Pädiatrische Neurochirurgie: Schonende Endoskopie und Mikrochirurgie für die Kleinsten
Through the establishment of multidisciplinary pediatric boards, it has been ensured that our young patients suffering from neurosurgical conditions receive individualized therapy based on the latest multidisciplinary research at the earliest possible stage.
Within the framework of our specialized pediatric outpatient clinic, appointments are available every Friday following prior telephone scheduling.
At our clinic, strong interdisciplinary collaboration with the University Departments of Pediatrics and Adolescent Medicine, Radiology and Nuclear Medicine, Anesthesiology, General Intensive Care Medicine and Pain Therapy, and Radiation Oncology forms the foundation of pediatric neuro-oncology. Each case is discussed within the framework of the interdisciplinary Pediatric Tumor Board, and individually tailored therapies are established at the highest international standard and according to international study protocols.
Neuro-oncological treatment pathway for children and adolescents:
In close cooperation with the Division of Neuroradiology and Musculoskeletal Radiology, new and innovative imaging techniques are continuously applied to optimize preoperative diagnostics and the planning of oncological procedures in children and adolescents. These structural and functional imaging methods include functional MRI, resting-state fMRI (functional magnetic resonance imaging), visualization of fiber tracts using DTI (diffusion tensor imaging), as well as MR spectroscopy. The data obtained from these techniques can be integrated into intraoperative neuronavigation and used directly in the operating room to support tumor resection.
In addition to imaging, intraoperative neurophysiological monitoring represents another important technique for monitoring neurological functions in tumors located in functionally critical areas (spinal cord/spinal, brainstem and central region/intracranial). In selected cases, intraoperative monitoring of language function during awake surgery is also performed, in collaboration with the Department of Neurology.
Immediate postoperative monitoring takes place either in the Pediatric Intensive Care Unit (PICU) or in the Intermediate Care Unit of the Pediatric Surgical Center. Subsequently, the children continue to receive care in the Pediatric Oncology ward (Level 9).
Hydrocephalus is the most common condition in pediatric neurosurgery, which is why a joint management approach with the Department of Pediatrics and Adolescent Medicine has been established for many years. Due to both congenital and acquired forms, such as those occurring after hemorrhages or infections, children can already be affected shortly after birth. The treatment concept developed together with neonatology for premature infants with very low to extremely low birth weight—using temporary, continuous external ventricular drainage systems and temporary, closed subcutaneous reservoirs—is continuously being further developed and optimized through the use of new technologies, such as modified catheters and ultra-thin endoscopes.
Neuroendoscopy has become established in modern neurosurgery both as an independent method and as a technique that complements microsurgery, enabling minimally invasive treatment of various neurosurgical conditions. Endoscopes specifically developed for children, in combination with electromagnetic neuronavigation, make it possible to perform procedures for intracranial cysts or complex forms of hydrocephalus even in premature infants and newborns.
Endoscopic third ventriculostomy represents not only a good alternative to shunt surgery in cases of obstructive hydrocephalus in children, but—given its success rate—can also be regarded as a first-line therapy in this age group.
Endoscopic ventriculostomies performed both in newborns only a few days old and in older children enable normalization of cerebrospinal fluid (CSF) flow in a high percentage of cases without the need for implantation of a shunt system.
Endoscopic septostomies and other intraventricular fenestrations, in combination with a shunt system, can improve the treatment of complex forms of hydrocephalus with isolated compartments of the ventricular system and are already used in premature infants.
Intra- and paraventricular cysts can also be successfully treated in children as young as a few days old through a single endoscopic fenestration.
Cysts of the central nervous system (CNS) can occur in different locations: in the middle cranial fossa at the temporal lobe (temporal or Sylvian arachnoid cysts), around the cerebellum, at the base of the skull, around the ventricles, or in the cerebral hemispheres.
Depending on their location, they can cause different symptoms. Many cysts are discovered incidentally; they often cause no symptoms and in many cases do not require treatment. Treatment is necessary if they compress the cerebrospinal fluid (CSF) pathways and thereby cause an accumulation of CSF, which is associated with increased intracranial pressure.
Arachnoid cysts are usually congenital. They develop during the early development of the brain due to a splitting of the arachnoid membrane (arachnoidea), one of the meninges, within which cerebrospinal fluid normally flows. As a result of this splitting and adhesion, CSF accumulates within the space and a fluid-filled cyst forms. Arachnoid cysts rarely grow; if they do, it is mainly during the first and second year of life. Therefore, regular follow-up examinations are particularly important during this period.
There are different therapeutic options, with microsurgical fenestration and resection as well as endoscopic fenestration being recommended depending on the location of the cyst. Intra- and paraventricular cysts in children have already been successfully treated from as early as a few days of age by a single endoscopic fenestration.
Endoscopic treatment is less invasive and offers the advantage of a smaller skin incision and a more limited surgical approach to the cyst. However, certain characteristics of cysts can make endoscopic treatment more difficult. In such cases, microsurgical treatment is preferable.
Between 1993 and 1995, an epilepsy surgery program for the pediatric age group was established at AKH Vienna. Since 2005, children have been cared for and evaluated within the Epilepsy Monitoring Unit of the Department of Pediatrics and Adolescent Medicine. The center at AKH Vienna is a referral center for children with drug-resistant epilepsy. Since 2019, our epilepsy center has been recognized within the “European Reference Network for Rare and Low Prevalence Complex Diseases – ERN” as the only reference center for epilepsies (ERN EpiCare) in Austria.
Our therapeutic procedures within epilepsy surgery include hemispherotomies, multilobar disconnections, focal resections—if necessary with intraoperative electrocorticography—callosotomies, and vagus nerve stimulator implantation. Many of these procedures are performed in Austria only at the Department of Neurosurgery of the Medical University. For invasive chronic monitoring, subdural and/or depth electrodes are implanted depending on the individual case.
Kraniosynostosen
Craniosynostoses arise from the premature closure of cranial sutures, which leads to changes in the shape of the entire skull. Due to an imbalance between bone formation and resorption, and the interaction between the cranial vault and the skull base via the dura mater, characteristic—more or less pronounced—skull shapes develop depending on the location of the prematurely fused suture. With timely surgical intervention, the skull shape can be normalized.
Abnormalities in the shape of a child’s skull can have various causes. Sometimes they are due to normal changes associated with the rapid skull growth during the first months and years of life, but in other cases they result from disturbances in bone growth that may affect both appearance and brain development. Diagnosis is often straightforward and possible without radiological imaging, but it can also be more complex and require the involvement of different medical specialties. In our pediatric specialty clinic, both the interdisciplinary assessment of abnormalities in skull growth and the planning of further treatment are carried out.
Changes in skull growth caused by craniosynostoses are being diagnosed and suspected with increasing frequency. Accordingly, both the number of outpatient consultations of patients with craniosynostoses and the number of surgical procedures are rising.
The most common form of craniosynostosis is sagittal synostosis. Premature closure of the sagittal suture leads to a long, narrow head shape, also referred to as dolichocephaly (long skull) or scaphocephaly (boat-shaped skull). The forehead often appears prominent, while the back of the head is narrow and sometimes protruding. Boys are affected more frequently.
If diagnosed early, treatment can be performed either through open surgery or with endoscopic assistance by removing the ossified suture over a sufficiently large area.
In somewhat older children, the surgical procedure involves reconstruction of parts of the skull bone in the parietal and occipital regions in order to achieve sufficient expansion of the cranial volume and improve the shape of the skull.
Trigonocephaly is a cranial shape deformity that is primarily characterized by a pointed or triangular forehead, which can restrict the space available for the brain in this region. It is caused by the premature closure of the metopic suture, which runs along the middle of the forehead. The severity of this condition can vary.
Treatment involves reconstruction of the entire frontal region. In this procedure, both the upper part of the forehead and the transition to the skull base—the upper borders of the eye sockets—are reshaped from a triangular configuration into a form that is straight at the front and rounded toward the sides.
Asymmetrical skull shapes can be caused by the unilateral premature closure of the coronal suture or, in rare cases, the lambdoid suture, and are referred to as plagiocephaly. This results in asymmetrical development of the skull base, the cranial vault, and parts of the facial skeleton.
Treatment is performed surgically through reconstruction of the asymmetrically deformed forehead to restore symmetry. This involves reshaping both the upper part of the frontal bone and the area adjacent to the skull base, including the upper borders of the eye sockets.
However, not all asymmetrical skull shapes are caused by craniosynostosis. The distinction can usually be made through clinical examination without radiological imaging and is important because, in these cases, treatment can often be carried out without surgery through physical therapy, and helmet therapy may also be considered.
In brachycephaly, also known as tower skull, the premature bilateral closure of the coronal sutures leads to a flattened forehead; the head becomes short, wide, and high. The eye sockets are shallow and small, which can result in exophthalmos (protruding eyes). This type of craniosynostosis is frequently associated with molecular genetic abnormalities. Increased intracranial pressure can occur at an early stage. For these patients, timely and regular interdisciplinary care is particularly important in order to ensure the best possible development.
Treatment consists of timely expansion of the cranial volume through a procedure known as fronto-orbital advancement. The frontal bone and the anterior frontobasal region are reshaped and advanced forward. In syndromic conditions, this procedure may need to be repeated to ensure normal brain development.
Both the initial consultation and the ongoing care of patients with suspected or diagnosed craniosynostoses take place in a weekly pediatric specialty clinic, where diagnostic evaluation, further neurosurgical management, and interdisciplinary assessment are carried out. In many cases, surgery is recommended at the optimal time, which
- in some cases can be very early, because a “smaller,” less extensive procedure is then possible.
- in some cases must be performed early, because only in this way can secondary damage be prevented.
- in many cases can also be scheduled electively in order to achieve the best possible outcome.
- in most cases, surgery is performed within the first year of life.
Surgical procedures for these patients are carried out in close cooperation with the University Clinic for Pediatrics and Adolescent Medicine, the University Clinic for Anesthesiology, General Intensive Care Medicine and Pain Therapy, and the University Clinic for Oral and Maxillofacial Surgery.
While open dysraphisms have become rare in newborns in Europe due to advances in prenatal medicine, it is primarily the closed spinal dysraphisms that benefit from comprehensive interdisciplinary care. Their neurosurgical treatment is optimized through the systematic use of complete intraoperative neurophysiological monitoring.
Malformations belonging to the spectrum of dysraphic disorders are rare conditions affecting the spine and brain and are associated with neurological impairments of varying severity. While open dysraphisms have become very rare due to advances in prenatal medicine, closed spinal dysraphisms are being diagnosed with increasing frequency.
They represent a complex group of disorders that require optimal interdisciplinary management in order to achieve the best possible outcome for these patients. The spectrum includes a wide range of conditions and severities, such as dermal sinus, tight filum, diastematomyelia (“split cord”), meningoceles, lipomyeloceles, and caudal regression syndrome. The primary role of neurosurgery in these conditions is to determine the optimal management strategy, particularly regarding the timing and course of possible surgical treatment. Surgical indications are continuously expanding, as technical advances increasingly allow safe surgical solutions even in complex cases.
An important component of the microsurgical technique is intraoperative neurophysiological monitoring, which has been systematically used at our clinic since 2003 and helps ensure the best possible medical and therapeutic outcome with the highest level of safety for patients.
Both the preoperative assessment and the long-term follow-up of these patients are carried out in an interdisciplinary setting together with specialists from the Clinical Division of Neuropediatrics, Neuroradiology, Pediatric Surgery and Urology, and Pediatric Orthopedics. Regular joint case discussions take place in the Pediatric Dysraphia Board. Initial consultations and further care for patients are available in our specialty outpatient clinic every Friday. Scheduling an appointment in advance is recommended.
The treatment of vascular malformations in childhood is carried out similarly to that in adults, following discussion in the Vascular Board. Microsurgery, endovascular procedures, and radiosurgery with the Gamma Knife are used on a case-by-case basis, either alone or in combination.
The specific early childhood condition of vein of Galen malformations is treated endovascularly in collaboration with the departments of Neonatology, Neuropediatrics, and Pediatric Intensive Care Medicine, as well as the Department of Pediatric Cardiology at the University Clinic for Pediatrics and Adolescent Medicine.
Clinical Working Group for Neuropediatrics
Head:
Ass.-Prof. Dr.med.univ. Andrea Reinprecht
Deputy:
Dr.med.univ. Dr.scient.med. Johannes Herta