Quality of Life at a 10-Year Follow-Up of Children Born Preterm with Post-Hemorrhagic Ventricular Dilatation: A Cohort Study.

BACKGROUND: Post-haemorrhagic ventricular dilatation (PHVD) is commonly seen in extremely preterm babies, carries significant morbidity, and may cause neonatal mortality. There is a lack of literature on the subsequent health-related quality of life (HRQoL) in childhood. The aim of this work was to assess the quality of life of preterm babies after PHVD at 10 years of age using two validated questionnaires. METHODS: Children with PHVD were assessed as part of the 10-year follow-up of the drainage, irrigation, and fibrinolytic therapy trial. The HRQoL outcome was measured using parent-reported EQ-5D-5L and HUI-3 questionnaires. Both questionnaires produce a summary score anchored at 1 (best health) and 0 (equivalent to death). RESULTS: Median scores at follow-up were 0.65 (IQR 0.36-0.84; n = 44) for the EQ-5D-5L and 0.52 (IQR 0.22-0.87; n = 51) for the HUI-3. Similar proportions had a score below 0.2 (HRQoL [20%], HUI-3 [21%]), while 20% had a HRQoL score above 0.80 compared to 34% using HUI-3. The most severe problems from the EQ-5D-5L were reported in the self-care, mobility, and activity domains, while the HUI-3 reported worse problems in ambulation, cognition, and dexterity domains. Infants with worse (grade 4) intraventricular haemorrhage had poorer HRQoL than those with grade 3 bleeds. CONCLUSION: Children who survive to 10 years of age after PHVD have on average lower HRQoL than their peers. However, the reported range is wide, with a quarter of the children having scores above 0.87 (similar to population norms), while a fifth have very low HRQol scores. Impact was not uniform across domains, with mobility/ambulation a concern across both measures.

Vision function in children 10 years after grade 3 or 4 intraventricular haemorrhage with ventricular dilation: A masked prospective study.

AIM: We examined children 10 to 11 years after grade 3 or 4 intraventricular haemorrhage and ventricular dilation (IVHVD) and investigated whether the grade of IVHVD affected their visual outcome. We explored associations between visual outcomes with cognitive outcomes and extra support at school. METHOD: The visual examinations were part of a 10-year follow-up study for children in a randomized trial. Testers followed a protocol and were masked to whether the child had experienced grade 3 or grade 4 IVHVD and all other data. RESULTS: Thirty-two children were tested: 24 were male and mean (standard deviation) age was 10 years 5 months (1 year 2 months); range 8 years 9 months to 12 years 9 months. All had at least one visual impairment. The median (interquartile range) number of impairments per child was six (six to nine) for children who experienced a grade 4 IVHVD compared with three (two to four) for children who experienced a grade 3 IVHVD (p = 0.003). Each extra vision impairment per child was associated with increased educational support at school, after adjustment for developmental age equivalence (odds ratio = 1.7 [95% confidence interval 1.1-2.6], p = 0.015). INTERPRETATION: Children who experience grade 3 or 4 IVHVD have a high level of visual morbidity at age 10 to 11 years. These children may have unmet visual needs and their outcomes might improve if these needs could be addressed. WHAT THIS PAPER ADDS: Parent-reported questionnaire responses underestimated directly assessed visual morbidity. Grade 4 intraventricular haemorrhage and ventricular dilatation (IVHVD) was followed by more vision impairments than grade 3 IVHVD. Simple tests of visual perceptual skills correlated with the neuropsychology tests. Children with supranuclear eye movement disorders were more likely to be receiving extra help at school. Each additional visual impairment increased the likelihood of extra educational support.

Presenting symptoms and time to diagnosis for Pediatric Central Nervous System Tumors in Qatar: a report from Pediatric Neuro-Oncology Service in Qatar.

INTRODUCTION: There are no previous published reports on primary pediatric tumors of the central nervous system (CNS) in Qatar. We undertook this retrospective cohort study to review the diagnosis of CNS tumors in children in Qatar to analyze the presentation characteristics including symptoms, referral pathways, and time to diagnosis. METHODS: All children registered with Pediatric Neuro-Oncology service (PNOS) were included in the study. Data from the time of diagnosis (October 2007 to February 2020) were reviewed retrospectively. Presenting symptoms were recorded and pre-diagnosis symptom interval (PSI) was calculated from the onset of the first symptom to the date of diagnostic imaging. RESULTS: Of the 61 children registered with PNOS during the study period, 51 were included in the final analysis. Ten children were excluded because they were either diagnosed outside Qatar (n = 7) or were asymptomatic at the time of diagnosis (n = 3). The median age was 45 (range 1-171) months. Common tumor types included low-grade glioma (LGG) (47.1%) and medulloblastoma/primitive neuro-ectodermal tumors (PNET) (23.5%). Nine children had an underlying neurocutaneous syndrome. Thirty-eight patients (74.5%) had at least one previous contact with healthcare (HC) professional, but 27 (52%) were still diagnosed through the emergency department (ED). Presenting symptoms included headache, vomiting (36.2%), oculo-visual symptoms (20.6%), motor weakness (18.9%), seizures, ataxia (17.2% each), irritability, cranial nerve palsies (12% each), and endocrine symptoms (10.3%). Median PSI was 28 days (range 1-845 days) for all CNS tumors. Longest PSI was seen with germ cell tumors (median 146 days), supratentorial location (39 days), and age above 3 years (30 days). Tumor characteristics of biological behavior (high-grade tumor) and location (infratentorial) were significantly associated with shorter PSI, as were presenting symptoms of ataxia, head tilt, and altered consciousness. CONCLUSIONS: Although overall diagnostic times were acceptable, some tumor types were diagnosed after a significant delay. The awareness campaign, such as the "HeadSmart" campaign in the United Kingdom (UK), can improve diagnostic times in Qatar. Further research is required to better understand the reasons for the delay.

Drainage, irrigation and fibrinolytic therapy (DRIFT) for posthaemorrhagic ventricular dilatation: 10-year follow-up of a randomised controlled trial.

BACKGROUND: Progressive ventricular dilatation after intraventricular haemorrhage (IVH) in preterm infants has a very high risk of severe disability and death. Drainage, irrigation and fibrinolytic therapy (DRIFT), in a randomised controlled trial (RCT), reduced severe cognitive impairment at 2 years. OBJECTIVE: To assess if the cognitive advantage of DRIFT seen at 2 years persisted until school age. PARTICIPANTS: The RCT conducted in four centres recruited 77 preterm infants with IVH and progressive ventricular enlargement over specified measurements. Follow-up was at 10 years of age. INTERVENTION: Intraventricular injection of a fibrinolytic followed by continuous lavage, until the drainage was clear, and standard care consisting of control of expansion by lumbar punctures and if expansion persisted via a ventricular access device. PRIMARY OUTCOME: Cognitive quotient (CQ), derived from the British Ability Scales and Bayley III Scales, and survival without severe cognitive disability. RESULTS: Of the 77 children randomised, 12 died, 2 could not be traced, 10 did not respond and 1 declined at 10-year follow-up. 28 in the DRIFT group and 24 in the standard treatment group were assessed by examiners blinded to the intervention. The mean CQ score was 69.3 (SD=30.1) in the DRIFT group and 53.7 (SD=35.7) in the standard treatment group (unadjusted p=0.1; adjusted p=0.01, after adjustment for the prespecified variables sex, birth weight and IVH grade). Survival without severe cognitive disability was 66% in the DRIFT group and 35% in the standard treatment group (unadjusted p=0.019; adjusted p=0.003). CONCLUSION: DRIFT is the first intervention for posthaemorrhagic ventricular dilatation to objectively demonstrate sustained cognitive improvement. TRIAL REGISTRATION NUMBER: ISRCTN80286058.

Ten-year follow-up of a randomised trial of drainage, irrigation and fibrinolytic therapy (DRIFT) in infants with post-haemorrhagic ventricular dilatation.

BACKGROUND: The drainage, irrigation and fibrinolytic therapy (DRIFT) trial, conducted in 2003-6, showed a reduced rate of death or severe disability at 2 years in the DRIFT compared with the standard treatment group, among preterm infants with intraventricular haemorrhage (IVH) and post-haemorrhagic ventricular dilatation. OBJECTIVES: To compare cognitive function, visual and sensorimotor ability, emotional well-being, use of specialist health/rehabilitative and educational services, neuroimaging, and economic costs and benefits at school age. DESIGN: Ten-year follow-up of a randomised controlled trial. SETTING: Neonatal intensive care units (Bristol, Katowice, Glasgow and Bergen). PARTICIPANTS: Fifty-two of the original 77 infants randomised. INTERVENTIONS: DRIFT or standard therapy (cerebrospinal fluid tapping). MAIN OUTCOME MEASURES: Primary - cognitive disability. Secondary - vision; sensorimotor disability; emotional/behavioural function; education; neurosurgical sequelae on magnetic resonance imaging; preference-based measures of health-related quality of life; costs of neonatal treatment and of subsequent health care in childhood; health and social care costs and impact on family at age 10 years; and a decision analysis model to estimate the cost-effectiveness of DRIFT compared with standard treatment up to the age of 18 years. RESULTS: By 10 years of age, 12 children had died and 13 were either lost to follow-up or had declined to participate. A total of 52 children were assessed at 10 years of age (DRIFT, n = 28; standard treatment, n = 24). Imbalances in gender and birthweight favoured the standard treatment group. The unadjusted mean cognitive quotient (CQ) score was 69.3 points [standard deviation (SD) 30.1 points] in the DRIFT group compared with 53.7 points (SD 35.7 points) in the standard treatment group, a difference of 15.7 points, 95% confidence interval (CI) -2.9 to 34.2 points; p = 0.096. After adjusting for the prespecified covariates (gender, birthweight and grade of IVH), this evidence strengthened: children who received DRIFT had a CQ advantage of 23.5 points (p = 0.009). The binary outcome, alive without severe cognitive disability, gave strong evidence that DRIFT improved cognition [unadjusted odds ratio (OR) 3.6 (95% CI 1.2 to 11.0; p = 0.026) and adjusted OR 10.0 (95% CI 2.1 to 46.7; p = 0.004)]; the number needed to treat was three. No significant differences were found in any secondary outcomes. There was weak evidence that DRIFT reduced special school attendance (adjusted OR 0.27, 95% CI 0.07 to 1.05; p = 0.059). The neonatal stay (unadjusted mean difference £6556, 95% CI -£11,161 to £24,273) and subsequent hospital care (£3413, 95% CI -£12,408 to £19,234) costs were higher in the DRIFT arm, but the wide CIs included zero. The decision analysis model indicated that DRIFT has the potential to be cost-effective at 18 years of age. The incremental cost-effectiveness ratio (£15,621 per quality-adjusted life-year) was below the National Institute for Health and Care Excellence threshold. The cost-effectiveness results were sensitive to adjustment for birthweight and gender. LIMITATIONS: The main limitations are the sample size of the trial and that important characteristics were unbalanced at baseline and at the 10-year follow-up. Although the analyses conducted here were prespecified in the analysis plan, they had not been prespecified in the original trial registration. CONCLUSIONS: DRIFT improves cognitive function when taking into account birthweight, grade of IVH and gender. DRIFT is probably effective and, given the reduction in the need for special education, has the potential to be cost-effective as well. A future UK multicentre trial is required to assess efficacy and safety of DRIFT when delivered across multiple sites. TRIAL REGISTRATION: Current Controlled Trials ISRCTN80286058. FUNDING: This project was funded by the NIHR Health Technology Assessment programme and will be published in full in Health Technology Assessment; Vol. 23, No. 4. See the NIHR Journals Library website for further project information. The DRIFT trial and 2-year follow-up was funded by Cerebra and the James and Grace Anderson Trust.

Surgical Outcomes of Single-Level Bilateral Selective Dorsal Rhizotomy for Spastic Diplegia in 150 Consecutive Patients.

OBJECTIVES: Selective dorsal rhizotomy (SDR) is used to improve spasticity, gait, and pain in children with spastic diplegia. There is growing evidence supporting its long-term benefits in terms of functional outcomes, independence, and quality of life. There is, however, little contemporary work describing the surgical morbidity of this irreversible procedure. The purpose of this study is to evaluate the surgical outcomes and complications of SDR at a single United Kingdom center. METHODS: Demographics, surgical, postoperative, and follow-up data for all patients undergoing SDR between 2011 and 2016 were collected from medical records. RESULTS: Preoperative Gross Motor Function Classification System levels in 150 consecutive patients were II (35%), III (65%), and IV (1%). Median age was 6 years and 58% were male patients. There were no deaths, cerebrospinal fluid leaks, returns to theater, or readmissions within 30 days. There were no new motor or sphincter deficits. Postoperative neuropathic pain was reported by 5.3% and sensory symptoms by 8.7%. Other complications included: postoperative nausea and vomiting (19.3%), superficial wound infection (3.3%), urinary retention (1.3%), headache (6.7%), and urine or chest infection (4.7%). Follow-up data were available for all patients (93% to 12 months, 72% to 24 months). Persistent neuropathic symptoms were reported in 6.5% at 24 months. CONCLUSIONS: SDR using a single-level approach is a safe procedure with low surgical morbidity. This study complements the growing evidence base in support of SDR for spastic diplegia and should help inform decisions when considering treatment options.

The outcome of surgical management of subependymal giant cell astrocytoma in tuberous sclerosis complex.

OBJECTIVES: The indications for surgery and outcomes of patients who underwent surgical removal of subependymal giant cell astrocytomas (SEGAs) in our institution between 2000 and 2011 were reviewed. METHODS: We reviewed the clinical details of 16 patients with a diagnosis of Tuberous Sclerosis Complex (TSC) who underwent surgery for SEGA in Bristol since 2000. We collected information on age, sex, epilepsy history and cognitive status. We reviewed the indications for surgery, age at surgery, surgical approach, and the size and location of the lesions. We analysed mortality, completeness of tumour resection, intraoperative blood transfusion, shunt placements, and surgical complications. RESULTS: 13 patients had surgery due to hydrocephalus. Increasing size of SEGA without hydrocephalus was an indication for surgery in two patients, and in one patient, the SEGA was removed because of its size and location at initial scan. 13 patients had complete tumour resection. One patient had tumour recurrence. Hydrocephalus failed to resolve or reoccurred in four patients post operatively necessitating shunt insertion. The surgical approach was transcortical in 14 patients and transcallosal in two. There was zero mortality in this series. There were no reports of cognitive decline or worsening epilepsy following surgery. CONCLUSION: Surgery is a safe and effective treatment for SEGA. It is the authors' view that surgery remains the most appropriate treatment strategy for SEGAs that are amenable to surgery. More work needs to be undertaken to assess prospectively the neurocognitive impact of surgery, and the relative advantages of different surgical approaches.

Comparison of infection rate with the use of antibiotic-impregnated vs standard extraventricular drainage devices: a prospective, randomized controlled trial.

BACKGROUND: External ventricular drainage (EVD) catheters provide reliable and accurate means of monitoring intracranial pressure and alleviating elevated pressures via drainage of cerebrospinal fluid (CSF). CSF infections occur in approximately 9% of patients. Antibiotic-impregnated (AI) EVD catheters were developed with the goal of reducing the occurrence of EVD catheter-related CSF infections and their associated complications. OBJECTIVE: To present an international, prospective, randomized, open-label trial to evaluate infection incidence of AI vs standard EVD catheters. METHODS: Infection was defined as (1) proven infection, positive CSF culture and positive Gram stain or (2) suspected infection: (A) positive CSF culture with no organisms identified on initial Gram stain; (B) negative CSF culture with a gram-positive or -negative stain; (C) CSF leukocytosis with a white blood cell/red blood cell count >0.02. RESULTS: Four hundred thirty-four patients underwent implantation of an EVD catheter. One hundred seventy-six patients in the AI-EVD cohort and 181 in the standard EVD catheter cohort were eligible for evaluation of infection. The 2 groups were similar in all clinical characteristics. Proven infection was documented in 9 (2.5%) patients (AI: 4 [2.3%] vs standard: 5 [2.8%], P = 1.0). Suspected infection was documented in 31 (17.6%) patients receiving AI and 37 (20.4%) patients receiving standard EVD catheters, P = .504. Duration of time to suspected infection was prolonged in the AI cohort (8.8 ± 6.1 days) compared with the standard EVD cohort (4.6 ± 4.2 days), P = .002. CONCLUSION: AI-EVD catheters were associated with an extremely low rate of catheter-related infections. AI catheters were not associated with risk reduction in EVD infection compared to standard catheters. Use of AI-EVD catheters is a safe option for a wide variety of patients requiring CSF drainage and monitoring, but the efficacy of AI-EVD catheters was not supported in this trial.

Spinal cord arterio-venous malformations in children: a rare but important diagnosis.

Spinal cord arterio-venous malformations (AVMs) are rare and the diagnosis is often delayed or missed. We describe two cases presenting in different ways, as subarachnoid haemorrhage (SAH) or myelopathy. A catheter angiogram confirmed the diagnosis following which they were treated with coil occlusion or embolisation, or a combination of both, without any complications.

The constant flow ventricular infusion test: a simple and useful study in the diagnosis of third ventriculostomy failure.

OBJECT: The evaluation of third ventriculostomy function in hydrocephalic patients is challenging. The utility of the constant flow infusion test in predicting response to shunt insertion in normal-pressure hydrocephalus, as well as in identifying shunt malfunction, has been previously demonstrated. The object of this study was to evaluate its usefulness in determining whether a revision CSF diversion procedure was indicated in patients presenting with recurring symptoms and persisting ventriculomegaly after endoscopic third ventriculostomy (ETV). METHODS: The authors conducted a prospective study of all patients who, after undergoing ETV at their institution, presented postoperatively with recurring symptoms and persisting ventriculomegaly. RESULTS: Forty-six patients (mean age 40.7 years, including 11 patients younger than 18 years) underwent 56 constant flow ventricular infusion tests (VITs) at a mean of 24.7 months post-ETV. Thirty-three patients with resistance to CSF outflow (R(out)) less than 13 mm Hg/ml/min underwent follow-up (median 17 months) and experienced resolution of symptoms. In 10 episodes R(out) was greater than 13 mm Hg/ml/min; the patients in these cases underwent revisional CSF diversion. Two patients demonstrated high and frequent B (slow) waves despite a low R(out); these patients also underwent successful revisions. Patients who improved after surgery had increased B wave activity in the plateau phase of the VIT (p = 0.01). Thirty-four patients underwent MR imaging at the same time; 4 had high R(out) despite evidence of flow across the stoma. These 4 patients underwent surgery and experienced resolution of symptoms. Of 9 patients without flow, R(out) was less than 13 mm Hg/ml/min in 4; these patients were successfully treated conservatively. CONCLUSIONS: The VIT is a useful and safe adjunct to clinical and MR imaging evaluation when ETV failure is suspected.

Preliminary evaluation of a novel intraparenchymal capacitive intracranial pressure monitor.

OBJECT: Intracranial pressure (ICP) monitors are currently based on fluid-filled, strain gauge, or fiberoptic technology. Capacitive sensors have minimal zero drift and energy requirements, allowing long-term implantation and telemetric interrogation; their application to neurosurgery has only occasionally been reported. The aim of this study was to undertake a preliminary in vitro and in vivo evaluation of a capacitive telemetric implantable ICP monitor. METHODS: Four devices were tested in air- and saline-filled pressure chambers; long-term capacitance-pressure curves were obtained. Devices implanted in a gel phantom and in a piglet were placed in a 3-T MR unit to evaluate MR compatibility. Four devices were implanted in a piglet neonatal hydrocephalus model; output was compared with ICP obtained through fluid-filled transduction and a strain-gauge ICP monitor. RESULTS: The capacitance-pressure relationship was constant over 4 weeks, suggesting minimal zero drift during this period. There were no temperature changes around the monitor. Signal loss at the sensor was minimal in both the phantom and the piglet. Over 114,000 measurements were obtained; the difference between mean capacitive ICP and fluid-transduced ICP was 1.8 ± 1.42 mm Hg. The correlation between ICP from the capacitive sensor and fluid-filled transducer (r = 0.97, p < 0.0001) or strain-gauge monitor (r = 0.99, p < 0.0001) was excellent. In vivo monitoring was restricted to 48 hours due to problems with robustness in the clinical environment. CONCLUSIONS: This preliminary study demonstrates minimal long-term zero drift in vitro, good MR compatibility, and good correlation with other methods of ICP monitoring in vivo in the short term. Further long-term in vivo study is required.

Randomized trial of drainage, irrigation and fibrinolytic therapy for premature infants with posthemorrhagic ventricular dilatation: developmental outcome at 2 years.

BACKGROUND: Preterm infants who develop posthemorrhagic ventricular dilatation (PHVD) have a high risk of cognitive and motor disability. No clinical intervention has been proven to reduce neurodevelopmental disability in such infants. We investigated whether drainage, irrigation, and fibrinolytic therapy (DRIFT), which aims to lower pressure, distortion, free iron, and cytokines, reduces death or severe disability in PHVD. METHODS: We randomly assigned 77 preterm infants with PHVD to either DRIFT or standard treatment (ie tapping off cerebrospinal fluid to control excessive expansion). Severe disability was assessed at 2 years' corrected age and included severe sensorimotor disability and cognitive disability (<55 on the Bayley Mental Development Index). RESULTS: Of 39 infants assigned to DRIFT, 21 (54%) died or were severely disabled versus 27 of 38 (71%) in the standard group (adjusted odds ratio 0.25 [95% confidence interval: 0.08-0.82]). Among the survivors, 11 of 35 (31%) in the DRIFT group had severe cognitive disability versus 19 of 32 (59%) in the standard group (adjusted odds ratio: 0.17 [95% confidence interval: 0.05-0.57]). Median Mental Development Index was 68 with DRIFT and <50 with standard care. Severe sensorimotor disability was not significantly reduced. CONCLUSIONS: Despite an increase in secondary intraventricular bleeding, DRIFT reduced severe cognitive disability in survivors and overall death or severe disability.

Prognosis of spina bifida in the era of prenatal diagnosis and termination of pregnancy.

OBJECTIVE: To evaluate the current outcome of a selected prenatally diagnosed spina bifida group. MATERIALS AND METHODS: We analyzed and followed up 74 cases of prenatally diagnosed spina bifida. RESULTS: Termination of pregnancy was chosen in 72% of the cases and 28% were live-born. Chromosomal defects were identified in 16%, although only 1.6% in isolated cases. Of the 21 live births, 3 died in the neonatal period. The other 18 (86%) were all alive after an average follow-up of 3 years and 6 months (range 5 months to 7 years and 4 months). From this group 11% are wheelchair-dependent, 87% of the patients older than 2 years of age are walking, 33% have had cerebral shunting and 72% have normal neurodevelopment. There was a better outcome in patients with closed defects; however, the rates of neuropathic bladder (50%) remain a concern. CONCLUSIONS: Even with prenatal diagnosis and a tendency towards apparently less severe defects in the cases in which the pregnancies continue, the prognosis in terms of morbidity needs to remain guarded.

Conservative management of patients with cerebrospinal fluid shunt infections.

OBJECTIVE: In patients with cerebrospinal fluid (CSF) shunt infection, removal of the shunt and antibiotic administration is the current standard of care. In 1986, we developed a protocol for the conservative management of patients with infected but functioning shunts. Treatment was based on the administration of a combination of intraventricular and systemic antibiotics. Intraventricular antibiotics were instilled via a separate access device. The purpose of this report is to describe our experience with this therapeutic intervention. METHODS: An observational study of all patients treated for CSF shunt infection between 1986 and 2003 was undertaken. Cure was defined by sterile CSF after completion of therapy and sterile shunt components at next revision or long-term freedom from recurrent infection (follow-up period, 6-88 mo). RESULTS: In total, 43 of 122 patients with CSF shunt infections were treated conservatively according to our protocol. Overall, 84% of these patients were cured, with a 92% success rate for patients with infections caused by bacteria other than Staphylococcus aureus. This included 30 coagulase-negative staphylococcal infections, of which two were treatment failures. We abandoned conservative treatment of patients with Staphylococcus aureus infections after early experience demonstrated that the success rate (four treatment failures in seven patients) was markedly lower than that for other pathogens. During the treatment and follow-up periods, there were three deaths, two of which were unrelated to shunt infection; treatment failure could not be completely excluded in the remaining patient. There was no toxicity related to intraventricular antibiotic administration. The incidence of shunt blockage among patients who were treated conservatively was not significantly different from that among a large cohort of patients with uninfected shunts. Ten patients received part of their courses of treatment as outpatients. CONCLUSION: The success rate of conservative management of patients with CSF shunt infections caused by coagulase-negative staphylococci is comparable with those in the published literature for patients treated conventionally. This form of management avoids surgical intervention, with its attendant risks, and is safe.

Obstructive sleep apnea syndrome with bilateral papilledema and vision loss in a 3-year-old child.

We describe bilateral papilledema and vision loss in a 3-year-old child with obstructive sleep apnea. Although lumbar puncture initially disclosed a normal opening pressure, cerebrospinal fluid (CSF) pressure monitoring during sleep confirmed intermittent episodes of elevated intracranial pressure corresponding to increased airway resistance. The association of obstructive sleep apnea and raised intracranial pressure is recognized in children with craniosynostosis but has not been reported in its absence.

Randomized clinical trial of prevention of hydrocephalus after intraventricular hemorrhage in preterm infants: brain-washing versus tapping fluid.

OBJECTIVE: Hydrocephalus is a serious complication of intraventricular hemorrhage in preterm infants, with adverse consequences from permanent ventriculoperitoneal shunt dependence. The development of hydrocephalus takes several weeks, but no clinical intervention has been shown to reduce shunt surgery in such infants. The aim of this study was to test a new treatment intended to prevent hydrocephalus and shunt dependence after intraventricular hemorrhage. METHODS: We randomly assigned 70 preterm infants who had gestational ages of 24 to 34 weeks and were progressively enlarging their cerebral ventricles after intraventricular hemorrhage to either (1) drainage, irrigation, and fibrinolytic therapy to wash out blood and cytokines or (2) tapping of cerebrospinal fluid by reservoir as required to control excessive expansion and signs of pressure (standard treatment). We evaluated outcomes at 6 months of age or hospital discharge (if later). RESULTS: Of 34 infants who were assigned to drainage, irrigation, and fibrinolytic therapy, 2 died and 13 underwent shunt surgery (dead or shunt: 44%). Of 36 infants who were assigned to standard therapy, 5 died and 14 underwent shunt surgery (dead or shunt: 50%). This difference was not significant. Twelve (35%) of 34 infants who received drainage, irrigation, and fibrinolytic therapy had secondary intraventricular hemorrhage compared with 3 (8%) of 36 in the standard group. Secondary intraventricular hemorrhage was associated with an increased risk for subsequent shunt surgery and more blood transfusions. CONCLUSIONS: Despite its logical basis and encouraging pilot data, drainage, irrigation, and fibrinolytic therapy did not reduce shunt surgery or death when tested in a multicenter, randomized trial. Secondary intraventricular hemorrhage is a major factor that counteracts any possible therapeutic effect from washing out old blood.

Conservative management of patients with cerebrospinal fluid shunt infections.

OBJECTIVE: In patients with cerebrospinal fluid (CSF) shunt infection, removal of the shunt and antibiotic administration is the current standard of care. In 1986, we developed a protocol for the conservative management of patients with infected but functioning shunts. Treatment was based on the administration of a combination of intraventricular and systemic antibiotics. Intraventricular antibiotics were instilled via a separate access device. The purpose of this report is to describe our experience with this therapeutic intervention. METHODS: An observational study of all patients treated for CSF shunt infection between 1986 and 2003 was undertaken. Cure was defined by sterile CSF after completion of therapy and sterile shunt components at next revision or long-term freedom from recurrent infection (follow-up period, 6-88 mo). RESULTS: In total, 43 of 122 patients with CSF shunt infections were treated conservatively according to our protocol. Overall, 84% of these patients were cured, with a 92% success rate for patients with infections caused by bacteria other than Staphylococcus aureus. This included 30 coagulase-negative staphylococcal infections, of which two were treatment failures. We abandoned conservative treatment of patients with Staphylococcus aureus infections after early experience demonstrated that the success rate (four treatment failures in seven patients) was markedly lower than that for other pathogens. During the treatment and follow-up periods, there were three deaths, two of which were unrelated to shunt infection; treatment failure could not be completely excluded in the remaining patient. There was no toxicity related to intraventricular antibiotic administration. The incidence of shunt blockage among patients who were treated conservatively was not significantly different from that among a large cohort of patients with uninfected shunts. Ten patients received part of their courses of treatment as outpatients. CONCLUSION: The success rate of conservative management of patients with CSF shunt infections caused by coagulase-negative staphylococci is comparable with those in the published literature for patients treated conventionally. This form of management avoids surgical intervention, with its attendant risks, and is safe.

Atypical external hydrocephalus with visual failure due to occult leptomeningeal dissemination of a pontine glioma. Case report.

The authors report on the case of a diffuse pontine glioma in a 5-year-old boy in whom radiologically and cytologically occult leptomeningeal metastases led to the development of an atypical "external" hydrocephalus, associated with grossly elevated intracranial pressure (ICP). Initial neuroimaging demonstrated only mild communicating ventricular dilation associated with a noticeable enlargement of the subarachnoid space, particularly over the surface of the frontal lobes; these features are not usually associated with significantly elevated ICP. Possible pathophysiological mechanisms resulting in this unusual clinical presentation are discussed. Early recognition of the severity of the raised ICP despite the paucity of clinical and radiological signs may have averted the development of blindness due to optic atrophy.