White Cord Syndrome after Thoracic Cord Decompression in a Pediatric Patient.

INTRODUCTION: White cord syndrome (WCS) is a rare phenomenon which causes acute neurologic deterioration after a chronically compressed spinal cord is surgically decompressed, resulting in a T2-hyperintense signal to appear in the cord. We present the first case of pediatric WCS in the thoracic spine and the first to show complete resolution of symptoms. CASE PRESENTATION: A 3-year-old girl presented with difficulty ambulating due to pain caused by T4-8 spinal cord compression from a mass of ganglioneuromatous tissue, consistent with her previously treated mediastinal neuroblastoma. She underwent laminoplasty and microsurgical debulking of the mass. She developed severe bilateral leg weakness on postoperative day 1. Magnetic resonance imaging (MRI) showed T2 hyperintensity in the spinal cord from T1 to T10 without enhancement, concerning for WCS. She was started on high-dose steroids and monitored. Her strength improved to 3/5 in both legs prior to discharge to inpatient rehabilitation. On the latest follow-up, 4 months after surgery, she did not demonstrate any neurologic deficits and was ambulating well. Follow-up MRIs have showed continued improvement in the T2 hyperintensity. DISCUSSION/CONCLUSION: Most cases in the literature occur in older adults with chronic cervical compression. Only 1 pediatric case, caused by a cervical arachnoid cyst, has been reported and only resulted in partial improvement. Our case is the first pediatric case of WCS to occur in the thoracic spine, the first pediatric case to demonstrate complete resolution, and the first case of any age to start to establish a length of time the compression is needed to be at risk for WCS.

Dynamic Occlusion of Distal Ventriculoperitoneal Shunt Catheter after Infusion Port Placement: A New Shunt Malfunction.

Shunt failure requiring reintervention remains a common complication of hydrocephalus treatment. Here, we report a novel cause of mechanical shunt obstruction in an adult patient: position-dependent intermittent occlusion via an infusion port catheter. A 51-year-old woman with a grade II oligodendroglioma presented in a delayed fashion following surgery with a pseudomeningocele. She underwent ventriculoperitoneal shunt placement due to communicating hydrocephalus, resolving her pseudomeningocele. Shortly thereafter, she underwent placement of a subclavian infusion port at an outside institution. Her pseudomeningocele returned. Imaging demonstrated close proximity of her port catheter to the shunt catheter overlying the clavicle. Her shunt was tapped demonstrating a patent ventricular catheter with normal pressure. She underwent shunt exploration after her pseudomeningocele did not respond to valve adjustment. Intraoperative manometry demonstrated head position-dependent distal catheter obstruction. Repeat manometry following distal catheter revision demonstrated normal runoff independent of position. Her pseudomeningocele was resolved on follow-up. To our knowledge, this is the only reported case of intermittent, position-dependent distal catheter obstruction. Shunted patients with concern for malfunction following subclavian infusion port placement should be evaluated for possible dynamic obstruction of their distal catheter when the two catheters are in close proximity along the clavicle.

Endoscopic Placement of Fourth Ventricular Catheter Using Seldinger Technique: Description of Technique and Case Series.

BACKGROUND: Neonatal intraventricular hemorrhage remains a significant source of morbidity in premature and low-weight patients. Approximately 15% of patients who require cerebrospinal fluid shunting develop trapped fourth ventricle (TFV). Surgical treatment presents challenges with short- and long-term complications. OBJECTIVE: To describe a technique that applies the Seldinger technique with image-guided endoscopy for direct visualization of catheter placement. METHODS: A guidewire is passed down the endoscope while it is positioned in the fourth ventricle. The endoscope is removed while the guidewire is held in place. The catheter is slid down the guidewire. The guidewire is removed and placement is confirmed with image guidance. RESULTS: Three patients, all less than 14 mo old, with history of prematurity and intraventricular hemorrhage with ventriculoperitoneal shunts, presented with loculated hydrocephalus with TFV. They each underwent image-guided endoscopic fenestration of the fourth ventricle with placement of a fourth ventricular catheter performed by our described technique. All 3 patients recovered well and were discharged on postoperative day 1. Follow-up imaging showed decompression of the fourth ventricle and good placement of the fourth ventricular catheter. None have had complications from catheter placement, and one revision of a fourth ventricular catheter was needed, which was completed with the same described technique. CONCLUSION: This technique is well suited for cases in which a fourth ventricular catheter or a difficult trajectory catheter is needed during endoscopic fenestration or when distorted anatomy is present that would make a straight trajectory with a pen endoscope more difficult or higher risk.

Venous Sinus Stenosis Treatment in Pediatric Idiopathic Intracranial Hypertension: Illustrative Case and Literature Review.

OBJECTIVE: To review the literature of venous sinus stenosis (VSS) treatment in children for idiopathic intracranial hypertension (IIH) and present our own institutional case. METHODS: A literature review was conducted using the PubMed and MEDLINE databases up to June 2020. From 134 studies that were screened, 6 studies were chosen for analysis that included patients <18 years old, a diagnosis of IIH that fit Dandy diagnostic criteria, and angiogram obtained to assess for VSS. IIH symptoms experienced in the pediatric population and efficacy of venous sinus stenting were analyzed. RESULTS: Eleven patients identified in the literature and 1 patient from our institution were included in the analysis. There was no statistically significant difference in pressure gradient response to stenting between male and female patients (P = 0.424) or patients with body mass index >90th percentile (P = 0.626). Larger decreases in pressure gradient after stent placement correlated with headache resolution (P = 0.0005). Patients who underwent unilateral stenting showed greater reduction in pressure gradient compared with patients who underwent bilateral stenting (average change 24 mm Hg vs. 5.75 mm Hg, P = 0.003). CONCLUSIONS: Our analysis showed that VSS treatment has the potential to be a safe option for IIH in pediatric patients. VSS treatment has shown similar results to traditional cerebrospinal fluid diversion procedures, with a lower complication rate and need for revision. More studies should be conducted to analyze the long-term efficacy and safety of VSS treatment in pediatric patients with IIH.

Umbilical hernia causing ventriculoperitoneal shunt dysfunction: illustrative cases.

BACKGROUND: When ventriculoperitoneal (VP) shunts and umbilical hernias coexist in the same patient, unique complications can occur. Typically, these are readily identified problems such as cerebrospinal fluid (CSF) fistulas or entrapment of the peritoneal catheter in the hernia. The authors present cases of two children whose VP shunt dysfunction resolved after repair of their umbilical hernias. OBSERVATIONS: The authors present two cases of infant patients with shunted hydrocephalus and umbilical hernias. In both cases, the patients presented with distal shunt malfunctions not due to infection. Their shunt function improved once the umbilical hernia was repaired by pediatric surgery. Neither has required shunt revision since umbilical hernia repair. LESSONS: Although there are case reports of VP shunts eroding through the umbilicus, developing CSF fistulas, or becoming trapped inside umbilical hernias, there is no case of VP shunt dysfunction caused by just the presence of an umbilical hernia. The authors suspect that the catheter may intermittently enter and exit the hernia. This may cause intermittent obstruction of the distal catheter, or inflammation in the hernia may occur that limits CSF absorption.

Continuous infusion of low-dose unfractionated heparin after aneurysmal subarachnoid hemorrhage: a preliminary study of cognitive outcomes.

OBJECTIVECognitive dysfunction occurs in up to 70% of aneurysmal subarachnoid hemorrhage (aSAH) survivors. Low-dose intravenous heparin (LDIVH) infusion using the Maryland protocol was recently shown to reduce clinical vasospasm and vasospasm-related infarction. In this study, the Montreal Cognitive Assessment (MoCA) was used to evaluate cognitive changes in aSAH patients treated with the Maryland LDIVH protocol compared with controls.METHODSA retrospective analysis of all patients treated for aSAH between July 2009 and April 2014 was conducted. Beginning in 2012, aSAH patients were treated with LDIVH in the postprocedural period. The MoCA was administered to all aSAH survivors prospectively during routine follow-up visits, at least 3 months after aSAH, by trained staff blinded to treatment status. Mean MoCA scores were compared between groups, and regression analyses were performed for relevant factors.RESULTSNo significant differences in baseline characteristics were observed between groups. The mean MoCA score for the LDIVH group (n = 25) was 26.4 compared with 22.7 in controls (n = 22) (p = 0.013). Serious cognitive impairment (MoCA ≤ 20) was observed in 32% of controls compared with 0% in the LDIVH group (p = 0.008). Linear regression analysis demonstrated that only LDIVH was associated with a positive influence on MoCA scores (ß = 3.68, p =0.019), whereas anterior communicating artery aneurysms and fevers were negatively associated with MoCA scores. Multivariable linear regression analysis resulted in all 3 factors maintaining significance. There were no treatment complications.CONCLUSIONSThis preliminary study suggests that the Maryland LDIVH protocol may improve cognitive outcomes in aSAH patients. A randomized controlled trial is needed to determine the safety and potential benefit of unfractionated heparin in aSAH patients.