Embryonal Tumor With Multilayered Rosettes of the Parietooccipital Region: A Case Report.

Embryonal tumor with multilayered rosettes is a rare and highly malignant early childhood brain tumor. We report a case of embryonal tumor with multilayered rosettes in the parietooccipital region of a 2-year-old girl. Histopathology of the tumor demonstrated amplification of the 19q13.42 locus and strong positivity for LIN28A. Treatment was multimodal and included 3 surgical resections, adjuvant chemotherapy with autologous stem cell rescue, and focal radiotherapy. The use of the agents vorinostat and isotretinoin, and the addition of focal radiation have not been extensively described in this patient population, but may attribute to our patient's sustained remission at 2.5-years follow-up.

Intradiploic Hematoma Associated With Synostosis in an Infant.

Intradiploic hematomas are extremely rare, particularly in newborns. Caused by bleeding between the inner and outer tables of the calvarium, they manifest with bony swelling of the skull. The authors present the first case of an intraosseous hematoma associated with synostosis, and the first report in a female patient. The clinical, radiological, surgical, and pathological characteristics of this lesion are discussed.

The preoperative incidence of raised intracranial pressure in nonsyndromic sagittal craniosynostosis is underestimated in the literature.

OBJECT: The presence of raised intracranial pressure (ICP) in untreated nonsyndromic, isolated sagittal craniosynostosis (SC) is an important functional indication for surgery. METHODS: A retrospective review was performed of all 284 patients presenting with SC to the Oxford Craniofacial Unit between 1995 and 2010. RESULTS: Intraparenchymal ICP monitoring was performed in 39 children following a standard unit protocol. Monitoring of ICP was offered for all patients in whom nonoperative management was considered on the basis of minimal deformity or in cases in which parents were reluctant to agree to corrective surgery. These patients presented at an older age than the rest of the cohort (mean age 56 months), with marked scaphocephaly (16/39, 41%), mild scaphocephaly (11, 28%), or no scaphocephalic deformity (12, 31%). Raised ICP was found in 17 (44%) patients, with no significant difference in its incidence among the 3 different deformity types. Raised ICP was not predicted by the presence of symptoms of ICP or developmental delay or by ophthalmological or radiological findings. CONCLUSIONS: The incidence of raised ICP in SC reported here is greater than that previously published in the literature. The lack of a reliable noninvasive method to identify individuals with elevated ICP in SC mandates consideration of intraparenchymal ICP monitoring in all patients for whom nonoperative management is contemplated.

Optic nerve sheath ultrasound in the assessment of paediatric hydrocephalus.

OBJECTS: Optic nerve sheath diameter has been linked with intracranial pressure in previous studies. Measurement of sheath diameter using transorbital ultrasound is proposed as a clinical assessment indicator of developing hydrocephalus in the paediatric population. METHODS: A retrospective review of optic nerve sheath ultrasound examinations performed in a clinical setting between 2000 and 2006 for assessment of hydrocephalus in a single institution. RESULTS: A total of 331 examinations were performed in 160 patients over the study period. A satisfactory image was possible in all cases. Sheath diameters were correlated with clinical case data from concurrent case records. CONCLUSION: Transorbital ultrasound is a reproducible, non-invasive technique for the assessment of optic nerve sheath diameter and is well tolerated in children. Our series revealed asymptomatic baseline value higher than in previous reports. Variation from individual case asymptomatic baseline was the most sensitive variable in the series in determining development of hydrocephalus. This technique is felt to be a useful adjunct in the assessment of hydrocephalus in the paediatric neurosurgical population.

Superior mesenteric artery syndrome (Wilkie's syndrome) complicating recovery from posterior fossa surgery in a child--a rare phenomenon.

INTRODUCTION: Superior mesenteric artery or Wilkie's syndrome is defined as compression of the third part of duodenum by superior mesenteric artery. It has not been previously reported in children after neurosurgical procedures. CLINICAL HISTORY: We present the case of a child who had persistent vomiting after surgery for posterior fossa astrocytoma complicated by low pressure hydrocephalus. CONCLUSION: Despite eventual hydrocephalus control, vomiting persisted, and she was diagnosed with superior mesenteric artery syndrome, this being confirmed radiologically and settled with appropriate nutritional management.

Spontaneous low-pressure headache complicating bag-piping.

We describe an interesting case of a 40-year-old lady who developed a spontaneous low-pressure headache whilst playing the bag-pipes. Playing wind instruments requires the generation of significant intra-thoracic pressure, which may have contributed to this musician's condition.

Predicting stroke risk in pediatric moyamoya disease with xenon-enhanced computed tomography.

OBJECTIVE: To determine whether estimates of regional cerebral blood flow (rCBF) using xenon computed tomography (XeCT) in children with moyamoya disease can predict stroke risk before and after treatment. METHOD: Seven patients with moyamoya disease underwent 22 serial Xe computed tomographic scans. Estimates of rCBF were obtained at three computed tomographic levels by use of a 5-minute inhalation of 28% Xe. Acetazolamide challenge was performed in eight scans. For comparison of abnormal vessel distribution and areas of infarction, 17 intra-arterial digital subtraction angiograms, 47 computed tomographic scans, and 15 magnetic resonance imaging scans were available. Follow-up exceeded 36 months in all patients. Mean follow-up for the interventional group was 65.2 months (n = 5; range, 37-109 mo) and 38 months for the nonoperative patients (n = 2; 36 and 40 mo). RESULTS: Of six Xe computed tomographic scans obtained at diagnosis, four revealed regions of oligemia, augmented vertebrobasilar flow, and regions of carotid steal after acetazolamide. In the delay between diagnosis and treatment, three patients had strokes in ischemic areas identified by XeCT. Of the 10 posttreatment scans obtained from 4 patients, 2 revealed improved tissue perfusion with angiography confirming successful encephaloduroangiomyosynangiosis. In 2 others, XeCT performed 6 months posttreatment revealed improved perfusion without angiographic change, and angiography at 1 year revealed failed encephaloduroangiomyosynangiosis and new native collaterals. None of the patients with improved rCBF had new strokes. Eleven of 14 Xe computed tomographic scans were obtained within 30 days of angiography. Comparison of these studies demonstrates that regions of oligemia were confined to areas associated with vessel stenosis and little neovascularity or collateral pathways. CONCLUSION: XeCT, particularly with acetazolamide challenge, objectively quantifies rCBF. Our preliminary data suggest that it may permit assessment of stroke risk in children with moyamoya disease and may predict surgical outcome earlier than angiography.

Mastoid air sinus abnormalities associated with lateral venous sinus thrombosis: cause or consequence?

BACKGROUND: Mastoiditis is a known cause of lateral venous sinus thrombosis (LST). We have encountered patients with LST associated with mastoid abnormality on MRI without any clinical signs of infection; the significance of these abnormalities is uncertain. This study examines the relationship of LST and mastoid air sinus abnormalities systematically. SUMMARY OF REPORT: We performed a retrospective clinical and radiological review of a series of 26 patients with cerebral venous thrombosis. Mastoid abnormalities were detected ipsilateral to 9 of 23 thrombosed lateral sinuses (39%) and 0 of 29 unaffected lateral sinuses (P<0.001). No patient had clinical evidence of mastoiditis. Eight of 9 patients with mastoid abnormalities were treated without antibiotics; all made uneventful clinical recoveries. Repeated MRI in 1 patient revealed reversal of the mastoid changes. CONCLUSIONS: The mastoid changes observed are likely to be due to venous congestion as a consequence of LST, not mastoiditis.