A European multicenter outcome study on the different perioperative airway management policies following midface surgery in syndromic craniosynostosis:a proposal for a Standard Operating Procedure

BACKGROUND: Perioperative airway management following midface advancements in children with Apert and Crouzon/Pfeiffer syndrome can be challenging, and protocols often differ. This study examined airway management following midface advancements and postoperative respiratory complications.METHODS: A multicenter, retrospective cohort study was performed to obtain information about the timing of extubation, perioperative airway management, and respiratory complications after monobloc / le Fort III procedures.RESULTS: Ultimately, 275 patients (129 monobloc and 146 Le Fort III) were included; 62 received immediate extubation and 162 delayed extubation; 42 had long-term tracheostomies and nine perioperative short-term tracheostomies. Short-term tracheostomies were in most centers reserved for selected cases. Patients with delayed extubation remained intubated for three days (IQR 2 - 5). The rate of no or only oxygen support after extubation was comparable between patients with immediate and delayed extubation, 58/62 (94%) and 137/162 (85%) patients, respectively. However, patients with immediate extubation developed less postoperative pneumonia than those with delayed, 0/62 (0%) versus 24/161 (15%) (P = 0.001), respectively. Immediate extubation also appeared safe in moderate/severe OSA since 19/20 (95%) required either no or only oxygen support after extubation. The odds of developing intubation-related complications increased by 21% with every extra day of intubation.CONCLUSIONS: Immediate extubation following midface advancements was found to be a safe option, as it was not associated with respiratory insufficiency but did lead to fewer complications. Immediate extubation should be considered routine management in patients with no/mild OSA and should be the aim in moderate/severe OSA after careful assessment.</p

A European multicenter outcome study on the different perioperative airway management policies following midface surgery in syndromic craniosynostosis:a proposal for a Standard Operating Procedure

BACKGROUND: Perioperative airway management following midface advancements in children with Apert and Crouzon/Pfeiffer syndrome can be challenging, and protocols often differ. This study examined airway management following midface advancements and postoperative respiratory complications.METHODS: A multicenter, retrospective cohort study was performed to obtain information about the timing of extubation, perioperative airway management, and respiratory complications after monobloc / le Fort III procedures.RESULTS: Ultimately, 275 patients (129 monobloc and 146 Le Fort III) were included; 62 received immediate extubation and 162 delayed extubation; 42 had long-term tracheostomies and nine perioperative short-term tracheostomies. Short-term tracheostomies were in most centers reserved for selected cases. Patients with delayed extubation remained intubated for three days (IQR 2 - 5). The rate of no or only oxygen support after extubation was comparable between patients with immediate and delayed extubation, 58/62 (94%) and 137/162 (85%) patients, respectively. However, patients with immediate extubation developed less postoperative pneumonia than those with delayed, 0/62 (0%) versus 24/161 (15%) (P = 0.001), respectively. Immediate extubation also appeared safe in moderate/severe OSA since 19/20 (95%) required either no or only oxygen support after extubation. The odds of developing intubation-related complications increased by 21% with every extra day of intubation.CONCLUSIONS: Immediate extubation following midface advancements was found to be a safe option, as it was not associated with respiratory insufficiency but did lead to fewer complications. Immediate extubation should be considered routine management in patients with no/mild OSA and should be the aim in moderate/severe OSA after careful assessment.</p

Use of a Fibula Free Flap for Mandibular Reconstruction in Severe Craniofacial Microsomia in Children with Obstructive Sleep Apnea

This is a retrospective study describing a multi-stage protocol for the management of severe mandibular hypoplasia in craniofacial microsomia (CFM) with accompanying obstructive sleep apnea (OSA). Patients with severe mandibular hypoplasia require reconstruction functionality and esthetical features. In the cohort, reconstructions based on free fibular flaps (FFF) may be the most effective way. Patients aged 4&ndash;17 years with severe mandibular hypoplasia were treated with FFF, which initially improved the respiratory function assessed on polysomnography (AHI). In the next stages of treatment of cases with respiratory deterioration, it was indicated to perform distraction osteogenesis (DO) of the mandible and the structures reconstructed with FFF. All surgeries were planned in accordance with virtual surgery planning VSP. The aim of the study was to prospectively assess the effectiveness of multi-stage mandibular reconstruction in craniofacial microsomia with the use of a free fibula flap in terms of improving respiratory failure due to obstructive sleep apnea (OSA). The FFF reconstruction method, performed with virtual surgical planning (VSP), is proving to be an effective alternative to traditional methods of mandibular reconstruction in patients with severe CFM with OSA

Treacher Collins Syndrome: Genetics, Clinical Features and Management

Treacher Collins syndrome (TCS) is associated with abnormal differentiation of the first and second pharyngeal arches, occurring during fetal development. Features of TCS include microtia with conductive hearing loss, slanting palpebral fissures with possibly coloboma of the lateral part of lower eyelids, midface hypoplasia, micrognathia as well as sporadically cleft palate and choanal atresia or stenosis. TCS occurs in the general population at a frequency of 1 in 50,000 live births. Four subtypes of Treacher Collins syndrome exist. TCS can be caused by pathogenic variants in the TCOF1, POLR1D, POLR1C and POLR1B genes. Genetically, the TCOF1 gene contains 27 exons which encodes the Treacle protein. In TCOF1, over 200 pathogenic variants have been identified, of which most are deletions leading to a frame-shift, that result in the formation of a termination codon. In the presented article, we review the genetics and phenotype of TCS as well as the management and surgical procedures utilized for treatment

Protocol for multi-stage treatment of temporomandibular joint ankylosis in children and adolescents

Treatment of temporomandibular ankylosis is challenging and frequently leads to re-ankylosis, relapse, dangerous complications and, in turn, the need for multiple operations. In this article, we present a protocol for the treatment of ankylosis of the temporomandibular joints that assumes earlier intervention with the assistance of 3D virtual surgical planning (3DVSP) and custom biomaterials for better and safer surgical outcomes. Thirty-three patients were treated due to either uni- or bilateral temporomandibular ankylosis. Twenty individuals received temporomandibular prosthesis, whereas seventeen required simultaneous 3D virtual surgical/planned orthognathic surgery as the final correction of the malocclusion. All patients exhibited statistically significant improvements in mouth opening (from 1.21 ± 0.74 cm to 3.77 ± 0.46 cm) and increased physiological functioning of the mandible. Gap arthroplasty and aggressive rehabilitation prior to temporomandibular prosthesis (TMJP) placement were preferred over costochondral autografts. The use of 3DVSP and custom biomaterials enables more precise, efficient and safe procedures to be performed in the paediatric and adolescent population requiring treatment for temporomandibular ankylosis

A Protocol for the Use of a Combined Microvascular Free Flap with Custom-Made 3D-Printed Total Temporomandibular Joint (TMJ) Prosthesis for Mandible Reconstruction in Children

Extended postoperative mandibular reconstructions due to orofacial disease involving the temporomandibular joint (TMJ) in immature patients remain a challenge as a result of ongoing growth, which is usually affected by the disease and treatment. Current reconstructive techniques based fully on alloplastic total joint replacement fail to meet fully the anatomical and functional requirements for the masticatory system and speech development. Fourteen children aged 12.6 ± 2.6 with tumors or congenital deformities affecting the mandible and TMJ were included in the study. Radical surgical treatment according to our own protocol was performed through microvascular anastomotic flap reconstruction of the soft tissues and bones, together with total TMJ custom replacements. Follow-up lasted 2–6 years. During the follow-up, increases in the mandible body (13% growth) and ramus (12% growth) were observed, both of which were related (p < 0.001). This is the first report concerning the immediate reconstruction of the mandible with ramus and total TMJ in children and adolescents that combines a free vascularized graft and total individual prosthesis of the TMJ. The presented technique enabled optimal function of the TMJ, growth of the mandible, and further rehabilitation of the patients. The technique was demonstrated to be safe, reliable, and provide good functional and cosmetic outcomes

Planning of physiotherapeutic procedure in patients after mandible reconstruction taking into account donor site: a literature review

Abstract Background Mandible tumors are very rare. One of the main methods of the treatments is resection of the tumor and then reconstruction of the mandible. The donor site is often distant tissue—fibula or ilium. Following this, it is necessary to improve the patient in two ways, on one hand restoring the function of the mandible, and on the other hand, improving the donor site area. For that reason, physiotherapy after tumor resection and reconstruction of the mandible is very complicated. The aim of this bibliographic review was to find the methods of the reconstruction of the mandible in the context of patients’ functional assessment after surgeries to create effective physiotherapeutic procedures in the feature. Methods PEDro, Medline (PubMed), Cochrane Clinical Trials were searched. Results 767 articles were found. 40 articles were included to this literature review. Conclusions Authors showed different kinds of surgeries strategy for patients with tumors of the mandible. They also showed manners of patients’ functional assessment in the localization of transplantation and donor site. It could be useful for physiotherapists during planning of comprehensive physiotherapy

Three-Dimensional Eyeball and Orbit Volume Modification After LeFort III Midface Distraction.

The aim of our study was to evaluate orbital volume modification with LeFort III midface distraction in patients with craniosynostosis and its influence on eyeball volume and axial diameter modification. Orbital volume was assessed by the semiautomatic segmentation method based on deformable surface models and on 3-dimensional (3D) interaction with haptics. The eyeball volumes and diameters were automatically calculated after manual segmentation of computed tomographic scans with 3D slicer software. The mean, minimal, and maximal differences as well as the standard deviation and intraclass correlation coefficient (ICC) for intraobserver and interobserver measurements reliability were calculated. The Wilcoxon signed rank test was used to compare measured values before and after surgery. P < 0.05 was considered statistically significant. Intraobserver and interobserver ICC for haptic-aided semiautomatic orbital volume measurements were 0.98 and 0.99, respectively. The intraobserver and interobserver ICC values for manual segmentation of the eyeball volume were 0.87 and 0.86, respectively. The orbital volume increased significantly after surgery: 30.32% (mean, 5.96 mL) for the left orbit and 31.04% (mean, 6.31 mL) for the right orbit. The mean increase in eyeball volume was 12.3%. The mean increases in the eyeball axial dimensions were 7.3%, 9.3%, and 4.4% for the X-, Y-, and Z-axes, respectively. The Wilcoxon signed rank test showed that preoperative and postoperative eyeball volumes, as well as the diameters along the X- and Y-axes, were statistically significant. Midface distraction in patients with syndromic craniostenosis results in a significant increase (P < 0.05) in the orbit and eyeball volumes. The 2 methods (haptic-aided semiautomatic segmentation and manual 3D slicer segmentation) are reproducible techniques for orbit and eyeball volume measurements