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Craniosynostosis : Types, Causes, Diagnosis and Treatment : Pediatrics National exit test

Craniosynostosis is a birth defect that affects the bones in a newborn baby's skull. In normal growth, the sutures (fibrous joints connecting the bones) remain open, allowing the skull to expand and accommodate the rapidly growing brain. In craniosynostosis, one or more of the sutures close prematurely, affecting the skull's shape and sometimes limiting brain growth.

1. Types of Craniosynostosis
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The type of craniosynostosis is determined by which suture(s) close prematurely.

Sagittal Synostosis (Scaphocephaly): The sagittal suture runs from the front to the back of the head. In this type, the head may appear long and narrow.
Coronal Synostosis (Anterior Plagiocephaly): There are two coronal sutures, running from each ear to the sagittal suture. Closure of one of these sutures can cause flatness on one side of the forehead and a raised eye socket.
Metopic Synostosis (Trigonocephaly): The metopic suture runs from the nose to the sagittal suture. Premature closure may lead to a pointed forehead (triangular shape).
Lambdoid Synostosis (Posterior Plagiocephaly): The lambdoid suture runs along the back of the head. Closure of this suture leads to a flatness on one side of the back of the head.

2. Causes
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Craniosynostosis may occur as part of a genetic syndrome or can be sporadic with no identifiable genetic cause.

Syndromic Craniosynostosis: This type includes conditions like Apert, Crouzon, and Pfeiffer syndromes. They often have a clear genetic cause and may be associated with other physical symptoms.
Nonsyndromic Craniosynostosis: This is the most common type, and its cause is usually unknown. It typically involves only one suture and is not associated with other physical or developmental problems.

3. Diagnosis
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Diagnosis is usually based on a physical examination and imaging studies.

Physical Examination: The doctor will examine the baby's head for abnormal shape, suture ridges, and fontanelle size.
Imaging Studies: X-rays, CT scan, or MRI can provide detailed images of the skull and brain, confirming the diagnosis.

4. Treatment
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The primary goal of treatment is to relieve pressure on the brain and allow for normal brain growth. The specific treatment may depend on the severity of the condition, the age at diagnosis, and the presence of other health problems.

Surgery: This is often the treatment of choice. It involves separating the fused suture to allow for normal brain and skull growth.
Helmet Therapy: Following surgery, a custom-fitted helmet may be used to help reshape the baby's head.

Monitoring: Regular follow-ups with a multidisciplinary team are essential to monitor the child's development and address any complications or new symptoms.

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