Long-term results of minimally invasive strip craniectomy without helmet therapy for scaphocephaly - a single-centre experience.

Scaphocephaly is the most common type of craniosynostosis and various surgical techniques are used for treatment. Due to late postoperative changes of the head shape, long-term outcome data is important for evaluating any new surgical technique. At our institution, minimally invasive strip craniectomy without regular helmet therapy is the standard treatment in scaphocephalic patients. Between October 2021 and February 2023, we retrospectively examined the skull shape of patients who underwent minimally invasive strip craniectomy for scaphocephaly using a 3D surface scan technique. The cephalic index (CI), the need for helmet therapy and additional cosmetic outcome parameters were investigated. We included 70 patients (72.5% male). The mean follow-up time was 46 (10-125) months and the mean CI was 75.7 (66.7-85.2). In 58 patients, the final cosmetic result was rated as "excellent/good" (mean CI: 76.3; 70.4-85.0), in 11 as "intermediate" (mean CI: 73.3; 66.7-77.6), and in one case as "unsatisfactory" (CI 69.3). The presence of a suboccipital protrusion was associated with a "less than good" outcome. The CI correlated significantly with the overall outcome, the presence of frontal bossing, and the interval between scan and surgery (age at scan). Minimally invasive strip craniectomy is an elegant and safe method to correct scaphocephaly. Our data show good cosmetic results in the long term even without regular postoperative helmet therapy.

Clinical variety and prognosis of intracranial arachnoid cysts in children.

Arachnoid cysts (AC) occur in different intracranial locations. Management and prognosis depend on the clinical presentation and treatment guidelines do not exist. With this study, we want to demonstrate the clinical variety of arachnoid cysts in children and place a focus on outcome factors in operated cases. This retrospective study of a consecutive single unit series of children, who underwent AC surgery between January 2010 and September 2019, provides demographic, clinical, imaging data, and information about surgical treatment and outcome. Overall, 63 patients (71.4 male) underwent surgery. Mean age was 50 months (0-191). Mean follow-up was 40 months (0-121). Eighty-one percent of patients presented with symptoms/signs of raised ICP. Focal neurological deficits were present in 15.9%, headache in 11.1% of children. Galassi cysts represented the predominant type (30.2%), followed by suprasellar (14.3%), quadrigeminal (12.7%), retrocerebellar, CPA and midline (each 11.1%), and hemispheric cysts (7.9%). Endoscopic and microsurgical fenestrations were performed in 27% and 58.7%, stent or shunt insertion in 6.3%/57.9% of the cases. In 33.3% of the cases one and in 12.7%, a second reintervention became necessary. Reoperation rate was significantly higher in children < 1 year (p = 0.003). Cyst volume decreased in 85.7%. Seventy percent of the patients were symptom free, 5% suffered from headache, and 22% from developmental disorders. All focal neurological symptoms resolved. Complication rate and outcome are depending on age and cyst location. Recurrence and revision rates are significantly higher in young infants (p = 0.003). Midline cysts with CCA are associated with developmental disorders.

Follow-up study to investigate symmetry and stability of cranioplasty in craniosynostosis - Introduction of new pathology-specific parameters and a comparison to the norm population.

PURPOSE: Cranioplasty and modulation of frontoorbital advancement (FOA) in children with craniosynostosis aims to achieve an attractive aesthetic and functional rehabilitation of the forehead area, comparable to that in unaffected children. Based on a three-dimensional surface scan, a cephalometric data evaluation with new parameters for the quantification of physiological and pathological cranial morphologies, and objective evaluation of postoperative follow-up in comparison to an age-equivalent standard population, were performed. MATERIALS AND METHODS: In a prospective study, 80 children were operated on with non-syndromic craniosynostosis (trigonocephalus, n = 30; plagiocephalus, n = 10; scaphocephalus, n = 38; brachycephalus, n = 2) and pre- and 3, 6, 12, 18 and 30 months postoperative three-dimensional surface scans were obtained (3DShape, Erlangen, Germany) and morphometrically measured (Onyx Ceph, Image Instruments, Chemnitz, Germany). In addition, 49 healthy children who were not operated on were measured at equivalent ages (n = 25 [6 months]; n = 20 [9 months]; n = 4 [12 months]). RESULTS: All patient groups showed stable long-term results with regard to shaping of the forehead. Cranioplasty in patients with scaphocephalus resulted in a significant widening of the anterior (73.9 ± 3.5 mm; p < 0.001) and posterior (132.2 ± 5.2 mm; p < 0.001) cranial width, with no significant difference from the norm population 1 year after surgery (p = 0.6597). As parameters for the correction of trigonocephaly, the frontal angle showed significant improvement (145.9 ± 3.7°; p < 0.001). While the parietal angle 12 months after surgery showed similar values as the norm population, the frontal angle was about 10° smaller than in healthy children (p = 0.0055), despite a clinically inconspicuous physiognomy. As part of the correction of plagiocephaly, the patients tended to relapse in the postoperative course, although there was no statistically significant difference in the frontal angle compared to that in the norm population (153.3 ± 3.9°; p = 0.06). While 6 months after surgery all patients showed a normal cranial volume development compared to healthy children of the same age, the volumes of brachycephalic patients remained below the norm (1244.2 ± 153.2 cm3; p = 0.0244). Overall, the analysis of the norm population showed a growing dispersion of measurement values with increasing age, which was observed to be more concentrated in the operated cranial morphologies. CONCLUSION: The determination of new pathology-specific morphometric parameters on the three-dimensional surface scan enables an objective quantification of physiological and pathological cranial morphologies of children. A comparison of operated children with a healthy, age-appropriate comparison group showed that preoperative and statistically significant deviations of the new measuring parameters in long-term follow-up could be normalized through surgical intervention, although this does not apply without limitations to children with coronary suture synostosis.

Trametinib Induces Neurofibroma Shrinkage and Enables Surgery.

Plexiform neurofibromas are congenital peripheral nerve sheath tumors characteristic of neurofibromatosis type 1 (NF1)-a frequent neurocutaneous disorder caused by mutations of the NF1 tumor suppressor gene. Since plexiform neurofibromas are a major cause of the burden of disease and may also progress to malignancy, many efforts have been undertaken to find a cure for these tumors. However, neither surgery nor medication has so far produced a breakthrough therapeutic success. Recently, a clinical phase I study reported significant shrinkage of plexiform neurofibromas following treatment with the MEK inhibitor selumetinib. Here, we report an 11-year-old NF1 patient with a large plexiform neurofibroma of the neck that had led to a sharp-angled kinking of the cervical spine and subsequent myelopathy. Although surgical stabilization of the cervical vertebral column was urgently recommended, the vertebral column was inaccessible due to extensive tumor growth. In this situation, treatment with the MEK inhibitor trametinib was initiated which resulted in a 22% reduction in tumor volume after 6 months of therapy and finally enabled surgery. These data show that MEK inhibitors may not lead to complete disappearance of NF1-associated plexiform neurofibromas but can be an essential step in a multimodal therapeutic approach for these tumors. The course of our patient suggests that MEK inhibitors are likely to play a significant role in providing a cure for one of the most devastating manifestations of NF1.

Conservative and operative management of iatrogenic craniocerebral disproportion-a case-based review.

INTRODUCTION: Craniocerebral disproportion (CCD) can occur as a sequela after shunting in early infancy. It can be understood as a disorder closely related to slit ventricle syndrome and chronic overdrainage syndrome. Here, we present two exemplary cases and summarize the pathophysiological, diagnostic, and therapeutic approaches to CCD. CLINICAL PRESENTATION: Two premature babies underwent shunting for posthemorrhagic hydrocephalus and presented in later childhood with recurrent episodes of symptomatic raised intracranial pressure (ICP) at 2 and 8 years of age, respectively. DIAGNOSIS AND MANAGEMENT: Both patients had unchanged ventricular size on cranial imaging and fulfilled the clinical diagnostic criteria of CCD. After confirming shunt patency, ICP monitoring was performed to diagnose intermittent intracranial hypertension. Different treatment pathways were pursued: While readjustment of a programmable shunt valve was sufficient to alleviate the raised ICP in the first case, a cranial expansion surgery was necessary in the second case. OUTCOME AND CONCLUSIONS: Both children were treated successfully after thorough assessment and careful choice of treatment approaches. This review provides detailed insight into CCD and highlights the importance of individual and critical decision-making in these complex patients.

Comparison between piezosurgery and conventional osteotomy in cranioplasty with fronto-orbital advancement.

BACKGROUND: Cranioplasty of patients with craniosynostosis requires rapid, precise and gentle osteotomy of the skull to avoid complications and benefit the healing process. The aim of this prospective clinical study was to compare two different methods of osteotomy. Piezosurgery and conventional osteotomy were compared using an oscillating saw and high speed drill while performing cranioplasties with fronto-orbital advancement. METHODS: Thirty-four children who required cranioplasty with fronto-orbital advancement were recruited consecutively. The operations were conducted using piezosurgery or a conventional surgical technique, alternately. Operative time, blood count, CRP and transfusion rate, as well as soft tissue injuries, postoperative edema, pain development and secondary bone healing were investigated. RESULTS: The average age of patients was 9.7 months. The following indications for craniosynostosis were surgically corrected: trigonocephaly (23), anterior plagiocephaly (8), brachycephaly (1), and syndromic craniosynostosis (2). Piezosurgery was utilized in 18 cases. There were no group differences with regard to the incidence of soft tissue injuries (dura, periorbita), pain, swelling, blood loss or bony integration. The duration of osteotomy was significantly longer in the piezosurgery group, leading to slightly increased blood loss, while the postoperative CRP increase was higher using the conventional method. CONCLUSIONS: The piezosurgery method is a comparatively safe surgical method for conducting osteotomy during cranioplasty. With regard to soft tissue protection and postoperative clinical course, the same procedural precautions and controls are necessary as those needed for conventional methods. The osteotomy duration is considerably longer using piezosurgery, although it is accompanied by lower initial postoperative CRP values.

Myelomeningocele - a single institute analysis of the years 2007 to 2015.

BACKGROUND: After the introduction of folate supplementation, the number of open spinal dysraphism was successfully reduced over time. In 2007, the department for pediatric neurosurgery was established in the children's hospital. Since then, newborns with myelomeningocele (MMC), the most common form of open neural tube defects (NTD), are treated here. The aim of this study is to present the concepts applied to and experiences resulting from treatment of MMC. METHODS: Records of all newborns with MMC treated surgically during the period January 2007 to August 2015 in our institution were analyzed. Children, who were previously operated in utero were excluded. The type of neural tube defect, its location, associated comorbidities, and ambulation were recorded. RESULTS: Forty-eight children (25 males, 23 females) with spinal dysraphism were included in the analysis. In nearly 90 % of the cases, the repair of the MMC was done on the day of delivery. The follow-up period ranges from 9 weeks to 8 9/12 years (loss of follow-up in 2 cases). In 19 %, the defect remained undetected during gestation and in one case, carbamazepine was taken despite pregnancy. In 36 children (75 %), we found a Chiari malformation type II (CMII) associated with myelomeningocele. 85.4 % suffered from hydrocephalus and implantation of a shunt was necessary. In cases of bladder impairment, an intermittent catheterization was the most common management (83.3 %); no bladder augmentation was required. Twelve children required orthopedic surgery. Twenty-three of 33 patients (70 %) are ambulatory w/wo orthoses and devices. The 13 children who are younger than 2 years were considered separately to assess the motor activity safely. CONCLUSIONS: Our data show that neural tube defects to this day can remain undetected despite medical care during pregnancy. The most common associated diseases with MMC are Chiari II malformations and hydrocephalus. In the seven cases of simultaneous repair of MMC with shunt implantation, no additional complications were encountered. An interdisciplinary approach was allowed in a high percentage independence and social continence.

Primary and secondary management of the Chiari II malformation in children with myelomeningocele.

OBJECT: Chiari malformation type II is almost exclusively found in patients with open spinal dysraphism. Etiology and pathophysiology are not yet completely understood, and management guidelines regarding the best follow-up and treatment of this pathological entity do not exist. In order to assess essential management aspects, literature and a series of secondary neurosurgical interventions in Chiari II patients have been reviewed. METHODS: A literature review regarding etiology, diagnostics, pathophysiology, and management of Chiari malformation type II (CMII) and a retrospective evaluation of a series (2009-2012) of secondary interventions in Chiari II patients have been performed. Inclusion criteria were ICD for myelomeningocele with or without hydrocephalus and ICD for Chiari malformation and neurosurgical OR procedure. Evaluated parameters were: patient demographics, primary management, secondary neurosurgical operations (cranio-cervical decompression, shunt revision, myelolysis) as well as specific findings pre- and postoperatively. Essential results from literature review and patients' series are compiled in order to define management recommendations. RESULTS: Fifty patients (28 f, 22 m; mean age, 7.1 years (range, 0.5-26 years)) with myelomeningocele-associated Chiari malformation type II were operated on between 2009 and 2012. Twenty-four patients had syringomyelia and scoliosis each, and 12 suffered from both. Orthopedic surgery for scoliosis or kyphosis had been performed in 13 cases. Shunt revision was performed in 38 cases, myelolysis in 17, and decompression of the foramen magnum in 14 (28 %). After a mean follow-up of 1.9 years, syringomyelia decreased from 24 to 16 cases. There was a postoperative reduction of neck pain (one third), sensorimotor (two fifths), and cranial nerve deficits (one half). CSF flow at the foramen magnum did not change visibly after surgery. Ventricular size improved in about half of the patients. Slit-like ventricles were found in nine (6 pre-surgical) and enlarged ventricles in nine (23 pre-surgical). Complication rate was 6 % (3/50) per cases, and no patient died or deteriorated neurologically after surgery. CONCLUSION: CMII-related management guidelines are not well defined, since clinical constellations and presentations are varying. Often associated findings are syringomyelia, hydrocephalus, and scoliosis, and symptomatic CMII may be triggered by more than one underlying condition. According to literature and clinical experience, management recommendations can be defined. The most important finding is that hydrocephalus is often involved in symptomatic CMII and must always be considered first in any symptomatic patient. Intrinsic brain stem dysfunctions cannot be treated surgically, and monitoring of vital functions is sometimes the only clinical means that can be offered to the patient. Knowledge of the complex background has led to improved follow-up programs for the affected children and thus also improved longtime survival.