CONGENITAL ANOMALIES & TRAUMATIC CONDITIONS

Hydrocephalus

• Normal CSF amount: 150 mL
• Normal CSF pressure (ICP): 10-15 mmHg
• CSF has no red blood cells
• CSF is produced by the choroid plexus, travels around the ventricular system and is reabsorbed by the subarachnoid villi
• Causes and Associated Factors:
• prematurity - ventricular system may be underdeveloped
• gram (-) meningitis - causes inflammation and subsequent edema
• aqueductal stenosis - a blockage in the Aqueduct of Sylvius, the passageway from the third to the fourth ventricle
• head injury - may cause inflammation on the brain post-trauma
• intracranial tumor - may impede flow of CSF depending on the area blocked
• Arnold-Chiari malformation - congenital herniation of the cerebellar tonsils leading to a blockage of flow of CSF from the brain to the spinal cord
• Dandy-Walker Syndrome - congenital absence of the the cerebellar vermis and an increased size of the fourth ventricle, increasing pressure on the brain
• myelomeningocele - neural tube defect which forms a sac on an unclosed area of the spine causing increased pressure of CSF
• Types
• Communicating - caused by decreased absorption of the subarachnoid villi
• Non-communicating - impediment of CSF flow within the brain's ventricular system
• Signs and Symptoms
• increased head circumference
• bulging fontanels
• dilated scalp veins
• separated skull sutures
• MacEwen's Sign (crackpot sound upon percussion)
• sluggish pupillary reflexes
• increased ICP
• change in level of consciousness
• setting sun sign - also called sunset eyes; a downward deviation of the eye, such that you can see the sclera in between the iris and the upper eyelid
• high pitched cry
• Diagnostic Tests
• CT scan - to check for bones and other hard tissue (e.g. tumors) blocking the ventricular system
• MRI - to check for soft tissue deformities (e.g. cysts, stenosis)
• daily measurement of head circumference
• Treatment
• removal of tumor
• shunting - diversion of flow of CSF to another body area where it is reabsorbed
• ventriculoperitoneal - from a ventricle to the peritoneum
• ventriculoatrial - from a ventricle to the heart's atrium
• lumboperitoneal - from the lumbar area of the spine to the peritoneum
• ventircular pleural - from a ventricle to the pleural space
• ventricular bypass - from one ventricle to another
• Complications:
• infection
• malfunction - obstruction or dislodged shunting catheter
• PreOp
• avoid increasing ICP
• AVOID sneezing, coughing, straining, Valsalva reflex
• DO NOT occluding the carotid artery
• hips slightly flexed and legs abducted
• support the neck muscles
• fluid restrictions
• administer stool softeners
• place sheep skin or lamb's wool under head for support
• change positions q2h
• small frequent feedings up until NPO
• PostOp
• avoid increasing ICP (see above)
• frequent neurologic assessment
• pain relievers as ordered
• antibiotics as ordered
• emotional support
• position on the unoperated side
• monitor for changes

Spina Bifida

• the higher the deformity, the more neurologic deficits presented
• Types
• Spina Bifida Occulta - not visible externally
• manifests with a skin dimple, port-wine nevi (wine-colored strained skin), tufts of hair, of subcutaneous lipoma (fat despoit)
• Spina Bifida Cystica - with an external sac-like protrusion
• Meningocele - protrusion of the meninges and CSF; (+) transillumination
• Myelomeningocele - protrusion of the meninges, CSF, and nerves; (-) transillumination
• Diagnostic Tests
• fetal ultrasound
• increased alpha-fetoprotein (16-18 weeks)
• chorionic villi sampling (9 weeks)
• MRI, CT, myelography (visualization of the flow of CSF in the spine)
• transillumination
• Treatment
• surgical closure within 1st 72 hours of life
• open fetal surgery
• PREVENTION: FOLIC ACID (0.4-4 mg/day during pregnancy)
• shunting
• prone position at rest
• side-lying position when feeding
• apply sterile dressing with NSS on area
• avoid increased ICP (see above)

Neurogenic Bladder Syndrome

• dysfunction of the urinary bladder due to spinal cord disease, injuries, and defects (e.g. spina bifida)
• Signs and Symptoms
• difficulty or complete inability to urinate
• Treatment
• intermittent catheterization
• vesicostomy - stoma creation on the abdominal wall and urinary bladder to provide for drainage
• urinary diversion - construction of a new urinary bladder from bowel/stomach
• oxybutynin (Ditropan) - improves bladder control and storage
• augmentation enterocystoplasty - improves bladder capacity

Cerebral Palsy

• Types
• Spastic - most common; hypertonicity of muscles, impaired motor skills
• Dyskinetic/Athetoid - may be caused by kernicterus (high unconjugated bilirubin at birth leading to brain damage); athetoid movements (slow, writhing, wormlike movements), drooling, dysarthria (poor articulation of words, "bolol-bolol"), poor motor skills
• Ataxic - may be caused by damage to cerebellum; poor motor skills, wide based gait, tremors
• Mixed/Dystonic - a mix of spastic and athetoid
• Causes
• cord coil
• intrauterine hypoxia
• birth trauma
• intrauterine infection, radiation exposure
• multiple births
• Treatment
• foot orthoses/braces - remedies fait problems
• rhizotomy - surgical release of nerves causing spasticity
• baclofen & botox injections - relieves spasticity
• stretching exercises
• seizure precautions
• promote safety
• use sturdy, padded furniture
• side rails up
• wear helmets
• use safety restraints as needed
• avoid polished floors
• talk slowly
• encourage participation during self care
• encourage activities that improve manual dexterity - drawing, writing
• PATIENCE
  

Skull Fractures

• Types
• Linear Skull Fracture - linear crack on the skull
• Comminuted/Depressed Skull Fracture - fractured area of skull breaks into pieces and are displaced inward; usually occurs from blunt force trauma e.g. rock to head, baseball bat to head, hammer to head
• Basillar Skull Fracture - fracture of the bones at the base of the skull
• Signs and Symptoms
• halo sign - blood stain surrounded by yellowish stain (indicative of CSF leakage)
• raccoon eyes - periorbital ecchymosis
• Battle's Sign - bruising behind the ear (for basillar skull fracture)
• blood in the sinuses
• bleeding from the ears or nose
• Injury Types
• Coup Injury - at point of impact (hits forehead on wheel after MVA)
• Contracoup Injury - at opposite side of impact (hits back of head to headrest after MVA)
• Primary Injury - impact damage (bruising on forehead)
• Secondary Injury - delayed events that follow injury (edema, infection, hypoxia)
• Emergency Management
• log rolling technique
• jaw thrust maneuver
• immobilize head and neck (e.g. cervical collar)
• decrease stimuli

Traumatic Brain Injury

• Concussion - reversible; may have temporary change of level of consciousness, headache, and nausea and vomiting
• Contusion - irreversible; actual damage to the brain
• Closed - force from object damages brain but doesn't penetrate skull
• Open - object penetrated the skull and into the brain
• Signs and Symptoms:
• altered levels of consciousness
• loss of reflexes
• pupillary abnormalities
• neurologic deficits
• change in vital signs
• sensory dysfunction
• headache
• vertigo
• seizures
• Management
• evacuation of clots
• mannitol - to reduce ICP
• phenytoin - for seizures
• analgesics
• monitor neurologic vital signs
• quiet environment
• wound care
• fluid regulation
• ventilatory support
• Complications:
• Intracranial Hemorrhage
• Epidural - blood collecting in between the dura and the skull
• Subdural - blood collecting in between the dura and the brain
• Intracerebral - blood collecting within the brain
• Diffuse Axonal Injury - disconnection of axons within the brain; patient immediately after the injury becomes comatose, decorticate, or decerebrate

Spinal Cord Injuries

•  Types
• Incomplete - only part of the spinal cord is damaged
• Complete - damage to an entire area of the spinal cord; may results in paraplegia (paralysis of lower extremities) or quadriplegia (paralysis of all extremities)
• Effects of Injuries
• Central Cord Syndrome
• motor deficits, bowel and bladder dysfunction
• injury to the central area of the spinal cord
• Anterior Cord Syndrome
• motor deficits, loss of sensation
• injury to the anterior area of the spinal cord or blockage of the anterior spinal artery
• Lateral Cord Syndrome (Brown-Sequard Syndrome)
• ipsilateral loss of sensation and paralysis (left side injured, right side manifestations)
• damage to half of the spinal cord transversely
• Autonomic Dysreflexia
• overstimulation of the ANS after injury
• BP > 200mmHG, diaphoresis, bradycardia, restlessness, bowel and blader distention, facial flushing, cognitive impairment
• Carpal Tunnel Syndrome - compression of the median nerve of the wrist from inflammation of the carpal tunnel; caused by repetitive movements of the wrist; symptoms include numbness and pain of the hand affected, muscle atrophy, and loss of grip strength
• Sciatica - injury to the sciatic nerve resulting in pain to the lower extremity
• Management
• emergency care
• immobilization
• spinal/back board
• patient must always be in an extended position
• patient must be twisted or flexed
• methylprednisolone - improves sensory and motor deficits
• surgery