Great Ormond Street Hospital for Children NHS Foundation Trust, developmental delay is a direct result of, the craniosynostosis – it appears to be an, association only. Premature closure and ossification of the metopic suture results in a triangular head shape called trigonocephaly and is characterized by a wedge-shaped forehead and frontotemporal narrowing. Some children with just a ridge or mild metopic synostosis don’t need any medical treatment. What is Metopic Synostosis? Craniosynostosis (kray-nee-o-sin-os-TOE-sis) is a birth defect in which one or more of the fibrous joints between the bones of your baby's skull (cranial sutures) close prematurely (fuse), before your baby's brain is fully formed. Click here for more information on metopic synostosis. If you are coming to GOSH for an outpatient appointment, only one carer per family will be allowed into the hospital. Note how forehead comes to a central point and there is constriction at both sides of the forehead. In this form, the baby’s head shape may be described as trigonocephaly which may vary from being mild to very severe. Although the majority are sporadic, Craniosynostosis syndromes may be associated with environmental and genetic factors. They will also have a pointed, almost triangular shape to the front and top of their skulls and eyes that appear too close together. Children with metopic, unicoronal or lambdoid synostosis were much more likely to have a learning problem than children with the most common form of single-suture craniosynostosis – sagittal synostosis. The estimated prevalence is 1 in 15,000 live births with a 3:1 male:female ratio. Between those plates are fibrous joints called sutures. Metopic craniosynostosis is also known as trigonocephaly – from the Greek for triangle-shaped. Risk factors include male sex, twin gestation, and in utero exposure to valproate. Summary. An infant born at term has nearly 40 percent of his or her adult brain volume, and this increases to 80 percent by three years of age. Reproduced with permission from the Barrow Neurological Institute. One type of craniosynostosis is called metopic synostosis (also referred to as trigonocephaly or metopic suture craniosynostosis). 24,25 Premature fusion of the metopic suture causes arrested growth of the cranium in the transverse direction anteriorly and increased anteroposterior growth. Right: face view after surgical correction of metopic synostosis. This results in abnormal head shapes and restriction to the growth of the brain. BACKGROUND: Premature closure and ossification of the metopic suture results in a triangular head shape called trigonocephaly and is characterized by a wedge-shaped forehead and frontotemporal narrowing. The brain grows rapidly in utero and during the first three years of life. What Does It Look Like? Observe the triangular forehead and hy-potelorism. It can affect one suture or several. 2000) c, d In a 21-month-old infant girl. The edges of the skull bones are called sutures, which normally close by age 2 to 3. For those who do, surgery has proven to be a successful approach. The multidisciplinary team will usually comprise craniofacial (skull and face) surgeons, neuro (brain) surgeons, ophthalmologists (eye specialists), geneticists and speech and language therapists with other specialists brought in as needed. There are different types of surgery depending, on the age of the child at diagnosis – the, team will discuss the options with the family. Corrective surgery does not, As children with metopic craniosynostosis, have a characteristic appearance, no specific. Craniosynostosis is a malformation that involves the early closure of a single or multiple sutures of the skull. The prematurely closed metopic suture is seen as a vertical band of hyperdensity in the midline. For those who … If left untreated, this can cause increased pressure within the skull (intracranial pressure) and can potentially result in cognitive impairment or developmental delays. The Craniofacial team in collaboration with the Child and Family Information Group. Metopic synostosis is an uncommon type of craniosynostosis, occuring in 4-10% of cases. Metopic Synostosis (trigonocephaly) This form of synostosis is relatively uncommon (less than 10 percent of cases) and is characterized by a bony ridge in the midline of the forehead, a triangularly shaped head, a narrow forehead and eyes that are positioned close together. Lambdoid synostosis Note the improved forehead contour and decrease in … Males are affected about three times as often as females. 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