Crouzon syndrome: Virtual planning of surgical treatment by application of internal distractors

Rahimov, Chingiz; Asadov, Ruslan; Hajiyeva, Gunel; Verdiyev, Nazim; Novruzov, Zaur; Farzaliyev, Ismayil

doi:10.4103/2231-0746.186144

Cited by 8 publications

(4 citation statements)

References 9 publications

(8 reference statements)

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“…Reports of the use of VSP for multisutural, syndromic, or other cases of complex synostosis remain limited to case reports or small case series. 2 , 13 , 14 In addition, there have been no reports made which focus on comparing directly the utility of VSP in cases of complex craniosynostosis to a similar cohort of cases where VSP was not used. As digital techniques for cranial modeling continue to advance and become more readily available, increased utilization of VSP for cases of skeletal facial reconstruction and complex craniosynostosis repair is anticipated.…”

Section: Trends In the Utilization Of Vsp And Role In The Management ...mentioning

confidence: 99%

The Role of Virtual Surgical Planning in Surgery for Complex Craniosynostosis

Imahiyerobo,

Valenti,

Guadix

et al. 2024

Plastic and Reconstructive Surgery - Global Open

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Background: Virtual surgical planning (VSP) decreases reliance on intraoperative subjective assessment of aesthetic and functional outcomes in craniofacial surgery. Here, we describe our experience of using VSP for complex craniosynostosis surgery to inform preoperative decision making and optimize postoperative outcomes. Methods: Chart review was performed for children treated with craniosynostosis at our institution from 2015 to 2021. Eight VSP maneuvers were defined and assigned to each patient when applicable: (1) complex cranioplasty: combined autologous and synthetic; (2) autologous cranioplasty; (3) synthetic cranioplasty; (4) vector analysis and distractor placement; (5) complex osteotomies; (6) multilayered intraoperative plans; (7) volume analysis; and (8) communication with parents. Outcomes between VSP and non-VSP cohorts were compared. Results: Of 166 total cases, 32 were considered complex, defined by multisutural craniosynostosis, syndromic craniosynostosis, or revision status. Of these complex cases, 20 underwent VSP and 12 did not. There was no difference in mean operative time between the VSP and non-VSP groups (541 versus 532 min, P = 0.82) or in unexpected return to operating room (10.5% versus 8.3%, P = 0.84). VSP was most often used to communicate the surgical plan with parents (90%) and plan complex osteotomies (85%). Conclusions: In this cohort, VSP was most often used to communicate the surgical plan with families and plan complex osteotomies. Our results indicate that VSP may improve intraoperative efficiency and safety for complex craniosynostosis surgery. This tool can be considered a useful adjunct to plan and guide intraoperative decisions in complex cases, reducing variability and guiding parental expectations.

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Section: Trends In the Utilization Of Vsp And Role In The Management ...mentioning

confidence: 99%

The Role of Virtual Surgical Planning in Surgery for Complex Craniosynostosis

Imahiyerobo,

Valenti,

Guadix

et al. 2024

Plastic and Reconstructive Surgery - Global Open

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“…(van der Meulen et al 2009 ; Johnson and Wilkie 2011 ; Cornelissen et al 2016 ; Tønne et al 2020 ) CS is caused by early fusion of the sutures. While craniofacial surgeons have been developing various techniques to advance the treatment of this condition (David and Sheen 1990 ; Jimenez and Barone 1998 ; Rahimov et al 2016 ; Delye et al 2018 ; Breakey et al 2021 ) molecular biologists have been unravelling its underlying genetics and have developed various mouse models presenting the condition. (Rice et al 2003 ; Eswarakumar et al 2004 ; Ishii et al 2015 ; Flaherty et al 2016 ; Katsianou et al 2016 ; Merkuri and Fish 2019 ; Lee et al 2019 ) The Crouzon mouse model, type Fgfr2 C342Y/ + , was developed in 2004, following the discovery of the genes responsible for early fusion of the coronal suture (joining the parietal and frontal bones) in humans.…”

Section: Introductionmentioning

confidence: 99%

Multiscale mechanical characterisation of the craniofacial system under external forces

Didziokas,

Jones,

Alazmani

et al. 2024

Biomech Model Mechanobiol

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Premature fusion of craniofacial joints, i.e. sutures, is a major clinical condition. This condition affects children and often requires numerous invasive surgeries to correct. Minimally invasive external loading of the skull has shown some success in achieving therapeutic effects in a mouse model of this condition, promising a new non-invasive treatment approach. However, our fundamental understanding of the level of deformation that such loading has induced across the sutures, leading to the effects observed is severely limited, yet crucial for its scalability. We carried out a series of multiscale characterisations of the loading effects on normal and craniosynostotic mice, in a series of in vivo and ex vivo studies. This involved developing a custom loading setup as well as software for its control and a novel in situ CT strain estimation approach following the principles of digital volume correlation. Our findings highlight that this treatment may disrupt bone formation across the sutures through plastic deformation of the treated suture. The level of permanent deformations observed across the coronal suture after loading corresponded well with the apparent strain that was estimated. This work provides invaluable insight into the level of mechanical forces that may prevent early fusion of cranial joints during the minimally invasive treatment cycle and will help the clinical translation of the treatment approach to humans.

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“…By creating a three-dimensional virtual model, the technology allows for presurgical determination of osteotomy lines, monobloc design, and the positioning of distraction devices. 25 This presurgical plan can then be translated to aid in the operating room through custom cutting guides, surgical splints, and fixation devices, resulting in improved postoperative outcomes. 26 In this case study, we review the evolution of treatment of a family with Crouzon syndrome spanning three generations, treated by three generations of craniofacial surgeons.…”

mentioning

confidence: 99%

Crouzon Syndrome Spanning Three Generations: Advances in the Treatment of Syndromic Midface Deficiency

Harmon,

Ferraro,

Rezania

et al. 2023

Plastic and Reconstructive Surgery - Global Open

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Background: Crouzon syndrome is an autosomal dominant genetic disorder characterized by craniosynostosis, midface retrusion, and exophthalmos. Over the past century, the treatment of craniofacial disorders like Crouzon syndrome has evolved significantly. Methods: An institutional review board–approved retrospective study was conducted to ascertain the treatment of three individuals with Crouzon syndrome from one family, complemented with a series of literature searches to examine the evolution of craniofacial surgical history. Results: Dr. David Williams Cheever developed the Le Fort I level to correct malocclusion, maxillomandibular malformations, and midface hypoplasia. Later, Dr. Paul Tessier introduced the Le Fort II and III osteotomies to treat syndromic midface hypoplasia. In 1978, Dr. Fernando Ortiz-Monasterio and Dr. Antonio Fuente del Campo published the first series of monobloc osteotomies, allowing for simultaneous correction of supraorbital and midface malformations, although complicated by blood loss and high infection rates. In 1992, McCarthy et al introduced the concept of gradual distraction to the craniofacial skeleton. In 1995, Polley et al performed the first monobloc advancement using external distraction. Subsequently, in 1997, Polley and Figueroa introduced a rigid external distraction device with multiple vector control to manage severe cleft maxillary hypoplasia. The technique was further refined and applied to treat syndromic midface hypoplasia, reducing complication rates. Currently, either external or internal distraction approaches are used to safely treat this challenging group of patients. Conclusion: The treatment of syndromic midface deficiency has significantly evolved over the past 50 years, as evidenced by this report of three generations of Crouzon syndrome.

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Crouzon syndrome: Virtual planning of surgical treatment by application of internal distractors

Cited by 8 publications

References 9 publications

The Role of Virtual Surgical Planning in Surgery for Complex Craniosynostosis

The Role of Virtual Surgical Planning in Surgery for Complex Craniosynostosis

Multiscale mechanical characterisation of the craniofacial system under external forces

Crouzon Syndrome Spanning Three Generations: Advances in the Treatment of Syndromic Midface Deficiency

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