Osmotic myelinolysis with malignant cerebellar edema occurring after DDAVP-induced hyponatremia in a child.

Central pontine myelinolysis (CPM) and extrapontine myelinolysis (EPM) are dire neurological disorders, characterized by severe damage to the myelin sheath of neurons, which typically result from rapid correction or overcorrection of systemic hyponatremia. For many years, both conditions have been considered universally fatal, though survivors have been reported more recently. Pediatric cases are rare. We present a 13-year-old boy with panhypopituitarism secondary to repair of a nasofrontal encephalocele in infancy, managed on long-term corticosteroid, deamino arginine vasopressin and thyroid hormone. He presented with severe hyponatremia (116 mEq/l), which during correction rapidly and unexpectedly increased to 176 mEq/l, resulting in profoundly impaired consciousness. Brain imaging revealed multiple bilateral changes in the basal ganglia, thalamus, pons and cerebral white matter, consistent with both CPM and EPM. Malignant cerebellar edema necessitated emergent suboccipital craniectomy, with subsequent improvement in level of consciousness and imaging postoperatively. However, he succumbed to acute cardiorespiratory arrest 8 weeks later. Nine similar cases from the literature are reviewed.

The association between intracranial tumours and multiple dyschondroplasia (Ollier's disease or Maffucci's syndrome): do children and adults differ?

In Ollier's disease (OD) and Maffucci's syndrome (MS), there is deforming dysplasia of cartilage, primarily but not exclusively involving the metaphyses and diaphyses of long bones. In a minority of patients with either of these rare syndromes, dysplasia can lead to sarcomatous degeneration, producing chondrosarcomas. There also appears to be an association with other neoplasms, which can include intracranial tumours. The primary objective of the current paper was to compare children/adolescents who have either OD or MS and an intracranial malignancy with their adult counterparts. All relevant cases in the medical literature were identified by electronically searching PubMed, SciSearch, Scientific Commons, Springer Link, and Google. Translate DotNet and Babelfish were used to translate non-English text. Non-parametric Pearson chi-square analyses were used to compare youths versus adults with respect to gender and geographic distribution (by continent), tumour histology and site of lesion, and the underlying enchondromatosis syndrome (OD vs. MS). All tests were 2-tailed, and P < 0.05 represented a statistically-significant difference. Forty-six patients with 47 intra-cranial malignancies were identified, with nine of the patients being 18 years old or less and categorized as youths. The incidence of intracranial chondrosarcomas peaked in the fourth decade of life, in parallel with the peak number of MS cases; conversely, both non-sarcomas and OD peaked in the third decade of life. Six of nine youths (67%) versus 17 of 36 adults with gender data (47%) were female (P = 0.30). There was no difference in geographic distribution by continent (P = 0.82). Four youths (44%) versus 16 adults (43%) had a chondrosarcoma (P = 0.95), and there was no statistically significant difference by tumour site (P = 0.42). However, seven (77%) of the youths had Ollier's disease as their underlying enchondromatosis syndrome, versus just 17 (46%) of the adults, a difference that approached statistical significance (P = 0.086). The association between enchondromatosis and intracranial malignancy seems to be roughly the same in youths versus adults, though Ollier's disease cases appear to predominate among youths.

Bilateral decompressive craniectomy for refractory intracranial hypertension in a child with severe ITP-related intracerebral haemorrhage.

We report a 13-month-old infant who developed acutely elevated intracranial pressure (ICP) as a result of a spontaneous intracerebral haemorrhage (ICH), secondary to idiopathic thrombocytopenic purpura (ITP). Her ICP remained severely elevated despite aggressive medical measures, with persistent obtundation, right hemiparesis and a dilated left pupil. Bilateral decompressive craniectomies (DCs) were performed, which resulted in a rapid decline in ICP. Ultimately, the patient regained consciousness and went on to complete neurological recovery. Tragically, she died of non-neurological, ITP-related complications 9 months later. In our review, we identified no other instances of bilateral DCs reported in the management of an infant with ITP and/or an ICH. We addressed three central questions: (1) Is there any value of DCs in children, and especially in infants, with elevated ICP? (2) Is there any value of DCs in the setting of non-traumatic ICH? And (3) is there any rationale for the use of bilateral versus unilateral DCs?

Intracerebral pneumatocele presenting after air travel.

In this report the authors discuss a patient who experienced symptoms of an acute right frontal, intraparenchymal pneumatocele while on an airplane descending to an international airport. This rare complication of an ethmoid sinus osteoma that eroded upward through the dura mater is described along with a literature review. A persistent headache and inappropriate behavior consistent with a frontal lobe syndrome brought the patient to clinical and imaging evaluation, which revealed a large right frontal lobe pneumatocele and an associated ethmoid sinus osteoma extending upward into the frontal lobe. Through a right frontal craniotomy, the air cavity was evacuated, the osteoma partially excised, and the dural defect closed using a vascularized pericranial flap. Postoperatively, the patient made an unremarkable recovery. For patients with air sinus osteomas extending into the cranial cavity, air travel or other barotrauma may result in a life-threatening tension pneumatocele.

Do intracranial neoplasms differ in Ollier disease and maffucci syndrome? An in-depth analysis of the literature.

OBJECTIVE: Ollier disease (OD) and Maffucci syndrome are closely related, very rare syndromes, that are both associated with multiple sites of chondrodysplasia. They differ primarily with the additional association of Maffucci syndrome with vascular abnormalities, including hemangiomas. Both are associated with chondrosarcomas and other nonsarcomatous neoplasms (NSN), but Maffucci syndrome is thought to have greater malignancy potential. We examined whether OD and Maffucci syndrome differ in the number, histology, in the location of intracranial malignancies, and in the demographics of such patients, as reported in the medical literature. METHODS: Relevant cases were identified by electronic searches on PubMed, SciSearch, Scientific Commons, Springer Link, and Google. Translate DotNet and Babelfish were used to translate non-English text. Unpaired Student's t tests were used to compare OD and Maffucci syndrome, and chondrosarcoma and NSN patients for mean age; Pearson chi analysis was used for comparisons of gender distribution, geographical distribution (by continent), site of lesion, and for OD versus Maffucci syndrome, tumor type (chondrosarcoma versus NSN). RESULTS: Forty-six patients with 47 intracranial malignancies were identified: 24 with OD, including 6 with a chondrosarcoma and 18 with an NSN; and 22 with Maffucci syndrome, including 13 with a chondrosarcoma, 8 with an NSN, and 1 with both. The 2 syndromes were statistically different in the distribution of chondrosarcomas versus NSNs (P = 0.002). All chondrosarcomas originated at the base of the cranium. All 18 NSNs in OD were of glial cell origin, but only 5 of 9 NSNs in Maffucci syndrome were glial; other tumors included pituitary adenoma, olfactory neuroblastoma, malignant chordoma, and spindle cell hemangioendothelioma. Patients with OD were more than 10 years younger than their Maffucci syndrome counterparts (24.7 versus 34.9 years; P = 0.002), as were patients with OD and chondrosarcoma versus those with Maffucci syndrome and chondrosarcoma (24.7 versus 36.2 years; P = 0.035). The 2 syndromes did not differ in overall sex distribution. OD and Maffucci syndrome differed in geographical distribution, with 10 Maffucci syndrome but no OD patients with malignancy either in Asia (7 patients) or South America (3 patients). Among NSNs, OD and Maffucci syndrome did not differ as to site of lesion within the brain. CONCLUSION: OD and Maffucci syndrome differ with respect to the distribution of intracranial malignancies by histology, and geographical and age distribution of cases, with OD patients younger by approximately a decade, and Maffucci syndrome patients more likely to live in Asia or South America.

Pediatric thalamic glioblastoma associated with Ollier disease (multiple enchondromatosis): a rare case of concurrence.

Ollier disease and Maffucci syndrome are rare syndromes in which there is deforming dysplasia of cartilage, primarily but not exclusively involving the metaphyses and diaphyses of long bones. In a minority of patients, dysplasia can lead to sarcomatous degeneration, producing chondrosarcomas. There also appears to be an association with other neoplasms. Little has been written about the association between Ollier disease and intracranial tumors, and these papers have largely consisted of case reports in adults. The authors present the case of a 6-year-old girl with left arm osseous changes consistent with Ollier disease and a biopsy-proven thalamic glioblastoma multiforme. They then examine the co-occurrence of brain tumors in conjunction with a dyschondroplasia syndrome in children and adolescents to assess the presentation, treatment offered, and disease course of similar cases. Eight other such cases were identified, 6 in patients with Ollier disease (ranging in age from 7 to 18 years), and 2 with Maffucci syndrome (both in late adolescence). Including our own patient, 7 of the 9 cases of comorbid dyschondroplasia and intracranial malignancy occurred in girls. Some patients presented soon after the acute onset of symptoms, and others had a more subtle, protracted course over as many as 2 years. Some tumors were deemed resectable and others not. In only 1 instance was follow-up beyond 1 year reported.