Syndromic Craniosynostosis: Complexities of Clinical Care

O’Hara, Justine; Ruggiero, Federica; Wilson, Louise C.; James, Greg; Glass, Graeme E.; Jeelani, Owase; Ong, Juling; Bowman, Richard; Wyatt, Michelle; Evans, Robert D.; Samuels, Martin; Hayward, Richard; Dunaway, David

doi:10.1159/000495739

Cited by 42 publications

(53 citation statements)

References 71 publications

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“…Craniosynostosis is defined as the early closure of one or more cranial sutures and can cause head shape abnormalities with both functional and appearance-related consequences [ 1 , 2 ]. Although exact timings and techniques vary between centres, many children referred to specialist centres undergo surgical correction in early childhood.…”

Section: Introductionmentioning

confidence: 99%

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

et al. 2020

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

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Section: Introductionmentioning

confidence: 99%

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

et al. 2020

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“…Examples of syndromic craniosynostosis include: Antley-Bixler syndrome, Apert syndrome, Carpenter syndrome, Crouzon syndrome, Muenke syndrome, Pfeiffer syndrome, and Saethre-Chotzen syndrome ( Wilkie and Morriss-Kay, 2001 ; Lenton et al, 2005 ). Of these syndromes, Muenke syndrome is the most common and presents with coronal suture synostosis ( O’Hara et al, 2019 ). However, in non-syndromic craniosynostosis the sagittal suture as the most frequently affected ( Kimonis et al, 2007 ; Flaherty et al, 2016 ).…”

Section: The Importance Of Suturesmentioning

confidence: 99%

The Intertwined Evolution and Development of Sutures and Cranial Morphology

White

Goswami

Tucker

2021

Front. Cell Dev. Biol.

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Phenotypic variation across mammals is extensive and reflects their ecological diversification into a remarkable range of habitats on every continent and in every ocean. The skull performs many functions to enable each species to thrive within its unique ecological niche, from prey acquisition, feeding, sensory capture (supporting vision and hearing) to brain protection. Diversity of skull function is reflected by its complex and highly variable morphology. Cranial morphology can be quantified using geometric morphometric techniques to offer invaluable insights into evolutionary patterns, ecomorphology, development, taxonomy, and phylogenetics. Therefore, the skull is one of the best suited skeletal elements for developmental and evolutionary analyses. In contrast, less attention is dedicated to the fibrous sutural joints separating the cranial bones. Throughout postnatal craniofacial development, sutures function as sites of bone growth, accommodating expansion of a growing brain. As growth frontiers, cranial sutures are actively responsible for the size and shape of the cranial bones, with overall skull shape being altered by changes to both the level and time period of activity of a given cranial suture. In keeping with this, pathological premature closure of sutures postnatally causes profound misshaping of the skull (craniosynostosis). Beyond this crucial role, sutures also function postnatally to provide locomotive shock absorption, allow joint mobility during feeding, and, in later postnatal stages, suture fusion acts to protect the developed brain. All these sutural functions have a clear impact on overall cranial function, development and morphology, and highlight the importance that patterns of suture development have in shaping the diversity of cranial morphology across taxa. Here we focus on the mammalian cranial system and review the intrinsic relationship between suture development and morphology and cranial shape from an evolutionary developmental biology perspective, with a view to understanding the influence of sutures on evolutionary diversity. Future work integrating suture development into a comparative evolutionary framework will be instrumental to understanding how developmental mechanisms shaping sutures ultimately influence evolutionary diversity.

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“…Modifications have been introduced for head shape correction in anterior plagiocephaly and metopic synostosis 21 – 23 . Spring assisted correction of lambdoid craniosynostosis has been reported, where it was part of a multi-sutural deformity 24 , 25 . The surgery requires insertion of spring distractors in the skull after osteotomies are performed to release the fused sutures; the springs, initially compressed, start opening resulting in an expansion force to the skull perpendicular to the osteomised cranial bone.…”

Section: Introductionmentioning

confidence: 99%

Computational modelling of patient specific spring assisted lambdoid craniosynostosis correction

Bozkurt

Borghi

Lande

et al. 2020

Sci Rep

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Lambdoid craniosynostosis (LC) is a rare non-syndromic craniosynostosis characterised by fusion of the lambdoid sutures at the back of the head. Surgical correction including the spring assisted cranioplasty is the only option to correct the asymmetry at the skull in LC. However, the aesthetic outcome from spring assisted cranioplasty may remain suboptimal. The aim of this study is to develop a parametric finite element (FE) model of the LC skulls that could be used in the future to optimise spring surgery. The skull geometries from three different LC patients who underwent spring correction were reconstructed from the pre-operative computed tomography (CT) in Simpleware ScanIP. Initially, the skull growth between the pre-operative CT imaging and surgical intervention was simulated using MSC Marc. The osteotomies and spring implantation were performed to simulate the skull expansion due to the spring forces and skull growth between surgery and post-operative CT imaging in MSC Marc. Surface deviation between the FE models and post-operative skull models reconstructed from CT images changed between ± 5 mm over the skull geometries. Replicating spring assisted cranioplasty in LC patients allow to tune the parameters for surgical planning, which may help to improve outcomes in LC surgeries in the future.

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Syndromic Craniosynostosis: Complexities of Clinical Care

Cited by 42 publications

References 71 publications

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

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

The Intertwined Evolution and Development of Sutures and Cranial Morphology

Computational modelling of patient specific spring assisted lambdoid craniosynostosis correction

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