Pediatric Flashcards
A 19-year-old man has suffered from complex-partial seizures since the age of 13. He has failed a trial of two anti-epileptic medications. His brain MRI reveals no focal abnormalities. What is the most appropriate next step in management?
a. Initiation of ketogenic diet
b. Addition of cannabidiol (CBD oil), a cannabis derivative
c. Evaluation by surgical epilepsy team for possible resective options
d. Addition of a third anti-epileptic medication
e. Implantation of a vagus nerve stimulator
c. Evaluation by surgical epilepsy team for possible resective options
A 10 year old boy presents with loss of consciousness followed by a lucid interval following a closed head injury. A head CT is shown (figure). Damage to which of the following vessels is most likely responsible for the imaging findings?
a. Superficial temporal artery
b. Middle meningeal artery
c. Vein of Labbe
d. Middle cerebral artery
e. Cerebral bridging vein
b. Middle meningeal artery
A 9 month old boy presents with scaphocephaly. X-rays confirm a sagittal synostosis. He a history of reflux disease that the family is treating with antacids. He is exclusively breast fed and his height and weight are in the 10th percentile, although occipitofrontal circumference is 80th percentile. In this case, which associated diagnosis is most likely?
a. Congenital Adrenal Hyperplasia
b. Prader-Willi Syndrome
c. Congenital Heart Disease
d. Rickets
e. Celiac Sprue
d. Rickets
A Central American patient presents with new onset seizures. CT shows hydrocephalus, and multiple calcified lesions. MRI demonstrates rim-enhancing cysts throughout the brain. What is the most likely etiology?
a. Schistosoma mansoni
b. Toxoplasma gondii
c. Plasmodium falciparum
d. Taenia solium
e. Entamoeba histolytica
d. Taenia solium
What is the most likely diagnosis demonstrated by the findings in the MRI images shown (see figures)?
a. Chordoma
b. Chiari 1 malformation with syrinx
c. Pilocytic astrocytoma
d. Basilar invagination
e. Multiple sclerosis
b. Chiari 1 malformation with syrinx
A 6-year-old girl presents with a deviated gluteal skin fold and lumbosacral dimple. A representative axial T1 non-contrast image from the patient’s lumbar MRI is shown (figure). If she is symptomatic, which is most likely?
a. Urinary incontinence
b. Flat feet
c. L5 paresthesias
d. Hypotonia
e. Anterior thigh pain
a. Urinary incontinence
A 14-year-old female presents with progressive difficulty writing. An MRI (figure) shows multiple nodular areas running along the cervical nerve roots and brachial plexus. Examination of her eyes also shows iris harmartomas. What is the most likely diagnosis?
a. Tuberous sclerosis
b. Neurofibromatosis-1
c. Neurofibromatosis-2
d. Von Hippel-Lindau
e. Ataxia-telangiectasia
b. Neurofibromatosis-1
Which of the following is the most effective sedative for pediatric trauma with severe traumatic brain injury and increased intracranial pressure?
a. Propofol
b. Sevoflurane
c. Dexmedetomidine
d. Thiopental
e. Fentanyl
e. Fentanyl
A 15-year-old girl with a history of epilepsy and learning disability presents with vomiting and somnolence. MRI shows obstructive hydrocephalus with a 3 cm calcified, contrast-enhancing intraventricular mass near the foramen of Monro, as well as several small subependymal nodules. Which cutaneous findings are most likely to be observed on physical examination?
a. Melanoblastosis cutis linearis (pigmented dermatitis)
b. Café-au-lait spots
c. Port-wine stain (nevus flammeus)
d. Adenoma sebaceum
e. Axillary freckles
d. Adenoma sebaceum
A 6-month-old girl with a lipomyelomeningocele is undergoing surgical detethering of their lumbar lipoma, filum sectioning, arachnoid adhesion lysis, and aggressive debulking of the intradural lipoma to the margins of the neural placode. What is the next surgical step?
a. Further lipoma resection until a gross total resection is achieved
b. Patch-graft duraplasty
c. Primary dural closure with a nonabsorbable monofilament suture
d. Primary dural closure with an absorbable, braided suture
e. Pial closure and tubularization of the placode/distal cord
e. Pial closure and tubularization of the placode/distal cord
An 8-year-old girl presents with obstructive triventricular hydrocephalus requiring endoscopic exploration of the ventricular system. What is the most appropriate site for surgical fenestration?
a. Lamina terminalis above the suprachiasmatic recess
b. Third ventricle floor anterior to the mammillary bodies
c. Pineal recess
d. Third ventricle floor anterior to the infundibular recess
e. Septum pellucidum superior to the fornices
b. Third ventricle floor anterior to the mammillary bodies
A 4-week-old, 26 week premature infant presents with a Grade IV intraventricular hemorrhage and posthemorrhagic hydrocephalus (Figure 1). Examination reveals stable vital signs, normal head circumference growth curve, and soft anterior fontanelle. Weight is 1.2 kg. What is the best initial management of the hydrocephalus?
a. Creation of a permanent ventriculoperitoneal shunt
b. Placement of a ventricular reservoir
c. Placement of external ventricular drain
d. Serial cranial ultrasounds with daily head circumference measurements
e. Serial lumbar punctures
d. Serial cranial ultrasounds with daily head circumference measurements
A 14 year-old is involved in a MVC and does not lose consciousness. He has a GCS of 15 and no focal deficits on neurological exam. He has no other injuries. His toxicology screen is negative. You remove his collar, and he has no palpable spinal tenderness or pain with range of motion. The correct next step would be which of the following?
a. Send patient for a MRI of the cervical spine
b. Remove the cervical collar and backboard
c. Send patient for a CT scan of the cervical spine
d. Obtain adequate cervical spine x-rays
e. Obtain flexion/extension cervical spine x-rays
b. Remove the cervical collar and backboard
An 8 year-old presents after an accident in gymnastics. He felt the immediate onset of sharp pain behind his right ear after a fall. In the emergency department an hour later he was neurologically intact. However, his head was immobile, being rotated to the right and tilted to the left with the left ear touching the left shoulder. A CT scan of the cervical spine was abnormal (Figure 1). What is the most appropriate initial treatment?
a. Rigid cervical orthosis
b. 15 pounds of cervical traction
c. Brooks fusion in situ with halo-vest immobilization
d. Open reduction and Harms fusion
e. Thermoplastic Minerva jacket
a. Rigid cervical orthosis
A 14-year-old male presented with months of slowly worsening tingling in his hands. His exam was intact. Diagnostic work-up included an MRI of the cervical and thoracic spine (figure). What is the most likely diagnosis?
a. Hemangiopericytoma
b. Astrocytoma
c. Neuroblastoma
d. Neurofibroma
e. Ependymoma
e. Ependymoma
What factor has had the single largest effect on the incidence of myelomeningocele in the United States over the past 30 years?
a. Antenatal diagnosis followed by termination
b. Changes in population demographics
c. Decreased use of seizure medication during pregnancy
d. Decreased numbers of children being born
e. Folate supplementation
e. Folate supplementation
A 5-year-old undergoes surgical resection of a posterior fossa medulloblastoma followed by chemotherapy and total neuroaxis radiotherapy, with a boost to the posterior fossa. Which endocrinological complication is most likely after radiotherapy for a posterior fossa tumor in childhood?
a. Cushing’s Syndrome
b. Addison’s Syndrome
c. Diabetes Insipidus
d. Growth Hormone Excess
e. Growth Hormone Deficiency
e. Growth Hormone Deficiency
What is the most common anatomic location of pediatric gangliogliomas?
a. Spinal cord
b. Temporal lobe
c. Brainstem
d. Frontal lobe
e. Parietal lobe
b. Temporal lobe
An 8-month-old girl with developmental delay presents to the emergency department with new onset seizure activity. On exam, the patient has slightly reduced muscular tone in the left upper and lower extremities. A non-contrast MRI is obtained (figure). What is the most likely diagnosis?
a. Porencephalic cyst
b. Arachnoid cyst
c. Holoprosencephaly
d. Schizencephaly
e. Lissencephaly
d. Schizencephaly
What is the most common side effect of vagus nerve stimulation?
a. Dyspnea
b. Cardiac arrhythmia
c. Wound infection
d. Dyspepsia
e. Voice hoarseness
e. Voice hoarseness
A 8-month-old infant was sent for imaging (figures) by his pediatrician because of irritability, poor feeding, and macrocephaly with a bulging fontanel. The preceding pregnancy and delivery had been unremarkable. The infant had been well up until this presentation, and review of systems was noncontributory. What additional diagnostic investigation is indicated?
a. Blood lead level
b. Measurement of parents’ head circumferences
c. Pyloric ultrasound
d. Dilated funduscopic examination
e. Urine amino acids
d. Dilated funduscopic examination
An 11-month-old female with a history of myelomeningocele repair and ventriculoperitoneal shunt placement presents with new stridor and vocal cord paralysis. What is the most appropriate next step in management?
a. Shunt evaluation
b. Tethered spinal cord release
c. Observation
d. EEG
e. Chiari decompression
a. Shunt evaluation
A 15 year-old boy complains of neck pain and intermittent pain about his lumbar myelomeningocele scar. A recent shunt series shows no disconnection and a head CT shows small dysmorphic ventricles. On exam, the patients is neurologically stable with chronic weakness of ankle dorsiflexion. What is the most likely source of the patient’s symptoms?
a. Ventricloperitoneal shunt malfunction.
b. Tethered spinal cord.
c. Ventriculoperitoneal shunt infection.
d. Aseptic Meningitis.
e. Chiari Il malformation.
a. Ventricloperitoneal shunt malfunction.
A 6-year-old with Down syndrome has a skull defect that has been present since birth (Figures 1 & 2). What is the correct diagnosis?
a. Dermoid cyst
b. Healed skull fracture
c. Aplasia cutis congenita
d. Bilateral parietal foramina
e. Acute skull fracture
d. Bilateral parietal foramina
In a patient with the MRI shown in the figure, a tumor in what other location would increase the probability of germinoma?
a. Suprasellar region
b. Frontal lobe
c. Orbit
d. Fourth ventricle
e. Brainstem
a. Suprasellar region
A 7-year-old child with a two week history of difficulty walking, dysarthric speech and facial weakness has the MRI shown in the figure. What is the most appropriate initial treatment?
a. Surgical debulking.
b. Whole-brain radiation.
c. Chemotherapy.
d. Gamma-knife.
e. Conformal radiotherapy.
e. Conformal radiotherapy.
A 10-year-old girl was playing outside when she fell and landed on her head. She experienced complete loss of motor and sensory function from the neck down that resolved over the next 15 minutes. A CT is shown (figure). What is the most appropriate treatment?
a. Transoral odontoidectomy
b. Posterior C1-2 fusion
c. Observation
d. External Rigid Collar
e. External Halo vest immobilization for 6-12 weeks
b. Posterior C1-2 fusion
A 16 year-old severe traumatic brain injury patient is on continuous IV propofol for ICP/CPP management. He develops a low urine output, mild fever, and blood drawn for laboratory analysis is cloudy. A diagnosis of propofol infusion syndrome is proposed. What laboratory value will best confirm the diagnosis?
a. Serum creatinine 1.2
b. Serum creatine kinase 75,000
c. ABG pH 7.35
d. Serum potassium of 3.4
e. Serum triglyceride 100
b. Serum creatine kinase 75,000
A patient with a baclofen pump presents with a temperature of 104 degrees F and hyperreflexia. What is the next appropriate treatment?
a. Broad spectrum antibiotics
b. Intravenous acetaminophen
c. Intrathecal baclofen administration
d. Intravenous dantrolene
e. Cooling blanket
c. Intrathecal baclofen administration
Which of the following answers most appropriately pairs a sign or symptom of hydrocephalus (or shunt malfunction in the setting of a patient with a shunt) and an appropriate age or patient population?
a. Adult - suture splaying
b. Infant - visual loss
c. Child - tense fontanel
d. Adult-progressive macrocephaly
e. Child - decline in IQ or school performance
e. Child - decline in IQ or school performance
A 5-month-old infant was a passenger in a car seat in a high-speed crash resulting in ejection. On exam, she only demonstrates right
parietal scalp swelling. A head CT scan shows separation of the lambdoid suture and faint hypodensity in the subjacent brain (Figure
1). CT scanning of the cervical spine and other routine trauma investigations were negative. She was discharged in stable condition
after 1 day. Which of the following is the most active concern for outpatient follow-up?
a. Chronic subdural hematoma
b. Traumatic meningoencephalocele
c. Hypopituitarism
d. Atlanto-occipital instability
e. Hydrocephalus
b. Traumatic meningoencephalocele
What primary intracranial tumor type has the highest frequency of BRAF-V600E mutations?
a. Schwannomas
b. Gangliogliomas
c. Meningiomas
d. Pituitary adenomas
e. Glioblastomas
b. Gangliogliomas
A 12-year-old African-American male with a history of asthma presents with fever, lethargy, seizure activity, and left hemiparesis.
Inflammatory markers are elevated. After stabilization, a CT head with contrast is obtained (figure). What is the most appropriate next
step in management?
a. Burrholes
b. Bedside subdural drain
c. Lumbar puncture
d. Craniotomy / craniectomy
e. Sinus surgery
d. Craniotomy / craniectomy
A four-year-old presents with occipital headaches and a lesion on brain MRI. He also has cafe-au-lait spots and axillary freckling. Patients with his disease are most likely to have a tumor in what intracranial location?
a. Cerebellum
b. Auditory canal
c. Brainstem
d. Cerebral cortex
e. Optic pathway
e. Optic pathway
What is the approximate mortality rate of stereotactic brainstem biopsy for suspected brainstem malignancy?
a. 20%
b. 15%
c. 5%
d. 10%
e. 1%
e. 1%
A 7-year-old female presents with worsening gait disturbance and upper back pain. A spinal MRI is shown. After surgical exposure,
frozen specimen analysis suggests juvenile pilocytic astrocytoma. What is the next best step in intraoperative management?
a. Divide dentate ligaments to permit spinal cord rotation
b. Multiple sample biopsies for diagnostic confirmation
c. Internal debulking and resection until the normal-abnormal boundary is indistinct
d. Gross total resection along the tumor capsule
e. Duraplasty and laminoplasty with no further tumor resection
c. Internal debulking and resection until the normal-abnormal boundary is indistinct
After being struck in the head, a 12 year old boy presents with a scalp laceration over a palpable skull deformity. A CT scan is obtained (Figure 1). His GCS is 14 (E3, M6, V5). What is the most appropriate management for this injury?
a. Venticulostomy for ICP management
b. Allow wound to heal by secondary intention
c. Bedside wound closure and IV antibiotics
d. Surgical exploration, fracture elevation, and debridement
e. Bedside irrigation, debridement, and wound closure
d. Surgical exploration, fracture elevation, and debridement
For which of the following diagnoses associated with hydrocephalus is it safest and most reasonable to perform a lumbar puncture?
a. Subarachnoid hemorrhage
b. Fourth ventricular ependymoma
c. Colloid cyst
d. Aqueductal stenosis
a. Subarachnoid hemorrhage
A 7-year-old girl presents with slowly worsening urinary incontinence (after previously being continent) and severe back pain radiating to the bottom of her feet. An MRI is shown. What is the most likely diagnosis?
a. Meningocele
b. Diastamatomyelia
c. Myelomeningocele
d. Lipomyelomeningocele
e. Fatty filum terminale
d. Lipomyelomeningocele
What is the most appropriate maintenance rate for IV fluid in a 2 week old child weighing less than 10 kg?
a, 4 ml/kg/hr
b. 20 ml/kg/hr
c. 2 ml/kg/hr
d. 10 ml/kg/hr
e. 1 ml/kg/hr
a, 4 ml/kg/hr
A 16-year-old boy presents with headaches, nausea, and blurred vision. His neurological examination is remarkable for impaired upgaze and convergence nystagmus. Contrast-enhanced MRI of the brain is shown (figure). Serum beta-HCG and alpha-fetoprotein levels are normal. What is the most likely diagnosis?
a. Meningioma
b. Germinoma
c. Endodermal sinus tumor
d. Choriocarcinoma
e. Embryonal carcinoma
b. Germinoma
A 4 year old child with symptomatic unilateral moyamoya syndrome was treated with surgical revascularization. At 1-year postoperatively, an angiogram revealed stable arteriopathy and excellent postsurgical collateral development. Cerebral blood flow studies show normalization of flow and clinically the child is symptom-free. What is the appropriate follow-up plan?
a. Clinical visits with annual CT imaging
b. Clinical visits annually with detailed neurologic examination, but no imaging is needed
c. Clinical visits annually with detailed neurologic examinations and MRI/MRA annually for 5 years
d.No follow-up is needed.
e. Clinical visits or imaging only if symptoms recur, otherwise just routine follow-up with the pediatrician
c. Clinical visits annually with detailed neurologic examinations and MRI/MRA annually for 5 years
Which of the following types of acceleration most likely results in diffuse axonal injury (DAI)?
a. Non-rotational
b. Non-linear
c. Rotational
d. Linear
e. Translational
c. Rotational
A 9-year-old girl visits her optometrist because of a 1-month progressive history of blurry vision and headaches. The optometrist notes papilledema. Imaging is obtained. What course of action is indicated?
a. LP shunt insertion
b. Conformal radiation therapy
c. Stereotactic biopsy
d. Endoscopic third ventriculostomy
e. Lumbar puncture for cytology and markers
d. Endoscopic third ventriculostomy
A 12 year-old with Down syndrome has abnormal flexion-extension cervical spine x-rays. The child has no significant neurologic complaints or neck pain. On exam, the child has full range of motion and no tenderness to palpation. The flexion-extension x-rays show a 7-8 mm atlantodental interval in flexion which reduces to 4 mm in extension. What is the most appropriate next step?
a. Observation with no repeat imaging necessary
b. Observation with repeat flexion-extension in the future
c. C1-2 wiring with halo placement and autologous fusion
d. C1-2 transarticular screw placement and autologous fusion
e. Occipital cervical fusion with sublaminar wires
b. Observation with repeat flexion-extension in the future
A 9 month old male with no significant medical history presents with one week of progressive irritability and emesis. There is no reported history of trauma. Examination shows a well-developed, irritable infant with a full fontanelle but without focal neurologic deficits. He has no external signs of trauma. Head circumference has increased from the 65th percentile to the 92nd percentile since his 6-month visit. Non-contrast head computed tomography (CT) is shown. What is the most likely diagnosis?
a. Non-accidental trauma
b. Ruptured arachnoid cyst
c. Subdural empyema
d. Intracranial hypotension
e. Benign enlargement of the subarachnoid spaces
a. Non-accidental trauma
The tumor represented in this photomicrograph most frequently occurs in association with what condition?
a. Sturge-Weber syndrome
b. Turcot syndrome
c. Von Hippel-Lindau syndrome
d. Neurofibromatosis-2
e. Tuberous sclerosis
d. Neurofibromatosis-2
A 2-year-old child presents with new-onset headaches, nausea and vomiting. Imaging reveals a solid, enhancing mass arising from the fourth ventricle floor and resultant mild obstructive hydrocephalus. Histopathologic evaluation of a representative portion reveals both perivascular pseudorosettes and true rosettes. Which of the following is true concerning the prognosis of this patient?
a. Surgical resection has little effect on survival.
b. Post-operative radiation therapy does not confer improved survival benefit.
c. The prognosis in this patient is better than that of a 2-year old child with the same tumor pathology located in the spine.
d. The prognosis is worse in this patient than in a 20-year-old with the same tumor.
d. The prognosis is worse in this patient than in a 20-year-old with the same tumor.
A newborn presents with seizures. MRI of the brain shows Probst bundles running superomedial to the lateral ventricles. This is indicative of what pathological process?
a. Kallman’s syndrome
b. Metabolic disorder
c. Holoprosencephaly
d. Agenesis of the corpus callosum
e. Focal cortical dysplasia
d. Agenesis of the corpus callosum
You are called to the NICU to see a 1 day old child who appears as demonstrated in figures 1 and 3. His CT scan is shown in figure 2. He has been stable, although with occasional apnea when agitated. On exam, he has a bulging fontanelle. His parents wish to pursue aggressive care. When should he undergo a cranial vault procedure?
a. At 6 months of age.
b. At 18 months of age.
c. At 24 months of age.
d. At 12 months of age.
e. In the next several days.
e. In the next several days.
What is the etiology of malignant cerebral edema in children after traumatic brain injury?
a. Cerebral hypoperfusion
b. Loss of autoregulation
c. Acute hydrocephalus
d. Venous sinus thrombosis
e. Nonconvulsive status epilepticus
b. Loss of autoregulation
Which medication has been shown to both reduce the risk of IVH and improve outcome in preterm infants?
a. Ibuprofen
b. Indomethacin
c. Corticosteroids
d. Phenobarbital
e. Vitamin E
c. Corticosteroids
A 2-month-old full-term previously healthy male presents with obstructive hydrocephalus due to an extensive hemorrhagic posterior fossa mass involving both cerebellar hemispheres and the vermis. Genetic analysis of a biopsy reveals a SMARCB1 / INI deletion in the long arm of chromosome 22 (22q11.2). What is the most likely diagnosis?
a. Medulloblastoma
b. Atypical teratoid/rhabdoid tumor (AT/RT)
c. WHO grade III ependymoma
d. Immature teratoma
e. Choroid plexus papilloma (CPP)
b. Atypical teratoid/rhabdoid tumor (AT/RT)
Which of the following is the most likely postoperative deficit seen in a right-handed 17-year-old male undergoing a left anterior temporal lobectomy for treatment of his medically-refractory epilepsy secondary to mesial temporal sclerosis?
a. Language and verbal memory deficits
b. Cranial nerve III palsy
c. Left superior quadrantanopsia
d. Left hemiparesis
e. Right sided sensorimotor apraxia
a. Language and verbal memory deficits
The axial noncontrast head CT (obtained for unrelated reasons) shown from an affected neonate results from which of the following deformities?
a. Metopic synostosis
b. Bicoronal synostosis
c. Unicoronal synostosis
d. Sagittal synostosis
e. Lambdoid synostosis
a. Metopic synostosis
An 8-year-old male complains of a painful swelling of his left forehead. Its onset was insidious, starting several weeks earlier. Radiographs are shown in Figures 1 and 2. What is the most likely diagnosis?
a. Osteoblastoma
b. Eosinophic granuloma
c. Fibrous dysplasia
d. Dermoid cyst
e. Osteoid osteoma
b. Eosinophic granuloma
Which of the following is most likely to help reduce infections associated with primary ventriculoperitoneal shunt insertion?
a. Decreasing number of OR personnel
b. Double gloving of OR personnel
c. Antibiotic irrigation
d. Administration of post-operative antibiotics for more than 24 hours
e. Injection of bacitracin in the shunt tract
b. Double gloving of OR personnel
What finding is more frequently associated with nasal and occipital dermoid cysts compared to other locations?
a. Cyst rupture
b. Malignant transformation
c. Intracranial extension
d. Hydrocephalus
e. Superinfection
a. Cyst rupture
c. Intracranial extension
A 19 year old male presents with tussive headaches located at the posterior base of the skull. Neurologic examination reveals weakness of the hands bilaterally with hypesthesia. MR of the brain and cervical spine are shown in the figures. What is the best initial management strategy for this presentation?
a. Ventriculoperitoneal shunt
b. Posterior fossa decompression
c. Anterior transoral odontoid resection
d. Posterior cervical decompression
e. Syringo-subarachnoid shunt
b. Posterior fossa decompression
What is the most common first line of therapy for vein of Galen malformation causing heart failure in children?
a. Medical management
b. Radiosurgery
c. Observation
d. Microsurgical treatment
e. Endovascular treatment
e. Endovascular treatment
An 18 year old functionally independent girl with tuberous sclerosis presents with progressive headaches. Imaging is shown (figure). What is the best treatment option for this patient?
a. Transcortical transventricular resection
b. Stereotactic radiosurgery
c. Everolimus therapy
d. Observation
e. Interhemispheric transcallosal resection
c. Everolimus therapy
A 10 year old child is referred to your interdisciplinary spasticity clinic. It is determined that he has spasticity as well as significant disabling dystonia in all four extremities, the face, and the neck. His symptoms have been medically refractory. What is the most appropriate treatment?
a. Botox injections
b. Deep brain stimulation
c. Stereotactic pallidotomy
d. Intrathecal baclofen therapy
e. Selective dorsal rhizotomy
d. Intrathecal baclofen therapy
What is the definitive treatment of atlanto-occipital dislocation (AOD)?
a. Rigid cranio-cervico-thoracic orthosis
b. Halo vest immobilization
c. Observation with serial radiographs
d. Cervical traction
e. Cranio-cervical internal fixation and fusion
e. Cranio-cervical internal fixation and fusion
A 5 year-old boy is brought to your clinic secondary to his parents’ concern that his congenital scoliotic curvature is progressing. Scoliosis films are performed demonstrating a unilateral unsegmented bar at T9-10 with a 40% levoscoliosis. What can you tell the parents regarding the cause of this deformation and its natural history?
a. Scheuermann disease with a rate of progression less than that with a formation failure
b. Formation failure with a high risk of continued, rapid progression of curvature
c. Neurulation failure with a low risk of progression of curvature
d. Embryologic differentiation failure with a low risk of progression of curvature
e. Segmentation failure with a high risk of continued, rapid progression of curvature
e. Segmentation failure with a high risk of continued, rapid progression of curvature
A pineal mass was completely resected and the pathologist diagnoses a mature teratoma. What is the best treatment for this patient?
a. Craniospinal irradiation
b. Fractionated local radiation therapy
c. Radiosurgery to the resection cavity
d. Observation
e. Chemotherapy
d. Observation
A 9-year-old male presented to his pediatrician with headaches and growth delay. He was found to have tumor in the region of the sella. Histology is shown. What is the most likely diagnosis:
a. Pilocytic astrocytoma.
b. Hypothalamic hamartoma.
c. Pituitary adenoma.
d. Craniopharyngioma.
e. Colloid cyst.
d. Craniopharyngioma.
A 4-year-old male presents with new onset headaches, nausea, and vomiting and is found to have a posterior fossa tumor. The tumor is resected and the histopathologic specimen is shown (figure 1). Which of the following is the most likely diagnosis?
a. Medulloblastoma
b. Pilocytic astrocytoma
c. Pilomyxoid astrocytoma
d. Ependymoma
e. Glioblastoma multiforme
a. Medulloblastoma
An ultrasound of a 29-year-old pregnant woman reveals a lumbar myelomeningocele in the fetus. What is the primary difference in outcome for fetal myelomeningocele repair as opposed to post-natal repair?
a. The likelihood of premature birth is not affected
b. The risk of symptomatic hindbrain herniation increases
c. The odds of walking without devices or braces decreases
d. The chance of maternal pregnancy complications is not affected
e. The rate of shunt placement for hydrocephalus decreases
e. The rate of shunt placement for hydrocephalus decreases
An asymptmatic 2-year-old child with macrocephaly has the finding seen on CT (Figure 1). He has no signs or symptoms of increased intracranial pressure. Six months later, a routine follow-up MRI is obtained (Figure 2) and repeat testing reveals mild developmental delay and early papilledema. There is no diffusion restriction. What is the most likely diagnosis?
a. Encephalomalacia
b. Abscess
c. Epidermoid
d. Astrocytoma
e. Arachnoid cyst
e. Arachnoid cyst
Hemangioblastomas are tumors characterized by prominent capillary vasculature. What syndrome predisposes an individual to this tumor?
a. Von Hippel-Lindau
b. Sturge-Weber
c. Neurofibromatosis type I
d. Neurofibromatosis type II
e. Tuberous sclerosis
a. Von Hippel-Lindau
A 6 year-old girl presents with progressive scoliosis. Physical examination reveals a club foot and a midline hairy nevus in the lumbar region. MRI is shown (Figure 1). What is the most likely diagnosis?
a. Diastematomyelia
b. Lipomyelomeningocele
c. Myelomeningocele
d. Neurenteric cyst
e. Dermal sinus tract
a. Diastematomyelia
A 9 month old boy presents with single sutural synostosis (figure 1). On exam his fontanelle is closed and there is no papilledema. What associated finding may be diagnosed in this patient?
a. Increased intracranial pressure
b. Chiari type II malformation
c. Syringomyelia
d. Dandy-Walker malformation
e. Tethered cord syndrome
a. Increased intracranial pressure
An 8 year-old child with a history of myelomeningocele presents with progressive back pain, scoliosis, right calf numbness, right leg weakness, incontinence, and bladder spasticity. Which of her symptoms is most likely to improve following a third untethering procedure?
a. Scoliosis
b. Back pain
c. Leg numbness
d. Bladder spasticity
e. Leg weakness
b. Back pain
A 4 year-old boy with a history of congenital hydrocephalus and VP shunt placement near birth presented one week following proximal shunt revision with lethargy, fevers to 38.4 C and erythema along the shunt tract. He underwent complete removal of the VP shunt system and placement of an external ventricular drain. CSF cultures grow MSSA and the patient is started on intravenous oxacillin, when should the shunt be replaced?
a. After 1 day of negative CSF culture
b. After 5 days negative CSF cultures
c. After 3 days negative CSF cultures
d. After 10 days of negative CSF cultures
e. After 21 days of negative CSF cultures
d. After 10 days of negative CSF cultures
A 6-month-old infant presents with enlarging head circumference, full anterior fontanelle, splitting of the cranial sutures and developmental delay. The results of a neurological examination are otherwise normal. Computed tomographic scans are obtained (Figures 1 and 2). What is the most likely diagnosis?
a. Aqueductal stenosis
b. Chiari III malformation
c. Retrocerebellar arachnoid cyst
d. Dandy-Walker malformation
d. Dandy-Walker malformation
Following resection of a frontal lobe AVM, a patient develops a sudden severe headache and decreased mental status. CT demonstrates a 4 cm hematoma, and subsequent angiography reveals residual AVM. Which management strategy is most advisable?
a. Blood pressure monitoring and repeat head CT
b. Craniotomy to address hematoma and residual AVM
c. Embolization of residual AVM
d. Elective radiosurgery for the residual AVM
e. Hypotensive therapy in the ICU
b. Craniotomy to address hematoma and residual AVM
A 10-year-old boy presents with headache and imbalance. Examination discloses mild papilledema, right-sided dysmetria, and ataxia. MRI with contrast is shown in the figure. What is the most important prognostic factor for this patient?
a. The extent of resection of the cyst walls
b. The presence of mitoses on histology
c. The presence of endothelial proliferation on histology
d. The extent of resection of the enhancing mass
e. The presence of hydrocephalus at presentation
d. The extent of resection of the enhancing mass
What molecular subgroup has the best prognosis in a child diagnosed with medulloblastoma?
a. Wnt
b. Sonic hedgehog (SHH)
c. Group 3
d. Group 4
e. Large cell
a. Wnt
A 4-year-old boy presents with progressive gait dysfunction, headache, and vomiting. A CT shows hyperdense areas suggestive of calcification. The MRI is shown (figures). What is the most likely diagnosis?
a. Medulloblastoma
b. Atypical teratoid / rhabdoid tumor (AT/RT)
c. Pilocytic astrocytoma
d. Ependymoma
e. Choroid plexus papilloma
d. Ependymoma
A 28-year-old with NF-1 has progressive headaches. Axial and coronal T1-weighted MR images with gadolinium are shown (Figures 1 and 2). The next morning she becomes suddenly unresponsive with a dilated right pupil. What is the most likely diagnosis?
a. Ganglioglioma
b. Meningioma
c. Metastatic tumor
d. Pilocytic astrocytoma
e. High-grade astrocytoma
e. High-grade astrocytoma
A 15 year-old male presents with severe low back pain. The pain responds poorly to acetaminophen but responds well to aspirin. CT demonstrates a 1.5 cm dense lytic lesion with a calcified nidus and circumferential sclerosis (figure). What is the diagnosis?
a. Fibrous dysplasia
b. Hemangioma
c. Osteochondroma
d. Osteoid osteoma
e. Oteoblastoma
d. Osteoid osteoma
A 16 year-old man with Down syndrome wishes to participate in the Special Olympics. Which of the following is recommended to screen for risk of injury?
a. EMG/NCV of single upper and lower extremity
b. Neurological exam
c. MRI scan of the cervical spine
d. Cervical spine x-rays
e. Cranial CT
d. Cervical spine x-rays
A patient who was shunted for hydrocephalus after intracerebral hemorrhage presents with severe headaches. Head CT is shown. What is the most likely diagnosis?
a. Post-hemorrhagic headache syndrome
b. Shunt malfunction
c. New intracranial hemorrhage
d. Shunt infection
e. Overdrainage
e. Overdrainage
During a pterional exposure of the sylvian fissure, you encounter significant brain swelling. In order to achieve rapid brain relaxation, identify the most appropriate point on the associated figure through which to place a ventriculosotomy and access the frontal horn of the lateral ventricle.
a. 2
b. 1
c. 4
d. 3
e. 5
a. 2
A 15-year-old presents with an incidental finding (figure). What is the most appropriate management strategy?
a. Surgical resection of the mural nodule.
b. Surgical resection of the mural nodule with a 1 cm margin.
c. Surgical resection of the mural nodule and entire cyst wall.
d. Surgical resection of the mural nodule with biopsy proven, tumor positive, cyst wall.
e. Drainage of the cyst to relieve mass effect.
a. Surgical resection of the mural nodule.
A 3 year old child with moyamoya is scheduled for an encephaloduroarteriosynangiosis (EDAS) indirect bypass. What is the most common cause of new perioperative neurological deficit?
a. Hyponatremia
b. Intracranial hemorrhage
c. Cerebral edema
d. Seizure
e. Cerebral ischemia
e. Cerebral ischemia
A 15 month old infant is brought to the emergency room 4 hours after a fall during which he sustained a cephalohematoma. He is opening his eyes when asked, is crying, and is holding onto a toy. Parents report that he has vomited twice. What is his GCS score?
a. Does not apply to preverbal children
b. Does not apply to children less then 5 years old
c. 15
d. 13
e. 12
d. 13
An 8-year-old boy was evaluated due to short stature and headaches. Sagittal MRI with contrast (figure) is most consistent with what diagnosis?
a. Arachnoid cyst
b. Craniopharyngioma
c. Medulloblastoma
d. Germinoma
e. Pituitary macroadenoma
b. Craniopharyngioma
What is a known possible side effect of corpus callosotomy for epilepsy?
a. Gerstmann syndrome
b. Superficial cerebral hemosiderosis
c. Hemibalismus
d. Hemiplegia
e. Disconnection syndrome
e. Disconnection syndrome
An 8 year-old presents with a small dimple located at the nasion which intermittently drains. MR imaging shows that this lesion does extend intracranially. His past- medical history is significant for two bouts of meningitis. What is the most common organism which causes meningitis in these cases?
a. Staphylococcus epidermidis
b. Staphylococcus aureus
c. Pseudomonas aeruginosa
d. Haemophilis influenzae
e. Propionibacterium acnes
b. Staphylococcus aureus
A 6-year-old female presents with progressive hemiparesthesia, hemiparesis, and sixth nerve palsy, all on the right side. Her CT and MRI are shown (figures). What is the most likely diagnosis?
a. Ependymoma
b. Meningioma
c. Central neurocytoma
d. Choroid plexus papilloma
e. Choroid plexus carcinoma
a. Ependymoma
A five-year-old right-handed boy has had medically intractable epilepsia partials continua for 4 years. Evaluation demonstrates developmental delay, worsening of verbal IQ and right-sided hemiparesis. EEG demonstrate several epileptogenic foci over the left parietal lobe. MRI imaging is shown. Aside from invasive monitoring, which of the following is the most appropriate surgical treatment?
a. Corpus callosotomy
b. Vagus nerve stimulation
c. Functional hemispherectomy
d. Parietal lobectomy
e. Selective amygdalohippocampectomy
c. Functional hemispherectomy
A 4-week-old was transferred to the Trauma Service from an outlying emergency department after an evaluation for a seizure that included a CT scan of the head (Figure 1). What additional diagnostic investigation is most appropriate?
a. Lumbar puncture
b. Cerebral angiography
c. Skeletal survey
d. Echocardiography
e. Urine amino acids
d. Echocardiography
A 17-year-old male presented with a heterogeneously contrast-enhancing mass within the spinal cord causing significant spinal cord expansion and compression. After laminectomy, an expansile intramedullary mass that appeared highly vascularized and infiltrative was found. A frozen section showed glioblastoma. What is the most appropriate next step in management?
a. Partial resection
b. Gross total resection
c. Primary dural closure without resection
d. Duraplasty without resection
e. En bloc resection
d. Duraplasty without resection
What type of tumor harbors the histopathological structure seen in the figure?
a. Glioblastoma
b. Pleomorphic xanthoastrocytoma
c. Pilomyxoid astrocytoma
d. Ependymoma
e. Anaplastic oligodendroglioma
b. Pleomorphic xanthoastrocytoma
