Determination of mandibular distraction osteogenesis most suitable parameters. An experimental study.

BACKGROUND: Distraction osteogenesis is a process of induced bone generation. Various protocols have been described for the management of the latency period, distraction speed and consolidation period, with greater or lesser success. OBJECTIVE: To better understand the process of mandibular distraction and establish the determining factors and their optimal times. MATERIAL AND METHODS: Twenty-seven dogs were studied, which had 54 distractors placed and that underwent unidirectional, bilateral mandibular distraction osteogenesis. The distraction processes were applied using six variants, two for each factor: latency period, distraction period and distraction speed. The changes were examined by means of bone biopsies and X-rays of the area at 0, 7, 14, 21, 45 and 55 days of the process. RESULTS: The most efficient osteogenic distraction parameters were a latency period of five days, a consolidation period of six weeks, distraction speed of 1 mm/day for distances of less than 20 mm, and 3 mm/day for longer distances. CONCLUSIONS: The sequential histological study allowed to observe the appearance of cellular elements (osteocytes, osteoclasts, osteoid matrix, trabeculate, etc.) and their participation in granulation tissue, newly-formed bone and compact mature bone.

ANTECEDENTES: Respecto a la distracción osteogénica (generación ósea inducida), con mayor o menor éxito han sido descritos diversos protocolos para el manejo del período de latencia, velocidad de distracción y período de consolidación. ­. OBJETIVO: Entender mejor el proceso de la distracción mandibular y establecer los factores determinantes y sus tiempos óptimos. MATERIAL Y MÉTODOS: Se estudiaron 27 perros sometidos a distracción osteogénica unidireccional, bilateral de la mandíbula. Los procesos de distracción se aplicaron con seis variantes, dos por cada factor (período de latencia, período de distracción y velocidad de distracción). Se estudiaron los cambios mediante biopsias del hueso y radiografías de la zona a los 0, 7, 14, 21, 45 y 55 días del proceso. RESULTADOS: Los parámetros de distracción osteogénica más eficientes fueron período de latencia de cinco días, período de consolidación de seis semanas, 1 mm diario de velocidad de distracción para distancias menores a 20 mm y 3 mm diarios para distancias mayores. CONCLUSIONES: El estudio histológico secuencial permitió observar la aparición de los elementos celulares (osteocitos, osteoclastos, matriz osteoide, trabeculado, etcétera) y su participación en el tejido de granulación, el hueso neoformado y el hueso maduro compacto.

Apert syndrome: neurosurgical outcomes and complications following posterior vault distraction osteogenesis.

PURPOSE: Posterior vault distraction osteogenesis (PVDO) has been utilized during the past 15 years to treat a variety of clinical features commonly presented by patients with Apert syndrome. The objective of this study is to determine the efficacy of PVDO in addressing both elevated intracranial pressure (ICP) and ectopia of the cerebellar tonsils (ECT) in young Apert patients. In addition, we aimed to determine the prevalence of hydrocephalus in Apert syndrome patients who underwent PVDO. METHODS: A retrospective study was made with a cohort of 40 consecutive patients with syndromic craniosynostosis (SC), previously diagnosed with Apert syndrome, who underwent PVDO between 2012 and 2022, and thereafter received at least 1 year of follow-up care. Demographic data and diagnosis, along with surgical and outcome data, were verified using medical records, clinical photographs, radiologic examination, and interviews with the parents of all cohort patients. RESULTS: The average patient age when PVDO was performed was 12.91 ± 10 months. The average posterior advancement distance achieved per patient was 22.68 ± 5.26 mm. The average hospital stay per patient was 3.56 ± 2.44 days. The average absolute and relative blood transfusion volumes were 98.47 ml and 17.63 ml/kg, respectively. Although five patients (14%) presented ECT preoperatively, this condition was completely resolved by PVDO in three of these five patients. One of the three patients whose ECT had completely resolved presented syringomyelia postoperatively, requiring subsequent extra dural foramen magnum decompression. All of the remaining four patients were asymptomatic for ECT for at least 1 year of follow-up, and none of these four patients required any additional treatments to address ECT. Two patients presented hydrocephalus requiring ventriculoperitoneal shunt placement. CONCLUSIONS: This study demonstrates that PVDO both reduces diagnosed elevated ICP symptoms and is partially effective in treating ECT in Apert syndrome patients. Hydrocephalus in Apert syndrome is an uncommon feature. The effectiveness of PVDO in addressing hydrocephalus is uncertain.

LeFort III Versus Monobloc Frontofacial Advancement: A Comparative Analysis of Soft Tissue Changes.

INTRODUCTION: The LeFort III and monobloc are commonly used midface advancement procedures for patients with syndromic craniosynostosis with well characterized postoperative skeletal changes. However, the differential effects of these procedures on facial soft tissues are less understood. The purpose of this study was to critically analyze and compare the effects of these 2 procedures on the overlying soft tissues of the face. METHODS: Frontal and lateral preoperative and postoperative photographs of patients undergoing monobloc or LeFort III were retrospectively analyzed using ImageJ to measure soft tissue landmarks. Measurements included height of facial thirds, nasal length and width, intercanthal distance, and palpebral fissure height and width. Facial convexity was quantified by calculating the angle between sellion (radix), subnasale, and pogonion on lateral photographs. RESULTS: Twenty-five patients with an average age of 6.7 years (range 4.8-14.5) undergoing monobloc (n=12) and LeFort III (n=13) were identified retrospectively and analyzed preoperatively and 6.4±3.6 months postoperatively. Patients undergoing LeFort III had a greater average postoperative increase in facial convexity angle acuity (28.2°) than patients undergoing monobloc (17.8°, P =0.021). Patients in both groups experience postoperative increases in nasal width ( P <0.001) and decreases in palpebral fissure height ( P <0.001). CONCLUSIONS: Both subcranial LeFort III advancements and monobloc frontofacial advancements resulted in significant changes in the soft tissues. Patients undergoing LeFort III procedures achieved greater acuity of the facial convexity angle, likely because the nasion is not advanced with the LeFort III segment.

Facial Fat Graft Injection Reduces Asymmetry and Improves Forehead Contour in Early Infancy Apert Syndrome Patients.

BACKGROUND: The objective of this study is to report the outcomes of a modified comprehensive Apert syndrome surgical protocol in which fat injection was performed during early infancy concurrent with postposterior vault distraction osteogenesis (PVDO) distractor removal. METHODS: A retrospective study was performed on 40 consecutive young patients with Apert syndrome who underwent PVDO and subsequent distractor removal between 2012 and 2022. Of these 40 patients, 12 patients underwent facial fat injection concurrent with distractor removal to treat residual supraorbital bar recession as part of a modified comprehensive Apert syndrome surgical protocol. Preoperative and postoperative severity of recession and irregularity was graded from 1 to 3, with 1 being less severe and 3 being the most severe. Recession severity was correlated with the number and type of suture fusion. The complication rate was stratified via a Clavien-Dindo scale. RESULTS: The average patient age was 14.3±5 months, with 5 males (41.6%) and 7 females (48.3%). The average hospital stay was 1.08 days. The average volume of free fat graft injection was 8.29±5 mL. According to the Likert scale, forehead morphology improved in 91.67% of the patients. Complete resolution of supraorbital bar recession was achieved in seven patients (58.33%), all of whom presenting a single suture synostosis. One patient with a cloverleaf skull presented a type IIIB complication. CONCLUSIONS: Facial fat grafting markedly reduces forehead asymmetry and improves forehead contour in Apert syndrome patients following PVDO. Total resolution of forehead recession directly correlated with a single suture fusion.

Therapeutic Management with Airway Clearance in Children with Robin Sequence and Association with Swallowing Outcomes: A Systematic Review and Meta-analysis.

Dysphagia in Robin Sequence can be present in varying degrees, requiring multidisciplinary management and specific swallowing assessment by a specialist. Most studies published to date have evaluated only respiratory outcomes, and the available evidence on the improvement of swallowing is questionable. To conduct a systematic review and meta-analysis of studies evaluating swallowing in children with Robin Sequence before and after airway clearance procedures. The research question was developed based on the PICO strategy. The literature search was performed in electronic databases and gray literature. Studies were selected by 3 independent reviewers. The risk of bias and level of evidence of the studies were assessed. A proportion meta-analysis was performed to calculate the prevalence of dysphagia after airway clearance procedures. The search identified 4938 studies, 5 of which were included. All studies had limitations in terms of design and sample size. The prevalence of dysphagia after airway clearance was obtained by analyzing treatment subgroups: mandibular distraction osteogenesis, mandibular distraction osteogenesis + tracheostomy tube, and nasopharyngeal tube. Clinical and/or instrumental assessment was assessed by a swallowing specialist. The meta-analysis was precluded by the limitations of the studies, especially regarding sample size, which affected the accuracy of the findings. Dysphagia remained unresolved in 55% of children (95% CI 1-99%). The methodological quality of the studies indicated a high risk of bias and very low level of evidence. It was not possible to confirm that airway clearance techniques used in Robin Sequence improve dysphagia.

Cadaveric Bone Allografting for Severe Mandibular Hypoplasia With Alleviation of Upper Airway Obstruction in Goldenhar Syndrome.

ABSTRACT: Severe congenital mandibular hypoplasia may cause significant upper airway obstruction, often necessitating immediate tracheostomy. Recent surgical advances have demonstrated early intervention with mandibular distraction osteogenesis over tracheostomy may yield desirable aesthetic and functional outcomes. Here we present a case of bilateral mandibular hypoplasia, severe on the right and mild on the left, secondary to Goldenhar syndrome yielding significant tongue-based upper airway obstruction that was surgically corrected with mandibular bone allograft reconstruction of the right condyle, ramus, and posterior half of the mandibular body. Postoperative polysomnography revealed significantly improved obstructive apnea and computed tomography demonstrated adequate placement of the bone allograft to overcorrect the affected hemi-mandible. To the authors' knowledge, this is the first report of immediate mandibular allografting alleviating tongue-based upper airway obstruction secondary to mandibular hypoplasia in an infant. Mandibular bone allografting may be a favorable alternative to immediate tracheostomy with delayed surgical intervention, though long-term follow up is needed to assess graft durability and maintenance of airway patency.

Treatment of Brachymetatarsia by Distraction Osteogenesis Using Monolateral External Fixator / Tratamento da braquimetatarsia por distração osteogênica com fixador externo monolateral

Abstract Objective To describe the profile of the patients and the results obtained with the use of an external fixator for metatarsal lengthening in brachymetatarsia. Methods A retrospective analysis of the medical records of patients with brachymetatarsia treated between January 2018 and April 2020 was performed. During this period, eight feet of six patients were operated on. Frequencies were estimated according to demographic and surgical aspects. Results All patients (100%; n= 6) were female, with a mean age of 28 years old, ranging from 15 to 48 years old, and were motivated to seek the orthopedic service due to aesthetic deformity. The deformity was bilateral in two patients and unilateral in four patients. The average lengthening time was 22 days ( ± 7.15, 95% confidence interval [CI]: 19.04-26.81). The lengthening speed was 0.5 mm/day, and the average total length of the lengthening was 11.46 mm ( ± 3.57; 95%CI: 9.52-13.40). Half of the patients (50%; n= 3) had local infection of the pins and were treated with antibiotics, and the others did not report any postsurgical complications. All patients denied pain or calluses after the surgical procedure and reported satisfaction with the results. Conclusion All patients were female and sought surgery for brachymetatarsia for aesthetic reasons. Osteogenic distraction at a rate of 0.5 mm/day resulted in successful lengthening of the metatarsal, with a low frequency of complications, good clinical outcomes, and high patient satisfaction.

Resumo Objetivo Descrever o perfil dos pacientes e os resultados obtidos com o uso de fixador externo para alongamento de metatarso em braquimetatarsia. Métodos Foi realizada uma análise retrospectiva dos prontuários de pacientes com braquimetatarsia tratados entre janeiro de 2018 e abril de 2020. Durante este período, foram operados oito pés de seis pacientes. Foram coletadas e estimadas as frequências em relação a aspectos demográficos e cirúrgicos. Resultados Todas as pacientes (100%; n= 6) eram do sexo feminino, com média de idade de 28 anos, variando de 15 a 48 anos, e motivadas a buscar o serviço de ortopedia em função da deformidade estética. O acometimento era bilateral em duas pacientes e unilateral em quatro pacientes. O tempo médio de alongamento foi de 22 dias ( ± 7,15; intervalo de confiança [IC] 95%: 19,04-26,81). A velocidade de alongamento foi de 0,5 mm/dia e o comprimento médio total do alongamento foi de 11,46 mm ( ± 3,57; IC95%: 9,52-13,40). Metade das pacientes (50%; n= 3) teve infecção local dos pinos e foi tratada com antibióticos; as demais não relataram nenhuma complicação pós-cirúrgica. As pacientes negaram dor ou calosidade após o procedimento cirúrgico e relataram satisfação com os resultados. Conclusão Todas as pacientes eram do sexo feminino e buscaram a cirurgia para braquimetatarsia por motivos estéticos. A distração osteogênica a uma taxa de 0,5 mm/dia resultou em alongamento bem-sucedido do metatarso, com uma baixa frequência de complicações, bons resultados clínicos e alta satisfação das pacientes.

Treatment of Sagittal Craniosynostosis Using Osteoplastic Parietal Craniotomy Flap With Distraction Osteogenesis: A Variation on Technique.

ABSTRACT: Late presentation sagittal craniosynostosis presents a unique challenge due to the decreased ability of the skull to repair the bony defects created by standard of care techniques. Distraction osteogenesis is a viable strategy to correct this defect in late presenting cases. The authors describe a variation in technique in which the temporalis muscle origin is retained, creating an osteoplastic bone flap with retained vascularity through the tem-poralis muscle. This may improve postoperative bony healing of bony defects in this compromised population. The authors present two patients who presented to them late with sagittal synostosis who were treated with distraction osteogenesis in which vascular continuity to the parietal bones is preserved through the temporalis muscle.

Endoscopic-Assisted Fronto-Orbital Distraction Osteogenesis: Initial Patient Report.

BACKGROUND: The purpose of this manuscript is to introduce a novel endoscopic-assisted approach for creation of osteotomies and distractor hardware placement for unicoronal craniosynostosis. METHODS: Only three small incisions are performed, one at the anterior fontanelle, pterional region, and upper lateral blepharoplasty location. Unicoronal strip craniectomy and osteotomies in temporal bone, nasofrontal junction, lateral orbital rim, sphenoid wing, orbital roof, and contralateral frontal bone are performed through these incisions with the assistance of a 30° 5-mm endoscope. One linear cranial distractor is inserted through the pterional incision and fixated across the unicoronal suturectomy. RESULTS: Endoscopic-assisted fronto-orbital distraction osteogenesis has been performed on 3 patients with unicoronal craniosynostosis. Median age at surgery was 5.4 months (95%CI 5.0-6.1). Median operative duration was 98 minutes (95%CI 91-112), and estimated blood loss was 25 mL (95%CI 15-150). Median length of distraction achieved was 25.7 mm (95%CI 21.9-28.0), based on lateral skull x-rays obtained on the last day of activation. Distractors were removed 2.3 months postoperatively (95%CI 2.1-3.1), and operative duration of distractor removal was 20 minutes (95%CI 19-29). There was 1 complication, a transient cerebrospinal fluid leak thought to have been caused by a pinpoint injury to the dura at the nasofrontal junction, which was managed expectantly and resolved spontaneously by the fourth postoperative day. CONCLUSIONS: Endoscopic-assisted fronto-orbital distraction osteogenesis is a promising treatment modality for children with unicoronal craniosynostosis combining the benefits of distraction osteogenesis with a minimally invasive approach. Additional experience and follow-up are needed to determine its utility, safety, and longevity.

Postoperative Changes in the Upper Airway Following Mandibular Distraction Osteogenesis in Pediatric Hemifacial Microsomia.

BACKGROUND: Patients with hemifacial microsomia (HFM) may undergo unilateral mandibular distraction osteogenesis (MDO) before skeletal maturity in an effort to improve facial symmetry. Mandibular distraction osteogenesis's effect on airway volumes have been studied in the past, though to our knowledge, none have accounted for the effect of head and neck posture on airway morphology. This study aimed to tackle this shortcoming, using imaging to analyze the upper airway of patients with HFM before and after surgical intervention with MDO. METHODS/DESCRIPTION: The authors retrospectively reviewed patients with a diagnosis of unilateral HFM whom underwent unilateral MDO with an oblique vector at age 4 to 14 years at a single institution from 2004 to 2019. Patients with pre- and post-MDO three-dimensional computed tomography scans of the upper airway within 12 months of distractor placement and removal, respectively, were included. Head and neck postures were determined by craniocervical, pitch, roll, and yaw angles. Pre- and post-operative pharyngeal airway volumes, pharyngeal surface area, minimum retropalatal cross-sectional areas (RP CSA) and retroglossal (RG) CSA and associated anteroposterior distances were measured using Mimics 22.0 (Materialise; Leuven, Belgium). Comparison was done using Kruskal-Wallis tests and linear mixed-effects models controlling for head and neck postures. RESULTS: Ten patients met inclusion criteria. Mean age at pre-distractor placement computed tomography scan was 99 ±â€Š35 months, and mean duration between pre- and post-surgery scans was 220 ±â€Š90 days. Head and neck posture were found to be significant predictors of all airway dimensions. After controlling for significant factors with fixed effects linear modeling, surface area was found to be significantly smaller in patients after MDO by 189.48 mm2 (F[10.8] = -3.47, P = 0.0053), compared to their preoperative measurements. Surgery was not a significant predictor of changes in airway volume (F[11.6] = 0.52, P = 0.61), minimum RP CSA (F[12.2] =  -0.64, P = 0.53), minimum RG CSA (F[12.6] =  -1.64, P = 0.13), RP anteroposterior distance (F[14.0] = 0.30, P = 0.77), or RG anteroposterior distance (F[20.0] =  -0.04, P = 0.97). CONCLUSIONS: Oblique vector MDO in patients with HFM is associated only with statistically significant changes in the surface area of the upper airway, and is not associated with statistically significant changes in dimensions like volume, CSA, or anteroposterior dimension. This is an important finding, as it may guide discussions surrounding risk/benefit ratio for MDO in childhood.

Forces Exerted in Craniofacial Distraction Osteogenesis.

BACKGROUND: The purpose of this study was to develop a methodology for quantifying linear forces of distraction osteogenesis, and thereafter apply this methodology to measure and compare distraction force magnitudes between cranial vault distraction osteogenesis (CVDO) and mandibular distraction osteogenesis (MDO). METHODS: Patients undergoing CVDO or MDO as inpatients had distraction forces acquired with a digital torque-measuring screwdriver. Torque measurements were then converted into linear distraction force values, which were then compared across distraction types and protocols with appropriate statistics. RESULTS: CVDO was performed on 7 patients (41.2%), and MDO was performed on 10 patients (58.8%). Across the entire cohort, the average maximum force per activation was 27.0 N, and the average elastic force was 10.7 N. Maximum force (CVDO: 52.9 N versus MDO: 12.9 N; P < 0.001) and elastic force (CVDO: 22.0 N versus MDO: 4.5 N; P < 0.001) were significantly higher in patients undergoing CVDO than MDO. Multivariate regression demonstrated that maximum activation force was significantly associated with sequential days of distraction (B= + 1.1 N/day; P < 0.001), distraction rate (B= + 8.9 N/mm/day; P = 0.016), distractor hardware failure (B= + 10.3 N if failure; P = 0.004), and distraction type (B= + 41.4 N if CVDO; P < 0.001). CONCLUSIONS: Cranial vault distraction requires significantly more linear distraction force than mandibular distraction. Maximum forces increase with each day of distraction, as well as with increased distraction rates. Linear distraction force methodology from this study may provide the foundation for future development of optimized procedure-specific or patient-specific distraction protocols.

Management of Segmental Tibial Bone Defects With a Motorized Intramedullary Bone Transport Nail: A Case Review With Follow-Up.

SUMMARY: Large segment bone defects of the tibia are challenging problems. Although caused by a wide range of conditions, tibial critical bone loss defects often require complex reconstructive plans with prolonged inability to weight-bear on the effected extremity. Reconstruction options frequently require harvesting of autograft leading to further morbidity. Distraction osteogenesis allows reconstruction of large segmental defects of the tibia while avoiding donor site morbidity. Historically, distraction osteogenesis of tibia was most reliably performed with circler ring external fixation. This process allowed early weight-bearing but unfortunately has considerable drawbacks. Negative effects include pin tract irritation and inability to wear normal clothes. The advent of the bone transport nail now allows management of tibial critical bone loss defects through distraction osteogenesis negating the need for external fixation. This new technique allows treatment of large segmental tibial defects by means of distraction osteogenesis with an all-internal device avoiding the negative effects of external fixation while simultaneously allowing early weight-bearing.

Craniometric and Volumetric Analysis of Posterior Vault Distraction Osteogenesis: 10 Year Update.

PURPOSE: The purpose of this study is to quantitatively establish the volumetric changes observed with posterior vault distraction osteogenesis in the anterior, middle, and posterior thirds of the cranial vault; characterize change in cranial length, width, and height, correlating these changes to demographic variables that may help identify why younger kids gain more volume; and describe the short-term position of the transport segment. METHODS: Multisuture craniosynostosis patients who underwent posterior vault distraction osteogenesis were retrospectively reviewed. Pediatric, dose-reduced, thin cut helically acquired head computed tomography scans were analyzed on Materialise Mimics v22 (Materialise, Ghent, Belgium). Pre and post-PVDO and "old" and "young" cohort were compared. RESULTS: Twenty-one patients met inclusion criteria. The change in cranial length was significantly less at a median of 14.9 mm [Interquartile range (IQR) 11.8, 31.6] compared to the X-ray measured AP distraction distance (30 mm [IQR 24, 33]; P < 0.001) and significantly less than the theoretically calculated distance (23 mm [IQR 19, 31]; P = 0.012). Median anterior cranial height was shorter post PVDO (82.9 mm, [IQR 64.8, 92.6] versus 78.7 mm [IQR 57.0, 88.7]; P = 0.030). The younger cohort saw a greater increase in total intracranial volume (median 335.1 mL [IQR 163.2, 452.3, median 37.1%] versus 144.6 mL [IQR 119.0, 184.8, median 12.0%]; P = 0.011). CONCLUSIONS: This is the first study to quantify volumetric changes to the anterior, middle, and posterior cranial vaults and demonstrates benefits of preforming PVDO at a younger age to help control turricephaly and produce greater percentage volumetric increases.

A Cohort Study of Strabismus Rates Following Correction of the Unicoronal Craniosynostosis Deformity: Conventional Bilateral Fronto-Orbital Advancement Versus Fronto-Orbital Distraction Osteogenesis.

BACKGROUND: The purpose of this prospective study is to compare perioperative morbidity and strabismus rates between traditional fronto-orbital advancement reconstruction (FOAR) and fronto-orbital distraction osteogenesis (FODO) in unicoronal craniosynostosis (UCS). METHOD: A consecutive group of 15 patients undergoing FODO for isolated UCS were compared to a contemporaneous group of 15 patients undergoing traditional FOAR for UCS. Patient age, operative time, blood loss, blood replacement, technical details of the surgery, length of stay, complications, and strabismus rates were documented and compared statistically using chi-square and Student t test with a significance value of 0.05. RESULTS: The 15 patients undergoing FODO were younger (6.3 and 9.8 months, P < 0.05), experienced less operative time for the initial procedure (111 versus 190 minutes, P < 0.01), less blood loss (26% versus 50% of total blood volume, P < 0.01), and less blood replacement (40% versus 60% of total blood volume, P < 0.05). One patient in the FODO group experienced a new-onset strabismus postoperatively compared with 5 in the FOAR group (P < 0.05). There were no complications requiring a return to the operating room in either group. CONCLUSIONS: Fronto-orbital distraction osteogenesis for the treatment of isolated UCS is associated with a favorable perioperative morbidity profile and a decreased incidence of postoperative strabismus compared with traditional FOAR. These positive factors are tempered by the need for an additional procedure for removal of the device and lack of long-term outcomes data on the technique.

Syndrome-related outcomes following posterior vault distraction osteogenesis.

PURPOSE: The most commonly occurring syndromic craniosynostoses are Apert syndrome, Crouzon syndrome, Pfeiffer syndrome, and Saethre-Chotzen syndrome. There is insufficient data regarding postoperative syndrome-related outcomes following the posterior vault distraction osteogenesis (PVDO) procedure, as well as data addressing whether or not additional procedures will be subsequently necessary to comprehensively treat children who undergo PVDO. Thus, the objective of this study is to describe and compare syndrome-related potential complications and outcomes associated with the PVDO procedure. METHODS: An observational retrospective study was performed on consecutive patients (n=24) with Apert syndrome, Crouzon syndrome, Pfeiffer syndrome, or Saethre-Chotzen syndrome, respectively, who underwent PVDO between 2012 and 2019. Demographic data (patient gender and age when the PVDO procedure was performed), diagnosis, surgery-related data, and outcome data (perioperative and midterm complications and need for additional surgery) were verified. RESULTS: Total relative blood transfusion volumes per kilogram for the patients were as follows: 22.75 ± 9.30 ml for Apert syndrome, 10.73 ± 2.28 ml for Crouzon syndrome (Apert versus Crouzon, p<0.05), 18.53 ± 8.08 ml for Pfeiffer syndrome, and 19.74 ± 9.12 ml for Saethre-Chotzen syndrome. None of the patients required a secondary procedure to alleviate intracranial pressure except for a Saethre-Chotzen patient. CONCLUSION: PVDO is an effective technique to address elevated intracranial pressure in SC patients that alleviates the need for secondary procedures at midterm follow-up. Apert syndrome patients presented relatively higher total blood transfusion rates than Crouzon syndrome patients who were operated on at a later age and weighed more.

Rib-based Distraction Device Implantation Before Age 3 Associated With Higher Unplanned Rate of Return to the Operating Room.

BACKGROUND: Surgical treatment of early-onset scoliosis (EOS) with rib-based implants such as the vertical expandable prosthetic titanium rib (VEPTR) is associated with a high rate of complications including surgical site infection, skin breakdown, and implant migration. Many of these complications warrant the need for unplanned reoperations, increasing the burden on an already vulnerable patient population, and introducing the further risk of infection. To provide insight into the risks of early intervention, we investigate the relationship between initial device implantation before the age of 3 and the rate of unplanned reoperation. METHODS: A retrospective review was performed of all patients at a single institution who had undergone VEPTR insertion for EOS with at least a 2-year follow-up from 2007 to 2016. Patients were stratified into the case-cohort (0 to 2 y of age) or the comparison cohort (3 to 10 y of age) based on age at the time of device implantation. Multivariate regression accounting for age and scoliosis etiology was performed to identify factors predictive of unplanned reoperation. RESULTS: A total of 137 of 185 patients treated with VEPTR were identified with 76 (56%) undergoing at least 1 unplanned reoperation during the study time period. There were 68 and 69 patients in the age 0- to 2-year and 3- to 10-year cohorts, respectively. Patients aged 0 to 2 years underwent a higher number of total procedures compared with those aged 3 to 10 (13.1±6.5 vs. 10.6±4.8, P=0.032). A significant difference was found in the rate of unplanned reoperation between the 2 cohorts with 44 (65%) patients aged 0 to 2 and 32 (46%) patients aged 3 to 10 undergoing at least 1 unplanned reoperation (P=0.031). Binary logistic multivariate regression accounting for age and scoliosis etiology demonstrated that patients aged 0 to 2 had a significantly greater odds of undergoing an unplanned reoperation (odds ratio=3.050; 95% confidence interval: 1.285-7.241; P=0.011) compared with patients aged 3 to 10 years. CONCLUSION: Overall, EOS patients aged 0 to 2 at initial VEPTR implantation are up to 3 times higher risk of undergoing an unplanned reoperation compared with those aged 3 to 10. LEVEL OF EVIDENCE: Level III.

Caso clínico: aproximación a la vía aérea difícil en neonatología. Síndrome de Treacher-Collins / Clinical case: approach to the difficult airway in neonatology. Treacher-Collins syndrome

Cuando los recién nacidos presentan obstrucción de la vía aérea, requieren un manejo urgente y experto para evitar la mortalidad y la morbilidad. La definición de vía aérea difícil se relaciona con problemas en la intubación endotraqueal o en la ventilación a presión positiva con bolsa y máscara o reanimador de pieza en T. El manejo debe basarse en la comprensión del mecanismo fisiopatológico responsable de la vía aérea difícil. Las causas en el recién nacido pueden ser congénitas y/o adquiridas.Se presenta el caso de una recién nacida con síndrome de Treacher-Collins tipo 1 [OMIM #154500] con una disostosis mandibulofacial, micrognatia, hipoplasia malar, paladar hendido, sin cardiopatía congénita, asociado con intubación extremadamente difícil

f newborns have an airway obstruction, they require urgent and expert management to avoid mortality and morbidity. The definition of difficult airway includes problems in endotracheal intubation or positive pressure ventilation with bag and mask or T-piece resuscitator. Management should be based on an understanding of the pathophysiological mechanism responsible for difficult airway. The causes of difficult airway in the newborn can be congenital or acquired.We present the case of a newborn with Treacher-Collins syndrome Type 1 [OMIM # 154500] with a mandibulofacial dysostosis, micrognathia, malar hypoplasia, cleft palate, without congenital heart disease, associated with extremely difficult intubation

Midface Morphology and Growth in Syndromic Craniosynostosis Patients Following Frontofacial Monobloc Distraction.

BACKGROUND: Facial advancement represents the essence of the surgical treatment of syndromic craniosynostosis. Frontofacial monobloc distraction is an effective surgical approach to correct midface retrusion although someone consider it very hazardous procedure. The authors evaluated a group of patients who underwent frontofacial monobloc distraction with the aim to identify the advancement results performed in immature skeletal regarding the midface morphologic characteristics and its effects on growth. METHODS: Sixteen patients who underwent frontofacial monobloc distraction with pre- and postsurgical computed tomography (CT) scans were evaluated and compared to a control group of 9 nonsyndromic children with CT scans at 1-year intervals during craniofacial growth. Three-dimensional measurements and superimposition of the CT scans were used to evaluate midface morphologic features and longitudinal changes during the craniofacial growth and following the advancement. Presurgical growth was evaluated in 4 patients and postsurgical growth was evaluated in 9 patients. RESULTS: Syndromic maxillary width and length were reduced and the most obtuse facial angles showed a lack in forward projection of the central portion in these patients. Three-dimensional distances and images superimposition demonstrated the age did not influence the course of abnormal midface growth. CONCLUSION: The syndromic midface is hypoplastic and the sagittal deficiency is associated to axial facial concavity. The advancement performed in mixed dentition stages allowed the normalization of facial position comparable to nonsyndromic group. However, the procedure was not able to change the abnormal midface architecture and craniofacial growth.