Results of Surgical Treatment of Occult Spinal Dysraphisms-A Single Centre Experience.

Occult spinal dysraphisms (OSDs) are caused by various defects in the embryogenesis of the spinal cord and represent an obstacle to the ascent of the conus, which allows the conus to pass from the lower levels of the spinal canal to the final position between L1 and L2 during normal foetal life. When an OSD tethers the spinal cord at the lower levels, it can lead to neurological symptoms, better known as tethered cord syndrome. Surgical treatment of OSD is primarily aimed at untethering the spinal cord. In asymptomatic patients, this can protect against the long-term development of neurological deficits. In symptomatic patients, this can halt or limit the progression of existing symptoms. The aim of this study is to examine all paediatric and adult patients diagnosed with OSD and treated in the Department of Neurosurgery at the University Medical Centre Ljubljana during the 5-year period of 2016-2021. All patients diagnosed with OSD during this period were included in the study. Patient characteristics, treatment modalities and outcomes were studied with the aim of describing the differences between the paediatric and adult population and defining the rationality of treating these pathological conditions. We included in the study 52 patients with 64 occult dysraphic lesions. Adults (>18 years old) represented 15/52 (28.8%) of all patients, while 37/52 (71.8%) were children. The most common OSDs were conus lipomas, followed by dermal sinus tracts, filum terminale lipomas and split cord malformations. Surgical treatment was performed in 35/52 (67.3%) cases, while conservative management was chosen in 17/52 (32.6%) cases. The preoperative presence of symptoms was statistically higher in adults than in children (p = 0.0098). Surgery on complex spinal cord lipomas was statistically related to a higher rate of postoperative neurological complications (p = 0.0002). The treatment of OSD is complex and must be based on knowledge of the developmental anomalies of the spine and spinal cord. Successful surgical treatment relies on microsurgical techniques and the use of neuromonitoring. Successful treatment can prevent or limit the occurrence of neurological problems.

Case Report: Two cases of multiples and atypical dermal sinus tracts.

Dermal sinus tracts (DSTs) are congenital lesions that connect the cutaneous ectoderm with the underlying neuroectodermal tissues. They are typically midline, solitary lesions. Multiple, and atypically located DSTs have been only rarely described. We present two cases of multiple and laterally located DSTs. The first presented with bacterial meningitis and two tracts in the right buttock, one of which entered the spinal canal through the S3 neural foramen. The second child had three midline lumbar DSTs, one subcutaneous dermoid cyst and one intradural epidermoid cyst. Complete surgical excision was achieved in both cases with good late follow up. Multiple or atypically located DSTs appear to carry the same risks of infection as the more common, midline, single tracts. Complete surgical excision is recommended to avoid the risks of neurological deterioration, in particular due to infection. Incomplete disjunction is the proposed developmental anomaly for DSTs, however the location of the cases presented here requires an alternative explanation.

Isolated Sagittal Craniosynostosis: A Comprehensive Review.

Sagittal craniosynostosis, a rare but fascinating craniofacial anomaly, presents a unique challenge for both diagnosis and treatment. This condition involves premature fusion of the sagittal suture, which alters the normal growth pattern of the skull and can affect neurological development. Sagittal craniosynostosis is characterised by a pronounced head shape, often referred to as scaphocephaly. Asymmetry of the face and head, protrusion of the fontanel, and increased intracranial pressure are common clinical manifestations. Early recognition of these features is crucial for early intervention, and understanding the aetiology is, therefore, essential. Although the exact cause remains unclear, genetic factors are thought to play an important role. Mutations in genes such as FGFR2 and FGFR3, which disrupt the normal development of the skull, are suspected. Environmental factors and various insults during pregnancy can also contribute to the occurrence of the disease. An accurate diagnosis is crucial for treatment. Imaging studies such as ultrasound, computed tomography, magnetic resonance imaging, and three-dimensional reconstructions play a crucial role in visualising the prematurely fused sagittal suture. Clinicians also rely on a physical examination and medical history to confirm the diagnosis. Early detection allows for quick intervention and better treatment outcomes. The treatment of sagittal craniosynostosis requires a multidisciplinary approach that includes neurosurgery, craniofacial surgery, and paediatric care. Traditional treatment consists of an open reconstruction of the cranial vault, where the fused suture is surgically released to allow normal growth of the skull. However, advances in minimally invasive techniques, such as endoscopic strip craniectomy, are becoming increasingly popular due to their lower morbidity and shorter recovery times. This review aims to provide a comprehensive overview of sagittal craniosynostosis, highlighting the aetiology, clinical presentation, diagnostic methods, and current treatment options.

Biparietal remodelling and total vault remodelling in scaphocephaly-a comparative study using 3d stereophotogrammetry.

PURPOSE: The aim of the study was to compare the results of two surgical techniques for the treatment of isolated sagittal synostosis (ISS) by means of 3D stereophotogrammetry. One technique, the Renier's "H" technique (RHT) comprised a biparietal expansion, the other, the total vault remodeling (TVR) included also a frontal remodeling. METHODS: The two groups of operated children were compared with a third control group of normocephalic children. The 3D scanning was performed in all children between 12 and 245 months of age. On each 3D image six measurements and indices have been made, with the aim of evaluating not only length and width of the head, but also the height. The cranial index (CI) was measured in a plane parallel to the nasion-tragus plane, at the intersection with the opisthocranion. RESULTS: Each of the three groups (RHT, TVR, control group) included 28 children. The measurements that were influenced by the correction of the frontal bossing, namely the CI and the sagittal length, were closer to normocephaly after TVR than after RHT. Lesser or no statistical difference was documented in the measurements evaluating the biparietal aspect and the height of the vertex, indicating that the biparietal expansion is effective in both procedures. CONCLUSION: Based on our results TVR results in a better esthetical outcome, particularly in relation to the direct surgical remodeling of the frontal bossing.

COVID-19 and Surgical Practice in Slovenia: Managing the Crisis in Neurosurgery during the COVID-19 Pandemic.

Worldwide, the novel coronavirus disease 2019 (COVID-19) has become a significant threat to global health. Worldwide, COVID-19 has affected the health service also in Slovenia. During this time, neurosurgery is facing difficulties in its service, both in emergency and elective surgeries. In the article, we describe the anti-COVID-19 measures taken at our neurosurgical department in a medical centre in Ljubljana, Slovenia, and analysed and compared the number of emergency and elective neurosurgical procedures during the time of the pandemic.

Craniofacial reconstructions in children with craniosynostosis.

BACKGROUND: Craniosynostosis is a rare congenital disease of the skull. They arise when one or more cranial sutures ossify prematurely. This causes an obstruction to normal brain growth and leads to specific deformations of the skull, which may result in intracranial hypertension and cognitive delay. MATERIALS AND METHODS: We have retrospectively analysed all children treated at the Unit of paediatric neurosurgery of the University Medical Centre Ljubljana between June 2015 and September 2020. The following items have been recorded: affected suture, underlying syndromic condition, hydrocephalus, Chiari malformation, raised intracranial pressure, age at surgery, surgical technique, need for multiple operations and surgical complications. RESULTS: During the study period, 71 children have been treated for craniosynostosis. The median postoperative follow-up was 31 months. There were: 54.9% sagittal, 25.3% metopic, 14.0% unicoronal, 1.4% bicoronal and 1.4% unilateral lambdoid craniosynostosis. Multiple sutures were affected in 2.8% cases. 7.0% of the cases were syndromic. Overall, 74 surgical procedures have been performed: frontoorbital advancement represented 40.5% of them; biparietal remodelling 32.4%: total cranial vault remodelling 22.9%; posterior distraction 2.7%; posterior expansion 1.3%. Median age at surgery was 12.8 months. CONCLUSIONS: The treatment of craniosynostosis is surgical and requires a multidisciplinary approach, with expertise in plastic and reconstructive surgery, maxillofacial surgery and neurosurgery. The aim of surgical treatment is to release the constrictive and deformative effect that the synostosis has on skull growth. This requires a remodelling of the neurocranium and, if necessary, of the viscerocranium. Beyond aesthetic purposes, the primary aim of surgical treatment is to permit a normal development of the brain.

Selective dorsal rhizotomy: short-term results and early experiences with a newly established surgical treatment in Slovenia.

BACKGROUND: Spasticity is characterised by an atypical increase of muscle tone, affecting normal movements and interfering with the patient quality of life. The medicines may limit the effects of the disease and selective dorsal rhizotomy (SDR) can be used for selected cases or cases refractory to medicine. We present the surgical technique and the short-term results of this newly established surgical treatment in Slovenia. METHODS: A retrospective analysis was performed of all patients that underwent the SDR from 2017 to 2019. The median follow-up was of 10 months. The following data have been collected: aetiology of spasticity, age at SDR, number of sectioned lumbar rootlets L1-S2, intraoperative disappearance of the H-reflex and intraoperative preservation of the bulbocavernosus reflex. The motor functions of all children have been classified by the Gross Motor Function Classification System (GMFCS) and Gross Motor Function Measure (GMFM-88). Twelve children underwent SDR, the median age at surgery was 9.6 years (min 3.9-max 16 years). RESULTS: A mean of 57.8% of dorsal rootlets L2-S1 have been cut, while at level L1 50% of the dorsal roots have been routinely sectioned. The median amount of S2 rootlets cut was 14.3%. Postoperatively, we observed a sudden decrease in muscle tone. In all patients, there was an improvement of the muscle tone and of the gait pattern. The GMFM improved from 187.8 to 208.3 after a follow-up of 6 months. CONCLUSIONS: There was no complication in terms of wound healing, cerebrospinal fluid fistula of neurological dysfunctions. Despite the relatively short follow-up, our early results confirm the efficacy of the SDR.

Chiari malformations in children: An overview.

Chiari malformations encompass various radiological and clinical entities, sharing the herniation of the rhombencephalic structures through the foramen magnum as a common characteristic. They can be symptomatic or asymptomatic. The therapeutic strategies for these malformations differ on the basis of the diverse pathophysiologic processes that cause them. As Chiari malformations are caused by various pathophysiologic processes, they must be recognized promptly to select the best treatment for each single case.

Myelomeningocele in Slovenia: results of a 10-year follow-up.

BACKGROUND: Myelomeningocele (MMC) is a congenital malformation that results from a failure in the process of neurulation. A multidisciplinary follow-up is required to manage and treat all associated sequelae. The aim of the study was to present the epidemiological data and the results of the multidisciplinary follow-up of children born in Slovenia with myelomeningocele (MMC) between 2007 and 2017. METHODS: We presented a retrospective analysis of all children born in Slovenia with between 2007 and 2017. The multidisciplinary follow-up included neurosurgical, urological, neurological, endocrinological and orthopedic expertise. RESULTS: Twenty children were treated in Slovenia for MMC from 2007 to 2017 (mean follow-up of 7.7 years). 9 MMC were thoracic, 2 higher lumbar, 5 lower lumbar and 4 sacral. Thirteen children needed a CSF shunt, 1 was treated with endoscopic ventriculostomy (ETV). Four children needed a craniocervical decompression and 2 needed a detethering procedure. 14 children had a neurogenic bladder and 17 referred bowel continence. Orthopedic correction of the lower limbs was required in 9 cases. 4 children had seizures, 10 had endocrinological deficits. Among 16 children attending school, 11 were inserted in special educational classes. CONCLUSIONS: The prevalence of MMC in Slovenia between 2007 and 2017 was 1/10000 births. Our follow-up results are comparable with those of previous, larger studies and confirm the efficacy of treating hydrocephalus with ETV in selected cases and with CSF shunt only in cases of clearly increased intracranial pressure. By adopting this strategy, we reduced the CSF shunt rate to 65%.

Endoscopic supracerebellar infratentorial approach to pineal and posterior third ventricle lesions in prone position with head extension: a technical note.

OBJECTIVE: The supracerebellar infratentorial (SCIT) approach is frequently used for the treatment of lesions in the pineal and posterior third ventricle region and can be performed in a sitting or prone position. We describe our experience with the purely endoscopic SCIT approach, using a prone position, with neck extension and head rotation. METHODS: We analysed all paediatric and adult patients operated on between July 2013 and January 2018 using the purely endoscopic SCIT approach. The surgical procedure was conducted through a paramedian subtorcular key-hole suboccipital craniotomy. The assistant held the endoscope and the surgeon used fine, long shaft instruments for bimanual tumour dissection. The prone position with head retroflexion allowed a gravity-aided enlargement of the anatomical surgical corridor between tentorium and cerebellum and enhanced venous blood drainage, similarly to the sitting or semi-sitting position, but with a decreased risk of air embolism. RESULTS: The approach allowed a complete removal of lesions located in the pineal region and in the posterior part of the third ventricle. Seven patients have been operated with this approach. All of them had the tumour completely removed. No permanent neurological deficits have been observed. CONCLUSIONS: The endoscopic SCIT approach enables effective and safe removal of pineal and posterior third ventricle lesions, even of moderate dimensions (up to 3 cm). The endoscope guarantees a detailed view and illumination of the deep-seated structures. The tumour resection can be performed with bimanual use of microsurgical instruments.

Endoscopic third ventriculostomy in obstructive hydrocephalus: A case report and analysis of operative technique.

BACKGROUND: The endoscopic third ventriculostomy (ETV) is a neuroendoscopical procedure that represents a more suitable alternative to the extracranial shunting. It consists of fenestrating the floor of the third ventricle and thus establishing a free flow of the cerebrospinal fluid from the ventricles to the site of resorption in the subarachnoid space. It offers a more physiological solution and a chance at a shunt-free life for children with hydrocephalus. The main indication for the procedure is obstructive hydrocephalus, however, it can also be useful in patients with other forms of hydrocephalus. CASE SUMMARY: We present a treatment flow of a 9-year-old patient, diagnosed with an obstructive hydrocephalus due to tectal glioma that was successfully treated with an ETV. We review the important factors influencing the success rate such as age, aetiology, shunt history, preoperative planning and visualisation of the basilar artery. CONCLUSION: Even though the ETV effectively controls obstructive hydrocephalus in more than 75% of all cases, the overall success rate of the procedure varies and could be approved by the correct preoperative patient selection.

Preoperative planning with a 3D model and repair of sphenoid dysplasia: a technical note.

INTRODUCTION: Sphenoid dysplasia is a distinctive marker of neurofibromatosis 1. It is a malformation of the sphenoid bone characterized by an enlargement of the superior orbital fissure, through which the intracranial content herniates, leading to a facial disfigurement and exophthalmos and to a potential visual deterioration. Surgical treatment has the aim of separating the intracranial from the intraorbital space. METHODS AND RESULTS: We present a new technique for the repair of sphenoid dysplasia, based on the preoperative planning on a 3D print of the skull base. CONCLUSIONS: The use of a 3D skull base model allows an effective surgical planning and a precise modelling of the titanium mesh, which is implanted to separate the intracranial from the intraorbital space.

Ureaplasma parvum ventriculitis related to surgery and ventricular peritoneal drainage.

Ureaplasma spp. usually causes genitourinary infections; few reports in the literature describe extragenital infections, usually in immunocompromised patients. We present a case of Ureaplasma parvum ventriculitis in an immunocompetent patient related to ventriculoperitoneal drainage and surgery. Ureaplasma parvum was detected with broad range 16S rRNA PCR and cultured on A8 agar.

TRANS-ENDOSCOPIC TREATMENT OF CRANIOPHARYNGIOMA AND RECOVERY FROM BLINDNESS IN ADULT PATIENT - A CASE REPORT.

We report a case of trans-endoscopic transventricular approach to a large cystic craniopharyngioma. Surgery was performed three days after visual acuity on both eyes deteriorated to blindness. Magnetic resonance imaging before surgery revealed a large lesion in the suprasellar region that severely compressed the optic chiasm and displaced the third ventricle upward. The lesion was operated through the trans-endoscopic transventricular approach, with the aim of urgent decompression of the optic apparatus. At first, wide ventriculo-cysto-cisternostomy was performed, and then tumor tissue was removed. Postoperatively, visual acuity significantly improved on one eye. Our case shows that this minimally invasive technique is safe and effective and can be an alternative treatment for large cystic craniopharyngiomas. The reported case also shows that loss of vision can still be recovered even after the 72-hour period in adults.

Selective dorsal rhizotomy in cerebral palsy spasticity - a newly established operative technique in Slovenia: A case report and review of literature.

BACKGROUND: Spasticity affects a large number of children, mainly in the setting of cerebral palsy, however, only a few paediatric neurosurgeons deal with this problem. This is mainly due to the fact that until 1979, when Fasano has published the first series of selective dorsal rhizotomy (SDR), neurosurgeons were able to provide such children only a modest help. The therapy of spasticity has made a great progress since then. Today, peroral drugs, intramuscular and intrathecal medicines are available, that may limit the effects of the disease. In addition, surgical treatment is gaining importance, appearing in the form of deep brain stimulation, peripheral nerve procedures and SDR. All these options offer the affected children good opportunities of improving the quality of life. CASE SUMMARY: A 15-year old boy is presented that was surgically treated for spasticity as a result of cerebral palsy. Laminotomy at L1 level was performed and L1 to S1 nerve roots were isolated and divided in smaller fascicles. Then, the SDR was made. CONCLUSION: We describe a patient report and surgical technique of SDR that was performed in Slovenia for the first time.

Vein of Galen aneurismal malformations - clinical characteristics, treatment and presentation: Three cases report.

BACKGROUND: The vein of Galen aneurismal malformations (VGAM) are rare arteriovenous malformations of the embryonic choroid plexus. They represent about 30% of paediatric neurovascular disorders and show diverse characteristics. The VGAM is constituted by a midline dilated venous structure that receives blood from abnormal macroscopic or microscopic arteriovenous shunting vessels. Two types of VGAM exist, the choroidal and the mural. The treatment represents a challenge with the therapeutic objective to preserve the normal brain development without creating new neurological deficits. CASE SUMMARY: We present three cases of VGAM in the early postnatal period and their treatment. All patents were treated with the endovascular technique, which was successful. According to our experience, the endovascular technique is a safe and efficient mode of VGAM treatment. CONCLUSION: The objective of treatment aims to child's normal neurological development. A proper selection of patients and a thorough diagnostic workup is of vital importance. When the endovascular treatment is performed, the primary aim is not a complete VGAM exclusion at one time, which could produce a sudden reversal of blood flow with consequent venous infarction and ischemia. The aim is therefore to occlude as much of the VGAM as needed to relieve the congestive cardiac failure, to gain time and to create the conditions for a normal maturation of the neurovascular system. With the use of endovascular techniques, which represent not only the first choice of treatment but also the only safe therapeutic modality, the natural history of VGAM and their risks may be avoided safely.

Risk factors for bad outcome in pediatric epidural hematomas: a systemic review.

BACKGROUND: Pediatric epidural hematomas (EDH) represent a neurosurgical emergency. Both surgical and conservative treatment can lead to a good clinical outcome. The aim of the study was to review our series of pediatric EDH and to determine the clinical and radiologic factors, which can influence the final outcome. METHODS: All children aged from 0 to 16 that have been treated between 2013 and 2017 for cranial EDH have been selected. RESULTS: Thirty children have been included in the study. Seventeen cases have been treated with surgical evacuation and 13 conservatively. Six months after the trauma, the outcome was excellent (mRS 0) in 25/30 (83.3%) cases, mild deficits (mRS 1-2) were present in 4/30 (13.3%), and severe deficits (mRS 3-5) in 1/30 (3.3%) cases. Only a GCS (Glasgow Coma Scale) below 8 at admission was significantly related to the presence of a neurologic deficit at 6 months (p = 0.048). CONCLUSIONS: EDH can be managed with excellent outcomes. Even in the presence of bad initial clinical and radiologic conditions, a correct treatment strategy can lead to a good recovery. In our series, only a GCS below 8 at admission was significantly related to the presence of neurological sequelae.

Craniosynostosis - Recognition, clinical characteristics, and treatment.

Craniosynostosis is a developmental craniofacial anomaly, resulting in impairment of brain development and abnormally shaped skull. The main cause of craniosynostosis is premature closure of one or more cranial sutures. It usually occurs as an isolated condition, but may also be associated with other malformations as part of complex syndromes. When left untreated, craniosynostosis can cause serious complications, such as developmental delay, facial abnormality, sensory, respiratory and neurological dysfunction, anomalies affecting the eye, and psychological disturbances. Thus, early diagnosis, expert surgical techniques, postoperative care, and adequate follow-up are of vital importance in treating craniosynostosis.

Clinical and neuroradiological features of the 9p deletion syndrome.

BACKGROUND: The 9p deletion syndrome is a rare condition, which associates trigonocephaly, facial dysmorphism and developmental delay. The neuroradiological aspects of this syndrome have not yet been described. The purpose of this article is to identify the clinical and neuroradiological features, that should be recognized by all specialists treating these children, for a proper and early diagnosis. METHODS: Among patients with trigonocephaly treated at our institution, we retrospectively analyzed the clinical and neuroradiological aspects of children with genetically confirmed 9p deletion syndrome. RESULTS: 6 patients were identified. Beside trigonocephaly, the most frequent clinical findings were small ears, long philtrum, upslanting palpebral fissures, flat nasal bridge and variable psycho-motor delay. Hypertelorism was present in 4 of 6 patient, which is opposite to the hypotelorism typical of non-syndromic trigonocephaly. Among neuroradiological findings, large, anteriorly rotated sylvian cisterns and altered shape of the septum pellucidum were found in all patients, as well as the compression of the frontal cortex due to the metopic synostosis (MS). A thin or dysmorphic corpus callosum and a diffuse white matter hypoplasia were present in more than half of the cases. Futhermore we compared these MRI findings with those of a control group of 30 non-syndromic trigonocephalies. CONCLUSIONS: Some recurrent neuroradiological alterations can be found in 9p deletion syndrome. The presence of these signs on MRI of a trigonocephalic patient should raise the suspicion of an underlying chromosomal alteration, such as the 9p deletion syndrome and prompt genetic investigations.