A multimethod assessment of psychosocial functioning and late effects in survivors of childhood cancer and hematopoietic cell transplant

Previous research in childhood cancer and hematopoietic cell transplant (HCT) survivorship has relied on the use of standardized questionnaires that assess symptoms of psychological functioning but do not sufficiently capture the cancer survivorship experience. Study aims are to quantitatively and qualitatively assess the psychosocial functioning of pediatric cancer and HCT survivors seen in a multidisciplinary survivorship clinic, determine survivorship concerns, and assess potential demographic and medical correlates of psychosocial outcomes. Data were collected using a retrospective chart review of a parent-report questionnaire of the child's psychological functioning, responses to a semistructured interview that qualitatively assessed adjustment to life after treatment, and documented medical late effects. Results indicated the majority of survivors had healthy psychological adjustment based upon a parent-report questionnaire. However, nearly 72% of survivors reported 1 or more survivorship concerns during the interview, with the primary concerns being current and future health or physical functioning, including the possibility of cancer recurrence. A content analysis of the interview responses indicated HCT survivors had more school or cognitive functioning concerns compared with survivors who did not have an HCT. Further research should use survivorship-specific measures to better identify survivors at risk and determine the impact of late effects on their quality of life.     

Magnetic resonance imaging of pediatric posterior fossa tumors.

MR detected abnormality in all 115 pediatric patients who subsequently had pathologically proven posterior fossa tumors. In 114, the initial magnetic resonance (MR) diagnosis was that of brain tumor. In 1, with less than 1-cm2 area of gadolinium enhancement, the significance of the initial finding was uncertain. Common posterior fossa tumor subgroups (brainstem gliomas, cerebellar astrocytomas, primitive neuroectodermal tumors, and ependymomas) have relatively consistent presentations on imaging studies. However, less common tumors mimic the MR appearance of more common ones, while common tumors may also have atypical appearances.     

Pediatric cerebellar cystic oligodendroglioma: case report and literature review

Oligodendrogliomas rarely occur in the posterior fossa of childhood and constitute approximately 1% of pediatric brain tumors. Only six pediatric posterior fossa oligodendroglioma cases have been reported to date and none of them were cystic. The authors present a seven-year-old girl with cystic, cerebellar midline localized tumor. A standard suboccipital craniectomy was performed and the tumor was histologically confirmed as oligodendroglioma. After operation the patient underwent radiation therapy and at one the-year follow-up, no recurrence of the tumor was observed.     

Psychosocial functioning in parents of adult survivors of childhood cancer

BACKGROUND: Although significant progress has been made in identifying long-term sequelae for adult survivors of childhood cancer, comparatively little attention has been paid to the functioning of their parents. In a previous study, we observed that a majority of adult survivors are accompanied to clinic visits by at least 1 parent, suggesting ongoing concern for their children's health. In the current study, we explore psychologic stressors that characterize this population and might account for this finding. PROCEDURE: Responses to measures of psychosocial functioning (ie, Brief Symptom Inventory, Impact on Family Scale, Impact of Events Scale, Parent Experience of Child Illness Scale) were compared between 27 parents of adult survivors (mean age=25.6 y) of pediatric cancer and 28 parents of current pediatric cancer patients (mean age=10.2 y) on, or within 1 year of, active treatment. RESULTS: Compared with parents of pediatric cancer patients on treatment, parents of adult survivors demonstrated few significant differences in overall psychologic functioning, posttraumatic stress symptoms, and adjustment to the disease experience. Indeed, the 2 groups differed only in their report of objective and family burden (eg, financial cost, time off from work, less time with family members), and in their levels of anger associated with the illness experience. CONCLUSIONS: Results suggest that parents who continue to accompany their adult child to clinic may remain psychologically vulnerable many years after the end of treatment, and that the impact of having a child with a life-threatening illness may not diminish even years into the child's survivorship.     

Hématome extradural de la fosse postérieure : à propos de 4 observations pédiatriques

Extradural hematoma of the posterior fossa is an uncommon complication of pediatric head injuries. Its prognosis has radically improved due to computed tomography. The authors report four cases of traumatic extradural hematoma of the posterior fossa concerning 18-months to 16-years old girls. The main clinical symptoms were those suggesting intracranial hypertension. Computed tomography, performed in all cases, leaded to the diagnosis. In three cases, there was occipital fracture, and the last case was, associated supratentorial lesions. Surgical evacuation of hematoma was performed in all cases, within less than six days. Good outcome was recorded among all children.     

Posterior fossa ependymoma with intense but transient disseminated enhancement but not metastasis

We report a pediatric patient with a posterior fossa ependymoma in whom the second of two magnetic resonance imaging (MRI) scans obtained preoperatively within a 10-day span showed intense but transient disseminated enhancement. However, following posterior fossa craniotomy for near total tumor resection, neither three MRI exams nor two craniospinal fluid samplings revealed evidence of disseminated disease. Concerned that the one MRI with disseminated enhancement indicated an elevated risk of leptomeningeal metastasis, we initiated craniospinal irradiation. However, we terminated this treatment after realizing that the bulk of the clinical evidence refuted the diagnosis of metastatic disease.     

The effect of cardiorespiratory fitness and physical activity levels on cognitive functions in survivors of childhood acute lymphoblastic leukemia

Abstract

Introduction. Most childhood acute lymphoblastic leukemia (ALL) survivors develop chronic treatment-related adverse effects several years after the end of the treatment. Regular physical activity and a good cardiorespiratory fitness can decrease the risks of neurological disturbances and increase cognitive function scores. The aim of this study was to examine the effect of good cardiorespiratory fitness and physical activity levels on cognitive functions.

Methods. We enrolled 219 survivors of childhood ALL. The participants underwent a cardiopulmonary exercise test, neuropsychological tests of executive functions (i.e. verbal fluency, cognitive flexibility, working memory, processing speed) and completed a physical activity questionnaire. We calculated the odds ratio to obtain the preventive fraction of physical activity and cardiorespiratory fitness levels on cognitive functions.

Results. The cohort is 52% male and 48% female. A total of 182 survivors (83%) have a cardiorespiratory fitness below their predicted (<100%). Our analyses show that there is an association between good cardiorespiratory fitness and processing speed (preventive fraction of 70% for dominant hand (p?<?0.01) and 65% for non-dominant hand (p?<?0.01)) and with cognitive flexibility identified as the category switching measure of the D-KEFS verbal fluency (preventive fraction of 61%; p?<?0.05).

Conclusion. Good cardiorespiratory fitness and good levels of physical activity were associated to a preventive fraction for most cognitive function parameters measured. Good cardiorespiratory fitness levels were significantly associated with a lower prevalence of deficits in processing speed (i.e., dominant hand and non-dominant hand) and in cognitive flexibility (i.e., category switching) in childhood acute lymphoblastic leukemia survivors.     

Fatal brainstem injury following proton radiation in a patient with medulloblastoma and a germline variant in RNF213

Brainstem injury occurs secondary to radiation to the posterior fossa in up to 2% of pediatric patients. It may occur after months to years after treatment. It has been associated with age less than 5 years and with comorbid conditions such as cerebrovascular disease, diabetes mellitus, and hypertension. Radiation necrosis is often symptomatic and can be fatal. A pathogenic variant in RNF213 was found in a patient who suffered fatal radiation necrosis. This mutation has been associated with moyamoya disease and may predispose to radiation necrosis.     

Cardiorespiratory fitness predicts clustered cardiometabolic risk in 10–11.9-year-olds

The aim of this study was to investigate levels of clustered cardiometabolic risk and the odds of being ‘at risk’ according to cardiorespiratory fitness status in children. Data from 88 10–11.9-year-old children (mean age 11.05?±?0.51 years), who participated in either the REACH Year 6 or the Benefits of Fitness Circuits for Primary School Populations studies were combined. Waist circumference, systolic blood pressure, diastolic blood pressure, glucose, triglycerides, high-density lipoprotein cholesterol, adiponectin and C-reactive protein were assessed and used to estimate clustered cardiometabolic risk. Participants were classified as ‘fit’ or ‘unfit’ using recently published definitions (46.6 and 41.9 mL/kg/min for boys and girls, respectively), and continuous clustered risk scores between fitness groups were assessed. Participants were subsequently assigned to a ‘normal’ or ‘high’ clustered cardiometabolic risk group based on risk scores, and logistic regression analysis assessed the odds of belonging to the increased cardiometabolic risk group according to fitness. The unfit group exhibited significantly higher clustered cardiometabolic risk scores (p?<?0.001) than the fit group. A clear association between fitness group and being at increased cardiometabolic risk (B?=?2.509, p?=?0.001) was also identified, and participants classed as being unfit were found to have odds of being classified as ‘at risk’ of 12.30 (95 % CI?=?2.64–57.33). Conclusion Assessing cardiorespiratory fitness is a valid method of identifying children most at risk of cardiometabolic pathologies. The ROC thresholds could be used to identify populations of children most at risk and may therefore be used to effectively target a cardiometabolic risk-reducing public health intervention.     

Impact of attention on social functioning in pediatric medulloblastoma survivors

Background: Parent-reported attention problems have been associated with social functioning in a broad sample of pediatric cancer survivors. Objective: The present study focused on a more homogeneous sample (pediatric medulloblastoma survivors), with the novel inclusion of self-reported attention ratings. Participants/Methods: Thirty-three pediatric medulloblastoma survivors, ages 7–18 years, completed a brief IQ measure and self-report of attentional and social functioning. Parents rated patients' attentional and social functioning. Results: Mean attention ratings were average based on both parent- and self-report, though parent ratings were significantly discrepant from normative means. No significant demographic or treatment-related predictors of self-reported attention problems were identified, whereas female gender was associated with greater parent-reported attention problems. Canonical correlation analysis revealed a significant association between parent-reported attention difficulties and social functioning in pediatric medulloblastoma survivors, but there was no association between self-reported attention problems and measures of social functioning. Conclusions: Consistent with existing literature in broader samples of pediatric cancer survivors, the present study further affirms attention deficits as an underlying contributor to social deficits in pediatric medulloblastoma survivors while also finding little relationship between self-reports of attention and social performance. Notably, present findings provide additional support suggesting that attention functioning is a more significant contributor to social outcomes for pediatric medulloblastoma survivors than the level of cognitive ability.     

Posterior fossa craniotomy: an alternative to craniectomy.

We describe a simple midline approach to posterior fossa lesions in children that involves removal and replacement of a bone flap. This approach offers several advantages over conventional suboccipital craniectomy. Elevation of the bone flap is facilitated by the relatively small size of the midline bony keel in children. This method is simple, safe and expeditious, and restores normal anatomical planes and improved protection to the contents of the posterior fossa. While this approach has been described previously for individual cases, the authors employ craniotomy as the standard method of access to the posterior fossa in pediatric patients, and suggest a straightforward technique for removing and replacing the bone flap.     

Transient eye closure after posterior fossa tumor surgery in children.

Transient cerebellar eye closure following posterior fossa tumor surgery in children has recently been observed. This phenomenon is characterized by a transient inability to open the eyes postoperatively with complete resolution within 4-6 days. Presented are four cases of transient cerebellar eye closure after posterior fossa tumor surgery in children. Possible mechanisms are reviewed with regard to historical works of cerebellar stimulation in man, and anatomical structures and pathways which might be involved.     

Posterior fossa problems in hydrocephalic children treated with shunts

Fourteen hydrocephalic children with functioning lateral ventricular shunts developed posterior fossa problems: posterior fossa cyst (7 patients); isolated fourth ventricle (6 patients), and concomitant dilatation of third and fourth ventricles (1 patient). Posterior fossa symptoms were present in only 2 children; 10 had nonspecific symptoms and 2 were asymptomatic. Computed tomography was the most useful investigation for diagnosis and management. Decompression of progressing posterior fossa lesions was attempted in 5 patients: cystoperitoneal shunt (3), marsupialization (1), and aspiration (1). All 5 patients had initial symptomatic relief but symptoms recurred subsequently; 2 eventually died of severe ventriculitis. The remaining 9 did not require posterior fossa surgery. Offprint requests to: P. K. H. Tam     

Photophobia, epiphora, and torticollis: a masquerade syndrome

Three young children presented with photophobia, epiphora, and torticollis as the initial manifestation of a posterior fossa tumor. In each case there was a delay in treatment due to the presumptive diagnosis of a local ocular inflammatory condition. We recommend that children with unexplained photophobia, epiphora, and torticollis undergo an imaging technique to evaluate the posterior fossa.     

Comparison of cardiorespiratory fitness and body mass index between rural and urban youth: Findings from the East of England Healthy Hearts Study

Background: The aim of the present study was to determine if there were differences in cardiorespiratory fitness (CRF) and body mass index (BMI) in rural versus urban youth. Methods: We measured BMI in 6628 10.0–15.9‐year‐olds and classified them as normal weight, overweight or obese. CRF was assessed with a 20‐m shuttle‐run test and subjects were classified as unfit, fit or highly fit. Rural or urban dwelling was calculated from postcodes. Results: There were no differences in frequency of BMI categories or CRF between rural and urban children (<13 years) or adolescents (>13 years). Rural adolescents were less likely to be fit (OR = 0.80; 95%CI 0.67–0.96) or highly fit (OR = 0.69; 95%CI 0.55–0.86) independent of their age, sex, race or level of deprivation. Conclusions: Whilst there were no differences evident in children <13 years, rural adolescents were significantly less likely to meet criterion‐based cut‐off points for CRF. Research to elucidate why such differences exist is warranted.     

Atypical teratoid/rhabdoid tumor of the central nervous system in children: an atypical series and review

Primary atypical teratoid/rhabdoid tumor (AT/RhT) of the central nervous system is a recently described, highly malignant neoplasm in infants and young children. This tumor is an unusual combination of mixed cellular elements, similar but not typical of teratomas, and rhabdoid cells. This tumor is most common in the posterior fossa in children less than 2 years, and is radiologically similar to medulloblastoma. No pathognomonic imaging features are present. The two tumors can be separated on histologic, molecular, and cytogenetic grounds. Separation of these two tumor types is crucial because the prognosis for AT/RhT is grim even with current multimodality treatment. We present four consecutive cases of AT/RhT, three in locations other than the cerebellum, seen at our institution in a 14-month period, indicating that this tumor may be more common than previously thought.     

Vertigo in children]

The main difference between childhood and adulthood vertigo is that the evaluation of symptoms and examination pose special challenges in pediatric patients, depending on age. Vertigo in pre-teenage children is similar to vertigo in adults. In contrast, although benign paroxysmal vertigo, vestibular neuronitis, and Ménière's vertigo occur in children, the distribution of these conditions is different from that seen in adults. After vestibular and cochlear investigations as well as a CT scan or MRI study to outrule a tumor of the posterior cerebral fossa, the diagnosis of vestibular vertigo is established. Often, the diagnosis proposed cannot be considered as final and requires reappraisal according to the long-term course. Collation of case-reports with highly accurate documents is essential in order to strive to develop more satisfactory approaches.     

Magnetic resonance imaging of lesions of the posterior fossa and upper cervical cord in childhood

Magnetic resonance imaging (MRI) promises to be an effective, noninvasive means of visualizing intracranial pathology. It should be especially useful in the evaluation of posterior fossa and cervical spinal cord disease of childhood; computed tomographic (CT) evaluation is frequently suboptimal in this region. MRI results are reported for 46 consecutively seen children with posterior fossa and/or cervical spinal cord disease (28 had brain malignancies; seven had congenital anomalies; three had cerebrovascular accidents). MRI was performed primarily by the partial saturation on a .12 Tesla resistive proton unit. All patients underwent concurrent CT evaluation. MRI demonstrated abnormalities in 96% of scans in patients with structural CNS disease (48 of 50). CNS malignancies were visualized in 100% (28 of 28) of children studied. MRI was especially useful in demonstrating the full extent of infiltrating gliomas and the anatomic location of other mass lesions. MRI frequently demonstrated disease to be more extensive than seen on CT. MRI was more sensitive than CT in documenting response to treatment and disease relapse in patients with infiltrating tumors. Cystic regions within tumors were poorly seen on MRI. Congenital anomalies were demonstrated in all patients evaluated and were better delineated using MRI than CT. MRI is sensitive in the evaluation of posterior fossa and cervical spinal cord disease of childhood and it has obvious advantages over CT; however, its specificity in such evaluations has yet to be proven.     

Psychological factors affecting pediatric inflammatory bowel disease

PURPOSE OF REVIEW: Psychological factors affecting pediatric inflammatory bowel disease, intervention and other therapeutic resources are reviewed. RECENT FINDINGS: Children with inflammatory bowel disease are at risk for more difficulties in psychosocial functioning than healthy children, particularly depression, anxiety and social difficulties. Psychosocial difficulties are generally similar to those found in other pediatric chronic illnesses and are clinically significant in only a subset of those with inflammatory bowel disease. Conflicting results have been reported for the areas of family dysfunction and body image, and few studies have been published in the areas of stress/coping and eating disorders. One pilot study suggests psychotherapy is effective for depressed adolescents with inflammatory bowel disease. SUMMARY: The scant existing research limits conclusions about which children are most at risk for experiencing problems. Future research should investigate a range of psychosocial outcomes and risk factors for developing problems. Prevention and intervention strategies aimed at improving psychosocial functioning in children with inflammatory bowel disease should be developed and evaluated.     

Neuropsychologic function in children with brain tumors: III. Interval changes in the six months following treatment

Twenty-six children with primary brain tumors were studied prospectively with regard to their sensorimotor, intellectual, academic, and emotional status. Serial evaluations were conducted after surgery (pre-irradiation) and six months after the completion of radiation therapy. The timing of the second evaluation was chosen so as to antedate the late effects of irradiation. Children over 6 years old displayed significant improvement of intellectual function over time, with only 11% exhibiting deterioration on one or more cognitive parameters. In contrast, 68% of younger children clinically deteriorated in one or more areas of intellectual functioning, with prominent difficulties in memory and selective attention for age. Children under 6 years old with supratentorial tumors were less likely than those with posterior fossa tumors to improve their cognitive performance. At the second evaluation, 23% of the patients were functioning below normal (IQ less than 80) intellectually, with 50% of the younger children and 11% of the older children receiving special educational assistance. Approximately 40-50% of the patients manifested emotional adjustment problems at each evaluation. The results are discussed in terms of the clinical need to follow similar patient populations with formal psychological assessments over time, and in terms of the difficulties involved in defining factors that determine the functional status of children surviving brain tumors.