Pediatric Flashcards
(98 cards)
A four-year-old presents with occipital headaches and a lesion on brain MRI. He also has café-au-lait spots and axillary freckling. Patients with his disease are most likely to have a tumor in what intracranial location?
A. Brainstem
B. Cerebellum
C. Cerebral cortex
D. Optic pathway
E. Auditory canal
The correct answer is optic pathway. The most common tumor in NF1 children is the optic-pathway glioma. These are detected in approximately 15%-30% of patients with NF1. The most common imaging abnormalities in patients with NF1 are focal areas of increased intensity on T2-weighted images. These areas occur in 60-80% of patients with NF1 and can occur in the basal ganglia, internal capsule, brainstem and cerebellum. These lesions tend to increase in numbers early in childhood and decrease later in childhood suggesting they are not tumors but rather abnormalities in myelination. Cerebellar and hemispheric gliomas are rare lesions in NF1 patients. Brainstem tumors are also less common and are differentiated from unidentified bright spots because the brainstem tumors produce definite mass effect.
What finding is more frequently associated with nasal and occipital dermoid cysts compared to other locations?
A. Intracranial extension
B. Hydrocephalus
C. Superinfection
D. Cyst rupture
E. Malignant transformation
Dermoid cysts are the most common congenital lesion of the scalp and calvarium encountered by the pediatric neurosurgeon. They result from failure of dysjunction of the cutaneous and neuroectoderm and contain epithelium, hair follicles, sebaceous glands, and other cutaneous elements (Figure 1). Nasal and midline occipital dermoid cysts are more likely to be associated with a small overlying pit or tract and have a higher risk of intracranial and intradural extension (generally between the leaves of the falx cerebri or falx cerebelli, respectively). Extracranial cysts present as palpable or enlarging lumps (Figure 2), or with local infection. Cysts with intracranial extension may also present due to recurring meningitis or, rarely, with intracranial mass effect. The treatment in all cases is surgical extirpation. Cyst rupture can occur with any dermoid cyst location. Hydrocephalus and malignant transformation are equally unlikely.
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. Bedside subdural drain
B. Craniotomy/craniectomy
C. Sinus surgery
D. Lumbar puncture
E. Burr holes
Craniotomy/Craniectomy. Male gender, African American race, age in the early teens, and a history of asthma impart an increased risk to intracranial suppurative infections. In a patient with hemiparesis, seizure, mass effect, and midline shift from a subdural empyema, the next appropriate step in management would be craniotomy (usually emergently). The goal of surgery is to drain the empyema to relieve mass effect and obtain microbiological specimens. Often the craniotomy is converted to a craniectomy due to underlying parenchymal edema. Large craniotomy / craniectomy bone flaps have been championed over burrholes or bedside subdural drain as purulent subdural collections tend to be extensive, loculated, and associated with cerebral edema. Identification of the infectious organism is via direct empyema sampling rather than lumbar puncture since the latter may precipitate herniation in the setting of intracranial mass effect from the empyema. Although sinus surgery is indicated for source control, it should occur after the craniotomy / craniectomy.
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. Intracranial hypotension
C. Benign enlargement of the subarachnoid spaces
D. Ruptured arachnoid cyst
E. Subdural empyema
The above clinical scenario describes a common presentation of non-accidental trauma. The child has a history consistent with increased intracranial pressure (irritability and vomiting) and has had a significant enlargement in head circumference. On examination, he is also noted to have a full fontanelle which raises further concern for elevated intracranial pressure. Imaging reveals enlargement of the subdural spaces with presence of a collection that is hyperdense to cerebrospinal fluid (CSF), consistent with bilateral subdural hematomas. This finding further raises the concern for non-accidental trauma. In cases of suspected abuse, it is important to involve the appropriate clinicians, authorities, and social work personnel for a complete assessment and evaluation of the child. Further testing may include skeletal survey and ophthalmologic evaluation to confirm the suspected abuse. Reporting requirements for abuse vary by state and institution, so clinicians should familiarize themselves with their proper local practices. Benign enlargement of the subarachnoid spaces (BESS) is characterized by accumulation of cerebrospinal fluid in the subarachnoid spaces due to an unclear etiology. Patients with this condition have enlarged head circumference and may have a full fontanelle, but generally do not have symptoms of raised intracranial pressure such as irritability and vomiting. Imaging in these cases shows enlarged convexity subarachnoid spaces with fluid isodense and isointense to CSF. In this patient, the presence of hyperdense fluid in the subdural space and symptoms of raised intracranial pressure are inconsistent with BESS. Subdural empyema is hypodense on CT. Ruptured arachnoid cyst would result in fluid isodense to CSF. A patient with intracranial hypotension would not have a full fontanel.
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. Fibrous dysplasia
B. Osteoblastoma
C. Dermoid cyst
D. Eosinophilic granuloma
E. Osteoid osteoma
Based on the insidious, subacute course and the typical imaging appearance of expansile lytic lesions with scalloped borders arising in the diploe and with variable involvement of the inner and outer tables, the presumptive diagnosis is eosinophilic granuloma (EG). After dermoid cyst and ossifying cephalohematoma, EG is the next most common bump on the skull in childhood. School age children with skull lesions should be staged under the direction of a pediatric oncologist, but they less commonly exhibit multisystem involvement, diabetes insipidus, or progressive development of new lesions. Aggressive disease is a concern in infants and toddlers. Curettage of skull lesions is indicated for diagnosis and for relief of discomfort, but if the clinical history and the imaging findings are sufficiently characteristic, the condition can be managed expectantly, presuming a systemic work up is negative. Isolated EG of the skull follows a self-limited course with healing over weeks to months. Doubt has been expressed whether treatment, such as curettage, local steroid injection or low-dose radiotherapy, has any effect on the rate or the completeness of the disappearance of isolated osseous lesions.
What is the etiology of malignant cerebral edema in children after traumatic brain injury?
A. Cerebral hypoperfusion
B. Loss of autoregulation
C. Nonconvulsive status epilepticus
D. Venous sinus thrombosis
E. Acute hydrocephalus
Loss of cerebral vascular autoregulation and subsequent hyperemia is hypothesized to contribute significantly to post-traumatic malignant edema. In these situations, the patient, usually a child, rapidly deteriorates and ICP escalates and fails to respond to typical ICP maneuvers. The condition is nearly always fatal. Although primarily studied in adults, seizures and nonconvulsive status epilepticus occur in one in five patients with moderate to severe closed head injury and result in increased ICP, but they are not felt to be the cause of post traumatic malignant edema. Venous sinous thrombosis can occur but is rarer and usually does not lead to such a rapid and irreversible decline. Acute hydrocephalus does not result in malignant edema and does not generally develop as rapidly. Loss of cerebral perfusion is the final common pathway of brain death in such patients and others who succumb from increased intracranial pressure.
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 12 months of age
C. At 18 months of age
D. At 24 months of age
E. In the next several days
In the next several days
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. Atypical teratoid/rhabdoid tumor (AT/RT)
B. Medulloblastoma
C. Choroid plexus papilloma
D. Ependymoma
E. Pilocytic astrocytoma
The most likely diagnosis in this patient is ependymoma. Common radiological findings of ependymomas are well-demarcated lesions, with variable enhancement after contrast injection, hypointense on T1, and hyperintense on T2/FLAIR. When located in the fourth ventricle, they appear to be originating from the floor of the fourth ventricle. Calcifications and cystic degeneration can be seen. The extension within the subarachnoid spaces of the posterior fossa (foramen of Luschka, foramen of Magendie, cerebellopontine angle) is commonly seen and highly suggestive of ependymoma.
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. Holoprosencephaly
B. Agenesis of the corpus callosum
C. Kallman’s syndrome
D. Focal cortical dysplasia
E. Metabolic disorder
Probst bundles are seen in patients with agenesis of the corpus callosum (ACC). They are longitudinal callosal fibers that have failed to cross the midline, and so instead form large tracts that run parasagitally, parallel to the medial walls of the lateral ventricle, causing invagination of the medial borders at the level of the frontal horns. This medial concavity of the frontal horns, along with the lumen of the third ventricle, creates the typical “bull’s head” appearance seen on a coronal MRI in a patient with agenesis of the corpus callosum. Though ACC may be caused by a multitude of factors, including various mutations, injury, or infection, it represents an insult to the developing brain during the 12th to 22nd week of gestation. Normal development of the CNS is a highly orchestrated process, with specific steps occurring at specific time points. The development of the human nervous system can be broken down into a few general processes, starting with neurulation, which occurs during the first four weeks of gestation. This is followed by extensive neuronal proliferation, generally occurring between 4 and 12 weeks of gestation. The newly generated neurons then migrate from their location of origin to their targeted locations in the adult brain. Neural migration begins at about 12 weeks of gestation and continues until birth, again, in a very stereotyped “inside-out” pattern, with structures such as the corpus callosum appearing at around 12 weeks, followed by migration of cells out toward the cerebral cortex. As neurons reach their final locations, apoptosis and synaptogenesis are critical processes for the development of mature neural networks. Probst bundles are not seen in the other answer choices.
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. Primary dural closure without resection
C. Duraplasty without resection
D. Gross total resection
E. En bloc resection
Duraplasty without resection
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. Dandy-Walker malformation
D. Retrocerbellar arachnoid cyst
In this case, the absence of vermis and the presence of hydrocephalus together suggest the diagnosis of Dandy-Walker malformation, which carries a high rate of association with other central nervous system (CNS) and non-CNS (especially cardiac) anomalies. Arachnoid cysts in the retrocerebellar space may be associated with deformation of the cerebellar vermis, 4th ventricle and even brainstem, and obstructive hydrocephalus. However midline cerebellar agenesis is generally not present. Mega cisterna magna is a normal variant, in which formation of the cerebellum is normal but a relatively large, non-compressive and non-isolated retrocerebellar CSF collection exists (enlarged cisterna magna). Aqueductal stenosis also causes obstructive hydrocephalus, but on the basis of obstruction of CSF flow at the level of the midbrain. While aqueductal stenosis may be found in children with Dandy-Walker malformation, it is not the best diagnostic choice in this case. Chiari III malformation consists of cerebellar and other posterior fossa tissue herniating through an enlarged foramen magnum and into a congenital cervical meningocele.
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. MRI scan of the cervical spine
B. Neurological exam
C. Cervical spine x-rays
D. EMG/NCV of single upper and lower extremity
E. Cranial CT
There is a significant incidence of craniovertebral instability in Down syndrome (14% - 24%). The prevalence of bony abnormalities such as os odontoideum, hypoplastic odontoid process, and rotatory atlantoaxial subluxation in Down syndrome causes concerns regarding participation in the Special Olympics. Therefore, it is recommended that screening cervical spine x-rays be obtained. Abnormal findings, such as an abnormal atlantodental interval, should lead to further evaluation.
What factor has had the single largest effect on the incidence of myelomeningocele in the United States over the past 30 years?
A. Folate supplementation
B. Antenatal diagnosis followed by termination
C. Changes in population demographics
D. Decreased numbers of children being born
E. Decreased use of seizure medication during pregnancy
The correct answer is folate supplementation. Folate is a coenzyme required for hematopoiesis and metabolism. A randomized double blind study in 1991 demonstrated 71% reduction in risk of repeat birth with neural tube defects (NTD) in the group taking 4 mg. Women with pregnancy complicated by NTD appear to utilize folate less effectively than others resulting in lower erythrocyte folate levels. Antenatal diagnosis followed by termination has had some effect but not to the degree seen with folate supplementation. Population changes may actually have increased the chances of neural tube defects as they are more common among Hispanics.
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. Neurofibromatosis-1
B. Ataxia-telangiectasia
C. Tuberous sclerosis
D. Neurofibromatosis-2
E. Von Hippel-Lindau
NF-1 Answer, neurofibromatosis-1 (NF1). This is one of the most common gentic disorders, and involves mutations/deletion of neurofibromin gene on 17q, which acts as a tumor suppressor by negatively regulating the ras oncogene. It is inherited in an autosomal dominant manner, though almost 50% of cases arise sporadically from new mutations. In an attempt to reflect the diverse nature of the disease, the NIH put out consensus criteria for the diagnosis of NF1 and NF2. These dignostic criteria are met if a person has two or more of the following: 1) Six or more cafe-au-lait macules that have a maximum diameter of greater than 5 mm in prepubertal patients and geater than 15 mm in post pubertal patients 2) Two or more neurofibromas of any type, or one plexiform neurofibroma 3) Freckling in the axillary or inguinal region 4) Optic glioma 5) Two or more lisch nodules 6) A characteristic osseous lesion, such as sphenoid wing dysplasia or thinning of long bone cortex 7) A first-degree relative with NF1 by the above criteria
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. Post-operative radiation therapy does not confer improved survival benefit.
B. Surgical resection has little effect on survival.
C. The Prognosis is worse in this patient than in a 20-year-old with the same tumor
D. The prognosis in this patient is better than that of a 2-year old child with the same tumor pathology located in the spine.
The prognosis is worse in this patient than in a 20-year-old with the same tumor The correct answer is that the prognosis is worse in this patient than in a 20-year old with the same tumor stage and grade. This child has a posterior fossa ependymoma. The prognosis of this tumor is primarily based on the extent of surgical resection. These tumors carry a worse prognosis in children, especially those younger than 3 years. Spinal ependymoma carry an improved prognosis when compared to intracranial masses. The treatment of ependymoma involves surgical resection and radiation therapy. It does not typically involve chemotherapy.
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. Internal debulking and resection until the normal-abnormal boundary is indistinct
B. Multiple sample biopsies for diagnostic confirmation
C. Divide dentate ligaments to permit spinal cord rotation
D. Gross total resection along the tumor capsule
E. Duraplasty and laminoplasty with no further tumor resection
Internal debulking and resection until the normal-abnormal boundary is indistinct In children, the most common intramedullary spinal cord tumor is a pilocytic astrocytoma. These tumors do not typically demonstrate a clear plane of demarcation from the spinal cord. While they appear well-circumscribed and often have a cystic appearance on imaging, spinal astrocytomas lack a true plane between tumor tissue and the normal spinal cord. Accordingly, the risk of resection must be weighed against neurological impairment. Despite the lack of a surgical plane of dissection, childhood spinal cord astrocytomas can be debulked internally and resected until the normal-abnormal boundary becomes indistinct.
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. Posterior fossa decompression
B. Anterior transoral odontoid resection
C. Ventriculoperitoneal shunt
D. Syringo-subarachnoid shunt
E. Posterior cervical decompression
Posterior fossa decompression Suboccipital or posterior fossa decompression has long been used as part of the surgical treatment for syringomyelia related to Chiari I malformation. In approximately 50% of patients with Chiari I malformation and syringomyelia is present. Posterior fossa decompression in patients with Chiari I malformation and syringomyelia is an effective and safe initial treatment. Anterior transoral odontoid resection, placement of a syringosubarachnoid shunt and posterior cervical decompression are typically reserved for patients if further abnormalities of the craniocervical junction exist or standard decompressive techniques fail or cannot be applied. Ventriculoperitoneal shunt placement is not an appropriate initial treatment option in the absence of hydrocephalus.
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. Pyloric ultrasound
B. Measurement of parents’ head circumferences
C. Blood lead level
D. Dilated funduscopic examination
E. Urine amino acids
Dilated Fundoscopic examination The diagnosis is chronic subdural hematoma. The differential diagnosis of macrocephaly in infancy includes hydrocephalus, chronic subdural hematomas, and benign macrocrania. Benign macrocrania is by far the most common of these 3 conditions. It is a transient, developmental phenomenon affecting otherwise healthy children who have flat or concave fontanels on physical examination. Often one parent has a head circumference at or above the 95th percentile suggesting familial component. It can be associated with early gross motor delay attributable to the disproportion between the size of the head and the development of neck and axial musculature, but, in the absence of any associated condition, long-term developmental prognosis is excellent. CT scans show expanded convexity subarachnoid spaces, prominent sulci and cisterns, and occasional mild ventricular dilatation. Distinction from chronic subdural hematoma on CT scan can sometimes be difficult. MR imaging allows visualization of blood vessels within the extra-axial collections, confirming that the extracerebral fluid is CSF in the subarachnoid spaces. Brain imaging normalizes later in childhood. No treatment is indicated.
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. Choroid plexus papilloma (CPP)
B. WHO grade III ependymoma
C. Medulloblastoma
D. Atypical teratoid/rhabdoid tumor (AT/RT)
E. Immature teratoma
Atypical teratoid/rhabdoid tumor (AT/RT) All of these tumors occur in infants (children <1 year old) and can present with obstructive hydrocephalus. All these tumors can occur in the posterior fossa; however, immature teratomas tend to occur supratentorially, as do choroid plexus papilloma, making these options less likely. In fact, CPP, unlike most brain tumors, have a reverse epidemiological occurrence: in adults, they tend to occur in the posterior fossa, but in children, they are almost always supratentorial. All these tumors can also hemorrhage, although much less likely in ependymoma, medulloblastoma and choroid plexus papilloma, compared to AR/RT. Ependymoma in the posterior fossa tend to involve the ventricular space and/or the cerebellopontine angle. Choroid plexus carcinoma involves the ventricular system as well, but more often in the lateral ventricles and trigone. AT/RT is the most likely diagnosis for a child this young, compared to the other options. AT/RT present in early childhood (median age less than 2-3 years), whereas medulloblastoma typically occur in mid-childhood (median age 6 years). Lastly, the genetic marker is exclusive to AT/RT. Rhabdoid cells are a hallmark of this tumor, but represent only a portion of the tumor and may not be detected on a biopsy sample. Otherwise, this tumor is radiographically and histologically indistinguishable from medulloblastoma or supratentorial primitive neuroecto dermal tumor (PNET). In the 2016 WHO classification scheme, AT/RT diagnosis requires confirmation of a genetic abnormality – loss of the SMARCB1 / INI tumor suppressor gene on chromosome 22. The age and presentation are most likely to be AT/RT, and with the genetic information provided in the question stem, AT/RT is the only option.
In semilobar holoprosencephaly, brain imaging is most likely to show what findings?
A. A near-normal ventricular system; lack of the genu of the corpus callosum, with the rest of the corpus callosum identifiable; near-normal thalami
B. A single ventricle; complete lack of corpus callosum and interhemispheric fissure; fused thalami
C. A normal ventricular system, corpus callosum, and thalami
D. A normal ventricular system and corpus callosum; failed separation of the posterior frontal and parietal lobes; near-normal thalami
E. A single ventricle anteriorly, with some separation of the occipital horns; dysgenesis of the anterior corpus callosum; may or may not have fused thalami
A single ventricle anteriorly, with some separation of the occipital horns; dysgenesis of the anterior corpus callosum; may or may not have fused thalami Brain imaging in this patient is most likely to show a single ventricle anteriorly, with some separation of the occipital horns; dysgenesis of the anterior corpus callosum; with or without fused thalami. Holoprosencephaly (HPE) the most common developmental disorder of the human forebrain, results from incomplete cleavage of the forebrain into two distinct hemispheres, and is often associated with midline craniofacial abnormalities. As with many congenital malformations, holoprosencephaly can range in severity from mild to severe. The severity of the craniofacial anomalies mirrors the severity of the brain anomalies. Most cases are so severe the fetus does not survive to term. Of the children that do survive, it is more common in females than males; is often associated with mutations of the sonic hedgehog gene (SHH), TGIF, ZIC2, or SIX3; and may be passed down in an autosomal dominant inheritance pattern, with approximately 80% penetrance. Traditionally, holoprosencephaly has been divided into three subtypes. Alobar HPE, the most severe survivable form, is characterized by complete absence of midline division. On neuroimaging, a single “monoventricle,” often associated with a dorsal cyst, complete lack of a corpus callosum and interhemispheric fissure, absence of the olfactory bulb and tracts, as well as fusion of the thalami and often other deep nuclei can be seen. In its most severe form, alobar HPE is associated with microcephaly, cyclopia, proboscis, and midline clefting.
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. New intracranial hemorrhage
B. Shunt malfunction
C. Overdrainage
D. Shunt infection
E. Post-hemorrhagic headache syndrome
Overdrainage The patient is suffering from overdrainage from the shunt from which has resulted in a subdural hygroma. Patients with shunts, particularly those with communicating hydrocephalus who may be only partially shunt dependent can have overdrainage through their shunt system resulting in signs and symptoms of low intracranial pressure. This is a complication from shunt placement described as overdrainage. The subdural hygromas can be treated with adjustment of the shunt valve setting or drainage depending on the clinical setting. Although patients can present with extraventricular hydrocephalus, this would be a less likely presentation and shunt malfunction with recurrent hydrocephalus would be more likely to present with ventricular enlargement in such a patient. There is no evidence of hemorrhage on CT and no history to suggest this diagnosis. Shunt infection would present with shunt malfunction, hydrocephalus, and systemic symptoms of meningitis and infection. A diagnosis of headache is one of exclusion and should never be considered in the setting of an abnormal imaging study that suggests a technical or structural explanation for the patient’s symptoms.
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. Intracranial hemorrhage
B. Seizure
C. Cerebral edema
D. Cerebral ischemia
E. Hyponatremia
Cerebral Ischemia Patients with moyamoya syndrome are at risk of stroke from pre and perioperative anesthetic maneuvers commonly used in other patients undergoing neurosurgical procedures. Because there is baseline ischemia and lack of cerebral autoregulatory reserve, factors which increase metabolic demand, such as hyperthermia and pain, increase the risk of perioperative stroke. Similarly hypocapnia, hypoxia and hypotension must be rigorously avoided in the operative and perioperative period. While moyamoya patients are also subject to other common causes of perioperative morbidity, such as hyponatremia, seizure, and hemorrhage, they are at particular risk for ischemic injury. Cerebral edema is uncommon in perioperative moyamoya patients due to the minimal manipulation involved in indirect bypass.
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 children less then 5 years old
B. 12
C. Does not apply to preverbal children
D. 15
E. 13
13 The child in this case opens his eye to voice (E3), is crying (V4), and holding onto a toy (M6). This gives him a pediatric GCS of 13. See a table below for pediatric GCS by age. The Pediatric GCS was developed for examination of preverbal children <5. The most sensitive and specific score is the combined score. While the motor subscore has been shown to be the most predictive score in adults with severe TBI, in children the verbal subscore is the most predictive.
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 Heart Disease
B. Congenital Adrenal Hyperplasia
C. Celiac Sprue
D. Prader-Willi Syndrome
E. Rickets
Rickets The patient described in the question has Rickets and osteomalacia due to insufficient dietary Vitamin D. There appears to be a strong association between Rickets of all forms (both normo and hypocalcemic) and craniosynostosis. Curranrino noted almost 1/2 of patients with hypophosphatemic Rickets (most with X-linked) suffered from sagittal synostosis. None of the other disease noted have any consistent association with craniosynostosis.
