1. Journal Club
Department of Neurosurgery
SCTIMST, Trivandrum
2nd Sept 2010
Adesh Shrivastava
JUNE 2010 Volume 5, Number 6
2. Transnasal odontoid resection followed by posterior
decompression and occipitocervical fusion in children
with Chiari malformation Type I and ventral brainstem
compression
Todd C. Hankinson, M.D.,1 Eli Grunste in, M.D.,2 Paul Gardner, M.D.,3
The odore J. Spinks, M.D.,4 and Rich ard C. E. Anderson, M.D.1
1Department of Neurosurgery, Columbia University, College of Physicians and Surgeons,
2Department of Otolaryngology-Head and Neck Surgery, New York Presbyterian Hospital,
Columbia University, College of Physicians and Surgeons, New York, New York; 3Department of
Neurological Surgery, University of Pittsburgh Medical Center Presbyterian, Pittsburgh,
Pennsylvania; and 4Department of Neurosurgery, Scott and White Neurosciences Institute, Texas
A&M College of Medicine, Temple, Texas
3. Introduction
• A minority of patients with CM1 have an associated
irreducible ventral compressive pathological features
such as basilar invagination or a severely retroflexed
odontoid process.
• This complicated situation demands additional
ventral decompression, the most common being
trans-orally.
4. Rationale for the article
• Problems with transoral approaches:
– the working area may be restricted by a small oral
cavity in young patients
– Superiorly located lesions may be difficult to reach
and may require splitting of the soft and/or hard
palate
– Oral palatal dysfunction from lower cranial nerve
palsies may be exacerbated by a transoral approach
– Intraoral retraction results in considerable airway
edema, and may require prolonged postoperative
intubation.
5. Rationale for the article
• The endoscopic transnasal approach, as described by Kassam
and colleagues, has been described for odontoid resection
primarily in the adult population.
• Authors present the first reported use of this approach in
pediatric patients with CM-I.
• The endoscopic transnasal approach was used in 2 children
who presented with CM-I complicated by irreducible ventral
brainstem compression.
• This method provided excellent surgical access to rostral
pathological entities, and avoided a transoral approach.
6. Case Report 1
• 15 year old boy of CM1
• C1 laminectomy one year back.
• Developed recurrent occipital headaches, hypernasal
speech, palatal and swallowing dysfuction, loss of
gag reflex and myepopathy.
• Admission MR and CT showed persistent CM1 with a
retroflexed odontoid process causing ventral
brainstem compression, platybasia and congenital
C2-3 posterior element autofusion.
• No evidence of syrinx.
8. Case Report 1
• Underwent endoscopic transnasal odontoidectomy
followed by posterior decompression and Oc-C2
fusion with iliac crest graft.
• Tracheostomy was done post op.
• At one month follow up the patient had complete
resolution of headaches, with normalization of
speech and swallowing functions and improvement
in myelopathy.
• At 9 months there is radiographic evidence of fusion
and stable symptom improvement.
9. Case Report 2
• 11 year old girl
• Mild developmental delay with progressive scoliosis.
• MR demonstrated CM1 with platybasia, syringobulbia and
syringomyelia.
10. Case Report 2
• Underwent endoscopic transnasal odontoidectomy followed by posterior
fossa decompression and Oc-C2 fusion with rib graft and BMP.
• Post op imaging revealed immediate syringobulbia resolution.
• At 18 months she was asymptomatic and scoliosis has not progressed.
11. ETO Technique
• Supine position with head slightly flexed but not turned.
• Oral endotracheal intubation and placement of electrophysiological
monitoring leads.
12. ETO Technique
• Adenoidectomy is performed using microdebrider and
electrocautery using tori of the eustachean tube as landmarks
for the latereal extent of the dissection.
• A partial posterior septectomy may be performed to widen the
exposure to the prevertebral region.
• Divide the prevertebral facia in midline and retract soft tissue to
expose the CI arch and the entire width of dens.
• Decompression is completed with drill, currette and kerrison
rongeurs and confirmed with stereotaxy.
• Patients are kept intubated, with cervical spine immobilization,
until posterior decompression and fusion are performed.
13. Discussion
• Authors present the first experience with using a transnasal
corridor to accomplish ventral decompression in children with
a CM-I that is complicated by ventral brainstem compression.
• A small oral cavity may impair the surgeon’s exposure and
preclude an adequate decompression transorally.
• Severe basilar invagination, for example, may require a more
superiorly directed trajectory than can be easily achieved
using a transoral approach without splitting of the soft and
hard palates.
• It is therefore necessary for pediatric neurosurgeons to be
familiar with an alternative corridor for ventral
decompression.
14. Discussion
• One major advantage of this approach is In
young children, for instance, or those with
craniofacial abnormalities such as midface
hypoplasia, micrognathia, or macroglossia,
transoral exposure may be very limited, in
part because a significant portion of the oral
cavity is occupied by the retractor.
15. Discussion
• Potential disadvantages of the endoscopic
transnasal corridor:
– Less exposure inferiorly with minimal access
below C2.
– Exposure may be limited by the patients nasal
anatomy.
16. Conclusion
• For the minority of children with a CM-I who
also present with irreducible ventral
brainstem compression, the endoscopic
transnasal corridor represents an alternative
to the more traditional transoral route for
ventral decompression.
17. Chiari malformation Type I in children younger
than age 6
years: presentation and surgical outcome
Gregory W. Albert, M.D.,1 Arnold H. Menezes, M.D.,1 Daniel R.
Hansen, M.D.,1
Jeremy D. W. Greenlee, M.D.,1 and Stuart L. Weinstein, M.D.2
Departments of 1Neurosurgery and 2Orthopaedics and
Rehabilitation, University of Iowa Hospitals and
Clinics, Iowa City, Iowa
18. Rationale for the article
• In the past, CM-I had been diagnosed primarily in adolescence or adulthood.
• Several authors have previously reported on CM-I in children.
• Most of these reports do not focus on the very young age group and instead
include patients up into their teenage years and, in some reports, into the
early part of the third decade.
• In 2002, authors reported their experience with the treatment of CM-I in
children who were younger than 6 years.
• This original report included an analysis of a series of 31 patients and
demonstrated that children age 0–2 years were more likely to present with
oropharyngeal dysfunction and those age 3–5 years were more likely to
present with scoliosis, syringomyelia, and/or headache.
19. Methods
• 39 patients
• Less than six years of age
• Further divided into 0-2 and 3-5 yrs age groups
• Every patient underwent atleast one procedure by the
senior author.
• Reviewed under presentation, treatment and outcome.
• Mean follow up was 42 months (0-146)
20. Results
• Age: 3 to 69 months (Mean = 40 months)
• Sex: 15 males and 24 females
21. Results
• The cohort was subdivided into 2 age ranges: 0–2 and 3–5
years.
• Statistically significant differences in the presentation with
regard to oropharyngeal dysfunction, scoliosis, and
syringomyelia.
• Patients aged 0–2 years presented primarily with
oropharyngeal dysfunction.
• Patients aged 3–5 years of age presented with scoliosis,
syringomyelia and headache.
22. Results
• Oropharyngeal dysfunctions:
– In 20 patients (51.3%)
– MC presentation was GE reflux
– Snoring
– Sleep apnea
– Choking
– Vomiting
• All patients with reflux experienced improvement in this
symptom following surgical treatment of the CM-I at a
mean follow-up of 22 months (range 9–35 months).
23. Results
• Syringomyelia:
– 23 patients
– 10 of these had associated scoliosis
– All experienced improvement after surgery
– One patient had recurrence
• Scoliosis:
– Eleven patients – one having levoscoliosis and the
rest having dextroscoliosis.
– Eight has thoracic and three had thoracic and
lumbar.
– Post surgery eight improved but three has
progression.
24. Results
• Headache:
– 18 had headache at presentation (Six <3 yrs)
– Valsalva induced headaches were documented in
five
– Post surgery:
• Thirteen experienced resolution
• One had initial resolution with later recurrence.
• One had partial reduction
• Two had no improvement.
25. Results
• Surgery:
– All underwent posterior fossa decompressio
– 32 underwent additional cervical decompression
primarily partial or complete C1 laminectomy.
– Cervical facia duroplasty was performed in 30 patients.
– Four patients required transoraltranspalatopharyngeal
decompression and eight underwent occipitocervical
fusion.
– Eight had more than one procedure.
– Two patients had wound infection, one had transient
neurological deficite (disconjugate gaze and gaze ataxia)
– One death unrelated to CM-I or its treatment.
26.
27. Discussion
• Presenting signs and symptoms in children
aged 0–6 years with CM-I are different than
those seen in older patients.
• Oropharyngeal dysfunction, syringomyelia,
and scoliosis are prominent in this patient
population, and the incidence of headaches is
lower than it is in adolescents and young
adults.
28. Conclusion
• Until the advent of MR imaging, CMs-I were typically diagnosed in
adolescence and adulthood.
• Children, particularly very young children, can present a diagnostic
challenge given the rarity of this disorder in individuals younger
than the age of 6 years.
• Also there was a dramatic difference in the signs and symptoms at
presentation between patients aged 0–2 years and those aged 3–5
years.
• Patients in the younger group were much more likely to have
oropharyngeal dysfunction, whereas those in the older group were
more likely to have syringomyelia, frequently associated with
scoliosis.
29. Conclusion
• Headaches were very rare in the younger group, although
they were reported in approximately half of the older
group.
• Most patients experienced improvement or complete
resolution of their preoperative signs and symptoms
following posterior fossa decompression.
• Thus recognition and early treatment of symptomatic CM-Is
in young children frequently lead to good outcomes.
30. Noninvasive detection of the
distinction between progressive
and compensated hydrocephalus
in infants: is it possible?
Paul H. Leliefeld, M.D.,1 Rob H. J. M. Gooskens, M.D., Ph.D.,2
Cees A. F. Tulleken, M.D., Ph.D.,1 Luca Regli, M.D., Ph.D.,1
Cuno S. P. M. Uiterwaal, M.D., Ph.D.,4 K. Sen Han, M.D., Ph.D.,1
and L. Jaap Kapp elle, M.D., Ph.D.3
Departments of 1Neurosurgery, 2Child Neurology, 3Neurology, and 4Julius
Center for Health Sciences and
Primary Care, Rudolf Magnus Institute of Neuroscience, University Medical
Center Utrecht,
The Netherlands
31. Rationale for the article
• Clinical signs and symptoms of hydrocephalus can be clear
and specific, but also subtle, nonspecific, or even absent and
it may be difficult to decide whether shunt placement is
indicated, especially in infants.
• It is often difficult to distinguish between compensated
hydrocephalus, without its risk of cerebral damage, and slowly
progressive hydrocephalus, with its risk of ongoing
parenchymal damage and serious cognitive impairment.
• Therefore, there is a need for the development of better
noninvasive detection methods to distinguish between
compensated and (slowly) progressive hydrocephalus.
32. Methods
• Authors hypothesized that the presence of a
normal CBF and the absence of interstitial edema
can help to differentiate between compensated
and slowly progressive hydrocephalus.
• Raised ICP in infants with slowly progressive
hydrocephalus may cause a 50% decrease in CBF
leading to increased ADC values, especially in the
periventricular white matter, indicating interstitial
edema without cytotoxic edema.
33. Methods
• 18 patients with compensated HCP
• Enlarged ventricles on MR imaging
• ICP within normal range
• No clinical features of raised ICP except
increased head circumference.
• Infants with parenchymal lesions were
excluded.
• Mean followup of 51.9 + 22.9 months.
34. Measurement of ICP
• AFP was measured in all the patients on the
day of MRI using the Rotterdam
Teletransducer.
• All the patients were asleep and without
sedation during the measurement which was
performed for at least 60 minutes.
35. Magnetic resonance imaging
• All patients underwent general anesthesia during the
MR imaging procedure, which was induced by a
pediatric anesthesiologist.
• The infants were asleep for approximately 45
minutes.
• The CBF measurement was completed at the end of
the MR imaging session, which gave the anesthetist
time to optimize the anesthesia for the age and
weight of the infants.
• Thus, all patients were probably in the same state of
sleep.
36. Magnetic Resonance Imaging
• Cerebral blood flow was measured at 1.5T
scannner and documented as ml/100cc of
brain/min.
• Flair images, DWI, Multishot EPI, and ADC
maps were obtained.
• Simultaneous MAPs were obtained and
compared with normal values to check for
whether BP changes accounted for possible
changes in CBF and ADC.
37. Results
• Ten girls and eight boys with clinically
diagnosed compensated hydrocephalus.
• Ages ranged from three days to 16 months.
• Mean ICP was 7.8 + 7 cm H2O and CBF was
53.5 ml/100 cc brain/min and fell in the
normal range.
• Mean ADC was 890 X 10-6 mm2/sec. and was
in the normal range.
38. Discussion
Leliefeld PH, Gooskens RHJM, Vincken KL, Ramos LMP, van der Grond J,
Tulleken CAF, et al: Magnetic resonance imaging for quantitative flow
measurement in infants with hydrocephalus: a prospective study. J Neurosurg
Pediatr 2: 163–170, 2008
- There occurs a 50 % decrease in CBF in infants with
slowly progressive hydrocephalus.
39. Discussion
Leliefeld PH, Gooskens RHJM, Braun KPJ, Ramos LMP, Uiterwaal CSP, Regli LPE,
et al: Longitudinal diffusion-weighted imaging in infants with hydrocephalus:
decrease in tissue water diffusion after cerebrospinal fluid diversion. Clinical
article. J Neurosurg Pediatr 4:56–63, 2009
- The ADC value measurement using DW imaging has
detected a pathological state of the brain parenchyma,
especially in the periventricular white matter, in infants
with progressive hydrocephalus.
40. Discussion
• The authors claim that this is the first study of infants
with compensated hydrocephalus to reveal a
discrimination possibility between progressive and
compensated hydrocephalus using MR imaging.
• And thus infants with compensated hydrocephalus
can be monitored noninvasively using repeated MR
imaging for the detection of possible decompensation
of the hydrocephalus.
41. Conclusion
• It is very important to gather as much information as
possible about patients with hydrocephalus because
there is a high rate of complications following CSF
drainage in these patients.
• Optimizing the right time of shunt insertion is
essential, particularly in the young infant considering
the effects of patient age at the time of first shunt on
infection and repeated shunt failure.
42. Conclusion
• Noninvasive measurement of CBF in infants with
compensated hydrocephalus reveals a normal CBF,
whereas CBF is decreased in infants with (slowly)
progressive hydrocephalus.
• The ADC values in infants with compensated
hydrocephalus are low to normal, whereas infants
with slowly progressive hydrocephalus have high
ADC values.
• Authors thus recommend including both these
additional imaging to the standard MR imaging
protocol for infants with hydrocephalus.
43. A simple method to reduce infection
of ventriculoperitoneal
shunts
Atiq-ur Rehman, F.R.C.S., F.I.C.S.,1 Tausif-ur Rehman, M.B.B.S.,2 Hassaa n H.
Bas hir,4
and Vikas Gupta, M.B.B.S.3
1Department of Neurosurgery, King Fahd Specialists Hospital, Al-Qassim, Saudi
Arabia; Departments of
2Neurosurgery and 3Neurology, University of New Mexico, Albuquerque, New
Mexico; and 4The Aga Khan
University Hospital, Karachi, Pakistan
44. Introduction
• Postoperative shunt infection is the most common
and feared complication of ventriculoperitoneal (VP)
shunt placement for treatment of hydrocephalus.
• Infection rates have not been low and range from 5
to 15%.
• The highest infection rates have been observed
during the 1st month of placement, in patients who
require serial revisions and in patients who have
undergone shunt revision after treatment for
infection.
46. Rationale for the article
• It is widely accepted that the source of infection lies
intraoperatively.
• Possible means of contamination:
– Surgeons glove
– Patient’s skin flora
• These transfer during the intraoperative shunt
handeling.
• The study aims to determine a simple, cost-effective
and practical method to reduce infection rates.
47. Methods
• Retrospective analysis of 111 patients with
obstructive hydrocephalus at King Fahad
Specialist hospital, Buraidah.
• Since infection rate was high, hospital decided
upon double glove strategy with removal of
outer pair before shunt handling.
• Initially no study was proposed, but as
infection appeared to decrease it was decide
to analyse the results.
48. Methods
• All cases were full term born at hospital with
congenital hydrocephalus between ages 0 and
6 weeks.
• Premature babies, meningomyeloceles, IVH,
Sepsis, meningitis, or fever in the past 48
hours were excluded.
• A Codman’s VP shunt with a small reservoir
was used in all cases.
49. Methods
• Group A: 54 patients between 2001 – 2003; using
conventional technique.
• Group B: 57 patients between 2004 – 2006; using
double glove technique.
• All patients postoperatively were monitored for
fever, tachycardia and WBC count.
• If any of these were positive then CSF study done
from the reservoir.
• Infection was defined as positive CSF culture.
• Follow up period was six months.
51. Discussion
• Methods universally applied to reduce infection include
preoperative systemic antibiotics and specific surgical
protocolincluding limited access to the operating room and
performing the procedure in the early morning to reduce bacterial
presence in the environment.
• To minimize contamination, a so-called no-touch technique is
adopted with antibiotic solution wound irrigation, double gloving,
and antibiotic-impregnated shunt use.
• However, evidence exists to discredit the effectiveness of antibiotic-
impregnated shunt. It has recently been shown that a combination
of intraventricular and systemic antibiotic use significantly reduces
infection rates compared with systemic use alone.
• While all these techniques have been reported to be effective
against shunt infections, it is still reasonable to seek a simpler and
cheaper alternative.
52. Conclusion
The study shows that a changing of gloves
before handling the shunt catheter may be a
simple and cost effective way to reduce the
burden of postoperative shunt infections.
53. Late obstruction of an endoscopic third
ventriculostomy
stoma by metastatic seeding of a recurrent
medulloblastoma
Flávio Nigri, M.D., Ph.D., Carlos Telles, M.D., Ph.D.,
and Marcus And ré Acioly, M.D.
Department of Surgical Specialties, Division of
Neurosurgery, Pedro Ernesto University Hospital, State
University of Rio de Janeiro; and Department of Neurology,
University of São Paulo, Brazil
54. Rationale for the article
• ETV has been considered as an established treatment
for tumoral obstructive hydrocephalus.
• Delayed closure of the ETV stoma has been
attributed to scarring involving the third ventricular
floor which is occasionally associated with rapid
deterioration and fatal outcome.
• This is a report on a unique case of late closure of the
ETV stoma caused by metastatic seeding of a
recurrentmedulloblastoma 9 years after the initial
treatment.
55. Case Report
• 18 year old male with raised ICP features.
• ETV for medulloblastoma induced HCP 9 years
back (surgery plus adjuvant RT and CT).
• Cranial MR imaging demonstrated
hydrocephalus and recurrent posterior fossa
tumor along with an enhancing mass at the
third ventricular floor, especially at the
infundibular region.
56. Case Report
• At a second endoscopic surgery, a reddish mass
located just anterior to the mammillary bodies
was resected, uncovering a patent stoma of the
previous ETV, which was further enlarged with a 3
fr fogarty catheter.
• Postoperatively, the symptoms of intracranial
hypertension were completely relieved.
• Histopathology of the third ventricular mass
confirmed the diagnosis of classical
medulloblastoma.
57. Case report
• One year after surgery, however, the patient
presented with widespread CSF medulloblastoma
dissemination in which several intraventricular
nodules were noted.
• The ETV stoma was again occluded, but a
ventriculoperitoneal shunt was inserted at this
time.
• The disease remained stable 1 year after
shunting, and then the tumor proceeded to grow
and disseminate.
• The patient died 9 months later.
58. Discussion
• Medulloblastoma has a well-recognized capacity
for CSF dissemination and is described as the
most common type of tumor to spread through
CSF pathways.
• Although previously hypothesized, secondary
obstruction of the stoma due to intraventricular
tumor dissemination of the CSF has not been
described until the present case.
59. Conclusion
• This case is the first reported instance of ETV
obstruction due to metastatic CSF seeding of a
medulloblastoma.
• In such cases of late failure of the ETV stoma
due to tumor obstruction, a second ETV can
be safely performed resulting in adequate
function of the stoma, even if limited by the
aggressive nature of the disease.
