CONGENITAL ANOMALIS OF THE SKULL AND FACE
The congenital cranial and facial anomalies vary from the simple tongue frenulum to craniofacial dysmorphias (such as craniosynostosis, clefts, or congenital nevi). Complex anatomy requires the collaboration of different specializations for full restoration.
Craniosynostosis is particularly worth mentioning. This is the premature closure of the points (called “sutures”) where, at the beginning of our lives, the individual bones that make up the skull join one another. This results in skull development disorders that can cause dysmorphia but also, in the most severe cases, affect the development of the brain with catastrophic consequences. Fortunately, early diagnosis and successful intervention can frequently lead to our young patients living a normal life.
CRANIOSYNOSTOSIS
Depending on the number of sutures and their degree of involvement, craniosynostosis may cause very distinct dysmorphias. Surgical treatment to open the prematurely closed suture prevents poor skull and brain development. The most common skull development disorders as a result of craniosynostosis are:
Scaphocephaly or dolichocephaly
This is the most common one and is due to the early closure of the sagittal suture (an elongated suture at the top of the head) causing large skull length and short width.
Trigonocephaly
This is due to the early closure of the suture present on the forehead causing a triangular forehead shape possibly affecting the distance between the ocular orbits.
Posterior plagiocephaly
This is often mistaken for craniosynostosis. In fact, it is the result of repeated placement of the infant’s head in the exact same position. This is often the case for premature infants or infants requiring prolonged hospitalization. The skull bones still being very thin and supple, combined with the pressure from a pillow or bed at exactly the same point of the head can causes a widening in that particular part of the skull and compensatory deformity in other parts. Often, the position of the ear is also asymmetrical (one ear is seen more anteriorly placed compared to the other). Treatment by repositioning the head at an early age or treatment with a special helmet often corrects this problem and surgery is only performed in severe cases.
Brachycephaly
This is an early closure of the coronal suture that will affect the shape of the forehead and possibly the symmetry of the (ocular) orbits, nose and ears.
Finally, there are more complex genetic syndromes (such as Crouzon syndrome and microcephaly) that may simultaneously involve more sutures, thus necessitating a more complex (multidisciplinary) surgical approach.
