Craniosynostosis is a birth defect in which one or more of the fibrous joints that link the baby’s skull bones (cranial sutures) close (fuse) prematurely, before the baby’s brain is fully formed. The brain will not stop growing, giving the head a misshapen appearance.
Sutures are normally kept flexible during childhood, giving your child’s brain time to grow. At the front of the skull, the sutures are attached at the large soft point (fontanel) at the top of the head. The anterior fontanel is the soft spot that can be felt just behind your baby’s forehead. The next largest point is at the back (rear). Each side of the skull has a small fontanel.
Craniosynostosis usually involves premature fusion of a single cranial suture, but it can affect more than one of your baby’s skull sutures (multiple-suture craniosynostosis). In rare cases, certain genetic syndromes cause craniosynostosis (syndromic craniosynostosis).
Craniosynostosis treatment in Delhi consists of surgery that corrects the shape of the head and enables normal brain growth. Early diagnosis and treatment allow your baby’s brain to have adequate space to grow and develop.
Although there may be neurological damage in severe cases, most children have normal cognitive development and have good cosmetic results after surgery. Early diagnosis and treatment are essential.
The signs of craniosynostosis are usually evident at birth, but may become more visible during the baby’s first few months of life. Signs and severity depend on how many sutures are fused and when the fusion occurs in brain development. This may include the following:
- A deformed skull, with the shape it takes depending on which sutures are affected
- An abnormal sensation or disappearance of the fontanelle of your baby’s skull
- Development of a hard, raised ridge along affected sutures
- Slow or no growth of the head as the baby grows
Types of craniosynostosis
There are many types of craniosynostosis. Most consist of the fusion of a single cranial suture. Some complex forms of craniosynostosis involve the fusion of multiple sutures. Most cases of multiple suture craniosynostosis are related to genetic syndromes and are called syndromic craniosynostosis.
The term given to each type of craniosynostosis depends on which sutures are affected. The types of craniosynostosis include the following:
- Sagittal (scaphocephaly). Premature fusion of the sagittal suture that runs from front to back across the top of the skull causes the head to grow long and narrow. Sagittal craniosynostosis results in a head shape called scaphocephaly and the most common type of craniosynostosis.
- Coronal. Premature fusion of one of the coronal (unicoronal) sutures running from each ear to the top of the skull can cause the forehead to flatten on the affected side and bulge on the unaffected side. It also causes the nose to turn and the eye socket to rise on the affected side. When both coronal sutures fuse prematurely (bicoronal), the head appears short and broad, often with the forehead tilted forward.
- Metopic. The metopic suture runs from the top of the bridge of the nose to the midline of the forehead, passing through the anterior fontanel and the sagittal suture. Premature fusion gives the forehead a triangular appearance and widens at the back of the head. This is also called trigonocephaly.
- Lambdoidea. Lambdoid synostosis is a rare type of craniosynostosis involving the lambdoid suture, which runs the length of the skull at the back of the head. It can cause one side of the baby’s head to look flat, one ear to be higher than the other, and the top of the head to tilt to one side.
Other reasons that can lead to deformed heads
A deformed head does not always indicate craniosynostosis. For example, if the back of your baby’s head appears flattened, it could be the result of spending too much time on one side of the head. This can be treated with frequent position changes or, if significant, with a helmet (cranial orthosis) to help reshape the head to a more normal appearance.
When should you see a doctor
The doctor will monitor your child’s head growth as a routine at check-up appointments. Talk to your paediatrician if you have concerns about the growth or shape of your baby’s head.
The cause of craniosynostosis is often unknown, but it is sometimes associated with genetic disorders.
- The non – syndromic craniosynostosis is the most common type of craniosynostosis and its cause is unknown, although it is believed to be a combination of genes and environmental factors.
- The syndromic craniosynostosis is caused by certain genetic syndromes, including Apert syndrome, Pfeiffer syndrome or Crouzon syndrome, which can affect the development of the baby’s skull. These syndromes generally also include other physical characteristics and health problems.
If not treated, craniosynostosis can cause, for example:
- Permanent deformity of the head and face
- Low self-esteem and social isolation
The risk of increased pressure within the skull (intracranial pressure) from simple craniosynostosis is small as long as the suture and head shape are surgically arranged. But babies with the preexisting syndrome can develop a pressure build-up inside the skull if it doesn’t expand enough to make room for the growing brain.
If left untreated, increased intracranial pressure can cause the following:
- Delays in development
- Cognitive impairment
- Lack of energy or interest (lethargy)
- Eye movement disorders
- Death, rarely
Craniosynostosis requires the evaluation of specialists, such as a pediatric neurosurgeon in Delhi or a specialist in plastic and reconstructive surgery. The diagnosis of craniosynostosis may include:
- Physical exploration. The doctor will feel your baby’s head for abnormalities such as suture ridges and facial deformities.
- Imaging studies. A computed tomography (CT) scan or magnetic resonance imaging (MRI) scan of your baby’s skull can detect if any sutures have fused. A cranial ultrasound can be used. Fused sutures are identified by their absence, because they are invisible once fused, or by the rim of the suture line. Photographs and a laser scanner can also be used to make precise measurements of the shape of the skull.
- Genetic analysis. If the doctor suspects a pre-existing genetic syndrome, genetic testing can help identify that syndrome.
Craniosynostosis Treatment in Delhi
Mild cases of craniosynostosis may not need treatment. The doctor may recommend a specially molded helmet to help reshape the baby’s head if the cranial sutures are open and the shape of the head is abnormal. In this situation, the molded helmet can help your baby’s brain grow and correct the shape of the skull.
However, for most babies, surgery is the main craniosynostosis treatment in Delhi. The type and timing of surgery depend on the type of craniosynostosis and whether there is a underlying genetic syndrome. Sometimes more than one surgery is needed.
The purpose of surgery is to correct the abnormal shape of the head, reduce or prevent pressure on the brain, create room for the brain to grow normally, and improve the baby’s appearance. This involves a planning and surgery process.
Imaging studies can help surgeons develop a plan for surgical procedures. Virtual surgical planning for the treatment of craniosynostosis uses high definition 3D computed tomography and MRI scans of your baby’s skull to build an individualized and computer-simulated surgical plan. Based on that virtual surgical plan, custom templates are built to guide the procedure.
A team that includes a head and face surgery specialist (craniofacial surgeon) and a brain surgeon in Delhi (neurosurgeon) generally performs the procedure. The surgery can be endoscopic or open. Both types of procedures generally produce very good cosmetic results with a low risk of complications.
- Endoscopic surgery. This minimally invasive surgery can be performed on babies up to 6 months of age. With a lighted tube and camera (endoscope) inserted through small incisions in the scalp, the surgeon removes the affected suture to allow your baby’s brain to grow normally. Compared to an open procedure, endoscopic surgery has a smaller incision, usually involves just one night in the hospital, and usually does not require a blood transfusion.
- Open surgery. Generally, for babies older than 6 months, open surgery is performed. The surgeon makes an incision in the scalp and cranial bones, and then reshapes the affected part of the skull. The position of the skull is held in place with plates and screws that are absorbable. Open surgery usually involves a three to four day hospital stay, and a blood transfusion is usually necessary. This is usually a one-time procedure, but in complex cases, multiple open surgeries are often required to correct the shape of the baby’s head.