Matthias Schulz, Yasin Oezkan, Andreas Schaumann, Miriam Sieg, Anna Tietze, and Ulrich-Wilhelm Thomale
Congenital intracranial cysts account for a significant portion of intracranial lesions in the pediatric population. The efficiency of surgical treatment in a pediatric cohort with intracranial arachnoid cysts (ACs) at different locations regarding clinical symptoms and mass effect was evaluated.
A retrospective study of all children who underwent surgical treatment of an intracranial AC during an 11-year period (2007–2018) was performed. Demographics, clinical symptoms, and radiological cyst size pre- and postoperatively, as well as the reoperation rate and possible treatment complications, were analyzed.
A total of 116 intracranial cysts at 8 different anatomical locations were surgically treated in 113 children (median age 5 years and 10 months) predominantly by endoscopic technique (84%). The complication rate was 3%, and the reoperation rate was 16%. Preoperative cyst enlargement was significantly more common in infants (p < 0.0001), as was the need for reoperation (p = 0.023). After a median radiological follow-up of 26 months, > 50% reduction of cyst volume was seen in 53 cysts (46%), and 47 cysts (40%) showed a 10%–50% reduction. Acute clinical symptoms improved: nausea and vomiting, accelerated head growth, and headaches improved at 100%, 92% and 89%, respectively. However, chronic symptoms responded less favorably after a median clinical follow-up of 26 months.
The strategy of predominantly endoscopic treatment with navigation planning is efficient to alleviate clinical symptoms and to significantly reduce the mass effect of pediatric intracranial cysts at different anatomical locations. The subgroup of infants requires close pre- and postoperative monitoring.
Philine Behrens, Anna Tietze, Elisabeth Walch, Petra Bittigau, Christoph Bührer, Matthias Schulz, Annette Aigner, and Ulrich-Wilhelm Thomale
A standardized guideline for treatment of posthemorrhagic hydrocephalus in premature infants is still missing. Because an early ventriculoperitoneal shunt surgery is avoided due to low body weight and fragility of the patients, the neurosurgical treatment focuses on temporary solutions for CSF diversion as a minimally invasive approach. Neuroendoscopic lavage (NEL) was additionally introduced for early elimination of intraventricular blood components to reduce possible subsequent complications such as shunt dependency, infection, and multiloculated hydrocephalus. The authors report their first experience regarding neurodevelopmental outcome after NEL in this patient cohort.
In a single-center retrospective cohort study with 45 patients undergoing NEL, the authors measured neurocognitive development at 2 years with the Bayley Scales of Infant Development, 2nd Edition, Mental Developmental Index (BSID II MDI) and graded the ability to walk with the Gross Motor Function Classification System (GMFCS). They further recorded medication with antiepileptic drugs (AEDs) and quantified ventricular and brain volumes by using 3D MRI data sets.
Forty-four patients were alive at 2 years of age. Eight of 27 patients (30%) assessed revealed a fairly normal neurocognitive development (BSID II MDI ≥ 70), 28 of 36 patients (78%) were able to walk independently or with minimal aid (GMFCS 0–2), and 73% did not require AED treatment. Based on MR volume measurements, greater brain volume was positively correlated with BSID II MDI (rs = 0.52, 95% CI 0.08–0.79) and negatively with GMFCS (rs = −0.69, 95% CI −0.85 to −0.42). Based on Bayesian logistic regression, AED treatment, the presence of comorbidities, and also cerebellar pathology could be identified as relevant risk factors for both neurodevelopmental outcomes, increasing the odds more than 2-fold—but with limited precision in estimation.
Neuromotor outcome assessment after NEL is comparable to previously published drainage, irrigation, and fibrinolytic therapy (DRIFT) study results. A majority of NEL-treated patients showed independent mobility. Further validation of outcome measurements is warranted in an extended setup, as intended by the prospective international multicenter registry for treatment of posthemorrhagic hydrocephalus (TROPHY).
Anna Tietze, Changho Choi, Bruce Mickey, Elizabeth A. Maher, Benedicte Parm Ulhøi, Ryan Sangill, Yasmin Lassen-Ramshad, Slavka Lukacova, Leif Østergaard, and Gorm von Oettingen
Mutations in the isocitrate dehydrogenase (IDH) genes are of proven diagnostic and prognostic significance for cerebral gliomas. The objective of this study was to evaluate the clinical feasibility of using a recently described method for determining IDH mutation status by using magnetic resonance spectroscopy (MRS) to detect the presence of 2-hydroxyglutarate (2HG), the metabolic product of the mutant IDH enzyme.
By extending imaging time by 6 minutes, the authors were able to include a point-resolved spectroscopy (PRESS) MRS sequence in their routine glioma imaging protocol. In 30 of 35 patients for whom this revised protocol was used the lesions were subsequently diagnosed histologically as gliomas. Of the remaining 5 patients, 1 had a gangliocytoma, 1 had a primary CNS lymphoma, and 3 had nonneoplastic lesions. Immunohistochemistry and/or polymerase chain reaction were used to detect the presence of IDH mutations in the glioma tissue resected.
In vivo MRS for 2HG correctly identified the IDH mutational status in 88.6% of patients. The sensitivity and specificity was 89.5% and 81.3%, respectively, when using 2 mM 2HG as threshold to discriminate IDH-mutated from wildtype tumors. Two glioblastomas that had elevated 2HG levels did not have detectable IDH mutations, and in 2 IDH-mutated gliomas 2HG was not reliably detectable.
The noninvasive determination of the IDH mutation status of a presumed glioma by means of MRS may be incorporated into a routine diagnostic imaging protocol and can be used to obtain additional information for patient care.