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Metopic craniosynostosis (trigonocephaly), ​ក្បាលរាងបីជ្រុង скачать в хорошем качестве

Metopic craniosynostosis (trigonocephaly), ​ក្បាលរាងបីជ្រុង 8 лет назад

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Metopic craniosynostosis (trigonocephaly), ​ក្បាលរាងបីជ្រុង

Dr Chor Ath Metopic craniosynostosis (trigonocephaly) Epidemiology and pathogenesis Trigonocephaly refers to the triangular appearance of the frontal skull. It is caused by the premature fusion of the metopic suture (metopic craniosynostosis). The metopic suture, also known as the median frontal suture, runs from the nasion to the bregma (anterior fontanelle in infants). The metopic suture normally fuses at around approximately 6 to 8 months of age (but can fuse as early as 2 months). The interdigitations of the suture are usually obliterated by age 7, but they can persist as an anatomic variant (not to be confused with a fracture). Metopic craniosynostosis can be present in isolation or in combination with other malformations. Craniosynostosis has multiple etiologies, including mutations in genes regulating fibroblast growth in syndromic forms (such as Saethre-Chotzen, Opitz, and Jacobsen syndromes), in utero infections, and teratogenic exposure. However, the cause of isolated metopic craniosynostosis is unknown, although it is likely multifactorial congenital with most cases being sporadic. The estimated incidence of all forms of craniosynostosis is 0.4 to 1 in 1,000. Metopic craniosynostosis is one of the most common forms of isolated craniosynostosis, representing 10% to 25% of cases. There is approximately a 3:1 predominance of males compared with females. Clinical presentation The abnormal head shape is usually identified in infancy. The classic triad of narrow forehead, biparietal widening, and hypotelorism (closeness of eyes) is only present in 14% of patients. There is a mild association with developmental delay, learning disabilities, attention deficit hyperactivity disorder (ADHD), autism spectrum disorder (ASD), and elevated intracranial pressures, especially in severe cases and if it goes untreated. Diagnosis Trigonocephaly is a physical exam finding. Metopic craniosynostosis is also often diagnosed by physical exam alone, although, especially when there is clinical uncertainty, a CT is often obtained for a more definitive diagnosis and is also useful for presurgical planning. Imaging features Imaging will demonstrate a trigonocephalic appearance of the frontal skull with a smaller anterior cranial vault and sometimes with biparietal widening. In young infants, the metopic suture will be prematurely fused, although it may normally fuse as early as 2 months of age. CT will often demonstrate ectocranial ridging of the metopic suture, although this is not entirely specific as mild forms can be seen physiologically. Endocranial notching of the metopic suture is a more specific imaging finding, sometimes seen as an internal invagination called the “omega sign.” Also seen are associated midface anomalies, including hypotelorism and teardrop-shaped orbits angulated toward the midline of the forehead ("surprised coon" sign). There is some association with underlying brain malformations such as gyral anomalies and callosal agenesis, particularly in syndromic cases. Treatment and prognosis Patients with craniosynostosis syndromes benefit from multidisciplinary teams that include neurosurgery; ear, nose, and throat (ENT); plastic surgery; and developmental pediatrics found at major pediatric centers to appropriately carry out individualized treatment and surgical reconstruction plans. To correct craniosynostosis, surgical procedures with cranial vault expansion are required to allow the brain sufficient space to grow and to normalize craniofacial anomalies. The most common procedure is fronto-supraorbital advancement, usually before the age of 1 year given evidence of better outcomes. References Birgfield CB, Saltzman BS, Hing AV, et al. Making the diagnosis: Metopic ridge versus metopic craniosynostosis. J Craniofac Surg. 2013;24(1):178-185. Birgfield CG, Heike CL, Saltzman BS, Hing AV. Clinical characteristics and surgical decision making for infants with metopic craniosynostosis in conjunction with other congenital anomalies. Plast Reconstr Surg Glob Open. 2013;1(7):e62. Lee BS, Hwang LS, Doumit GD, et al. Management options of non-syndromic sagittal craniosynostosis. J Clin Neurosci. 2017;39:28-34. Wang JC, Nagy L, Demke JC. Syndromic craniosynostosis. Facial Plast Surg Clin N Am. 2016;24(4):531-543. Weinzweig J, Kirschner RE, Farley A, et al. Metopic synostosis: Defining the temporal sequence of normal suture fusion and differentiating it from synostosis on the basis of computed tomography images. Plast Reconstr Surg. 2003;112(5):1211-1218.

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