Decompressive Cranial Vault Remodeling in Osteosclerotic Robinow Syndrome

We present a novel application of endocranial burr contouring for cranial vault expansion as a surgical adjunct during decompressive craniectomy in patients with cranial osteosclerosis. A 16-year-old female with osteosclerotic Robinow syndrome complicated by slit ventricle syndrome presented with refractory intracranial hypertension following external ventricular drain placement. Symptoms included severe headaches and altered mental status. Given the severe intracranial volume restriction secondary to massive calvarial thickening (2.5 cm), the patient was taken to the operating room for urgent surgical decompression. After frontal and parietal craniectomy, burr and osteotome contouring were used to remove two-thirds of the endocranial calvarial bone flap thickness resulting in a 9% cranial vault expansion while preserving an overall normal head size. There were no immediate postoperative complications. At over 3 years postoperatively, the patient had reduced headaches, maintained adequate shunt function, and has not required further vault reconstruction.

A Mathematical Model to Simulate Intracranial Pressure and Unified Interpretation of Several Disease Entities

BACKGROUNDMany clinical phenomena related to cerebrospinal fluid(CSF) and intracranial pressure (ICP) are often contrary to common sense and difficult to explain by classical theory. Such as slit ventricle syndrome, normal intracranial pressure hydrocephalus / low pressure hydrocephalus, paradoxical herniation, and so on. Many authors have different theories about them but can’t have an unified explanation.OBJECTIVEWe try to simulate the above CSF disorders and ICP conduction with a mathematical method, and make theoretical interpretations to them.METHODSWe introduced a mathematical model based on several well-accepted hypothesesto simulate human CSF physiology and propose that ICP curve should be an U-shaped curve (especially, we introduce the hypothesis that CSF also play a role of decompression). Maple software was used to draw charts according to our formula. We use the theory and intuitive charts to explain those illnesses one by one.RESULTSThe formula: ICP = μ · MAP − δ · Vα · μ · MAP + θ · Vβ · μ · MAP + C, and corresponding diagrams was conducted.CONCLUSIONThis mathematical model is a supplement to the classical Monro-Kellie’s theory, the curve and coordinate system can be used to analyze different pathophysiological states and give a reasonable unified explanation to them.

ENDOSCOPY FOR ACHIEVEMENT OF SHUNT-INDEPENDENT CONDITION IN TREATMENT OF SLIT VENTRICLE SYNDROME

Objective: Objective: To demonstrate the results of neuroendoscopic operations for achievement of shunt-independent condition in treatment of slit ventricle syndrome. Methods: Forty-three cases were considered: adult patients aged 18 years and over – 5 (11.63%), and children aged 0-17 years – 38 (88.37%). Pediatric patients were divided into the following age groups: under 1 year – 6 (13.95%); 1-2 years – 7 (16.28%); 2-17 years – 25 (58.14%). The interval between operations was an average of 2 years 6 months (from 1 week to 16 years), and the period of observation after surgery – an average of 41.8 months (from 1 week to 100 months). Results: In children under 1 year, the efficiency was 83.3%; 1-2 years old – 71.4%; 2-17-years old – 92%; 60% of adult patients aged 18 and over. The effectiveness of operations performed using standard rigid endoscopes was 72.1%, and using a semi-rigid needle-shaped endoscope – 95.2%. In total, the overall efficiency of the ETV, aqueductoplasty, choroid plexus coagulation, cystocysternostomy, septostomy, ventricular stenting, revision and reconstruction of VPS, plasty and stenting of Foramen of Magendie and Lyushka, independently from the age of the patients, type of endoscopes was 83%. In 68.3% of cases shunt-independent state was achieved. Conclusions: Endoscopic methods have shown their high effectiveness in the treatment of patients with slit ventricle syndrome and other complications of hydrocephalus. Using a more advanced modern endoscopic technique, such as a semi-rigid needle endoscope in our case, allows to restore CSF circulation even in the most distal sections of the interpeduncular and prepontine cisterns, as well as safely remove and install a ventricular catheter with minimal trauma to surrounding structures and achieve a shunt-independent state. Keywords: Slit ventricle syndrome, endoscopic ventriculocysternostomy, obstructive hydrocephalus, semi-rigid needle-shaped neuroendoscope, aqueductoplasty, septostomy.