Optical Detection of Intracranial Pressure and Perfusion Changes in Neonates With Hydrocephalus.

OBJECTIVE: To demonstrate that a novel noninvasive index of intracranial pressure (ICP) derived from diffuse optics-based techniques is associated with intracranial hypertension. STUDY DESIGN: We compared noninvasive and invasive ICP measurements in infants with hydrocephalus. Infants born term and preterm were eligible for inclusion if clinically determined to require cerebrospinal fluid (CSF) diversion. Ventricular size was assessed preoperatively via ultrasound measurement of the fronto-occipital (FOR) and frontotemporal (FTHR) horn ratios. Invasive ICP was obtained at the time of surgical intervention with a manometer. Intracranial hypertension was defined as invasive ICP ≥15 mmHg. Diffuse optical measurements of cerebral perfusion, oxygen extraction, and noninvasive ICP were performed preoperatively, intraoperatively, and postoperatively. Optical and ultrasound measures were compared with invasive ICP measurements, and their change in values after CSF diversion were obtained. RESULTS: We included 39 infants, 23 with intracranial hypertension. No group difference in ventricular size was found by FOR (P = .93) or FTHR (P = .76). Infants with intracranial hypertension had significantly higher noninvasive ICP (P = .02) and oxygen extraction fraction (OEF) (P = .01) compared with infants without intracranial hypertension. Increased cerebral blood flow (P = .005) and improved OEF (P < .001) after CSF diversion were observed only in infants with intracranial hypertension. CONCLUSIONS: Noninvasive diffuse optical measures (including a noninvasive ICP index) were associated with intracranial hypertension. The findings suggest that impaired perfusion from intracranial hypertension was independent of ventricular size. Hemodynamic evidence of the benefits of CSF diversion was seen in infants with intracranial hypertension. Noninvasive optical techniques hold promise for aiding the assessment of CSF diversion timing.

Prophylactic Decompression for Cervical Stenosis in Jeune Syndrome: Report From a Single Institution.

STUDY DESIGN: This is a level IV retrospective descriptive study at a single institution. OBJECTIVE: The objective of the study was to determine the preoperative signs or symptoms prompting cervicomedullary imaging in Jeune syndrome. SUMMARY OF BACKGROUND DATA: Jeune syndrome is a rare autosomal recessive disorder that results in pulmonary compromise from abnormal development of the thorax. Multiple medical comorbidities complicate timely diagnosis of cervicomedullary stenosis, which neurologically jeopardizes this patient population with regards to improper cervical manipulation. Currently, explicit screening of the cervicomedullary junction is not advocated in national guidelines. METHODS: The User Reporting Workbench and Center for Thoracic Insufficiency Syndrome (CTIS) Safety Registry was queried for patients with Jeune syndrome under the age of 18 with cervicomedullary stenosis with or without suboccipital craniectomy/craniotomy evaluated at the authors' institution from January 1, 2007 to August 21, 2018. The primary outcome was the clinical reason for cervicomedullary screening. Secondary outcomes were: age at time of surgery, preoperative myelopathy (spasticity, urinary retention), hydrocephalus, postoperative deficits (respiratory, motor, swallowing difficulty), and need for cervical fusion. RESULTS: Of 32 patients with Jeune syndrome, four (12.5%) had cervicomedullary stenosis requiring decompression. The average age at surgery was 5.25 months (2-9 mo). Two patients underwent imaging due to desaturation events while the other two patients were diagnosed with cervical stenosis as an incidental finding. No patients exhibited clinical myelopathy. Two patients had baseline preoperative swallowing difficulties. None of the patients postoperatively required cervical fusions, nor did they exhibit respiratory deficits, motor deficits, or worsening swallowing difficulties. CONCLUSION: Jeune patients should be routinely screened for cervicomedullary stenosis and undergo subsequent prophylactic decompression to minimize or eliminate the development of irreversible neurologic compromise. LEVEL OF EVIDENCE: 4.