PEDIATRIC NEUROLOGICAL CONDITIONS

1. PEDIATRIC NEUROLOGICAL CONDITIONS Unit 4: Part 2 Module 3

2. REFERENCES C 290- Nelson�s Essentials of Pediatrics C 306- Toronto Sick Kids manual 277- Toronto Notes 2001 Anti-infective Guidelines Class handout

3. OUTLINE Embryonic Development of the CNS Primary Pathways and Symptoms Of Neurological Disorders in Children Neural Tube Defects Acute Pediatric Coma Seizure Disorders

4. OUTLINE Headaches Head Injuries Meningitis Hypotonia in the Neonate Hydrocephalus Childhood Neurological Malignancies

5. EMBRYONIC DEVELOPMENT OF THE CNS CNS arises from the neural plate of embryonic ectoderm Neural plate gives rise to neural tube Neural tube forms the brain , spinal cord and neural crest cells which form the peripheral NS, meninges, melanocytes and adrenal medulla NT begins to form on day 22 of gestation The lumen of the NT forms the ventricles and central canal of the spinal cord

6. PRIMARY PATHWAYS OF CNS DISORDERS Congenital anomalies e.g NTD- spina bifida Congenital inborn errors of metabolism e.g. Infection - e.g meningitis Neoplasm - brain tumors Vascular anomalies - e.g. AV malformation

7. PRIMARY PATHWAYS OF CNS DISORDERS Nutritional deficiency Autoimmunity Cellular degeneration Intoxication - acute poisoning Trauma - e.g acute head injury Anoxia

8. PRIMARY SIGNS & SYMPTOMSOF CNS DISORDERS Cognitive deficits- memory,mood , concentration, personality changes Seizures Headache Dizziness These S&S may be congenital or acquired, acute or chronic, progressive or static, reversible or irreversibleThese S&S may be congenital or acquired, acute or chronic, progressive or static, reversible or irreversible

9. PRIMARY SIGNS & SYMPTOMSOF CNS DISORDERS Vision or hearing loss Impairment of swallowing or respiration Weakness/numbness/paresthesia Difficulty walking/talking Incontinence

10. NEURAL TUBE DEFECTS Spina bifida - Defective closure of the caudal end of NT at the end of 4th week of gestation - Results in anomalies of the lumbar and sacral vertebrae or spinal cord - Range of severity of CNS affects - Preventable with pre-conceptual Folic acid supplements 0.4 mg /day When a fetus is developing, the arches of the vertebra grow and close around the spinal cord to protect it. In some cases, however, part of the arch (known as the "laminar arch") fails to develop and does not close around the spinal cord. This is called "spina bifida."Sometimes there are no problems found in patients with this defect (spina bifida oculta). At other times, however, it can lead to severe neurological problems. It can occur at any level of the spine or even at several levels in the same patient. The most common location is towards the bottom of the spine. There is an increased risk of spina bifida occurring in the child of a parent who has the condition. When a fetus is developing, the arches of the vertebra grow and close around the spinal cord to protect it. In some cases, however, part of the arch (known as the "laminar arch") fails to develop and does not close around the spinal cord. This is called "spina bifida."Sometimes there are no problems found in patients with this defect (spina bifida oculta). At other times, however, it can lead to severe neurological problems. It can occur at any level of the spine or even at several levels in the same patient. The most common location is towards the bottom of the spine. There is an increased risk of spina bifida occurring in the child of a parent who has the condition.

11. NEURAL TUBE DEFECTS Spina bifida "oculta" (meaning "hidden" in latin) - There may be no signs or symptoms - The spinal arch has not closed, but the spinal cord underneath has retained its normal position and is not damaged - Skin of back intact, small dimple or tuft of hair may be present over affected vertebrae - A child could grow up and never know that he or she has the defect These patients only have an increased risk of developing a condition called "spondylolisthesis." In other patients with a lipomeningocele ("lipo" meaning "fat"), a fatty mass pokes through the open part of the spinal arch. The soft mass can be felt in the back. A dimple in the back or a hairy spot may also be seen. These patients only have an increased risk of developing a condition called "spondylolisthesis." In other patients with a lipomeningocele ("lipo" meaning "fat"), a fatty mass pokes through the open part of the spinal arch. The soft mass can be felt in the back. A dimple in the back or a hairy spot may also be seen.

12. More ominous signs of spinal cord damage that might result if the cord or the membranes covering it have protruded through the open arch (called "myelomeningocele") are muscle weakness, atrophy (i.e. a decreased size) of the calf or thigh muscle, and loss of function of the bladder. At birth, a child with spina bifida should be seen by an experienced neurologist. This doctor can ask for consultations with a neurosurgeon, pediatric orthopaedic surgeon, or urologist as needed. Genetic counseling is generally available for patients. It is also important to avoid exposure to latex and latex containing products. Patients with spina bifida are more likely to get a clubfoot More ominous signs of spinal cord damage that might result if the cord or the membranes covering it have protruded through the open arch (called "myelomeningocele") are muscle weakness, atrophy (i.e. a decreased size) of the calf or thigh muscle, and loss of function of the bladder. At birth, a child with spina bifida should be seen by an experienced neurologist. This doctor can ask for consultations with a neurosurgeon, pediatric orthopaedic surgeon, or urologist as needed. Genetic counseling is generally available for patients. It is also important to avoid exposure to latex and latex containing products. Patients with spina bifida are more likely to get a clubfoot

14. ANENCEPHALY Defective closure of the rostral neural tube results in anencephaly or encephalocele Neonates with anencephaly have a rudimentary brainstem , or midrain , no cortex or cranium Rapidly fatal condition if born alive

17. NEURAL TUBE DEFECTS Diastemamyelia - A bone or fibrous band divides spinal cord in two longitudinal sections - Associated lipoma may be present, which tethers cord to vertebra - S&S include weakness, numbness in feet, urinary incontinence, decreased or absent reflexes in feet - Rx - surgery to free cord

18. ENCEPHALOCELE Skull defect with exposure of meninges alone or meninges and brain Sometimes defect can cause protrusion of frontal lobe through the nose Recurrence rate in subsequent pregnancies of NTD or cranial defects is about 10%Recurrence rate in subsequent pregnancies of NTD or cranial defects is about 10%

20. MACROCEPHALY Results from: Macrocrania- increased skull thickness Hydrocephalus- enlarged ventricles Megalencephaly -enlargement of brain Causes of Macrocrania include diseases of bone metabolism or hypertrophy of bone marrow secondary to hemolytic anemia Causes of megaencephaly include disorders causing proliferation of brain tissue e.g neurofibromatosis, tuberous sclerosis or an accumulation of abnormal metabolic substances as seen in Tay Sach�s Causes of Macrocrania include diseases of bone metabolism or hypertrophy of bone marrow secondary to hemolytic anemia Causes of megaencephaly include disorders causing proliferation of brain tissue e.g neurofibromatosis, tuberous sclerosis or an accumulation of abnormal metabolic substances as seen in Tay Sach�s

21. MICROCEPHALY Causes include: - Premature closure of skull sutures ((craniosynostosis) - Microencephaly - small brain due to insult ( infectious, toxic, metabolic, vascular) sustained in the perinatal or early infancy period e.g rubella,CMV, Fetal alcohol syndrome - Genetic disorder - microencephaly vera - Many syndromes and metabolic disorders are associated See table 18-1, PG 769, Nelson�s

22. PEDIATRIC COMA Most common pattern in children is diffuse impairment of cerebral hemispheres Less commonly results from brainstem dysfunction

23. PEDIATRIC COMA Useful mnemonics for Differential Dx of causes: T - trauma I - insulin/hypoglycemia/inborn errors of metabolism/intususception P - Psychiatric S - seizures, stroke, shock, shunt malfunction

24. PEDIATRIC COMA A- alcohol abuse E- electrolytes, encephalopathy, endocrinopathy I - infection O - overdose/ingestion U - uremia

25. PEDIATRIC COMA-INITIAL APPROACH Primary Survey - ABC;s - C-spine precautions - Pediatric Glasgow Coma Scale - Vital signs including rectal temperature - Check for signs of obvious trauma - Check for S&S of raided ICP - Hypoglycemia- give glucose 0.5 g/kg( D50W, 1-2 ml/kg IV empirically - chemstrip sugar low - Narcan empirically 0.1 mg/kg if pupils small/pinpoint

26. PEDIATRIC COMA-INITIAL APPROACH Secondary survey - History - known underlying cause, acute fever, trauma, ingestion, PMH, Medications, allergies, last meal - General CPX including CNS exam - Look for evidence of infection, intoxication, traumatic and metabolic causes - Fontanelle, neck stiffness, neck bruits, fundi( retinal hemorrhages), oculomotor movements - Breathing patterns - Motor responses ( focalizing/lateralizing signs)

27. PEDIATRIC COMA-INITIAL APPROACH Investigations - depends on potential etiology and clinical condition Blood work may include: - CBC, cultures, glucose, electrolytes, BUN, creatinine, Calcium, magnesium, LFT�s, ammonium, blood clotting screen, ABG

28. PEDIATRIC COMA-INITIAL APPROACH Diagnostic Imaging - CT of head essential if focal causes suspected e.g trauma not if diffuse cause e.g infection - CRX, C-spine XR, Flat plate of Abdomen, limb XR - urinalysis, C&S, latex agglutination - LP- CSF analysis - ECG - EEG LP contraindicated in profound coma, evidence of increased ICP or if hemodynamic instabilityLP contraindicated in profound coma, evidence of increased ICP or if hemodynamic instability

29. PEDIATRIC COMA-INITIAL APPROACH Further management directed at underlying cause Serial Glasgow coma scale assessments Maintain homeostasis with: - Oxygen/CO2 - IV fluids - Acid base , electrolytes - Nutrition

30. PEDIATRIC GLASGOW COMA SCALE Eye Opening Spontaneous: 4 To speech: 3 To pain: 2 No Response: 1

31. PEDIATRIC GLASGOW COMA SCALE Best Verbal Response Oriented (Infant coos or babbles): 5 Confused (Infant irritable cries): 4 Inappropriate words (Infant Cries to pain): 3 Incomprehensible sounds (Infant Moans to pain): 2 No Response: 1

32. PEDIATRIC GLASGOW COMA SCALE Best Motor Response Obeys (Infant moves spontaneous/purposefully): 6 Localizes (infant withdraws to touch): 5 Withdraws to pain: 4 Abnormal Flexion to pain (Decorticate): 3 Extensor Response to pain (Decerebrate): 2 No Response: 1

33. PEDIATRIC GLASGOW COMA SCALE Scoring- Total: 3-15 Minor head injury 13-15 Moderate head injury 9-12 Severe head injury (coma): <= 8 Confers Significant Mortality Risk <= 8

34. SEIZURES Classification of Seizures Partial Generalized Most seizures in children involve loss of consciousness and tonic-clonic movements, but auditory, visual or olfactory disturbance, behavioral change or absences in attention may also occur.Most seizures in children involve loss of consciousness and tonic-clonic movements, but auditory, visual or olfactory disturbance, behavioral change or absences in attention may also occur.

35. SEIZURES Simple Partial Seizure - Affects only part of brain (focal, motor or sensory) - Formerly called focal seizures May progress to generalized seizures Complex Partial Seizure Partial seizure with affective or behavioral changes Absence seizures( spells) The history is important, because the anticonvulsants used for partial seizures differ from those used for generalized seizures. The history is important, because the anticonvulsants used for partial seizures differ from those used for generalized seizures.

36. SEIZURES Febrile Seizure - Associated with temperature >38�C - Occurs in children <6 years old (prevalence is 2% to 4% among children <5 years old) - No signs or history of underlying seizure disorder - Often familial - Uncomplicated and benign if seizure is of short duration (<15 minutes), only 1 in 24 hours,and normal CNS exam after seizure - Involves tonic-clonic movements, bilaterally Associated with otitis, roseola, URI, gastroenteritis, pneumonia Keep a high index of suspicion for meningitis in children < 1 yearAssociated with otitis, roseola, URI, gastroenteritis, pneumonia Keep a high index of suspicion for meningitis in children < 1 year

37. SEIZURES HISTORY Previous episodes (i.e., known seizures) Nature of Current Seizure episode - Onset (sudden or gradual), time , setting, duration of seizure - Whether consciousness has been regained since onset of seizure activity - post ictal state - drowsiness, stupor, headache, transient paralysis

38. SEIZURES HISTORY ( continued) Sequence of seizures Type of seizure (generalized or partial) Association with fever, cyanosis, incontinence Association with head injury Ingestion of poisonous substance or other poisoning (e.g., lead encephalopathy)

39. SEIZURES HISTORY ( continued) Medication use -compliance with anticonvulsant therapy if child known to have seizures Other chronic disease Allergies Symptoms of inter-current illness (e.g., fever, malaise, cough) Growth/Developmental history Family Hx. Of seizure disorders

40. SEIZURE DISORDERS Differential Diagnosis - Epilepsy - Drugs (non-compliance with prescription, withdrawal syndrome, overdose, multiple drug abuse) - Hypoxia - Brain tumor - Infection (e.g., meningitis) - Metabolic disturbances (e.g., hypoglycemia, uremia, liver failure, electrolyte disturbance) - Head injury

41. SEIZURE DISORDERS Diagnostic tests - Pulse oximetry - Glucose, electrolytes, calcium, magnesium - Anticonvulsant drug levels in on Rx - Diagnostic Imaging for undiagnosed cases; - EEG - CT + or - MRI - ? LP- CSF analysis

42. SEIZURE DISORDERS Complications - Hypoxia during seizures - Status epilepticus - Arrhythmia - Injury during seizure (e.g., from a fall) - Brain damage - Death

43. MANAGEMENT ACUTE SEIZURE ABCs are the first priority: Ensure airway is clear and patent Suction secretions as necessary Insert oropharyngeal airway Assist ventilation as needed by means of Ambu-bag with oxygen

44. MANAGEMENT ACUTE SEIZURE Oxygen as necessary to maintain oxygen saturation >97% Start IV therapy with normal saline, adjusting rate according to state of hydration Nurse child in side-lying position Keep child warm Give nothing by mouth until child has fully recovered

45. MANAGEMENT ACUTE SEIZURE Pharmacologic Interventions Lorazepam (Ativan), 0.05�0.10 mg/kg IV (maximum 4 mg per dose), repeat q10min for 2 more doses (administer slowly over 5 minutes, maximum rate 2 mg/min) OR Diazepam (Valium), 0.3 mg/kg IV (maximum 5 mg per dose for child <5 years old, 10 mg per dose for child >5 years old) Repeat q5-10 min for 2 more doses (administer slowly over 5 minutes, maximum rate 2 mg/min) Drug of choice for neonatal seizures is phenobarbital Risks of Drug Therapy � Hypotension � Respiratory depression Drug of choice for neonatal seizures is phenobarbital Risks of Drug Therapy � Hypotension � Respiratory depression

46. MANAGEMENT ACUTE SEIZURE If cannot get IV access - give per rectum - diazepam (Valium) 0.5 mg/kg per dose PR (maximum dose 10 mg) - repeat q5�10min for total of 2 doses (maximum rate 2 mg/min) If unable to achieve IV access, diazepam can be given effectively by the rectal route, as follows. Use IV solution without dilution and administer by inserting the smallest possible syringe or a small catheter affixed to the end of a syringe (if the dose is less than 5 mg, a tuberculin syringe is ideal): The medication should be placed a distance of 4 cm into the rectum, adjacent to the rectal mucosa. The buttocks should be elevated and squeezed together for 5 minutes to avoid evacuation of the rectal contents after administration of the drug. Two doses may be given, 5�10 minutes apart. If unable to achieve IV access, diazepam can be given effectively by the rectal route, as follows. Use IV solution without dilution and administer by inserting the smallest possible syringe or a small catheter affixed to the end of a syringe (if the dose is less than 5 mg, a tuberculin syringe is ideal): The medication should be placed a distance of 4 cm into the rectum, adjacent to the rectal mucosa. The buttocks should be elevated and squeezed together for 5 minutes to avoid evacuation of the rectal contents after administration of the drug. Two doses may be given, 5�10 minutes apart.

47. MANAGEMENT ACUTE SEIZURE Monitoring and Follow-Up - Admit - Identify focal neurological deficits, clinical conditions e.g infection - Observe for return to normal level of consciousness - Monitor vital signs, ABCs, pulse oximetry (if available) - Monitor closely for continued seizure activity

48. MANAGEMENT CHRONIC SEIZURE Provide reassurance Client Education - Explain prognosis - most children outgrow - Emphasize importance of adhering to medication regimen - Counsel about first aid during seizures - Advise supervision during swimming, use of helmets - Advise that the child be treated as a normal child would be - Advise about possible teratogenic effects of medications (e.g., phenytoin) for sexually active females

49. MANAGEMENT CHRONIC SEIZURE Commonly Used Anticonvulsants carbamazepine (Tegretol) lamotrigine (Lamictal) phenobarbital (Phenobarb) - kids < 2 phenytoin (Dilantin) primidone (Mysoline) valproic acid (Depakene) vigabatrin (Sabril) Anticonvulsants are tailored to the specific type of seizure. Monotherapy is ideal, but 10% to 15% of patients need two or more medications. Poor compliance is the major cause of seizure recurrence. Anticonvulsants are tailored to the specific type of seizure. Monotherapy is ideal, but 10% to 15% of patients need two or more medications. Poor compliance is the major cause of seizure recurrence.

50. MANAGEMENT CHRONIC SEIZURE Monitoring and Follow-Up - Follow up every 6 months if seizures are well controlled, more frequently if child is having breakthrough seizures - Assess adherence to medication regimen - Monitor serum drug levels every 6 months if stable, more frequently if necessary - Refer urgently if child is having breakthrough seizures - Consider neurological follow-up if symptoms are not controlled on current medications

51. HEADACHES Occurs in 20% of school-age children. Onset may occur at any age The most common causes of headache in children: - benign vascular headaches (leading to migraine) - muscle contraction (leading to tension headaches) In the absence of other symptoms, recurrent headaches of more than 3 months� duration are rarely due to an organic cause. Headaches of relative recent onset (<3 weeks) that are increasing in frequency and severity are worrisome. Vascular headaches (migraine) are common in children, who often have incomplete manifestations of this condition This type of headache should be considered in any recurrent type of headache Variants of Migraine - Acute confusional state - Benign paroxysmal vertigo - Cyclic vomiting In the absence of other symptoms, recurrent headaches of more than 3 months� duration are rarely due to an organic cause. Headaches of relative recent onset (<3 weeks) that are increasing in frequency and severity are worrisome. Vascular headaches (migraine) are common in children, who often have incomplete manifestations of this condition This type of headache should be considered in any recurrent type of headache Variants of Migraine - Acute confusional state - Benign paroxysmal vertigo - Cyclic vomiting

52. HEADACHES Vascular Organic Causes - Arteriovenous malformation - Berry aneurysm - Cererbral infarction - Intracranial hemorrhage

53. HEADACHES Other causes Infection Trauma Toxic Effects Psychogenic Organic -Traction Muscle Contraction-Tension Infection-Brain abscess ,Dental infection,Encephalitis Meningitis Sinusitis (chronic) Trauma-Neck injury,Post-concussion syndrome, Subdural hematoma Toxic Effects, Carbon monoxide, Heavy metal poisoning (e.g., lead) Non-medicinal agents, Excess intake of vitamins Psychogenic- Conversion,Depression, Factitious Traction- Brain tumors ,Hydrocephalus Hypertension Muscle Contraction-Tension Infection-Brain abscess ,Dental infection,Encephalitis Meningitis Sinusitis (chronic) Trauma-Neck injury,Post-concussion syndrome, Subdural hematoma Toxic Effects, Carbon monoxide, Heavy metal poisoning (e.g., lead) Non-medicinal agents, Excess intake of vitamins Psychogenic- Conversion,Depression, Factitious Traction- Brain tumors ,Hydrocephalus Hypertension

54. HEADACHES Food allergy or sensitivity Refractive error Ocular muscle imbalance Temporomandibular joint (TMJ) dysfunction Hypertension

55. HEADACHES History - CHLORIDE PEPPS Associated symptoms - ROS PMH Family HX Social Hx - stress, environmental factors CPX including Neurological exam Gather history from many sources, including the affected child and his or her parents (or caregiver) and teachers. It is best to get a description of both the initial and the most recent headaches. Children >4 years old may be able to give a good description of their symptoms. Associated Symptoms (Functional Inquiry) - Nausea and vomiting with or without abdominal pain (typical of migraine) - Photophobia, facial pain, fever - Transient neurologic signs - Acute confusion, hemiplegia, ophthalmoplegia, syncope, vertigo, paresthesias, phonophobia - Depression - Anorexia, declining school performance, insomnia, weight loss - Other medical problems - Past medical history - Family history of headaches Gather history from many sources, including the affected child and his or her parents (or caregiver) and teachers. It is best to get a description of both the initial and the most recent headaches. Children >4 years old may be able to give a good description of their symptoms. Associated Symptoms (Functional Inquiry) - Nausea and vomiting with or without abdominal pain (typical of migraine) - Photophobia, facial pain, fever - Transient neurologic signs - Acute confusion, hemiplegia, ophthalmoplegia, syncope, vertigo, paresthesias, phonophobia - Depression - Anorexia, declining school performance, insomnia, weight loss - Other medical problems - Past medical history - Family history of headaches

56. HEADACHES Physical exam- Physical findings are usually minimal with headaches - Blood pressure usually normal - Temperature may be elevated with infectious process (e.g., meningitis) - Height and weight

57. HEADACHES HEENT - Pained facial expression - Nuchal rigidity ( neck stiffness) - Funduscopic examination (disks, blood vessels); results usually normal - Spasm or tenderness of neck muscle, tenderness of TMJ

58. HEADACHES HEENT Exam - Deficits of cranial nerves - Purulent rhinorrhea - Halitosis, dental abscesses - Cephalic bruits: use bell of stethoscope over the fronto-temporal areas and orbits

59. HEADACHES Neurologic Examination - Level of consciousness, - Mental status: general demeanor, confusion, depression, stress - Cutaneous lesions (caf� au lait spots) - Focal abnormalities (e.g., tics, limb paresis) - Sensory deficits - Abnormal deep tendon reflexes

60. CLINICAL CHARACTERISTICS OF SPECIFIC HEADACHES Organic -Traction - Headaches increase rapidly in frequency and severity - Headache is worst upon awakening in the morning, diminishes during the day - Headache wakens child from sleep - Aggravated by coughing or valsalva maneuver - May be relieved by vomiting - Associated symptoms: focal neurological findings; altered gait; changes in behavior, personality, cognition or learning ability In 88% of children with a brain tumor, abnormal neurological signs will be evident within the first 4 months after onset of headache. In 88% of children with a brain tumor, abnormal neurological signs will be evident within the first 4 months after onset of headache.

61. CLINICAL CHARACTERISTICS OF SPECIFIC HEADACHES Migraine - Headache - pulsatile (throbbing) - Headaches are periodic, separated by symptom-free intervals - Associated with at least three of the following symptoms: abdominal pain and nausea or vomiting, aura (motor, sensory, visual), family history of migraine - Unilateral or bilateral - Headache relieved by sleep In infancy , very young children signs of migraine could include spells of irritability, sleepiness, pallor, and vomiting Affects 4-5 % of school age children Female/male 2:1 after puberty Headache may be bilateral in kids , associated with photophobia, phonophobiaIn infancy , very young children signs of migraine could include spells of irritability, sleepiness, pallor, and vomiting Affects 4-5 % of school age children Female/male 2:1 after puberty Headache may be bilateral in kids , associated with photophobia, phonophobia

62. CLINICAL CHARACTERISTICS OF SPECIFIC HEADACHES Tension Headache - Band-like tightness or pressure in the bifrontal, occipital or posterior cervical regions -` Seen at any age - Lasting for days or weeks but not disrupting regular activities - Not associated with a prodrome - Associated symptoms: tight neck muscles, sore scalp, nausea, vomiting and aura are uncommon Many children have insight into cause of stress e.g poor self esteem, fear of school failure, fear of bullying at school etc, Associated features - sudden mood changes,fatigue, poor sleep, withdrawal from social activities Many children have insight into cause of stress e.g poor self esteem, fear of school failure, fear of bullying at school etc, Associated features - sudden mood changes,fatigue, poor sleep, withdrawal from social activities

63. CLINICAL CHARACTERISTICS OF SPECIFIC HEADACHES Refractive Error - Persistent frontal headache, which is worse while reading or doing schoolwork TMJ Dysfunction - Temporal headache - Associated symptoms: local jaw discomfort, malocclusion (crossbite), decreased range of motion of mouth, click with jaw movement, bruxism (grinding of teeth)

64. CLINICAL CHARACTERISTICS OF SPECIFIC HEADACHES Chronic Sinusitis - Frontal headache - Tenderness to percussion over the frontal, maxillary or nasal sinuses - Associated symptoms: prolonged rhinorrhea and congestion, chronic cough and postnasal drip, anorexia, low-grade fever, malaise It is unusual for children <10 years old to have recurrent headaches secondary to chronic sinusitis

65. HEADACHES Complications - Recurrent or chronic headaches can be debilitating and may cause absences from school and social withdrawal - Intracranial lesions, masses or infections are life-threatening Diagnostic Tests - Most headaches can be diagnosed from the history and physical examination. - For recurrent or chronic headache, diagnostic information may include daily headache record - CT scan if suspect organic cause

66. MANAGEMENT OF HEADACHES Goals of treatment depend on the cause Acute - Rule out serious organic pathology - Relieve pain Recurrent or Chronic - Relieve pain - Prevent recurrence - Avoid disruption of normal life tasks, such as attending school

67. MANAGEMENT OF HEADACHES Consult a physician immediately in the following circumstances: - Concern about an underlying organic cause for headaches - Uncertainty about the diagnosis - Headaches are chronic and unresponsive to simple analgesia

68. MANAGEMENT OF HEADACHES Supportive reassurance and education are appropriate for non-organic headaches : - Advise parents or caregiver that headaches in children are common and real - Reassure family that headache is unlikely to indicate brain tumor - Explain underlying pathophysiology of vascular and muscle contraction headaches (which are benign and have a favorable prognosis) - Counsel about avoiding factors that trigger headaches

69. MANAGEMENT OF HEADACHES - Identify stressors and advise on how to deal with them - Counsel about use of medications (dose, frequency, side effects) - Relaxation and Imagery Therapy - Abdominal breathing exercises - Visual imagery exercises

70. MANAGEMENT OF HEADACHES For tension headaches and mild -moderate migraines, - Acetaminophen (Tylenol), 10-15 mg/kg per dose (usually analgesic of choice) - Children >6 years old may be given 325 mg, and children >12 years old may be given 325-650 mg PO q4h prn. OR - Nonsteroidal anti-inflammatory drugs (NSAIDs): ibuprofen (Motrin), 5-10 mg/kg per dose PO q8h prn, to daily maximum of 40 mg/kg

71. MANAGEMENT OF HEADACHES Avoid narcotics Migraine prophylaxis Conduct follow-up visits: - Review headache diary if unable to identify cause on first visit, as well as to monitor management - Reinforce balanced health habits of sleep, exercise and diet Referral to pediatrics any child with acute symptoms in whom organic pathology is evident or cannot be ruled out without investigation or any child whose symptoms persist despite Rx On the advice of a physician, migraine prophylaxis may be ordered, but this is rarely necessary in young childrenReferral to pediatrics any child with acute symptoms in whom organic pathology is evident or cannot be ruled out without investigation or any child whose symptoms persist despite Rx On the advice of a physician, migraine prophylaxis may be ordered, but this is rarely necessary in young children

72. HEAD INJURIES HISTORY-Ascertain the following: - Mechanism of injury - Time of injury - Loss of consciousness (a brief seizure at the time of injury) may not be clinically significant - Loss of memory , amnesia - Irritability Head trauma is common among children and results in a significant number of visits to emergency clinics. Children are more predisposed than adults to head injury because their head to body ratio is greater, their brains are less myelinated and thus more prone to injury, and their cranial bones are thinner. Although the incidence of mass lesions is lower among children than among adults, children are more likely to suffer from a unique form of brain injury called malignant brain edema. In addition, children may lose relatively large amounts of blood from scalp lacerations and subgleal hematomas and may present in hemorrhagic shock. Head trauma may be due to child abuse or serious neglect by a parent or caregiver. In all cases, a thorough history should be obtained of past injuries and of the circumstances surrounding the present injury. It may be impractical to review old records for all children with head injuries, but in suspicious cases these records must be reviewed and appropriate follow-up arranged. Head trauma is common among children and results in a significant number of visits to emergency clinics. Children are more predisposed than adults to head injury because their head to body ratio is greater, their brains are less myelinated and thus more prone to injury, and their cranial bones are thinner. Although the incidence of mass lesions is lower among children than among adults, children are more likely to suffer from a unique form of brain injury called malignant brain edema. In addition, children may lose relatively large amounts of blood from scalp lacerations and subgleal hematomas and may present in hemorrhagic shock. Head trauma may be due to child abuse or serious neglect by a parent or caregiver. In all cases, a thorough history should be obtained of past injuries and of the circumstances surrounding the present injury. It may be impractical to review old records for all children with head injuries, but in suspicious cases these records must be reviewed and appropriate follow-up arranged.

73. HEAD INJURIES History (continued) - Visual disturbance - Disorientation - Abnormal gait - Lethargy, pallor or agitation may indicate severe injury - Vomiting - Symptoms of increased intracranial pressure (vomiting, headache, irritability) Many children will vomit two or three times after even a minor head injury. However, protracted vomiting and retching, associated with other symptoms or signs, indicates a more severe head injury. The child�s complete medical history must be obtained. Evidence of conditions such as a predisposition to seizures or bleeding problems is important and will affect the clinical management. Many children will vomit two or three times after even a minor head injury. However, protracted vomiting and retching, associated with other symptoms or signs, indicates a more severe head injury. The child�s complete medical history must be obtained. Evidence of conditions such as a predisposition to seizures or bleeding problems is important and will affect the clinical management.

74. HEAD INJURIES Physical Examination - Vital Signs - Tachypnea: - Bradycardia (with hypertension - Cushing response): - Hypertension - Hypotension Tachypnea: rapid heart rate may signify blood loss, in which case evidence of other injuries should be sought Bradycardia with hypertension (Cushing response): usually a late response in children with increased intracranial pressure and therefore not very reliable Hypertension: late sign of increased intracranial pressure Hypotension signifies shock: look for other injuries, since shock is not a usual sign of brain injuryTachypnea: rapid heart rate may signify blood loss, in which case evidence of other injuries should be sought Bradycardia with hypertension (Cushing response): usually a late response in children with increased intracranial pressure and therefore not very reliable Hypertension: late sign of increased intracranial pressure Hypotension signifies shock: look for other injuries, since shock is not a usual sign of brain injury

75. HEAD INJURIES Signs of Skull Fracture - Hematotympanum - Periorbital or post-auricular ecchymosis - Cerebrospinal fluid otorrhea or rhinorrhea - Depressed fracture or penetrating injury - Palpate scalp for hematomas and contusions, underlying depressions, which may signify depressed skull fracture Before suturing any lacerations, explore all full-thickness skull lacerations to ensure that the underlying bone is intact. Before suturing any lacerations, explore all full-thickness skull lacerations to ensure that the underlying bone is intact.

76. HEAD INJURIES Neurologic Examination - Pediatric Glasgow coma scale - Papilledema - Pupillary light reflexes (PERRLA) - Cranial nerve examination - Movement of extremities - Abnormal posture (decorticate or decerebrate) - Muscle flaccidity, spasticity - Plantar responses Injuries to other areas such as the thorax or abdomen should be sought and treated promptly, since they may contribute to morbidity and death. Clues to increased intracranial pressure: � Decrease in Glasgow coma score of 2 points or more � Abnormality or changes in pupillary size and reaction to light � Respiratory abnormalities � Development of paresis in absence of shock � Hypoxia � Seizures � Elevation of blood pressure � Decrease in heart rate � Decrease in respiratory rate Maintain a high index of suspicion for child abuse Injuries to other areas such as the thorax or abdomen should be sought and treated promptly, since they may contribute to morbidity and death. Clues to increased intracranial pressure: � Decrease in Glasgow coma score of 2 points or more � Abnormality or changes in pupillary size and reaction to light � Respiratory abnormalities � Development of paresis in absence of shock � Hypoxia � Seizures � Elevation of blood pressure � Decrease in heart rate � Decrease in respiratory rate Maintain a high index of suspicion for child abuse

77. HEAD INJURIES Classification of HI � Canadian Pediatric Society Mild Moderate Severe

79. HEAD INJURIES MANAGEMENT MILD INJURY Children with mild intracranial injury may be discharged home An instruction sheet should be given to the parents or caregiver concerning observation and precautions See �Home Care Instructions� for minor head injuries

80. FNIHB- Pediatric Guidelines for Nurses in Primary careFNIHB- Pediatric Guidelines for Nurses in Primary care

81. HEAD INJURIES MODERATE TO SEVERE INJURY - ABC�s first priority - C-spine control - Suture scalp lacerations, as major blood loss can occur - Start IV therapy with normal saline to keep vein open (unless the child is in shock from other injuries) - Restrict fluids to 60% of normal intake (except in cases of shock) - Oxygen MODERATE TO SEVERE INJURY Management Priorities ABCs must be assessed before any detailed history-taking or neurologic examination. Instability of the cardiorespiratory system may be due to severe intracranial injury, intracranial hypertension or injury to other areas, such as the thorax or the abdomen. Prompt ventilatory support and treatment of shock are mandatory, since these factors, if left uncorrected, will result in secondary intracranial traumaMODERATE TO SEVERE INJURY Management Priorities ABCs must be assessed before any detailed history-taking or neurologic examination. Instability of the cardiorespiratory system may be due to severe intracranial injury, intracranial hypertension or injury to other areas, such as the thorax or the abdomen. Prompt ventilatory support and treatment of shock are mandatory, since these factors, if left uncorrected, will result in secondary intracranial trauma

82. HEAD INJURIES MODERATE TO SEVERE INJURY - Elevate head of bed by 30� to 45� - Place head and neck in midline position - Minimize stimuli (e.g., suctioning and movement) - To control increased intracranial pressure: above measures plus establish controlled hyperventilation - CT scan of head - C-spine x-ray

83. HEAD INJURIES MODERATE TO SEVERE INJURY - Diuretics if intracranial pressure is increased (and there is documented deterioration) despite measures outlined above:mannitol, 0.5-1 g/kg IV - Monitor ABCs, vital signs, pulse oximetry, level of consciousness (with serial pediatric Glasgow coma scores), intake and output Prevention strategies Use of helmets for all sports activities is best single strategy Use of proper child restraints in vehiclesPrevention strategies Use of helmets for all sports activities is best single strategy Use of proper child restraints in vehicles

84. MENINGITIS Definition - Inflammation of the meningeal membranes of the brain or spinal cord - Most cases (70%) occur in children <5 years old - May be secondary to other localized or systemic infections (e.g., otitis media). Causes - Meningitis may be caused by bacteria, viruses, fungi and (rarely) parasites.

85. MENINGITIS Bacterial - In children <1 month old: group B Streptococcus, Escherichia coli - In children 4-12 weeks old: E. coli, Hemophilus influenzae type B, Streptococcus pneumoniae, group B Streptococcus, Neisseria meningitidis (meningococcal) - In children 3 months to 18 years old: Streptococcus pneumoniae (most common cause), N. meningitidis, H. influenza type B (rare) - Mycobacterium tuberculosis

86. MENINGITIS Viral - Approximately 70 strains of enteroviruses Fungal - Candida Aseptic - Lyme disease

87. MENINGITIS Transmission - Meningitis caused by H. influenzae: airborne droplets and secretions - Meningococcal meningitis (caused by N. meningitidis): direct contact with droplets or secretions

88. MENINGITIS Incubation - Meningitis caused by H. influenzae: 2-4 days - Meningococcal meningitis (caused by N. meningitidis): 2-10 days Contagion - Meningitis caused by H. influenzae: moderate; high risk of transmission in daycare centers and other crowded environments - Meningococcal meningitis (caused by N. meningitidis): low; spreads most rapidly in crowded conditions

89. MENINGITIS Communicability - Meningitis caused by H. influenzae: as long as organisms are present; non-communicable within 24-48 hours after treatment is started - Meningococcal meningitis (caused by N. meningitidis): until organism is no longer present in secretions from nose and mouth

90. MENINGITIS HISTORY - In children <12 months old - Usually preceded by URTI - High fever - Irritability - Child sleeps "all the time� - Child is "not acting right� - Child cries when moved or picked up

91. MENINGITIS History- Infant < 12 months - Child won't stop crying - "Soft spot bulging"? - Vomiting - Poor feeding - Seizures may develop - Rash (purple spots

92. MENINGITIS History- Older children - Preceding URI - Fever - Photophobia - Headache that becomes increasingly severe - Headache made worse with movement, especially bending forward - Vomiting( without nausea)

93. MENINGITIS History- Older children - Neck pain - Back pain - Changes in level of consciousness, progressing from irritability through confusion, drowsiness and stupor to coma - Seizures may develop - Rash (purple spots)

94. MENINGITIS PHYSICAL EXAM - Temperature- elevated - Tachycardia or bradycardia with increased intracranial pressure - Blood pressure normal (low if septic shock has occurred) - Child in moderate-to-acute distress - Flushed Perform a full head and neck examination to identify a possible source of infection. Perform a full head and neck examination to identify a possible source of infection.

95. MENINGITIS PHYSICAL EXAM ( continued) - Level of consciousness variable - Possible enlargement of the cervical nodes - Focal neurologic signs: - photophobia - nuchal rigidity (in children >12 months old) - positive Brudzinski's sign ( in children >12 months) - positive Kernig's sign (in children >12 months ) Petechiae with or without purpura may be present in meningococcal meningitis -Shock (septic) Petechiae with or without purpura may be present in meningococcal meningitis -Shock (septic)

96. MENINGITIS DIFFERENTIAL DIAGNOSIS - Bacteremia - Sepsis - Septic shock - Brain abscess

97. MENINGITIS COMPLICATIONS - Seizures - Coma - Blindness - Deafness - Death - Palsies of cranial nerves III, VI, VII, VIII

98. MENINGITIS Diagnostic Tests - Blood culture x3, drawn 15 minutes apart - Urine for routine and microscopy, culture and sensitivity - Throat swab for culture and sensitivity - CBC, lytes, creatinine,glucose, INR,platelets, ABG�s - LP It is important to culture several specimens before initiating antibiotic therapy in cases of suspected meningitis, to increase the chance of isolating the organism. Consultation with a physician should be attempted before initiating collection of these specimens. It is important to culture several specimens before initiating antibiotic therapy in cases of suspected meningitis, to increase the chance of isolating the organism. Consultation with a physician should be attempted before initiating collection of these specimens.

99. MENINGITIS Management - Admit- Bed rest - Nothing by mouth - Foley catheter (optional if the child is conscious) - Start IV therapy with normal saline, and adjust rate according to state of hydration Consult a physician immediately. Do not delay starting antibiotics if this diagnosis is suspected. If you are unable to contact a physician, follow the guidelines below for IV antibiotics. Nonpharmacologic Interventions - - Restrict fluid to 50% to 60% of maintenance requirements (unless the child is in septic shock) Do not overload with fluids, as this could lead to brain edema. Consult a physician immediately. Do not delay starting antibiotics if this diagnosis is suspected. If you are unable to contact a physician, follow the guidelines below for IV antibiotics. Nonpharmacologic Interventions - - Restrict fluid to 50% to 60% of maintenance requirements (unless the child is in septic shock) Do not overload with fluids, as this could lead to brain edema.

100. MENINGITIS Pharmacotherapy Antipyretic - Acetaminophen (Tylenol) 10�15 mg/kg q4h prn Antibiotics- Infants <6 Weeks Old - Ampicillin (Ampicin), 75 mg/kg per dose, IV q6h (maximum 2.5 g/dose) AND Gentamicin (Garamycin), 2.5 mg/kg per dose q8h . Give initial antibiotic dose as soon as possible. Any delay once diagnosis is suspected is unacceptable- within . Give initial antibiotic dose as soon as possible. Any delay once diagnosis is suspected is unacceptable- within

101. MENINGITIS Antibiotics -Infants 6 Weeks to 3 Months Old Ampicillin (Ampicin), 75 mg/kg per dose, IV q6h (maximum 2.5 g/dose) AND Ceftriaxone (Rocephin), 80 mg/kg per dose, IV q12h (for the first 48 hours) (maximum 2 g/dose, 4 g/day)

102. MENINGITIS Antibiotics -Children 3 Months to 18 Years Old ceftriaxone (Rocephin), 80 mg/kg per dose, IV q12h (for the first 48 hours) (maximum 2 g/dose, 4 g/day)

103. MENINGITIS Monitor ABCs, vital signs Level of consciousness, intake Urine output Watch for focal neurological symptoms Monitor serum sodium Monitor serum sodium - iaapropriate ADH secretion is frequent complication during first 3 days Monitor serum sodium - iaapropriate ADH secretion is frequent complication during first 3 days

104. MENINGITIS Prevention and Control - Meningitis Caused by Hemophilus influenzae - A vaccine is now routinely given to infants as part of the usual childhood immunizations. - The type of vaccine and the immunization schedule vary by province - The vaccine is usually given at 2, 4, 6 and 18 months of age, along with the DPTP vaccine.

105. MENINGITIS Meningococcal Meningitis -prevention - Vaccines for certain subtypes are available and are sometimes used in epidemics Chemoprophylaxis for household contacts: Rifampin (Rifadin) Infants <1 month old: 5 mg/kg bid for 2 days Children: 10 mg/kg bid for 2 days Adults: 600 mg bid for 2 days Unfortunately, the vaccine does not include the subtype (type B) that commonly causes the disease in the Canadian North. Furthermore, the vaccine is not very effective. Unfortunately, the vaccine does not include the subtype (type B) that commonly causes the disease in the Canadian North. Furthermore, the vaccine is not very effective.

106. CASE STUDY Elaine, a 7 month old girl is brought in by mother for 6 month well child visit Child born at term , birth weight 7.5 pounds Pregnancy healthy, mom thought fetus was kicking less than in utero than she had with her previous baby Labor /delivery were uneventful

107. CASE STUDY Health to date good, feeding well Showed visual attention at 2-3 weeks, smiled socially at one month Pushed self up on arms while prone at 2 months, Rolled over at 4 months, no longer does this No longer reaches for mobile in crib or toys like rattle No attempts to sit up- cannot balance

108. CASE STUDY Physical Examination results - Infant lies quietly on table , watches examiner intently - Growth parameters including head circumference are normal - Vital signs are normal - See-saw breathing, frog leg posture noted - Cranial nerves are normal except eyes did not follow past the midline, and head turning strength was decreased

109. CASE STUDY Physical Examination results - When she is pulled to a sitting position by the hands , her head lags far behind and her arms are fully extended at the elbows - She could not raise her arms off table - When a rattle was placed in her hand , she manipulates the toys which she regards from the corner of her eye - Deep tendon reflexes were absent - Pain sensation intact

110. CASE STUDY Resolution Elaine was judged to have hypotonia with a neuromuscular cause in part because of her alert appearance and absent DTR�s Neuropathic abnormalities on EMG and muscle biopsy confirmed a diagnosis of spinal muscular atrophy Family received genetic counseling and become involved in a support group Elaine died of respiratory failure in a chronic care hospital at age 17 months

111. HYPOTONIA IN ONFANTS Definition Lower-than-normal muscular resistance to passive motion across a joint Muscle strength is a key component of this resistance In infants who cannot cooperate because of their young age with testing muscle power , tone can be effectively used as a measure of muscle power Identifying the level of the lesion within the nervous system i.e upper motor neuron, spinal cord, anterior horn cell, peripheral nerves, myoneural junction, muscle fibers in the motor unit, is most important in determining the etiology of hypotonia in infantsIn infants who cannot cooperate because of their young age with testing muscle power , tone can be effectively used as a measure of muscle power Identifying the level of the lesion within the nervous system i.e upper motor neuron, spinal cord, anterior horn cell, peripheral nerves, myoneural junction, muscle fibers in the motor unit, is most important in determining the etiology of hypotonia in infants

112. ETIOLOGY-HYPOTONIA IN INFANTS Central causes - Neonatal asphyxia and /or intracranial hemorrhage - Chromosomal disorders - trisomy 21 - CNS malformations- NTD�s - Metabolic/endocrine causes - hypothyroidism, celiac disease, inborn errors of metabolism) - Benign congenital hypotonia ( a Dx of exclusion only) - Direct trauma - spinal cord transection

113. ETIOLOGY-HYPOTONIA IN INFANTS Anterior horn Cell - Spinal muscle atrophy( genetic) Peripheral nerve - Guillain-Barre syndrome, hereditary neuropathies, metabolic, toxins, trauma

114. ETIOLOGY-HYPOTONIA IN INFANTS Neuromuscular junction - Myasthenia gravis, infant botulism Muscle - Muscular dystrophies( Duchenne), congenital myopathies, myotonic dystrophy

115. NEUROLOGIC SIGNS of CENTRAL LESION Alertness - decreased, Cry -decreased Muscle power- normal--> decreased Muscle bulk - normal---> decreased Fasiculations- absent DTR�s - increased Primitive newborn reflexes persist or reappear Plantar response- extensor Sensation - normal

116. NEUROLOGIC SIGNS of ANTERIOR HORN CELL LESION Alertness normal, Cry- normal/weak Muscle power- decreased Muscle bulk - proximal atrophy Fasiculations- present DTR�s - decreased to absent Primitive newborn reflexes - absent Plantar response- flexor or nonreactive Sensation - normal

117. NEUROLOGIC SIGNS of PERIPHERAL LESION Alertness/Cry normal Muscle power- decreased Muscle bulk - distal atrophy Fasiculations- variable DTR�s - decreased Primitive newborn reflexes - absent Plantar response- flexor or non-reactive Sensation - decreased

118. NEUROLOGIC SIGNS - LESION NEUROMUSCULAR JUNCTION Alertness normal, Cry weak Muscle power- fluctuating weakness Muscle bulk - normal Fasiculations- absent DTR�s - normal to decreased Primitive newborn reflexes - absent Plantar response- flexor Sensation - normal

119. NEUROLOGIC SIGNS - MUSCLE LESION Alertness normal Cry normal/weak Muscle power- decreased Muscle bulk - decreased Fasiculations- absent DTR�s - decreased Primitive newborn reflexes - absent Plantar response- flexor to nonreactive Sensation - normal

120. ASSESSMENT OF HYPOTONIA  HISTORY Onset (acute or gradual), progression Past history of any acute illness (e.g., meningitis) Family history of myopathy Social history: infant�parent interaction, siblings� history (many babies are �floppy� because of lack of stimulation)

121. ASSESSMENT OF HYPOTONIA  Associated Symptoms: Respiratory Feeding difficulties Seizures Fasciculations Ptosis Delays in reaching developmental milestones

122. ASSESSMENT OF HYPOTONIA  Inappropriate weight gain Maternal health problems (e.g., hypertension, diabetes mellitus) Physiologic insults during pregnancy or delivery Maternal use of neurotoxic drugs Neonatal problems- Apgars, sepsis, respiratory Family History

123. ASSESSMENT OF HYPOTONIA  PHYSICAL EXAM General survey- dysmorphic features Vital signs General physical examination to rule out any underlying cause Complete - detailed CNS exam Assessment of developmental milestones for age Assessment of primitive reflexes *** Dysmorphic features- hypoplastic mandible, high arched palate In assessing neuromuscular function in a younger child, observation is more the rule than formal testing; useful activities to observe include walking, running, climbing stairs, lying on floor and rising to a standing position unassisted, smiling, tightly closing eyes and speaking/cryingDysmorphic features- hypoplastic mandible, high arched palate In assessing neuromuscular function in a younger child, observation is more the rule than formal testing; useful activities to observe include walking, running, climbing stairs, lying on floor and rising to a standing position unassisted, smiling, tightly closing eyes and speaking/crying

124. MANAGEMENT OF HYPOTONIC INFANT Diagnostic approach - CBC, electrolytes, BUN, creatinine, calcium. CPK, bilirubin, LFT�s, urinalysis - Consider Head U/S, CT scan, MRI, LP if central cause suspected - Chromosomes studies if dysmorphic features - Referral to Pediatric neurology - timing dependent on suspected underlying cause and urgency of clinical condition Prognosis is variable depending on underlying mechanism. Genetic conditions may progress to be fatal, some infantile weakness for example that associated with congenital myopathy may improve with time and RxReferral to Pediatric neurology - timing dependent on suspected underlying cause and urgency of clinical condition Prognosis is variable depending on underlying mechanism. Genetic conditions may progress to be fatal, some infantile weakness for example that associated with congenital myopathy may improve with time and Rx

125. MANAGEMENT OF HYPOTONIC INFANT Diagnostic approach - DNA analysis for dystrophies - EMG, nerve conduction studies - Tensilon test for myasthenia gravis - Muscle/nerve biopsies

126. HYDROCEPHALUS Increased CSF volume Communicating hydrocephalus: - Results from unsatisfactory absorption of CSF by the arachnoid gratulations or overproduction of CSF by the choroid plexus Non-communicating hydrocephalus: - Results from an obstruction to CSF flow , causing enlargement of only those ventricles proximal to the obstruction

127. HYDROCEPHALUS Congenital- Aqueductal anomalies - Primary aqueductal stenosis, or secondary to intrauterine infections i.e. varicelal, mumps, TORCH - Dandy-Walker malformation - Chiaria malformation - Myelomeningocele

128. HYDROCEPHALUS Acquired - Post meningitis - Post hemorrhage- (SAH, IVH) - Masses - vascular malformations, neoplastic

129. HYDROCEPHALUS Clinical presentation -age related - Increased head circumference - Irritability, lethargy, poor feeding, vomiting -infant - Headache, lethargy, vomiting- older child - Bulging anterior fontanelle - Widened cranial sutures - Cracked pot sound on cranial percussion - Scalp vein dilatation Head tilt, head bobbingHead tilt, head bobbing

130. HYDROCEPHALUS Clinical presentation - - Sunset sign - eyes deviate downward - Episodic bradycardia, apnea - Loss of color and peripheral vision(older child) Cranial nerve palsies - e.g abnormal pupil size/reactivity, EOM�s, nystagmus Spasticity limbs - Hyperreflexia, clonus

131. HYDROCEPHALUS Diagnostic Investigations Ultrasound of skull- through anterior fontanelle - Shows ventricular enlargement CT of head - Shows ventricular enlargement, peri-ventricualr lucency, narrow/absent sulci, +/- 4 th ventricular enlargement

132. HYDROCEPHALUS RX: Serial Spinal taps Surgery- remove obstruction if possible Shunts Acetazolamide- decreases blood flow to choroidal arteries , therefore decreasing CSF production Long term disability is variable depending on the underlying etiologyLong term disability is variable depending on the underlying etiology

133. HYDROCEPHALUS Complications - Shunt blockages - Infection of shunt - Over shunting - Seizures - Blindness - Cranial nerve dysfunction - ICP - Cognitive impairment

134. CHILDHOOD MALIGNANCIES Cancer is the most common cause of disease related deaths in children 1-19 years Incidence has increased slowly, but mortality rates have declined significantly Common cancers in childhood include: - Leukemias - Lymphomas - Brain tumors

135. CHILDHOOD MALIGNANCIES Clinical Clues/Manifestations Hematologic - Pallor, anemia, petechiae, thrombocytopenia, neutropenia, fever, paryngitis - Signifies bone marrow infiltration - Example- Leukemia, neuroblastoma

136. CHILDHOOD MALIGNANCIES Clinical Clues/Manifestations Systemic - Fever, weight loss, night sweats, painless lymphadenopathy ( Hodgkins, Non-Hodgkins lymphoma) - Bone pain, limp, arthralgias ( Osteosarcoma, Ewing�s sarcoma) - Cutaneous lesions ( neuroblastoma, leukemias)

137. CHILDHOOD MALIGNANCIES Clinical Clues/Manifestations - Soft tissue mass ( Osteosarcoma, Ewing�s sarcoma) - Vomiting/ Diarrhea, abdominal mass (Lymphoma) - Thoracic mass- Lymphoma, neuroblastoma - Visual disturbances, headache, ataxia, cranial nerve palsies, papilledema ( Brain tumour)

138. CHILDHOOD MALIGNANCIES Clinical Clues/Manifestations Ophthalmologic signs - Leukokoria ( retinoblastoma) - Peri-orbital ecchymosis ( neuroblastoma) - Ptosis, miosis (neuroblastoma) - Exopthalmous, proptosis ( orbital tumour) lymphoma,

139. BRAIN TUMOURS Primarily infratentorial involving cerebellum, midbrain, brainstem Glial( cerebellar astrocytomas most common) Presenting S&S - Poor feeding, Vomiting , FTT( failure to thrive) - Arrest or regression of developmental milestones - Morning headache, increased head circumference ( hydrocephalus) - Diploplia, nystagmus,papilledema - Focal neuro deficits, seizures , ataxia

140. BRAIN TUMOURS Diagnosis Comprehensive history and complete CPX Careful CNS exam Rule out other causes - infection/trauma/metabolic CT head and/or MRI Referral to Pediatric neurosurgery

141. LYMPHOMA Third most common childhood cancer Hodgkins - Occurs in older child > 15 years, similar to adult - Presents as a painless firm lymhadenopathy Non- Hodgkins - Occurs in younger child 7-11 years - Rapid growing , commonly metastesizes - Common disease sites abdomen, mediastinal mass, head/neck mass - S&S vary according to site affected

142. NEUROBLASTOMA Most common cancer occurring in the first year of life Neural crest tumour arising from sympathetic tissues Adrenal medulla- 45 % Retroperitoneal- 25% Posterior mediastinum - 20% Pelvis - 4% Neck - 4%

143. NEUROBLASTOMA Presents a s a neck mass or chest mass, or abdominal mass( adrenal gland) Hypertension, headache,palptation,sweating ( increased catecholamines), diarrhea, hypokalemia, FTT, are other possible S&S Direct extension to spinal cord - compression Metastases common at presentation

144. NEUROBLASTOMA Referral to Pediatric oncology Diagnostic tests can include: - LFT�s, renal function, ferritin, urine( VMA, HVA) - CT scan chest, abdomen - Bone scan - Bone marrow examination - Tissue biopsy Rx: surgery/radiation/chemotherapy +/- bone marrow transplant Age and staging are important prognostic indicators Age and staging are important prognostic indicators