Care and three-year outcomes of children with Benign Epilepsy with Centro-Temporal Spikes in England.

PURPOSE: Benign Epilepsy with Centro-Temporal Spikes (BECTS) is a pediatric epilepsy with typically good seizure control. Although BECTS may increase patients' risk of developing neurological comorbidities, their clinical care and short-term outcomes are poorly quantified. METHODS: We retrospectively assessed adherence to National Institute for Health and Care Excellence (NICE) guidelines relating to specialist referral, electroencephalogram (EEG) conduct and annual review in the care of patients with BECTS, and measured their seizure, neurodevelopmental and learning outcomes at three years post-diagnosis. RESULTS: Across ten centers in England, we identified 124 patients (74 male) diagnosed with BECTS between 2015 and 2017. Patients had a mean age at diagnosis of 8.0 (95% CI = 7.6-8.4) years. 24/95 (25%) patients were seen by a specialist within two weeks of presentation; 59/100 (59%) received an EEG within two weeks of request; and 59/114 (52%) were reviewed annually. At three years post-diagnosis, 32/114 (28%) experienced ongoing seizures; 26/114 (23%) had reported poor school progress; 15/114 (13%) were diagnosed with a neurodevelopmental disorder (six autism spectrum disorder, six attention-deficit/hyperactivity disorder); and 10/114 (8.8%) were diagnosed with a learning difficulty (three processing deficit, three dyslexia). Center-level random effects models estimated neurodevelopmental diagnoses in 9% (95% CI: 2-16%) of patients and learning difficulty diagnoses in 7% (95% CI: 2-12%). CONCLUSIONS: In this multicenter work, we found variable adherence to NICE guidelines in the care of patients with BECTS and identified a notable level of neurological comorbidity. Patients with BECTS may benefit from enhanced cognitive and behavioral assessment and monitoring.

CSF diversion in refractory idiopathic intracranial hypertension: single-centre experience and review of efficacy.

PURPOSE: The current surgical management strategies for refractory cases of idiopathic intracranial hypertension (IIH) remain unresolved. We evaluated the outcome of our paediatric patients who were offered a CSF diversion procedure in order to control their symptoms. METHODS: We retrospectively reviewed the medical notes of the patients under 16 years of age, who presented in our centre from 2005 to 2010, with a confirmed diagnosis of IIH, and ultimately had a lumboperitoneal shunt (LPS). We describe their immediate postoperative course, shunt-related complications and recent outcome. RESULTS: Seven patients presented at a mean age of 8.7 years. Two presented with significant visual loss and had a shunt acutely; the remaining five presented with headaches and were shunted within 2 years. In the immediate postoperative period two patients experienced low-pressure symptoms. All patients required shunt revisions; in total 15 revisions took place, mainly secondary to symptomatic overdrainage or obstruction. After a mean follow-up of 26 months, two patients have diminished visual acuity at least on one side; only one patient became headache-free, despite resolution of the CSF pressure post diversion in four out of the six remaining patients. CONCLUSIONS: All patients required shunt revisions and 6/7 (85.7%) had persisting headaches at their last follow-up. It is apparent that once functional, the LPS seems adequate to lower the CSF pressure but not effective in eliminating symptoms.

Outcome of CDH infants following fetoscopic tracheal occlusion - influence of premature delivery.

PURPOSE: To evaluate the mortality and morbidity of infants with congenital diaphragmatic hernia who had undergone fetal endoscopic tracheal occlusion (FETO) and whether this was influenced by premature birth. METHODS: The gestational age at delivery, lung-head ratio (LHR) pre and post FETO, neonatal outcomes, and respiratory, gastro-intestinal, neurological, surgical, and musculoskeletal problems at follow up of consecutive infants who had undergone FETO were determined. Elective reversal of FETO was planned at 34 weeks of gestation. RESULTS: The survival rate of the 61 FETO infants was 48%, with 84% delivered prematurely. Thirty-one delivered <35 weeks of gestation. Their survival rate was 18%. Twenty-three of 24 infants who had emergency balloon removal were born <35 weeks of gestation. Survival was related to gestational age at delivery (OR 0.55, 95% CI 0.420, 0.77, p<0.001) and the duration of FETO (OR 0.73, 95% CI 0.59, 0.91, p<0.005). Infants born prior to 35 weeks of gestation compared to those born at ≥ 35 weeks required a longer duration of ventilation (median 45 days versus 12 days, p<0.001), and a greater proportion had surgery for gastro-oesophageal reflux (50% versus 9%, p=0.011). CONCLUSION: These results emphasize the need to reduce premature delivery following FETO.