Cerebellar mutism syndrome in pediatric head trauma with cerebellar injury.

PURPOSE: Cerebellar mutism syndrome (CMS) after cerebellar injury in pediatric head trauma is a poorly recognized condition that is not properly diagnosed or treated in our daily practice. We aimed to clinically identify this syndrome after isolated posttraumatic cerebellar injury and to propose pathophysiological explanation. METHODS: We retrospectively analyzed 8 consecutive children presenting with isolated cerebellar injury over 16 years. Clinical presentation, radiological type and localization of injury, clinical initial CMS symptoms, and long-term neurocognitive outcome were reviewed. RESULTS: Out of 8 patients presenting with isolated traumatic cerebellar injury, we diagnosed 2 cases with initial clinical symptoms of CMS. Both patients had an injury damaging median structures of the posterior fossa, especially the fourth ventricle and dentate nuclei. Initial symptoms lasted more than 1 month for one patient, who still presented concentration difficulties almost 1 year after the head injury. CONCLUSION: CMS after traumatic cerebellar injury does exist, even if it seems to be a very rare entity. It has to be better detected and studied in order to enrich pathophysiological knowledge about CMS of all etiologies and to bring our concerned patients the suitable follow-up and rehabilitative care that they could benefit from.

Anorexia: an early sign of fourth ventricle astrocytoma in children.

INTRODUCTION: Paediatric low-grade astrocytomas of the fourth ventricle are rare tumours, generally revealed by hydrocephalus. However, some patients present with a history of severe anorexia. It might be a harbinger, which if recognized, could lead to earlier diagnosis. We decided to examine our database in order to evaluate the incidence and signification of anorexia in this context. METHODS: Retrospective monocentric study of cases of low-grade astrocytomas of the fourth ventricle operated between 1991 and 2012 in our paediatric neurosurgery department. We particularly observed the clinical presentation and long-term clinical, oncological and radiological evolution. Non-parametrical tests were used (Mann-Whitney, Fisher). RESULTS: We reviewed 34 cases, 31 pilocytic astrocytomas and 3 diffuse astrocytomas, 16 boys and 18 girls, (M/F ratio 0.89). Mean age at diagnosis was 8 years old. Seven presented with notable anorexia, the average BMI in this group was ≤2 standard deviation (SD); with clinical signs evolving for 11.5 months. Twenty-seven children had no anorexia; average BMI in this group was +1 SD, with clinical evolution for 6 months on an average of p < 0.05. We found no significant difference regarding hydrocephalus or tumour location. In all children with anorexia, body mass index improved markedly in the postoperative follow-up, which lasted, on average, for 6 years. CONCLUSION: Anorexia with stunted body weight curve is a non-exceptional presentation in children with low-grade astrocytomas of the fourth ventricle. Unexplained or atypical anorexia with negative etiologic assessment should prompt cerebral imaging. Clinical improvement after surgical resection, could suggest a possible interaction between tumour tissue and appetite-suppressing peptide secretion.

Adult outcome of pediatric hydrocephalus.

INTRODUCTION: The outlook of pediatric hydrocephalus has spectacularly improved over the past decades; however, the adult outcome is still poorly documented. Determining the healthcare profile of these patients is important in order to organize the management of this growing population. We decided to review our pediatric hydrocephalus database for pediatric patients treated for hydrocephalus and followed up into adulthood. METHODS: Our institution has a virtual monopoly for pediatric hydrocephalus, serving a four-million-plus population; the transition to adult care is also managed in the same institution. We retrospectively reviewed patients younger than 18 treated for hydrocephalus since 1980 and followed up beyond the age of 20. RESULTS: We reviewed 456 patients, with a mean initial age of 55.6 months, and followed up for a mean of 24.2 years. In 81 patients (17.8%), the last shunt operation occurred after 20 years; 22 of these (4.9% of the total) having never been revised earlier. Sixteen patients (3.5%) could be weaned of their shunt. Thirteen patients died in adult age, 5 of these dying of shunt-related causes. The most prominent sequels were motor (46.5%) and cognitive (47.6%); only 82 patients (18.0%) had no sequel at all. Intelligence quotient values were ≥80 in 54.5% of tested patients; however, schooling was normal in only 41.4%, and only 33.7% was employed in the competitive labor market. CONCLUSION: Adults treated for hydrocephalus in childhood require a life-long follow-up. Late mortality is low but not null, morbidity is high, and many patients require shunt surgery during adulthood. The transition from child to adult neurosurgery needs to be organized for these vulnerable patients.

Morbidity and tumor-related mortality among adult survivors of pediatric brain tumors: a review.

INTRODUCTION: The health status of adult survivors of pediatric brain tumors is poorly documented. Documenting their health risks regarding tumor recurrence or new tumor, endocrine, and cognitive sequels, as well as their social outcome, would help to define their medical needs when they become adults. Identifying risk factors of late morbidity could help to improve treatment protocols in order to reduce the burden of sequels. PURPOSE: We decided to review retrospectively our pediatric oncology database, in order to study the oncological and clinical outcome of adult patients treated for brain tumor during childhood. MATERIALS AND METHODS: We selected patients treated under the age of 18, and followed clinically after the age of 20. RESULTS: We studied 207 patients, aged 20.2-45 years at last control. Forty eight (23%) presented with late progression of the initial tumor, and 29 (14%) developed new tumors requiring surgery; 7 died of tumor progression. The main sequels were endocrine in 88 patients (44%), and cognitive in 86 (43%); only 36 patients (18%) had no sequel at all. The Karnofsky score was ≥ 80 (independent) in 73%; however, only 48% of evaluable patients were normally employed. Among the variables related to initial treatment, reoperation was the most significant factor influencing negatively outcome; however, young age at irradiation was the only significant factor predicting poor employment status. CONCLUSIONS: Adult patients treated for brain tumor in childhood are at significant risk of tumor progression, and many patients require prolonged oncological follow-up. Complex disabilities require the organization of efficient child-to-adult transition for these vulnerable patients.

Radiation-induced tumors in children irradiated for brain tumor: a longitudinal study.

BACKGROUND: Radiation-induced tumors (RIT) are increasingly recognized as delayed complications of brain irradiation during childhood. However, the true incidence is not established, their biology is poorly understood, and few guidelines exist regarding the long-term follow-up of irradiated children. METHODS: We studied retrospectively patients irradiated for brain tumor under 18 years and followed in our institution since 1970. RIT were defined as new masses, different from the original tumor, occurring after delay in irradiated areas, and not related to phacomatosis. RESULTS: Among 552 irradiated patients, 42 (7.6%) developed one or more RIT, 26 months to 29 years after irradiation (mean 12.8 years). The cumulated incidence was 2.0% at 5 years and 8.9% at 10 years. Of the patients, 73.8% were adult at the time of diagnosis of RIT, and 75% were diagnosed within 18.1 years after irradiation. We identified 60 cavernomas, 26 meningiomas, 2 malignant gliomas, 1 meningosarcoma, and 6 thyroid tumors. Compared with meningiomas, cavernomas appeared earlier, in children irradiated at an older age, and with a male predominance. Although RIT were correlated with higher irradiation doses, 80.9% of these occurred at some distance from the maximum irradiation field. Twenty-five lesions were operated in 20 patients; three patients died because of progression of the RIT. CONCLUSION: A significant number of patients undergoing irradiation for brain tumor during childhood develop a RIT, often during adulthood. Our data suggest that radiation-induced cavernomas result from angiogenetic processes rather than true tumorigenesis. Protracted follow-up with MRI is warranted in children irradiated for brain tumor.

Subdural hematoma in infants: can it occur spontaneously? Data from a prospective series and critical review of the literature.

BACKGROUND: Subdural hematomas (SDH) in infants often result from nonaccidental head injury (NAHI), which is diagnosed based on the absence of history of trauma and the presence of associated lesions. When these are lacking, the possibility of spontaneous SDH in infant (SSDHI) is raised, but this entity is hotly debated; in particular, the lack of positive diagnostic criteria has hampered its recognition. The role of arachnoidomegaly, idiopathic macrocephaly, and dehydration in the pathogenesis of SSDHI is also much discussed. PURPOSE: We decided to analyze apparent cases of SSDHI from our prospective databank. MATERIALS AND METHODS: We selected cases of SDH in infants without systemic disease, history of trauma, and suspicion of NAHI. All cases had fundoscopy and were evaluated for possible NAHI. Head growth curves were reconstructed in order to differentiate idiopathic from symptomatic macrocrania. RESULTS: Sixteen patients, 14 males and two females, were diagnosed with SSDHI. Twelve patients had idiopathic macrocrania, seven of these being previously diagnosed with arachnoidomegaly on imaging. Five had risk factors for dehydration, including two with severe enteritis. Two patients had mild or moderate retinal hemorrhage, considered not indicative of NAHI. Thirteen patients underwent cerebrospinal fluid drainage. The outcome was favorable in almost all cases; one child has sequels, which were attributable to obstetrical difficulties. CONCLUSION: SSDHI exists but is rare and cannot be diagnosed unless NAHI has been questioned thoroughly. The absence of traumatic features is not sufficient, and positive elements like macrocrania, arachnoidomegaly, or severe dehydration are necessary for the diagnosis of SSDHI.

Confessed abuse versus witnessed accidents in infants: comparison of clinical, radiological, and ophthalmological data in corroborated cases.

BACKGROUND: The diagnosis of inflicted head injury (IHI) or accidental trauma (AT) in infants is based on clinical, radiological, and/or ophthalmological findings such as subdural hematoma (SDH), encephalopathy, retinal hemorrhage (RH), and signs of impact. As a consequence, the diagnostic value of these criteria is difficult to assess because of a circularity bias. PURPOSE: In order to assess the predictive value of these diagnostic criteria avoiding circularity, we studied prospectively corroborated cases of head injuries in infants, comparing IHI with AT. MATERIAL AND METHODS: We selected 45 cases of confessed IHI and 39 cases of AT having occurred in public places. Patients were systematically evaluated clinically, with computed tomography scanner and ophthalmological evaluation by a trained ophthalmologist. RH was rated as absent, mild, and severe, according to the depth and extent of the RH. RESULTS: Brain ischemia was found in 26.7% of IHI; the most prominent elements in favor of IHI were SDH, severe RH, and absence of signs of impact; the predictive diagnostic values of these three features were 0.685, 0.961, and 0.830, respectively; however, only severe RH in the absence of ocular impact was specific of IHI. When all three features were combined, the specificity was 100%, but the sensitivity was only 24.4%. CONCLUSION: Our study confirms the high diagnostic value of RH, SDH, and signs of impact for the differential diagnosis between AT and IHI. The evaluation of head injuries in infants requires a high level of awareness and thorough and systematic examination by a trained multidisciplinary team.

Craniopharyngioma and hypothalamic obesity in children.

BACKGROUND AND PURPOSE: Obesity is a major concern in children treated for craniopharyngioma and is caused by hypothalamic damage. The role of aggressive surgical removal has been questioned, leading some authors to recommend a minimalist approach. In order to test this hypothesis, we decided to study obesity in craniopharyngioma and the factors related to it. MATERIALS AND METHODS: We reviewed retrospectively our series of pediatric craniopharyngiomas operated since 1981. The body-mass index (BMI) was calculated for each patient pre- and at several intervals postoperatively and expressed as standard deviations (SD) adjusted for age and gender. RESULTS: We operated on 45 cases, which were followed up for a mean duration of 11.0 years. Initial resection was total in 25 cases (55.6%). No patient died because of surgery or tumor progression; two died with delay presumably because of endocrine failure. At last control, 28 patients (62%) had obesity (BMI over +2SD). Hypothalamic involvement was significantly correlated with preoperative and postoperative BMI. Subtotal tumor resection was significantly associated with obesity at last control. Reoperation for tumor recurrence was associated with a significantly higher BMI. CONCLUSIONS: Our results suggest that obesity results from hypothalamic lesions caused by the tumor rather than by surgery. The postoperative weight gain appears to result from the continued impact of preoperative hypothalamic damage. The high rate of tumor recurrence in children, with the risk of additional damage to the hypothalamus, incites us to recommend total resection whenever it appears safe during initial surgery.

Shunt revision for asymptomatic failure: surgical and clinical results.

OBJECTIVE: Shunt obstruction represents a permanent threat for patients with shunts, and its prevention and treatment are important parts of neurosurgeons' duties. Although there is little discussion regarding the need to reoperate for treatment of symptomatic shunt failure (SSF), the need to reoperate for treatment of asymptomatic shunt failure (ASF) is debated, as are the guidelines for the follow-up monitoring of patients with shunts. The goal of this study was to assess the effects of systematic follow-up monitoring and shunt revision for ASF; we reviewed our database to compare the results of shunt revision for ASF versus SSF. METHODS: We defined ASF as shunt failure diagnosed for an asymptomatic patient during a systematic consultation. In our institution, children who receive shunts for treatment of hydrocephalus are systematically monitored in an outpatient clinic, with clinical examinations and plain x-rays. Among 1,564 children with shunts, who were monitored for a mean of 10.7 years, 1106 (70.7%) required at least one shunt revision. The indication for the first revision was SSF in 609 cases and ASF in 305 cases; the indication was not specified in 192 cases. We studied the surgical outcomes after the first shunt revision and compared the results for SSF and ASF. RESULTS: After the first revision, shunt infections and subsequent shunt failure were significantly less frequent in the ASF group, compared with the SSF group. The interval between the first shunt revision and subsequent shunt failure was significantly longer in the ASF group. CONCLUSION: Our data support the practice of systematic follow-up monitoring for patients with shunts, for the early diagnosis and systematic treatment of ASF.

Hemispheric cerebral gliomas in children with NF1: arguments for a long-term follow-up.

BACKGROUND: Neurofibromatosis type 1, or peripheral neurofibromatosis, is the most form of common phakomatosis. In some instances, it can be associated with tumors of the central nervous system. CASE REPORT: We describe four cases of hemispheric cerebral glioma in children with NF1. Two of these tumors were pilocytic astrocytomas and two, ependymomas. We reviewed the literature on hemispheric cerebral gliomas in NF1 patients in an attempt to find characteristic features of and some explanations for these lesions. CONCLUSION: We conclude that surgical resection of these tumors should be proposed whenever possible, with due consideration for their progressive nature and the uncertainties about malignancy. We advocate yearly clinical and neuroradiological follow-up over a long period in NF1 children, since they may develop additional CNS tumors during their lifetime.