Pattern of Closure of Skull Base Synchondroses in Crouzon Syndrome

Coll, Guillaume; Sakka, Laurent; Botella, Céline; Dang, N. Pham; Collet, Corine; Zérah, Michel; Arnaud, Éric; Rocco, Federico Di

doi:10.1016/j.wneu.2017.09.208

Cited by 32 publications

(31 citation statements)

References 23 publications

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“…The widened and shortened anterior cranial fossa happens in the early development of the Crouzon’s syndrome infant. 15 This increased SN-FH angular measurement is consistent, in time and space, with the anterior cranial fossa and its abnormal increased orbit height. 11 …”

Section: Discussionsupporting

confidence: 64%

Facial Malformation in Crouzon’s Syndrome Is Consistent with Cranial Base Development in Time and Space

Forte

Sawh-Martinez

et al. 2018

Plastic and Reconstructive Surgery - Global Open

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Section: Discussionsupporting

confidence: 64%

Facial Malformation in Crouzon’s Syndrome Is Consistent with Cranial Base Development in Time and Space

Forte

Sawh-Martinez

et al. 2018

Plastic and Reconstructive Surgery - Global Open

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“…33 Furthermore, it may occur simultaneously with premature cranial base synchondrosis closure causing cranial base shortening. 34 In brachycephalic dogs, and especially in cavalier King Charles spaniels, the cranial base spheno-occipital synchondrosis closes prematurely, restricting rostrocaudal skull expansion. 35 Furthermore, in Chihuahuas, a short skull base at the caudal cranial fossa and increased proximity of the cervical vertebrae both cause overcrowding of the CCJ which predisposes both to SM and CM/SMrelated clinical signs.…”

Section: Possible Causes Of Pfsmentioning

confidence: 99%

Persistent fontanelles in Chihuahuas. Part I. Distribution and clinical relevance

Kiviranta

Rusbridge

Lappalainen

et al. 2021

Veterinary Internal Medicne

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Background: The Chihuahua dog breed is known for frequent occurrence of a bregmatic fontanelle on the dorsal skull. A common conception is that this skull defect is a clinically irrelevant finding. No studies, however, describe its prevalence or whether it is accompanied by other persistent fontanelles (PFs). Although Chihuahuas are predisposed to Chiari-like malformation (CM) and syringomyelia (SM), it is unknown whether PFs occur more commonly in dogs with clinical signs that are caused by CM or SM.

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“…As a result, children with craniosynostosis are more likely to have sutural diastasis [19]. Additionally, in children, craniosynostosis can occur simultaneously with premature cranial base synchondrosis closure [30].…”

Section: Persistent Fontanellesmentioning

confidence: 99%

Persistent fontanelles in Chihuahuas and inter- and intra-rater reliability of fontanelle area measurement in computed tomography images

Kiviranta¹,

Rusbridge²,

Lappalainen³

et al. 2020

Preprint

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Background: The Chihuahua dog breed is known for frequent occurrence of a bregmatic fontanelle on the dorsal skull. A common conception is that this skull defect is clinically irrelevant in Chihuahuas. No studies, however, describe the prevalence of this malformation, whether it is accompanied by fontanelles at other locations on the skull or how to assess the severity of these lesions. Our primary aim was, by using computed tomography imaging, to describe the presence, number, and location of persistent fontanelles (PF) at cranial sutures on dorsal, lateral and caudal cranial surfaces in Chihuahuas. The secondary aim was to develop a method to measure the fontanelle area in computed tomography images by using the closed polygon tool of Osirix Medical Imaging Software. Results: Of the 50 dogs evaluated, 46 (92%) had either one or several PFs. The mean ± SD number of affected cranial sutures per dog was 2.4 ± 2.3 (range 0-10), and mean ± SD number of PFs was 2.8 ± 3.0 (range 0-13). Of the 46 dogs with affected sutures, 7 (15%) had no PF at a location typical for a bregmatic fontanelle. The inter-rater reliability of the fontanelle area measurement was almost “perfect”, and intra-rater reliability reached “excellent” agreement. Conclusions: PFs are almost ubiquitous in the examined group of Chihuahuas. They are located at dorsal, lateral, and caudal surfaces of the cranium, and hence are not all recognized reliably by palpation in adult dogs. Though the pathogenesis of the PFs described here is unknown, bone-deficient lesions may occur due to congenital defects in cranial bone ossification, delayed closure of cranial sutures, or bone resorption, as is observable in children with craniosynostosis (premature cranial suture closure). Because the imaging findings described in the Chihuahuas of this study are similar to findings among children with craniosynostosis/premature cranial base synchondrosis closure, this growth disorder may be a predisposing factor for the PFs described here. Further studies are necessary to evaluate the pathogenesis and clinical relevance of these lesions. Due to high inter- and intra-rater reliability of the method of fontanelle area measurement it may be useful in future studies.

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Pattern of Closure of Skull Base Synchondroses in Crouzon Syndrome

Cited by 32 publications

References 23 publications

Facial Malformation in Crouzon’s Syndrome Is Consistent with Cranial Base Development in Time and Space

Facial Malformation in Crouzon’s Syndrome Is Consistent with Cranial Base Development in Time and Space

Persistent fontanelles in Chihuahuas. Part I. Distribution and clinical relevance

Persistent fontanelles in Chihuahuas and inter- and intra-rater reliability of fontanelle area measurement in computed tomography images

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