Flashcards in Paediatric Deck (205):
What is the success rate for ETV with tectal glioma?
What score is used to calculate success of ETV?
Kulkarni et al 2010 ETV success score based on :
Age - Older the better
Etiology - bad if post infectious and high if tumour
Previous shunt - better if no previous shunt
What else can be done in addition to ETV for obstructive hydrocephalus?
Choroid plexus coagulation
What is better ETV or shunt?
Recent meta-analysis has shown that outcomes are the same due to side-effects of both procedures.
What is the rate of shunt failure in one year for children
What are the reasons for shunt failure in young children?
Obstruction, infection, over-drainage and loculation
Level 1 evidence for neonatal hydrocephalus?
Don't give acetazolamide / serial LPs
What is the risk of VPS in neonates?
Risk of necrotising enterocolitis (NEC) as preterm so may get infected
Child may be too small to shunt
How is neonatal hydrocephalus best treated?
Ventriculosubgaleal shunt +/- reservoir until the child is old enough to shunt
What is the differential diagnosis of an intracranial cyst?
What is the classification for middle cranial fossa arachnoid cysts?
1 - free communication with cistern
2 - little communication
3 - no communication
What is the pathogenesis of arachnoid cyst?
Ball valve mechanism
Fluid produced from cyst wall
Does operating on an arachnoid cyst alter seizure frequency?
What is a hydatid cyst?
The larval stage of tape worm echinococcus granulosus
How is a hydatid cyst resected?
Dowling hydrodissection technique to deliver the cyst
What is the difference between cavum septum, cavum vergae and cavum velum interpositum?
What is the origin of a colloid cyst?
Neuroepithelial remnant of the paraphysis / ependyma
Midline inclusion cyst
What are the contents of a colloid cyst?
Mucin, blood, cholesterol
What is the natural history of colloid cyst?
Over 50 y
What is the differential diagnosis of a colloid cyst?
CSF flow artifact
RARE - aneurysm / glioma / meningioma / pineal region tumour / germinoma
What are the causes of non-obstructive hydrocephalus?
Excess production - choroid plexus papilloma / carcinoma
Reduced absorption - immaturity of arachnoid villi, venous hypertension, IVH
What is the incidence of craniosynostosis?
1 in 2500
Where is the metopic suture?
What deformity does metopic suture craniosynostosis cause?
What does coronal suture craniosynostosis cause?
Brachycephaly if bilateral or anterior plagiocephaly if unilateral
What is the differential diagnosis of posterior plagiocephaly?
Positional vs unilateral lambdoid suture closure
What are the causes of secondary synostosis?
Microcephaly - not due to genetic suture abn but due to lack of brain growth
How can lambdoidal plagiocephaly be differentiated from positional plagiocephaly?
Position of the ear is moved forward with positional and posterior with lambdoid suture fusion
What age do sutures ossify (not close!)
What is the classical correction of scaphocephaly
Barrel stave osteotomies to widen the skull
Performed age 6 - 8 months
Followed by helmeting for 6-12 months if they are young
What does 'copper beating' of the skull suggest in craniosynostosis?
What are the important parts of treatment for brachiocephaly?
Frontal craniotomies followed by fronto-orbital advancements
What syndromes cause craniosynostosis?
These all have FGF mutations:
Apert - syndactyly and flat midface
Crouzon - orbital hypertelorism
Pfeiffer - syndactyly, web neck
How do you perform a classical repair of metopic suture synostosis?
Fronto-orbital advancement and resection of the synostosed suture
What is dolichocephaly?
Long narrow head. Scaphocephaly is a type of dolichocephaly and signifies a pointed suture.
Do you need a CT scan to treat scaphocephaly?
What is spinal dysraphism?
What are the lemon and banana signs?
On 20 week USS: Anteriorly the skull flattens and look like a lemon; Posteriorly there looks to be a banana over where the ambient cisterns are; These are classical findings of a child with a myelomeningocoele.
What embryological abnormality causes spinal dysraphism?
Abnormal closure of the neural tube during primary neurulation
(note secondary neurulation is from S2 down)
What conditions are caused by abnormalities of secondary neurulation?
Thickened filum with tethered cord
** something else
What is MOMS?
Management of myelomeningocoele study - RTC
What are the outcomes of MOMS?
Decreased shunts and chiari 2 in infants with IU repair
What is the prognosis of IU repair of myelomeningocoele?
Sacral will walk
Lumbar 50:50% normal walking
Thoracic will not walk
When should a myelomeningocoele be repaired in a term baby?
Closure within 48 hours; Wrap it in plastic
How does shunt malfunction present in a patient with previous myelomeningocoele repair?
Shunt malfunction can result in leg pain / weakness then think about chiari 2, syrinx, dermoid, arachnoid and tethered cord.
Patient with hairy patch on lower back but otherwise normally. Diagnosis?
Split cord malformation (normal walking and bowel etc)
Patient with spinal cord malformation presenting with recurrent meningitis
What is a sacrococcygeal sinus / pit?
These are low between the gluteal fold and are not of clinical significance as do not connect with the cord
Where is the conus in the newborn?
L3 - returns to L1/2 by 3 months!
When should you operate on a dermal sinus?
As soon as safe due to risk of infection
Where do paediatric tumours occur?
1 year - 75% infratentorial
What is infantile desmoplastic astrocytoma?
WHO grade 1 - can have neuronal differentiation and is called desmoplastic infantile ganglioglioma
Supratentorial mass lesion with cartilage, respiratory epithelial and neural cells. Diagnosis?
What are the histological features of medulloblastoma?
Homer-Wright Rosettes, small blue cells high density
What does the term desmoplastic mean?
Lots of collagen
Which medulloblastoma molecular subtype has the best prognosis?
Wnt (wingless) abnormality
Group 3 have the worse prognosis - have MYC amplification
What are the cerebellar embryonal tumours?
ATRT, medulloblastomas and ETANTR
What are ATRT?
Atypical rhabdoid/teratoid tumour
What are ETANTR?
These are part of a family of Embryonal tumours with multilayered rosettes (ETMRs)
What do ependymal rosettes look like?
Here the cells coalesce around an ependymal lined cavity
What are the features of a desmoplastic infantile glioma?
No restriction, large cyst, no calcification and durally based!
4 year old with hyperdense midline posterior fossa tumour. Diagnosis?
What are the factors for risk stratification of medulloblastomas?
High risk is any of: Age 1.5 cc
What is the management of high risk medulloblastomas?
Radio and chemotherapy gives 20-65% 5 year survival if >3 years
Chemotherapy only and bone marrow rescue if
DD of suprasellar tumours in children?
Chiasmatic hypothalamic glioma - solid or cystic, no calcification and increased restricted diffusion
What is the management of hypothalamic low grade gliomas?
Pineal region tumour with calcification, heterogenous with calcifications and CSF tumour marker negative. Diagnosis?
Mature teratoma - needs surgical resection!
Approaches to the pineal region?
Supracerebellar infratentorial or Occipital Transtentorial
Which vein attaches to the vein of galen from inferiorly?
Precentral cerebellar vein
Differential diagnosis of a pineal region tumour?
Germ cell tumours - germinomatous or non-germinomatous germ cell tumours
Pineal tumours (pineoblastoma / cytoma)
Gliomas - tectal plate
Others - choroid plexus tumour / cavernoma
What does raised CSF bHCG suggest?
What does raised CSF AFP suggest?
When should a pineal region tumour be biopsied?
If the tumour markers are absent. If germinoma then no resection; If teratoma, glioma or pineal origin then resect the tumour;
What is the diagnostic yield of stereotactic biopsy for pineal region tumours?
90% (1% mortality and 1% major morbidity)
Pineal region tumour - ETV or biopsy first?
ETV first as this is main goal. No risk of dissemination of tumour cells by doing ETV before biopsy.
Is endoscopic or stereotactic biopsy of pineal region tumours better?
Stereotactic yield 90%; Endoscopic biopsy 80%;
What are the types of Chiari malformation?
1 - >5mm tonsillar herniation below the FM; Assoc with syrinx
2 - Herniation of the vermis; Assoc with MMC
3 - Occipital or high cervical encephalocoele
4 - Cerebellar aplasia or hypoplasia
(Chiari 1.5 is tonsilllar herniation with elongated brainstem and 0 is where there is no tonsillar herniation but CSF block at the FM)
What are the features of Chiari 1?
Headache - cough related
Syrinx related features:
Syrinx motor / sensory abn
What is the incidence of Chiari 1 in asymptomatic population?
2.5% are asymptomatic and 1% are symptomatic
Why image the entire spine in a child with a chiari malformation?
Tethered cord / MMC / Syrinx
What is the natural history of a child with a Chiari 1 (asymptomatic)?
50% stable, 38% improved and 12% worsened;
No neurological deficit occured
Strahle et al 2011
What is the first neurological sign with a syrinx?
Loss of reflexes
Should a duroplasty be performed with Chiari FMD?
Durham et al 2008 showed no difference in outcome but the re-operation rate was less, however CSF complication rates were higher with opening the dura!!
Features of Chiari 2
Up running nerve roots
Beaking of the tectum
Enlarged massa intermedia
Cerebral heteratopia / polymicrogyria
Skull craniolacunia (dysplasia of the skull)
Low transverse sinus
Small posterior fossa
Should prophylactic chiari decompression be done with MMC?
No as syrinx will only become symptomatic in 15-30%
What are the symptomatic complications of Chiari 2?
Medullary compression - poor feeding, prolonged apnoea and stridor (vocal cord palsy)
Upper cervical spine root injury
What operation should be performed for Chiari 2?
Basically a cervical laminectomy NOT an FMD;
Usually don't need to remove the suboccipital bone
Probably don't need to open the dura in the majority of these patients.
Preterm infant; always stiff and MRI shows reduced white matter in one hemisphere? Diagnosis?
What is the most common cause of intractable epilepsy in children that undergo surgery?
42% is due to FCD
What is FCD?
Focal cortical dysplasia is a disruption of normal cortical lamination
What is the Longterm outcome with VNS for epilepsy?
Up to 50% have >50% reduction in seizure frequency
What is the distribution of tumours in an infant?
<1y = 75% supratentorial
>1y = 75% infratentorial
What is the histology of a desmoplastic .... paediatric tumour?
What is the rosette feature of medulloblastoma?
What stains for connective tissue?
Medulloblastoma with strong reticulin staining?
Desmoplastic / nodular type
What are the types of SHH medulloblastoma?
P53 mutant and non-P53 mutant
Which molecular medulloblastomas are histologically desmoplastic?
SHH group if non P53 mutant
(Anaplastic if SSH p53 mutant)
What are ATRTs?
Tumour of children <5 y
Supra or infratentorial
INI1 protein (SMARCB1 gene)
Which ependymal tumours have the worst prognosis?
Supratentorial RELA positive
What mutation is associated with diffuse midline Gliomas
Very poor prognosis!!
What mutation occurs with Pilocytic astrocytomas?
What are the urinary biomarkers of Pilocytic astrocytomas?
Look up paper
What are the radiological features of a desmoplastic infantile glioma?
No restricted diffusion
Can be durally based
Look up the rest
What paediatric tumour is hyperdense on CT?
Which children have ATRF?
What are the factors that make a medulloblastoma high risk?
High risk =
Tumour residual >1.5cc
What is the medullo high risk treatment protocol >3years?
Rt to craniospinal axis with vincrinstine
Post fossa Rt boost
What is the medullo high risk treatment protocol <3years?
Stem cell transplant
How do manage a DIPG?
No need for biopsy (unless abnormal radiological features)
No role for surgery
What is the prognosis?
Median survival 9 months
Suprasellar lesions with solid or cystic features, bright enhancement, no calcification and increased diffusivity?
Chiasmatic / hypothalamic glioma
(pilocytic or pilomyxoid astrocytoma)
What are the treatment for deep / optic pilocytic astrocytomas?
Chemotherapy!! Only 50% respond;
If no response then Surgical resection
What factor favours teratoma in the pineal region?
Calcification and marker negative
Needs surgical resection
What is a homogeneous tumour in the pineal region?
What vein is encountered in the midline during a supracerebellar infratentorial approach?
Precentral cerebellar vein
What marker are sent for pineal region tumours?
bHCG (germinoma if <50; NGGCT if >50)
What do you do if the tumour markers are negative with a pineal region tumour?
If suspected teratoma then resect
Otherwise biopsy (stereotactic if small ventricles or endoscopic with ETV - needs 2 burr holes if rigid scope)
With pineal region tumour - do you do ETV or biopsy first?
ETV first and biopsy second as the main goal is to treat hydrocephalus. If the biopsy bleeds ++ you might not be able to do the ETV
Does ETV cause tumour CSF spread?
No evidence of increased risk
Which condition has narrowing at the jugular foramen?
What is the treatment for tectal glioma?
How do you calculate the chance of ETV success?
Kulkarni et al 2010:
Age (max 50)
Etiology (max 30)
Previous shunt (max 10)
What procedure can you do to improve the success of ETV?
Choroid plexus coagulation (need more than 90% to be coagulated so need a flexible scope!)
What is the shunt failure rate?
40% in the first year and 10% the year after
What are the types of germinal matrix haemorrhage?
What interventions are done for hydro secondary to IVH in perinatal period
Subgaleal reservior to buy some time until pt is older and then shunt the patient
EVD - risk of infection
VP shunt - risk of NEC and infection
ETV - likely unsuccessful, no information
See the systemic review in JNS:paeds 2014 for hydrocephalus management in paeds
What is craniosynostosis?
Premature closure of 1 or mor calvarial suture
What is craniostenosis?
The skull is too small for the brain
What is an elongated boat shaped head called?
Scaphocephaly (sagittal sinus)
What is the most common type of craniosynostosis?
How do you classify craniosynostosis?
Isolated vs syndromic
Single vs multiple
Primary vs secondary
What are the secondary causes of craniosynostosis?
Which type of craniosynostosis is increasing in frequency?
What is the mercedes benz syndrome?
Bilateral lambdoid and posterior sagittal suture synostosis?
What tests can you do for craniosynostosis?
USS of the sutures
CT (high radiation!)
What is the difference between dolichocephaly and scaphocephaly?
Dolichocephaly is positional whilst scaphocephaly is craniosynostosis
What happens the eyes with anterior plagiocephaly?
Orbital dystopia (eye is pulled up on the synostosis side!) Needs an orbital advancement
How can you differente positional from craniosynostosis plagiocephaly?
The eyes are not affected with positional - So called Harlequin eye!
What is metopic suture?
Between the two frontal bones
What is caused by metopic suture synostosis?
Trigonocephaly - do a CT scan if you think they have other midline fusion abnormalities such as cleft lip etc
How you differential lambdoid synostosis from posterior positional plagiocephaly?
The ear goes back with and the mastoid is lower with lambdoid fusion
Do you do a CT scan for lambdoid synostosis?
Yes as the diagnosis is difficult
How do you prevent blood loss in paediatrics?
Can you take a craniotome across an open suture?
No - It will tear the sinus!!
Do you put the bone back after craniosynostosis surgery?
<6 months - don't put bone back
>6 months - put bone back
What technique do you know for fixing scaphocephaly?
Clam shell procedure with barrel stave cuts
Put helmet on after to prevent AP growth and promote medial-lateral growth
What is moved forward with fronto-orbital advancement?
What are the common syndromes with craniosynostosis?
Which has polysyndactyly
What is clover leaf skull?
Tree-foil appearance seen with Pfeiffer's syndrome
Which syndromic craniosynostosis has hydrocephalus?
Pfeiffer > Crouzon > Apert
What is the genetic condition behind craniosynostosis?
Which patients with craniosynostosis should you do genetic testing?
What is a Chiari 0
Syrinx with CSF block at the FM but no tonsillar decent
What is platybasia?
Flattening of the skull base - clivus is shallow with angle >145 with the anterior skull base
What proportion of children have an asymptomatic chiari 1?
Which Chiari patients need a sleep study?
History of snoring
Do you operate on an asymptomatic chiari?
What is the natural history of an asymptomatic Chiari malformation?
Would you operate on an asymptomatic Chiari with syrinx?
Most do not deteriorate and there is no evidence to support surgery. Reasonable to observe or do surgery.
What can be mistaken for the tonsils during Chiari 2 operations?
Is agenesis of the corpus callosum associated with Chiari 2?
What is craniolacunia?
Think areas in the skull
Is enlarged mass intermedia associated with Chiari 2?
YES! Also beaking of the tectum
Which patients with Chiari 2 have the worst prognosis?
Stridor with bilateral VC palsy at birth!
Where is the transverse sinus in Chiari 2?
Very low near the FM
What is the treatment for Chiari 2?
Cervical laminectomy not FM decompression
Should you decompress a medullary cyst below the tonsils in a patient with Chiari 2?
Do you need to open the dura with Chiari 2?
What is the lemon and banana sign associated with?
On in utero USS is associated with Myelomeningocoele (Chiari 2 seen on MRI)
Myelomeningocoele is associated with which developmental process?
How can you differentiate a problem with primary or secondary neurulation?
Secondary is below S1/2; Associated with sacral agenesis and rectal malformation
Which conditions are associated with failure of gastrulation?
Split cord malformation
What conditions are associated with secondary neurulation failure?
What is the MOMS study?
RCT into the prenatal repair of myelomeningocele:
Reduces shunt requirement
Reduces Chiari 2
Improved lower limb neurology
Do you need to do a C-section for myelomeningoceole?
No evidence either way
What should you rule out in patient with back pain and myelomeningocele?
What is the most likely malformation associated with a hairy patch on the lower back of a child?
Split cord malformation = diastomatomyelia (gastrulation problem)
What are the types of split cord malformation?
type 1 = 2 dural sacs with 2 cords separated by spur
type 2 = 1 dural sac with 2 cords
What do you do with a ventral dural defect with a split cord malformation?
What causes a skin abnormality and a low lying cord?
Limited dorsal myeloschisis
What is Limited dorsal myeloschisis
Disorder of primary neurulation resulting in incomplete fusion of the neuropore. Associated with a skin pit / crater (look like cigarette burns). A band of fibrofatty tissue from the skin attaches to the spinal cord and tethers it!
What are the problems with dermal sinuses?
What is the saccular form of limited doral myeloschisis?
Cyst at the skin with connection to the cord that needs to be disconnected intradurally. Cysts can be incontinuity with the syrinx (myelocystocoele).
What is it called when there is a large lipoma tethering the cord?
Dorsal, transitional or transitional conus lipoma - they slowly loose neurological function so cord needs untethering and aggressive removal of all the fat!
What are occult dysraphism?
Closed myelomeningocele - the neurologicla deficit is usually due to tethering
At what age does the conus reach the same position as a child?
Why operate on spinal dyraphism
Remove portal for infection
Eosinophilia granulomatosis in bone
Observe (or curettage / steroids to lesion)
Bone punched out lesion, thickened at edges. Midline near ant fontanelle.
Resect it. No need for craniotomy.
Growing skull fracture complication
Types of hypertonia
What is spasticity?
Velocity dependent hypertonia
Causes of abnormal gait in a child with CP
Scales for spasticity
Pros and cons of selective dorsal rhizotomy vs baclofen
In paediatric thalamic tumours can grade be estimated from imaging?
NO -- pilocytic and anaplastic all look the same. Need to fully resect the lesion.
Causes of paediatric infarction?
What conditions are associated with Moyà Moyà
Atretic parietal encephalocoele
Look it up!
What are the scalp masses in a baby?
Make a list...