Gorham-Stout disease (GSD) is an intractable disease characterized by massive osteolysis caused by abnormal lymphangiogenesis in bone. In rare cases of GSD, CSF abnormalities develop. The authors present the case of a 19-year-old woman with GSD presenting with orthostatic headache due to intracranial hypotension (5 cm H2O). The clinical course of this case was very unusual. Orthostatic headache was associated with a CSF leak from the thigh after pathological fractures of the femur and pelvis. The chronic CSF leak led to acquired Chiari malformation (CM) with syringomyelia. After an epidural blood patch, her neurological status improved; however, after the complete arrest of the CSF leak from the thigh, she presented with severe nonpostural headache and progressive visual acuity loss with optic papilledema. A ventriculoperitoneal shunt was placed to treat intracranial hypertension (50 cm H2O). Headache improved and optic papilledema decreased after shunt surgery. This case shows that dynamic CSF abnormalities may lead to reversible CM in patients with GSD. Sealing a CSF leak rather than performing suboccipital decompression is recommended for acquired CM resulting from a CSF leak.
Shoko Yoshimoto, Keisuke Takai, Koichi Takahashi, Toshio Yasui, and Makoto Taniguchi
Brendan F. Judy, Jordan W. Swanson, Wuyang Yang, Phillip B. Storm, Scott P. Bartlett, Jesse A. Taylor, Gregory G. Heuer, and Shih-Shan Lang
Evaluation of increased intracranial pressure (ICP) in the pediatric craniosynostosis population based solely on ophthalmological, clinical, and radiographic data is subjective, insensitive, and inconsistent. The aim of this study was to examine the intraoperative ICP before and after craniectomy in this patient population.
The authors measured the ICP before and after craniectomy using a subdural ICP monitor in 45 children. They regulated end-tidal carbon dioxide and the monitoring site under general anesthesia to record consistent ICP readings.
The average age of the patient population was 29 months (range 3.8–180.5 months). Thirty-seven patients (82.2%) were undergoing initial craniosynostosis procedures. All craniosynostosis procedures were categorized as one of the following: frontoorbital advancement (n = 24), frontoorbital advancement with distraction osteogenesis (n = 1), posterior vault distraction osteogenesis (n = 10), and posterior vault reconstruction (n = 10). Nineteen of 45 patients (42.2%) had syndromic or multisuture craniosynostosis. The mean postcraniectomy ICP (8.8 mm Hg, range 2–18 mm Hg) was significantly lower than the precraniectomy ICP (16.5 mm Hg, range 6–35 mm Hg) (p < 0.001). Twenty-four patients (53%) had elevated ICP prior to craniectomy, defined as ≥ 15 mm Hg. Only 4 (8.9%) children had papilledema on preoperative funduscopic examination (sensitivity 17%, specificity 100%, negative predictive value 51%, and positive predictive value 100%). There were no significant differences in elevated precraniectomy ICP based on type of craniosynostosis (syndromic/multisuture or nonsyndromic) or age at the time of surgery. Patients undergoing initial surgery in the first 12 months of life were significantly less likely to have elevated precraniectomy ICP compared with patients older than 12 months (26.3% vs 73.1%, p = 0.005).
In this study, the authors report the largest cohort of syndromic and nonsyndromic craniosynostosis patients (n = 45) who underwent precraniectomy and postcraniectomy ICP evaluation. A craniectomy or completed craniotomy cuts for distractors effectively reduced ICP in 43/45 patients. The authors’ findings support the notion that papilledema on funduscopy is a highly specific, however poorly sensitive, indicator of increased ICP, and thus is not a reliable screening method. These findings indicate that even nonsyndromic patients with craniosynostosis are at risk for increased ICP. Furthermore, patients who present prior to 12 months of age appear less likely to have elevated ICP on presentation. Further studies with other noninvasive imaging of the retina may be useful as an adjunct tool for determining elevated ICP.
Tarek Y. El Ahmadieh, Cody B. Wolfe, Joyce Koueik, Bradley E. Weprin, Bermans J. Iskandar, and Angela V. Price
Neuroendoscopy has demonstrated safety and efficacy in the treatment of a host of pediatric neurosurgical pathologies. With the increase in its applicability, several associated complications have been described in the literature. A common practice in pediatric neurosurgery is the use of Gelfoam sponge pledget in the burr hole, followed by bone fragments and dust (obtained from the created burr hole), to cover the dural defect. This technique is used to enhance burr hole sealing and potentially prevent CSF leakage from the surgical site. Reports on intracranial bone dust migration associated with this technique are scarce. The authors report 2 cases of intracranial migration of bone fragments after an endoscopic third ventriculostomy and an endoscopic colloid cyst resection. The bone fragment migration was thought to be caused by negative pressure from a lumbar puncture in one case and external trauma to the head in the other. As endoscopy becomes more widely used, it is important to be aware of this potential complication that may in some cases require an intervention. A review of the cases reported in the literature is provided and a technique is suggested to help prevent this complication.
Surya Sri Krishna Gour, Mohit Agrawal, and Sachin A. Borkar
Zahid Hussain Khan, Masoud Nashibi, and Seyed Amir Javadi
Aiko Terada, Masaki Komiyama, Tomoya Ishiguro, Yasunari Niimi, and Hidenori Oishi
The authors performed a nationwide study in Japan to evaluate the annual detected rate of pediatric intracranial arteriovenous (AV) shunts such as brain AV malformations (BAVMs), pial AV fistulas (PAVFs), vein of Galen aneurysmal malformations (VGAMs), and dural AV fistulas (DAVFs). These rates were revealed for the first time and showed that VGAM, DAVF, and PAVF were relatively common but that BAVMs were extremely rare in neonates and infants.
Mark R. Kraemer, Joyce Koueik, Susan Rebsamen, David A. Hsu, M. Shahriar Salamat, Susan Luo, Sara Saleh, Taryn M. Bragg, and Bermans J. Iskandar
Ventricular shunts have an unacceptably high failure rate, which approaches 50% of patients at 2 years. Most shunt failures are related to ventricular catheter obstruction. The literature suggests that obstructions are caused by in-growth of choroid plexus and/or reactive cellular aggregation. The authors report endoscopic evidence of overdrainage-related ventricular tissue protrusions (“ependymal bands”) that cause partial or complete obstruction of the ventricular catheter.
A retrospective review was completed on patients undergoing shunt revision surgery between 2008 and 2015, identifying all cases in which the senior author reported endoscopic evidence of ependymal tissue in-growth into ventricular catheters. Detailed clinical, radiological, and surgical findings are described.
Fifty patients underwent 83 endoscopic shunt revision procedures that revealed in-growth of ventricular wall tissue into the catheter tip orifices (ependymal bands), producing partial, complete, or intermittent shunt obstructions. Endoscopic ventricular explorations revealed ependymal bands at various stages of development, which appear to form secondarily to siphoning. Ependymal bands are associated with small ventricles when the shunt is functional, but may dilate at the time of obstruction.
Ventricular wall protrusions are a significant cause of proximal shunt obstruction, and they appear to be caused by siphoning of surrounding tissue into the ventricular catheter orifices.
Michael M. Safaee, Soren Jonzzon, Giselle Y. López, Shailesh Asaikar, Tarik Tihan, Orit A. Glenn, and Nalin Gupta
Calcifying pseudoneoplasms of the neuraxis (CAPNONs) are rare, nonneoplastic lesions of the CNS. Their radiographic features have been well described, with prominent calcifications seen on CT imaging and generally uniform hypointensity on T1- and T2-weighted MRI sequences, with variable patterns of contrast enhancement. They are not associated with significant perilesional edema. The authors present an unusual case of an 8-year-old boy who was found to have a 2.5-cm right frontal mass that demonstrated reduced signal on T2-weighted sequences, heterogeneous contrast enhancement, and extensive perilesional edema on MRI sequences. The differential diagnoses included a chronic infection or neoplasm. He underwent gross-total resection of a firm, calcified mass that had clear boundaries between it and the surrounding gliotic brain. Pathological analysis demonstrated a well-circumscribed lesion with islands of lamellar calcifications and intervening spindle cells, consistent with a CAPNON. At 8 months after surgery the patient remained seizure free, and MRI revealed no evidence of residual lesion and significant improvement in perilesional edema. This particular case highlights the potential for unusual presentation of CAPNON and the rare presence of perilesional edema.
Geoffrey P. Colby, Bowen Jiang, Matthew T. Bender, Narlin B. Beaty, Erick M. Westbroek, Risheng Xu, Li-Mei Lin, Jessica K. Campos, Rafael J. Tamargo, Judy Huang, Alan R. Cohen, and Alexander L. Coon
Intracranial aneurysms in the pediatric population are rare entities. The authors recently treated a 9-month-old infant with a 19-mm recurrent, previously ruptured, and coil-embolized left middle cerebral artery (MCA) pseudoaneurysm, which was treated definitively with single-stage Pipeline-assisted coil embolization. The patient was 5 months old when she underwent resection of a left temporal Grade 1 desmoplastic infantile ganglioglioma at an outside institution, which was complicated by left MCA injury with a resultant 9-mm left M1 pseudoaneurysm. Within a month, the patient had two aneurysmal rupture events and underwent emergency craniectomy for decompression and evacuation of subdural hematoma. The pseudoaneurysm initially underwent coil embolization; however, follow-up MR angiography (MRA) revealed aneurysm recanalization with saccular enlargement to 19 mm. The patient underwent successful flow diversion–assisted coil embolization at 9 months of age. At 7 months after the procedure, follow-up MRA showed complete aneurysm occlusion without evidence of in-stent thrombosis or stenosis. Experience with flow diverters in the pediatric population is still in its early phases, with the youngest reported patient being 22 months old. In this paper the authors report the first case of such a technique in an infant, whom they believe to be the youngest patient to undergo cerebral flow diversion treatment.