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Jahangir Sajjad, Chandrasekaran Kaliaperumal, Niamh Bermingham, Charles Marks and Catherine Keohane

This 40-year-old man presented with a 5-month history of progressive right-sided headache associated with visual blurring. He also had a history of epilepsy but had been seizure free with medication for the past 10 years. An initial CT scan of his brain performed 16 years previously had revealed a small area of calcification in the right parietal region. In the current presentation, he had a left-sided homonymous hemianopia but no other neurological deficits. A CT scan of his brain showed a much larger calcified, partly cystic lesion in the right parietal region. Because he was symptomatic, the lesion was excised and the cyst was drained. Histological examination of the excised tissue showed an unusual primary tumor that was difficult to classify but had some features of angiocentric glioma. The heavy calcification, mixed-density cell population, and regions with features of angiocentric glioma were most unusual. The patient remained asymptomatic 5 years after surgery, and follow-up scans did not show recurrence.

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John A. Emelifeonwu, Drahoslav Sokol, Pasquale Gallo, Jothy Kandasamy and Chandrasekaran Kaliaperumal

The authors report a case of a child with hypothalamic-origin pilocytic astrocytoma and hydrocephalus, which was refractory to treatment with a ventriculoperitoneal shunt due to high CSF protein content. With parental education, the child's hydrocephalus was managed long-term in the community with a long-tunnelled external ventricular drain, which was maintained by his parents. To the authors' knowledge this is the first report of this management option as a long-term measure. No harm has come to the patient. The authors propose long-term, long-tunnelled external ventricular drain as a viable treatment option for such patients.

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Ingrid Kieran, Zaitun Zakaria, Chandrasekaran Kaliaperumal, Declan O'Rourke, Alan O'Hare, Eoghan Laffan, John Caird, Mary D. King and Dylan J. Murray

The authors describe the case of a 3-year-old boy with a giant congenital vertex hemangioma who underwent presurgical embolization with Onyx (ethylene-vinyl alcohol copolymer dissolved in dimethyl sulfoxide) and Glubran (N-butyl-2-cyanoacrylate). This vascular tumor had no intracranial vascular communication as assessed by pre-embolization MRI and catheter angiography. All embolizations were performed by direct percutaneous injection. One week following the last embolization procedure the child presented with a 24-hour history of ataxia and extrapyramidal tremor. He was diagnosed with a possible immune-mediated reaction to Onyx or Glubran, which was treated with an urgent surgical excision of the hemangioma followed by intravenous administration of immunoglobulin and steroids. To the authors' knowledge, this is the first case of possible immune-mediated toxicity secondary to either Onyx or Glubran administration. This case highlights the need for awareness of potential toxic reactions to these embolic agents in the treatment of hemangiomas in the pediatric patient.

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Zaitun Zakaria, Chandrasekaran Kaliaperumal, Darach Crimmins and John Caird

OBJECTIVE

The aim of this study was to assess the outcome of neurosurgical treatment in children with bleeding diathesis and also to evaluate the current management plan applied in the authors’ service.

METHODS

The authors retrospectively analyzed all cases in which neurosurgical procedures were performed in pediatric patients presenting with intracranial hematoma due to an underlying bleeding tendency over a 5-year period at their institution. They evaluated the patients’ neurological symptoms from the initial referral, hematological abnormalities, surgical treatment, neurological outcome, and scores on the Pediatric Glasgow Outcome Scale–Extended (GOS-E Peds) obtained 1 year after the last operation.

RESULTS

Five patients with a bleeding diathesis who underwent surgery for intracranial hematoma were identified; the diagnosis was hemophilia A in 3 cases, idiopathic thrombocytopenic purpura in 1 case, and severe aplastic anemia in 1 case. Intracerebral hematoma (ICH) (n = 4) and acute subdural hematoma (n = 1) were confirmed on radiological investigations. In 2 of the 4 patients with ICH, the diagnosis of bleeding diathesis was made for the first time on presentation. Four patients (all male) were younger than 2 years; the patient with severe aplastic anemia and spontaneous ICH was 15 years old and female. The duration of symptoms varied from 24 hours to 5 days. Neurological examination at 1 year’s follow-up showed complete recovery (GOS-E Peds score of 1) in 3 cases and mild weakness (GOS-E Peds score of 2) in 2 cases.

CONCLUSIONS

Neurosurgical management of patients with bleeding diathesis should be carried out in a tertiary-care setting with multidisciplinary team management, including members with expertise in neuroimaging and hematology, in addition to neurosurgery. Early diagnosis and prompt treatment of a bleeding diathesis is crucial for full neurological recovery.

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Steven Tominey, Chandrasekaran Kaliaperumal and Pasquale Gallo

OBJECTIVE

Contention exists regarding appropriate classification and management of spinal lipomas (SLs). Given the heterogeneity of SLs, omissions and overlap between surgically incomparable groups exist in conventional classification systems. The new classification of spinal lipoma (NCSL) recently proposed by Morota et al. delineates morphology by embryological pathogenesis and the resultant operative difficulty. Here, the authors aimed to validate the NCSL by applying it to patients who had been operated on at their institution.

METHODS

All children who had undergone resection for SL between 2014 and 2018 were included in this analysis. MRI studies were independently reviewed and classified by three adjudicators. Baseline characteristics, inter-adjudicator agreement, coexisting anomalies and/or malformations, and postoperative outcomes and complications were analyzed.

RESULTS

Thirty-six patients underwent surgical untethering for SL: NCSL type 1 in 5 patients (14%), type 2 in 14 patients (39%), type 3 in 4 patients (11%), and type 4 in 13 patients (36%). All classification was agreed on first assignment by the adjudicators. Radical or near-radical resection, which was attempted in all patients, was always possible in those with type 1 and 4 SL, but never in those with type 2 and 3 SL. Neurological stabilization and/or improvement were observed in all patients at the last follow-up.

CONCLUSIONS

The NCSL was found to be a logical and reproducible system to apply in this SL population. All cases were successfully classified with a high degree of inter-assessor agreement. Widespread establishment of a commonly adopted and clinically useful classification system will enable clinicians to improve patient selection as well as discussion with patient representatives during the decision-making process.