An Algorithm for Managing Syndromic Craniosynostosis Using Posterior Vault Distraction Osteogenesis

Swanson, Jordan W.; Samra, Fares; Bauder, Andrew R.; Mitchell, Brianne; Taylor, Jesse A.; Bartlett, Scott P.

doi:10.1097/prs.0000000000002127

Cited by 79 publications

(56 citation statements)

References 26 publications

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“…There have been several technical modifications to theoretically minimize step-off deformity, including the use of barrel stave osteotomies with green stick out fracture, loosely lagging the out fractured segments to the transport segment, and placing the low occipital osteotomy in a position where the curvature of the skull is in the plane of the intended movement of the transport segment such that there is no appreciable overjet. 2,7,[17][18][19] The goal of this study was to investigate patient specific factors and surgical techniques that affect occipital step-off deformity. We hypothesize that older age at the time of PVDO may be associated with greater occipital step-off deformity, and that the use of occipital barrel staves, shorter distraction distances, and an occipital osteotomy made below the inion may be protective against occipital step-off deformity.…”

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confidence: 99%

Risk Factors for Occipital Step-Off Deformities in Posterior Vault Distraction Osteogenesis

Zapatero

Carlson

Card

et al. 2021

Journal of Craniofacial Surgery

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Purpose: The goal of this study was to investigate patient specific factors and surgical techniques that affect occipital step-off deformity in children undergoing posterior vault distraction osteogenesis (PVDO). Methods: Patients who underwent PVDO were retrospectively reviewed and included if a high resolution three-dimensional computed tomography scan was available 1-year post-distractor removal. Two craniofacial surgeons were blinded to individual subjects and presented with 3 still images of three-dimensional bone reconstructions and asked to rate the degree of step off: none, mild, moderate, or severe. The data were analyzed with multinomial logistic regressions and other appropriate statistics.Results: Forty-one patients met inclusion criteria. All patients had multisuture or bicoronal craniosynostosis. Multinomial logistic regression identified age at PVDO (Cox and Snell ¼ 0.487, P < 0.001) as a predisposing factor and one potentially protective factor, height of osteotomy (Cox and Snell ¼ 0.394, P ¼ 0.004) in univariate models. Post-hoc pairwise Mann-Whitney U test revealed there was decreased step-off rating when comparing osteotomy below torcula (median 0, [IQR 0, 3]) and above torcula (median 3 [IQR 1, 4]; P ¼ 0.036) as well as a decreased step-off when below inion (median 0 [IQR 0, 1]; P ¼ 0.001) was compared to above the torcula. Conclusions: Older age at PVDO predict increased risk of developing an occipital step-off deformity after PVDO, while design of the occipital osteotomy below the inion is protective. Barrel staves with greenstick outfracture did not lower the risk of occipital step-off in this sample, the reasons for which are unclear.

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confidence: 99%

Risk Factors for Occipital Step-Off Deformities in Posterior Vault Distraction Osteogenesis

Zapatero

Carlson

Card

et al. 2021

Journal of Craniofacial Surgery

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“…In addition, it has been reported that posterior volume expansion sometimes leads to improvement of anterior vault morphology, 73 and may thereby minimize the number of craniofacial procedures in the first 5 years of life. 74…”

Section: Openmentioning

confidence: 99%

Update in Management of Craniosynostosis

Xue

Buchanan

Hollier

2022

Plastic &Amp; Reconstructive Surgery

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raniosynostosis refers to skull deformities caused by premature fusion of one or more sutures (Fig. 1). The incidence is estimated to be one in 2500 births. 1,2 More than 80 percent are isolated or nonsyndromic. The suture affected has a gender predisposition, with sagittal synostosis being four times more common in boys, and unicoronal synostosis 1.5 times more common in girls. 1,2 The pathogenesis of premature fusion in craniosynostosis remains unclear. There have been many hypotheses over the years, including intrauterine constraint, 3-7 hypophosphatemia and rickets, 8,9 maternal smoking, 10,11 and excessive use of antacids during infancy. 12 None were strongly supported.With the development of better genetic sequence technologies, more and more mutations have been identified in individuals with congenital abnormalities, and craniosynostosis is no exception. Recently, isolated sagittal synostosis has been linked to a mutation in the BBS9 gene 13 and metopic synostosis to a mutation in the FREM1 gene, 14 but the clinical significance of these findings is unclear. Other genes identified include SMAD6, which account for 7 percent of nonsyndromic sagittal and metopic synostosis, and 10 percent of familial cases. 15 A recent study demonstrated that the SMAD6 mutation may lead to poorer neurocognitive outcome as well. 16 Several mutations have been identified for syndromic craniosynostosis. Of these, the most common are the fibroblast growth factor receptor genes (FGFR1, FGFR2, and FGFR3). FGFR is a group of 22 proteins that regulate proliferation, differentiation, and migration of cells that are essential for wound healing, angiogenesis, and limb development. There are several craniosynostosis syndromes linked to mutations in this group of genes. Mutation in FGFR2 is linked to Apert, Crouzon and Beare-Stevenson syndromes. Pfeiffer syndrome is linked to mutations in both FGFR1 and FGFR2 mutations. Muenke syndrome and Crouzon with acanthosis nigricans are linked to FGFR3 mutations. 17 Saethre-Chotzen syndrome is associated with more than 100 mutations in the TWIST1 gene, 18 which codes for a transcription factor that specifies and maintains cellular identity.

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“…Patients with Apert, Crouzon, or Pfeiffer syndromes frequently undergo PVDO due to the severity of their synostosis and high risk for developing pansynostosis with significant cranial growth restriction. 30 While patients with Muenke and Saethre-Chotzen syndromes may undergo PVDO, the majority are treated with FOA due to the decreased severity of their synostosis. 31,32 Endoscopic strip craniectomy with helmet therapy is rarely used for the treatment of syndromic craniosynostosis as many patients require cranial vault remodeling shortly after.…”

Section: Syndromic Craniosynostosismentioning

confidence: 99%

Pediatric Fronto-Orbital Skull Reconstruction

et al. 2021

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Craniofacial surgery in children is a highly challenging discipline that requires extensive knowledge of craniofacial anatomy and pathology. Insults to the fronto-orbital skeleton have the potential to inflict significant morbidity and even mortality in patients due to its proximity to the central nervous system. In addition, significant aesthetic and ophthalmologic disturbances frequently accompany these insults. Craniosynostosis, facial trauma, and craniofacial tumors are all pathologies that frequently affect the fronto-orbital region of the craniofacial skeleton in children. While the mechanisms of these pathologies vary greatly, the underlying principles of reconstruction remain the same. Despite the limited data in certain areas of fronto-orbital reconstruction in children, significant innovations have greatly improved its safety and efficacy. It is imperative that further investigations of fronto-orbital reconstruction are undertaken so that craniofacial surgeons may provide optimal care for these patients.

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An Algorithm for Managing Syndromic Craniosynostosis Using Posterior Vault Distraction Osteogenesis

Abstract: Therapeutic, III.

Cited by 79 publications

References 26 publications

Risk Factors for Occipital Step-Off Deformities in Posterior Vault Distraction Osteogenesis

Risk Factors for Occipital Step-Off Deformities in Posterior Vault Distraction Osteogenesis

Update in Management of Craniosynostosis

Pediatric Fronto-Orbital Skull Reconstruction

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