynostosis, this study compared outcomes of craniotomy combined with springs and H-craniectomy for non-syndromic sagittal synostosis. METHODS: Comparisons were performed using available pre- and postoperative imaging and follow-up data from the two craniofacial national referral centers in Sweden, which perform two different surgical techniques: craniotomy combined with springs (Gothenburg) and H-craniectomy (Renier's technique; Uppsala). The study included 23 pairs of patients matched for sex, preoperative cephalic index (CI), and age. CI, total intracranial volume (ICV), and partial ICV were measured before surgery and at 3 years of age, with volume measurements compared against those of pre- and postoperative controls. Perioperative data included operation time, blood loss, volume of transfused blood, and length of hospital stay. RESULTS: Craniotomy combined with springs resulted in less bleeding and lower transfusion rates than H-craniectomy. Although the spring technique requires two operations, the mean total operation time was similar for both methods. Of the three complications that occurred in the group treated with springs, two were spring-related. Importantly, the compiled analysis of changes in CI and partial volume distribution revealed that craniotomy combined with springs resulted in superior morphological correction. CONCLUSIONS: The findings showed that craniotomy combined with springs normalized cranial morphology to a greater extent than H-craniectomy based on changes in CI and total and partial ICVs over time.

Treatment of orbital fractures - a critical analysis of ophthalmic outcomes and scenarios for re-intervention.

BACKGROUND: Malplaced implants in orbital reconstruction may lead to serious complications and necessitate re-intervention. The aim of this study was to describe outcomes, complications and scenarios of re-intervention in a historical case series of orbital fractures treated with free-hand orbital wall reconstruction. The main hypothesis was that early re-interventions are mainly because of malplaced implants in the posterior orbit. METHODS: Retrospective review of 90 patients with facial fractures involving the orbit, reconstructed with radiopaque orbital wall implants, from 2011 to 2016. Data were obtained from medical records and computed tomography images. Recorded parameters were fracture type, ocular injury, ocular motility, diplopia, eye position, complications and re-interventions. Secondary reconstructions because of enophthalmos were volumetrically evaluated. RESULTS: Early complications requiring re-intervention within 1 month were seen in 12 (13%) patients, where all except two were because of malplaced implants. The implant incongruence was without exception found in the posterior orbit. Late complications consisted of four (4%) cases of ectropion and five (5%) cases of entropion that needed corrective surgery. The majority of the patients with eye-lid complications had undergone repeated surgeries. Secondary orbital surgeries were performed in nine (10%) patients. Five of these patients had secondary reconstruction for enophthalmos and associated diplopia. None of these patients became completely free from either enophthalmos or diplopia after the secondary surgery. CONCLUSION: Re-intervention after orbital reconstruction is mainly related to malplaced implants in the posterior orbit. Incomplete results in patients requiring secondary surgery for enophthalmos infer the importance of accurate restoration of the orbit at primary surgery. Abstract presented at: Swedish surgery Week 2021 and SCAPLAS 2022.

Longitudinal data on speech outcomes in internationally adopted children compared with non-adopted children with cleft lip and palate.

BACKGROUND: At the beginning of the 21st century, international adoptions of children with cleft lip and/or palate increased dramatically in Sweden. Many children arrived partially or totally unoperated, despite being at an age when palatoplasty has usually been performed. To date, the speech development of internationally adopted (IA) children has been described up to age 7-8 years, but later development remains unstudied. AIMS: To investigate speech development between ages 5 and 10 years in children born with cleft lip and palate (CLP) adopted from China and to compare them with non-adopted (NA) children with CLP. A secondary aim was to compare the frequencies of secondary palatal surgery and number of visits to a speech and language pathologist (SLP) between the groups. METHODS & PROCEDURES: In a longitudinal study, 23 IA children from China were included and matched with 23 NA children born in Sweden. Experienced SLPs blindly reassessed audio recordings from routine follow-ups at ages 5 and 10 years. Velopharyngeal function (VPF) was assessed with the composite score for velopharyngeal competence (VPC-Sum) for single words and rated on a three-point scale (VPC-Rate) in sentence repetition. Target sounds in words and sentences were phonetically transcribed. Per cent correct consonants (PCC) were calculated at word and sentence levels. For in-depth analyses, articulation errors were divided into cleft speech characteristics (CSCs), developmental speech characteristics (DSCs) and s-errors. Information on secondary palatal surgery and number of visits to an SLP was collected. OUTCOMES & RESULTS: VPF differed significantly between the groups at both ages when assessed with VPC-Sum, but not with VPC-Rate. Regardless of the method for assessing VPF, a similar proportion in both groups had incompetent VPF but fewer IA than NA children had competent VPF at both ages. IA children had lower PCC at both ages at both word and sentence levels. More IA children had CSCs, DSCs and s-errors at age 5 years, and CSCs and s-errors at age 10. The development of PCC was significant in both groups between ages 5 and 10 years. The proportion of children receiving secondary palatal surgery did not differ significantly between the groups, nor did number of SLP visits. CONCLUSIONS & IMPLICATIONS: CSCs were more persistent in IA children than in NA children at age 10 years. Interventions should target both cleft and DSCs, be comprehensive and continue past the pre-school years. WHAT THIS PAPER ADDS: What is already known on this subject At the beginning of the 21st century, IA children with cleft lip and/or palate arrived in Sweden partially or totally unoperated, despite being at an age when palatoplasty has usually been performed. Studies up to age 7-8 years show that adopted children, compared with NA peers, have poorer articulation skills, demonstrate both cleft-related and developmental articulation errors, and are more likely to have velopharyngeal incompetence. Several studies also report that adopted children more often require secondary palatal surgery due to fistulas, dehiscence or velopharyngeal incompetence compared with NA peers. What this paper adds to existing knowledge This longitudinal study provides additional knowledge based on longer follow-ups than previous studies. It shows that the proportion of children assessed to have incompetent VPF was similar among IA and NA children. It was no significant difference between the groups regarding the proportion that received secondary palatal surgery. However, fewer IA children were assessed to have a competent VPF. Developmental articulation errors have ceased in most IA and all NA children at age 10 years, but significantly more adopted children than NA children still have cleft-related articulation errors. What are the potential or actual clinical implications of this work? Speech and language therapy should target both cleft-related and developmental articulation errors. When needed, treatment must be initiated early, comprehensive, and continued past the pre-school years, not least for adopted children.

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

OBJECTIVE: 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. METHODS: 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. RESULTS: 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. CONCLUSIONS: 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.

Secondary Le Fort III after Early Fronto-Facial Monobloc Normalizes Sleep Apnea in Faciocraniosynostosis: A Cohort Study.

BACKGROUND: This study aims to assess the improvement of sleep apnea after secondary Le Fort III facial advancement with distraction (LF3) in faciocraniosynostosis (FCS) patients with sleep apnea who have previously undergone fronto-facial monobloc advancement (FFMBA) with distraction. METHODS: Patients having undergone secondary LF3 were selected from a cohort of FCS patients with documented sleep apnea who had previously undergone fronto-facial monobloc advancement. Patient charts and polysomnographic records were reviewed. Apnea-hypopnea index (AHI) was recorded before and at least 6 months after secondary LF3. The primary outcome was normalization of AHI (less than 5/h was considered normal). Hierarchical multilevel analysis was performed to predict postoperative AHI evolution. RESULTS: Seventeen patients underwent a secondary LF3, 7.0 ± 3.9 years after the primary FFMBA. The mean age was 9.6 ± 3.9 years. A total of 15 patients (88%) normalized their AHI. Two of four patients were decannulated (50%). There was a statistically significant decrease in AHI (preoperative AHI 21.5/h vs. 3.9/h postoperatively, p=0.003). Hierarchic multilevel modeling showed progressive AHI decrease postoperatively. CONCLUSION: Secondary LF3 improves residual or relapsing sleep apnea in FCS patients who have previously had FFMBA.

"When the surgery was over, I felt like the worst part had passed": experiences of parents of children with craniosynostosis.

PURPOSE: Parents of children scheduled for surgery often experience emotional distress and anxiety. This study aimed to explore parents' experiences of hospital care after their child's craniosynostosis surgery and their perception of support during the year after discharge. DESIGN AND METHODS: A purposive sample of 19 parents of 12 children with nonsyndromic craniosynostosis, who had undergone surgery, was recruited from one of two national centers in Sweden. An interview was conducted ~1 year after the child's surgery, from September 2017 to August 2018. The interviews followed a semistructured interview guide, were recorded, transcribed verbatim, and analyzed using inductive content analysis. RESULTS: The analysis yielded six categories with subcategories as follows: (1) cared for and confident: the hospital staff was perceived as kind, professional, and reliable. (2) Alone and abandoned: sometimes, parents found it hard to initiate contact with professionals during hospitalization and after discharge. (3) The importance of information: thorough information was perceived as essential and the need for information varied during postsurgery period. (4) Feelings of worry: some parents remained worried about risks during recovery and were concerned about comorbidities and development. (5) Alright after all: parents felt that the worst part had been before surgery. (6) The need for support: parents were generally satisfied with the support offered and they often received support from family and friends, or other parents through social media/online forums. PRACTICE IMPLICATIONS: Healthcare professionals must be responsive to what support parents need at different stages in the care process and be aware that parents sometimes hesitate to initiate contact and ask for help and support. Support from healthcare professionals to everyone in the follow-up program, as a default, might be more accessible or acceptable for some parents. Providing online support from professionals should be considered and caregivers could also facilitate peer support among parents, either face-to-face or online.

Incidence of Non-Syndromic and Syndromic Craniosynostosis in Sweden.

ABSTRACT: Premature craniosynostosis is a rare condition, with a wide range of incidence estimations in the literature. The aim of this study was to establish the current incidence among the Swedish population. Since the surgical care for these children is centralized to the 2 centers of Sahlgrenska University Hospital and Uppsala University Hospital, the 2 craniofacial hospital registries were examined for surgically treated children, all having a computed tomography verified diagnosis. Results show an incidence of 7.7 cases per 10,000 live births, including 0.60/10,000 syndromic craniosynostosis. Due to information programs among health care staff and a system for early diagnosis through rapid communication, these results seem to mirror the true incidence of craniosynostosis in the Swedish population. The updated incidence data will facilitate healthcare planning and make future studies of possible changes in craniosynostosis incidence more accurate.

Greater Palatal Cleft Width Predicts an Increased Risk for Unfavorable Outcomes in Cleft Palate Repair.

OBJECTIVE: To investigate the impact of cleft width and cleft type on the need for secondary surgery and velopharyngeal competence from a longitudinal perspective. DESIGN: Retrospective, longitudinal study. SETTING: A single multidisciplinary craniofacial team at a university hospital. PATIENTS: Consecutive patients with unilateral or bilateral cleft lip and palate and cleft palate only (n = 313) born from 1984 to 2002, treated with 2-stage palatal surgery, were reviewed. A total of 213 patients were included. MAIN OUTCOME MEASURES: The impact of initial cleft width and cleft type on secondary surgery. Assessment of hypernasality, audible nasal emission, and glottal articulation from routine follow-ups from 3 to 16 years of age. The assessments were compared with reassessments of 10% of the recordings. RESULTS: Cleft width, but not cleft type, predicted the need for secondary surgery, either due to palatal dehiscence or velopharyngeal insufficiency. The distribution of cleft width between the scale steps on a 4-point scale for hypernasality and audible nasal emission differed significantly at 5 years of age but not at any other age. Presence of glottal articulation differed significantly at 3 and 5 years of age. No differences between cleft types were seen at any age for any speech variable. CONCLUSIONS: Cleft width emerged as a predictor of the need for secondary surgery as well as more deviance in speech variables related to velopharyngeal competence during the preschool years. Cleft type was not related to the need for secondary surgery nor speech outcome at any age.

Does Coronal Suturectomies and Occipital Barrel Staves Make a Difference in Early Reconstruction for Sagittal Craniosynostosis?

BACKGROUND: Various surgical methods are used for early treatment of nonsyndromic sagittal craniosynostosis. The craniofacial centers in Uppsala and Helsinki fundamentally both use the H-Craniectomy: Renier's technique. However, the Helsinki group systematically adds coronal suturectomies to prevent secondary coronal synostosis and posterior barrel staves to address posterior bulleting. The effects of these additions in early treatment of sagittal craniosynostosis are currently unknown. METHODS: Thirty-six patients from Uppsala and 27 patients from Helsinki were included in the study. Clinical data and computed tomography scans were retrieved for all patients. RESULTS: The Helsinki patients had a smaller preoperative Cranial index (CI) (65 vs 72) and a smaller preoperative width (10.1 vs 11.2). There was no difference in postoperative CI, corresponding to a difference in change in CI. Regression analysis indicated that the larger change in CI in the Helsinki group was mainly due to a lower preoperative CI allowing for a larger normalization. The Helsinki patients had less growth in length (1.5 vs 2.1 cm) and more growth in width (2.3 vs 1.9 cm). There were no differences in head circumference or surgical complications. Secondary coronal synostosis was present in 43% of the Uppsala group at 3 years of age, while calvarial defects located at sites of previous coronal suturectomies and posterior barrel staving were seen in the Helsinki group 1 year postoperatively. CONCLUSIONS: Lower preoperative CI appears to be the main factor in determining the amount of normalization in CI. Prophylactic coronal suturectomies do not seem to benefit preservation of coronal growth function since the modification correlates to less sagittal growth and more growth in width.

Ophthalmological findings in children with non-syndromic craniosynostosis: preoperatively and postoperatively up to 12 months after surgery.

AIMS: Craniosynostosis is a congenital condition characterised by premature fusion of one or more cranial sutures. The aim of this study was to analyse ophthalmic function before and after cranial surgery, in children with various types of non-syndromic craniosynostosis. METHODS: Children referred to Uppsala University Hospital for surgery of non-syndromic craniosynostosis were examined preoperatively. Visual acuity was measured with Preferential Looking tests or observation of fixation and following. Strabismus and eye motility were noted. Refraction was measured in cycloplegia and funduscopy was performed. Follow-up examinations were performed 6-12 months postoperatively at the children's local hospitals. RESULTS: One hundred twenty-two children with mean age 6.2 months were examined preoperatively. Refractive values were similar between the different subtypes of craniosynostosis, except for astigmatism anisometropia which was more common in unicoronal craniosynostosis. Strabismus was found in seven children, of which four had unicoronal craniosynostosis.Postoperatively, 113 children were examined, at mean age 15.9 months. The refractive values decreased, except for astigmatism and anisometropia in unicoronal craniosynostosis. Strabismus remained in unicoronal craniosynostosis. Two new cases with strabismus developed in unicoronal craniosynostosis and one in metopic, all operated with fronto-orbital techniques. No child had disc oedema or pale discs preoperatively or postoperatively. CONCLUSION: Ophthalmic dysfunctions were not frequent in children with sagittal craniosynostosis and preoperative ophthalmological evaluation may not be imperative. Children with unicoronal craniosynostosis had the highest prevalence of strabismus and anisometropia. Fronto-orbital techniques used to address skull deformity may be related to a higher prevalence of strabismus postoperatively.

Secondary Coronal Synostosis After Early Surgery for Sagittal Craniosynostosis: Implications for Cranial Growth.

ABSTRACT: Secondary Coronal Synostosis (SCS) in patients operated for non-syndromic Sagittal Craniosynostosis is a postoperative phenomenon with unclear implications. The aim of this study was to investigate whether SCS is a negative or a benign occurrence in the postoperative course. The authors hypothesized that SCS is related to reduced cranial growth and intracranial hypertension. Thirty-one patients operated for SC at an early age with the H-craniectomy technique were included in the study. Associations between SCS and cranial shape, growth, and signs of intracranial hypertension were analyzed. Intracranial volume distribution was assessed by measuring partial intracranial volumes defined by skull base landmarks. A total of 12/31 patients developed SCS during the postoperative course. The presence of SCS was associated with a higher prevalence of gyral impressions and a larger normalization of Cranial Index due to less growth in the anteroposterior plane. The SCS group had a smaller postoperative intracranial volume due to less posterior intracranial volume as well as less growth in head circumference. Whether this is a growth restriction caused by the SCS or a secondary effect of less primary brain growth remains to be determined. However, the correlation between SCS, less cranial growth and gyral impressions does imply that SCS should be taken into consideration during clinical follow-up as a potentially adverse event.

Nonsyndromic Craniosynostosis Is Associated with Increased Risk for Psychiatric Disorders.

BACKGROUND: Craniosynostosis is one of the most common craniofacial malformations demanding surgical treatment in infancy. Data on overall psychiatric morbidity among children with nonsyndromic craniosynostosis remain limited. This study investigated the risk of psychiatric disorders in nonsyndromic craniosynostosis. METHODS: The authors reviewed a register-based cohort of all individuals born with nonsyndromic craniosynostosis in Sweden between 1973 to 1986 and 1997 to 2012 (n = 1238). The nonsyndromic craniosynostosis cohort was compared with a matched community cohort (n = 12,380) and with unaffected full siblings (n = 1485). The authors investigated the risk of psychiatric disorders, suicide attempts, and suicides by using Cox regression adjusted for perinatal and somatic factors, season and birth year, sex, parental socioeconomic factors, and parental psychiatric disorders. RESULTS: Children with nonsyndromic craniosynostosis had a higher risk of any psychiatric disorder (adjusted Cox-derived hazard ratio, 1.70; 95 percent CI, 1.43 to 2.02), including intellectual disability (adjusted Cox-derived hazard ratio, 4.96; 95 percent CI, 3.20 to 7.70), language disorders (adjusted Cox-derived hazard ratio, 2.36; 95 percent CI, 1.57 to 3.54), neurodevelopmental disorders (adjusted Cox-derived hazard ratio, 1.30; 95 percent CI, 1.01 to 1.69), and other psychiatric disorders (adjusted Cox-derived hazard ratio, 1.43; 95 percent CI, 1.11 to 1.85). Full siblings with nonsyndromic craniosynostosis were more likely, in the crude analyses, to be diagnosed with any psychiatric disorder, including intellectual disability, language disorders, and neurodevelopmental disorders compared with nonaffected siblings. The higher risk for any psychiatric disorder and intellectual disability remained after adjusting for confounders. CONCLUSIONS: Children with nonsyndromic craniosynostosis demonstrated higher risks of any psychiatric disorder compared with children without nonsyndromic craniosynostosis. This risk cannot fully be explained by familial influences (i.e., genetic or environmental factors). CLINICAL QUESTION/LEVEL OF EVIDENCE: Risk, III.

A progressive and complex clinical course in two family members with ERF-related craniosynostosis: a case report.

BACKGROUND: ERF-related craniosynostosis are a rare, complex, premature trisutural fusion associated with a broad spectrum of clinical features and heterogeneous aetiology. Here we describe two cases with the same pathogenic variant and a detailed description of their clinical course. CASE PRESENTATION: Two subjects; a boy with a BLSS requiring repeated skull expansions and his mother who had been operated once for sagittal synostosis. Both developed intracranial hypertension at some point during the course, which was for both verified by formal invasive intracranial pressure monitoring. Exome sequencing revealed a pathogenic truncating frame shift variant in the ERF gene. CONCLUSIONS: Here we describe a boy and his mother with different craniosynostosis patterns, but both with verified intracranial hypertension and heterozygosity for a truncating variant of ERF c.1201_1202delAA (p.Lys401Glufs*10). Our work provides supplementary evidence in support of previous phenotypic descriptions of ERF-related craniosynostosis, particularly late presentation, an evolving synostotic pattern and variable expressivity even among affected family members.

Head-compliant microstrip split ring resonator for non-invasive healing monitoring after craniosynostosis-based surgery.

A soft and highly directive, proximity-coupled split-ring resonator fabricated with a liquid alloy, copper and polydimethylsiloxane (PDMS) is presented. The same was designed for sensing osteogenesis of calvarial bone. As dielectric properties of bone grafts in ossifying calvarial defects should change during the osteogenesis process, devices like this could monitor the gradual transformation of the defect into bone by differentiating changes in the dielectric properties as shifts in the resonance frequency. Computational Software Technology (CST) Microwave Studio®-based simulation results on computational head models were in good agreement with laboratory results on head phantom models, which also included the comparison with an in-vivo measurement on the human head. A discussion based on an inductive reasoning regarding dynamics' considerations is provided as well. Since the skin elasticity of newborn children is high, stretching and crumpling could be significant. In addition, due to typical head curvatures in newborn children, bending should not be a significant issue, and can provide higher energy focus in the defect area and improve conformability. The present concept could support the development of soft, cheap and portable follow-up monitoring systems to use in outpatient hospital and home care settings for post-operative monitoring of bone healing after reconstructive surgical procedures.

Parents' Experiences of Their Child's Craniosynostosis and the Initial Care Process.

Craniosynostosis is usually diagnosed in early infancy. Treatment almost always involves surgery and care is optimally organized around an interdisciplinary team of specialists at a craniofacial center. This study aimed to investigate Swedish parents' experiences of having a child with craniosynostosis and their perceptions of the initial care process. Semistructured telephone interviews were conducted with 20 parents (10 fathers and 10 mothers) of children with nonsyndromic craniosynostosis who were undergoing surgery at the Uppsala Craniofacial Center. A thematic data analysis revealed 6 themes presented in a timeline following the parents' journey from detection of their child's abnormal skull shape to waiting for surgery: Detection of the abnormal skull shape, thoughts, and feelings before the appointment with the craniofacial team, an appointment with the craniofacial team, searching the Internet and social media, waiting for surgery, and suggestions for improvement. Although meeting with the craniofacial team was considered informative, parents expressed concerns about surgery and their infant's long-term prognosis were evident. Most parents had no previous knowledge about craniosynostosis and craniofacial syndromes and wished for more information already at the time of its detection. The Internet was used both at the time of suspicion that something was wrong with the child and later to learn about risks and consequences, alternative treatments and prognosis.

Dr. Tillman et al. Reply.

We read with interest the commentary of Dr. Zablow1 on our publication on neurodevelopmental disorders and orofacial clefts (OFC).2.