Neuropediatri Flashcards
2.5 year old child presents with macrocephaly and poor height and weight gain. He has just begun to walk, but has age appropriate language development. A head CT scan and MRI show a large pineal region lesion. Serum beta HCG and AFP are negative. The MOST appropriate procedures is:
A. Endoscopic biobsy and CSF shunt placement
B. Cerebral angiogram
C. Stereotactic Biopsy
D. Open biopsy and possible resection
B. Cerebral angiogram
In this case, the pineal region lesion is not a timor, but a vein of galen malformation. Biopsy or attempt at open resection would be disastrous. This case emphasizes the need to carefully iamge, and in appropriate cases obtain angiography, for pediatric cases that may involve vascular lesions. Vein of galen malformation present variably by age. Newborn children typically present in severe congestive heart failure due to high flow shunting through the malformation, and often macrocephaly. Older children may present first with hydrocephalus, usually with a slower flow lesion. Yasargil classified vein of galen malformation in the following manner. 1 : pure fistula of ACA or PCA branch to vein of galen, 2 : fistulous thalamoperforating vessels to vein of galen, 3 : combined lesion, and 4 : plexiform AVMs. Treatment options include endovascular techniques (both transarterial and transvenous), observation with management of hydrocephalus and rarely, open surgical techniques. Prognosis is related to age at presentation, cardiac status and presence of calcifications in the basal ganglia, as a marker of cerebral injury due to steal. In order children, non-operative management or hydrocephalus management alone can be successful. In this case, angiography showed a slow flow lesion which was seen to thrombose on flow
A 10 year old boy with a history of aqueductal stenosis and CFS shunt present with nausea, vomitting and headache and a temperature of 100.5 he had a shunt revision for similiar symptoms 3 month ago. His brother has been sick lately too, with diarrhea and abdominal pain. His CT scan shows a ventricular catheter in good position and normal ventricular size. A shunt series is norma. The MOST appropriate diagnotic test is :
A. None
B. Shunt exploration
C. Shunt tap
D. ICP monitoring
C. Shunt tap
Pasients presenting with sign and symptoms of shunt failure within the first few months after prior shunt failure are at high risk of shunt infection. Studies indicate that recent shunt revision is a risk factor for both shunt infection and mechanical shunt failure. Nevertheless, the symptoms of shunt failure are sometimes non-spesific. Shunt infection occurs at a rate of up to 12% after shunt placement and may present either as repeated shunt failure, or with overt symptoms and signs of infection. CSF analysis is essential for diagnosis.
A 10 year-old girl presents with signs of accelerated pubertal stage, convergence-retraction nystagmus, and impaired upward gaze. Which of the following lesions would likely be the cause of the findings described?
A. Pontine glioma
B. Pineal region tumor
C. Acqueductal stenosis
D. Multiple sclerosis
B. Pineal region tumor
Pineal region tumors occur in children of approximately 10 years of age. These lesions can result in a dorsal midbrain syndrome, which includes a triad of vertical gaze palsy, pupillary light-near dissociation and convergence retraction nystagmus. Convergence-retraction nystagmus is best seen with a down going optokinetic drum. While congenital aqueductal stenosis cam present with gaze palsies, it is not associated with pubertal acceleration and patiens most often an earlier age with increased head circumference or headache
While multiple sclerosis (MS) could cause gaze palsies such as that described in this question, MS usuallly present in the fourth decade of life, is much less common than pineal region tumors in this age group, and is not associated with accelerated puberty. Pontine gliomas also often present with diploplia due to abducens palsies. However, the ocular finding difer significantly from the dorsal midbrain syndrome and pontine gliomas are not associated with pubertal acceleration.
A 10-year-old boy presents compalining of headache and imbalance. Examination discloses mild pailedema, right-sided dysmetria, and taxia. Magnetic resonance imaging with gadolinium enhancememnt is shownin figure 1. The MOST important prognostic factor this patient is :
A. The extent of resection of the enhancing mass
B. The extent of resecion of the cyst walls
C. The presence of endothelial proliferation of histology
D. The presence of mitoses on histology
E. The presence of hydrocephalus at presentation
A. The extent of resection of the enhancing mass
Approximately one third of posterior fossa tumors in children are astrocytomas (with the other most common diagnoses being medulloblastoma and ependymoma). The large majority of astrocytoma are benign, and most occur in the cerebellum. The most common pathological diagnosis in juvenille pilocytic astrocytoma (JPA), with less frequent occurence of anaplastic astrocytomas and glioblastomas. The typical magnetic resonance imaging or computed tomographic appearence of JPA is a brightly enhancing mural nodule within a non-enhancing cyst, although some tumors may be mostly solid. Pathological features that carry a poor prognosis in other tumors, including frequent mitoses and endothelial proliferation, are compatible with world health organization grade 1 designation and favorable prognosis in cases of JPA. Rosenthial fibers are common but can also be seen in other pathological entities. Total incision of the enhancing nodule or mass in a cerebellar astrocytoma often results in indefinite disease-free survival. Adjecent cyst walls typically do not enhance and are not neoplastic. JPAs of similiar pathological
A 15 year old male presents with severe low back pain. The pain responds poorly to ….. but responds well to aspirin. CT demonstrates a 1.5 cm dense lytic lesion of with a calcified nidus and circumferential sclerosis. What is the diagnosis?
A. Osteochondroma
B. Osteoid osteoma
C. Hemangioma
D. Oteoblastoma
B. Osteoid osteoma
This child has an osteoid osteoma which typically presents between 10-20 years of age. The spine is involved in 10% of cases. CT reveals dense sclerosis around a lytic lesion with a central calcified nidus of osteoid and woven bone. They are usually <2cm. Large lesion should raise the suspicion of osteoblastoma. Symptoms include scoliosis and pain that respondsto nonsteroidal anti-inflammatory medications. Hemangiomas present a at peak age of 30-50 years with a female predominance. 75% are spinal and usually present in the vertebral body. CT demonstrates a honeycomb pattern. Most are asymptomatic and require no intervention.
A 15 year old patient with a history of prematurity and post-hemorrhagic hydrocephalus presents to the emergeny department with an intermittent fever of no higher than 100.2 deg . F, anorexia, and mild headache. His peripheral WBC is normal, at 9.3. The patient and his mother both deny the possibility of VP shunt malfunction. He states that “this is totally different than the symptoms i had 8 months ago when my shunt was blocked”. The MOST likely diagnos is :
A. Gastroenteritis
B. Pseudotumor cerebri
C. Proximal shunt obstruction
D. Bowel perforation
E. Abdominal pseudocyst
E. Abdominal pseudocyst
Fastidious skin bacteria may cause chronic ventricular shunt infection, which often presents as a shunt related abdominal pseudocyst. Presenting symptoms include intermittent low grade fever, abdominal fullness,anorexia, nausea and vomitting, ang headache. Fever >38,5 degrees C, rigors, meningismus, and peripheral leukocytosis are rare. Infection typically occurs weeks to months after the most recent surgical accessor or tap of the shunt system, representing the source of bacterial contamination. Ventricular shunt tap from a valve or reservoir may yield a normal or slightly elevated cerebrospinal fluid white blood cell cont. Cultures may grow slowly (up to a week after sampling) or may be negative in the case of some diffucult to culture organisms. Work-up include imaging of the abdomen to identify a peritoneal pseudocyst around the distal shunt catheter, which indicates the presence
A- 2-year-old child undergoes computed tomography for the preliminary complaint of macrocephaly (figure 1). He is developmentally normal and has normal results of the neurological examination without papilledema. Six months later, routine follow-up magnetic resonance imaging shows enlargement of the left middle fossa abnormality (figure 2) and repeat tasting reveals mild developmental delay and early papilledema. Which diagnosis is MOST likely:
A. Arachnoid cyst
B. Epidermoid
C. Abscess
D. astrocytoma
A. Arachnoid cyst
Extra-axial cyst in the central nervous system of children are most commonly arachonid cyst, although dermoid, epidermoid, neureneric, and other cyst types may occur. True arachnoid cyst have cerebrospinal fluid (CSF) imaging characteristics on computed tomographic scans and magnetic resonance images (including diffusion weighted imaging). Arachonoid cyst in the intracranial space are not commonly found in the middle fossa-sylvian fisure (appoximately 40%), posterior fossa (approximately 25%),and other locations (suprasellar, quadrigerminal cistern, interhemispheric fissure, cerebral convexity). Arachonid cyst may spontaneously enlarge, involute, or remain static. Cyst hemorrhage and associated subdural hemorrhage also are seen. Indications for surgical management are controversional but may include progressive macrocephaly, cyst enlargement, hemorrhage, headache, raised intracranial pressure, seizure, or neurological deficit. Surgical options include cyst peritoneal shunt placement. Craniotomy for cyst fenestration, and endoscopic cyst fenetration unless the cyst is fenetrated into central CSF cisterns
A year old presents with headache and vomitting. CT and MR imaging reveals the presence of a 4th ventricular tumor. A suboccipital craniotomy is performed for total resection of a medulloblastoma. Resection is followed protocol based chemotherapy and total neuroaxis radiotherapy. With a boost to the posterior fossa. Which endocrinological complication is the MOST likely after radiotherapy for a posterior fossa tumor in childhood?
A. Diabetes insipidus
B. Cushing’s syndrome
C. Growth hormone deficiency
D. Addison’s syndrome
C. Growth hormone deficiency
Central nervous system (CNS) radiotherapy in childhood poses particular risks of hipopituitarism, in particular of short stature from growth hormone deficiency. Diabetes insipidus is uncommon. Gigantism, caused by growth hormone excess in childhood, is not seen. Pituitary neoplasms caused by previous radiotherapy for other diagnoses are extremely rare. Endocrine dysfunction, shortstature and developmental delay caused by early childhood radiation generally preclude the use of cranial radiotherapy in children less than 3 years old.
A 6 year old child with a traumatic brain injury is intubated and appears agitated. His CT scan obtained within the last hour shows a left parietal contusion. Intracranial pressure monitoring via ventriculostomy shows sustained pressure above 20 mmHg, despite bucking againts the ventilator with minimal stimulation. His vital signs are within age appropriate limits and he apprears well hydrated. What is the most appropriate pharmacological management of this patient?
A. Morphine and versed
B. Pentobarbital
C. Vecuronium
D. Propofol
A. Morphine and versed
Sedation and analgesia are important adjuncts to the management of intracranial hypertension after taumatic brain injury. The correct answer in this case is morphine and versed. They facilitate maintenance of mechanical ventilation, vascular access catheters and ventriculostomyor other ICP monitors. Pain and agitation increase cerebral metabolism, thereby producing an increase in cerebral blood flow, volume and intracranial pressure. The advantages of these agents must be weighed against their adverse effects on blood pressure and the neurological examination. Paralytics and high dose barbiturates, while an option, eliminate the neurological examination. Propofol, while an exellent choice of rapidly reversible sedation in adult patients is contraindicated for long term sedation in the ICU and has been linked to a progressive acutely fatal cardiomyophaty and acidosis in children. Paralytics and high dose barbiturates eliminate the ability to perform a neurological examination, and paralytics without sedation are contraindicated.
A 9-year-old male presented to his pediatrian with headaches and growth delay. Pathological specimens of his tumor obtained at operation are shown in the photomicrographs. What is the MOST likely diagnosis:
A. Colloid cyst
B. Craniopharyngioma
C. Pilocytic astrocytoma
D. Hypothalamic hamartoma
E. Pituitary adenoma
B. Craniopharyngioma
Craniopharyngiomas represent 1-2% of all intracranial neoplasms and about 10% of the tumors of the sellar region. The current WHO classification identifies two variants: adamantinomatous (fig 1) and papillary (fig 2). The adamantinomatous type tend to be mostly cystic lesions, while the papillary craniopharyngioma is most commonly a solid lesion. Some tumors may contain variable proportions of these two histologic patterns. In children, most tumors present with endocrinologic abnormalities, such as growth retardation and diabetes insipidus, and/or visual loss. In adults, symptoms of compressive effects including visual defects and hypopituitarism may be present. Mild hyperprolactinemia due to stalk compression may also be present.
A term infant is born with a cranial malformation in the occipital region (fig 1). Which diagnosis is MOST likely?
A. Dermal sinus tract
B. Chiari III malormation
C. Myelomeningocele
D. Anencephaly
E. Encephalocele
E. Encephalocele
Congenital encephaloceles are calvarial and dural defects containing neural tissue and cerebrospinal fluid (CSF). Occipital encephaloceles are most common in north America, whereas frontoethmoidal (sincipital) encephaloceles are more common in southeast Asia. Congenital basal (transethmoidal, transsphenoidal) encephaloceles are rare. Occipital encephaloceles show a 70% female predominance. The extracranial position of some neural tissue generally results in relative microcephaly. Repair involves the resection of gliotic and nonfunctional neural tissue and dural/cranial repair. Hydrocephalus and/or CSF leak not uncommonly complicate repair, thus, placement of a ventricular shunt is often necessary. Myelomeningocele refers to similiar dysraphic defect containing CSF and neural tissue, but which are dorsally open and occur in the spine. Dermal sinus tracts in the region ofthe inion present as tiny pits with one or a few dark, dyplastic hairs; they are often missed on a causal physical examination. Chiari III malformation involves herniation of posterior fossa content through a dysraphic foramen magnum and cervical level dysraphic defect. Anencephaly is a severe open dysraphic defect of the entire telencephalon.
An 8 year old girl present with years of chronic headaches that have worsened during the past 6 months and prgressive difficulty in school. She had previously been an ‘A student’ but her grades have dropped to Cs, and she complains of problems with memory, attention, and coordination. She has mild papilledema. You recommend endoscopic treatment of her obstructive triventricular hydrocephalus rather than VP shunt placement. During endoscopic exploration of the ventricular system, the MOST appropriate site for surgical fenestration is:
A. Septum pellucidum superior to the fornices
B. 3rd ventricle floor anterior to the infundibular recess
C. 3rd ventricle floor anteior to the mammillary bodies
D. Lamina terminalis above the suprachiasmatic recess
C. 3rd ventricle floor anteior to the mammillary bodies
Triventricular hydrocephalus, dilation of the lateral and third ventricles, result from obstruction of cerebrospinal fluid flow in the posterior third ventricle, most commonly resulting from conginetal acqueductal stenosis, hamartomas of the rectal plate, or pineal region tumors. Macrocephalus, headache, papilledema, visual loss, cognitive deterioration, and other signs of raised intracranial pressure, including death, may result. The gold standard therapy for >40 years has been ventricular shunting, most commonly to the peritoneal space. However, ventricular shunts are subject to mechanical and infectious complications and may result in extra-axial fluid or blood collections due to cortical mantle collapse, particularly in patients such as this one with severe ventriculomegaly. Alternatively, endoscopic third ventriculostomy represents a potentially curative single surgical intervention by which the posterior third ventricular blockage is bypassed into the ventral subarachnoid space(prepontine cistern). The ventriculoscope is introduced via a pre-coronal.
Children of mothers with diabetes mellitus have an increased insidence of which spinal disorder?
A. Meningocele manque
B. Sacral agenesis
C. Spinal dysraphism
D. Thoracic hemivertebrae
E. Intraspinal lipomas
B. Sacral agenesis
Sixteen percent of children with sacral agenesis or caudal regression are born to diabetic mothers and 1% of diabetic mothers have children with these conditions. The overall incidence of spinal dysraphism has decreased 26% in the US by folate supplementation of cereal making this as well as meningocele manque´, and intraspinal lipomas incorrect choices. Active or passive exposure to tobacco smoke has been linked to preterm delivery or low birth weight, another example of a maternal activity or condition being linked to a neonatal condition. There is no data to support neonatal thoracic hemivertebrae being increased in mothers with diabetes.
Current pediatric head injury guidelines suggest maintenance of the minimum cerebral perfusion pressure above 40 to 65 mmHg, depending on :
A. Skull thickness
B. Patient age
C. Status of fontanelle
D. Core temperature
E. Time since injury
B. Patient age
As in adult patients, the insidence of intracranial hypertension incerases with a declining GCS score. Limited clinical studies have suggested that a sustained ICP of > 20 is associated with significantly worse outcome than if less than 20. Intuitevely, for younger children and particulary infants, ICP treatment thresholds of ,10 would seem to offer better outcomes, but no study has to this point has demonstrated this. Cerebral pefusion pressure targets for children are imprecisely known. One study has suggested that CPP of <40 mmHg is associated with a poor outcome, while expert opinion holds that velues between 40-65 mmHg, dependent on age, are appropriate targets. An open fontanella does not precludethe development of intracranial hypertension, nor obviate the utility of ICP monitoring. Diffuse cerebral edema after trauma without intracranial hemorrhage is thought to be more common in children than adults, and may be an indication for ICP monitoring even when no evert mass lesion is present. ICP monitoring for children even with GCS scores of 9 or greater mey be indicated for certain mass lesions or if the patient cannot be followed with serial neurological examinations
The incidence of myelomeningocele has decreased during the past decade. Before the 1980s, the incidence was 1 to 2 per 1000 live births. The current incidence is 3 per 10.000 live births. One reason for this decline may be the recommendation by physicians and obstetricians for women to supplement their folic acid intake. The american college of obstetrics and gynecology recommends the following supplementation guideline to all women from menarche to menopause:
A. 400 micrograms every day
B. 800 microgramsduring first trimester
C. 4 gram every day
D. 4 gram every week
E. 400 micrograms after positive pregnancy test
A. 400 micrograms every day
The incidence of infants born with spina bifida has been declining for 50 years in the most areas of the word. The incidence in 1984 was 5.9 per 10.000 live births. The factors contributing to decline include improved maternal nutrition, prenatal screening and diagnosis, selective pregnancy termination, undetermined enviromental changes, and vitamin supplementation, aspecially folat. The incidence decrease from east to west in the united states. It is higher among females and is much less common among blacks and asians. It is increased with poverty and poor nutrition. The american college of obsetrics and gynecology recommends 400 micrograms daily of folic acid supplementation (in addition to a healthy diet) to all women capable of becoming pregnant. Because the full benefit of folic acid is seen in mothers who begin supplementation at least 3 months before conception and continue it throughout pregnancy.
