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Craniosynostosis refers to the premature fusion of skull sutures. Some cases are linked to genetic factors, while others have no specific cause and relate to the environment in which the skull develops.
In general, craniosynostosis occurs in the womb, so an experienced pediatrician can often detect it right after birth. However, some babies are born with one fused suture, and over time, nearby sutures may also fuse, leading to increasingly abnormal head shapes. If an abnormal head shape is noticed, seek prompt medical evaluation. By age one, a baby's brain volume triples, so insufficient space can cause issues.
If a specialist diagnoses craniosynostosis, they may first arrange a CT scan of the skull. While frontal craniosynostosis is sometimes effective, other types are visible on CT scans. Since the fontanelle closes by eight months, diagnosis via CT is not possible after that point and requires specialist assessment.
Worldwide, surgical treatment for craniosynostosis can be divided into two main categories: procedures handled solely by pediatric neurosurgeons or those involving collaboration with pediatric neurosurgeons and craniofacial surgeons, depending on hospital practices.
This collaboration stems from different surgical models. Pediatric neurosurgeons focus on the brain, while craniofacial surgeons emphasize aesthetics, working together in a team approach for the procedure.
Simply put, pediatric neurosurgeons care for the baby's brain, and pediatric craniofacial surgeons focus on appearance, collaborating on surgeries where each handles their expertise.
Craniofacial surgeons not only reshape the skull but can also perform procedures on the midface. Some cases of craniosynostosis accompany incomplete midface development, causing airway narrowing that may require future midface surgery. Others involve hypertelorism needing eye spacing reduction or even orthognathic surgery to correct jaw fusion, or nasal bone adjustments for crooked noses—all within the scope of craniofacial surgeons.
"I've heard about endoscopic suturectomy with small incisions. Is it suitable for my baby?"
In recent years, endoscopic suturectomy has become popular in North America, often followed by helmet molding to reduce the risks of major surgery.
Traditional surgery for early suture fusion involves removing and reassembling the skull to allow brain expansion and improve appearance, but it carries higher risks due to blood loss and complexity.
Endoscopic suturectomy uses smaller incisions with less blood loss, but it's not ideal for every case of craniosynostosis. Surgery must occur early to prevent worsening skull deformity, typically between 2-4 months. Even with small incisions, risks remain, especially since the small opening can be hard to manage with special equipment, potentially leading to bleeding. Post-surgery, a molding helmet is essential for effectiveness, worn for about a year, usually requiring 2-3 helmets, all at personal cost and worn consistently.
Additionally, not all types of craniosynostosis are suitable; sagittal craniosynostosis with long anterior-posterior dimensions is most appropriate, while others, like unilateral coronal craniosynostosis, yield poorer results.
However, early detection of abnormal head shape and diagnosis of premature fusion allow consultation with specialists about endoscopic options, providing at least some choices.
"If I choose not to have surgery for my baby, will the head shape worsen?"
Currently, there's no definitive answer. Collecting data on untreated cases with complete head shape records is challenging.
A study from a major Canadian children's hospital, not yet published, tracked 30 untreated patients long-term and found head shapes remained consistent.
In clinical practice, some cases worsen over time, while others progress from one fused suture to two or three.
To address this, first understand suture functions and normal fusion times:
Sutures on the skull allow for molding during birth passage and enable rapid brain growth.
Brain volume triples by age one, reaches about 80% of adult size by two, and 90% by three. Skull sutures, except for the frontal fontanelle which closes by eight months, fully fuse in adulthood. Early fusion can impact brain development variably, but quantification is difficult.
Some babies have early anterior fontanelle closure, raising concerns about craniosynostosis, but it's not always related and requires specialist consultation.
How does early suture fusion affect my baby?
Craniosynostosis may lead to several issues:
"I'm not concerned about my baby's appearance. Do they still need surgery?" Many parents ask this.
Theoretically, one fused suture raises intracranial pressure risk by about 17%, while two or more raise it to 47%.
Increased intracranial pressure poses life risks and requires treatment, but what if pressure isn't elevated?
Measuring intracranial pressure in infants is challenging; current non-invasive methods include clinical assessment by neurosurgeons, brain ultrasounds, or ophthalmoscope exams.
These require specialist help and can't be done at home.
Including brain development, developmental issues, or pressure effects on the brain, without surgery, it's impossible to predict or guarantee future impacts on the baby.
"I've read articles saying surgery before or after age one greatly affects intelligence."
Recent studies increasingly show craniosynostosis-related skull abnormalities "may" correlate with intelligence development, and surgery can improve intelligence and learning abilities.
These studies come from large medical centers in North America and Europe, reviewing patient data and comparing surgery timing with intelligence test results.
"Intelligence" tests include IQ, learning skills like reading, behavior, or academic performance. For common sagittal craniosynostosis, surgery before or after one year shows statistical differences in intelligence scores; recent research indicates earlier major skull expansion surgery yields better intelligence outcomes.
Globally recognized surgery timing is around 8-12 months, when babies are larger, anesthesia risks lower, for posterior skull expansion or anterior reshaping. This is when skull bones are thinner, surgery easier, and post-surgery suture fusion smoother.
For popular North American endoscopic small-incision suturectomy, optimal timing is 2-4 months, requiring early detection, but not all craniosynostosis types respond well.
"If I decide against surgery now, can we wait until the baby is older and more aware before proceeding?"
As mentioned, optimal surgery timing balances risk, ease, fusion ability, and brain development impact.
Later surgery makes bone fusion harder, potentially requiring additional skull repair before school age, increasing procedures.
Skull development involves growth and thickening, with later-forming "sinuses" at brain-face interfaces. Delaying surgery increases difficulty, complications, and bleeding risks, as these sinuses are prone to infection.
Thus, surgery becomes harder with age. If delaying until adolescence for appearance reasons, careful consideration is advised.