Craniosynostosis is the premature fusion of skull sutures, restricting skull growth in some areas and enhancing it in others. The main types are: - Scaphocephaly from sagittal suture fusion, causing a long narrow head. - Trigonocephaly from metopic suture fusion, pushing the forehead forward in a triangular shape. - Plagiocephaly from unilateral coronal or lambdoid fusion, causing an asymmetric "skewed" head shape. - Brachycephaly from bilateral coronal fusion, restricting forward and backward growth for a short wide head. - Oxycephaly from coronal plus other suture fusion,

1. HYDROCEPHALUS , SPINA BIFIDA AND
CRANIOSYNOTOSIS

2. Hydrocephalus
 Hydrocephalus is a condition characterized by an excess of cerebrospinal fluid (CSF)
within the ventricular and subarachnoid spaces of the cranial cavity .
 Normally a delicate balance exists between the rate of formation and absorption of
CSF: the entire volume is absorbed and replaced every 12 to 24 hours. In
hydrocephalus, this balance is disturbed.
 Hydrocephalus may be recognized at birth, or it may not be evident until after a few
weeks or months of life

5. CSF ( cerebro spinal fluid )
 It is a clear, colorless body fluid found in the brain and spinal cord.
 It is produced by the specialized ependymal cells in the choroid plexuses of the
ventricles of the brain, and absorbed in the arachnoid granulations .
 About 20 ml of CSF is secreted in an hour .
 About 500mL is generated every day

7. Production

8. Functions of CSF
 CSF acts as a cushion or buffer for the brain, providing basic
mechanical and immunological protection to the brain inside the skull.
 Serves a vital function in cerebral autoregulation of cerebral blood
flow .
 Carry nutrient to the brain and spinal cord and remove waste
substances .

9. Etiology
Congenital hydrocephalus
 Intrauterine infections: Rubella, cytomegalovirus, toxoplasmosis, intracranial
bleeds, intraventricular haemorrhage .
 Congenital malformations: Aqueduct stenosis, Dandy-Walker syndrome
(posterior fossa cyst continuous with fourth ventricle), Arnold-Chiari syndrome
(portions of cerebellum and brainstem herniating into cervical spinal canal, blocking
the flow of CSF to the posterior fossa) ,Midline tumors obstructing CSF flow .

10. Etiology cont …
Acquired hydrocephalus
 Tuberculosis, chronic and pyogenic meningitis , Post-intraventricular
haemorrhage
 Posterior fossa tumors: Medulloblastoma, astrocytoma, ependymoma
 Arteriovenous malformation, intracranial haemorrhage, ruptured
aneurysm . Hydrocephalus ex vacuo

11. Classification
 Communicating hydrocephalus - There is no blockage in the CSF pathway but
reabsorption may be affected. Excess CSF may be produced in papilloma of choroid
plexus.
 Obstructive or non communicating hydrocephalus - The block is at any level in the
ventricular system, commonly at the level of aqueduct or foramen of Luschka and
Magendie . This blockage causes increased pressure on the brain or spinal cord .

12. Clinical Manifestations
 Enlarging head size
 Delayed closure of fontanel and sutures .
 Associated symptoms include headache, nausea, vomiting, personality
and behavior disturbances such as irritability, head banging, apathy
and drowsiness .
 Scalp veins become prominent and dilated.
 Papilledema, pyramidal tract signs and cranial nerve palsies may occur

13. Cont.…..
 Sun set sign
 Macewen sign
 Distortion of the brainstem may lead to bradycardia,
systemic hypertension and altered respiration rate .

14. Diagnostic Evaluation

15. Management
 Medical Management : only in mild cases
• Acetazolamide at a dose of 25-100 mg/kg/day
diminishes CSF production in mild, slowly
progressive hydrocephalus .
• Oral glycerol has also been used for similar
purpose

16. Surgical Management
Ventriculoperitonial(VP)shunt :
 A catheter is inserted into the ventricle with
tubing tunnelled subcutaneously down the
thorax and then further tunnelled into the
peritoneal cavity where the CSF is absorbed.
 VP shunt drains excess CSF and decreases
the pressure in brain .

17. Shunt systems
 It has three components:
(1) a ventricular catheter
(2) a one way valve
(3) a distal catheter
 The ventricular catheter is a straight piece of
tubing, closed on the proximal end and usually
with multiple holes for the entry of CSF along
the proximal two centimeters of the tube

18. Complications of VP shunt
 Infection
 Blockage
 Symptoms of shunt malfunction or infection include headache, fever, drowsiness
, convulsions, increased head circumference and bulging fontanelle .

19. ventriculoatrial (VA) Shunt
 The CSF is shunted from the cerebral ventricles into the right atrium of the heart. The
CSF is then shunted directly into the blood circulation.
 A silicon catheter is inserted in lateral ventricle & down through the internal jugular vein
into left atrium of the heart .
 It is reserved for older children who have attained most of their somatic growth &
children with abdominal pathology. It requires repeated lengthening as child grows

21. Ventriculopleural shunt
 This type of shunt drains fluid from the lateral ventricle to the pleural cavity .Drainage of the CSF
may cause hydrothorax, necessitating either removal of the shunt or a thoracentesis.

22. Third ventriculostomy
 It is a surgical procedure for treatment of hydrocephalus in which an opening is
created in the floor of the third ventricle using an endoscope placed within the
ventricular system through a burr hole .
 it is used for no communicating obstructive hydrocephalus

24. Nursing Management
Assessment :
 Obtaining accurate vital and neurologic signs is necessary before and after surgery.
 Measurement of the newborn’s head is essential.
 If the fontanelles are not closed, carefully observe them for any signs of bulging.
 Observe, report, and document all signs of Increase ICP.
 Determine the level of knowledge family members have about the condition

25. Nursing diagnosis
Ineffective cerebral Tissue Perfusion related to Increased intracranial
pressure
 Assess vital signs hourly, noting for any irregularity in breathing and heart
rate and rhythm and measure the pulse pressure .
 Measure the head circumference and appearance of anterior fontanelle
 Elevate the head of the bed gradually about 15-45 degrees as indicated
 Administer diuretics, carbonic hydrase, corticosteroids as ordered .
 Assess neurological status (such as mental status, motor, and balance,
reflexes )

26. Risk for Impaired Skin Integrity related to pressure from physical immobility
 Assess the general condition of the patient
 keep the newborn’s head turned away from the operative site until the physician allows a
change in position .
 Inspect the dressings over the shunt site immediately after the surgery, every hour for
the first 3 to 4 hours
 Maintain the aseptic techniques .
 Assess the pressure points frequently (sacrum. Heels, bones)
Deficient Knowledge related to the family’s understanding of the child’s condition and
home care
 Encourage the parents to provide as normal lifestyle as possible
 Explain how to recognize signs and symptoms of increased ICP. Subtle signs include changes in
school performance,intermittent headache, and mild behaviour changes
 Encourage the child to participate in age appropriate activities as tolerated.
 Encourage the parents to provide as normal lifestyle as possible.

27. Risk for Infection related to the presence of a shunt.
 Monitor the signs & symptoms of infection
 Follow aseptic technique for performing any procedure to the patient to prevent the
chances of infection
 Administer antibiotics as prescribed.
 Provide drain care , catheter care to the patient .
Anxiety related to the family caregiver’s fear of the surgical outcome
 Explain to the family the condition and the surgical procedure .
 Encourage them to express their anxieties and ask questions
 Provide feedback to the family in their understandable terms .
 Clear thee doubts of the family
 Provide counselling

28. Spina Bifida

29. Definition
 spina bifida is a failure of the posterior laminae of the vertebrae to close;
this leaves an opening through which the spinal meninges and spinal cord
may protrude .
 It is a neural tube defect
 Due to deficiency of folic acid and zinc .

30. Etiology
 Maternal risk factors include zinc and folate deficiency, alcohol, radiation
exposure, insulin dependent diabetes mellitus (IDDM), and use of valproate and
carbamazepine during pregnancy .
 Family history - Previous NTD affected pregnancy increses risk that a woman
will have another NTD-affected pregnancy
 Obesity
 Genetic Factors - genetic condition such as Patau's syndrome, Edwards'
syndrome or Down's syndrome.
 Race/ethnicity (NTDs are more common among white women than black
women, and more common among Hispanic women than non-Hispanic women

31. Types
 Spina bifida occulta
 Spina bifida cystica
Meningocele
Myelomeningocele

32. Spina Bifida Occulta
 Occult means hidden
 The outer part of some of the vertebrae is not completely closed.
 The splits in the vertebrae are so small that the spinal cord does not protrude.
 The skin at the site of the lesion may be normal, or it may have some hair growing
from it; there may be a dimple in the skin, or a birthmark.

34. Spina bifida cystica
 Meningocele: A posterior meningocele is the least common form of spina bifida.
In this form, a single developmental defect allows the meninges to herniate between
the vertebrae.
 As the nervous system remains undamaged, individuals with meningocele are
unlikely to suffer long-term health problems
 No nerve roots are involved, so no paralysis or sensory loss below the lesion appears
 The sac may rupture or perforate, introducing infection into the spinal fluid and
causing meningitis

36. Myelomeningocele
 The most severe of all forms of Spina Bifida.
 The spinal cord and dura mater protrudes from the opening in the spine. Because
the spinal cord leaves the protective bone tube of the spine and grows in the sack,
damage to the nerves occur at this point.
 In some cases, the skin covering the area will be absent requiring surgical closure
 Complete paralysis involves the lower trunk and legs, as well as bowel and bladder
sphincters

39. Clinical Manifestations
Physical problems :
 Leg weakness and paralysis
 Orthopedic abnormalities (i.e., club foot, hip dislocation, scoliosis)
 Bladder and bowel control problems, including incontinence, urinary tract infections, and
poor kidney function
 Pressure sores and skin irritations
 Abnormal eye movement

40. Cont.…
Neurological problems :
 The Arnold Chiari II malformation
 Hydrocephalus also occurs because the displaced cerebellum interferes with the
normal flow of cerebrospinal fluid, causing an excess of the fluid to accumulate
Academic problem :
Individuals with spina bifida may struggle academically, especially in the subjects of
mathematics and reading

41. Diagnostic Evaluation
 AFP level - An elevated AFP level in the maternal serum or amniotic fluid indicates the
probability of central nervous system abnormalities .
 Fetal ultrasound - Open spina bifida can usually be detected during pregnancy by fetal
ultrasound.
 Genetic counseling and genetic testing – Amniocentesis ( performed at 14 and 16
weeks gestation.)
 (MRI), ultrasonography, computed tomography (CT), and myelography

42. Prevention
 Primary prevention includes preconception folate supplementation to all
prospective mothers
 Counseling of family with a previous child with NTD is essential
 Folate supplementation reduces recurrence risk by 70%. Zinc and vitamin A
supplementation is also advised .
 Dose for primary prevention is 0.4 mg per day. A mother who has previously
delivered a child with NTD should receive 4 mg per day of folic acid in subsequent
pregnancies

43. Surgical Management
 Surgery includes closure of the defect and a VP shunt (if associated with
hydrocephalus). Early closure prevents neurological deterioration.
 Open lesions draining CSF should be closed within 24 hr. Closed lesions should
be operated within 48 hr.

44. Lorber's criteria for selective surgery : Surgery is not recommended if
there is severe
 paraplegia at or below L3 level
 kyphosis or scoliosis
 gross hydrocephalus, associated gross congenital anomalies
 intracerebral birth injuries and neonatal ventriculitis before closure of back
.

45. Nursing Management
 Risk for Infection related to vulnerability of the myelomeningocele sac
 Risk for Injury related to neuromuscular impairment
 Compromised Family Coping related to the perceived loss of the perfect newborn
 Deficient Knowledge of the family caregivers related to the complexities of caring for a
newborn with serious neurologic and musculoskeletal defect

46. Craniosynotosis

47. Definition
 Craniosynostosis is the premature fusion of one or more cranial sutures, either major (e.g.
metopic, coronal, sagittal, and lambdoid) or minor (frontonasal, temporosquamosal, and
frontosphenoidal ) .
 Virchow's law dictates that, when premature suture closure occurs, growth of the skull typically
is restricted perpendicularly to the fused suture and enhanced in a plane parallel to it, thus trying
to provide space for the fast-growing brain
 The resulting skull deformity is termed as scaphocephaly, plagiocephaly or trigonocephaly based
on the suture involved.

49. Scaphocephaly
 Premature sagittal suture closure restricts growth in a perpendicular plane, thus the head will
not grow sideways and will remain narrow .
 Compensatory growth occurs forward at the coronal suture and backward at the lambdoid
suture giving respectively a prominent forehead, called frontal bossing, and a prominent back
portion of the head, called coning .
 When viewed from sideways the resulting shape of the head will look a bit like a boat

51. Trigonocephaly
 Results from the premature closure of the metopic suture.
 Compensatory growth occurs at both the coronal sutures, thereby pushing the forehead
forwards .
 Trigonocephaly is also a Greek-derived word, which can be translated as triangular-
shaped head

53. Plagiocephaly
 Plagiocephaly can be subclassified as anterior plagiocephaly or posterior plagiocephaly
 Anterior plagiocephaly : is a clinical description of unilateral coronal synostosis. Children born with
unilateral coronal synostosis develop due to compensatory mechanisms a skew head .
 The sagittal suture 'divides' the coronal suture in two halves; unilateral meaning that either the
right side or the left side to the sagittal suture is fused

54.  Posterior plagiocephaly : Unilateral lambdoid synostosis is also called posterior
plagiocephaly, indicating that this gives, just like unilateral coronal synostosis, a 'skew
head'. The difference is that this time, the deformity mostly shows at the occiput.

55. Brachycephaly
 Brachycephaly, or a 'short head', is the result of a closure of both the coronal sutures.
 child's head with a restriction of growth in the forward direction and in the backward
direction , recessed frontal bones and a flattened occiput.
 Compensatory growth will occur sideways, due to the sagittal suture, and upwards, due to
the lambdoid sutures

57. Oxycephaly
 The premature closure of the coronal suture plus any other suture, like the lambdoid suture .
 Also known as turricephaly and high-head syndrome/ Tower head .

59. Other craniosynostosis types and their
signs
 Apert syndrome : An abnormal skull shape, small upper jaw, and fusion of the fingers and toes.
 Crouzon syndrome : A craniofacial birth abnormalities with bilateral coronal suture fusion.
Anterior and posterior of skull shortness, flat cheek bones and a flat nose are their features.
 Pfeiffer syndrome : Abnormalities of the skull, hands, and feet wide-set, bulging eyes, an
underdeveloped upper jaw, beaked nose.
 Saethre–Chotzen syndrome : Short or broad head. the eyes may be spaced wide apart and have
palpebral ptosis (droopy eyelids), and fingers may be abnormally short and webbed .

60. Etiology
 Biomechanical factors : fetal head constraint during pregnancy
 Environmental factors : maternal smoking and the maternal exposure to amine-containing
drugs.
 Hormonal factors : Hyperthyroid induced craniosynostosis is a hormone mediated premature
closure

61. Clinical Manifestations
 Craniosynostosis causes a change in the normal shape of the head. Depends on type of suture
involved
 Elevated ICP
 If left untreated, some children may then develop learning disabilities or problems with their
eyesight.
 Slow or no growth of the head as your baby grows

62. Diagnostic evaluation
 History
 Physical Examination
 CT scan
 X ray

63. Management
 Helmet therapy - Cranial remolding helmets are usually made of a hard outer
shell with a foam lining. Gentle, persistent pressures are applied to capture the
natural growth of a baby’s head while inhibiting growth in the prominent areas and
allowing for growth in the flat regions .
 child is diagnosed with deformational plagiocephaly, brachycephaly or
scaphocephaly and is less than 12 months old, cranial remolding may be prescribed
to correct the shape of the baby’s head .
 The average treatment with a helmet is usually three month

65. Surgical Management
Real-time Stereotactic-Endoscopic Craniectomy:
 Minimally invasive technique can be used to treat patients with unicoronal, bicoronal and metopic
synostosis .
 It is reserved only for young infants (less than 3 months of age)
 A small camera (endoscope) is used that is registered to the patient's radiologic images (CT) to
ensure precise removal of the pathologic suture and release of the other bones to permit normal
bone growth .

66. Endoscopic Strip Sagittal Craniectomy
 An endoscopic strip sagittal craniectomy is also minimally invasive and is used with
younger infants with sagittal craniosynostosis.
 Small incisions are made in the front and back of the head.
 A strip of bone encasing the fused suture approximately two inches wide is removed
under endoscopic guidance

67. Standard Craniosynostosis Surgery
Strip Sagittal Craniectomy -- This technique is used for children who are not candidates for
endoscopic procedures.
 An incision is made over the top of the head from ear to ear and the scalp is moved forward. A
strip of bone encasing the fused suture approximately two inches wide is removed from behind
the "soft spot" to the back of the head .
Frontal-Orbital Advancement (FOA) -- This procedure is recommended for older infants with
severe metopic synostosis or coronal synostosis.
 An incision is made over the top of the scalp from ear to ear.
 The bone of the skull is removed from above the eyes to behind the forehead. The bone is
reformed and then replaced.

68.  Frontal-Occipital Reversal (FOR) -- This procedure is advised for children 6 months of
age or older who have fusion of the sagittal suture or have multiple sutures that are fused .
 An incision is made over the top of the scalp from ear to ear.
 The scalp is drawn back and the skull bone is removed from above the eyes to the back of
the head
 The bone is divided into several pieces, which are reshaped and then replaced over the
crown of the head. Tiny plates and screws are used to fix the bones into proper position.

69. Nursing Management
Pre operative care :
 Provide counselling to the parents.
 Prepare patient for surgery .
 Check for informed consent
 Arrange for blood products .
 Encourage mothers for breastfeeding before and after surgery

70. Post operative care :
 After surgery for craniosynostosis, patients return to the pediatric
intensive care unit for monitoring of hemodynamic status and
level of consciousness.
 Monitor vitals of the patient
 Keep head end elevated
 Monitor for signs of hypovolemia as there is increased risk of
bleeding
 Monitor laboratory values for haematocrit , platelets .
 Monitor intake and output
 Monitor output of the subgleal drain