Congenital hydrocephalus in a trisomy 9p gained child:a case report

BACKGROUND: Hydrocephalus caused by excessive liquor production due to choroid plexus hyperplasia is a rare condition that may necessitate unusual treatment paradigms. It can be seen in trisomy 9p where coexisting congenital heart disease additionally may complicate the therapeutic approach as illustrated in the current case report. CASE PRESENTATION: At 20 months of age, a Caucasian girl with trisomy 9 and family history of an older brother and twin sister having the same syndrome displayed sign of congenital hydrocephalus due to increasing head circumference. Magnetic resonance imaging revealed enlarged lateral ventricles and a prominent choroid plexus, and the girl was treated with a ventriculoperitoneal shunt, which 2 days later had to be replaced with a ventriculoatrial shunt as cerebrospinal fluid formation greatly exceeded the ability of the patient’s abdominal absorptive capability. At 16 years of age, the patient was diagnosed with cardiomyopathy and diminished ejection fraction. Some months later, she was admitted to the neurosurgical ward showing signs of shunt dysfunction due to a colloid cyst in the third ventricle. Cystic drainage through endoscopic puncture only helped temporarily. Revision of the shunt system showed occlusion of the ventricular drain, and replacement was merely temporary alleviating. Intracerebral pressure was significantly increased at around 30 mmHg, prompting externalization of the drain, and measurements revealed high cerebrospinal fluid production of 60–100 ml liquor per hour. Thus, endoscopic choroid plexus coagulation was performed bilaterally leading to an immediate decrease of daily cerebrospinal fluid formation to 20–30 ml liquor per hour, and these values where stabilized by pharmaceutical treatment with acetazolamide 100 mg/kg/day and furosemide 1 mg/kg/day. Subsequently a ventriculoperitoneal shunt was placed. Follow-up after 1 and 2 months displayed no signs of hydrocephalus or ascites. CONCLUSIONS: High cerebrospinal fluid volume load and coexisting heart disease in children with trisomy 9p may call for endoscopic choroid plexus coagulation and pharmacological therapy to diminish the daily cerebrospinal fluid production to volumes that allow proper ventriculoperitoneal shunting

Endoscopic removal of lumbar disc herniation

The technical development has caused a reintroduction of endoscopic techniques directed towards degenerative spine disease. A summary of the endoscopic procedure is given in this review. The spinal canal is reached through an inter-laminar or transforaminal access. In comparison with open surgery the percutaneous transforaminal access seems especially advantageous for the removal of paramedian and/or foraminal herniated disc material. However, careful patient selection is required, as the restricted manoeuverability and working zone of the endoscope and patient specific pathoanatomy in some cases will disfavour endoscopy.</p

Study protocol for ACTIVE study:safety and feasibility evaluation of external ventricular drainage with ACTIVE fluid exchange in intraventricular hemorrhage-a phase 2, multi-center, randomized controlled trial

Background: Primary intraventricular hemorrhage (IVH) or IVH secondary to intracerebral (ICH) and subarachnoid hemorrhage (SAH) are known to have a very poor prognosis, with an expected mortality between 50 and 80% (Hinson et al. Current Neurology and Neuroscience Reports 10:73–82, 2010). Clearance of IVH might improve patient outcome. Methods: The study is designed as an investigator-initiated, comparative, prospective, multi-center, 1:1 randomized phase 2 trial evaluating the efficacy and safety of active irrigation in external ventricular drainage (intervention arm—IRRAflow) compared to passive external ventricular drainage (control arm—EVD). The trial will enroll 58 patients with primary or secondary IVH. Major eligibility criteria include age ≥18 years of age, IVH documented on head CT or MRI scan (Graeb score ≥3), need of cerebrospinal fluid drainage, deterioration of consciousness or medical sedation at the time of enrollment, and indication for active treatment evaluated by the treating physicians. Exclusion criteria included patients with fixed and dilated pupils and pregnant or nursing women. The primary endpoint of the study is catheter occlusion evaluated by time to first observed occlusion from VC placement. Secondary endpoints include clearance of ventricular blood as measured by head CT scan, rates of catheter-related infection and shunt dependency, length of intensive care unit stay, functional status—Extended Glascow Outcome Scale (eGOS) and modified Rankin scale (mRS) at discharge to rehabilitation and 90 days—and mortality rates at 30 days and 90 days. Discussion: With no standardized treatment for IVH and a poor prognosis, new treatments are needed. IVH patients often need CSF drainage to treat hydrocephalus and to decrease ICP. Standard treatment with passive external ventricular drainage is related to an increased risk of infections which is found in up to 22% of treated cases. The passive VC is known to have a risk of occlusion and is seen in 19–47% of the cases. We hypothesize that the use of active fluid change using the IRRAflow system will be safe and feasible and will reduce the occlusion and infection rates in patients with IVH. Trial registration: ClicalTrials.gov NCT05204849. Registered 15 December 2021.</p