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[Craniosynostosis, a pediatric perspective]
José Pablo Fernández V1, Gabriela Chica H2, Andrés Goycoolea R1
1Instituto de Neurocirugía Asenjo, Santiago, Chile.
Insights
Craniosynostosis, the premature fusion of skull sutures, causes abnormal head shapes. Early recognition and surgical intervention, ideally before age one, improve outcomes and prevent complications like increased intracranial pressure.
Area of Science:
- Pediatric Neurosurgery
- Craniofacial Surgery
- Medical Genetics
Background:
- Craniosynostosis involves premature fusion of cranial sutures, leading to abnormal head shape.
- This rare condition requires timely recognition and neurosurgical referral to prevent complications.
- Delayed diagnosis is linked to intracranial hypertension and impaired brain development.
Purpose of the Study:
- To review the clinical and genetic features of craniosynostosis.
- To describe classification based on skull shape and key diagnostic signs.
- To emphasize timely recognition and management for better patient outcomes.
Main Methods:
- Literature search of PubMed, SciELO, and EMBASE databases.
- Keywords included: craniosynostosis, plagiocephaly, scaphocephaly, brachycephaly.
- Selection of Spanish and English articles, prioritizing systematic reviews and guidelines.
Main Results:
- Craniosynostosis can occur in isolation or with other deformities.
- Classification depends on affected sutures, dictating skull shape and associated malformations.
- Late diagnosis is common, leading to complications; early surgery offers better results.
Conclusions:
- Abnormal head shape should prompt suspicion of craniosynostosis, even when isolated.
- Surgical management before one year of age is associated with a better prognosis.
- Timely intervention reduces comorbidity and enhances aesthetic outcomes.
Abstract:
Craniosynostosis is defined as the premature fusion of one or more skull sutures, characterized by an abnormal shape of the head. It is a rare condition but should be recognized and timely referred to Neurosurgery in order to prevent complications. The objective of this review is to describe the most frequent clinical and genetic characteristics of this pathology, its classification according to the shape of the skull, and the most characteristic signs to achieve timely recognition. A search for scientific articles in Pubmed, Scielo, and EMBASE databases was performed using the terms craniosynostosis, plagiocephaly, scaphocephaly, and brachycephaly. We selected articles in Spanish and English that described the characteristics of the pathology and about its management, choosing systematic reviews or recommendations from scientific societies when available. Craniosynostosis may occur in isola tion or associated with other deformities. Its classification depends on the affected suture(s), leading to the characteristic shape of the skull and the presence of other malformations. This condition is usually diagnosed and referred late, which is associated with complications such as intracranial hy pertension and impaired brain development. Early surgery has less comorbidity and better esthetic results. In conclusion, the abnormal shape of the skull must raise the suspicion of craniosynostosis, even if it occurs in isolation. Surgical management before one year of life is associated with a better prognosis.
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