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.

[Optic canal stenosis in Crouzon syndrome: a case report and literature review]. / Stenoz zritel'nykh kanalov pri sindrome Kruzona: klinicheskii sluchai i obzor literatury.

BACKGROUND: Incidence of Crouzon syndrome is 1 per 25.000-31.000 newborns. This syndrome is extremely rarely accompanied by optic canal stenosis. OBJECTIVE: To present a patient with Crouzon syndrome and optic canal stenosis, to discuss the management of such patients considering own and literature data. MATERIAL AND METHODS: A 6-year-old boy presented with Crouzon syndrome (verified by molecular genetic research, i.e. FGFR2 gene mutation). The patient underwent 3 surgeries for craniosynostosis and hydrocephalus. Nevertheless, visual acuity progressively decreased despite patent ventriculoperitoneal shunt. Examination revealed severe decrease in visual functions with optic disc congestion under secondary atrophy. MRI data on subarachnoid CSF accumulation over both optic nerves potentially indicated optic canal stenosis. This assumption was confirmed by 3D CT. RESULTS: The patient underwent decompression of both optic canals with subsequent improvement of visual functions. CONCLUSION: Vision decrease following Crouzon syndrome may be due to optic canal stenosis. Decompression may be effective, even in long-term course of disease, and improve visual functions.

Morphology of the Occipital Bones and Foramen Magnum Resulting From Premature Minor Suture Fusion in Crouzon Syndrome.

To identify skull-base growth patterns in Crouzon syndrome, we hypothesized premature minor suture fusion restricts occipital bone development, secondarily limiting foramen magnum expansion.Skull-base suture closure degree and cephalometric measurements were retrospectively studied using preoperative computed tomography (CT) scans and multiple linear regression analysis.Evaluation of multi-institutional CT images and 3D reconstructions from Wake Forest's Craniofacial Imaging Database (WFCID).Sixty preoperative patients with Crouzon syndrome under 12 years-old were selected from WFCID. The control group included 60 age- and sex-matched patients without craniosynostosis or prior craniofacial surgery.None.2D and 3D cephalometric measurements.3D volumetric evaluation of the basioccipital, exo-occipital, and supraoccipital bones revealed decreased growth in Crouzon syndrome, attributed solely to premature minor suture fusion. Spheno-occipital (ß = -398.75; P < .05) and petrous-occipital (ß = -727.5; P < .001) suture fusion reduced growth of the basioccipital bone; lambdoid suture (ß = -14 723.1; P < .001) and occipitomastoid synchondrosis (ß = -16 419.3; P < .001) fusion reduced growth of the supraoccipital bone; and petrous-occipital suture (ß = -673.3; P < .001), anterior intraoccipital synchondrosis (ß = -368.47; P < .05), and posterior intraoccipital synchondrosis (ß = -6261.42; P < .01) fusion reduced growth of the exo-occipital bone. Foramen magnum morphology is restricted in Crouzon syndrome but not directly caused by early suture fusion.Premature minor suture fusion restricts the volume of developing occipital bones providing a plausible mechanism for observed foramen magnum anomalies.

The foramen magnum in scaphocephaly.

PURPOSE: The foramen magnum (FM) presents various alterations in craniosynostoses, such as brachycephaly or Crouzon syndrome. However, to date, no study has been devoted to its morphology and morphometry in scaphocephaly, which is the most common of cranial deformities resulting from premature fusion of cranial sutures. METHODS: We assessed the morphology and morphometry of FM using preoperative thin-cut CT scans of 107 children with non-syndromic sagittal craniosynostosis aged 1-12 months (mean age 5.38 months). A series of sagittal and transverse dimensions were taken and the FM area was calculated in each case. Obtained data were compared to the age-matched control group of 101 normocephalic children. RESULTS: Dolichotrematous type of FM was dominant in the scaphocephaly group and observed in 63/107 cases (58.9%). The mean FM area in the scaphocephaly group was 519.64 mm2 and was significantly smaller compared to the control group (p = 0.0011). The transverse diameter and anterior sagittal diameter were also significantly smaller (p = 0.0112 and p = 0.0003, respectively). CONCLUSION: The area of FM in scaphocephaly is smaller compared to normal individuals. This is associated with a significant reduction of the width of FM in children with sagittal craniosynostosis. FM in scaphocephaly is larger than in other reported series of children with brachycephaly or Crouzon syndrome.

Robot-assisted frontofacial correction in very young children with craniofacial dysostosis syndromes: a technical note and early functional outcome.

OBJECTIVE: In this study, the authors aimed to 1) retrospectively analyze the early functional outcomes in a cohort of very young children with craniofacial dysostoses who underwent robot-assisted frontofacial advancement (RAFFA) or robot-assisted midface distraction (RAMD), and 2) analyze the utility of robotic assistance in improving the accuracy and safety of performing transfacial pin insertion for RAFFA or RAMD. METHODS: A retrospective analysis of a cohort of 18 children (age range 1-42 months at presentation), who underwent RAFFA or RAMD from February 2015 to February 2021 in the craniofacial unit at Amrita Institute of Medical Sciences and Research Centre in Kochi, India, was performed. Inclusion criteria were patients who had undergone RAFFA in a single stage or RAMD where the cranial vault had been addressed earlier, had been addressed on follow-up, or had not been addressed and had follow-up of at least 6 months. RESULTS: Overall, 18 children with syndromic craniosynostosis underwent LeFort level III midface distraction, with or without RAFFA, from February 2015 to February 2021 at a single center in India. The patients' ages ranged from 6 to 47 months at the time of the procedure. All patients had significant obstructive sleep apnea (OSA), significant ocular issues, and disturbed sleep as determined by the authors' preoperative protocol. Clinically significant intracranial pressure issues were present in 17 patients. None of the patients had injury due to the transfacial pin trajectory such as globe injury, damage to the tooth buds, or the loss of purchase during the active distraction phase. The mean distraction achieved was 23 mm (range 18-30 mm) (n = 16/18). Of the 18 patients, 10 (56%) had an excellent outcome and 6 (33%) had a satisfactory outcome. In all cases, the degree of OSA had significantly reduced after surgery. Eye closure improved in all patients, and complete closure was seen in 11 patients. On follow-up, the functional gain remained in 14 of 16 patients at the final follow-up visit. The distraction results were stable during the follow-up period (mean 36 months [range 6-72 months]). CONCLUSIONS: The early RAFFA and RAMD protocols investigated in this study gave a significant functional advantage in very young patients with craniofacial dysostoses. The results have demonstrated the accuracy and safety of robotic assistance in performing transfacial pin insertion for RAFFA or RAMD.

Crouzon's Syndrome with a Dominant Sinus Pericranii Draining Transverse Sinus: Report of a Rare Association and Review of Literature.

INTRODUCTION: Crouzon's syndrome and sinus pericranii (SP) are rare entities. Only few cases having both the features are reported. SP most commonly drains in relation to superior sagittal sinus and their communication to major posterior dural sinuses is rare. CASE REPORT: We report a rare case of Crouzon's syndrome with SP at a suboccipital location with termination of left transverse sinus into the SP draining further through the extracranial suboccipital and extravertebral cervical venous plexi into external jugular veins. Distal transverse sinus and sigmoid sinus on the left side were absent. CONCLUSION: Crouzon's syndrome with SP is an extremely rare entity. SP with communication to major posterior dural venous sinuses is also rare and mostly associated with multi-suture craniosynostosis. Management depends on the volume of venous blood they are draining. Most of them are dominant type and their occlusion is not feasible. Preoperative diagnosis of a dominant SP is essential for proper surgical planning as it needs to be preserved mandatorily to prevent cerebral venous infarction.

Monobloc Differential Distraction Osteogenesis.

ABSTRACT: Midface advancement at the monobloc level can be the seminal life event for patients with craniofacial dysostosis. Monobloc reconstruction, when planned appropriately, can simultaneously and definitively address multiple functional and aesthetic deficiencies in these patients. The application of distraction has reduced the morbidity experienced with traditional monobloc surgery. The purpose of this study is to report on the outcomes, stability, and growth in younger patients after monobloc advancement in syndromic craniosynostosis patients. The authors report a consecutive series of thirty patients with craniofacial dysostosis treated through monobloc differential distraction osteogenesis. Detailed history, photographic, and long-term radiographic data are reviewed, including a subset of patients who were skeletally immature at the time of their treatment. Differential distraction allows control of midface pitch, roll, and yaw, optimizing functional and aesthetic outcomes. There were no infectious complications requiring reoperation. The average surgical age for all patients was 12.5 years. For the 7 patients age <7 years, average age was 6 years. For all patients, the mean horizontal movement was 12 mm at nasion and 10 mm at A-point. At mean follow-up (4.8 years entire group and 6.2 years age <7 years group) a positive horizontal advancement of 1.1 mm at nasion and 0.8 mm at A-point was observed. More pronounced positive horizontal changes were seen in the age <7 years group. Monobloc differential distraction osteogenesis affords safe and precise repositioning of the midface. The advancement is skeletally stable and young patients show moderate continued growth.

Correction of Midface Deficiency in Patient With Crouzon Syndrome by Orthognathic Surgery and Patient Specific Facial Implant: Case Report.

ABSTRACT: Crouzon syndrome, also known as craniofacial dysostosis, is an autosomal dominant inherited disease characterized by early cranial fusion and consequential craniofacial malformations. In patients with Crouzon syndrome, the growth of the midface is affected due to early fusion of the cranial base, which results in exophthalmos, ocular ptosis, midface deficiency, and maxillary retrognathism. Frontofacial advancement using Le Fort III osteotomy is the conventional method for treating patients with Crouzon syndrome. However, this procedure has drawbacks such as extensive operation field and high possibility of serious complications (eg, meningitis). For patients with mild symptoms, facial esthetics and proper occlusion can be promoted through conventional orthognathic surgery, but midface deficiency cannot be completely resolved. Thus, in this case report, midface augmentation was performed for a patient with Crouzon syndrome by undergoing conventional orthognathic surgery, along with patient-specific implants made using a 3D virtual technique. Implants were 3D printed using polyetherketoneketone and simultaneously implanted during the orthognathic surgery. After the surgery, not only were the patient's occlusion and facial esthetics improved, but also exophthalmos and ocular ptosis were reduced by the midfacial augmentation effect of patient-specific implants placed in the midface. Since the implants were made exactly as what surgeons have intended through computer-aided design (CAD)/computer-aided manufacturing (CAM) and 3D printing techniques, problems such as under-/over-correction were avoided. In addition, the possibility of implant malpositioning was minimized using surgical stents as implants were passively fitted on the patient's bone surface. This case verified that the application of 3D technology to the field of oral and maxillofacial surgery can reduce the invasiveness of surgery and improve the accuracy of the operation. Therefore, by using cutting-edge technologies, the field of oral and maxillofacial surgery is expected to be developed further in the near future.

Crouzon Syndrome Anatomy, Usefulness of Vestibular Orientation.

ABSTRACT: Spatial resolution of computerized tomographic scanner has reached a level to which accurate anatomic measurements could be done in. Three-dimensional accurate measurements require a reliable referential system. In craniology landmarks are usually selected in the skull base. For craniofacial malformation the classical landmarks are of no help so the authors have used the vestibular orientation to study a series of 50 Crouzon syndrome computerized tomographic scan and compare the results (shape, position, linear mensuration, volumes…) with 122 controls in unaffected patients. The authors have confirmed Crouzon description of a high level of polymorphism in phenotypes, the authors propose an organigram to understand the abnormal growth patterns in crouzon syndrome, which lead to such consequences. This polymorphism implies that the planning of surgical treatment should be tailored for each case.

Characterization of Treatment Modalities for Patients With Syndromic Craniosynostosis in Relation to Degree of Midface Hypoplasia and Patient's Age Using Longitudinal Follow-Up Data.

ABSTRACT: The purpose of this study was to investigate the type and frequency of use of treatment modalities (Tx-Mods) in patients with syndromic craniosynostosis (SC) using longitudinal follow-up data. A total of 28 patients with SC (24 Crouzon, 2 Apert, and 2 Antley-Bixler syndromes), who were treated at the Department of Orthodontics, Seoul National University Dental Hospital, Seoul, South Korea between 1998 and 2020, was included. According to the degree of midface hypoplasia (MH) at the initial visit (T1), the patients were divided into the mild-MH (78°≤SNA < 80°, n = 8), moderate-MH (76≤SNA < 78°, n = 7), and severe-MH (SNA < 76°, n = 13) groups. T1-age and Tx-Mods, including cal-varial surgery (CALS), orthopedic treatment (OPT), fixed orthodontic treatment, and midface advancement surgery in childhood (MAS-child) and adulthood (MAS-adult), were investigated. Complexity of MAS-adult was graded as follows: 0, no surgery; 1, orthognathic surgery; 2, distraction osteogenesis (DOG); 3, combination of distraction osteogenesis and orthognathic surgery. Then, statistical analysis was performed. Percentage distribution of Tx-Mods was 71.4% in CALS, 21.4% in MAS-child, 42.9% in OPT, 100% in fixed orthodontic treatment, and 89.3% in MAS-adult. 92.9% of patients underwent MAS more than once. The number of MAS increased according to the severity of MH ( P < 0.05). The complexity of MAS-adult increased as T1-age and severity of MH increased (all P < 0.05); whereas it decreased when CALS and OPT were performed (all P < 0.05). However, MAS in childhood did not guarantee the avoidance of additional MAS in adulthood ( P > 0.05). These findings may be used as basic guidelines for successful treatment planning and prognosis prediction in patients with SC.

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

BACKGROUND: 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. METHODS: 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. RESULTS: 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. CONCLUSION: Late posterior vault distraction osteogenesis can improve behavioral, learning skills and visual acuity as shown in this 9-year-old Crouzon patient.

Surgical Result and Identification of FGFR2 Variants Using Whole-Exome Sequencing in a Chinese Family With Crouzon Syndrome.

ABSTRACT: Crouzon syndrome is considered as one of the most common craniosynostosis syndromes with a prevalence of 1 in 65,000 individuals, and has a close relationship with variants in fibroblast growth factor receptor 2. Here the authors described a Crouzon syndrome case, which was asked for surgery treatment for the symptom of multisuture craniosynostosis. Mild midfacial retrusion, larger head circumference, proptosis, pseudo-prognathism, and dental malposition could also be found obviously. Then fronto-orbital advancement and cranial cavity expansion were performed to the child. After whole-exome sequencing (WES) and Sanger sequencing, gene variants in the exons 2 and 3 of FGFR2 were detected. And protein tyrosine 105 replaced by cysteine in the extracellular region of FGFR2 was also detected. After operation, she presented a satisfactory anterior plagiocephaly and scaphocephaly correction, and the result was satisfied by surgeons and her parents. Variants detected using WES have further research prospect.

An Elderly Patient With Crouzon Syndrome Treated With Monobloc Distraction.

Monobloc advancement by distraction osteogenesis is the treatment of choice in patients with syndromic craniosynostosis. This procedure is usually performed at 18 to 24 months/5 to 10 years of age. Herein, we present the case of a male patient with Crouzon syndrome who underwent monobloc advancement at the age of 62 years. Although the patient lived a normal life (employed, married, and being a father of a daughter), he visited our hospital for surgical improvement in facial esthetics. The patient underwent monobloc advancement by distraction osteogenesis. He was satisfied with the postoperative esthetic improvement and did not experience any major complications. This case highlights the fact that patients with syndromic craniosynostosis desire esthetic improvement and suggests that multidisciplinary treatment involving both the neuro and plastic surgeons is important in such cases.

Frontofacial Reconstruction Technique Modification With Preservation of Blood Supply to the Monobloc Segment.

ABSTRACT: Craniosynostosis syndromes, including Apert Syndrome, Pfeiffer Syndrome, and Crouzon Syndrome, share similar phenotypes, including bicoronal craniosynostosis, midface hypoplasia, hypertelorism, and exorbitism. The standard surgical treatment for these craniofacial abnormalities is monobloc osteotomy with distraction osteogenesis. Complications of this technique include the failure of osteogenesis or resorption of the frontal bone. The authors propose an alternative surgical technique with a frontal arch in continuity with the midface segment to ensure vascularization to anterior and posterior borders of distraction. A case report of an 8-year-old female patient with Apert Syndrome is reported using our technique. Our frontal arch monobloc distraction procedure preserves blood supply to a cranial component of the monobloc segment site that becomes the anterior portion of distraction rather than with the traditional devascularized frontal bone flap. This technique modification should improve osteogenesis outcomes by preventing resorption or failure of bone formation.

An Auxiliary Cranioparietal Anchorage System to Improve the Stability of the Rigid External Distraction Device in Children With Crouzon Syndrome.

ABSTRACT: Rigid external distraction (RED) device instability because of thin skulls in syndromic craniosynostosis patients remains challenging. The authors propose the use of an auxiliary cranioparietal anchorage system to improve RED device stability in patients with Crouzon syndrome. Five consecutive patients with Crouzon syndrome underwent Le Fort III osteotomy with midface advancement using a modified external device. Cephalometric analysis and three-dimensional computed tomography images were evaluated preoperatively and after device removal. Mechanical analyses of the RED device with and without the auxiliary cranioparietal anchorage system were performed. Both postoperative photographs and follow-up computed tomographs showed obvious midfacial advancement with no intracranial pin perforation or external frame migration. Mechanical analysis showed that the new system reduced the possibility of postoperative external frame migration. The auxiliary cranioparietal anchorage system might be considered an adjunct to the RED device in patients with Crouzon syndrome to avoid postoperative complications.

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

BACKGROUND: 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. METHODS: 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. RESULTS: 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. CONCLUSIONS: PVDO effectively prevented hydrocephalus and resolved CMIs, successfully alleviating intracranial pressure and maximizing clinical outcomes for patients with severe Crouzon syndrome.

Comprehensive management of Crouzon syndrome: A case report with three-year follow-up.

Crouzon syndrome is one of the most common craniosynostosis facial syndromes caused by a mutation in the fibroblast growth factor receptor 2 (FGFR2) gene. Less commonly, there is a mutation of the FGFR3 gene which results in Crouzon syndrome syndrome with acanthosis nigricans. It involves the premature fusion of sutures of the cranial vault, base, orbital and maxillary region. The clinical presentation of this congenital deformity depends on the pattern and timing of sutural fusion. The present report describes the features and management of this syndrome in an 18-year-old woman. The patient presented with a hypoplastic maxilla, deficient midface, exorbitism due to shallow orbits, severe crowding and bilateral crossbite. A multidisciplinary approach involving orthodontics and surgical intervention with distraction osteogenesis brought about marked improvement in the facial profile, occlusion and upper airway. The aesthetics and function were greatly enhanced, and the results were found to be stable at the end of three years.

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.

Crouzon syndrome: posterior fossa volume studies in vestibular orientation.

INTRODUCTION: With the increasing possibilities of surgical treatment addressing the complex spectrum of defects in Crouzon syndrome (CS), it is of utmost importance to obtain accurate assessment of the malformation. Evaluating the volume of the posterior fossa is of great importance since many authors, considering that its volume is always decreased, favor posterior fossa enlargement as a first step in the treatment of CS. MATERIAL AND METHODS: We studied CT scans of children with CS, which were vestibular-oriented (VO) in order to conform with physiological landmarks, and we measured intracranial volume by manual segmentation; these abnormal CT scans were superposed with VO 3D-CT scanners of age-matched controls. We studied the volume index of the posterior fossa for each CS patient defined as the ratio of its volume in CS patients with the normal for age calculated from normal controls. RESULTS: We studied the imaging of 41 children with CS and 70 control. Among CS patients, the volume of the posterior fossa was increased in 10, compared with control. We found closure of the sphenoidal synchondrosis was correlated with age and with the width of the posterior fossa, but not with its length nor with the posterior fossa volume index. CONCLUSION: Segmentation on VO-CT scanner and superposition with CT scanners of normal controls is a powerful tool for the study of the impact of CS or other synostoses on volume and shape. We found that CS is more heterogeneous than previously thought, and surgical strategies should be adapted accordingly.

Disappointing results of spring-assisted cranial vault expansion in patients with Crouzon syndrome presenting with sagittal synostosis.

The aim of this study was to report on a single center's experience with spring-assisted cranial vault expansion (SAE) in patients with Crouzon syndrome and sagittal suture synostosis. Strip craniotomy with SAE has resulted in successful outcomes with low complication and revision rates in patients with isolated scaphocephaly. However, recent experience suggests that outcomes in patients with Crouzon syndrome and sagittal synostosis (SS) who undergo SAE are less favorable compared with the outcomes of those who undergo frontobiparietal (FBP) expansion. The authors reviewed both operations performed at a single center and noticed an upward expansion of the skull, which may be related to ventriculomegaly, with concurrent intracranial hypertension and poor aesthetic outcome. All patients diagnosed with Crouzon syndrome and SS who were treated with SAE required a revision FBP operation. Based on this outcome, the authors consider Crouzon syndrome a contraindication for correcting SS with springs.