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UC San Francisco

UC San Francisco Previously Published Works

Title
Fellowship Training in the Emerging Fields of Fetal-Neonatal Neurology and Neonatal
Neurocritical Care

Permalink
https://escholarship.org/uc/item/186307jp

Authors
Smyser, CD
Tam, EWY
Chang, T
et al.

Publication Date
2016-10-01

DOI
10.1016/j.pediatrneurol.2016.06.006

Peer reviewed

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University of California
Pediatric Neurology xxx (2016) 1e6

Contents lists available at ScienceDirect

Pediatric Neurology
journal homepage: www.elsevier.com/locate/pnu

Topical Review

Fellowship Training in the Emerging Fields of Fetal-Neonatal


Neurology and Neonatal Neurocritical Care
Christopher D. Smyser MD, MSCI a, b, c, *, Emily W.Y. Tam MDCM, MAS, FRCPC d,
Taeun Chang MD e, Janet S. Soul MDCM, FRCPC f,
Steven P. Miller MDCM, MAS, FRCPC d, Hannah C. Glass MDCM, MAS, FRCPC g, h, i
a
Department of Neurology, Washington University, St. Louis, Missouri
b
Department of Pediatrics, Washington University, St. Louis, Missouri
c
Department of Radiology, Washington University, St. Louis, Missouri
d
Department of Paediatrics, The Hospital for Sick Children and University of Toronto, Ontario, Canada
e
Department of Neurology, Children’s National Health System, Washington, DC
f
Department of Neurology, Boston Children’s Hospital and Harvard Medical School, Boston, Massachusetts
g
Department of Neurology, Benioff Children’s Hospital, University of California, San Francisco, California
h
Department of Pediatrics, Benioff Children’s Hospital, University of California, San Francisco, California
i
Department of Epidemiology and Biostatistics, Benioff Children’s Hospital, University of California, San Francisco, California

abstract
BACKGROUND: Neonatal neurocritical care is a growing and rapidly evolving medical subspecialty, with increasing
numbers of dedicated multidisciplinary clinical, educational, and research programs established at academic in-
stitutions. The growth of these programs has provided trainees in neurology, neonatology, and pediatrics with
increased exposure to the field, sparking interest in dedicated fellowship training in fetal-neonatal neurology.
OBJECTIVES: To meet this rising demand, increasing numbers of training programs are being established to provide
trainees with the requisite knowledge and skills to independently deliver care for infants with neurological injury
or impairment from the fetal care center and neonatal intensive care unit to the outpatient clinic. This article
provides an initial framework for standardization of training across these programs. RESULTS: Recommendations
include goals and objectives for training in the field; core areas where clinical competency must be demonstrated;
training activities and neuroimaging and neurodiagnostic modalities which require proficiency; and programmatic
requirements necessary to support a comprehensive and well-rounded training program. CONCLUSIONS: With
consistent implementation, the proposed model has the potential to establish recognized standards of professional
excellence for training in the field, provide a pathway toward Accreditation Council for Graduate Medical
Education certification for program graduates, and lead to continued improvements in medical and neurological
care provided to patients in the neonatal intensive care unit.
Keywords: neonatal neurocritical care, neonatal neurology, fetal neurology, fellowship, education, neonatology, neurophysiology,
neuroradiology
Pediatr Neurol 2016; -: 1-6
Ó 2016 Elsevier Inc. All rights reserved.

Introduction neurological morbidities faced by surviving infants has led


to fundamental alterations in medical and neurological care
Over the past several decades, increased understanding provided in the neonatal intensive care unit (NICU).1,2 Novel
of the developing brain and the short- and long-term neuroprotective strategies to prevent and/or mitigate the
effects of brain injury and improve neurological outcomes
have been established and implemented.1-3 Ancillary
Article History:
testing that requires age-specific interpretation, such as
Received February 13, 2016; Accepted in final form June 7, 2016
* Communications should be addressed to: Dr. Smyser; 660 South
electroencephalography (EEG) and magnetic resonance
Euclid Avenue, Campus Box 8111; St. Louis, Missouri 63110-1093. imaging (MRI), has become commonplace. New and
E-mail address: smyserc@neuro.wustl.edu increasingly available neurodiagnostic technologies to

0887-8994/$ e see front matter Ó 2016 Elsevier Inc. All rights reserved.
http://dx.doi.org/10.1016/j.pediatrneurol.2016.06.006
2 C.D. Smyser et al. / Pediatric Neurology xxx (2016) 1e6

identify and evaluate neurological impairment have been local program structure and resources, these recommen-
developed and adapted. As a result, neonatal neurocritical dations (developed based on our own existing programs
care has emerged as one of the most rapidly evolving sub- and consensus agreement where programs differ) can be
specialties in all of medicine. used as foundation for establishment and continued
Concomitant with these advances in NICU clinical care, development of programs at individual institutions.
several centers have established dedicated integrative fetal-
neonatal neurology clinical and research programs.1,2,4-7 At Overall goals and objectives
most centers, these programs consist of multidisciplinary
collaborations including neurology, neonatology, neuro- The overarching goal of fetal-neonatal neurology
surgery, neuroradiology, palliative care, and obstetrics. The fellowship training should be to successfully provide in-
advent of these programs distinctly changed the clinical struction for physiciansdincluding both child neurologists
practice of neonatal neurocritical care. Protocols for and neonatologistsdin the knowledge and skills necessary
consistent and comprehensive evaluation and management to practice as a specialized consultant and collaborator in
of common neurological conditions have been developed. the care of neonates with or at risk for neurological injury or
Specialty consultants are involved earlier and more impairment in the NICU.
frequently, often as early as fetal consultation. Electro- To achieve this, the trainee must acquire comprehensive
physiological and radiological testing adapted for the medical knowledge in the diagnosis and management of
developing brain are used to evaluate patients and develop neurological conditions affecting neonates, including con-
individualized care plans. Furthermore, neonatal neurolo- ditions arising during fetal development and as a result of
gists are playing an increasing role in rigorous neonatal preterm birth. This includes expert skills in neurological
follow-up programs. The overarching result has been examination of the newborn at all gestational ages. Also
establishment of a brain-directed model of critical care and needed is knowledge in neuroembryology, etiologies of
improvements in patient care and family relationships.6-8 brain injury, genetic and metabolic conditions, neuromus-
Subsequently, as many as one third of all NICU patients at cular diagnoses, and epilepsies presenting in the antenatal,
some institutions are evaluated by neonatal neurocritical perinatal, or early postnatal period. Knowledge of the pri-
care programs, leading to 100s of encounters each year at mary newborn medical conditions that accompany these
large academic centers.2 As a result of this growth, neuro- neurological conditions and their therapies is also essential
critical care programs of this type have become financially (e.g., respiratory failure, pulmonary hypertension, etc.).
beneficial.9 Expertise in the interpretation and integration of results
The growth of these clinical programs has provided from diagnostic modalities in common use in the NICU is
trainees in neurology, neonatology, and pediatric training important. Understanding of current treatment options in
programs with consistent and expanded exposure to the treatment of newborns and young children is also
neonatal neurocritical care as an important component of required. This knowledge should enable the graduate to
their training, sparking growing interest in training in the provide counseling of families antenatally, care and support
field.10 Increasingly, specialized training beyond the tradi- of newborns and families during the neonatal period in the
tional residency program is required to attain the requisite NICU, and continued ongoing assessment and management
experience and expertise necessary to function indepen- of NICU graduates in follow-up.
dently and provide state-of-the-art care in this area of child To this end, the fetal-neonatal neurology fellowship
neurology.11 This requirement has led many individuals to must provide adequate exposure and supervised training in
pursue additional training in fetal-neonatal neurology, neurological patient care from the fetal care center or NICU
often through independent mechanisms. In an attempt to to the outpatient clinic settings. Postnatal care in the
meet these growing clinical and training demands, neonatal neurology follow-up clinic should be established
increasing numbers of training programs in fetal-neonatal in collaboration with rehabilitation services and develop-
neurology have been established through varied funding mental pediatricians and include exposure to complex
mechanisms. Some institutions have capitalized on local follow-up care for developmental delay and epilepsy, as
neurocritical care programs in adult and pediatric medicine, well as cognitive, behavioral, and neuromotor disabilities.
where training opportunities have been established over Focused training on neurophysiological and neuroimaging
the last decade.12 Certification in Critical Care Neurology is tools is also needed. Education in patient-centered care,
now offered through the United Council of Neurological ethical and humanistic aspects of care, communication
Specialties13; however, there is currently no formal skills, and palliative care is also of high importance in a field
accreditation for fetal-neonatal neurology and neonatal where parents are faced with life-changing diagnoses for
neurocritical care. their yet unborn or newborn child. The framework must
This article represents an initial step toward standardi- also exist to enable the trainee to gain exposure to clinical or
zation of training in fetal-neonatal neurology and neonatal basic science research in neonatal neuroscience, to provide
neurocritical care. We include recommendations regarding opportunities for ongoing contribution to the field of fetal-
goals and objectives for training in the field, core areas neonatal neurology, and to develop the skills for evidence-
where clinical competency must be demonstrated, recom- based practice and learning.
mended training activities, neuroimaging and neuro- Although there is currently no accreditation for training
diagnostic modalities that require proficiency, and in neonatal neurocritical care in the United States or Can-
programmatic requirements to support a comprehensive ada, these activities should be centered on federal guide-
and well-rounded training program. Although newly lines (Accreditation Council for Graduate Medical Education
established training programs will likely differ based on [ACGME] or Royal College of Physicians and Surgeons of
C.D. Smyser et al. / Pediatric Neurology xxx (2016) 1e6 3

Canada, respectively), focusing on the hallmark compo- TABLE 2.


Commonly Encountered Fetal and Neonatal Neurological Conditions
nents of patient care, medical knowledge, practice-based
learning and improvement, interpersonal and communi-  Neonatal encephalopathy/hypoxic-ischemic encephalopathy
cation skills, professionalism, and systems-based practice to  Seizures, acute symptomatic seizures and neonatal onset epi-
provide the framework for future accreditation. lepsies, as well as status epilepticus
 Stroke, arterial and venous
 Intracranial hemorrhage, including intraventricular hemorrhage
 White matter injury
 Neurological sequelae of congenital heart disease and critical
Core competencies
illness
 Meningitis/encephalitis and in utero infections
Most trainees will arrive in neonatal neurology fellow-  Hypotonia/neuromuscular disorders
ship programs having completed core programs in pediatric  Hydrocephalus, posthemorrhagic and congenital
neurology or neonatology. Yet, with the expansion of  Inborn errors of metabolism
neonatal neurocritical care programs, there is also  Neurogenetic and complex malformation syndromes
increasing interest from other specialty pathways including  Brain malformations and cerebral vascular malformations
cardiac intensive care and developmental pediatrics. Given
the heterogeneity of prefellowship training, all trainees
must develop proficiency in a number of key areas directly Inpatient training
related to fetal-neonatal neurology (Table 1).2,4,7,13-16 These
competencies should be developed as they relate to the Inpatient training experience with the neurocritical care
fetus where applicable and in a postmenstrual age-specific service is necessary for the trainee to master the evaluation
manner in the care of term and preterm neonates. and management of acute and/or subacute neonatal
Principal among these is the ability to perform a neuro- neurological conditions. The trainee must become adept
logical examination in a critically ill newborn, recognize the with acute bedside management of encephalopathy, status
acute versus subacute versus chronic neurologically epilepticus, and other life-threatening neurological condi-
affected newborn, and become confident at independently tions. This includes knowledge of current national guide-
diagnosing and managing common neurological conditions lines (e.g., therapeutic hypothermia and brain monitoring)
(Table 2). The trainee must also develop a level of comfort in and expertise to adapt these to a specific center’s resources.
imparting information regarding these diagnoses to fam- Trainees must also become proficient in neurological
ilies to aid them in acute decision-making and forming prognostication, including understanding the impact of
long-term care plans in collaboration with the maternal- neuroprotective therapies and developmental care and
fetal medicine or ICU teams. exposures to pain, infection, surgery, and critical illness.
Finally, they must develop skills enabling effective
communication with families. Specific recommendations
include the following:
Recommended training activities
 A minimum of six months of direct care or consultation
Recommended activities include direct patient care for inpatient neonates with neurological disorders who
balanced with formal didactic education and subspecialty are admitted to the NICU to develop the skills and
rotations (Table 3). Across all activities, programs should
develop a detailed set of learning objectives to provide the TABLE 3.
trainee with understanding of the skills and knowledge Suggested Training Schedule
they are to acquire.
Clinical pathway, 1 year
 Neonatal neurocritical care service, 6-8 months, depending on
TABLE 1. the trainee’s residency experience
Core Competencies  Outpatient clinics, 1-2 months (or minimum two half-days/
month if concurrent with inpatient rotations)
 Perform age-appropriate neurological examinations
 Diagnose and manage common and rare neonatal neurological B Neonatal neurology clinic, 1-2 clinics/month
disorders B Fetal consults, includes participation in both inpatient and
 Understand the pathophysiology of common conditions and outpatient consults
mechanism of action of neuroprotective therapies B Other clinics including high-risk infant follow-up, physiatry,
 Understand proper use of ancillary neurodiagnostic and neuromuscular and cerebrovascular clinics, 1-2 clinics/month
neuroimaging tools to improve assessment, management, and  Neuroradiology, 1 month
prognostication (see Neurodiagnostic and neuroimaging tools  Electrophysiology, 1 month
requiring proficiency section)  Elective, 2-3 months (research and/or clinical rotations based
 Synthesize available clinical information to predict neurological upon trainee interests)
outcomes
Research pathway, 2-3 years
 Understand evidence-based medicine as it applies to fetal and
 1 year clinical pathway as above (elective rotations may be used
neonatal neurology, including current guidelines and practice
for targeted research activities)
parameters
 1-2 years clinical, translational, or bench research
 Be sensitive to ethical issues that arise in fetal-neonatal
 Coursework relevant to the research pathway, e.g.,
neurology
 Develop effective communication skills with families and other B Master’s degree in clinical research or epidemiology
health care providers B Master’s degree in data science or bioinformatics
4 C.D. Smyser et al. / Pediatric Neurology xxx (2016) 1e6

expertise to direct neurological evaluation and man- neonatal nursing, neurology, neurosurgery, neuroradiology,
agement. (REQUIRED) neurophysiology, pathology, genetic/metabolic, palliative
 If newborns with cardiac anomalies are cared for in a care, and neurorehabilitation (including developmental
separate cardiac ICU (CICU), training should include at care) subspecialties. Specific recommendations include the
least one month providing neurology consultation for following:
neonates in the CICU to achieve familiarity with the
unique course and management of newborns with  Structured multidisciplinary educational program in
congenital heart disease with neurologic injury. fetal-neonatal neurology that includes didactic lectures,
(REQUIRED) supplemented by journal clubs, morbidity and mortality
rounds, and/or ethics conferences. See Appendices 1
Outpatient training and 2 for expanded lists of relevant neonatal and fetal
neurology topics to be considered for inclusion in
The overarching goal of outpatient training is for the didactic curricula. (REQUIRED)
trainee to understand the long-term clinical course of  Bedside teaching rounds and/or case conferences that
neurological conditions that present in the fetal and incorporate neurophysiology, neuroradiology, and fetal
neonatal periods. The trainee must become adept at diag- and placental pathology. (REQUIRED)
nosing and providing initial evaluation and management  Fellows shall also attend local conferences relevant to
for the developmental disabilities that arise subsequent to fetal-neonatal neurology, including neonatology,
fetal-neonatal neurological conditions, including neuro- maternal-fetal medicine, neurology, genetics, pathol-
motor disabilities (e.g., cerebral palsy), epilepsy, cognitive ogy, and neuroimaging conferences.
dysfunction, autonomic dysfunction, cerebral visual
impairment, and state regulation (e.g., sleep, mood). Neurodiagnostic and neuroimaging tools requiring
Trainees should become familiar with available equipment, proficiency
therapies, and medical and surgical treatments for spas-
ticity, cerebral palsy, and cerebral visual impairment. The Neurodiagnostic and neuroimaging tools are used in the
trainee is also expected to gain valuable exposure to neonatal neurocritical care setting to determine both
methods for prenatal evaluation of neurological disorders, diagnosis and prognosis and are a key component of patient
develop familiarity in interpreting fetal MRI, be aware of management and discussions with families.1-3 For both
available fetal surgical therapies and/or trials (e.g., in utero neurodiagnostic and neuroimaging tools, the neonatal
neural tube defect repair), and be familiar with local neurologist must become proficient in the following as they
termination laws and counseling for fetal consults. Finally, apply to the term and preterm neonate:
the trainee should become familiar with local and regional
resources available to the patient and their families such as  Indications and guidelines for performing monitoring
early intervention services, state Medicaid programs, and imaging studies17
federal disability programs, and local pediatric hospice  Interpretation of monitoring and imaging study results
programs. Specific recommendations include the following:  Management decisions based on monitoring and
imaging study results
 Outpatient rotation(s) in neonatal neurology clinics, as  Communication of study results to allied health pro-
well as exposure to fetal neurology consults, infant fessionals and parents
development or high-risk follow-up clinics, and phys-
iatry or rehabilitation clinics (and/or equipment, spina Neuroimaging
bifida, and spasticity clinics). Suggested exposure is one
to two months (or minimum two half-days/month if Cranial ultrasound
concurrent with inpatient rotations). (REQUIRED) Cranial ultrasound (CUS) is the most commonly used
 Elective rotations in EEG, neuroimaging, maternal-fetal imaging modality in the NICU.1,2 It is noninvasive and can be
medicine (including inpatient and outpatient fetal used readily at the bedside. Neonatal studies should
consults, fetal imaging, fetal cardiology, and fetal routinely provide coronal, sagittal, and posterior fossa views
pathology), palliative care, and perinatal pathology of the brain. Cerebral blood flow and intracranial pressure
(including fetal-pediatric autopsy and placental pa- can also be assessed using Doppler studies. CUS is used
thology), if available and depending upon the trainee’s principally for screening examinations and serial assess-
career goals. (SUGGESTED) ments of cerebral injury in term and preterm infants,
although it is limited in its ability to detect injury in specific
brain regions.
Didactic training
Magnetic resonance imaging
A formal, multidisciplinary education curriculum specific MRI provides valuable information regarding cerebral
to fetal-neonatal neurology is mandatory. The trainee development and acute and chronic brain injury not
is expected to actively participate in conferences and available from other imaging modalities.1,2,18 Magnetic
lead fetal-neonatal neurology teaching sessions for resonance spectroscopy also enables assessment of brain
medical students and resident physicians. Didactic injury and cerebral metabolism (i.e., for infants with sus-
conferences should include contributions from maternal- pected inborn errors of metabolism). Over the past decade,
fetal medicine, neonatology, pediatric critical care, procedures to study term and preterm infants safely
C.D. Smyser et al. / Pediatric Neurology xxx (2016) 1e6 5

without the use of sedating medications have become neurological disorders to provide the trainee with educa-
increasingly established. Growing numbers of studies have tion in congenital and acquired neurological disorders of
correlated neonatal neuroimaging results with neuro- the newborn. Finally, training should include exposure to
developmental outcomes in high-risk neonatal populations. and/or participation in research relevant to fetal-neonatal
Trainees should spend at least one month during the neurology, whether clinical, translational, or basic science.
training period reviewing CUS and MRI studies with board- Specific recommendations include the following:
certified pediatric radiologists and neuroradiologists expe-
rienced in the interpretation of neonatal studies. The Inpatient
trainee should become adept in identifying postmenstrual
age-specific normal patterns, common patterns of injury  Level III/IV NICU. (REQUIRED)
(e.g., changes in diffusion-weighted and perfusion imaging  An inpatient neonatal neurocritical care service that
in infants with stroke or hypoxic-ischemic injury), and/or manages a broad range of preterm and term newborns
developmental abnormality (e.g., malformations of cortical with common and rare neurological disorders.
folding) and anticipated changes in patterns of abnormality (REQUIRED)
with time across both modalities. As part of these activities,  Subspecialists with expertise in the diagnosis and
trainees should also gain exposure to interpretation of management of newborns with congenital or acquired,
magnetic resonance spectroscopy and fetal MRI studies central and peripheral neurological disorders of
with concerns for central nervous system abnormalities. the fetus and newborn. This includes neurologists,
Finally, although not used routinely in the NICU, trainees neonatologists, neurophysiologists, neuroradiologists,
must gain experience in interpretation of neonatal head neurosurgeons, perinatal pathologists, neuropatholo-
computerized tomography studies as portable and/or gists, and geneticists/metabolic specialists. (REQUIRED)
standard studies may be performed to assist in clinical  Inpatient rounds closely integrated with the NICU
decision-making when access to other imaging modalities team to form a comprehensive understanding of the
is limited because of critical illness. (REQUIRED) medical issues primary or secondary to the neuro-
logical condition, discuss the priority of neurological
Neurophysiological monitoring plans, and convey a cohesive message to families.
(REQUIRED)
Electroencephalogram  Inpatient neonatal neurocritical care rounds led by a
Continuous video EEG is the gold standard for detecting child neurologist with at least 25% of clinical activities
seizures in neonates.19,20 Brain monitoring using EEG and dedicated to fetal-neonatal neurology. (SUGGESTED)
adapted montage amplitude-integrated EEG (aEEG) is used  Readily available inpatient EEG monitoring and neuro-
to assess degree of encephalopathy and the presence of imaging optimized for the newborn brain (as per
seizures in neonates who are admitted to the neurocritical neurodiagnostic and neuroimaging tools requiring
care unit. proficiency section). (REQUIRED)
Trainees should spend at least one month during the  Experts in palliative care and ethics to help address the
training period reviewing EEGs with a board-certified unique difficulties of decision-making and care of
neurophysiologist who has experience with neonatal newborns with severe congenital or acquired neuro-
monitoring to become adept at determining background logical disorders and/or complex medical problems that
EEG pattern and seizures from artifact. Proficiency in aEEG include neurological disorders. (REQUIRED)
training can be accomplished with frequent bedside prac-  Rehabilitative services, including physical and occupa-
tice and assistance from review articles and an aEEG atlas.21 tional therapy, physiatry, and lactation support.
(REQUIRED) (REQUIRED)
 A maternal-fetal medicine clinical service with exper-
Optical near-infrared spectroscopy tise in providing care for fetuses with neurological
Near-infrared spectroscopy (NIRS) is a noninvasive concerns beginning antenatally and continuing through
method for trending of brain tissue oxygenation. It is useful the time of delivery and immediate postnatal course.
to guide hemodynamic management to optimize cerebral (REQUIRED)
oxygenation in critically ill term and preterm neonates.22,23  Specialized bedside nurses and/or nurse practitioners
Trainees should gain exposure to NIRS technology and with expertise in neurological conditions in newborns.
interpretation of neonatal NIRS results. (REQUIRED) These individuals can be key members in training,
together with social workers and care coordinators who
Institutional programmatic requirements for fellowship assist families in navigating the health care system.
training (SUGGESTED)

Multiple programmatic requirements are necessary to Outpatient


establish and support a fetal-neonatal fellowship that pro-
vides comprehensive and balanced training that meets the  A multidisciplinary, longitudinal neonatal neurology
needs and interests of individual candidates. Principal outpatient clinic, which includes access to neuropsy-
among these is a sufficiently broad clinical and research chologists, therapists, physiatrists, and nurses.
infrastructure to support well-rounded training at an aca- (REQUIRED)
demic center. In addition, there must be inpatient and  A fetal neurology consult service with specialized fetal
outpatient care with adequate volume and variety of neuroimaging. (REQUIRED)
6 C.D. Smyser et al. / Pediatric Neurology xxx (2016) 1e6

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C.D. Smyser et al. / Pediatric Neurology xxx (2016) 1e6 6.e1

Appendix 1. Neonatal neurology topics to be considered for Neurogenetics


incorporation into didactic curricula 1. Chromosomal and genetic disorders
2. Neurocutaneous disorders
3. Inborn errors of metabolism with neonatal
Neurodiagnostic Tools presentations
1. Neurologic examdnormal newborn; Apgar score; 4. Leukodystrophies
Sarnat score 5. Mitochondrial disorders
2. EEG 6. Catastrophic neurodegenerative disorders of fetal or
3. aEEG; aEEG versus EEG neonatal onset
4. EEG trend algorithms
5. Evoked studiesdbrainstem auditory evoked Neuromuscular
response (BAER); visual evoked potential (VEP); so- 1. Neonatal hypotonia
matosensory evoked potential (SSEP) 2. Arthrogryposis
6. Cranial ultrasound and Doppler 3. Common neonatal neuromuscular disorders
4. Critical illness myopathy
7. CT scansdportable; standard
8. Anatomical MRIeT1; T2; diffusion-weighted image 5. Neurodiagnostic tools
(DWI) Neuroprotection
9. Advanced MRIediffusion tensor image (DTI); func- 1. Neurosupportive care
tional connectivity MRI (fcMRI); magnetic resonance 2. Therapeutic hypothermia
spectroscopy (MRS); perfusion 3. Erythropoietin
10. Near-infrared spectroscopy (NIRS) 4. Clinical trialsdxenon; cord stem cells; mucomyst
11. Placental pathologies 5. Seizure monitoring and treatment
12. Autopsies
Fetal, Perinatal and Neonatal Strokes
Brain Development 1. Incidence; causes
1. Normal neuroembryology 2. Neurodiagnostic evaluation
2. Developmental neurobiology 3. Protocols
3. Myelination 4. Outcomes
4. Brain malformationsdDandyeWalker malforma-
tions; encephalocele; agenesis of the corpus callosum End of Life Issues
5. Spine malformationsdneural tube defects 1. Brain death
6. Migrational disordersdlissencephaly; pachygyria; 2. Futile or palliative care
schizencephaly, heterotopias 3. Autopsies
7. Hindbrain malformations
Outcomes and Disabilities
Development 1. Developmental delay
1. Developmental assessments (age, visual, motor, so- 2. Cortical visual impairment
cial, IQ, pros and cons) 3. Cerebral palsy
2. Prechtl and fidgety movements 4. Intellectual disabilitiesdattention deficit hyperactiv-
3. Outcome measuresdNICU Network Neurobehavioral ity disorder; learning disabilities; mental retardation
Score (NNNS); Dubowitz score; Gross Motor Function
Classification System (GMFCS); Alberta Infant Motor Therapies and Rehabilitation
Scale (AIMS); Bayley III 1. Feeding
4. Outcomes of preterm infants 2. Visual therapy
5. Outcomes of hypoxic-ischemic encephalopathy (HIE) 3. Physical and Occupational Therapy
6. Outcomes of cerebral palsy 4. Speech therapy
5. Treatments for spasticitydbaclofen; botox and
Neonatal Seizures and Epilepsy phenol injections; tendon release
1. Etiology of neonatal seizures 6. Communication devices
2. Neonatal seizure evaluation and workup 7. Equipmentdbath chairs; standers; activity chairs;
3. Neuromonitoring ankle-foot orthoses (AFOs); wheelchairs; walkers;
4. Neonatal seizure therapiesdanticonvulsant medica- gait trainers
tions; lidocaine
5. Neonatal onset epilepsies Other
6. Risk for postnatal epilepsy 1. Neonatal abstinence syndrome
2. Meconium aspiration syndrome/pulmonary
Neonatal Brain Injuries hypertension
1. Traumadextradural hemorrhage; subdural hemor- 3. Congenital diaphragmatic hernia
rhage; subgaleal bleeds, skull fractures 4. Necrotizing enterocolitis
2. Premature infantsdintraventricular hemorrhage 5. Hydrops fetalis
(IVH); periventricular leukomalacia; ventriculitis; 6. Gastroschisis/omphalocele/tracheoesophageal
posthemorrhagic ventricular dilatation fistula
3. Term infantsdHIE/neonatal encephalopathy; intra- 7. Hyperbilirubinemia
cranial hemorrhage; IVH; infections (TORCH, etc.) 8. Hypo/hyperglycemia
6.e2 C.D. Smyser et al. / Pediatric Neurology xxx (2016) 1e6

9. Brachial plexus injury 3. Gyration development


10. Pain management and analgesic exposure/use 4. Cerebellar and vermian development
11. Anesthetic exposure/use 5. Myelination
12. In utero exposure to drugs of abuse
Common Chromosomal and Genetic Syndromes
13. In utero exposure to selective serotonin reuptake
1. Trisomiesd21; 13; 18
inhibitors (SSRIs) and other neuropsychiatric
2. Microarray abnormalities
medications
3. Tuberous sclerosis
14. Maternal mental health
4. Dystroglycanopathies
15. Epidemiology and public health
5. Neurocutaneous melanosis
CNS Maldevelopment
Appendix 2. Fetal neurology topics to be considered for
1. Cerebrumdanencephaly; holoprosencephaly; schi-
incorporation into didactic curricula
zencephaly; focal versus diffuse polymicrogyria;
pachygyria; lissencephaly; transcortical or sub-
ependymal heterotopias; hemimegalencephaly
Fertility Issues and Testing 2. Corpus callosumddysgenesis or complete agenesis;
1. In vitro fertilization/intrauterine insemination associated conditions
2. Preimplantation genetic diagnosis 3. Septum pellucidumdcavum septum pellucidum;
cavum vergae; cavum velum interpositum; associated
Prenatal Screening conditions
1. Maternal serum screening 4. Posterior fossadmega cisterna magna; Blake pouch
2. First-trimester screening cyst; arachnoid cysts; occipital encephalocele;
3. Cell-free DNA/noninvasive prenatal testing (NIPT) DandyeWalker malformation
4. Neonatal Autoimmune Thrombocytopenia (NAIT) 5. Brainstemdcongenital muscular dystrophies; Joubert
testing syndrome; pontotegmental dysplasia; pontocerebellar
5. TORCH testing hypoplasia; brainstem disconnection syndrome
6. Carrier testing 6. Cerebellum and vermisdvermian hypoplasia; cere-
7. Thrombophilias bellar dysplasia; rhomboencephalosynapsis
8. Chorionic villus sampling (CVS) and amniocentesis 7. Craniumdencephalocele
9. Fetal ultrasound and Doppler 8. Spinedneural tube defects
10. Fetal MRIs
11. Fetal magnetoencephalogram (MEG) Ventriculomegaly
12. Autopsies 1. Idiopathic
2. Cerebral malformation
Placenta 3. Obstructive hydrocephalus
1. Anatomy and function
2. Biomarkers Vascular
3. Ultrasounddlocation; Doppler 1. Intraventricular hemorrhages
4. MR imaging 2. Intraparenchymal hemorrhages
5. Pathologydnormal 3. Extracranial hemorrhages
6. Pathologydsubchorionic hemorrhages; prematurity 4. Ischemic strokes
5. Vascular malformations
Growth Issues
1. IUGR versus SGA versus asymmetric growth Infections
2. Normative growth curvesdbiparietal diameter 1. TORCH (toxoplasmosis; rubella; cytomegalovirus
(BPD); head circumference; brain volumes [CMV]; herpes simplex virus [HSV])
3. Microcephalyd2SD versus 3SD 2. Lymphocytic choriomeningitis virus (LCMV)
4. Oligo- versus polyhydramnios 3. HIV
5. Cerebral blood flow and metabolism 4. Zika virus
6. Fetal circulation and brain development 5. Parechovirus
6. Parvovirus
Development
1. Prechtl movements Neuromuscular
2. Swallowing 1. Clubfoot
2. Arthrogryposis
Maternal Conditions
Early-Onset Neurodegenerative Disorders
1. Hypothyroidism
1. Inborn errors of metabolism (IEM)dnonketotic
2. Mood disorders and SSRIs
hyperglycinemia (NKH); sulfite oxidase deficiency;
3. Epilepsy and antiepileptic drugs
glutaric aciduria type 1; Smith-Lemli-Optiz
4. Multiple sclerosis
2. Mitochondrial disordersdpyruvate dehydrogenase
CNS Development deficiency; pyridoxal-5-phosphate deficiency; Leigh
1. Neuroembryology syndrome; pontocerebellar hypoplasia
2. Developmental neurobiology 3. LeukodystrophiesdZellweger; Aicardi Goutieres
C.D. Smyser et al. / Pediatric Neurology xxx (2016) 1e6 6.e3

Brain Tumors 5. Comfort or palliative care


1. Tuberous sclerosis 6. Autopsies and genetic testing
2. Neurocutaneous melanosis
Outcomes and Disabilities
3. Germ cell tumorsdteratoma
1. Immediate postnatal issues
Fetal Seizures 2. Short-term issues
1. Early infantile epileptic encephalopathiesdNKH; 3. Long-term issues
ARX; channelopathies; pontocerebellar hypoplasia
Therapies
End of Life Issues 1. Fetal infectionsdHIV; CMV; Toxoplasmosis
1. Stillbirths 2. AutoimmunedNAIT
2. Terminationdstate and federal laws; induction 3. Fetal surgerydmyelomeningocele; tracheoesophageal
versus extraction fistula; congenital diaphragmatic hernia
3. Postnatal counseling and planning 4. Vasculardarteriovenous malformations; lymphatic
4. Recurrence risk counseling malformations

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