| 1. |
- Kljajić, Marizela, et al.
(författare)
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Children Treated for Nonsyndromic Craniosynostosis Exhibit Average Adaptive Behavior Skills with Only Minor Shortcomings
- 2021
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Ingår i: Plastic and reconstructive surgery. - 1529-4242. ; 147:2, s. 453-464
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Tidskriftsartikel (refereegranskat)abstract
- Copyright © 2021 by the American Society of Plastic Surgeons. BACKGROUND: Adaptive behavior skills are important when assessing cognitive functions related to daily life; however, few studies have assessed these skills in patients treated for nonsyndromic craniosynostosis. In this study, the authors assessed the adaptive behavior skills of children treated for craniosynostosis and examined whether their outcomes are related to surgical technique. METHODS: The Adaptive Behavior Assessment System, 2nd Edition, parent report was used for children (age, 7 to 16 years) treated for sagittal (n = 41), metopic (n = 24), and other rare synostoses (n = 8). Background data, including intelligence quotient, were controlled for confounders. RESULTS: All evaluated children treated for craniosynostosis were estimated as lower in all aspects of adaptive behavior skills (full-scale, conceptual, social, and practical composites; effect size, 0.36 to 0.44) as compared with norms. The sagittal group showed shortcomings in social composite (effect size, 0.48) and subscales measuring self-care and self-direction, although no difference was observed between spring-assisted surgery and pi-plasty regarding outcomes of adaptive behavior skills. In addition, children treated for metopic synostosis showed results indicating shortcomings with adaptive behavior according to the full-scale, conceptual, and social composites (effect size, 0.53 to 0.61) relative to norms. Furthermore, attrition analysis revealed no significant differences between responders (rate, 80.2 percent) and nonresponders. CONCLUSION: These results found that children treated for craniosynostosis display average adaptive behavior skills, and that the two surgical techniques used to treat sagittal synostosis did not differ in their behavioral outcomes. CLINICAL QUESTION/LEVEL OF EVIDENCE: Therapeutic, III.
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| 2. |
- Kljajić, Marizela, et al.
(författare)
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Sustained attention and vigilance of children treated for sagittal and metopic craniosynostosis.
- 2020
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Ingår i: Child neuropsychology : a journal on normal and abnormal development in childhood and adolescence. - : Informa UK Limited. - 1744-4136. ; 26:4, s. 475-488
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Tidskriftsartikel (refereegranskat)abstract
- Attention problems are common in patients with craniosynostosis. Craniosynostosis is a rare condition, studies face challenges of selection bias, small sample sizes, and wide age ranges. The aim of the study was to assess the sustained attention and vigilance of children treated for sagittal and metopic craniosynostosis. To reduce selection bias, we included children that had previously undergone surgery for craniosynostosis, were between 8 and 16years, and lived close to the craniofacial centre. The Connors Continuous Performance Test (3rd edition) was used to measure sustained attention and vigilance (n =61; response rate: 76.3%). Attrition analysis revealed no differences between responding and non-responding groups regarding background variables. One identified difference between the SS (n =28) and MS (n =23) groups involved significantly better performance by the SS group in the hit-reaction time (HRT) test relative to the MS group (p<0.05). Compared with the norms, the SS group showed significantly worse response style, detectability, omissions, commissions, perseverations, HRT response speed (HRT-SD), HRT inter-stimulus interval change (HRT-iC) (p<0.01 for all), and variability (p<0.05). The MS group showed significantly worse detectability, HRT-SD, variability (p<0.01 for all), commissions, perseverations and HRT-iC (p<0.05 for all) as compared with norms. No differences regarding attention was detected for the two different surgical techniques used for correction of sagittal synostosis. There were shortcomings in sustained attention and vigilance as compared with the norms in the SS and MS groups, although the deviations were small.
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| 3. |
- Fischer, Sara, et al.
(författare)
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Comparisons of Intracranial Volume and Cephalic Index After Correction of Sagittal Craniosynostosis With Either Two or Three Springs
- 2021
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Ingår i: The Journal of craniofacial surgery. - 1536-3732. ; 32:8, s. 2636-2640
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Tidskriftsartikel (refereegranskat)abstract
- ABSTRACT: In this retrospective study, the authors determined changes in intracranial volume (ICV) and cephalic index (CI) in patients with sagittal craniosynostosis and operated with craniotomy combined with either 2 or 3 springs. The authors included patients (n=112) with complete follow-up that had undergone surgical correction for isolated sagittal craniosynostosis with craniotomy combined with springs between 2008 and 2017. All patients underwent computed tomography examination preoperative, at the time of spring extraction, and at 3years of age. Intracranial volume was measured using a semiautomatic MATLAB program, and CI was calculated as the width/length of the skull. The authors found that craniotomy combined with 2 springs increased the ICV from a preoperative value of 792±113mL (mean±standard deviation) to 1298±181mL at 3years of age and increased the CI from 72.1±4.1 to 74.6±4.3, whereas craniotomy combined with 3 springs increased the ICV from 779±128mL to 1283±136mL and the CI from 70.7±4.3 to 74.8±3.7. The relative increase in ICV was 65±21% in the two-spring group and 68±34% in the three-spring group (P value=0.559), and the relative increase in CI was 3.6±3.3% in the two-spring group as compared with 6.0±5.0% in the three-spring group (P=0.004). These findings demonstrated that use of 3 springs resulted in additional absolute and relative CI-specific effects as compared with 2 springs during the time when the springs were in place, with this effect maintained at 3years of age.
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| 4. |
- Malmqvist, Carina, et al.
(författare)
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Simplified Postoperative Care After Spring-assisted Strip Craniotomy for Sagittal Synostosis: A Prospective Before-and-After Study.
- 2021
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Ingår i: The Journal of craniofacial surgery. - 1536-3732. ; 32:4, s. 1507-1510
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Tidskriftsartikel (refereegranskat)abstract
- Since the development of spring-assisted techniques for corrective craniofacial surgery, routine postoperative admission to intensive care units (ICUs) has been questioned. However, close monitoring is necessary if the continuous infusion of morphine is used as recommended for better pain relief. In this study, the authors evaluated a simplified postoperative protocol without continuous morphine infusion and no indwelling urinary catheter following spring-assisted surgery (SAS) for sagittal synostosis. Ten children were cared for according to a standard protocol with postoperative intravenous (i.v.) infusion of morphine and an indwelling urinary catheter, and 11 consecutive children were treated according to a simplified protocol with pain relief based on intermittent injections of morphine and clonidine [according to Face, Legs, Activity, Cry, Consolability (FLACC) scores >4] without the indwelling catheter. A Mann-Whitney U test was used for comparison of distributions between the two groups. The results revealed no differences between groups regarding the proportion of FLACC scores >4, total amount of administered i.v. morphine and clonidine, total volume of buffered glucose infused, time to first feeding on breast milk or substitute, or the length of stay. Despite the inherent limitations of our small observational study, the authors concluded that at our institution, it was possible to exclude a standard continuous i.v. infusion of morphine and an indwelling urinary catheter from our postoperative care protocol without decreasing the quality of pain relief in children submitted to SAS for sagittal synostosis. This finding supports downgrading the level of care from the ICU to a regular ward after limited immediate postoperative observation.
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| 5. |
- Mellgren, Jonas, 1997, et al.
(författare)
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Improved Facial and Skull-base Symmetry Following Osteotomy and Distraction of Unilateral Coronal Synostosis.
- 2024
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Ingår i: Plastic and reconstructive surgery. - 1529-4242. ; 153:2, s. 447-456
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Tidskriftsartikel (refereegranskat)abstract
- Unilateral coronal synostosis (UCS) results in a surgically demanding deformation, as the deformity is asymmetric in the calvarium but also presents with facial scoliosis and orbital dystopia. Traditional cranioplasties correct the forehead but have little effect on the face and orbits. Here, we describe a consecutive series of patients operated for UCS with osteotomy of the fused suture combined with distraction osteogenesis (FOD).Fourteen patients [mean age: 8.0 months (range: 4.3-16.6 months)] were included in this study. We measured and compared the orbital dystopia angle (ODA), anterior cranial fossa deviation (ACFD), and anterior cranial fossa cant (ACFC) between results from preoperative computed tomography and those at distractor removal.Blood loss was 6.1 mL/kg (range: 2.0-15.2 mL/kg), and length of stay was 4.4 days (range: 3.0-6.0 days). We observed significant improvements in the ODA from [median (95% confidence interval)] -9.8° (-12.6° to -7.0°) to -1.1° (-3.7° to -1.5°) (p<0.001), ACFD from 12.9° (9.2-16.6°) to 4.7° (1.5-7.9°) (p<0.001), and ACFC from 2.5° (1.5-3.5°) to 1.7° (0.0-3.4) (p=0.003).The results showed that osteotomy combined with a distractor for UCS straightened the face and relieved orbital dystopia by affecting the nose angle relative to the orbits, correcting the deviation of the cranial base in the anterior fossa, and lowering the orbit on the affected side. Furthermore, this technique demonstrated a favorable morbidity profile with low perioperative bleeding and a short inpatient period, suggesting its potential to improve the surgical treatment of UCS.
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| 6. |
- Michaëlsson, Isak, et al.
(författare)
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Circulating Brain-Injury Markers After Surgery for Craniosynostosis
- 2023
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Ingår i: World Neurosurgery. - : Elsevier BV. - 1878-8750 .- 1878-8769. ; 173
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Tidskriftsartikel (refereegranskat)abstract
- Objective: Historically, there have been few quantitative methods for effectively evaluating outcomes after surgery for craniosynostosis. In this prospective study, we assessed a novel approach for detecting possible postsurgery brain injury in patients with craniosynostosis. Methods: We included consecutive patients operated on for sagittal (pi-plasty or craniotomy combined with springs) or metopic (frontal remodeling) synostosis at the Craniofacial Unit at Sahlgrenska University Hospital, Gothenburg, Sweden, from January 2019 to September 2020. Plasma concentrations of the brain-injury biomarkers neurofilament light (NfL), glial fibrillary acidic protein (GFAP), and tau were measured immediately before induction of anesthesia, immediately before and after surgery, and on the first and the third postoperative days using single-molecule array assays. Results: Of the 74 patients included, 44 underwent craniotomy combined with springs for sagittal synostosis, 10 underwent pi-plasty for sagittal synostosis, and 20 underwent frontal remodeling for metopic synostosis. Compared with baseline, GFAP level showed a maximal significant increase at day 1 after frontal remodeling for metopic synostosis and pi-plasty (P = 0.0004 and P = 0.003, respectively). By contrast, craniotomy combined with springs for sagittal synostosis showed no increase in GFAP. For neurofilament light, we found a maximal significant increase at day 3 after surgery for all procedures, with significantly higher levels observed after frontal remodeling and pi-plasty compared with craniotomy combined with springs (P < 0.001). Conclusions: These represent the first results showing significantly increased plasma levels of brain-injury biomarkers after surgery for craniosynostosis. Furthermore, we found that more extensive cranial vault procedures resulted in higher levels of these biomarkers relative to less extensive procedures.
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| 7. |
- Selvaggi, Gennaro, 1973, et al.
(författare)
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Ethical Considerations in Surgery for Single-suture Craniosynostosis.
- 2023
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Ingår i: The Journal of craniofacial surgery. - 1049-2275 .- 1536-3732. ; 34:7, s. 1922-1926
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Tidskriftsartikel (refereegranskat)abstract
- Single-suture craniosynostosis (SSC) describes the premature fusion of one cranial suture, which restricts cranial growth and consequently results in unaffected regions presenting a compensatory expansion. Surgery can redistribute intracranial volume, reduce the risk of elevated intracranial pressure, and improve head shape, potentially leading to improved neurocognitive function and social acceptance. However, there is limited evidence that surgery for SSC improves neurocognitive function and social acceptance. Given the inherent surgical risks and uncertainty of outcomes, the conditions under which this surgery should be allowed remain uncertain. Here, we discuss ethical questions regarding the permissibility of surgery, value of neurocognitive function and social acceptance, research ethics associated with SSC, patient autonomy and parental roles, and the process of recommending surgery and obtaining consent. Because surgery for SSC has become a routine procedure, its practice now presents a relatively low risk of complications. Furthermore, having acquired an understanding of the risks associated with this surgery, such knowledge fulfils the principle of non-maleficence although not beneficence. Thus, we advocate that surgery should only be offered within Institutional Review Board-approved research projects. In these situations, decisions concerning enrollment in scientific research involves health care providers and parents or guardians of the child, with the former acting as gate-keepers upon recognition of a lack of coping skills on the part of the parent or guardian in dealing with unforeseen outcomes. To minimize associated surgical risks and maximize its benefits, there exists a moral obligation to refer patients only to highly specialized centers.
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| 8. |
- Söfteland, Madiha Bhatti, 1977, et al.
(författare)
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Correction of Unicoronal Synostosis With Springs: Two Patients With Improved Facial Symmetry
- 2024
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Ingår i: JOURNAL OF CRANIOFACIAL SURGERY. - 1049-2275 .- 1536-3732. ; 35:1, s. 10-12
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Tidskriftsartikel (refereegranskat)abstract
- Objective: Surgical correction of unicoronal synostosis (UCS) entails extensive cranioplasties which do not address facial scoliosis. This paper presents the first results with springs that motivated the shift from extensive cranioplasties to dynamic techniques for surgical correction of UCS.Methods: Two cases of UCS were operated with a linear osteotomy combined with springs. The deviation in facial symmetry (orbital dystopia angle) and skull base angles were measured on pre and postoperative computed tomography scans until 3 years of age.Results: The facial scoliosis was corrected. At spring removal, the orbital dystopia angle had gone from a 9.2 to 13.2-degree deviation preoperatively to a 0.5 to 0.9-degree overcorrection compared with the ideal 0-degree deviation. Also, the skull base deviation improved.Conclusion: Linear osteotomy combined with springs corrects the facial scoliosis in UCS. These cases indicate that dynamic methods may be beneficial for improving the results of surgical correction of UCS.
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| 9. |
- Tarnow, Peter, 1963, et al.
(författare)
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Incidence of Non-Syndromic and Syndromic Craniosynostosis in Sweden
- 2022
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Ingår i: Journal of Craniofacial Surgery. - : Ovid Technologies (Wolters Kluwer Health). - 1049-2275. ; 33:5, s. 1517-1520
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Tidskriftsartikel (refereegranskat)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.
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| 10. |
- Topa, Alexandra, 1978, et al.
(författare)
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The value of genome-wide analysis in craniosynostosis
- 2024
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Ingår i: FRONTIERS IN GENETICS. - 1664-8021. ; 14
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Tidskriftsartikel (refereegranskat)abstract
- Background: This study assessed the diagnostic yield of high-throughput sequencing methods in a cohort of craniosynostosis (CS) patients not presenting causal variants identified through previous targeted analysis.Methods: Whole-genome or whole-exome sequencing (WGS/WES) was performed in a cohort of 59 patients (from 57 families) assessed by retrospective phenotyping as having syndromic or nonsyndromic CS.Results: A syndromic form was identified in 51% of the unrelated cases. A genetic cause was identified in 38% of syndromic cases, with novel variants detected in FGFR2 (a rare Alu insertion), TWIST1, TCF12, KIAA0586, HDAC9, FOXP1, and NSD2. Additionally, we report two patients with rare recurrent variants in KAT6A and YY1 as well as two patients with structural genomic aberrations: one with a 22q13 duplication and one with a complex rearrangement involving chromosome 2 (2p25 duplication including SOX11 and deletion of 2q22). Moreover, we identified potentially relevant variants in 87% of the remaining families with no previously detected causal variants, including novel variants in ADAMTSL4, ASH1L, ATRX, C2CD3, CHD5, ERF, H4C5, IFT122, IFT140, KDM6B, KMT2D, LTBP1, MAP3K7, NOTCH2, NSD1, SOS1, SPRY1, POLR2A, PRRX1, RECQL4, TAB2, TAOK1, TET3, TGFBR1, TCF20, and ZBTB20.Conclusion: These results confirm WGS/WES as a powerful diagnostic tool capable of either targeted in silico or broad genomic analysis depending on phenotypic presentation (e.g., classical or unusual forms of syndromic CS).
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| 11. |
- Andine, A., et al.
(författare)
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Cleft Palate in Apert Syndrome: A Descriptive Study of Incidence and Surgical Outcome
- 2023
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Ingår i: The Cleft Palate-Craniofacial Journal. - 1055-6656.
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Tidskriftsartikel (refereegranskat)abstract
- Objective Apert syndrome (AS) is a rare congenital craniofacial disorder that requires a multidisciplinary approach to treatment and multiple surgeries. Given that cleft palate (CP) is presented in some of these cases, this poses an additional risk of aggravating obstructed airways after closure. The timing and outcome of CP repair in these patients remains disputed and requires additional attention. Design This retrospective analysis included patients diagnosed with CP and AS, born between 1950 and 2020, and treated at our institution. Data were collected from medical records and evaluated using descriptive statistics. Setting Data analyses were conducted at Sahlgrenska University Hospital in Gothenburg, Sweden. Patients/Participants A registry of 83 patients with AS resulted in a cohort of 26 patients also presenting with CP. Main Outcome Measures Postoperative complications, requirement for intensive care, and reoperations following CP repair. Results CP incidence among all registered patients was 31%. Patients undergoing CP repair at low age (mean: 22.5 months) tended to experience more frequent postoperative complications and requirements for intensive care. Among the evaluated cohort with medical records describing CP repair (n = 14), 29% experienced postoperative complications, all of which involved aggravation of obstructed airways. Conclusions This study highlights the importance of airway assessment before and after CP repair in AS. The findings suggest that surgical outcomes might benefit from postponing CP repair, avoiding combined surgeries, and operating in two stages when indicated. However, additional and larger studies are required.
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