Can sagittal craniosynostosis be corrected in adulthood
Synonyms: skull deformity, premature (premature) skull suture closure
The term craniosynostosis actually describes a premature closure of the cranial sutures. Skull sutures are the bone growth zones where the head can rapidly increase in size in infancy. This provides enough space for the rapidly developing brain and prevents the brain from coming under pressure or even from being damaged if there is not enough space (see skull and head shape).
Due to the changes in the bones of the skull base, it can also lead to accompanying deformities of the facial skull. The indication for an operative correction arises not only from possible neurological symptoms (developmental delay, pain, visual disturbances etc.), but also from the aesthetic aspect, which can seriously influence the further development of the child and his psyche.
For the correction of skull deformities, Children's Neurosurgery Leipzig is the first center to use MRT-based, individually manufactured CAD templates and resorbable implants made for your child, on which the head shape of your child can be calculated and modeled in advance. In this way, an optimal correction, symmetry and aesthetic result is achieved, especially with corrections involving the midface. Due to the optimal planning and fit, it is also possible to shorten the operation time, which also benefits the little patient. The resorbable implants make second operations to remove titanium material superfluous.
Pediatric Neurosurgery Leipzig conducts two 14-day consultation hours with colleagues from oral and maxillofacial surgery, in which all aspects of craniofacial changes, sutures and positioning deformities can be diagnosed and treated. Leipzig is one of the few centers in Germany that has many years of experience and consistently interdisciplinary care for patients.
The growth of the bony skull is mediated via the skull base and the meninges and is primarily linked to the growth of the brain, which also dictates the rate of increase in size. The cranial sutures are essentially named sagittal sutures, lamda sutures, coronary sutures and frontal sutures (metopica) according to their direction or shape and course (see Figures 1 and 2). Two connective tissue openings in the middle line at the intersection of the large sutures form the so-called large and small fontanels as a pulsation and expansion reserve. At around two to five months of age, the posterior, triangular, smaller fontanel closes. By the age of one year, the skull is almost 90% complete. At the age of 2 the sutures close, so that further bone growth takes place only very slowly via an internal breakdown and external build-up. The large anterior fontanel closes at the age of two. 95% of the growth is achieved by the age of 6, from the age of about 7 years there is hardly any further growth. The facial skull has a completely different dynamic over time and develops into the final face of the adult until puberty.
All so-called primary craniosynostoses can be explained by a growth disturbance of the skull bone mostly in the area of the base and thus an asymmetry or distortion. They occur with increasing frequency in about 1: 1000 births. The occurrence of a craniosynostosis can also be determined by a lack of growth of the brain or an incorrect production of cerebrospinal fluid. These so-called secondary craniosynostoses usually do not need to be treated.
The craniosynostosis should not be confused with a position-related flattening of the head, which can typically and very often occur on the back of the head (position-related plagiocephaly). This is explained by the most preferred supine position of the child, which has been recommended since the 1990s to avoid sudden infant death syndrome. In this case, attention should be paid to a change in lying habits and the child should be checked again after a few weeks.
The diagnosis of craniosynostosis as well as positional plagiocephaly is essentially based on the clinical examination. An ultrasound examination can confirm the often clinically clear diagnosis of suture synostosis and thus help avoid unnecessary radiation exposure of the child. If further clarification is required, a native X-ray examination of the head is recommended. Computed tomography of the head offers the highest resolution and makes the bony structures with the cranial sutures visible in detail. The type and scope of any operation that may be necessary in the case of a craniosynostosis should be made dependent on this examination.
The therapy depends on the affected skull sutures and the extent of the skull deformity.
The minimally invasive endoscopic correction of craniosynostoses can already take place at 6 - 8 weeks of life and is particularly gentle due to the significantly shorter duration of the operation, the keyhole technique with minimal wound surface. Due to the very early correction, complex reconstruction or plating of cranial bones is not necessary. As a rule, removing the pathologically altered suture and notches on the bone is sufficient to normalize the growth of the skull. Helmet treatment can often be dispensed with after the operation thanks to positioning therapy.
A open surgical correction should have occurred between the 4th and 15th month of life, depending on the location and extent of the synostosis. The beneficial effect on the further development of the child is greatest at this early age. In old age, more extensive operations on the skull bone are often necessary due to the advanced ossification. The surgical corrections must take into account the individual circumstances, such as the course of the blood vessels and the nature of the bones, and require a special surgical reconstruction. The position-related head deformities are an exception. Depending on the extent of the position-related plagiocephaly, a wait-and-see attitude may be justified (see helmet therapy).
The most common form of premature cranial suture closure is the so-called sagittal suture synostosis, which leads to a scaphocephalus (long skull). It corresponds to an elongated, narrow head with a keel-like, bony protrusion in the area of the sagittal suture (arrow suture) or in the area of the forehead and the back of the head. The unilateral coronary synostosis leads to so-called plagiocephaly, a head and facial skull deformity with flattening of the affected side. In addition to reconstructing the forehead area, surgical correction should also include the eye sockets (orbit) of the face. The same procedure applies to bilateral coronary synostosis, which leads to more pronounced brachycephaly with a high and steep forehead shape. Frontal suture synostosis (trigonocephalus), which is characterized by a prominence of the forehead with accompanying bony changes, also in the eye sockets, is less common. The lambda synostosis, which causes a unilateral or bilateral flattening of the back of the head, is just as rare.
Genetically determined syndromes such as Apert, Crouzon or Carpenter syndrome, which can show bony malformations of the arms and legs or internal organs in addition to the closure of several cranial sutures, must first be subjected to a careful pediatric diagnosis in order to achieve a treatment plan that is as holistic as possible to be able to create. Here, the opening of the cranial sutures is absolutely necessary, as several sutures are usually affected at the same time and this considerably restricts brain growth and thus the development of the child.
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