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Trigonocephaly, also known as metopic synostosis, refers to the premature closure of the forehead suture. This condition occurs when the cranial suture in the forehead fuses earlier than expected. Understanding the typical timeline for suture closure is crucial for diagnosis.
The forehead suture is unique as it is the first cranial suture to close, typically around eight months of age. Other sutures usually remain open until puberty. Early diagnosis is essential, often requiring clinical expertise alongside imaging like CT scans after eight months to confirm the condition.
In Western populations, trigonocephaly affects approximately 1 in 15,000 newborns. It is three times more common in boys than girls. About 6% of cases are linked to genetic factors, while 15% involve additional abnormalities, such as corpus callosum atrophy, heart issues, limb deformities, or urinary tract problems.
Increased Intracranial Pressure: Similar to other forms of craniosynostosis, confirming elevated brain pressure is key. The risk for trigonocephaly is around 8-15%. If present, intervention is necessary.
Appearance Changes: The head may appear triangular from above, with a pointed forehead, sunken orbits, and a narrower eye frame. Eyes might seem crossed, though vision is usually unaffected, and strabismus can occur.
Developmental Impacts: Premature closure can affect frontal lobe development, influencing cognitive functions like emotion, problem-solving, memory, language, judgment, personality, and communication. Approximately 31-33% of affected children experience these issues.
From the top, the head resembles a triangle with a pointed forehead and sunken areas above the eye sockets. Variations exist; milder cases may not show a sharp point but still feature sunken orbits and closer eye spacing, giving a cross-eyed appearance. The eye frames are wider at the top and narrower below.
Treatment for craniosynostosis follows similar principles. Functional needs take priority—if intracranial pressure is elevated, surgery is required. Otherwise, aesthetic concerns may guide decisions. The optimal surgery window is 9-12 months, as younger skulls have better healing potential. Delaying increases complications and blood loss.
Early detection (within four months) allows for endoscopic-assisted suture release, followed by custom helmet therapy. Traditional suture release has evolved with helmets for better outcomes, though results vary. For trigonocephaly, surgery involves reshaping the forehead and orbits, similar to unilateral coronal synostosis correction. An incision at the crown enables neurosurgeons and pediatric surgeons to remove and reshape bone, expanding brain space and improving appearance.
Post-surgery, patients stay in the neurosurgery intensive care unit for 1-3 days, then transfer to pediatric wards. Discharge typically occurs after about a week. Swelling may prevent eye opening initially, peaking around day three, with gradual improvement by day four. Normal diet and behavior return before discharge.
The primary goal of surgery is to expand the forehead and brow bones for increased brain capacity, as demonstrated in simulations.
Surgical focus is on the orbital and forehead bones, leading to post-operative swelling of the forehead and eyelids. Eyes open gradually 3-5 days later. Initial unevenness resolves as bones absorb over about 18 months. Future adjustments may be needed due to unpredictable bone growth.