Pediatric craniopharyngiomas: magnetic resonance imaging assessment for hypothalamus-pituitary axis dysfunction and outcome prediction.

BACKGROUND: In adamantinomatous craniopharyngiomas, tumor topographical categories, cystic component volume, and magnetic resonance signal intensity may impact prognosis. OBJECTIVE: To identify magnetic resonance imaging (MRI) variables associated with pituitary-hypothalamic axis dysfunction and predictive of outcome in children with cystic adamantinomatous craniopharyngiomas. MATERIALS AND METHODS: We evaluated 40 preoperative MRIs of adamantinomatous craniopharyngiomas to classify tumor topography, volume, and signal intensity of the cystic components and peritumoral edema. Volumes and normalized signal intensity minimum values were extracted from coronal T2-weighted images (nT2min). Radiological variables were compared to pituitary-hypothalamic axis dysfunction-related clinical data and surgical outcomes. RESULTS: Adamantinomatous craniopharyngiomas were categorized into five topographic classes (12 patients, sellar-suprasellar; seven patients, pseudo-intraventricular; six patients, strict intraventricular; 14 patients, secondary intraventricular; one patient, not strict intraventricular). All cases exhibited a predominant (30 patients, 80%) or total (10 patients, 20%) cystic tumor component and displayed low nT2min percentage values compared to cerebrospinal fluid (42.3% [interquartile range 28.4-54.6%]). Significant associations between tumor topographic classes and pituitary dysfunction (P<0.001), and between peritumoral edema and hypothalamic dysfunction (P<0.001) were found. Considering extent of surgical removal and tumor relapse, volume of the cystic tumor component displayed a positive correlation (P=0.002; r=0.48; P=0.02; r=0.36), while nT2min intensity values exhibited a negative correlation (P=0.01; r= - 0.40; P=0.028; r= - 0.34). CONCLUSION: Severe hypothalamic-pituitary axis dysfunction is associated with tumors along the pituitary stalk and peritumoral edema. Tumor invasion of the third ventricle, tight adherence to the hypothalamus, larger volumes, and lower nT2min intensity of the tumor cystic component are independent predictors of extent of adamantinomatous craniopharyngioma excision and recurrence.

Effects of osteopathic approach in infants with deformational plagiocephaly: an outcome research study.

BACKGROUND: The aim of the present study is to investigate the effects of the osteopathic approach in infants with deformational plagiocephaly at 12-month follow up and to evaluate parental satisfaction and safety of the treatments. METHODS: The authors reviewed charts of infants with a medical diagnosis of deformational plagiocephaly consecutively seen in a private paediatric outpatient in Rome, Italy, between December 2016 and February 2018. After applying the exclusion criteria, infants under 6 months of corrected age, with diagnosis of deformational plagiocephaly and assessed with a plagiocephalometry were included. RESULTS: 37 infants (mean age 16,6±5,7 weeks; 19 females) were eligible for this study, generating 35 unilateral deformational plagiocephaly (21 right-sided) and 18 brachycephaly (16 infants had both). After an average of 6.5±2.8 osteopathic manipulative treatments plus postural advice, a significant reduction of all indices of the plagiocephalometry assessments was reported at 12-month follow up. Oblique Diameter Difference Index decreased from 109 ± 4.5 to 103.7 ± 1.9 (Mean diff - 5.3 CI 95% {-5.83; -3.41}, p<0.001); Cranial Proportional Index decreased from 94.9 ± 3.8 to 85.6 ± 2.4 (mean diff -9.3 CI 95% {-11.39; -7.37}, p<0.001); Ear Deviation Index decreased from 7.7 ± 3.3 to 3 ± 1.6 (mean diff -4.7 CI 95% {-6.1; -2.84}, p <0.001). CONCLUSIONS: Osteopathic approach could be considered an interesting option in the treatment of infants with deformational plagiocephaly. Parents were overall satisfied with this approach. No adverse events or reactions were reported in this study. Multicentre randomized controlled trials are recommended.

Vestibular evoked myogenic potentials in children affected by myelomeningocele.

PURPOSE: The aim of the present study is to establish if the vestibular evoked myogenic potentials (VEMPs) could be used as a clinical test for the evaluation of vestibular function in children affected by myelomeningocele (MMC). MATERIALS AND METHODS: Fifteen children, aged between 3 and 17 years, who had been affected by MMC were investigated. Data obtained from these children were compared with normal data from healthy children of the same age. Electromyographic activity of sternocleidomastoid muscle was recorded, while children were laid supine and asked to raise their head off the bed in order to activate their neck flexors bilaterally. The saccular receptors were acoustically stimulated with a logon of 500 Hz at an intensity of 130 dB peSPL presented monaurally through earphones. In each recording, we analyzed latencies and amplitudes of the p13-and n23 waves and the amplitude ratio between the two ears. RESULTS: VEMPs were detected to be normal in 13 patients. In particular, the mean p13 and mean n23 latencies were 15.7 (±1.4) and 21.7 (±1.1) ms, respectively; the mean amplitude value was 84.7 (±36.6), while the mean amplitude ratio was 17.4 (±12). A comparison of latencies and amplitude ratios between the children and healthy control group did not reveal any significant difference. On the contrary, a comparison of amplitude values between the two groups showed significant differences. CONCLUSION: In conclusion, vestibulocollic reflex is normal in patients affected by MMC, and VEMPs could represent a valid and noninvasive technique eligible to investigate the vestibular functions in these children.

Preoperative and postoperative neurological, neuropsychological and behavioral impairment in children with posterior cranial fossa astrocytomas and medulloblastomas: the role of the tumor and the impact of the surgical treatment.

INTRODUCTION: The aim of the present study was to prospectively investigate if a correlation might exist between preoperative and postoperative neurological conditions, neuroradiological/intraoperative findings and results of a complete neuropsychological evaluation in children with posterior fossa medulloblastomas and astrocytomas. MATERIALS AND METHODS: Of the 65 children admitted at the Pediatric Neurosurgery of the UCSC of Rome between January 2005 and October 2009, 41 were selected; the only two exclusion criteria were represented by age under 24 months and severe neurological conditions, seen that in both cases it would not have been a possible reliable evaluation. All children underwent a preoperative and immediate postoperative complete MR study. Hydrocephalus was graded on the Evans score; brainstem infiltration was defined on intraoperative findings. Neuropsychological assessment consisted of a battery of tests tailored on the patient's age, cognitive level, and level of cooperation. Post operative neuropsychological evaluation was performed at a mean time of 2.5 min (2 mos, max 4.5 mos) from the operation, before any eventually needed adjuvant treatment (i.e., chemotherapy, radiotherapy). RESULTS: Concerning neurological status, we found a statistically significant relation between the presence of oculomotor impairment and both verbal fluency deficits (p = 0.044) and imagery disorders (p = 0.03); also, the presence of ataxia/dysmetria was significantly correlated to attention dysfunction (p = 0.01) and, more tightly, to planning dysfunction (p = 0.006). For neuroradiological/intraoperative features, Intelligence Quotient (IQ) impairment was significantly correlated to the intraoperative evidence of tumor infiltration of the brainstem (p = 0.003), a severe hydrocephalus at diagnosis (p = 0.001) and the histological diagnosis of medulloblastoma (MB) (p = 0.002). For selective skills, a significant correlation was found between linguistic processing deficits and the evidence of dentate nuclei infiltration (blindly defined on MR); procedural memory defects and imagery disorders related to the severity of the hydrocephalus (p = 0.02), infiltration of the brain stem (p = 0.01) and a histological diagnosis of MB (p = 0.01). After surgery no patient showed a worsening of his/her cognitive profile; the relationships between clinical, intraoperative, and radiological findings were substantially confirmed. DISCUSSION: Our results support the hypothesis that when present, neuropsychological impairment is already present at diagnosis and that the most statistically significant factors, which might be related with cognitive deficits in the preoperative as well as in the postoperative period, are tumor infiltration of the brainstem, the severity of hydrocephalus, and a histological diagnosis of MB.