Week 4: Congenital Disorders

Question 1

Describes defects of fusion of the neural tube involving the
neural tube itself, or overlying bone or skin.
• Occur in the spine or the head

Answer 1

Dysraphism

Question 2

are among the most common congenital

abnormalities.

Answer 2

NTD

Question 3

, reduce the incidence

of neural tube defects.

Answer 3

Prenatal vitamins, especially folic acid

Question 4

Your spinus process is split into two it did not close and it is
occult meaning the skin is intact you do not know that
something is wrong underneath the skin. It is usually
asymptomatic.

Congenital absence of posterior vertebral elements, with
intact skin
• Asymptomatic unless associated with other developmental
abnormalities

Answer 4

SPINA BIFIDA OCCULTA

Question 5

Most of the time you can’t see these patients symptomatic.
There are subtle signs like ______ (left pic with hair at the
back)

Answer 5

Faun’s tail

Question 6

Two types of cord malformation: Type A and Type B

Answer 6

Type A – has a bony spur in between the spinal cord and
there are two dural covering
 Type B – the whole spinal cord is inclose in one single sheet
of dura pero split siya

Question 7

Congenital absence of posterior vertebral elements with
protrusion of the meninges through the defect, with
underlying neural structural abnormalities.
 There is a protrusion of the spine, spinal cord, dura , CSF
into the posterior elements because the vertebral elements
are absent or defect.

• Common findings: neurologic, urologic and othopaedic
syndrome

Answer 7

myelomeningocele

Question 8

• Herniation of brain encased in meninges through the skull
that forms an intracranial mass

Most occur over the convexity of the skull. More rarely, the
tissue protrudes through the skull base into the sinuses.

Answer 8

Encephalocele

Question 9

Tx for encephalocele

Answer 9

excision of the herniated tissue and closure of the defect

Question 10

Most patients with encephaloceles and meningoceles have
impaired cognitive development. Patients with greater amounts of herniated neural tissue tend to have more severe
cognitive deficit. T or F?

Answer 10

T

Question 11

Most common site of encephalocele is in the?

Answer 11

Foramen cecum

Question 12

Congenital small posterior fossa
• Cerebellum and Brainstem herniates thru the foramen magnum
• Hydrocephalus
• Constellation of other congenital malformations

Answer 12

Chiari Malformations

Question 13

Type of Chiari Malformation: herniation of cerebellar tonisls, sometimes with syringomyelia

Answer 13

Type 1

Question 14

Type of chiari malformation, herniation of cerebellar vermis accompanied by lumbar myelomeningocele

Answer 14

Type 2 (Arnold chiari malformation)

Question 15

Occipital encephalocele, usually c syringomyelia, a tethered cord and hydrocephalus. what type of chiari malformation?

Answer 15

Type 3

Question 16

Lack of cerebellar development, not compatible with life, what type of chiari malformation?

Answer 16

Type 4

Question 17

• Enlarged posterior fossa
• Cystic dilatation of the 4th ventricle
• Vermian hypoplasia
• Supratentorial Hydrocephalus

Answer 17

Dandy walker malformation

Question 18

• Abnormal early fusion of a cranial suture line with resultant restrictions of skull growth in the affected are and compensatory bulging
• Skull growth occurs at the cranial sutures for the first 2 years of life.

Answer 18

Cranial Synostosis/ craniosyntosis

Question 19

• A pathologic condition in which an excessive CSF is collected in the cranial cavity

Answer 19

Hydrocephalus

Question 20

• CSF is primarily produced by the ______

* Absorbed by the __________

Answer 20

Choroid plexus, absorbed by the arachnoid granulation

Question 21

rate of prod of CSF?

Answer 21

Produced at a rate of 0.3-0.5 mL/min and approximately 500 mI/day

Question 22

Fxn of CSF?

Answer 22

• Maintenance of the internal environment
• Intracerebral transport of pharmacologically active agents
• Substitute for the normal lymphatic system

Question 23

rate limiting enzyme in the production of CSF?

Answer 23

carbonic anhydrase

Question 24

what drug is a carbonic anydrase inhibitor?

Answer 24

acetazolamide

Question 25

Or it is a non-obstructive type of hydrocephalus. Nasa labas ng ventricles yung block because of an infection and the subarachnoid granulations are scarred.

Answer 25

Communicating Obstructionq

Question 26

Most common cause of congenital hydrocephalus

Answer 26

Aqueductal stenosis

Question 27

4th ventricular outlet obstruction

Answer 27

dandy walker cyst

Question 28

aka cracked pot on percussion?

Answer 28

Macewen’s sign

Question 29

Transillumination test in hyderocephalus findings?

Answer 29

The whole cranial content will illuminate because of increased fluid. Mas maraming tubig, so it will absorb light. Iilaw yung ulo ng patient, parang yung sa Halloween, yung pumpkin, jack o lantern, ganon ang itsura ny

Question 30

MMSE perfect score?

Answer 30

30

Question 31

dye is introduced into the ventricles and x rays are taken to outline the ventricles

Answer 31

Ventriculogram

Question 32

air is injected into the subarachnoid space and x rays are taken to determine the outline of the ventricles

Answer 32

pneumoencephalogram

Question 33

how do you divert CSF?

Answer 33

o Third ventriculostomy
o Shunting
▪ Ventriculoperitoneal
▪ Ventriculoatrial
▪ Ventriculopleural
▪ Ventriculosubdural
▪ Lumboperitoneal

Question 34

This is the most commonly done neurosurgical procedure worldwide. Hundreds are done in a daily basis.

Answer 34

VENTRICULIPERITONEAL SHUNT INSERTION

Question 35

complications of ventriculo shunting?

Answer 35

infection
obstruction
subdural hygroma
subdural hematoma
shunt migration
shunt disconnection or fracture
pseudocyst in the peritoneum

Question 36

psudocyst is a marker of inflammation. T or F?

Answer 36

T