Repetera-utvalda Flashcards
1
Q
A
2
Q
5 most common etiologies to hcph in peds?
A
- congenital w/o MMC 38%
- congenital with MMC 29%
- perinatal hemorrhage 11%
- tumor 11%
- previous infection 7.6%
- trauma/SAH 4.7%
3
Q
Why is the scalp veins enlarged and engorged in child hcph?
A
Reversed flow from intracerebral sinuses.
4
Q
What is Macewens sign?
A
cracked pot sound on percussing dilated/engorged scalp veins in young child hcph.
5
Q
Rule of thumb for normal OCF?
A
Equals distance from crown to rump.
6
Q
typical radiological findings of benign external hydrocheplaus/external hydrocephalus? When does it resolve?
A
- enlarged subarachnoidal space over frontal poles in first year of life.
- normal/minimally enlarged ventricles.
- cortical vein sign
- usually resolve spontaneously by 2yo.
7
Q
Clinical signs of benign external hcph
A
- normal child, perhaps a bit late in motorf due to large head
- enlarged cisterns and widening of anterior interhemispheric fissure.
- usually resolve spontaneously by 2yo.
8
Q
Arrested hcph
A
No progression or deleterious sequelae due to hcph that would require the presence of a CSF shunt.
9
Q
What needs to be done for pt w apparent shunt independence?
A
- follow closely. Reports of death as long as 5 years after apparent shunt independence.
10
Q
how common is entrapped 4th ventricle?
A
2-3% of shunted pt
11
Q
presentation of entrapped 4th ventricle
A
- H/A
- swallowing difficulties
- If the 4th venricle compress the facial colliculus; facial biplegia and/or bilateral abducens paresis might occur.
- reduced level of consiousness
- nausea/vomiting
- “incidental finding”
12
Q
What are the most common etiologies for aqueductal stenosis?
A
- Inflammation following hemorrhage or infection (syphilis, TB)
- Neoplasm - especially brainstem astrocytoma and tectal situated gliomas. LIPOMAS
- Quadrigeminal plate arachnoidal cysts.
13
Q
How many percent of congenital hcph is casued by aqueduct stenosis?
A
70%
14
Q
How old is a child USUSALLY when a hcph is developed from a congenital aqueductstenosis?
A
Usually at birth, but up to 3 mo.
15
Q
What are the three options for treating non- tumoral AqS?
A
- Shunting
- Torkildsen shunting
- Endoscopic third ventriculostomy
16
Q
What is the most common craniosynostosis post shunting?
A
dolichocephaly from sagittal synostosis
17
Q
what is the craniosynostos from sagittal synostosis called?
A
Dolichocephaly
18
Q
what fontanelle is located at the junction of coronal, sagittal and frontal sutures
A
the anterior fontanelle
19
Q
when does the anterior fontanelle usually fuse?
A
18-24mo
20
Q
Where is the posterior fontanelle situated?
A
At the junction of sagittal and lambdoid suture
21
Q
when does the posterior fontanelle fuse?
A
at 2-3 mo age.
22
Q
What is caused by a premature fusion of the sagittal suture?
A
scaphocephaly
23
Q
When does normal fusion of the sagittal suture normally occur?
A
At approximately 22-24 months of age.
24
Q
what is the most common form of craniosynostosis?
A
Scaphocephaly = Dolichocephaly
25
What suture is the most common to give rise to craniocynostosis?
sagittal suture.
26
What happens to the skulle shape in case of scaphocephaly?
Den växer ffa framför och bakom sutura sagittalis, som ett hundben -narrow elongated skull. (impediment of lateral growth).
27
How many % of craniosynostosis does scaphoencephaly encount?
50%
28
What is the Male:female ratio of scaphoencephaly?
3:1
29
What syndrome is associated to scaphoencephaly?
Marfan syndrome
30
What is different w scaphoencephaly to other craniocynostosis?
* near normal IQ.
* NOT associated to hydrocephalus.
31
what acrocephalosyndactyly types of syndromes are associated to craniosynostosis?
Apert syndrome, carpenter syndrome, crouzon syndrome
32
What type of anomali is associated to craniosynostosis?
choanal atresia
33
What syndrome is coronal craniosynostosis correlated to?
Crouzons syndrome and Aperts syndrome.
34
When does primary craniosynostosis occur?
prenatally
35
WHat etiologies are there to secondary craniosynososis?
* metabolic ex rickets - D vitaminbrist
* toxic ex valproat, methotrexat
* hematologic - sickle cell, thalassemi
* structural - due to lack of brain growth.
36
Can craniocynostosis be caused by shunting?
That has never been proven.
37
How often does increased ICP occur in single suture craniosynostosis?
11%. HIgher in multiple.
38
What to look for clinically in suspected craniosynostosis?
*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.
39
How many % of patients with non-syndromic sagittal synostosis have elevated ICP?
44%!
40
Surgical treatment of Sagittal craniocynostosis?
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.
41
Under what sensitive period after the longitudinal strip surgery for sagittal craniosynostosis is a redo needed if the opening heals?
up to 6 mo age.
42
Which is the most common craniosynostosis, with 80% male pt?
sagittal synostosis
43
What is Apert syndrome?
AKA type 1 acrocephalosyndactyly.
* Brachycephaly
* Syndactyly
* Maxillary hypoplasia
Predominantly skull and limb malformations. Congenital cardiac anomalies 10%. Congenital genourinary anomalies. Symphalangism.
1/800000 pregnancies. Older paternal age.
44
Cardinal symptoms in Syringomyelia?
* Bilateral UE impaired ability for pain sensation.
* Bilateral UE impaired temperature sensation
* Spastic paraparesis.
45
When does the few arachnoidal cysts that become symtomatic usually become symtomatic?
In early childhood.
46
When a suture ossifies, normal growth changes. How?
The growth 90 degrees to the suture terminates and instead it proceeds parallel to the suture. ( så om coronarsuturen sluter blir huvudet långsmalt )
47
What sydrome is associated to higher PATERNAL age?
Apert syndrome.
48
Dandy Walker malformation is often associated to something else. WHat?
Hcph.
49
Name 5 differential diagnostics to Dandy Walker malformation
* Dandy Walker variant
* Persistent Blakes pouch cyst
* Retrocerebellar arachnoid cyst
*Joubert syndrome
* Enlarged cisterna magna
50
What is Joubert syndrome?
Abscence or underdevelopment of cerebellar vermis
51
Which tyoe of craniosynostosis might cause Amblyopia?
coronal
52
Name two syndromes/malformations in which vermis is underdeveloped/hypoplastic or agenesis of vermis is shown?
* Joubert syndrome - unerdeveloped
* Dandy Walker - hypoplastic or agenesis
53
How does the posterior fossa look in "Mega cisterna magna"?
The whole posterior fossa is enlarged due to the enlarged cistern. Normal vermis, normal 4th v, no mass effect.
54
What can happen with retrocerebellar arachnoid cyst that does not occur in cisterna magna?
The arachnoid cyst may anteriorly displace the 4th ventricle and cerebellum, causing a significant mass effect. Vermis is intact in both situations.
55
What is a Dandy walker variant?
When not all of the (3) Dandy Walker criteria are fullfilled;
* Vermian hypoplasia/agenesis
* Enlarged 4th ventricle
* Enlarged posterior fossa.
56
What malformation show *enlarged posterior fossa, *hypoplasia or agenesis of vermis and *cystic dilatation of the fourth ventricle. The fourth ventricle is also distorted and enchased by NEUROGLIAL VASCULAR membrane?
Dandy Walker malformation
57
What more is seen in Crouzons syndrome?
hypoplasia of the midface.
58
What more is seen in Apert syndrome?
Syndactyly
59
What is plagiosephaly?
Flattened or concave forehead on the affected side and compensatory prominence of the contralateral forehead.
TYPISK TENTAFRÅGA
60
Bilateral coronal craniosynostosis is seen in Aperts syndrome. What is it called and how does it look?
ACROcephaly.
Broad flattened forehead.
61
What is Apert syndrome?
AKA type 1 acrocephalosyndactyly.
* Brachycephaly
* Syndactyly
* Maxillary hypoplasia
Predominantly skull and limb malformations. Congenital cardiac anomalies 10%. Congenital genourinary anomalies. Symphalangism.
1/800000 pregnancies. Older paternal age.
62
What may be the diagnosis if you see a tetraventricular hcph and a communicating 4th ventricle and posterior fossa cyst? Vermis and medial parts of cerebellum hemispheres might also look hypoplastic.
That is probably a persistent Blakes pouch cyst.
63
What are the two most difficult differential diagnostics regarding cysts in the 4th ventricle?
To differentiate between Dandy Walker, a Dandy walker variant and a persistent Blakes pouch cyst. The difference should be a tetraventricular hcph in Blakes pouch. Hcph is common in Dandy Walker too, but then there is an enlarged posterior fossa present.
64
It is possible to look for positioning of the choroid plexus of the fourth ventricle when differentiating between arachnoidal cyst, Blakes pouch and Dandy walker/dandy walker variant.
What differs in the Choroid plexus?
* Choroid plexus of the fourth ventricle does not exist in Dandy Walker malformations.
* The position of the Choroid plexus is normal in case of an arachnoidal cysts.
* The Choroid plexus is DISPLACED in a Blakes pouch cyst.
65
Where is the fourth ventricle choroid plexus situated in a Dandy Walker malformation?
It does not exist.
66
What does abscence of anterior CC with precence of some posterior CCindicate?
Some form of holoproencephaly.
67
Where are lipomas usually found intracranially?
Near the midsagittal plane.
Particularly over CC and often associated to agenesis of CC.
68
Where are hypothalamic hamartomas usually situated?
At tuber cinerum
69
How does hypothalamic hamartomas usually present?
* Precocious puberty
* Seizures
* Developmental delay
* Aggressive behavoiur/rage attacks.
70
Hypothalamic hamartomas are associated with a very special form of seizures. What is it called?
Gelastic seizures.
71
How are gelastic seizures usually presenting?
Brief unprovoked laughter.
72
Why does hypothalamic hamartoma patients get precocious puberty?
Due to GnRH release from hamartoma cells.
This is the most common tumouros change to cause precocious puberty.
73
Where is tuber cinerum situated?
In the floor of the third ventricle, between the infundibular stak and the mammillary bodies.
74
Apert syndrome and Crouzon syndrome are similar. WHat differs?
In apert syndrome, cleft palpate, complex syndactyli and mental retardation is seen, whereas all this is normal in Crouzon.
75
hydranencephaly
post-neurolation defect.
Only small bands of cerebrum remains.
Thought to be due to bilateral ICA infarcts.
Cavity filled with CSF
76
How to differ hcph and hydranencephaly?
By EEG. Hydranencephaly doesnt show any activity at all. Extreme hcph show abnormal activity and background activity.
Another way, for the typical hydranencephaly is angiography that will not show any anterior circulation.
77
How does hydranencephaly children act?
Normal initially.
But at 6mo still primitive reflexes.
rarely progress beyond social smiling and seizures are common.
78
What is Holopros encephaly?
Failure of the telencephalic vesicle to cleave into 2 cerebral hemispheres.
From severe alobar to semilobar and lobar.
79
80% of Holoprosencephaly have a genetic defect. Which?
Trisomy 13.
80
Why is it important to differentiate microcephaly from craniocynostosis?
Treatment might improve cerebral development in craniosynostosis pt.
81
Risk factors for microcephaly?
* infections during pregnancy: Rubella, toxoplasmosis, cytomegalovirus, zika virus
* Severe malnutrition
*maternal exposure to cocain or alkcohol
*interruption of blood supply during pregnancy
82
prenatal detection of neural tube defects are done in several ways. Name 3.
* Serum alpha-fetoprotein
* Ultrasound
* Amniocentesis
83
How high sensitivity does sAFP have for spina bifida?
91%
84
How high sensitivity does sAFP have for anencephaly?
100%
85
How sensitive is ultrasound for spina bifida?
90-95%
86
Another name for spinal dysraphism
Spina Bifida
87
What three types of spinal dysraphism are there?
* Spina bifida occulta
* Meningocele
* Myelomeningocele
88
Spina bifida occulta may be associated to another deformity that might give problems. Which?
Tethered cord.
89
What are the most common symtoms of tethered cord?
* gait disturbance
* leg weakness
* atrophy
* urinary disturbance
* foot deformities.
90
How many percent of patients w MM develop hcph?
65-85% (over 80% before age 6mo)
91
How common is latex allergy in MM children?
up to 73% thpught to be iatrogenically caused.
92
What might be good with intrauterine closure of MM defect?
* reduced chiari II defect
* possibly reduced incident of hcph.
Obs does not improve distal neurologic deficits.
93
If a MM lesion is ruptured, what needs to be done?
* beta-lactamase-resistant pencillin
* Gentamicin
* cover lesion with wet sponges not to dry out.
*Keep child in trendelenburg position on the stomach to keep pressure off lesion.
94
Levels of MMs and associated problems
* complete paralysis - Th12
* Possible problems w clawing toes with growth - S2
läs mer page 282
95
How many other additional abnormalities are seen in a MM child on average?
2-2.5.
96
What additional abnormalities needs looking out for before surgery?
* pulmonary immaturity - may preclude surgery.
* Bladder - start pt on regular urinary catheterizations and consult urologist
* Assess scoliosis and kyphosis
97
Why is closure prefered before 36h from birth?
Due to colonization and increased risk of infection.
98
What are the most important late problems/issues for MM patients?
* hcph or shunt malfunction
* syringomyelia
* Thethered cord syndrome (70% have on imaging but only minority is symtomatic)
* scoliosis
* Dermoid tumor at MM site incidence 16%
* Chiari II formation
99
What is the outcome with modern treatment for MM ?
* 85% of infants survive. Most common early cause of death is Chiari, late is shunt malfunction.
* 80% normal IQ. -mental retardation usually correlated to shunt infection.
* 40-85% ambulatory with bracing! But most use wheelchairs for ease.
* 3-10% normal urine continence, most use intermittent catheterization.
100
Why is it important to notice a dermal sinus?
Some connect with coccyx, some with the dural tube.
SOme form a epidermoid cyst containing keratine. Some contain sebum and hair
101
Why has Klippel-feil patients short neck with limitation in movement?
Due to 2-more cervical vertebra fused.
Ranging from only fusion of the body-congenital block vertebra; to fusion of the entire vertebra.
102
What clinical associations are usually seen e Klippel-Feil syndrome?
* scoliosis 60%
* Sprengels deformity 25%
* facial asymmetry, torticollis, with more!
Non-neural are unilateral deafness 30!%, unilateral loss of a kidney, with more
103
What is (strangely) the most common needed surgery for KLippel-Feil syndrome pt?
Fusion of unstable non-fused segments. At the risk of further loss of mobility!
104
What should you expect if a MM pt has:
* increasing scoliosis
* increasing spasticity
* worsening gait
* deteriorating urodynamics
1! always rule out shunt dysfunction
2. Pain? - tethered cord until proven otherwise
3. No pain - syringomyelia until proven otherwise
4. May be due to Chiari II malformation -brainstem compression.
105
What is a "split cord malformation"?
embryologically developed double spinal cord
106
Type 1 split cord malformation?
* 2 hemicords
* both have their own central canal and surrounding pia and separate dural tube.
* SEPARATED BY A RIGID OSSEOCARTILAGINOUS MEDIAN SEPTUM.
* abnormalities of the spine is seen by the split.
107
! Obs! In surgery of tethered cord in split cord formation 1!!
The median septum should be removed before detethering!! Otherwise the cord may retract up against the septum. Auch.
