The Foramen magnum in isolated and syndromic brachycephaly

Background

Though the foramen magnum (FM) is often altered in complex craniosynostosis, no study analysed the FM dimensions in patients with brachycephaly specifically.

Patients and methods

We measured the FM area, sagittal and transverse diameters on preoperative CT scans in patients with bicoronal synostosis (n?=?40) and age-matched control group (n?=?18). Our study included 16 children with FGFR3 p.Pro250Arg mutation (mean age 6.1 months), 10 with TWIST-1 mutation (mean age7.6 months) and 14 patients with isolated bicoronal synostosis (mean age 6.1).

Results

We observed a significantly smaller FM area in FGFR3 group compared to control group and isolated brachycephaly group (p?=?0.001 and p?=?0.038, respectively). The mean FM area in FGFR3 group was 426.13 mm² (p?=?0.001), while in TWIST-1 group was 476.34 mm² (p?=?0.103), and in isolated brachycephaly group 489.43 mm² (p?=?0.129) compared to control group: 528.90 mm². The posterior segment of the sagittal diameter of the FM and its width as well as the bi-interoccipital synchondrosis diameters were significantly smaller in FGFR3 group compared to control group. In TWIST-1 group, the only altered dimension was the FM anterior segment of the sagittal diameter (p?=?0.008). We did not observe any significant alteration of FM in patients with isolated brachycephaly compared to control group.

Discussion and conclusions

The FM area is significantly altered in FGFR3-related brachycephaly, whereas in patients with Saethre–Chotzen syndrome (TWIST-1 mutation) the mean FM area is similar to control group. This study confirms the importance of FGFRs on FM growth whereas TWIST-1 seems to have a minor role.     

Optical coherence tomography: a quantitative tool to screen for papilledema in craniosynostosis

Purpose

Our aim was to evaluate if optical coherence tomography (OCT) can be used as an alternative for fundoscopy to screen for increased intracranial pressure (ICP) in children with craniosynostosis

Methods

We performed a prospective cohort study at the Dutch Craniofacial Centre. We included 38 patients with nonsyndromic scaphocephaly and Crouzon’s syndrome aged 3–8 years old, in whom we scored complaints suggestive of increased ICP and performed fundoscopy and OCT. Main outcome measures total retinal thickness (TRT) which was measured on 58 OCT scans.

Results

Forty-three percent of fundoscopies revealed pathologic changes of the papil in at least one eye. Retinal thickness was increased in patients with an abnormal fundoscopy as compared to patients with a normal papil (TRT p?<?0.001). Patients with Crouzon’s syndrome had a significantly increased retinal thickness as compared to patients with scaphocephaly (TRT p?<?0.001).

Conclusions

The current study demonstrates that OCT in children with craniosynostosis is feasible. It confirms that retinal thickness increases in case of papilledema. Given the quantitative character, OCT has a high potential as an alternative tool to screen for papilledema in craniosynostosis and other pediatric populations.     

Does optic nerve sheath diameter on MRI decrease with clinically improved pediatric hydrocephalus?

Introduction

Serial change in ventricular size is recognized as an imperfect indicator of ongoing hydrocephalus in children. Potentially, other radiographic features may be useful in determining the success of hydrocephalus interventions. In this study, optic nerve sheath diameter (ONSD), optic nerve tortuosity, and optic disk bulging were assessed as indicators of hydrocephalus control in children who underwent endoscopic third ventriculostomy (ETV) or posterior fossa tumor resection.

Methods

Sixteen children underwent ETV or tumor resection for treatment of hydrocephalus. T2-weighted axial magnetic resonance images of the orbit were obtained, and the ONSD was measured posterior to the optic globe, pre- and post-intervention. Evidence of optic disk bulging and optic nerve tortuosity was also assessed. Ventricular size was estimated using the frontal and occipital horn ratio (FOR).

Results

There was a significant reduction in the ONSD post-ETV (n?=?9) and after tumor resection (n?=?7). Average preoperative ONSD was 6.21 versus 5.71 mm postoperatively (p?=?0.0017).There was also an 88 % (p?=?0.011) and 60 % (p?=?0.23) reduction in optic disk bulging and tortuosity, respectively. The FOR normalized in the tumor resection group but not the ETV group. After intervention, all patients showed improvement in signs and symptoms of hydrocephalus.

Conclusion

In our study population, ONSD decreased in response to measures to reduce hydrocephalus. Optic disk bulging also appears to resolve. Serial reduction in ONSD, and optic disk bulging may be indicators of improved hydrocephalus following pediatric neurosurgical interventions.     

Sleep apnoea in syndromic craniosynostosis occurs independent of hindbrain herniation

Purpose

Hindbrain herniation (HH) is frequently found in syndromic craniosynostosis. It may cause impairment of the respiratory centres and manifest as sleep-disordered breathing. Our aim was to quantify sleep apnoea caused by HH in children with syndromic craniosynostosis.

Methods

Seventy-one children with syndromic and complex craniosynostosis (aged 0–18 years) underwent prospective magnetic resonance imaging and a sleep study. The position of the cerebellar tonsils and respiratory parameters were evaluated and analysed. None of the included patients had undergone previous foramen magnum decompression.

Results

HH was present in 35 % of the patients and was more frequent in children with Crouzon syndrome (63 %) than in other types of craniosynostosis (p?=?.018). There was a positive association between the position of the cerebellar tonsils and papilledema (p?=?.002). Sleep studies of children with craniosynostosis and HH were not different from those without HH. Obstructive sleep apnoea syndrome was not more prevalent in children with HH compared to those without HH (p?=?.12). A cluster analysis using indices of apnoea revealed that three new clusters between which age was significantly different (p?=?.025).

Conclusion

Sleep apnoea in syndromic craniosynostosis is not caused by HH. Rather, our evidence suggests that sleep-disordered breathing in craniosynostosis may be caused by brain stem immaturity in young children or upper airway obstruction. Therefore, as long as the child remains asymptomatic, our preferred management of HH is to be conservative and provide regular neurosurgical follow-up.     

Microsurgical outlet restoration in isolated fourth ventricular hydrocephalus: a single-institutional experience

Purpose

Isolated fourth ventricles as a consequence of shunted posthemorrhagic hydrocephalus can cause significant brainstem compression and subsequent clinical deficits in children. Several treatment options have been described. We report the clinical and radiological outcome after microsurgical fenestration of fourth ventricular outlet foramen via a suboccipital approach.

Methods

In nine patients (age, 9 to 87?months; median age, 21?months), microsurgical reopening of formerly occluded outlet foramen of the fourth ventricle was performed under electrophysiological monitoring. Pre- and postoperative clinical outcome as well as radiological results are reported.

Results

Mean follow-up for all children was 25?months. Three children older than 3?years were less significantly involved, the remaining six showed tremendous long tract signs and lower cranial nerve deficits. All children exhibited a remarkable improvement of their preexisting neurological deficits post-surgery. Despite successful fenestration, one child required additional internal drainage of fourth ventricle to the lateral ventricles due to malabsorption. Median diameters of the fourth ventricle changed markedly after surgery with anterior–posterior (a.p.) extension from 3.8 to 2.9?cm, lateral extension from 4.2 to 2.8?cm (p?=?0.018), and craniocaudal extension from 5.8 to 4.7?cm, respectively. Also, the pontine a.p. diameter increased significantly from 0.8 to 1.5?cm (p?=?0.022).

Conclusion

The clinical and radiological outcomes after microsurgical fenestration in children with an isolated fourth ventricle are very promising. This treatment modality is a safe and effective shunt-free option when electrophysiological monitoring and thorough preoperative neuroradiological work-up are applied.     

Diffusion tensor imaging of the superior cerebellar peduncle identifies patients with posterior fossa syndrome

Introduction

Posterior fossa tumors are the most common brain tumor of children. Aggressive resection correlates with long-term survival. A high incidence of posterior fossa syndrome (PFS), impairing the quality of life in many survivors, has been attributed to damage to bilateral dentate nucleus or to cerebellar output pathways. Using diffusion tensor imaging (DTI), we examined the involvement of the dentothalamic tracts, specifically the superior cerebellar peduncle (SCP), in patients with posterior fossa tumors and the association with PFS.

Methods

DTI studies were performed postoperatively in patients with midline (n?=?12), lateral cerebellar tumors (n?=?4), and controls. The location and visibility of the SCP were determined. The postoperative course was recorded, especially with regard to PFS, cranial nerve deficits, and oculomotor function.

Results

The SCP travels immediately adjacent to the lateral wall of the fourth ventricle and just medial to the middle cerebellar peduncle. Patients with midline tumors that still had observable SCP did not develop posterior fossa syndrome (N?=?7). SCPs were absent, on either preoperative (N?=?1, no postoperative study available) or postoperative studies (N?=?4), in the five patients who developed PFS. Oculomotor deficits of tracking were observed in patients independent of PFS or SCP involvement.

Conclusion

PFS can occur with bilateral injury to the outflow from dentate nuclei. In children with PFS, this may occur due to bilateral injury to the superior cerebellar peduncle. These tracts sit immediately adjacent to the wall of the ventricle and are highly vulnerable when an aggressive resection for these tumors is performed.     

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.     

Calcifications associated with pediatric intracranial arterial aneurysms: incidence and correlation with pathogenetic subtypes

Background and purpose

Little is known about calcifications associated with pediatric intracranial arterial aneurysms (IAA). We sought to characterize calcifications associated with pediatric IAA according to aneurysm pathogenetic subtype.

Materials and methods

Patients with IAA less than 20 years of age were retrospectively identified. Three fellowship-trained neuroradiologists independently reviewed each patient’s CT studies for calcifications of the parent artery or aneurysm. Aneurysmal calcification (ANC) was correlated with characteristics of the patient (age, sex) and aneurysm pathogenetic subtype, size, morphology, rupture status, and location.

Results

Thirty-three patients (mean age 10 years) with 43 IAA were analyzed. There were no parent artery calcifications. Nine IAA were calcified. IAA in children with non-hemodynamic risk factors (arteriopathy, trauma, infection, tumor) were more commonly calcified than idiopathic IAA (p?=?0.029). More than one third of the pediatric IAAs in this group (arteriopathy, infection trauma, tumor) were calcified. IAA?≥?10 mm were more likely to be calcified (p?=?0.03). IAA that were ruptured at presentation were less likely to be calcified (p?=?0.03). ANC was not significantly associated with patient age (≤10 years vs. >10 years), sex, morphology (fusiform vs. saccular) or location (anterior vs. posterior circulation).

Conclusion

Aneurysmal but not parent artery calcifications are associated with a significant minority of pediatric IAA. Pediatric ANCs are associated with underlying non-hemodynamic vascular risk factors (arteriopathy, infection, trauma, and tumor), size ≥10 mm and non-hemorrhagic presentation.     

Self-Regulatory Fatigue,Quality of Life,Health Behaviors,and Coping in Patients with Hematologic Malignancies

Background

Self-regulatory fatigue may play an important role in a complex medical illness.

Purpose

Examine associations between self-regulatory fatigue, quality of life, and health behaviors in patients pre- (N?=?213) and 1-year post-hematopoietic stem cell transplantation (HSCT; N?=?140). Associations between self-regulatory fatigue and coping strategies pre-HSCT were also examined.

Method

Pre- and 1-year post-HSCT data collection. Hierarchical linear regression modeling.

Results

Higher self-regulatory fatigue pre-HSCT associated with lower overall, physical, social, emotional, and functional quality of life pre- (p’s?p’s?p?p?p?p’s?Conclusion This is the first study to show self-regulatory fatigue pre-HSCT relating to decreased quality of life and health behaviors, and predicting changes in these variables 1-year post-HSCT.     

Endoscopic third ventriculocisternostomy in hydrocephalic children under 2 years of age: appropriate or not? A single-center retrospective cohort study

Purpose

Treating hydrocephalus can be difficult in children under the age of 2 years because a high amount of uncertainty exists as to which treatment to perform. In this retrospective cohort study, we analyzed children under the age of 2 years with hydrocephalus undergoing an endoscopic third ventriculocisternostomy (ETV) with respect to ETV outcome.

Methods

In 59 consecutive patients under the age of 2 years, an ETV was performed between 1999 and 2010 at the Erasmus MC, Sophia Children’s Hospital. Demographics, etiology of hydrocephalus, and radiological data were extracted retrospectively from the patients’ medical records and operative reports and related to outcome. ETV Success Score (ETVSS) was used to retrospectively calculate the probability of success related to the actual outcome.

Results

In this series, 42.4 % of patients had a successful ETV. The only statistically significant finding concerned age. The failed ETV patients appeared to be younger (0.52?±?0.60 vs. 0.86?±?0.56 year, p?=?0.005), and when using a cutoff age of 6 months only, five out of 32 infants had a successful ETV (p?=?0.002). Of the children with an arachnoid cyst, 57.1 % were treated successfully with an ETV. Of the five patients with a high probability of ETV success, four (80 %) were indeed successfully treated with ETV (p?=?0.049).

Conclusions

Our data confirm the overall ineffectiveness of an ETV in children under the age of 6 months. Nevertheless, using the ETVSS is recommended to aid in the decision-making process even in patients under the age of 6 months.     

Visual function in infants with congenital hydrocephalus with and without myelomeningocoele

Objectives

Congenital hydrocephalus without or with associated myelomeningocoele has impaired visual function as a potential complication. The present study was embarked on to determine the frequency of optic nerve deficits and refractive errors in this group of children and document any relationship to neuroradiological measurements.

Materials and methods

All infants with congenital hydrocephalus associated with myelomeningocoele (MHC) and congenital hydrocephalus without myelomeningocoele (HC) were prospectively studied. The children underwent clinical neuro-ophthalmological evaluation and neuroimaging. Radiological confirmation and severity of hydrocephalus was by Evans ratio (frontal and occipital) and third ventricular diameter.

Results

There were 50 children (27 boys and 23 girls, median and mean age of 6 and 5.4 months, respectively) included in the study. Eighteen patients (36 %) had no or poor visual tracking and fixation, while nine (18 %) patients had optic atrophy. Optic atrophy was significantly associated with the HC group (p?=?0.007), while the MHC group was significantly associated with a lower Evans ratio (occipital ratio, p?=?0.000; frontal ratio, p?=?0.000). Forty-nine patients had anisometropia. The refractive errors were more commonly hypermetropia (46 patients). This was not significantly associated with HC or MHC (0.309).

Conclusion

Optic atrophy rarity in MHC is probably due to early presentation of the patients and lower Evans ratio (occipital and frontal). Evans ratio is a good predictive index for optic atrophy in infantile congenital hydrocephalus. Refractive errors frequency is not dependent on an association of myelomeningocoele with or without hydrocephalus.     

Results of posterior cranial vault remodeling for plagiocephaly and brachycephaly by the meander technique

Objective

Several techniques to remodel the posterior calvarium in order to increase intracranial volume (ICV) and to improve cosmetic appearance are reported. This study presents the results of meander technique in patients with brachycephaly and posterior plagiocephaly.

Methods

During December 2011 and July 2013, a total of 12 children (median age: 15 months) underwent posterior cranial vault remodeling by the meander technique (brachycephaly, n?=?6; posterior plagiocephaly, n?=?6). The available pre- and postoperative MRIs were assessed with regard to ICV, cranial index (CI) and asymmetry index (AI) as well as the position of the cerebellar tonsils.

Results

No intra- or postoperative complications were observed. Blood transfusions were necessary in nine of 12 patients. A significant increase of the ICV from 1,178.4?±?134.5 to 1,293.0?±?137.5 cm³ (p?p?p?p?p?p?Conclusion The presented surgical technique is considered to be safe. The technique is capable to significantly increase ICV and improve cosmetic appearance of the remodeled calvarium. Further evidence that posterior cranial vault remodeling influences the position of the cerebellar tonsils is added by the results of the study.     

The Effects of Parental Illness and Other Ill Family Members on the Adjustment of Children

Background

This study addresses limitations of prior research that have used group comparison designs to test the effects of parental illness on youth.

Purpose

This study examined differences in adjustment between children of a parent with illness and peers from ‘healthy’ families controlling for the effects of whether a parent or non-parent family member is ill, illness type, demographics and caregiving.

Methods

Based on questionnaire data, groups were derived from a community sample of 2,474 youth (‘healthy’ family, n?=?1768; parental illness, n?=?336; other family member illness, n?=?254; both parental and other family illness, n?=?116).

Results

The presence of any family member with an illness is associated with greater risk of mental health difficulties for youth relative to peers from healthy families. This risk is elevated if the ill family member is a parent and has mental illness or substance misuse.

Conclusions

Serious health problems within a household adversely impact youth adjustment.     

Does Affective Valence During and Immediately Following a 10-Min Walk Predict Concurrent and Future Physical Activity?

Background

Affect may be important for understanding physical activity behavior.

Purpose

To examine whether affective valence (i.e., good/bad feelings) during and immediately following a brief walk predicts concurrent and future physical activity.

Methods

At months?6 and 12 of a 12-month physical activity promotion trial, healthy low-active adults (N?=?146) reported affective valence during and immediately following a 10-min treadmill walk. Dependent variables were self-reported minutes/week of lifestyle physical activity at months?6 and 12.

Results

Affect reported during the treadmill walk was cross-sectionally (month?6: ???=?28.6, p?=?0.008; month 12: ???=?26.6, p?=?0.021) and longitudinally (???=?14.8, p?=?0.030) associated with minutes/week of physical activity. Affect reported during a 2-min cool down was cross-sectionally (month 6: ???=?21.1, p?=?0.034; month 12: ???=?30.3, p?<?0.001), but not longitudinally associated with minutes/week of physical activity. Affect reported during a postcool-down seated rest was not associated with physical activity.

Conclusions

During-behavior affect is predictive of concurrent and future physical activity behavior.     

Association of Acute Coronary Syndrome-Induced Posttraumatic Stress Disorder Symptoms with Self-Reported Sleep

Background

Symptoms of posttraumatic stress disorder (PTSD) after acute coronary syndrome (ACS) are associated with recurrent ACS events and mortality. Poor sleep may be a mechanism, but the association between PTSD and sleep after ACS is unknown.

Purpose

This study aims to estimate the association between ACS-induced PTSD symptoms and self-reported sleep.

Methods

ACS-induced PTSD symptoms were assessed 1-month post-ACS in 188 adults using the Impact of Events Scale-Revised. Sleep was assessed using the Pittsburgh Sleep Quality Index. Linear and logistic regression models were used to determine whether PTSD symptoms were associated with self-reported sleep, independent of sociodemographic and clinical covariates.

Results

In adjusted models, ACS-induced PTSD symptoms were associated with worse overall sleep (β?=?0.22, p?=?0.003) and greater impairment in six of seven components of sleep (all p values <0.05).

Conclusions

ACS-induced PTSD symptoms may be associated with poor sleep, which may explain why PTSD confers increased cardiovascular risk after ACS.     

The Fgfr2 W290R mouse model of Crouzon syndrome

Purpose

This study aimed to review and discuss the utility of the Fgfr2 ^W290R mouse mutant as a model of human Crouzon syndrome.

Methods

A review of current and past scientific literature on Fibroblast Growth Factor Receptor-2 (FGFR2) protein domain structure, FGFR mutations associated with human Crouzon syndrome, and phenotypic and molecular changes combined with recent observations and experimental data of the Fgfr2 ^W290R mouse mutant was conducted. A comparison of the Fgfr2 ^W290R mouse mutant with another mouse model of Crouzon syndrome, Fgfr2 ^C342R mouse mutant, was also performed. Finally, possible future research directions using the Fgfr2 ^W290R mutant mice were discussed.

Results

The Fgfr2 ^W290R heterozygous mouse exhibits defects characteristic of human Crouzon syndrome. At the molecular level, the defects observed in the mouse mutant are due to the dysregulation of signaling of both the IIIb and IIIc isoforms of Fgfr2. The involvement of the IIIb isoform of FGFR2 in the etiopathology of Crouzon syndrome is a novel finding in the craniosynostosis literature field. Dysregulated signaling of both IIIb and IIIc isoforms causes a broad spectrum of changes that explain some of the defects observed clinically in humans. Several of the defects observed in the Fgfr2 ^W290R homozygous mouse mutant are attributable to a loss-of-function mechanism in contrast to the frequently reported gain-of-function receptor function associated with mutated FGF receptors in craniosynostosis.

Conclusions

The Fgfr2 ^W290R mouse model can be used as a model system to further investigate the cellular, molecular, and biochemical mechanisms of Crouzon syndrome.     

Incidence, predictors and early post-operative course of diabetes insipidus in paediatric craniopharygioma: a comparison with adults

Purpose

This study aims to determine the incidence, predictors, early post-operative course of diabetes insipidus (DI) in paediatric craniopharyngiomas(CP) and compare the findings with adults.

Methods

Retrospective analysis of clinical, biochemical, radiological and operative data for 102 consecutive CP surgeries (45 paediatric and 57 adult cases) was done. Bivariate and multivariate analyses were done to determine the predictors of DI. The incidence of the triphasic response and electrolyte abnormalities in the first post-operative week was compared between children and adults.

Results

Children had larger tumours and higher incidence of cystic tumours and hydrocephalus. Preoperative DI was close to 15 % in both the age groups. Radical/subtotal excision was achieved in 58 % of children and 53 % of adults. The incidence of post-operative DI was 80 % and 63 % in children and adults, respectively. Children had significantly higher incidence of permanent DI (55.6 %). Radical excision in children (p?=?0.000); previous tumour surgery (p?=?0.014) and new onset hypopituitarism (p?=?0.019) in adults were associated with permanent DI. The triphasic response (23 %), wide intra-day serum sodium fluctuations and hyponatraemia were more common in children.

Conclusions

Post-operative DI is a frequent and significant cause of morbidity in children undergoing surgery for CP. Children have a higher incidence of permanent DI. Radical excision is a predictor of permanent DI in children, whereas previous tumour excision and new onset hypopituitarism were predictors of permanent DI among adults. The management of post-operative DI is more difficult in children and the treating physician needs to be alert to detect the triphasic response.     

Correlating brain volume and callosal thickness with clinical and laboratory indicators of disease severity in children with HIV-related brain disease

Background

Objective MRI markers of central nervous system disease severity may precede subjective features of HIV encephalopathy in children. Previous work in HIV-infected adults shows that brain atrophy was associated with low CD4 and with neuropsychological impairment. Significant thinning of the corpus callosum (CC), predominantly anteriorly, was also found in HIV-infected adults and correlated with CD4 levels. These findings have not been tested in children.

Purpose

The aim of this study was to determine if brain volume and midsagittal CC linear measurements (thickness and length) on MRI in children with HIV-related brain disease correlate with clinical and laboratory parameters of disease severity.

Methods

Retrospective MRI analysis in children with HIV-related brain disease used a volumetric analysis software and a semi-automated tool to measure brain volume and callosal thickness/length, respectively. Each measure was correlated with clinical parameters of disease severity including Griffiths Mental Development scores (GMDS), absolute CD4 counts (cells/mm³), nadir CD4 (the lowest CD4 recorded, excluding baseline), duration of HAART, and decreased brain growth.

Results

Thirty-three children with HIV-related brain disease were included. Premotor segment of the CC mean thickness correlated with age (p?=?0.394). Motor CC maximum thickness correlated significantly with general developmental quotient (p?=?0.0277); CC length correlated with a diagnosis of acquired microcephaly (p?=?0.0071) and to CD4 level closest to date of the MRI scan (p?=?0.04).

Conclusions

Length of the CC and the “motor CC segment” may represent surrogate clinical biomarkers of central nervous system disease severity and with decreased level of immunity in HIV-infected patients that precede established HIV encephalopathy.     

Neurodevelopmental functioning of infants with untreated single-suture craniosynostosis during early infancy

Purpose

Single-suture craniosynostosis (SSC) is a congenital craniofacial disorder, in which premature fusion of one of the skull sutures restricts and distorts growth of the cranium and underlying brain. This disorder of prenatal onset occurs during a critical phase of rapid growth and development of the immature brain. Craniosynostosis carries a known risk of developmental impairment. The neurodevelopmental sequelae of SSC prior to treatment remains however incompletely understood. This study sought to determine the neurodevelopmental sequelae of untreated single-suture craniosynostosis during early infancy.

Methods

Fifty-six consecutive patients with unoperated SSC (sagittal, metopic and unicoronal) comprised the sample cohort. Patients were aged between 4 and 16?months (M?=?8.9?months, SD?=?2.9?months). Neurodevelopmental functioning was assessed with the mental (Mental Development Index) and motor (Psychomotor Development Index) scales of the Bayley Scales of Infant Development, second edition.

Results

Children with SSC displayed significantly lower mean mental (M?=?97.7, SD?=?6.7, p?M?=?87.7, SD?=?13.0, p?Conclusions Untreated SSC is associated with an increased incidence of developmental delay during early infancy, with motor skills appearing the most vulnerable to impairment during this developmental phase.     

Effects of a Multiple Health Behavior Change Intervention for Colorectal Cancer Survivors on Psychosocial Outcomes and Quality of Life: a Randomized Controlled Trial

Background

Multiple health behavior change can ameliorate adverse effects of cancer.

Purpose

The purpose of this study was to determine the effects of a multiple health behavior change intervention (CanChange) for colorectal cancer survivors on psychosocial outcomes and quality of life.

Methods

A total of 410 colorectal cancer survivors were randomized to a 6-month telephone-based health coaching intervention (11 sessions using acceptance and commitment therapy strategies focusing on physical activity, weight management, diet, alcohol, and smoking) or usual care. Posttraumatic growth, spirituality, acceptance, mindfulness, distress, and quality of life were assessed at baseline, 6 and 12 months.

Results

Significant intervention effects were observed for posttraumatic growth at 6 (7.5, p?p?=?0.033), spirituality at 6 months (1.8, p?=?0.011), acceptance at 6 months (0.2, p?=?0.005), and quality of life at 6 (0.8, p?=?0.049) and 12 months (0.9, p?=?0.037).

Conclusions

The intervention improved psychosocial outcomes and quality of life (physical well-being) at 6 months with most effects still present at 12 months. (Trial Registration Number: ACTRN12608000399392).