key: cord-0053606-pgkx1jki
authors: Kim, Michael; Stein, Alan A; Overby, Philip; Kleinman, George; Nuoman, Rolla; Al-Mufti, Fawaz; Pisapia, Jared M; Muh, Carrie R
title: Pediatric Multi-System Inflammatory Syndrome With Fatal Cerebral Edema in a Patient With COVID-19
date: 2020-11-16
journal: Neurosurgery
DOI: 10.1093/neuros/nyaa447_571
sha: aeb2c2d67283b71c7aabd38c22bd9b6aacccc8f3
doc_id: 53606
cord_uid: pgkx1jki

INTRODUCTION: There are increasing reports of a pediatric multi-system inflammatory syndrome associated with coronavirus disease 2019 (COVID-19) that presents with varying clinical features, but includes features of Kawasaki disease or toxic shock syndrome. Symptoms include fever, rash, abdominal pain, vomiting, and diarrhea. Many patients present without any respiratory symptoms and testing for SARS-CoV-2 is often negative. METHODS: A retrospective chart review was performed. RESULTS: A 7-year-old previously healthy male presented with 3 days of fevers up to 102.4F, headaches, abdominal pain, and intractable vomiting. Both parents had tested positive for SARS-CoV-2 four weeks prior. Nasopharyngeal swab tested positive for SARS-CoV-2 RNA. Echocardiogram was normal. CT venogram of his head was negative for any pathology. He developed severe neck pain and persistent headache during his hospitalization. Soon after receiving hydroxychloroquine, he developed a facial rash and altered mental status with episodes of aphasia, agitation, and pinpoint pupils. He then became unresponsive with left gaze deviation. A non-contrast head CT and CT angiography were negative. He was given levetiracetam and cefazolin and transferred to the pediatric intensive care unit. An electroencephalogram (EEG) showed no epileptiform activity. Over the following 7 hours, the EEG demonstrated left frontotemporal slowing, which progressed into a loss of fast activity over the right hemisphere with increased delta activity in the left hemisphere, then abruptly changed to generalized voltage attenuation.He rapidly lost brainstem reflexes, developing fixed and dilated pupils. Repeat CT scan revealed diffuse cerebral edema with loss of gray-white differentiation. Lab results then were consistent with severe inflammation. An intracranial pressure monitor revealed pressures greater than 76 mmHg. His exam soon became consistent with brain death. Pathologic evaluation showed diffuse cerebral edema with perivascular mononuclear infiltrates. CONCLUSION: The cause of this pediatric multi-system inflammatory syndrome is unclear and the mechanism by which SARS-CoV-2 affects the nervous system is unknown. Pediatric patients with COVID-19 and neurologic symptoms should be closely monitored as they can rapidly decline due to fulminant cerebral edema.

INTRODUCTION: Spontaneous intracranial hypotension (SIH) is an uncommon and generally self-limiting condition caused by low cerebrospinal fluid (CSF) volume usually secondary to a CSF-leak. This process results in a downward traction of the brain, causing headaches, subdural fluid collections and possible brain herniation. 16-57% of patients are expected to suffer from subdural hematomas (SDH). SIH treatment prior or after SDH management still remains controversial.

METHODS: Procedure/Clinical findings A 48-year-old man presented with an orthostatic headache of 3-months duration that worsened for the last few hours. A CT-scan showed SDH. A bilateral craniotomy with drain placement was performed. The drain was removed 24 hours later at a bedside procedure, triggering headache, tonic posture, right ocular deviation and altered mental status. A new CT-scan showed SDH recurrence. After a second intervention, SIH was diagnosed. An epidural blood patch (EBP) was performed. With no change in the clinical outcome, SDH recurred and a third intervention with drain placement was performed. In the post-op period, normal sterile saline was infused into the subdural space at the same time the drain was being removed.

RESULTS: The patient was discharged with no recurrence of neurologic signs after a 24-month follow-up. It is hypothesized that drain removal causes a direct communication between the atmosphere and intrathecal space, further decreasing the intracranial pressure in SIH patients. These would cause either brain herniation or recurrence of SDH. To avoid a sudden decrease of intracranial pressure and mask the effect of atmospheric pressure, we infused saline solution into the subdural space simultaneous to drain retraction.

CONCLUSION: SIH is a frequently misdiagnosed disorder. No standardized treatment has been established for SDH/SIH presentation as some centers perform EBP either before or after hematoma evacuation. To our knowledge, this is the first report of clinical resolution with transoperative subdural saline infusion. Further consensus needs to be reached as the prognosis of SDH/SIH can be largely impacted by an approach proven to be safe and free of long-term complications.

Trigeminal Neuralgia Due to a Tributary of the Petrosal Vein Passing Through the Substance of the Trigeminal Nerve at its Root Entry Zone: A Video Record Ahmed Abougamil; Tarel Rayan INTRODUCTION: Management of trigeminal neuralgia includes medical treatment, minimally invasive procedures or surgical micro vascular decompression (MVD). MVD is now considered the benchmark surgical procedure for intractable TN of any variety. Here we report a female patient suffered from TN who had the offending vessel totally inside the trigeminal nerve.

METHODS: We present a case of a 52-year old female who had a 2-year history of a typical left TN involving the second and third trigeminal branches. Carbamazepine was ineffective in achieving pain relief initially. MRI brain demonstrated offending vascular loop near the right trigeminal nerve complex. Surgical decompression was performed through left retrosigmoid approach where the offending vein was found passing through the trigeminal nerve itself. Microvasucalr decompression was completed successfully through simple coagulation and transaction of the vein releasing its anchoring effect upon the trigeminal nerve.

RESULTS: Postoperatively, the patient didn't suffer any deficits secondary to sacrificing the vein and her trigeminal neuralgia was completely relieved.

CONCLUSION: The normal anatomy of the veins in this region is quite variable and the venous structures causing a neurovascular contact are more or less inconsistent. To the best of our knowledge and despite the wide range and progress in microvascular decompression surgery for trigeminal neuralgia, this is the first video recorded case in the literature for a neurovascular conflict caused by a vein passing through the trigeminal nerve itself. INTRODUCTION: There are increasing reports of a pediatric multisystem inflammatory syndrome associated with coronavirus disease 2019 (COVID-19) that presents with varying clinical features, but includes features of Kawasaki disease or toxic shock syndrome. Symptoms include fever, rash, abdominal pain, vomiting, and diarrhea. Many patients present without any respiratory symptoms and testing for SARS-CoV-2 is often negative.

METHODS: A retrospective chart review was performed.

RESULTS: A 7-year-old previously healthy male presented with 3 days of fevers up to 102.4F, headaches, abdominal pain, and intractable vomiting. Both parents had tested positive for SARS-CoV-2 four weeks prior. Nasopharyngeal swab tested positive for SARS-CoV-2 RNA. Echocardiogram was normal. CT venogram of his head was negative for any pathology. He developed severe neck pain and persistent headache during his hospitalization. Soon after receiving hydroxychloroquine, he developed a facial rash and altered mental status with episodes of aphasia, agitation, and pinpoint pupils. He then became unresponsive with left gaze deviation. A non-contrast head CT and CT angiography were negative. He was given levetiracetam and cefazolin and transferred to the pediatric intensive care unit. An electroencephalogram (EEG) showed no epileptiform activity. Over the following 7 hours, the EEG demonstrated left frontotemporal slowing, which progressed into a loss of fast activity over the right hemisphere with increased delta activity in the left hemisphere, then abruptly changed to generalized voltage attenuation.He rapidly lost brainstem reflexes, developing fixed and dilated pupils. Repeat CT scan revealed diffuse cerebral edema with loss of gray-white differentiation. Lab results then were consistent with severe inflammation. An intracranial pressure monitor revealed pressures greater than 76 mmHg. His exam soon became consistent with brain death. Pathologic evaluation showed diffuse cerebral edema with perivascular mononuclear infiltrates.

CONCLUSION: The cause of this pediatric multi-system inflammatory syndrome is unclear and the mechanism by which SARS-CoV-2 affects the nervous system is unknown. Pediatric patients with COVID-19 and neurologic symptoms should be closely monitored as they can rapidly decline due to fulminant cerebral edema.

rates and substantial morbidity. Limited understanding of pathogenesis warrants identification of crucial genetic drivers underlying CH and their impact on brain development.

METHODS: Exome analysis of 381 radiographically-confirmed, neurosurgically-treated sporadic CH probands (including 232 caseparent trios) identified genes with rare de novo or transmitted mutations conferring disease risk. Transcriptome analyses identified mid-gestational brain modules and cell-types enriched for cohort-determined CH risk genes, known genes previously implicated in isolated and syndromic forms of CH, and risk genes of Autism Spectrum Disorder (ASD) and Developmental Disorder (DD).

RESULTS: Exome analysis reveals 9 high confidence genes and 55 probable risk genes harboring CH-linked mutations. Together, cohort-determined and known CH genes enrich in a single network ("yellow" module) associated with ASD and DD. Functional profiling of the yellow module yields terms of cell and neuronal differentiation, congenital anomalies of craniofacial development, and behavioral abnormalities. Cohort-determined and known CH genes together enrich in nascent migrating excitatory neurons and cycling mitotic progenitors, occupying earlier stages of differentiation than ASD-and DD-enriched cell-types.

CONCLUSION: Genetic drivers of CH converge in a neurodevelopmental network and in early neurogenic cell-types, implicating genetic disruption of early brain development as a primary patho-mechanism for a significant subset of CH patients. Transcriptional overlap with ASD and DD may explain persistence of these conditions in CH patients despite surgical intervention, while greater potency of CH-enriched neural precursors may account for increased frequency of structural brain abnormalities in CH than in ASD or DD alone. METHODS: A retrospective review was conducted of medical records of 294 CM-I patients (<20 years), operated on at a single institution between 2001-2015 by the senior author (RGE). Imaging findings of tonsillar descent, associated syrinx (syringomyelia or syringobulbia), basilar invagination, clinical assessment of CM-I attributable symptoms, and scoliosis were recorded. Clinical outcomes, including syrinx resolution, symptom resolution, impact on scoliosis progression and complications were compared for 3 groups: bone only/posterior fossa decompression (PFD), with duraplasty (PFDwD), with duraplasty and obex exploration (PFDwDO).

Congenital Hydrocephalus (CH) affects

/1,000 live births and costs the US healthcare system over $2 billion annually. Surgical cerebrospinal fluid diversion exhibits high failure