This document discusses various neurological conditions and diseases that can cause macrocephaly in infants and children. It describes conditions present at birth such as caput succedaneum, subgaleal hemorrhage, cephalohematoma, osteopetrosis, subdural hematomas, benign enlargement of the subarachnoid space, megalencephaly, vein of Galen aneurysm, and hydrocephalus. Hydrocephalus and its causes, clinical presentation, assessment, treatments including shunts, and complications are discussed in detail. Posthemorrhagic hydrocephalus as a consequence of intraventricular hemorrhage is also outlined.

1. Neurological conditions
and diseases
Post Basic Paediatrics
18 April 2012

2. Neurological conditions and diseases
 Part I
 At birth (Congenital, acquired)
 Macrocephaly
 Microcephaly
 Spine defect
 Other developmental defect
 Birth trauma/HIE
 Part II
 During development (Congenital, acquired)
 Meningitis
 Seizure
 Headache
 Stroke/Vascular
 Neoplasm/Tumour
 Trauma
 Coma

3. Neurological conditions
and diseases
Part I
At birth (Congenital, acquired)

4. At birth (Congenital, acquired)
 Macrocephaly
 Microcephaly
 Spine defect
 Other developmental defect
 Birth trauma/HIE

5. Macrocephaly
 Macrocephaly during the neonatal period results from
enlargement of any component or “space” of the head
 The components or spaces of the head most likely to enlarge are
the scalp, skull, subdural space, subarachnoid space, brain
parenchyma, intraparenchymal vessels, and ventricles.

6. Macrocephaly: Causes
 SCALP
 caput succedaneum,
 subgaleal hemorrhage, and
 cephalohematoma

7. Caput Succedaneum
 Caput succedaneum is due to edema between the skin and the
epicranial aponeurosis.
 It presents as a mass, usually located in the vertex, that crosses
the sutures and extends over several bones.
 The mass is soft, superficial, and pitting.
 The edema results from compression of the scalp by the uterus
or suction on the scalp if a vacuum extractor was used during
delivery

8. Subgaleal Hemorrhage
 Subgaleal hemorrhage is due to blood between the epicranial
aponeurosis and the external periosteum.
 Subgaleal hemorrhage presents as an evenly spread mass
throughout a large portion of the scalp.
 The mass is firm, fluctuant, crosses suture lines, and increases in
size after birth (sometimes at an alarming speed)

9. Cephalohematoma
 Cephalohematoma presents as a localized mass that does not
cross suture lines.
 It is usually unilateral and over the parietal bone.
 The blood collects between the external periosteum and the
bone.
 The mass is firm, tense, and confined to an individual bone.
 The edge of the mass may feel like a ridge.
 Underlying linear fracture is detected in 10% to 25% of cases.
 Cephalohematoma is produced by forces that tend to separate
the periosteum from the bone.

10. Macrocephaly: Causes
 SKULL -Osteopetrosis
 Osteopetrosis is a disorder characterized by overgrowth of
brittle bones.
 This results in thick, dense, and fragile bones.
 The bony tissue overgrowth results in encroachments of the:
 (1) bone marrow leading to anemia,
 (2) cranial nerves foramina leading to deafness, blindness, or other
signs of cranial nerve dysfunction,
 (3) Pacchioni bodies producing communicating hydrocephalus and
macrocephaly.

11. Macrocephaly: Causes
 SUBDURAL SPACE -Subdural hematomas
 Progressive increases in head circumference
may be noted during the third week of life.
 Subdural hematomas present with irritability or
hyperalertness, or with signs of focal cerebral
disturbances such as seizures, hemiparesis, or
gaze preference.
 The causes of subdural hematomas are trauma
and coagulation disorders.
 Subdural hematoma is diagnosed by CT of the
brain.

12. Macrocephaly: Causes
 SUBARACHNOID SPACE
 Patients with benign enlargement of the subarachnoid space
are usually not born macrocephalic;
 However, some patients with this condition may have
excessive head growth during the neonatal period.
 The presence of bilateral enlarged frontal subarachnoid
spaces (>5.7 mm), widening of the Sylvian fissure (>7.6 mm)
and other sulci, and normal or minimally enlarged ventricles
establishes the diagnosis.
 The anterior fontanelle is large and soft to palpation.
 Family members, most often the father, may also
have a large head

13. Macrocephaly: Causes
 BRAIN PARENCHYMA -megalencephaly
 Parenchymal space enlargement occurs in
 neurocutaneous disorders,
 Soto syndrome,
 metabolic megalencephalies, and
 some degenerative disorders.
 Brain Tumors

14. Macrocephaly: Causes
 Vein of Galen Aneurysm
 Neonates with aneurysm of the vein of Galen may be
macrocephalic at birth.
 The most common neonatal presentations of vein of
Galen aneurysm in the neonatal period are cardiac
failure, cerebral infarction, or cerebral bleed.
 Macrocephaly can be caused by the large size of the
vein of Galen aneurysm, but most often it is caused
by an obstruction of the aqueduct of Sylvius.
 A cranial bruit is often present in neonates with vein
of Galen aneurysm.

15. Macrocephaly: Causes
 Hydrocephalus
 Increased amount of CSF within the ventricles of the brain
 May be caused by obstruction of CSF flow or by
overproduction or inadequate reabsorption of CSF
 May result from congenital malformation or be secondary to
injury, infection, or tumor

16. Hydrocephalus
 Types:
 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

17. Hydrocephalus: Causes
 Congenital
 Aqueductal anomalies
- Primary aqueductal stenosis, or secondary to
intrauterine infections i.e. varicella, mumps, TORCH
- Dandy-Walker malformation
- Chiari malformation
- Myelomeningocele

18. Hydrocephalus: Causes
 Acquired
- Post meningitis
- Post hemorrhage- (SAH, IVH)
- Masses - vascular malformations, neoplastic

19. Clinical Presentation
 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

20. 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

21. Hydrocephalus: Assessment
 Assessment findings depend on
 age of onset and
 amount of CSF in the brain
 Infant to 2 years:
 Enlarging head size, bulging, non-pulsating fontanels,
downward rotation of eyes (sunset), poor feeding, vomiting,
lethargy, irritability, high-pitched cry and abnormal muscle
tone
 Older Children:
 Changes in head size less common
 Signs of increased ICP (vomiting, ataxia, headache) common
 Alteration in consciousness and papilloedema late signs

22. Hydrocephalus: Assessment
 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
 Treatments:
 Serial Spinal taps
 Surgery- remove obstruction if possible
 Shunts
 Acetazolamide- decreases blood flow to choroidal arteries ,
therefore decreasing CSF production

23. Hydrocephalus: Assessment
 Complications:
- Shunt blockages
- Infection of shunt
- Over shunting
- Seizures
- Blindness
- Cranial nerve dysfunction
- ICP
- Cognitive impairment

24. Shunts
 Insertion of a flexible tube into the lateral ventricle of the brain
 Catheter is the threaded under the skin and the distal end
positioned in the peritoneum (common) or the right atrium
 Shunt drains excess CSF from the lateral ventricles; fluid is the
absorbed by the peritoneum or absorbed in the general
circulation via the right atrium

25. Shunts : Nursing Interventions
 Pre-operative
 Monitor head circumference
 Monitor for signs of ICP
 Small frequent feedings
 Post-operative
 Position on opposite side of surgery or back
 Avoid sedation
 Monitor for signs of ICP
 Educate parents concerning signs and symptoms of shunt
infection or shunt malfunction

26. Macrocephaly: Causes
 Posthemorrhagic Hydrocephalus
 Posthemorrhagic hydrocephalus is the most common type of
hydrocephalus in the neonatal period.
 Posthemorrhagic hydrocephalus may be communicating or
noncommunicating.
 It is usually the consequence of intraventricular hemorrhage.
 Intraventricular hemorrhage usually occurs as a consequence
of germinal matrix hemorrhage.
 Germinal matrix hemorrhages are unusual after 34 weeks
gestational age.

27. Macrocephaly: Causes
 Posthemorrhagic Hydrocephalus
 Germinal matrix hemorrhages are classified based on brain
ultrasound in four grades.
 Grade I intraventricular hemorrhage refers to the
presence of subependymal bleed;
 Grade II intraventricular hemorrhage refers to
extension of the subependymal bleed into the
ventricles but without ventricular dilatation;
 Grade III intraventricular hemorrhage refers to
subependymal bleed with extension of the bleed into
the ventricles and hydrocephalus; and
 Grade IV intraventricular hemorrhage refers to
subependymal bleed with extension of the bleed into
the parenchyma as a result of venous infarcts

28. At birth (Congenital, acquired)
 Macrocephaly
 Microcephaly
 Spine defect
 Other developmental defect
 Birth trauma/HIE

29. 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

30. 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

31. At birth (Congenital, acquired)
 Macrocephaly
 Microcephaly
 Spine defect
 Other developmental defect
 Birth trauma/HIE

32. Neural Tube Defects
 Spina bifida
 Diastematomyelia

33. Spina Bifida (myelodysplasia)
 Neural tube defects that develop during the first trimester of
fetal development
 Defect can occur at any place along the spinal canal
 Unknown etiology; thought to be associated with folic acid
deficiency in mother’s diet prenatally
 Degree of disability dependent on location of the defect & if
spinal nerves involved

34. Spina Bifida (myelodysplasia)
 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 defect
 Preventable with pre-conceptual Folic acid supplements 0.4
mg /day

35. Spina Bifida
 Occulta
 Meningocele
 Myelomeningocele

36. Spina bifida “Occulta"
 Spina bifida "occulta" (meaning "hidden" in latin)
 Posterior vertebral arches fail to fuse
 No herniation of meninges or spinal cord
 May have a tuft of hair or dimpling over the lumbarsacral area
 No loss of function

37. Meningocele
 Posterior vertebral arches fail to fuse
 Sac-like protrusion containing meninges and cerebral spinal fluid
 No spinal nerve involvement

38. Myelomeningocele
 Sac-like herniation containing meninges, CSF, and spinal nerves
imbedded in the wall of the sac
 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

39. Nursing Care – Spina Bifida
 Neurological status
 Assess degree of sensation at or below lesion
 Leg movement
 Neurogenic bladder
 Measure head circumference
 High risk of hydrocephalus
 High risk for infection
 High risk for impaired skin integrity
 Altered urinary elimination
 Bowel incontinence/constipation
 Impaired physical mobility

40. Nursing Care – Spina Bifida
 Sac
 Monitor for leakage of spinal fluid
 Monitor skin integrity of sac
 Assess for infection- Sac or systemic
 Position infant on side or abdomen
 Apply wet, sterile, saline dressing
 Do not allow sac to dry out

41. Nursing Care – Post-operative
 Defect/sac is surgically closed within 48 hours
 Observe for latex allergies
 Neurogenic bladder: straight catheterization
 Neurogenic bowel: bowel management program
 Monitor for signs/ symptoms of hydrocephalus

42. Diastematomyelia
- A bone or fibrous band divides spinal cord in two longitudinal
sections
- Associated lipoma may be present, which tethers cord to
vertebra
- Signs and symptoms include weakness, numbness in feet, urinary
incontinence, decreased or absent reflexes in feet
- Treatment - surgery to free cord

43. At birth (Congenital, acquired)
 Macrocephaly
 Microcephaly
 Spine defect
 Other developmental defect
 Birth trauma/HIE

44. Encephalocele
 Skull defect with exposure of meninges alone or
meninges and brain
 Sometimes defect can cause protrusion of
frontal lobe through the nose

45. At birth (Congenital, acquired)
 Macrocephaly
 Microcephaly
 Spine defect
 Other developmental defect
 Birth trauma/HIE

46. Hypoxic-ischemic
Encephalopathy
 Hypoxic ischemic encephalopathy (HIE) refers to the CNS
dysfunction associated with perinatal asphyxia.
 HIE is of foremost concern in an asphyxiated neonate because
of its potential to cause serious long-term neuromotor sequelae
among survivors.
 A simple and practical classification of HIE by severity of
manifestations provided by Levene

47. Hypoxic-ischemic
Encephalopathy
 Hypoxic-ischemic encephalopathy often involves the brain and
the brainstem.
 Very severe hypoxic-ischemic encephalopathy may involve the
brain, brainstem, spinal cord, and muscle.
 Magnetic resonance imaging of the brain in neonates with
hypotonia due to hypoxic-ischemic encephalopathy shows loss
of gray-white matter interface, cortical necrosis, or neuronal loss
of the basal ganglia and thalamus.

48. Mental Retardation
 Significant below average intellectual functioning
which is associated with impaired learning
difficulties
 Causes
 Pre-natal
 Perinatal
 Post-natal

49. Mental Retardation: Causes
• Pre-natal -Genetic Disorders
• Chromosomal aberrations- e.g. Down syndrome (trisomy
21 )
• Disorders with autosomal-dominant inheritance- e.g.
Tuberous sclerosis
• Disorders with autosomal-recessive inheritance-
metabolic disorder; e.g. Phenylketonuria
• X-linked mental retardation- Fragile X syndrome
• Maternal infections- e.g. Rubella infection during the first
month of pregnancy
• Toxic substances- fetal alcohol syndrome
• Toxemia of pregnancy and placental insufficiency

50. Mental Retardation: Causes
• Perinatal (This period refers to 1 week before birth to 4 weeks
after birth )
• Infections -e.g. herpes simplex type 2
• Delivery problems – e.g. birth asphyxia
• Other perinatal problems
• Retinopathy of prematurity
• Hyperbilirubinemia

51. Mental Retardation: Causes
• Postnatal
• Infections
• Bacterial and viral infections of the brain during childhood may cause
meningitis and encephalitis and result in permanent damage
• Toxic substances –e.g. Lead poisoning
• Other postnatal causes
• Childhood malignancies, brain tumors
• Trauma
• Psychosocial problems –e.g. Severe maternal mental illness
• Unknown causes
• no cause can be identified in approximately 30% of cases of severe
mental retardation and in 50% of cases of mild mental retardation

52. MR – Classifications
 Mild
 Slow learner, can work, marry, have children, may need
assistance with crisis
 Moderate
 Needs life supervision
 Severe
 Needs a caretaker for basic needs
 Profound

53. Interventions
 Goal is to promote
 Optimal development
 Family support
 Community referrals

54. Cerebral Palsy
 A non-progressive motor disorder of the CNS resulting in
alteration in movement and posture
 Cause is trauma, hemorrhage, anoxia or infection before, during
or after birth
 1/3 of children have some degree of mental retardation
 Classified as:
 Spastic
 Spasticity (hypertonicity of muscle groups)
 Athetoid
 Worm-like movements of extremities
 Ataxic
 Disturbed coordination
 Mixed

55. Cerebral Palsy – Assessment
 May have hypertonicity or hypotonia of varying degrees on
different extremities
 May have scissoring of the legs
 Absence of expected reflexes or presence of reflexes that extend
beyond expected age
 Failure to meet developmental milestones
 Difficulty swallowing
 Altered speech

56. Nursing Care
 Impaired physical mobility
 Self-care deficit
 Altered nutrition: less than body requirements
 High risk for injury related to neuromuscular,
perceptual or cognitive impairments

57. Treatment
 Self-care is a goal for all children
 Team approach
 Nutrition
 Increased caloric intake
 Special feeding devices
 Community referrals
 Emotional support

58. At birth (Congenital, acquired)
 Macrocephaly
 Microcephaly
 Spine defect
 Other developmental defect
 Birth trauma/HIE

59. Thank You