Complex Craniosynostosis Research Articles

Application of Computerized Surgical Planning in Craniosynostosis Surgery.

Craniosynostosis, a medical condition characterized by premature fusion of one or multiple cranial sutures, has historically been treated through surgical correction. Computerized Surgical Planning (CSP) and three-dimensional (3D) modeling have gained significant popularity across craniofacial surgery. Through a collaborative effort between surgeons and engineers, it is now possible to virtually execute a surgical plan based on preoperative imaging using computed tomography scans. The CSP workflow involves several elements including virtual 3D modeling, CSP computer-aided surgical guide design, manufacturing of guides and templates, and intraoperative implementation. Through the gradual optimization of this workflow, it has been possible to achieve significant progress in the surgical process including improvements in the preoperative planning of complex craniosynostosis cases and reduction of intraoperative time. Furthermore, CSP and 3D modeling have had a positive impact on surgical simulation and residency training, along with patient education and counseling. This article summarizes the CSP workflow in the treatment of craniosynostosis and the implications of this treatment modality on medical trainee education and patient management.

Craniofacial Surgery in a Low-Resource Setting: The Successes, Challenges, and Prospects

Background:Craniofacial Trauma and Anomalies affect a substantial proportion of the global society, especially those in the low-and middle-income countries (LMICs). The literature stresses the concept of a team approach for craniofacial surgery and the need for the interaction of many specialists to provide optimal care for the patient. Craniofacial surgery is practiced mainly in the advanced countries of Europe and America. This report describes the establishment of craniofacial care in an African country. It reports on 6-year early outcomes of craniofacial surgery in a low-resourced country, counting the successes, challenges, and prospects. Methods: The establishment of an international collaboration that facilitated the training in craniofacial surgery is described. A 6-year retrospective review of craniofacial surgery cases managed by the Multidisciplinary Cleft and Craniofacial team at the Komfo Anokye Teaching Hospital (KATH) was done from January 2016 to December 2022. Results: Two Ghanaian surgeons were trained in Alabama, USA (a maxillofacial surgeon and a neurosurgeon), then returned to Kumasi to begin a Craniofacial practice with the help of the craniofacial team in Children of Alabama Birmingham Al, USA. A total of 646 procedures were performed (616 cases of orofacial cleft, 20 cases of craniofacial anomalies, and 10 cases of craniofacial trauma involving the cranial vault or base of the skull). Ninety percent of the cases were cleft lip and palate, with the remainder being a mixture of isolated and syndromic craniosynostosis, craniofacial trauma, encephalocele, and craniofacial fibrous dysplasia. Conclusion: The Commonest craniofacial procedure performed was cleft lip and palate repair. Other surgeries were for the repair of velopharyngeal incompetence (VPI) and atypical facial clefts. Complex craniosynostosis and encephaloceles are also now being repaired at KATH. The foundation for a sub-regional center of excellence in cleft and craniofacial surgery is being laid in Ghana at KATH.

The Role of Virtual Surgical Planning in Surgery for Complex Craniosynostosis.

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. 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. 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%). 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.

A unique presentation of Crouzon-like syndrome: Complex craniosynostosis in the absence of genetic mutations or familial predisposition – A case report

Background: Crouzon syndrome is a rare genetic disorder characterized by premature fusion of skull sutures during skull development, resulting in various craniofacial abnormalities and complex craniosynostosis is a condition in which more than one such sutures of the skull fuse prematurely. Case Description: Herein, we present a case of a 5-year-old male diagnosed with Crouzon-like syndrome and complex craniosynostosis involving multiple cranial sutures, including metopic, sagittal, coronal (right and left), and lambdoid sutures, and without any identifiable mutations on karyotyping. The patient underwent successful surgical intervention with a satisfactory outcome, highlighting the importance of early diagnosis and intervention to prevent or minimize associated neurological manifestations and craniofacial abnormalities. Conclusion: Our case report underscores the involvement of multiple cranial sutures in complex craniosynostosis and the absence of identifiable mutations or family history of similar craniofacial abnormalities, providing important insights into the diagnosis and management of this condition.

Multisutural cranial reconstruction in a case of Crouzon syndrome with complex craniosynostosis and no mutations: A case report

Multisutural cranial reconstruction in a case of Crouzon syndrome with complex craniosynostosis and no mutations: A case report

EP08.27: Prenatal diagnosis of complex craniosynostosis and right cleft lip: a case report

EP08.27: Prenatal diagnosis of complex craniosynostosis and right cleft lip: a case report

The Use of 3D Printed Models for Surgical Simulation of Cranioplasty in Craniosynostosis as Training and Education.

The advance in imaging techniques is useful for 3D models and printing leading to a real revolution in many surgical specialties, in particular, neurosurgery. We report on a clinical study on the use of 3D printed models to perform cranioplasty in patients with craniosynostosis. The participants were recruited from various medical institutions and were divided into two groups: Group A (n = 5) received traditional surgical education (including cadaveric specimens) but without using 3D printed models, while Group B (n = 5) received training using 3D printed models. Group B surgeons had the opportunity to plan different techniques and to simulate the cranioplasty. Group B surgeons reported that models provided a realistic and controlled environment for practicing surgical techniques, allowed for repetitive practice, and helped in visualizing the anatomy and pathology of craniosynostosis. 3D printed models can provide a realistic and controlled environment for neurosurgeons to develop their surgical skills in a safe and efficient manner. The ability to practice on 3D printed models before performing the actual surgery on patients may potentially improve the surgeons' confidence and competence in performing complex craniosynostosis surgeries.

Comparison of Internal and External Distraction in Frontofacial Monobloc Advancement: A Three-Dimensional Quantification.

Crouzon syndrome is characterized by complex craniosynostosis and midfacial hypoplasia. Where frontofacial monobloc advancement (FFMBA) is indicated, the method of distraction used to achieve advancement holds an element of equipoise. This two-center retrospective cohort study quantifies the movements produced by internal or external distraction methods used for FFMBA. Using shape analysis, this study evaluates whether the different distraction forces cause plastic deformity of the frontofacial segment, producing distinct morphologic outcomes. Patients with Crouzon syndrome who underwent FFMBA with internal distraction [Hôpital Necker-Enfants Malades (Paris, France)] or external distraction [Great Ormond Street Hospital for Children (London, United Kingdom)] were compared. Digital Imaging and Communications in Medicine files of preoperative and postoperative computed tomographic scans were converted to three-dimensional bone meshes and skeletal movements were assessed using nonrigid iterative closest point registration. Displacements were visualized using color maps and statistical analysis of the vectors was undertaken. Fifty-one patients met the strict inclusion criteria. Twenty-five underwent FFMBA with external distraction and 26 with internal distraction. External distraction provides a preferential midfacial advancement, whereas internal distractors produce a more positive movement at the lateral orbital rim. This confers good orbital protection but does not advance the central midface to the same extent. Vector analysis confirmed this to be statistically significant ( P < 0.01). Morphologic changes resulting from monobloc surgery differ depending on the distraction technique used. Although the relative merits of internal and external distraction still stand, it may be that external distraction is more suited to addressing the midfacial biconcavity seen in syndromic craniosynostosis. Therapeutic, III.

Practical Algorithm for the Management of Multisutural Craniosynostosis with Associated Chiari Malformation and/or Hydrocephalus

Introduction: The association between multisutural craniosynostosis with Chiari malformation (CM), venous hypertension, and hydrocephalus is widely described in the literature, especially in children with paediatric craniofacial syndromes. Some efforts have been done in the last years to understand the complex pathogenetic mechanisms underlying this association, and several theories have been proposed. In particular, it is now accepted that the hypothesis of the overcrowding of the posterior fossa due to precocious suture fusion is the cause of the cerebellar herniation in syndromic and non-syndromic patients, against the theory of intrinsic cerebellar anomalies, ventriculomegaly, and venous hypertension. However, whatever the pathophysiological mechanism, it is still unclear what the best management and treatment of CM and hydrocephalus are in multisutural craniosynostosis patients. The aim of this study was to report our 25 years’ experience in treating paediatric patients affected by these rare pathologies in order to propose a simple and effective therapeutic flow chart for their management. Materials and Methods: We retrospectively collected data of each patient who underwent a cranial vault remodelling (CVR) for complex multisutural craniosynostosis in our institution in the last 25 years, while monosutural craniosynostosis was excluded. We recorded data concerning type of craniosynostosis and craniofacial syndromes, presence of ventriculomegaly, and CM at presentation and clinical and radiological follow-up. Therefore, we evaluated the final outcomes (improved, stable, deteriorated) of these patients and created a practical flow chart that could help physicians choose the best surgical treatment when different pathological conditions, as Chiari malformation I (CMI) or hydrocephalus, affect complex craniosynostosis children. Results: Thirty-nine patients (39 out of 55; 70.9%), with an isolated multisutural craniosynostosis at presentation, underwent a two-step CVR as first surgery; 36 patients (92.3%) had an improved outcome, 2 patients (5.1%) had a stable outcome, and 1 patient (2.56%) had a deteriorated outcome. Other eight children (8 out of 55; 14.5%) had a radiological evidence of asymptomatic CMI at presentation. In this group, we performed CVR as first surgery. As for the final outcome, 7 patients had an improved outcome (87.5%) with good aesthetic result and stability or resolution of CMI. Finally, 7 patients (7 out of 55; 12.7%) presented a various combination of CMI and ventriculomegaly or hydrocephalus at presentation. Among them, 3 patients had an improved outcome (42.8%), and 4 patients had a deteriorated outcome (57.1%). Discussion: The prevalence of one pathological condition with associated symptoms over the others was the key factor leading our therapeutic strategy. When craniosynostosis is associated with a radiological CM, the assessment of clinical symptoms is of capital importance. When asymptomatic or pauci-symptomatic, we suggest a CVR as first step, for its efficacy in reducing tonsillar herniation and solving CM symptoms. When craniosynostosis is associated with ventricular enlargement, the presence of intracranial hypertension signs and symptoms forces physicians to first treat hydrocephalus with a ventriculo-peritoneal shunt or endoscopic third ventriculostomy. For patients with various degrees and severity of ventriculomegaly and associated CM, the outcomes were very heterogeneous, even when the same therapeutic strategy was applied to patients with similar starting conditions and symptoms. This is maybe the most unexpected and least clear part of our results. Despite the proposed algorithm comes from a clinical experience on 85% successfully treated patients with multiple craniosynostosis, more extensive and deep studies are needed to better understand CM and hydrocephalus development in such conditions.

10. Comparison of Internal and External Distraction in Frontofacial Monobloc Advancement: A Three-Dimensional Quantification

Crouzon syndrome is characterised by complex craniosynostosis and midfacial hypoplasia. Where frontofacial monobloc advancement (FFMBA) is indicated, the method of distraction used to achieve advancement holds an element of equipoise. This two-centre retrospective cohort study quantifies the movements produced by internal or external distraction methods used for FFMBA. Using shape analysis, this study evaluates if the different distraction forces cause plastic deformity of the frontofacial segment, producing distinct morphological outcomes.

The impact of obstructive sleep apnea on growth in patients with syndromic and complex craniosynostosis: a retrospective study

Given the high prevalence of OSA in children with syndromic and complex craniosynostosis (SCC) and the consequences of untreated OSA, it is important to assess their nutritional status and growth. Yet, literature regarding growth in children with SCC remain scarce. Therefore, this study aimed to (1) illustrate the growth pattern in SCC, (2) determine the impact of OSA on this growth pattern, and (3) evaluate the effect of surgical treatment of OSA on growth over time. A retrospective study was performed in children with SCC, who were treated at the Dutch Craniofacial Center (Rotterdam, Netherlands). Growth variables (height, weight, weight-for-age standard-deviation-score (SDS), weight-for-height SDS, and height-for-age SDS) and degree of OSA (obstructive apnea–hypopnea index) were assessed. Of the 153 children with SCC, 38 (25%) were acutely malnourished at some point during follow-up, of whom 21 had disease-related acute malnutrition. Children with moderate-severe OSA had significant lower weight-for-height SDS compared to children without OSA (p = 0.0063). Growth parameters (weight-for-age SDS, weight-for-height SDS, height-for-age SDS) in children with SCC without OSA were not impaired as they did not differ from the normal healthy population, with exception of the patients with Saethre-Chotzen syndrome (SCS) who had a significantly lower SDS for height-for-age. Conclusion: Children with SCC have a substantial chance of developing acute malnutrition at some point during growth. Additionally, in children with moderate-severe OSA, a significant lower SDS for weight-for-height is present, indicating the importance of assessing the weight and growth pattern in children who are clinically suspected for OSA.What is Known:• Obstructive sleep apnea is seen in up to two-thirds of the children with syndromic and complex craniosynostosis.• Presence of obstructive sleep apnea is associated with intracranial hypertension and an increased risk of metabolic, cardiovascular, and neurocognitive consequences later in life. Untreated obstructive sleep apnea may lead to impaired growth and weight gain, which can result in growth failure.What is New:• Craniosynostosis patients with moderate-severe obstructive sleep apnea had significant lower weight-for-height standard deviation scores (SDS), compared to children without obstructive sleep apnea. • Children with syndromic and complex craniosynostosis without OSA did not significantly differ from the normal healthy population in regard to weight-for-age SDS, weight-for-height SDS, and height-for-age SDS.

Posterior Calvarial Distraction in older paediatric population: single centre paediatric neurosurgery craniofacial unit outcomes.

Posterior calvarial distraction (PCD) is a safe and effective technique used to increase cranial vault volume and therefore reduce intracranial pressure in children with complex craniosynostosis. Optimal timing and method used for PCD is controversial. This procedure is usually performed in children younger than 2years. Literature regarding calvarial distraction in older children is sparse and limited. We report our single-centre experience with PCD in children aged 6 and above to outline the applications, benefits and challenges of employing this technique in an older paediatric population. A retrospective analysis of a database on craniofacial cases from 2006 to 2021 was performed. Patients undergoing PCD were identified and children aged 6 and above at the time of operation were included. Data on demographics and clinical outcomes were obtained from electronic records and relevant imaging was reviewed. All cases were reviewed prior to a decision for surgery by the multidisciplinary craniofacial team (composed of neurosurgery, maxillofacial and plastics teams) and underwent surgery in our paediatric craniofacial centre. Overall, 98 PCD cases were identified during the study period, of which 20 cases were identified as having undergone PCD at age 6 or above with mean age of 8.8years (range 6-18). The most common indication was pansynostosis associated with raised intracranial pressure. Four cases had calvarial remodelling previously and represented with symptoms of raised intracranial pressure sometime after their initial surgery requiring PCD as rescue procedure. Average duration of inpatient stay was 5.85days. The average duration of follow-up was 3.5years (0.3 to 11years). Mean distraction distance achieved was 22.5mm (18-29mm). Five patients experienced complications related to wound infection or distractor. Follow-up assessment in all patients demonstrated evidence of vault expansion and symptomatic improvement and resolution of intracranial pressure signs. Comparison with younger cohort did not reveal any difference in any parameters except lower rate of transfusion in the older cohort compared to young cohort (5% vs 38%). Posterior calvarial distraction in older children is safe and effective for vault expansion and treatment of raised intracranial pressure in selected cases. A multidisciplinary craniofacial team approach is crucial for appropriate case selection and management in order to optimise outcomes.

Comparison of Perioperative Anesthetic Concerns in Simple and Complex Craniosynostosis Cases: A Retrospective Study

Abstract Background We compared intraoperative and postoperative anesthetic parameters between simple (S-single suture) and complex (C-more than one suture) craniosynostosis cases. Materials and Methods Data was collected from a retrospective review of anesthetic and postoperative records of patients who underwent craniosynostosis correction surgery between April 2014 and August 2019. Pearson’s chi-square and independent sample t-tests were used for analysis. Results We analyzed data of 98 patients (S-simple craniosynostosis: 40 and C-complex craniosynostosis: 58). Statistically significant differences between simple and complex cases were seen only in the incidence of difficult airway, failed extubation, and requirement of postoperative ventilation. Massive blood loss (S: 23.21 mL/kg; C: 22.71 mL/kg) and difficult airway (S: 2.04%; C: 19.39%) were the most common intraoperative adverse events encountered. Metabolic abnormalities, hemodynamic instability, and hypothermia occurred in few patients. The most common postoperative issue was pyrexia (S: 15.31%; C: 17.35%). Anemia and coagulopathy needing transfusion of blood products and vitamin K injection were seen in a significant number of cases. Three nonsyndromic patients developed seizures. Conclusion The incidence of difficult airway and failed extubation with postoperative ventilation is more frequent following surgery for complex craniosynostosis than simple craniosynostosis. Complications secondary to blood loss and airway issues were the primary cause of morbidity and mortality. A staged approach to surgical management resulted in decreased surgical blood loss among the children with complex craniosynostosis.

Quantified Retinal Morphology and Its Association With Papilledema and Visual Acuity in Syndromic and Complex Craniosynostosis: An Optical Coherence Tomography Study

This study is the first to evaluate the prevalence of retinal thinning and the correlation with papilledema and visual acuity (VA) in a large population with craniosynostosis. Prospective clinical cohort study. All and complex patients syndromic and complex with craniosynostosis who visited the only national referral center between 2018 and 2020 were included. Retinal layers were segmented using optical coherence tomography. Patients were seen by an ophthalmologist for VA assessment and fundoscopy. Multivariate regression models were developed to evaluate correlations between retinal thickness, papilledema and VA. We included 127 patients. Retinal thinning was most prevalent in the peripapillary retinal nerve fiber layer (≤38%). A longer duration of papilledema in early childhood correlated with an increased peripapillary retinal nerve fiber layer and total retinal thickness optic nerve head later in life (+0.3 ± 0.2, P=.04 and +1.0 ± 1.0, P=.003); however, its thickness was not correlated with the VA (P=.20 and P=.53). Macular retinal thinning was associated with a worse VA (P=.01); however, it was not correlated with the duration of papilledema (P=.95). Following a preventative treatment strategy for syndromic and complex craniosynostosis, the prevalence of retinal ONH thinning is low. Although the prevalence of peripapillary retinal nerve fiber layer thinning is considerable, its thickness is not correlated with VA. In contrast, macular thinning is correlated with worse VA scores and should, therefore, be evaluated during follow-up. Future studies should evaluate the (1) causative mechanism for macular thinning, (2) correlation between the time to surgery and macular thinning, and (3) results of reactive treatment strategies and compare those results to the current study.

Venous anomalies in hypoplastic posterior fossa: unsolved questions.

Anomalous intracranial venous anatomy is described in patients with syndromic craniosynostosis andis of significant importance when it comes to surgical morbidity. However, it is still controversial its origin, type ofcirculation in each syndrome, how it behaves over time, when it can be interrupted and wether it needs to bestudied. The purpose of this paper is to discuss these issues by reviewing the literature. A literature search was performed using the PubMed database with a focus on papers including detaileddescriptions of the venous outflow in complex and syndromic craniosynostosis. Search details used were thefollowing: ("veins"[MeSH Terms] OR "veins"[All Fields] OR "venous"[All Fields]) AND ("abnormalities"[Subheading]OR "abnormalities"[All Fields] OR "anomalies"[All Fields]) AND syndromic[All Fields] AND ("craniosynostoses"[MeSH Terms] OR "craniosynostoses"[All Fields] OR "craniosynostosis"[All Fields]). Studies that exposed details ofvenous anomalies found in syndromic or complex craniosynostosis were selected. Of a total of 211 articles found, 11 were selected for this review. Of these, 5 were case reports, 5retrospective studies, and only 1 prospective study. From the 6 series of cases presented, 5 discussed therelationship between jugular foramen stenosis (JFS) and collateral venous drainage. The authors discuss data fromthe literature for each leading question presented: 1-collateral circulation: is it an intrinsic trouble, a consequenceof stenosis of the cranial base foramina or related to raised intracranial pressure (ICP)?; 2-what venousanomalies should we search for, and what is the best exam to study them?; 3-collateral circulation changes withtime?; 4-can neurosurgeons interrupt the collateral circulation?; 5-should we study all complex types ofcraniosynostosis? The importance of the study of the venous outflow in patients with complex craniosynostosis is evidentin the literature. The real relationship between intracranial hypertension, hypoplastic skull base foramen, Chiari Imalformation, hydrocephalus, and venous collateral circulation remains unknown. Prospective studies focusing onmolecular biology analysis will possibly solve all of these leading questions.

Complex craniosynostosis in the context of Carpenter's syndrome.

Carpenter's syndrome or acrocephalopolysyndactyly type II is a rare genetic autosomal recessive disease, with an incidence estimated at 1 per 1 million births. Common findings of a brachydactyly, polysyndactyly, and a trefoil-like skull with extreme brachycephaly due to fusion of the bilateral coronal, sagittal and lambdoid sutures. We report a 12-month-old male who was referred to our care for evaluation of a craniofacial deformity-a trefoil-like skull, flattened and receding forehead, bulging of temporal bones, hypertelorism, exorbitism, and polysyndactyly in the upper and lower limbs and psychomotor delay. Head computed tomography (CT) with 3D reconstruction revealed craniosynostosis with fusion of the coronal, metopic, and sagittal sutures. Correction of the craniofacial deformity was performed with satisfactory aesthesis of the craniofacial bones at 2years of follow-up. Early correction of craniofacial deformity in Carpenter's syndrome is usually safe within 6 to 12months. Venous drainage abnormalities and ectatic emissary veins can lead to significant bleeding and may be detected on MR angiography. Significant skull weakening may lead to bony fragmentation while creating cranial flaps and is best evaluated with 3D CT imaging. Taking these pitfalls into consideration decreases the chances of aborting the surgery and may lead to better overall outcomes.

A Neonate with an Abnormally Shaped Head

A female newborn has an abnormally shaped head (Figs 1 and 2). Figure 1. Profile of infant. Figure 2. View of top of infant’s head. ### Prenatal and Birth Histories ### Presentation (Day 1) The infant’s respiratory distress resolved 2 hours after birth and she transitioned off CPAP. Dysmorphic features were noted on physical examination at 2 days of age and hence, further evaluation was undertaken. ### Vital Signs ### Physical Examination (Day 2)

Morphological and surgical results in sagittal synostosis: early craniectomy versus later cranioplasty.

Morphological correction is one of the main aims of surgery for sagittal synostosis (SSO). Different surgical techniques have been developed; however, few studies have compared the different surgical protocols. The morphological outcome is poorly documented, because a consensual evaluation tool is lacking. We performed a prospective study of children operated for SSO in our institution. Children were operated whenever possible at 4 months for craniectomy; by default, children underwent cranioplasty at or after 9 months. The morphological outcome of all children was evaluated using traditional craniometry with head circumference (HC) and the cephalic index (CI), and with the Rotterdam scaphocephaly morphology score (RSMS), a total of semi-quantitative assessments of morphological hallmarks. Craniectomy was significantly associated with a shorter operation time and hospital stay, and a better impact on HC and CI measurements, compared with cranioplasty. The RSMS was markedly improved after surgery in both groups; however, we found no significant difference in improvement between the two groups. Although the transfusion rate and the prevalence of developmental delay were lower in the craniectomy group, and reoperations for calvarial lacunae or complex craniosynostosis occurred only this group, these differences were not significant. Our results support early surgery with craniectomy whenever possible; however, cranioplasty at a later age is a very acceptable by-default indication. In addition to classical craniometry, morphological evaluation using the RSMS or a similar quantitative scale appears highly desirable for future studies.

Posterior Cranial Vault Distraction Osteogenesis Utilizing a Posterior-Superior Distraction Vector in the Treatment of Mercedes Benz Pattern Craniosynostosis.

Bilateral lambdoid and sagittal synostosis, or Mercedes Benz Syndrome, is a rare complex craniosynostosis resulting in frontal bossing, a tapered posterior fossa, and an anteriorly displaced cranial vertex. Its ideal surgical correction must result in posterior expansion, skull elongation, and caudal repositioning of the vertex. We present a craniometric analysis of skull changes with posterior-superior distraction and introduce a novel craniometric measure: vertex position. In this study, a retrospective review was performed to analyze outcomes of posterior cranial vault distraction osteogenesis (PVDO) using a posterior-superior distraction vector from 2016 to 2019. Cranial vertex position was measured as a fraction of the occipitofrontal diameter from rostral to caudal (0-1.0). Four patients underwent PVDO at mean age 10.61 ± 3.16 months utilizing a posterior-superior distraction vector. Linear distraction distance averaged 30.30 ± 0.90 mm with a mean consolidation period of 3.98 ± 0.72 months. Mean corrected change in intra-cranial volume was 236.30 ± 3.71 mL, at an average rate of 7.81 ± 2.00 mL/mm of distraction. Increases in anterior cranial height (7.83 ± 2.51 mm), middle cranial height (8.43 ± 4.21 mm), posterior cranial height (13.15 ± 7.45 mm), and posterior cranial fossa height (21.99 ± 8.55 mm) were observed. Cranial vertex demonstrated a mean posterior movement of 0.18 ± 0.13. PVDO utilizing a posterior-superior distraction vector for management of nonsyndromic bilateral lambdoid and sagittal synostosis effectively increases intracranial volume and height and provides an esthetic outcome with posterior movement of the cranial vertex.

Serial Posterior Cranial Vault Distraction for the Treatment of Complex Craniosynostosis.

Posterior cranial vault distraction is an important modality in the management of craniosynostosis. This surgical technique increases intracranial volume and improves cranial aesthetics. A single procedure is often inadequate in patients with complex multisuture craniosynostosis, as some will go on to develop intracranial hypertension despite the operation. Considering the negative effects of intracranial hypertension, some patients may warrant 2 planned distractions to prevent this scenario from ever occurring. Three patients with complex multiple-suture synostosis and severe intracranial volume restriction (occipital frontal head circumferences [OFCs] <1st percentile) were treated with 2 planned serial posterior cranial vault distractions at the institution between 2013 and 2018. Demographics, intraoperative data, and postoperative distraction data were collected. The OFC was recorded pre- and postdistraction, at 3- and 6-month follow-up appointments. Patients had a corrected average age of 18 weeks at the time of their initial procedure. There was an average of 38 weeks between the end of consolidation and the time for their 2nd distraction procedure. There was an average age of 79 weeks at the time of the 2nd procedure. All patients had a substantial increase in OFC and improvement of the posterior calvarium shape. The average increase in OFC was 5.2 cm after first distraction and 4.3 cm after 2nd distraction. No postoperative complications were encountered. Planned serial posterior cranial vault distraction is a safe and effective strategy for increasing intracranial volume, improving aesthetic appearance, and preventing the consequences of intracranial hypertension in patients with multisuture craniosynostosis and severe intracranial volume restriction.