What is Scaphocephaly?

Scaphocephaly, also known as dolichocephaly, is a condition where a baby’s head shape is elongated from front to back. This condition can often correct itself as the baby grows and develops. However, concerns may arise when an infant’s head shape appears abnormal. Some head shape variations correct over time, while others, like Scaphocephaly, may require medical intervention. This essay explores the possibility of Scaphocephaly correcting itself, when parents should be concerned about their baby’s head shape and the importance of early intervention. Scaphocephaly is the most common form of Craniosynostosis and can vary in severity.

Scaphocephaly is a craniosynostosis where the sagittal suture fuses too early, causing an elongated and narrow head shape. It’s the most common type in 1 in 5,000 live births. Unlike positional skull deformities, which usually resolve on their own, Scaphocephaly typically requires medical evaluation and treatment, unlike positional skull deformities.

Does Scaphocephaly resolve on its own?

Scaphocephaly is a skull shape that cannot naturally correct itself due to premature suture fusion, causing the skull to grow excessively in length. Unlike mild positional flattening, this condition requires medical intervention and can persist if left untreated. In rare cases, it can lead to increased intracranial pressure. Unlike mild head shape abnormalities, Scaphocephaly requires medical intervention if significant deformity or complications occur, unlike mild head shape abnormalities that may improve over time.

Causes and symptoms

Causes 

Scaphocephaly is a common condition caused by premature fusion of the sagittal suture, prolonged pressure on the back of the head, genetic predisposition, and multiple births. It is more common in babies born on their backs, who may have a genetic predisposition to the condition, and those born in multiple births are more likely to develop it.

Symptoms

The head is elongated, with a narrow forehead due to premature fusion of the sagittal suture and a prominent occipital bone at the back. 

When Infant Head Shape Deviation Warrants Medical Evaluation

Newborns often have irregularities in head shape, but some require more attention. Parents should consult a healthcare professional if they notice persistent flattening or asymmetry, a long, narrow head shape, raised ridges or hard spots, closed or small fontanelle, unusual head growth patterns, developmental delays or neurological symptoms, or if their baby’s head appears disproportionately long and thin, Scaphocephaly, raised ridges or hard spots, closed or small fontanelle, rapid or slow head growth, or developmental delays or neurological symptoms.

• Persistent flat spots, also known as plagiocephaly or brachycephaly, are a flat area on one side or the back of the head, often accompanied by uneven ears or forehead. Causes include positional plagiocephaly or thoracicollis. Significant asymmetry may occur if the flat spot persists for 4-6 months, and severe cases may require helmet therapy.
• Scaphocephaly is a long, narrow head shape caused by premature fusion of the sagittal suture. It’s a concern if the shape doesn’t improve, there’s a family history of Craniosynostosis, or the baby has other developmental issues.
• Oxycephaly or Turricephaly is an abnormally pointed or tall head, often caused by Cranial Synostosis affecting multiple sutures. It should be considered when a baby has feeding difficulties, developmental delays, or abnormal head growth patterns.
• Craniosynostosis is an abnormal skull shape with ridges, a rugged ridge along skull sutures, and a lack of soft spots. It may be caused by premature suture closing. If the head shape doesn’t improve over time, there’s increased pressure, or head circumference growth is too fast or slow, it’s a concern.
• Macrocephaly or Microcephaly is characterized by a large or small head, often due to genetic conditions, brain growth issues, infections, or fluid buildup. It is considered a concern when head growth is rapid or slow, developmental delays, seizures, or other neurological signs are present.

Why Early Diagnosis and Treatment of Scaphocephaly Matters

Early diagnosis and treatment of Scaphocephaly are crucial for several reasons. It can improve the head shape, reduce the risk of complications, enhance brain development, and improve surgical outcomes. Younger children have a more malleable skull, making achieving a more normal head shape easier. Untreated Scaphocephaly can lead to increased intracranial pressure, vision problems, hearing problems, sleep apnea, and cognitive and developmental delays. Early treatment can relieve this pressure, allowing the brain to develop normally.

Scaphocephaly treatment varies based on the child’s age and severity. Surgical options include Cranial Vault Remodeling (CVR) for infants, Endoscopic Strip Craniectomy for younger infants, and Helmet Therapy for milder cases. CVR reshapes the skull by cutting and repositioning bones for normal growth. Endoscopic Strip Craniectomy is minimally invasive and uses helmet therapy postoperatively to guide development. Helmet Therapy can help reshape the skull over time. Early intervention can lead to better outcomes, a more natural-looking head shape, and reduced risk of complications. Early treatment can also reduce long-term consequences, such as vision or hearing loss, cognitive and developmental delays, and emotional and psychological difficulties.

Early treatment can also improve self-esteem in children with untreated Scaphocephaly. It can also reduce the financial burden of scaphocephaly treatment, as surgery and other treatments performed at a younger age are less expensive. Parents can also have peace of mind knowing their child receives the best possible care and has a better chance of achieving optimal outcomes.

Prognosis and long-term outcomes

Early treatment for Scaphocephaly can lead to normal development and improved head shape in most children. However, untreated cases may result in skull deformity and potential complications like headaches, vision problems, or developmental issues. Early surgery generally results in an excellent prognosis, with most children experiencing normal brain development and improved skull shape. Delayed treatment may lead to persistent skull deformities. Regular paediatrician or craniofacial specialist monitoring is crucial to ensure the head shape improves and no complications arise.

Scaphocephaly is a condition that doesn’t correct itself and requires medical attention for proper management. Parents should monitor their baby’s head shape and seek professional advice if abnormalities persist. Early intervention, including surgery and helmet therapy, ensures optimal outcomes, prevents complications, and allows for normal skull and brain development. Early diagnosis and treatment are essential for achieving optimal outcomes, reducing the risk of complications, and ensuring the child’s best possible quality of life. If Scaphocephaly is suspected, consult a pediatric neurosurgeon or craniofacial specialist for the best action.

About Dr Vishakha : 

Dr Vishakha Basavraj Karpe is a highly skilled senior consultant at Rainbow Children’s Hospital in Banjara Hills and Hydernagar Hyderabad. She is known for her comprehensive care approach and is one of the few dedicated leading paediatric neurosurgeons in the city and India with over ten years of extensive experience in pediatric neurosurgery. Her expertise includes treating hydrocephalus, spinal dysraphism, craniosynostosis, paediatric brain infections, brain and spine tumours and stroke surgery. 

She has a special interest in craniosynostosis surgery, which is done only in very few centres in India.

Proficiency of Dr Vishakha:

1. 1. Hydrocephalus (increased fluid in the brain): The procedure involves an endoscopic third ventriculostomy and CSF diversion (VP shunt) to treat complex hydrocephalus.
   2. 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.

• Spinal dysraphisms(Spina Bifida)- (spinal abnormalities present by birth) – surgical repair

• Encepahaocles repair surgery.

1. 1. Vascular conditions and stroke surgeries: revascularization surgeries for moya moya disease.

• Pediatric brain and spine tumour surgeries.

1. 1. Pediatric brain and spine infection surgeries: Endoscopic and open surgeries for brain and spine infections.

• Pediatric traumatic brain and spinal injury.

• Antenatal counselling for congenital fatal neurosurgical conditions.