Craniosynostosis

Question 1

What is the most common craniosynostosis post shunting?

Answer 1

dolichocephaly from sagittal synostosis

Question 2

what is the craniosynostos from sagittal synostosis called?

Answer 2

Dolichocephaly

Question 3

How often does microcephaly occur after shunting?

Answer 3

6%. If complete synostosis has not occured, it might be reversibel if IC hypertension reoccured.

Question 4

where is the anterior fontanelle situated?

Answer 4

At the junction of coronal, sagittal and frontal sutures

Question 5

what fontanelle is located at the junction of coronal, sagittal and frontal sutures

Answer 5

the anterior fontanelle

Question 6

when does the anterior fontanelle usually fuse?

Answer 6

18-24mo

Question 7

Where is the posterior fontanelle situated?

Answer 7

At the junction of sagittal and lambdoid suture

Question 8

what is situated at the junction of sagittal and lambdoid suture

Answer 8

the posterior fontanelle

Question 9

when does the posterior fontanelle fuse?

Answer 9

at 2-3 mo age.

Question 10

What is caused by a premature fusion of the sagittal suture?

Answer 10

scaphocephaly

Question 11

When does normal fusion of the sagittal suture normally occur?

Answer 11

At approximately 22-24 months of age.

Question 12

what is the most common form of craniosynostosis?

Answer 12

Scaphocephaly = Dolichocephaly

Question 13

What suture is the most common to give rise to craniocynostosis?

Answer 13

sagittal suture.

Question 14

What happens to the skulle shape in case of scaphocephaly?

Answer 14

Den växer ffa framför och bakom sutura sagittalis, som ett hundben -narrow elongated skull. (impediment of lateral growth).

Question 15

How many % of craniosynostosis does scaphoencephaly encount?

Answer 15

50%

Question 16

What is the Male:female ratio of scaphoencephaly?

Answer 16

3:1

Question 17

What syndrome is associated to scaphoencephaly?

Answer 17

Marfan syndrome

Question 18

What is different w scaphoencephaly to other craniocynostosis?

Answer 18

• near normal IQ.
• NOT associated to hydrocephalus.

Question 19

what does kephale mean in greek?

Answer 19

head.

Question 20

what does skaphe mean in greek?

Answer 20

a light boat.

Question 21

what acrocephalosyndactyly types of syndromes are associated to craniosynostosis?

Answer 21

Apert syndrome, carpenter syndrome, crouzon syndrome

Question 22

What type of anomali is associated to craniosynostosis?

Answer 22

choanal atresia

Question 23

What syndrome is coronal craniosynostosis correlated to?

Answer 23

Crouzons syndrome and Aperts syndrome.

Question 24

When does primary craniosynostosis occur?

Answer 24

prenatally

Question 25

WHat etiologies are there to secondary craniosynososis?

Answer 25

• metabolic ex rickets - D vitaminbrist
• toxic ex valproat, methotrexat
• hematologic - sickle cell, thalassemi
• structural - due to lack of brain growth.

Question 26

Can craniocynostosis be caused by shunting?

Answer 26

That has never been proven.

Question 27

How often does increased ICP occur in single suture craniosynostosis?

Answer 27

11%. HIgher in multiple.

Question 28

Which tyoe of craniosynostosis might cause Amblyopia?

Answer 28

coronal

Question 29

What to look for clinically in suspected craniosynostosis?

Answer 29

*Bony prominence of the suture
* No ability to cause realtive movement over “the suture” (gently with the thumbs)
* Lack of normal luency over the suture on x-ray or CT.
* CT is the radiology of choice 1st hand.
* MRI if suspected other abnormalities.
– measurements may be very difficult to interpret and be normal even in a odd shaped skull.

Question 30

What type of skulldeformity does dagittal synostosis cause?

Answer 30

A palpable keel-like sagittal ridge and DOLIDOCHOcephaly OR SCAPHOcephaly

Men kom ihåg. På Beito sade handledaren att de brukar frå förlängt huvud med “bossing” både fram o bak - som ett hundben i axial vy.

Question 31

What is dolidochencephaly?

Answer 31

An elongated skull frontal and frontal bossing

Question 32

What is scaphocephaly?

Answer 32

elongated skull with prominent occiput.

Question 33

How many % of patients with non-syndromic sagittal synostosis have elevated ICP?

Answer 33

44%!

Question 34

Surgical treatment of Sagittal craniocynostosis?

Answer 34

Longitudinal strip craniectomy from coronal suture to the lambdoid suture. GREAT care not to injure the underlying dura over sinus sagittalis. Within 3-6 mo age….

Usually extensive cranial remodelling is done after 1 yr age.

Question 35

Under what sensitive period after the longitudinal strip surgery for sagittal craniosynostosis is a redo needed if the opening heals?

Answer 35

up to 6 mo age.

Question 36

Which is the most common craniosynostosis, with 80% male pt?

Answer 36

sagittal synostosis