Fever in a child with cerebrospinal fluid access device or shunt: a pragmatic approach to management.

Children with shunts commonly present with fever, and often the focus of infection will be unrelated to their shunt. However, as shunt infections may present with few or even no specific symptoms, evaluation of a child with a shunt presenting with fever should be careful and comprehensive to ensure shunt infections are not missed. Treatment of an infected shunt involves removal of the shunt followed by a long course of antibiotics; missing or partially treating shunt infections can result in significant morbidity and potentially even mortality. Our experience of managing children with shunts presenting with fever is that many non-specialist clinicians have little experience in this area so initial management may not always be appropriate. Those children who are most at risk of shunt infection are those who within the preceding 8 weeks have had insertion, revision or access of their shunt or chemotherapy device, or have had abdominal surgery in the presence of a ventriculoperitoneal shunt. We have chosen 8 weeks as a pragmatic time point, as in our experience the vast majority of children who have had shunt infections have presented within this period. The caveat is that this should not be used as an absolute cut-off where there is strong suspicion of shunt infection or no clear focus at a later time point.

A Donation After Circulatory Death Program Has the Potential to Increase the Number of Donors After Brain Death.

OBJECTIVES: Donation after circulatory death has been responsible for 75% of the increase in the numbers of deceased organ donors in the United Kingdom. There has been concern that the success of the donation after circulatory death program has been at the expense of donation after brain death. The objective of the study was to ascertain the impact of the donation after circulatory death program on donation after brain death in the United Kingdom. DESIGN: Retrospective cohort study. SETTING: A national organ procurement organization. PATIENTS: Patients referred and assessed as donation after circulatory death donors in the United Kingdom between October and December 2013. INTERVENTIONS: None. MEASUREMENTS AND MAIN RESULTS: A total of 257 patients were assessed for donation after circulatory death. Of these, 193 were eligible donors. Three patients were deemed medically unsuitable following surgical inspection, 56 patients did not proceed due to asystole, and 134 proceeded to donation. Four donors had insufficient data available for analysis. Therefore, 186 cases were analyzed in total. Organ donation would not have been possible in 79 of the 130 actual donors if donation after circulatory death was not available. Thirty-six donation after circulatory death donors (28% of actual donors) were judged to have the potential to progress to brain death if withdrawal of life-sustaining treatment had been delayed by up to a further 36 hours. A further 15 donation after circulatory death donors had brain death confirmed or had clinical indications of brain death with clear mitigating circumstances in all but three cases. We determined that the maximum potential donation after brain death to donation after circulatory death substitution rate observed was 8%; however due to mitigating circumstances, only three patients (2%) could have undergone brain death testing. CONCLUSIONS: The development of a national donation after circulatory death program has had minimal impact on the number of donation after brain death donors. The number of donation after brain death donors could increase with changes in end-of-life care practices to allow the evolution of brain death and increasing the availability of ancillary testing.

Fetal MRI demonstrating vein of Galen malformations in two successive pregnancies--a previously unreported occurrence.

PURPOSE: Vein of Galen malformations are rare and are usually detected in utero using ultrasonography. No definite genetic predisposition has been described in the literature. We present a case with two successive pregnancies complicated by vein of Galen malformations, which were assessed using fetal MRI. The putative role of genetic mutations is also discussed. METHODS: A 30-year-old primigravida presented in the third trimester with a fetus diagnosed with vein of Galen malformation on sonography. MRI and MR angiography were performed for further assessment. The subsequent pregnancy was again complicated by vein of Galen malformation. In addition to MRI, genetic analysis was carried out on both fetuses and on the parents. RESULTS: MR angiography revealed that both fetuses suffered from the choroidal sub-type of vein of Galen malformation, with multiple arterial feeders fistulating onto a midline venous pouch. The visualised anatomy obtained was far superior than on sonography and allowed categorisation of vein of Galen malformation sub-type. Genetic analysis on the mother and both fetuses showed variant RASA1 gene mutation. CONCLUSIONS: This case demonstrates that fetal MRI is a powerful tool in the investigation of in utero neurovascular malformations. A genetic mutation was identified, but this was of uncertain significance.

Management of extensive frontal cranioplasty defects.

Cranioplasty is a medical technique to correct cranial bone defects. Depending on the size and location of the defect, a bone substitute can be used to replace the missing bone. Frontal bone defects are important to patients in terms of cosmetics because they are visible. Advances in computer design allow the production of customized implants with improved cosmetic and functional results. This report describes hybrid optimization of three-dimensional technological methods along with traditional methods toward the manufacture of deep-buried titanium implants, restoring frontal skull defects for 4 patients. A three-dimensional model was produced from the computed tomographic scan data of 3 patients using an in-house three-dimensional printer. A new approach was followed in treating the fourth patient. The defect was restored using preoperative scan before cranioplasty. These data were transported digitally into the defect skull to recreate the bone contour required, and a three-dimensional model was produced from the "new" digital model using the three-dimensional printer. Defect areas of the patients were large and measured 101.21 × 123.35 (vertical × horizontal) in average (mm). Conventional wax-up of the defect was carried to restore normal conformity. A titanium sheet (0.5 mm) was swaged into the desired shape; however, convexity of the defect area makes titanium swaging challenging, especially at the deep lateral undercuts. Making side flanges at reasonable lengths made it easy to swage without creasing. Three-dimensional models aided to produce accurately fitting plates. Finally, the sequential method of using both digital and manual procedures is a low-cost, reliable, accurate, and reproducible method.

Infective endocarditis caused by Arcanobacterium haemolyticum: a case report.

Arcanobacterium haemolyticum is an organism that commonly causes pharyngitis and wound infections. It does not usually cause systemic invasive disease. The organism presents a difficult diagnostic problem because the Clinical Microbiology laboratory has a propensity to view them as diphtheroid organisms of the Corynebacterium species, thus contaminants or normal flora. We describe a case of a 21-year-old female who had endocarditis with cerebral emboli due to Arcanobacterium haemolyticum. This rare condition is associated with significant mortality and to the best of our knowledge; this is the first successfully treated case of A. haemolyticum endocarditis complicated by embolic phenomenon.

Homozygous loss of ADAM3A revealed by genome-wide analysis of pediatric high-grade glioma and diffuse intrinsic pontine gliomas.

Overall, pediatric high-grade glioma (pHGG) has a poor prognosis, in part due to the lack of understanding of the underlying biology. High-resolution 244 K oligo array comparative genomic hybridization (CGH) was used to analyze DNA from 38 formalin-fixed paraffin-embedded predominantly pretreatment pHGG samples, including 13 diffuse intrinsic pontine gliomas (DIPGs). The patterns of gains and losses were distinct from those seen in HGG arising in adults. In particular, we found 1q gain in up to 27% of our cohort compared with 9% reported in adults. A total of 13% had a balanced genetic profile with no large-scale copy number alterations. Homozygous loss at 8p12 was seen in 6 of 38 (16%) cases of pHGG. This novel deletion, which includes the ADAM3A gene, was confirmed by quantitative real-time PCR (qPCR). Loss of CDKN2A/CDKN2B in 4 of 38 (10%) samples by oligo array CGH was confirmed by fluorescent in situ hybridization on tissue microarrays and was restricted to supratentorial tumors. Only ∼50% of supratentorial tumors were positive for CDKN2B expression by immunohistochemistry (IHC), while ∼75% of infratentorial tumors were positive for CDKN2B expression (P = 0.03). Amplification of the 4q11-13 region was detected in 8% of cases and included PDGFRA and KIT, and subsequent qPCR analysis was consistent with the amplification of PDGFRA. MYCN amplification was seen in 5% of samples being significantly associated with anaplastic astrocytomas (P= 0.03). Overall, DIPG shared similar spectrum of changes to supratentorial HGG with some notable differences, including high-frequency loss of 17p and 14q and lack of CDKN2A/CDKN2B deletion. Informative genetic data providing insight into the underlying biology and potential therapeutic possibilities can be generated from archival tissue and typically small biopsies from DIPG. Our findings highlight the importance of obtaining pretreatment samples.

Coil embolization of ruptured middle cerebral artery aneurysms in the first 2 months of life. Report of two cases.

Intracranial artery aneurysms are very rare in infants. There have been no previous reports of coil embolization to branches of the middle cerebral artery (MCA) in infants. The authors describe successful embolization of ruptured aneurysms of the right frontoopercular MCA branch in a 31-day-old infant, and of the left sylvian MCA branch in a 54-day-old infant. One of the cases involves a dissection flap. The authors also review cases in which coil embolization was used for intracranial aneurysms in the first 2 months of life.