What is Craniosynostosis?

Craniosynostosis is a congenital condition causing premature closure of skull sutures, impacting head shape and brain growth, necessitating early diagnosis and effective treatment.

Types of Craniosynostosis:

1. Sagittal Craniosynostosis: Sagittal Craniosynostosis is a common form of Craniosynostosis, characterized by premature fusion of the sagittal suture, crucial for normal skull growth and expansion.
2. Coronal Craniosynostosis: Coronal Craniosynostosis occurs when coronal sutures close prematurely, causing head shape changes and facial asymmetry.
3. Metopic craniosynostosis: Metopic Craniosynostosis is a condition where the metopic suture, which connects the nose to the skull, prematurely closes, causing forehead shape changes and potentially causing physical and developmental issues.
4. Lambdoid craniosynostosis: Lambdoid Craniosynostosis is a rare condition affecting the shape of the head’s back, affecting cranial and facial symmetry, and occurs when one or both lambdoid sutures close prematurely.

Causes of Craniosynostosis:

Syndromic Craniosynostosis is linked to genetic syndromes like Apert, Crouzon, Pfeiffer, Saethre-Chotzen, and Muenke syndrome, often involving mutations in specific genes. Non-syndromic Craniosynostosis, a sporadic condition not linked to any syndromes, is heightened by pregnancy tobacco smoke exposure. Older paternal age is also linked to a higher risk of genetic mutations. Certain medications, such as fertility treatments or antiepileptic drugs, have been linked to increased risk. Intrauterine constraints or abnormal uterine shape can also contribute to premature closure of sutures. Hormonal variables during pregnancy may also be important.

Symptoms of Craniosynostosis:

Sagittal Craniosynostosis is distinguished by a long, thin head with a prominent forehead and occiput. It can be unilateral, bilateral, metopic, or lambdoid, with asymmetrical foreheads, flattening of the back, and a recessed brow. Facial asymmetry occurs when facial features are misaligned, particularly in unilateral or lambdoid Craniosynostosis. Fontanelle changes may be absent or smaller than normal, and a ridge may be felt along the affected suture. Head circumference may not increase as expected during routine check-ups. Developmental delays may occur in severe cases or those associated with syndromes. Increased intracranial pressure is rare, with symptoms including irritability, vomiting, poor feeding, a bulging fontanelle, and lethargy. Neurological symptoms include seizures, vision problems, or hearing loss in severe or untreated cases. 

Diagnosis of Craniosynostosis:

Craniosynostosis diagnosis involves a combination of physical examination, imaging studies, and genetic testing. A detailed medical history is taken, including any family history of the condition. The doctor examines the baby’s head shape, size, and symmetry, checking for ridges along sutures and facial features. X-rays provide an initial view of the skull and suture lines, while CT scans provide detailed images of the skull and sutures, often with 3D scans for a comprehensive view. MRI is sometimes used to assess the brain and intracranial structures, especially for neurological issues.

Treatment for Craniosynostosis:

Craniosynostosis is a condition requiring surgery to correct the abnormal head shape, promote normal brain growth, and alleviate potential complications like increased intracranial pressure. The type and timing of surgery depend on the severity of the condition, suture type, and syndromic or non-syndromic status.

Regular check-ups with a craniofacial team are essential for monitoring for developmental delays or complications.

Prognosis:

Early diagnosis and treatment of Craniosynostosis can lead to minimal complications in children, but untreated cases can cause developmental delays, increased intracranial pressure, and psychological effects. Support groups and comprehensive care involving neurosurgeons, plastic surgeons, geneticists, and paediatricians ensure optimal outcomes.

Advancements in surgical techniques and multidisciplinary approaches have significantly improved the prognosis for children with Craniosynostosis, a condition that can be effectively managed with timely diagnosis and treatment.

About Dr Vishakha

Dr. Vishakha Karpe, a highly skilled Senior Paediatric Neurosurgeon at Rainbow Children’s Hospital, Banjara Hills, and Hyder Nagar in Hyderabad, is one of India’s leading paediatric neurosurgeons with extensive experience in paediatric neurosurgery. With over nine years of dedicated practice, she is among the few in India working extensively in this field.

With extensive experience in paediatric neurosurgical conditions, she focuses on comprehensive care, including precise surgery and educating parents about the complete case management protocol. She is an efficient and passionate medical professional, pursuing ethical practice and ensuring patient care after surgery.

Proficiency of Dr Vishaka:

Hydrocephalus (increased fluid in the brain): The procedure involves an endoscopic third ventriculostomy and CSF diversion (VP shunt) to treat complex hydrocephalus.

1. Craniosynostosis (abnormal head shape due to untimely cranial sutures fusion) surgeries: Helmet therapy is a technique that is used in both endoscopic and open surgery.
2. Spinal dysraphisms(Spina Bifida)- (spinal abnormalities present by birth) – surgical repair
3. Encepahaocles repair surgery.
4. Vascular conditions and stroke surgeries: revascularization surgeries for moya moya disease.
5. Pediatric brain and spine tumour surgeries.
6. Pediatric brain and spine infection surgeries: Endoscopic and open surgeries for brain and spine infections.
7. Pediatric traumatic brain and spinal injury.
8. Antenatal counselling for congenital fatal neurosurgical conditions.

Dr Vishaka specializes in craniosynostosis surgery, which is only done in a few centres in India. Dr Vishaka Patil, M.B.B.S, DNB (AIIMS) New Delhi, M.Ch (IPGMER SSKM) became a Member of  “The Royal College of Surgeons, Edinburgh” (U.K.) a highly successful and best pediatric neurosurgeon in Hyderabad, Telangana with 13 years of experience, is among the topmost pediatric neurosurgeons in the Rainbow group of hospitals at Hyder Nagar and Banjara Hills.