What Is Multisuture & Syndromic Craniosynostosis?
Multisuture Craniosynostosis is a condition in which more than one of the soft joints of the skull fuses earlier than usual. It is usually associated with an abnormal gene causing one of the craniofacial syndromes.
How It Affects Your Child
The head may be flat (Brachycephaly), tall (turricephaly), flat and tall (turribrachycephaly) or small (Microcephaly). It can come with anomalies of the hands, ears, brain or internal organs. Very often it compresses the brain and raises the pressure inside the skull, which can lead to blindness and brain damage. Many children have an underdeveloped midface (midface hypoplasia), causing airway problems and Obstructive Sleep Apnoea, and shallow eye sockets (exorbitism) that can let the eyeballs dislocate and be damaged. A significant proportion of children have some degree of learning disability.
How It Is Diagnosed
Your craniofacial surgeon will examine your child thoroughly. A skull X-ray, a 3D CT scan and an MRI scan of the head are usually needed, and intracranial pressure monitoring is occasionally required. Genetic testing is usually done to identify the gene involved.
How It Is Treated
Treatment needs a multidisciplinary team: craniofacial surgeon, neurosurgeon, orthodontist, respiratory physician, ENT surgeon, ophthalmologist, speech pathologist, paediatrician and clinical psychologist. Dr Vybhav Deraje organises regular multidisciplinary meetings to decide the right course for each child.
Many children need surgery to relieve raised intracranial pressure and improve the head shape, often more than one operation at different stages of childhood.
Common Syndromes
- Crouzon syndrome
- Apert syndrome
- Pfeiffer syndrome
- Muenke syndrome
- Saethre-Chotzen syndrome
Stages of Treatment
- First year of life — posterior cranial vault expansion to release the pressure inside the skull and improve intracranial anomalies such as Chiari malformation.
- Late first or second year — Fronto-Orbital Advancement and Cranial Vault Remodelling to bring the forehead forward and reshape the skull, delayed as long as possible to allow for growth.
- After the age of 3 — Le Fort III midface advancement if breathing problems and sleep apnoea need it.
- Other procedures as your child's condition requires.
Posterior Cranial Vault Expansion — What to Expect
After assessment by a paediatric neuro-anaesthetist and ophthalmologist, your child is admitted on the day of surgery. The operation takes 3–4 hours under general anaesthesia, with monitoring lines placed, and almost all children need a blood transfusion. Through an ear-to-ear cut, the neurosurgeon protects the brain while the craniofacial surgeon expands the bones at the back of the head to make more room for the brain.
Your child spends the first night in the ICU. Swelling of the head and face (the eyes may close for 3–4 days) settles over the week, and children usually go home on the sixth or seventh day. Follow-up is with the craniofacial surgeon, neurosurgeon and ophthalmologist, with CT and MRI scans to monitor progress. Gaps in the skull close over 2–3 years and the scar is usually hidden in the hair.
Fronto-Orbital Advancement and Cranial Vault Remodelling
A 4–5 hour operation under general anaesthesia, with a blood transfusion in almost every case. The craniofacial surgeon brings the forehead forward and reshapes the forehead and skull bones while the neurosurgeon protects the brain. Recovery is as above: a night in the ICU, swelling for about a week and discharge on the sixth or seventh day, with follow-up scans and regular multidisciplinary review.
Le Fort III Midface Advancement
The middle part of the face — the upper jaw and cheekbones — is brought forward to support the eyeballs and improve the airway and sleep apnoea. Your child first sees the orthodontist, and a CT scan is used for virtual surgical planning. The operation takes 6–7 hours and always needs a blood transfusion; cuts are made from ear to ear and inside the mouth, and the bones are fixed in their new position with titanium plates and screws.
After a night in the ICU and about a week of swelling, children usually go home on the sixth or seventh day and eat a soft diet for the first few weeks. A sleep study is usually repeated a few months later to measure the improvement.
Possible Complications
This is major surgery and families should be fully informed. Infection, bleeding, scarring, swelling and pain are the common complications, and the team manages them routinely. A small tear in the covering of the brain is sometimes seen and is usually repaired during the operation. Injury to the eye or brain is very rare. Death is a remote theoretical possibility; Dr Deraje's team has not seen it in their practice.
In Syndromic Craniosynostosis, raised intracranial pressure can return at any time, and repeat surgery may then be needed.
Travelling from Another State or Country?
Families come to Dr Vybhav Deraje from across India and abroad. Send your child’s photographs and CT scan to the clinic first — Dr Vybhav can review them on a video consultation, and the clinic confirms the dates before you book travel. Keyhole surgery is possible only before 6 months of age, so contact the clinic early.
Planning Your Visit from Outside BangaloreVideos on Multisuture & Syndromic Craniosynostosis
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Frequently Asked Questions
Which syndromes cause Multisuture Craniosynostosis?
Common ones are Crouzon, Apert, Pfeiffer, Muenke and Saethre-Chotzen syndromes. Genetic testing is usually done to identify the gene involved.
How many operations will my child need?
Often more than one, at different stages: Posterior Vault Expansion in the first year, forehead and skull reshaping in the late first or second year, and Le Fort III midface advancement after age 3 if breathing needs it.
Why is a midface advancement needed?
Many children with Syndromic Craniosynostosis have an underdeveloped midface, which narrows the airway and causes sleep apnoea and leaves the eyes poorly protected. Le Fort III advancement brings the midface forward to improve breathing and support the eyes.
Who is involved in my child's care?
A multidisciplinary team: craniofacial surgeon, neurosurgeon, orthodontist, respiratory physician, ENT surgeon, ophthalmologist, speech pathologist, paediatrician and clinical psychologist, meeting regularly to plan each step.
Other Types of Craniosynostosis
- Sagittal Synostosis — Long, narrow head (Scaphocephaly) — the most common single-suture type.
- Metopic Synostosis — Triangular, keel-shaped forehead (Trigonocephaly).
- Unicoronal Synostosis — One side of the forehead flattened, with a slanted skull (Anterior Plagiocephaly).
- Bicoronal Synostosis — Both coronal sutures fused — a flat, wide head (Brachycephaly).
- Lambdoid Synostosis — Back of the head slanted or twisted to one side — the rarest type.
This page is general information written by Dr Vybhav Deraje for families. It does not replace an individual consultation; treatment depends on clinical assessment.