Posterior fossa arachnoid cyst masking a delayed diagnosis of hyperparathyroidism in a child.

Background. Primary hyperparathyroidism in childhood is a very rare entity, often being diagnosed late after the onset of its presenting symptoms. It most commonly affects patients in their fourth decade of life and beyond. The inclusion of primary hyperparathyroidism in the differential diagnosis is necessary when evaluating patients presenting with nonspecific symptoms such as polyuria, fatigue, weight loss, abdominal pain, nausea, and vomiting. Methods. We report the case of an eleven-year-old girl presenting with three years history of headaches, visual disturbance, along with episodes of emotional lability. Neuroimaging confirmed a large posterior fossa arachnoid cyst. It was decided to manage this lesion conservatively with surveillance. Only after further hospital admissions with recurrent loss of consciousness, dizziness, and nausea to add to her already existing symptoms, a full biochemical and endocrine assessment was performed to look for more specific causes for her presentation. These pointed to a diagnosis of primary hyperparathyroidism. Conclusions. The inclusion of primary hyperparathyroidism in the differential diagnosis should be considered when evaluating paediatric patients presenting with nonspecific (neurological, gastrointestinal, and renal) symptoms in order to establish a prompt diagnosis of the disorder and to avoid severe complications of prolonged hypercalcaemia and end-organ damage.

Virchow-Robin spaces in idiopathic normal pressure hydrocephalus: a surrogate imaging marker for coexisting microvascular disease?

BACKGROUND: Virchow-Robin spaces (VRSs) surround perforating cerebral arteries and are reported to be found with increasing frequency with advancing age. In addition, some studies indicate an association between VRSs and vascular dementias. The present study examined the incidence of VRSs in patients with idiopathic normal pressure hydrocephalus (INPH) and considered their use as a potential surrogate imaging marker of coexisting microvascular disease in patients with this condition. METHODS: The MRI incidence of VRS in the centrum semiovale (CS), basal ganglia (BG), mesencephalon (MES), and the subinsular (SI) region was measured in 12 patients with INPH and in 12 control subjects, using the scoring system proposed by Patankar et al. (Am J Neuroradiology 26:1512, 2005). Historical control data were also used for further comparison. RESULTS: All 12 INPH patients had clearly visible VRSs, distributed in the CS (all 12), basal ganglia (11/12), SI region (9/12), and MES region (6/12). The mean Patankar scores of the INPH group were BG 2.25, CS 1.66, SI 0.91, and ME 0.5. The respective scores for our control group were 1.41, 1.5, 1.16, and 0.16, and for historical controls were 1.46, 0.51, 0.96, and 0.51. There were, however, no statistically significant differences between the INPH patients and either of the control groups. No correlation was found between age and the overall incidence of VRS. CONCLUSION: This preliminary study suggests that there may be a higher incidence of VRSs in patients with INPH, when compared with normal patients of similar age, but our small numbers prevent us from demonstrating statistical significance, and larger studies are clearly required.

Suppurative intracranial complications of sinusitis in adolescence. Single institute experience and review of literature.

The objective of this study was to investigate the outcome of neurosurgical treated children with suppurative intracranial complications (SIC) of sinusitis over the past 28 years in our hospital. We reviewed the cases notes of a series of 11 consecutive paediatric patients, who were subjected to surgery for sinusitis-induced SIC, retrospectively. Eleven children (10 males and only one female) were underwent neurosurgical procedure in our hospital between 1978 and 2006. Their age at the time of diagnosis ranging from 13 to 17 years (mean 15.27 years, SD 1.737). The commonest presenting symptoms were headaches (81.8%) followed by vomiting (45.5%) and swelling of the forehead (45.5%). The most often involved sinus was the frontal sinus (63.6%) and sinus surgery was performed in eight (72.72%) of 11 cases. The neurosurgical procedures carried out included burr hole drainage or aspiration of abscess in five cases, craniotomy and evacuation of empyema in seven cases and craniectomy in two cases. Four (36.4%) of 11 patients had more than one neurosurgical operation due to re-accumulation of pus and worsening of their clinical status. Most common pathogen was Streptococcus species (81.9%), and anaerobes were isolated in three (27.3%) cases. Postoperative antibiotic treatment lasted from 26 to 70 days (mean 45.45 days, SD 15.280). Epilepsy was diagnosed in two patients, postoperatively. During the follow-up period, persistent focal neurological deficits were present in five (45.5%) of 11 patients. Interestingly, five (45.45%) cases occurred over the last 2 years (2005-2006) and the other six over the previous 16 years (1978-2006). Prompt and aggressive medical and neurosurgical intervention is required, aiming to minimize the morbidity and mortality and also to maximize the favourable outcome of those children.

Outcome of children with posterior fossa medulloblastoma: a single institution experience over the decade 1994-2003.

AIM: While the impact of radiotherapy in the management of medulloblastoma was recognised, the introduction of chemotherapy was investigated in clinical trials and shown to confer an additional advantage. We reviewed the outcome of a series of consecutive patients to assess the impact in a population-based clinical establishment. MATERIALS AND METHODS: A series of 38 children treated for medulloblastoma at Birmingham Children's Hospital between 1994 and 2003 was analysed. The effect of surgery, radiotherapy, chemotherapy and metastasis on survival was analysed. RESULTS: The overall 5-year survival rate was 61.4% for the 36 patients who had resective surgery, while 2 patients had biopsy only and died within a few months. There was no operative mortality. The incidence of hydrocephalus needing permanent shunting was higher in the first 3 years of life (p = 0.007, chi-square). The 5-year survival rate of patients with total and sub-total excision of medulloblastoma was 61.1% and 61.8%, respectively. The 5-year survival rate of patients older than 3 years was 73.4% and for patients under 3 years was 36.3% (p = 0.007, log rank). Metastases at presentation did not influence survival. All deaths occurred in the first 32 months. CONCLUSION: The contribution of chemotherapy in the improvement of the overall survival appears more evident in children younger than 3 years or presenting with metastases. The absence of significant difference in survival between patients with total or sub-total excision of medulloblastoma supports the view that total excision of medulloblastoma can be avoided when the risk for potential intra-operative damage and consequent neurological deficits is high.

Assessment of the influence of operative factors in the success of endoscopic third ventriculostomy in children.

OBJECTIVE: To determine if operative factors correlate with success of endoscopic third ventriculostomy (ETV) in the treatment of hydrocephalus. MATERIALS AND METHODS: The ETV procedure video of 33 hydrocephalic children was reviewed. Mean age at operation was 76 months (range: 1-196). Success was defined as no need for shunt in the long term. We calculated the relative size of stoma as the percentage of stoma diameter to the distance between posterior clinoid-basilar artery. Factors analysed were: intra-operative haemorrhage, stoma size, thick/double third ventricular floor, pre-pontine adhesions presence, brisk cerebro-spinal fluid (CSF) flow through the stoma as well as hydrocephalus cause, previous shunt presence, CSF infection or haemorrhage and previous ETV. Analysis was performed using chi-square, linear regression, and one-way ANOVA. RESULTS: Overall ETV success rate was 42%. Mean stoma size was 37%. For the entire group, none of the operative factors correlated statistically with success. Previous shunt presence adversely correlated with success (p=0.008). The highest success rate was in the aqueduct stenosis group. In patients without previous shunt (n=17), stoma size over 30% tended towards significance (p=0.094), CSF leak was adversely associated with ETV success (p=0.041) and mean stoma size was 41.3% in successful ETV and 27.8% in unsuccessful ETV (p=0.072). In patients with previous shunt (n=16), thin third ventricular floor was a negative predisposing factor (p=0.057). CONCLUSION: This study did not demonstrate a correlation between the presence of pre-pontine adhesions, double or thickened floor of third ventricle and ETV success. In patients without previous shunt, stoma size may correlate with success. CSF leak was strongly associated with failure.

Infantile clivus chordoma without clivus involvement: case report and review of the literature.

BACKGROUND: We present a giant clival chordoma with disseminated disease but without involvement of the clivus. To our knowledge, this is the youngest child and only the second case, presenting without base of skull involvement, in paediatric literature and the fourth reported case of a chordoma in a patient with tuberous sclerosis. DISCUSSION: We discuss the subtle presentation, difficulties in diagnosis and management and also review the literature.