Craniosynostosis

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Craniosynostosis is a condition in which one or more of the fibrous sutures in an infant  skull prematurely fuses by turning into bone (ossification), thereby changing the growth pattern of the skull. Because the skull cannot expand perpendicular to the fused suture, it compensates by growing more in the direction parallel to the closed sutures which  results in an abnormal head shape and abnormal facial features. In cases in which the compensation does not effectively provide enough space for the growing brain, craniosynostosis results in increased brain pressure  leading to visual impairment, impairment of mental development combined with a significant reduction in IQ and behavioral problems. Craniosynostosis occurs in one in 2000 births.

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The types of craniosynostosis are

• Sagittal synostosis– It is the most common type of craniosynostosis.When this suture closes too early, the head will grow long and narrow (scaphocephaly). It is the most common type of craniosynostosis.

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• Coronal synostosis – This is the second most common type of craniosynostosis.The right and left coronal sutures run from each ear to the sagittal suture at the top of the head. When one of these sutures closes too early, the infant  may have a flattened forehead on the side of the skull that closed early (anterior plagiocephaly). The baby’s eye socket on that side might also be raised up and his or her nose could be pulled toward that side.

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• Bicoronal synostosis – This type of craniosynostosis occurs when the coronal sutures on both sides of the baby’s head close too early. In this case, the head will grow broad and short (brachycephaly).

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• Lambdoid synostosis – The lambdoid suture runs along the backside of the head. If this suture closes too early, the baby’s head may be flattened on the back side (posterior plagiocephaly). This is one of the rarest types of craniosynostosis.

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• Metopic synostosis – The metopic suture runs from the baby’s nose to the sagittal suture at the top of the head. If this suture closes too early, the top of the head shape may look triangular, meaning narrow in the front and broad in the back (trigonocephaly). This is one of the rarest types of craniosynostosis.

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Diagnosis

Clinically and radiologically by USG, CT scan.

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Treatment

Patients with mild deformity cases can be managed conservatively and others will require surgery. The primary goal of surgical intervention is to allow normal cranial vault development to occur.The surgical procedure is meant to relieve pressure on the brain, correct the craniosynostosis, and allow the brain to grow properly. Results are best when surgery is performed within 6 months of age. There are multiple types of surgery used to treat craniosynostosis, including strip craniectomy, spring-assisted craniectomy and cranial vault remodeling. Endoscopic surgeryresults are good if surgery is done within three months of age.

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