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Two types of metopic-sagittal synostosis. a Type 1, scaphocephalic type. Upper column: with the sagittal ridge (eight cases). Lower column: without the ridge (19 cases). b Type 2, non-scaphocephalic (eight cases total; no sagittal ridges evident). A metopic ridge was confirmed in all patients. c The smaller anterior fossa, the very thin and wider sphenoid ridge (arrows), and marked digital markings
Source publication
Purpose:
We have diagnosed 35 cases of the supposedly rare condition metopic-sagittal synostosis in the past 20 years. Here, we introduce their clinical symptoms, neuroradiological findings, and surgical treatment methods, as well as discuss the relevant literature.
Methods:
Subjects included 35 patients (33 boys and 2 girls; mean age 4.2 years;...
Citations
... Although our cohort included pediatric patients only and is not comparable with Morrison et al's study, the most affected sutures in our series were bicoronal, followed by sagittal, metopic, unicoronal, multiple, and bilateral lambdoid synostosis. 1 Previously published series have shown different ordinaries regarding the most affected sutures. [1][2][3][4][5][6] The most common type of craniosynostosis was sagittal synostosis (scaphocephaly), followed by coronal synostosis. [2][3][4][5][6] In the bicoronal synostosis (brachycephaly) cases, the coronal sutures on both sides of the child's head close too early. ...
... [1][2][3][4][5][6] The most common type of craniosynostosis was sagittal synostosis (scaphocephaly), followed by coronal synostosis. [2][3][4][5][6] In the bicoronal synostosis (brachycephaly) cases, the coronal sutures on both sides of the child's head close too early. Thus, the child's head will grow broad and short. ...
... Thus, the child's head will grow broad and short. 3,4,6 Pogliani et al reported 16 (57.1%) sagittal and 8 (28.6%) metopic synostoses among the true craniosynostosis group, who were first diagnosed with the USG. 5 Sloan et al reported 107 (42.8%) sagittal, 30 (12.0%) multiple suture, 30 (12.0%) unilateral lambdoid, and 28 (11.2%) ...
Craniosynostosis is a skull malformation occurring due to the premature fusion of one or more cranial sutures. This pathological entity is a relatively commonly observed congenital malformation and is reportedly seen in 1/1,700–1,900 live births. The study aimed to evaluate the surgical outcomes of the open cranial vault remodeling (OCVR) in children with craniosynostosis.
Medical records of 76 children with craniosynostosis who were diagnosed at the neurosurgery departments of our centers for 11 years (from January 2010 to December 2020) were retrospectively examined. Among them, 54 consecutive children who underwent OCVR were included in this study. Surgical outcomes were discussed with a related literature review.
Fifty-four (32 males and 22 females) consecutive children received OCVR for craniosynostosis with a mean age of 12.6 ± 7.1 months. Eight children were syndromic. Three children were shunt-induced craniosynostosis. Syndromic children were four with Apert, two with Pfeiffer, and two with Crouzon syndrome. Twelve children were brothers/sisters. The misshapen skull was the most commonly recorded symptom in 49 children (90.7%). The most affected sutures were bicoronal craniosynostosis found in 20 children. The complication rate was 9.3% ( n = 5). Two of these five children needed reoperation for optimal remodeling. One child died postoperatively in the intensive care unit due to cardiac arrest.
These findings demonstrated that the OCVR approach is an efficient surgical method to get good outcomes. Satisfactory results with an acceptable complication rate can be obtained with expert hands. Further studies are warranted to support these findings.
Scaphocephaly (SC) is defined as an elongation of the anteroposterior axis of the skull resulting from the abnormal fusion of the sagittal suture. This study describes the "Peau d'ours" technique and results for correcting SC. We conducted a consecutive and retrospective analysis of patients treated from 2011 to 2016. We evaluated the gender, age, and surgical outcomes. A total of 53 patients were enrolled with a mean age of 19 months old. The advantages of this technique are healthy coronal and lambdoid suture preservation and symmetrical parietal bone flap opening. This technique is safe and simple to reproduce, allowing good surgical outcomes with a low incidence of secondary craniosynostosis. This technique is ideal for patients older than six months old.
Combined sagittal and metopic craniosynostosis is an uncommon condition that may result in a wide spectrum of cranial morphologies. This occurs as a result of the compound effects of both prematurely fused sutures. The authors present an incidence of sagittal craniosynostosis with a specific altered head morphology, and delayed diagnosis that the authors believe occurred due to structural changes associated with concurrent metopic suture synostosis This patient underscores the limitations of relying on classic diagnostic patterns and highlights the critical role of noninvasive imaging in the diagnosis of craniosynostosis.
In addition to the clinical examination, ultrasonography (US), high-resolution computed tomography (CT), and magnetic resonance imaging (MRI) are the most important diagnostic tools to differentiate morphological changes of congenital midface anomalies in children. Preoperative treatment planning is often based on a combination of both cross-sectional imaging techniques to provide optimal care for complex reconstructive approaches. Ultrasonography and MRI as non-irradiating imaging modalities are preferred for prenatal and postnatal diagnostics. Despite exposure to X-rays, CT is the method of choice for imaging bony malformations of the midface and skull base including craniosynostosis and branchial arch diseases. However, tailored low flip angle gradient-echo MRI sequences may have the potential to replace CT for the diagnosis and monitoring skull deformities in infants. The strength of MR imaging lies in its high-resolution, multisequential soft tissue imaging, which is particularly useful for congenital midface masses, craniofacial syndromes, and craniocervical soft tissue malformations. The knowledge of characteristic cross-sectional imaging features together with the familiarity of normal anatomy, anatomical variants, and embryogenesis is critical if pathological conditions of the midface in children are to be distinguished. Characteristic imaging features of nonsyndromic and syndromic craniosynostosis, branchial arch diseases, and craniofacial soft tissue malformations are discussed.
Sagittal craniosynostosis is the most common of all craniosynostoses. Patients with sagittal craniosynostosis exhibit a typical cranial appearance, including scaphocephaly, and an elongated head, with a fused and ridged sagittal suture. Moreover, some recent reports described atypical sagittal craniosynostosis accompanied by autism, speech delay, and hyperactivity. This condition, known as delayed-onset craniosynostosis, is rarely reported, given that it is difficult to determine if the condition is congenital or has a delayed-onset.
This report describes the clinical course and treatment of 2 brothers with atypical sagittal synostosis. The shapes of their heads were not indicative of scaphocephaly and the younger brother exhibited delayed-onset sagittal synostosis. Their father and paternal grandmother exhibited similar cranial morphologies. Therefore, we hypothesized the involvement of a familial factor in the etiology of atypical sagittal synostosis in these patients.
Craniosynostosis with early closure of skull sutures is categorized either as simple or as syndromic if present with other congenital anomalies such as midface hypoplasia, syndactyly, or joint contractures. Some children have a single affected suture, whereas some have multiple affected sutures. Skull shape varies depending on the affected sutures and the treatment needs to be done in the period of skull growth, which depends on age and skull shape. No consensus has been established for treatment decisions. New less invasive treatment methods such as distraction osteogenesis and molding helmets have been developed, but unsolved problems continue to be present. Here we discuss chronological changes in treatment methods, current status and problems, treatment algorithms, and research strategies for pathophysiology and prevention.
Purpose:
The purpose of this study was to evaluate the indications, safety, and short-term outcomes of posterior vault distraction osteogenesis (PVDO) in patients with no identified acrocephalosyndactyly syndrome (study) and to compare those to a syndromic cohort (controls).
Methods:
Demographic and perioperative data were recorded and compared across the study and control groups for those who underwent PVDO between January 2009 and December 2016. Univariate analysis was conducted using χ and Fisher exact tests for categorical variables, and Mann-Whitney U test for continuous variables.
Results:
Sixty-three subjects were included: 19 in the nonsyndromic cohort, 44 in the syndromic cohort. The cohorts had similar proportion of subjects exhibiting pansynostosis (42.1% of nonsyndromic versus 36.4% of syndromic, P = 0.667). The nonsyndromic cohort was significantly older (4.04 ± 3.66 years versus 2.55 ± 3.34 years, P = 0.046) and had higher rate of signs of raised intracranial pressure (68.4% versus 25.0%, P = 0.001) than the syndromic cohort. There was no significant difference in perioperative variables or rate of complications (P > 0.05). The mean total advancement distance achieved was similar, 27 ± 6 mm in the nonsyndromic versus 28 ± 8 mm in the syndromic cohort (P = 0.964). All nonsyndromic subjects with signs of raised intracranial pressure demonstrated improvement at an average follow-up of 22 months.
Conclusion:
As in the syndromic patient, PVDO is a safe and, in the short-term, effective modality for cranial vault expansion in the nonsyndromic patient. The benefits and favorable perioperative profile of PVDO may therefore be extended to patient populations other than those with syndromic craniosynostosis.
