Posterior Vault Distraction Osteogenesis Research Articles

Distraction osteogenesis in craniosynosotosis: technical note

Introduction: Craniosynostosis is a group of diseases whose main characteristic is the premature fusion of one or more cranial sutures. They can be subdivided into non-syndromic and syndromic. The posterior vault distraction osteogenesis (PVDO) has become the standard procedure for managing the intracranial hypertension of these patients. The objective of this paper is to report the experience of the Sobrapar Hospital (Campinas, Brazil) in treating and following up on syndromic craniosynostosis patients subjected to PVDO and highlight the lessons learned throughout this process. Methods: The medical records of the patients treated at the Sobrapar Hospital between 2013 and 2023 were reviewed. Results: The total of patients initially candidates to PVDO was 79, and 75 of them proceeded to the surgery. The mean age of surgery was 8,16 months old (ranging from 4 months to 12 years old). The length of stay ranged from 2 to 14 days, with a mean of 3,29 days. The distraction achieved ranged from 13 mm to 30 mm, with a mean distance of 23 mm. The postoperative complications were mostly cutaneous. CSF leak occurred in 3 patients. Conclusion: PVDO provides a progressive formation of a vascularized bone, its intracranial volume expansion is greater than the FOA, increasing the posterior fossa’s size (even when the craniotomy is supratorcular) and it improves Chiari’s symptoms, even when the radiologic abnormalities are not necessarily solved. A specialized and multidisciplinary team is essential to improving the success rates of the surgery.

Changes in Ventricular Volume After Posterior Vault Distraction Osteogenesis in Patients With Syndromic and Nonsyndromic Craniosynostosis.

Little is known about the response of the ventricular system to cranial vault surgery in patients with craniosynostosis. This study aims to evaluate the changes in the cerebral ventricular system in response to posterior vault distraction osteogenesis (PVDO) in patients with syndromic and nonsyndromic craniosynostosis. A single-institution retrospective review of all patients with craniosynostosis undergoing PVDO from 2000 to 2022 was completed. Patients were included for analysis if they had pre and postoperative cranial computed tomography scans. Ventricular volume (VV) and intracranial volume (ICV) were calculated using segmentation software. Both patients with syndromic synostosis and nonsyndromic synostosis (NSS) experienced a significant increase in ICV after PVDO, but only patients with NSS experienced a significant VV change ( P = 0.004). After normalization by ICV, total, lateral, and third VV changes retained significance with percentage increases of 114%, 117%, and 89%, respectively ( P < 0.05 for all). The differing results between cohorts reinforce the concept that the intracranial milieu is different between patients with syndromic synostosis and NSS. The results of the NSS cohort suggest that these patients may exist in a compensated state in which a reduction in cerebral blood flow and VV allows for the maintenance of parenchymal health to prevent the development of intracranial hypertension. Further studies may explore VV as a surrogate marker of ICP elevation, and the utility of cranial vault remodeling on nonsynostotic pathologies with cephalocranial disproportion.

Genetic Heterogeneity, Craniofacial Surgical Burden, and Surgical Techniques in Patients With Saethre-Chotzen Syndrome.

While genotype correlates with phenotype in patients with many forms of syndromic craniosynostosis, the relationship between molecular diagnosis and craniofacial surgical history in patients with Saethre-Chotzen syndrome (SCS) is more variable. This manuscript characterizes that relationship and evaluates operative trends in these patients over the past 3 decades. Demographic information, molecular diagnosis, and craniofacial surgical history in patients born with SCS between 1989 and 2023 were compared with appropriate statistics, including t tests and analysis of variance. Thirty-five patients with SCS were included, and there was no difference in total craniofacial procedures among those with TWIST1 substitutions (2.1 ± 1.6), duplications (3.0 ± 4.2), insertions (3.5 ± 0.7), or deletions (2.4 ± 1.9; P = 0.97). Cranial expansion rates were also similar across all genetic diagnoses (P>0.05), and surgical incidence was similar across patients with unicoronal, bicoronal, and multisuture involvement (P > 0.05). Those with an initial fronto-orbital advancement had a lower incidence of secondary cranial vault procedures compared with those with an initial posterior vault distraction osteogenesis (29% versus 71%, P < 0.05), though this did not control for phenotypic severity. On average, total cranial vault surgical burden (1.35 ± 0.67 versus 1.75 ± 0.46) and cranial expansion surgical burden (1.40 ± 0.68 versus 1.88 ± 0.64) between the fronto-orbital advancement-first and posterior vault distraction osteogenesis-first cohorts were similar (P = 0.11, P = 0.17, respectively). While SCS is molecularly and phenotypically heterogeneous, genetic diagnosis does not appear associated with rates of craniofacial surgery. Additional prospective study of correlations between genotype, severity of craniofacial manifestations, and treatment algorithms is warranted; but, in the end, it may be that this highly variable form of syndromic craniosynostosis warrants tailored, expectant management.

Vaulting further: cranial vault expansion for craniocerebral disproportion without primary craniosynostosis.

Treatment of subjects with refractory idiopathic intracranial hypertension (IIH) or shunted hydrocephalus with chronic shunt complications is challenging. What is the role for cranial vault expansion, particularly utilizing posterior vault distraction osteogenesis (PVDO), in these cases? This study assesses medium-term efficacy of cranial vault expansion in this unique patient population. A retrospective review was conducted of patients who underwent cranial vault expansion from 2008 to 2023 at the Children's Hospital of Philadelphia. Subjects who did not have a diagnosis of primary craniosynostosis were included in the study. Demographic information, medical history, and perioperative details were collected from medical records. Primary outcomes were the rate of CSF diversion procedures and resolution of presenting signs and symptoms. Secondary outcomes were perioperative and 90-day complications and reoperation requirement. Among 13 included subjects, nine (69.2%) patients had a primary diagnosis of shunted hydrocephalus and 4 (30.8%) patients had IIH. Twelve (92.3%) subjects underwent posterior vault distraction osteogenesis (PVDO) and one (7.7%) underwent posterior vault remodeling (PVR). All 4 patients with IIH demonstrated symptomatic improvement following PVDO, including resolution of headaches, vomiting, and/or papilledema. Among 9 patients with shunted hydrocephalus, CSF diversion requirement decreased from 2.7 ± 1.6 procedures per year preoperatively to 1.2 ± 1.8 per year following cranial vault expansion (p = 0.030). The mean postoperative follow-up was 4.1 ± 2.1years and four (30.8%) patients experienced complications within 90days of surgery, including infection (n = 2), CSF leak (n = 1), and elevated ICP requiring lumbar puncture (n = 1). Four (30.8%) patients underwent repeat cranial vault expansion for recurrence of ICP-related symptoms. At most recent follow-up, 7 of 9 patients with shunted hydrocephalus demonstrated symptomatic improvement. Cranial vault expansion reduced intracranial hypertension-related symptomology as well as the rate of CSF diversion-related procedures in patients with refractory IIH and shunted hydrocephalus without craniosynostosis, and should be considered in those who have significant shunt morbidity.

Early posterior vault distraction osteogenesis changes the syndromic craniosynostosis treatment paradigm: long-term outcomes of a 23-year cohort study

BackgroundEarly surgical management of syndromic craniosynostosis varies widely between centers, with patients typically undergoing multiple intracranial procedures through childhood. This study evaluates the long-term impact of early posterior vault distraction osteogenesis (PVDO) versus conventional treatment paradigms on the number and timing of subsequent craniofacial procedures.MethodsWe retrospectively analyzed the longitudinal operative patterns of patients with syndromic craniosynostosis treated from 2000 to 2023 with greater than five years of follow-up. Outcomes of patients who underwent early PVDO and conventional vault reconstruction were compared.ResultsFifty-five patients met the inclusion criteria (30 PVDO and 25 conventional). Age at initial vault surgery was similar between the PVDO and conventional cohorts (7.6 vs. 8.8 months), as were baseline craniometrics (p > 0.05). Multiple fronto-orbital advancement (FOA) procedures were performed in only 1/30 (3%) PVDO-treated patient versus 12/25 (48%) conventionally-treated patients (p < 0.001). Twelve (40%) PVDO-treated patients did not undergo FOA at all. Among patients with Apert and Crouzon syndromes, fewer PVDO-treated patients required FOA prior to midface surgery (33% vs. 92%, p = 0.004) or repeat FOA (6% vs. 50%, p = 0.005) compared to conventionally-treated patients. Among patients with Muenke and Saethre–Chotzen syndromes, a similar proportion of patients required FOA in the PVDO and conventional cohorts (91% vs. 100%, p = 0.353), though no PVDO-treated patients required repeat FOA (0% vs. 44%, p = 0.018).ConclusionsEarly PVDO is associated with a reduction in the high burden of both FOA and, critically, revisionary frontal procedures which are commonly seen in conventionally-treated patients with syndromic craniosynostosis.

Distraction Osteogenesis and Endoscopic Suturectomy in the Treatment of Rachitic Craniosynostosis: A Case Series and Updated Literature Review With Discussion of Cephalometrics

Background: Individuals with genetic or nutritional rickets frequently suffer from craniosynostosis. While cranial vault remodeling is most often performed, other approaches, such as distraction osteogenesis and endoscopic suturectomy, are infrequently reported. Here, we discuss the status of the current literature and describe 2 patients with rachitic craniosynostosis, one of whom had previously undergone endoscopic suturectomy, who both successfully underwent distraction osteogenesis. Case description: Two male patients with rickets-associated craniosynostosis, 1 genetic and 1 nutritional, underwent posterior-vault distraction osteogenesis (PVDO). The patient with genetic rickets previously underwent endoscopic-assisted cranial suturectomy of the sagittal suture with barrel-staving at an outside hospital and subsequently re-fused all cranial sutures 19 months postoperatively necessitating reintervention. Complete distraction was confirmed by X-ray and both patients recovered without complication and without re-synostosis at 18 month follow-up. The patient with nutritional rickets required subsequent fronto-facial advancement because of poor midface growth. Conclusion: PVDO effectively treats rachitic craniosynostosis associated with both genetic and nutritional etiologies. Long-term follow up is warranted to monitor for skull-base involvement and associated midface retrusion in these patients.

Posterior Vault Distraction in the Acute Setting.

Posterior vault distraction osteogenesis (PVDO) has been shown to resolve acute intracranial hypertension (AIH) while carrying an acceptable perioperative morbidity profile. PVDO has been associated with symptomatic improvement and fewer surgeries in those requiring ventriculoperitoneal shunts. The authors' experience using PVDO as an acute intervention is presented, demonstrating its safety and efficacy for management of AIH. Four cases of children with craniosynostosis that underwent PVDO in the acute setting are outlined. All patients presented with papilledema and symptoms of AIH. One patient with slit ventricle syndrome (SVS) presented with a nonfunctioning shunt following multiple shunt revisions. No intraoperative complications during distractor placement or removal were reported. Distraction protocol for all patients began on postoperative day 1 at 1-2 mm per day, resulting in an average total distraction of 30.25 mm. For the 3 cases with no shunt, the average length of stay was 7 days. As part of the planned treatment course, the patient with SVS required externalization of the shunt during distraction followed by early distractor removal and planned shunt replacement. One case of surgical site infection (in an immunocompromised patient) required premature distractor removal during the consolidation period. Computed tomography (CT) in all patients indicated increased intracranial volume following distraction, and symptomatic improvement was reported. Six-month follow-up showed resolution of papilledema in all patients. The authors' experience using PVDO in the acute setting is reported, alongside a review of current literature, in order to provide supporting evidence for the efficacy of PVDO as a tool for resolving AIH.

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

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. 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. The average patient age when PVDO was performed was 12.91 ± 10 months. The average posterior advancement distance achieved per patient was 22.68 ± 5.26mm. The average hospital stay per patient was 3.56 ± 2.44 days. The average absolute and relative blood transfusion volumes were 98.47ml and 17.63ml/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. 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.

D36. Early Posterior Vault Distraction Osteogenesis is changing the Syndromic Craniosynostosis Treatment Paradigm: Outcomes of A 20-year Cohort Study

D36. Early Posterior Vault Distraction Osteogenesis is changing the Syndromic Craniosynostosis Treatment Paradigm: Outcomes of A 20-year Cohort Study

Impact of Anterior and Posterior Vault Distraction Osteogenesis (A-PVDO) and 3D-Printed Positioning/Shaping Templates in Apert Syndrome: A Case Series Study.

This study seeks to examine the impact of anterior and posterior vault distraction osteogenesis (A-PVDO) in conjunction with 3D-printed positioning and shaping templates for the management of Apert syndrome. From January 2018 to February 2022, a retrospective analysis was conducted on 6 cases of Apert syndrome employing fronto-orbital 3D-printed positioning and molding templates. The cranium underwent surgical modification in accordance with the template's configuration and was affixed with absorbable plates. Subsequently, distraction devices were applied, encompassing both anterior and posterior craniotomies. The evaluation encompassed clinical outcomes, complications (including cerebrospinal fluid leakage and infection), safety, and the feasibility of the distraction osteogenesis procedure. Six patients diagnosed with Apert syndrome underwent treatment involving the integration of fronto-orbital 3D-printed positioning and shaping templates in conjunction with anterior and posterior cranial distraction osteoplasty. Follow-up durations ranged from 18 to 32 months (average: 22mo). No instances of fronto-orbital retraction, cerebrospinal fluid leakage, or intracranial infection were noted during the follow-up period. The sole reported complication entailed an infection at the extension rod site in 1 case. All patients conveyed satisfaction with the treatment outcomes. The application of 3D-printed positioning and shaping templates in tandem with anterior and posterior cranial distraction osteogenesis demonstrates efficacy in addressing Apert syndrome. Notably, significant enhancements in head shape and orbit were observed, and the incidence of postoperative complications such as cerebrospinal fluid leakage and infection remained minimal. Moreover, long-term follow-up affirmed stability.

The Use of Resorbable Fixation for Posterior Cranial Vault Distraction Osteogenesis

Summary: Posterior vault distraction osteogenesis (PVDO) traditionally relies on the use of cranial distractors affixed with titanium screws to the posterior cranial vault transport segment to allow for expansion of intracranial volume. We describe a novel technique using the Synthes RAPIDSORB IPS resorbable fixation system for stable fixation of the distraction hardware for PVDO. In a retrospective review of our experience with the IPS system, there was no instance of hardware or fixation failure. This off-label use of the IPS system results in a significant reduction in operative time for hardware removal while allowing for a more limited dissection, which can help reduce devascularization of the bony regenerate, bony relapse, and other complications.

Effects of Posterior Vault Distraction Osteogenesis on Ventricular Morphology and Volume

Effects of Posterior Vault Distraction Osteogenesis on Ventricular Morphology and Volume

Oxycephaly-systematic review, case presentation, and diagnostic clarification.

Oxycephaly is a specific phenotype of multi-suture craniosynostosis that is often misrepresented. This study aims to review the relevant literature, clarify the diagnostic criteria, and present an alternate approach to its management. Published literature regarding oxycephaly was reviewed from 1997, when the largest series was published, until 2022. All cases at a single institution were then retrospectively reviewed. Over the last 25years, four studies met the inclusion criteria, none of which specifically defined oxycephaly. One case, in one study, was potentially consistent with the phenotype. An institutional review yielded two patients who met the original diagnostic criteria set forth by Renier and Marchac. Both patients had unexplained speech delays, mild retinal nerve fiber layer thickening, and diffuse inner table scalloping, along with the characteristic oxycephalic phenotype. One patient also had a direct intracranial pressure (ICP) measurement of 25mmHg, and the other had a Chiari I malformation. Both were treated with posterior vault distraction osteogenesis (PVDO) to alleviate the cephalo-cranial disproportion while simultaneously allowing for turricephaly correction. Oxycephaly presents with late onset multi-suture fusion. Patients have patent sutures at birth. Midface hypoplasia and known syndromic associations are absent. Patients demonstrate supraorbital recession, an obtuse fronto-nasal angle, and turricephaly without substantial brachycephaly. Over 60% of patients have symptomatic ICP elevation, the presentation of which ranges from headaches to rapidly progressive blindness. This rare form of craniosynostosis is particularly virulent and likely often missed due to diagnostic ambiguity and its relatively mild phenotype.

Prior fronto-orbital advancement associated with complications from transcranial midface surgery in patients with syndromic craniosynostosis.

Our center adopted posterior vault distraction osteogenesis (PVDO) as a first-line intervention for cranial expansion in syndromic craniosynostosis in 2008, and we have a growing cohort of patients undergoing transcranial midface advancement who have not had prior fronto-orbital advancement (FOA). The purpose of this study was to evaluate whether a history of FOA influences the risk profile of transcranial midface advancement in patients with syndromic craniosynostosis. Patients undergoing transcranial fronto-facial advancement from 2000 to 2022 were retrospectively divided into cohorts based on preceding history of fronto-orbital advancement (FOA- and FOA+). Perioperative outcomes including operative time, length of stay, intraoperative dural injury, and complications (Clavien-Dindo score) were compared between groups with appropriate statistics. Thirty-eight patients were included (15 in FOA- group and 23 in FOA+ group). The overall complication rate was 47% (10% minor, 37% major). Compared to the FOA- group, the FOA+ group had a higher incidence of dural tears (65% v 20%, p = 0.006) and major complications (48% v 13%, p = 0.028). These findings were recapitulated in multivariate logistic regression controlling for other predictors. Prior FOA is associated with increased rates of major complications and dural tears in patients with syndromic craniosynostosis undergoing fronto-facial surgery. Options for cranial vault expansion that avoid the frontal region, such as PVDO, may favorably alter the risk profile of fronto-facial advancement.

Behavioral, Learning Skills, and Visual Improvement in Crouzon Syndrome Patient Following Late Posterior Vault Distraction Osteogenesis.

It is well accepted among craniofacial surgeons that surgery does not improve mental status but does prevent worsening or deterioration of cognitive and mental function. In this report, we describe significant improvement in behavioral, learning skills and visual acuity for a Crouzon patient who underwent late posteriorvault distraction osteogenesis. A 9-year-old Crouzon patient was referred to our hospital, presenting severe intracranial hypertension. The patient had previously undergone a strip craniectomy in early infancy at another medical institution, but there was no subsequent follow-up. Magnetic resonance imaging showed Chiari type I malformation and fundoscopy revealed papilledema. At the time of referral, the patient was not attending a regular school, had not acquired reading skills, was unable to concentrate, and could not accomplish school-related tasks that were standard for children in his age group. The patient underwent posterior vault distraction osteogenesis and showed concentration improvement and acquisition of fluent reading skills. Chiari type I malformation resolved as well as papilledema. visual acuity improved at Snellen test preoperatively from 20/200 to 20/60 at postoperative test. Ventricle size remained unchanged subsequent to performance of the posterior cranial vault distraction. The occipital veins were less visible upon clinical examination and less pronounced when palpated, and the bruit had completely disappeared. Late posterior vault distraction osteogenesis can improve behavioral, learning skills and visual acuity as shown in this 9-year-old Crouzon patient.

How Low Should We Go? Safety and Craniometric Impact of the Low Occipital Osteotomy in Posterior Vault Remodeling.

A larger volume cranial vault expansion is likely facilitated by a low posterior cranial osteotomy beneath the torcula; however, this may impart an increased risk of venous bleeding. The authors compared the safety of infratorcular versus supratorcular osteotomy in patients undergoing posterior vault reconstruction or posterior vault distraction osteogenesis and analyzed volumetric changes. Patients undergoing initial posterior vault reconstruction or distraction osteogenesis between 2009 and 2021 at the authors' institution were grouped by occipital osteotomy location and analyzed retrospectively. Craniometric analysis was performed if patients had high-resolution computed tomography scans available within 180 days preoperatively and postoperatively. A total of 187 patients were included: 106 (57 percent) who underwent posterior vault distraction osteogenesis and 81 (43 percent) who underwent posterior vault reconstruction. Infratorcular osteotomy was more common in reconstruction [ n = 65 (80 percent)] than in distraction osteogenesis [ n = 61 (58 percent); p < 0.002]. Blood transfused was similar between low and high osteotomy cohorts in the distraction osteogenesis ( p = 0.285) and reconstruction ( p = 0.342) groups. However, median transfused blood volume per kilogram of patient weight was greater in the low versus high osteotomy distraction osteogenesis ( p = 0.010) and reconstruction ( p = 0.041) cohorts. Intraoperative venous sinus injury was rare. In the distraction osteogenesis cohort, there was increased median intracranial volumetric gain in the low (263 ml) compared with the high osteotomy cohort (127 ml; p = 0.043); however, when controlled for distraction distance, only a trend was observed ( p = 0.221). Patients undergoing distraction osteogenesis showed a larger median intracranial volume increase (168 ml) compared with those undergoing reconstruction (73 ml; p < 0.001). Infratorcular osteotomy can be performed safely in most patients undergoing cranial vault remodeling and does not appear to be associated with greater hemodynamic instability or sinus injury. Therapeutic, III.

Ten Years of Posterior Cranial Vault Expansion by Means of Distraction Osteogenesis: An Update and Critical Evaluation.

The goal of this study was to describe the 10-year evolution of the authors' surgical technique and institutional perioperative outcomes using posterior vault distraction osteogenesis (PVDO) in patients with syndromic and multisuture craniosynostosis. The authors performed a retrospective cohort study of patients who underwent PVDO for treatment of syndromic and multisuture craniosynostosis at a single institution over a 10-year period. Demographic data, perioperative outcomes, distraction patterns, and complications were analyzed. Outcomes of patients in the first 5 years (early cohort) were compared with those of the latter 5 years (late cohort). One hundred ten patients underwent a total of 118 PVDO procedures. Patients with a syndromic diagnosis represented 83.6 percent of the cohort ( n = 92) and were significantly younger than patients with a nonsyndromic diagnosis at the time of first PVDO (median [25th percentile, 75th percentile] 14.1 [6.6, 40.1] versus 42.7 [15.2, 59.6] months; p = 0.014). Mean distraction distance in the anterior-posterior direction was 30.8 mm (SD, 7.4). Compared with the early cohort, PVDO in the late cohort had faster median operative times (144.0 [123.0, 189.0] minutes versus 161.0 [138.0, 199.0] minutes; p = 0.038), lower estimated blood loss as a percentage of blood volume (28.5 [20.6, 45.3] versus 50.0 [31.1, 95.8]; p < 0.001), and lower blood replacement as percentage of blood volume (39.5 [23.8, 59.1] versus 56.3 [37.8, 110.1]; p = 0.009). This 10-year experience with PVDO demonstrates continued overall safety and efficacy with improved perioperative outcomes over time. Although PVDO has become the authors' first line of expansion in syndromic craniosynostosis, shortcomings such as need for device removal, infection concerns, and potential for cerebrospinal fluid leak merit attention by the craniofacial community. Therapeutic, III.

Posterior vault distraction osteogenesis in Apert syndrome

Apert syndrome is an uncommon autosomal dominant condition with a varied clinical spectrum which includes premature cranial and facial suture fusion and complex upper and lower limb syndactilies. In this manuscript, we share our posterior vault distraction osteogenesis (PVDO) experience regarding Apert syndrome patients so that a larger patient population may benefit. In addition, we aimed to determine an onset of elevated intracranial pressure in the long term follow up.&#x0D; A retrospective study was performed on consecutive patients with syndromic craniosynostosis who were diagnosed with Apert syndrome, underwent PVDO between 2012 and 2019, and received at least 2 years of follow-up care. Demographic data, diagnosis, surgery related data, and outcome data (perioperative and mid-term complications and need for additional surgery), were verified by means of medical records, clinical photographs, radiographic images, and interviews with all included patients and/or their parents.&#x0D; The present study included 14 patients who were diagnosed respectively with Apert syndrome (n = 14). The average posterior advancement distance achieved was 19.37 ± 3.0 mm. Two patients included in this study presented major complications, but neither experienced permanent sequelae or a lethal course. Two Apert patients presented cerebral spinal fluid (CSF) leakage (Clavien–Dindo type II). One patient presented a onset of elevated intracranial pressure at 4 years of follow up.&#x0D; PVDO is an effective procedure at alleviating intracranial pressure and present a low complication rate.&#x0D; &#x0D; &#x0D;

Posterior Vault Distraction Outcomes in Patients With Severe Crouzon Syndrome Resulting from Ser347Cys and Ser354Cys Mutations.

In this study, the authors present the outcomes of 4 patients with a severe form of Crouzon syndrome characterized by mutation of fibroblast growth factor receptor 2 (FGFR2) c.1040 C > G p.Ser347Cys or the pathogenic c.1061C > G p.Ser354Cys variant of FGFR2, who underwent posterior vault distraction osteogenesis (PVDO) to alleviate elevated intracranial pressure. Patients with diagnosed Crouzon syndrome who were found by genetic testing to have an FGFR2 c.1040 C > G p.Ser347Cys mutation or the c.1061C > G p.Ser354Cys variant were included. Outcome data and presence of hydrocephalus, Chiari Malformation type I (CMIs), and the presence/absence of a tracheostomy were recorded. Three patients with the FGFR2 c.1040 C > G p.Ser347Cys mutation and 1 with the pathogenic FGFR2 c.1061C > G p.Ser354Cys variant were identified as having characteristics of severe Crouzon syndrome. The mean age at PVDO was 15 months and the mean posterior advancement was 20 mm. All 4 patients experienced sufficient relief of the elevated intracranial pressure from the PVDO to prevent the need for shunt placement, stabilize the ventricular dimensions (n = 2), and resolve the CMIs (n = 2). Intracranial pressure screening ruled out malignant cerebrospinal fluid volume increase. PVDO effectively prevented hydrocephalus and resolved CMIs, successfully alleviating intracranial pressure and maximizing clinical outcomes for patients with severe Crouzon syndrome.

Transverse Distraction Osteogenesis for Posterior Narrowing of the Cranial Vault.

Although conventional posterior vault distraction osteogenesis is known to achieve the greatest improvement in intracranial volume, anteroposterior expansion of the cranial vault is not always ideal in certain head shapes. Transverse posterior vault distraction osteogenesis is an alternative reconstructive strategy in patients presenting with recalcitrant multisuture craniosynostosis resulting in scaphocephaly with posterior narrowing. The authors present a multimedia demonstration of placing the cranial hinge points at the superior anterior aspect of the bone segments in order to achieve increased intracranial volume and significant improvement in posterior head shape, while preserving normal anterior skull dimensions.