Lars Bøgeskov

A report of nine newborns with congenital brain tumours

BackgroundAlthough rare, brain tumours represent one of the relatively larger groups of congenital neoplasias. Most studies on congenital neoplastic disease deal with several types of neoplasms and are dominated by leukaemias, retinoblastomas and systemic solid tumours. Few studies are dedicated to congenital brain tumours. We present nine newborns (four boys and five girls) who were diagnosed with congenital brain tumours during the 8-year period 1 January 1992–31 December 1999 at our institution, which covers all paediatric neuro-oncology cases for Eastern Denmark.EpidemiologyTwo of the cases were referred from Western Denmark for surgery, and were therefore excluded from the calculation of incidence. During the same period, a total of 172 children below the age of 15xa0years were diagnosed as having primary central nervous system tumours. The seven remaining congenital cases thus represent 4% of all paediatric brain tumour cases in the area (95% confidence interval 1.7–8.3%). The population of the referral area is 2.383×106, and based on the total number of living births, the incidence of congenital brain tumour was calculated to be 2.9 per 100,000 live births. The ages of the mothers were 28–33xa0years, corresponding to the present mean age of 31xa0years for Danish primipara. The gestational age varied between 35 and 42xa0weeks, and the birth weights were 3,044–4,790xa0g.Risk factorsTwo patients with p53-related glioblastoma multiforme (GBM) had relatives with p53-related neoplasms. In one case, the mother was treated for cancer of the ovary with surgery and chemotherapy 2xa0months before conception.Clinical featuresIn five of the cases, brain abnormality was suspected antenatally. The clinical features of the newborns were limited to enlarged head circumferences, associated hydrocephalus, and asymmetric skull growth.Diagnosis and treatmentThree babies were treated with complete tumour resection. In the remaining six cases, a guided or open biopsy to obtain histology was made after CT/MRI imaging. The histological diagnoses were teratoma in four cases, GBM in two cases, anaplastic astrocytoma in two cases and, finally, haemangioma capillare in one case.OutcomeFour of the patients (44%) are still alive, including two patients with totally resected combined orbital/intracranial teratomas, one patient with a totally resected haemangioma and one patient with anaplastic astrocytoma who did not receive any treatment apart from supportive care. The survival lengths of the five neonates who died varied between 1xa0day and 51xa0days.

The Usefulness of Dynamic O-(2-18F-Fluoroethyl)-l-Tyrosine PET in the Clinical Evaluation of Brain Tumors in Children and Adolescents

Experience regarding O-(2-18F-fluoroethyl)-l-tyrosine (18F-FET) PET in children and adolescents with brain tumors is limited. Methods: Sixty-nine 18F-FET PET scans of 48 children and adolescents (median age, 13 y; range, 1–18 y) were analyzed retrospectively. Twenty-six scans to assess newly diagnosed cerebral lesions, 24 scans for diagnosing tumor progression or recurrence, 8 scans for monitoring of chemotherapy effects, and 11 scans for the detection of residual tumor after resection were obtained. Maximum and mean tumor-to-brain ratios (TBRs) were determined at 20–40 min after injection, and time–activity curves of 18F-FET uptake were assigned to 3 different patterns: constant increase; peak at greater than 20–40 min after injection, followed by a plateau; and early peak (≤20 min), followed by a constant descent. The diagnostic accuracy of 18F-FET PET was assessed by receiver-operating-characteristic curve analyses using histology or clinical course as a reference. Results: In patients with newly diagnosed cerebral lesions, the highest accuracy (77%) to detect neoplastic tissue (19/26 patients) was obtained when the maximum TBR was 1.7 or greater (area under the curve, 0.80 ± 0.09; sensitivity, 79%; specificity, 71%; positive predictive value, 88%; P = 0.02). For diagnosing tumor progression or recurrence, the highest accuracy (82%) was obtained when curve patterns 2 or 3 were present (area under the curve, 0.80 ± 0.11; sensitivity, 75%; specificity, 90%; positive predictive value, 90%; P = 0.02). During chemotherapy, a decrease of TBRs was associated with a stable clinical course, and in 2 patients PET detected residual tumor after presumably complete tumor resection. Conclusion: Our findings suggest that 18F-FET PET can add valuable information for clinical decision making in pediatric brain tumor patients.

Indications for shunt insertion or III ventriculostomy in hydrocephalic children, guided by lumbar and intraventricular infusion tests

Abstract The best therapeutic management for infantile hydrocephalus is not always obvious. Traditionally, shunt insertion has been performed when CSF dynamics have been considered abnormal. However, in cases of noncommunicating hydrocephalus endoscopic III ventriculostomy (ETV) has become a well-established treatment modality, but despite clinical and radiological information clinical improvement is not obtained in all cases. A reliable preoperative investigative procedure helping to select hydrocephalic children for ETV, shunt insertion or no operation, is urgently needed. We report three cases of infantile hydrocephalus, in which our operative management was guided by the results of cerebrospinal (CSF) infusion tests. With a lumbar infusion test we assessed the CSF resorption capacity, and thus whether shunting was indicated. Comparing the results with those of an intraventricular infusion test, we assessed the presence of any structural blockage of the CSF circulation between the ventricles and the subarachnoid compartment, which would indicate a possible effect of an ETV. Performance of both a lumbar infusion test and a subsequent intraventricular infusion test in hydrocephalic children seems to provide valuable information for the decision-making on surgery.

Facial Asymmetry in Children with Unicoronal Synostosis Who Have Undergone Craniofacial Reconstruction in Infancy.

Objective Quantitatively assess 3D spatially detailed soft-tissue facial asymmetry in children who had undergone craniofacial reconstruction for Unicoronal Synostosis (UCS), and compare the facial asymmetry to control patients. It was hypothesized that there would be no significant differences in the facial asymmetry between the groups. Design Clinical, retrospective follow-up study. Methodological study. Setting Primary care center. Patients/Participants Twenty-two children with UCS were selected after review of records. Inclusion criteria: isolated UCS; surgically treated for UCS within the first 19 months of life, without secondary reconstruction; and DNA analysis for the Muenke mutation. An age- and sex-matched control group was employed. Interventions The UCS group had undergone bilateral craniotomy of the frontal bone with unilateral supraorbital rim advancement. Main outcome Measure(s) Using 3D surface scanning, a detailed map of 3D asymmetry presenting the amount of asymmetry in the sagittal, vertical, and transverse directions was calculated for six facial subregions. Results The facial asymmetry in the UCS group was significantly larger than in the control group for all regions, to the largest extent in the sagittal direction (level of significance: 5%). The regions with the most pronounced asymmetry were cheeks (mean: 5.45 mm; SD: 1.83 mm), forehead (mean: 5.00 mm; SD: 1.57 mm), and eyes (mean: 4.26 mm; SD: 1.44 mm). Conclusions Ninety percent of the UCS patients in the study had significant facial asymmetry throughout the facial area. The study demonstrates a methodology of facial asymmetry quantification well suited for soft-tissue surgical outcome evaluations and long-term follow-up studies in patients with craniofacial anomalies.