Craniosynostosis: Understanding the Misshaped Head - prodportal.clinicacolsanitas.com
Craniosynostosis: Understanding the Misshaped Head
Márquez JC, Herazo Bustos C, Wagner MW. https://doi.org/10.1148/rg.2021200127Abstract
Craniosynostosis is defined as the premature closure of one or more cranial sutures, which alters the configuration of the child’s head (Figure). The morbidity and mortality are related to intracranial hypertension, hydrocephalus, Chiari 1 deformity, upper airway obstructions, and developmental delay, especially in syndromic cases. Eighty-five percent of cases are nonsyndromic, and of these, 75% are single-suture synostoses and nearly 60% of those are sagittal synostosis.
Sagittal synostosis (scaphocephaly) typically spares the skull base. It manifests with frontal and occipital prominence, a narrow and elongated skull, a flat vertex, and ridging of the suture.
Figure. Three-dimensional CT reconstructions show the types of alterations of head shape in children.
The second most common type is coronal synostosis, which can be unilateral or bilateral, with the former being more common. This deformity is called anterior plagiocephaly with ipsilateral exophthalmos, frontal bone flattening, and contralateral bossing. Bicoronal synostosis (brachycephaly) manifests with a widened transverse diameter of the skull, harlequin deformity, and hypertelorism.
Metopic synostosis (trigonocephaly) is less common. Triangular or pearlike shape, parieto-occipital bossing, and narrow anterior cranial fossa are characteristic cranial features. Orbital features include quizzical orbits and lateral orbital hypoplasia.
The least common type is lambdoid synostosis (posterior plagiocephaly). It is characterized by occipitoparietal flattening, mastoid bulging, and contralateral occipitoparietal and frontal bossing. The keys to differentiate lambdoid synostosis from positional plagiocephaly are the trapezoidal shape of the skull and posterior displacement of the ear.
Syndromic synostoses are caused by specific mutations and manifest with multiple systemic alterations. Apert syndrome is caused by a mutation in the FGFR2 gene. The craniofacial findings include coronal synostosis (rarely, cloverleaf skull), midface hypoplasia, and hypertelorism. Further musculoskeletal anomalies include syndactyly, vertebral fusion (often at C5-C6), and joint deformities.
Crouzon syndrome is caused by mutations of the FGFR2 or FGFR3 gene. Craniofacial features are coronal synostosis (the sagittal and lambdoid sutures can also be affected), midface hypoplasia, and exophthalmos. Systemic features include hearing loss due to auditory meatus atresia, orbital defects, and cervical spine fusion.
Pfeiffer syndrome is caused by mutations in the FGFR1 or the FGFR2 gene. There are three types, with different degrees of fusion of the coronal and sagittal sutures. Type 1 is the classic form. Frequent manifestations include midface hypoplasia, hypertelorism, broad thumbs and toes, brachydactyly, and syndactyly. Type 2 manifests with cloverleaf skull, extreme proptosis, and ankylosis or synostosis of elbows. Type 3 is similar but without the cloverleaf skull.
The neuroradiologist’s role in pediatric assessment is the evaluation of the head shape with regard to premature fusion of the sutures, which can be depicted at CT. The radiology report should include a section about the vascular anatomy, including the course of the venous sinuses, large scalp or bridging veins (relevant for surgical planning), venous thrombosis, and arterial variants. In addition, craniovertebral junction anomalies and signs of increased intracranial pressure need to be assessed carefully. Postoperative complications such as stroke, hemorrhage, cerebrospinal fluid leaks, intracranial collections, and venous damage or thrombosis must be ruled out.
In the online presentation, the anatomy and embryology of the skull and the main craniosynostoses are presented, as well as the protocol for low-dose CT.
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