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Address: Calwerstr. 7
72076 Tübingen


E-mail address: face@​med.​uni-​tuebingen.​de


frontend.sr-only_#{element.icon}: +49 7071 29-83799


Craniosynostoses - growth disorders of the skull

Craniosynostoses

Growth disorders of the skull

A craniosynostosis is a premature ossification of one or more of the so-called cranial sutures, which is accompanied by a circumscribed or general growth disorder of the cranial skeleton. The growth impulse comes from the rapidly enlarging brain, not from the bone itself.

There are four main cranial sutures (frontal, coronal, sagittal, and lambdoid), which, with the exception of the frontal suture, which ossifies already towards the end of the first year of life, normally do not close until the second to third decade of life. The open cranial sutures in childhood ensure, on the one hand, the displacement of the cranial bones during birth and, on the other hand, the rapid growth of the skull due to the brain growing very fast in the first year of life.

In case of a premature closure of one or more cranial sutures, a growth retardation of the skull and resulting characteristic deformation of the skull occurs.

Father lies with child on chest
The general goal of therapeutic measures for craniosynostosis is to eliminate, mitigate, or prevent as many of the negative consequences of synostosis as possible, thereby creating optimal conditions for the child's development.

Interdisciplinary consultation

Contact

Interdisciplinary consultation
for Craniofacial Anomalies

frontend.sr-only_#{element.contextual_1.children.icon}: Neuropediatric Outpatient Clinic,
Children's Hospital C3
Hoppe-Seyler-Str. 1
72076 Tübingen, Germany


Directions

Thursday 3-5 pm
1x monthly

+49 7071-29-83806


Departments involved:
Neuropediatrics
(OA Dr. Jan Kern, Dr. Karin Haas-Lude)
Neurosurgery
(Prof. Dr. Martin Schuhmann)
Oral and maxillofacial surgery
(Prof. Dr. Dr. Michael Krimmel)


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Therapy

The general goal of therapeutic measures for craniosynostosis is to eliminate, mitigate or prevent as many of the negative consequences of synostosis as possible, thereby creating optimal conditions for the child's development. Depending on the form and characteristics of the synostosis, aesthetic aspects or reasons such as lowering of increased pressure in the head or protection of the eyes by enlarging the eye socket are in the foreground.

Depending on the form of synostosis, there are two surgical principles, which are performed in joint work by pediatric neurosurgery and oral and maxillofacial surgery.

Passive remodeling

In passive remodeling, as used for sagittal suture synostosis, for example, the correspondingly ossified cranial suture and the adjacent bones of the cranial calvaria are removed. Within a few months, the bone is remodeled by the body (called reossification) and the skull is remodeled in the interval in between according to the shaping force of the growing brain. This operation should be performed between the 5th-6th month of life, because at this time of maximum brain growth the internal pressure in the head is highest and the reossification also occurs without problems. Surgery at an earlier time, e.g. at 3 months of age, requires additional treatment with a helmet to support the reshaping. This early endoscopically assisted therapy offers advantages especially in metopica and coronary suture synostoses, as a later, reshaping and major surgery can often be avoided.

Active remodeling

Active remodeling is used, for example, in frontal suture/metopicasynostosis or isolated coronal suture synostosis at the age of 10-12 months. In this case, the skull bone in the area of the forehead and the roof of the orbit, including the ossified suture, is removed, divided into suitable segments, actively remodeled and then reassembled in the desired shape and fixed to the base of the skull. This improves the deformity in the area of the forehead and ocular skeleton and, in the case of syndromal craniosynostoses, also creates space in the head.

In some synostoses, a backward extension of the skull is also applied.

The most important risk of these operations is relevant blood loss, which is mainly due to the large wound area. In this case, the administration of blood reserves and clotting factors is often necessary despite all blood-saving surgical techniques and measures taken during the operation as a rule. After the operation, the children spend one night in our pediatric intensive care unit to ensure optimal care and monitoring of blood values. The total inpatient stay is between 4-7 days, depending on the type of surgery and the age of the child.

The probability of a craniosynostosis recurring after a successful operation, i.e. the space for the brain becoming too small so that a second operation is necessary, is a maximum of 10% for isolated synostoses and at least 60% for syndromes with many affected sutures.


Follow-up

After surgery, children are followed up regularly for years in our craniofacial consultation by a team of pediatric neurosurgeons, oral and maxillofacial surgeons, and neuropediatricians to detect any recurrences early.

Forms of craniosynostoses

Scientific illustration
Premature suture closure with respective resulting characteristic head shape compared to the normal skull shape in open skull sutures. The closed suture is no longer visible in the respective image.

In sagittal suture synostosis, the altered direction of growth results in an elongated, narrow skull shape with a protruding back of the head and prominent forehead (so-called scaphocephalus). The fontanel is often narrow or completely closed and a bony groin can be palpated in the area of the sagittal suture.

Metopicasynostosis results in a head shape that is pointed in the forehead region often with a palpable bony ridge at the metopic suture. The shape of the forehead may not develop normally and there is often retraction in the temporal region or over the lateral region of the orbits. As a compensatory reaction, one can often observe an increased growth of the skull in the posterior region. All changes together lead to a classic triangular head shape (so-called trigonocephalus), especially when viewed from above.

In unilateral coronal synostosis, asymmetry of the forehead and also face develops (so-called facial scoliosis) with flattening of the forehead and orbit on the affected side and protrusion of the forehead on the opposite side (so-called anterior plagiocephalus). The eye on the affected side often appears larger, and the nose may deviate to the opposite side. Bilateral coronal synostosis is very rare and leads to increased skull growth in width (so-called brachycephalus). A very high and flat forehead develops, the eye sockets may be reduced in size. Often the skull grows compensatory in height (so called turricephalus).

In lambdoid synostosis, there is a unilateral asymmetry of the back of the head (so-called posterior plagiocephalus) with flattening on the affected side and compensatory protrusion on the opposite side. Asymmetry of the ears may be noticeable when viewed from the front. The main differential diagnosis to the very rare lambdoid suture synostosis is the so-called and relatively frequent "positional plagiocephalus" in which the lambdoid sutures are open.

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