Craniosynostosis - An overview

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Craniosynostosis is a congential condition where the growth plates (sutures) of the babies' skull fuse prematurely.

It can be primary or secondary. Primary Craniosynostosis is a condition where the fusion of the sutures is the primary event. Secondary Craniosynostosis is fusion of sutures secondary to a brain issue or sometimes post surgery.

Craniosynostosis is a congenital condition. As with all congenital conditions, it can be due to several factors:

1. Genetic reasons
2. Family history
3. Elderly maternal age
4. Conception through IVF
5. Hypothyroidism during pregnancy
6. Specific drugs & medicines intake during 1st trimester of pregnancy
7. Exposure to radiation or viral fevers during 1st trimester of pregnancy
8. Alcohol & Smoking during pregnancy

Craniosynostosis leads to 2 main problems:

1. Abnormal head shape
2. Raised intracranial pressure

Depending on the suture that is fused, Craniosynostosis leads to different kinds of abnormal head shapes.

1. Trigonocephaly - Triangular Head shape
2. Plagiocephaly - Twisted Head shape
3. Scaphocephaly - Long and narrow Head Shape
4. Brachycephaly - Flat Head Shape
5. Turricephaly - Tall Head Shape

In some babies, Craniosynostosis can lead to raised pressure in the brain. This can in-turn lead to:

1. Pressure on the optic nerve - Leading to Papilloedema - leading to vision loss
2. Chiari I Malformation
3. Developmental delays
4. Behavioural issues
5. Headaches
6. Vomiting
7. Seizures
8. Sleep apnoea
9. Exophthalmos

Craniosynostosis can be widely classified into 2 types - Non syndromic & Syndromic. In Syndromic cases, a specific gene like FGFR2, FGFR3, TWIST1, EFNB1, MSX2 are mutated and detected. In Non syndromic cases, no such genes can be detected.

Non-syndromic:

1. Metopic synostosis
2. Unicoronal synostosis
3. Sagittal synostosis
4. Bicoronal synostosis
5. Multisuture synostosis
6. Lambdoid synostosis

Syndromic:

1. Apert syndrome
2. Crouzon syndrome
3. Pfeiffer syndrome
4. Muenke syndrome
5. Saethre-Chotzen syndrome
6. Carpenter syndrome

The diagnosis of craniosynostosis is predominantly clinical. But your surgeon might advise the following in certain circumstances:

1. X Ray Skull
2. CT scan of Head
3. MRI of Brain
4. Eye test
5. B scan of the Optic nerves
6. Intracranial pressure monitoring
7. Routine blood test
8. Genetic testing

The treatment is primarily surgical in nature.

In Non-syndromic single suture craniosynostosis, our team's protocol is as follows:

1. Age less than 6 months - Endoscopic strip craniectomy & Orthotic Helmet Therapy
2. Age more than 6 months - Open Fronto orbital advancement or Cranial Vault Remodelling surgery

In Syndromic craniosynostosis cases, usually the babies need multiple surgeries and is tailored according to each baby's need at a particular point of time. They might need further surgeries on the face in addition to surgeries on the skull for full correction of the deformity.

We also perform innovative treatments like Spring-assisted cranial expansion in certain specific scenarios.