Department of Neurosurgery, All India Institute of Medical Sciences, Bhopal –

462020.

Thesis Topic:-Prospective Study For Evaluation Of CSF Flow Dynamics Across

Foramen Magnum In Adult Chiari Malformation/Syringomyelia Complex Patients
And Its Clinical Correlation With Outcomes After Surgery

Candidate:-
Dr. Manas Prakash
Senior Resident (academic)
Department of Neurosurgery
AIIMS, Bhopal

Guide:-
Dr. AdeshShrivastava
Associate Professor
Department of Neurosurgery AIIMS, Bhopal

Co-guide:- Co-Guide:-
Dr. RadhaSarawagi Gupta Dr. Pradeep Chouksey
Associate Professor Associate Professor
Department of Radio diagnosis Department of Neurosurgery

AIIMS, Bhopal AIIMS, Bhopal AIIMS, Bhopal AIIMS, Bhopal

1
TABLE OF CONTENTS

2
3
LIST OF FIGURES

Figure 1 Morphology of herniated tonsil in patients with Chiari malformations .................... 20

Figure 2 Chairi 0 and Chiari 1 malformation ............................................................................. 22

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ABBREVIATIONS

AAD- Atlantoaxial Dislocation

ADL- Activities of Daily Living

CCOS- Chicago Chiari Outcome Score

COPI- Chiari Outcome Predictability Index

CSF- Cerebrospinal Fluid

CVJ- Craniovertebral Junction

ECF- Extra Cellular Fluid

FMD- Foramen magnum decompression

FMD+LD- Foramen magnum decompression and lax duraplasty

FMD+LD+C1- Foramen magnum decompression and lax duraplasty and C1 arch Excision

mCCOS- Modified Chicago Chiari Outcome Score

SS- Vertebral level where Syringomyelia starts

SE - Vertebral level where Syringomyelia ends

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6
 INTRODUCTION

Chiari 1 malformation refers to cerebellar tonsillar descent below foramen magnum (1–3). Chiari 1

malformation is a debilitating disease, with varying symptoms, ranging from headache, to myelopathic

features, to involvement of lower cranial nerves ((4–7)). However, Chairi like symptoms may arise in

patients with compromised posterior fossa in absence of tonsillar descent (Chiari 0 malformation)((8)).

The exact pathophysiology of Chiari

1malformation is unclear. The notion of a Chiari “0” malformation disputes the concept that tonsillar

herniation is even necessaryfor a Chiari malformation–like pathophysiology to exist.(9-11)

Chiari 1 malformation is found frequently in association with syringomyelia (cystic cavity in spinal

cord)(4-6). Multiple theories have been put forward to explain the relationship between syringomyelia

and Chiari 1. Malformation(5,6,12–14), however, each have its own shortcomings(15,16). It is the

leading cause of syringomyelia (17–19) and also seen with osseous abnormalities of skull base.

Despite of investigation and discussion of the pathogenesis responsible for the progression of

syringomyelia associated with Chiari I malformation, the pathophysiological mechanisms remain

poorly defined.

Treatment for Chiari 1 malformation aims at establishing CSF flow at the level of foramen

magnum(6,20) or to fix the atlantoaxial joint in view of instability(21,22). The aim of these surgeries is

to halt and reverse the progression of syringomyelia as well as to improve the quality of life of the

patients(4,23,24). However, outcome of surgery varies amongst patients(4,25–27).

In conducting cerebrospinal fluid (CSF) flow studies, investigators have attempted to correlate the

clinical severity of these lesions with general flow velocity or bulk flow at the foramen magnum;

however, these techniques have not allowed consistent prediction of symptomatology, explanation of

the presence of syringomyelia, or the assessment of the hydrodynamic characteristics of the

decompression.

In the presence of unclear pathophysiology, various treatment methodologies and varied outcome. it is

7
difficult to explain the expected surgical outcome to the patient. There are various studies on surgical

outcome but a very few on predictors of outcome(23,25,26,28).

There is a need for a tool for helping neurosurgeons to stratify their patients and prognosticate them

about their expected outcome.

We will study various clinical and radiological factors and CSF Flow parameters (pre-operative and

post-operative), which affect the post-surgical outcome of patients. This will help to stratify patient, in

accordance with their expected outcome.

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REVIEW OF LITERATURE

History

Hans Chiari described malformations of hindbrain in relation to skull base in 1891in his

publication ‘Ueber Veränderungen des Kleinhirns infolge von Hydrocephalie des

Grosshirns’ (concerning changes in the cerebellum due to hydrocephalus of the cerebrum) in

(7,8). He described Chiari 1 malformation as peg-like elongation of cerebellar tonsils which

accompany the medulla oblongata into the spinal canal(7).

However, before Hans Chairi, Theodor Langhans, in 1881, had published ‘regarding cavity

creation in the spinal cord as a consequence of obstruction to blood flow’ (23), where he

made observations regarding cavity in spinal cord and its relation with tonsillar herniation,

which he termed as ‘pyramidal tumours’ (24). This was probably one of the oldest paper on

relationship of Chiari 1 malformation with syringomyelia (24).

Syrinx is a fluid-filled cavity within the spinal cord (25). Syringomyelia refers to resulting

disease complex because of syrinx (25). Charles Estienne (1503–1564), provided the first

description of cavities in the spinal cord, and of the central spinal canal, in his treatise ‘La

dissection du corps Human’ in year 1543. Charles Prosper Ollivier d'Angers (1796–1845),

was the first person to coin the term syringomyelia (26,27).

Chiari Malformation and Syringomyelia

Chiari 1 Malformation

Chiari1 malformation refers to cerebellar tonsillar descent below foramen magnum (7,28–

30). It is the leading cause of syringomyelia (30–32) and also seen with osseous

abnormalities of skull base.

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The true incidence of Chiari 1 malformation is not known. Meadows and colleagues (33)

found that of 22,591 patients who underwent MRI of the head, 175 (0.775%) were found to

have tonsillar herniation, extending more than 5 mm below the foramen magnum.

Aboulezz et al. (34) in 1985, studied 82 patients and concluded that tonsillar herniation up to

3 mm was considered normal, between 3 and 5 mm it was borderline, and clearly pathologic

when it exceeded 5 mm.

In 1986, Barkovich et al (35) analysed 200 selected ‘normal’ patients. They found that, the

normal position of the tonsils was from 8 mm above to 5 mm below foramen magnum.

Lowest threshold of tonsillar herniation, for predicting symptomatic Chiari 1 malformation

patients was 2mm with 100% sensitivity and specificity is 98.5%.

Mikulis et al, 1992, believed that, to look for a single reference standard, which indicates the

normal distance of the cerebellar tonsils from the foramen magnum was inappropriate unless

age was considered (36). Based on their study, they suggested age wise extent of tonsillar

herniation to consider Chiari 1 malformation as given in Table 1.

Decade of life Distance below foramen magnum (mm)

1st 6

2nd to 3rd 5

4th to 8th 4

9th 3

Note-The indicated distances below the foramen magnum are more than 2 SDs outside the

normal range.

By Mikulis et al (36)

Table 1 Suggested Criteria for Ectopia of the Cerebellar Tonsils as a Function of Age

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Milhorat et al. (4), 1999, defined Chiari 1 malformation as tonsillar descent below foramen

magnum. According to them, tonsillar descent of less than 5 mm did not exclude the

diagnosis.

Brandon W. Smith et al (37), 2013, published analysis of 2400 patients undergoing MRI and

organized them into 8 age groups in relation to cerebellar tonsil position. They concluded that

tonsillar descent follows an essentially normal distribution and varies significantly with age.

Patients with pegged morphology (Figure 1) were more likely to have a tonsil location at

least 5 mm below the foramen magnum (85%), when compared with those having

intermediate (38%) or rounded (2%) morphology (p < 0.0001). Female sex was associated

with a lower mean tonsil position (p < 0.0001). Patients with a lower tonsil position also tend

to have an asymmetrical tonsil position, usually lower on the right (p < 0.0001). He

postulated that probably Chiari symptoms and the formation of spinal syringes were the result

of crowding at the foramen magnum, which also cause abnormal cerebrospinal fluid (CSF)

movement at the craniocervical junction (37,38).

a. b. c. d.

a. Pegged morphology of tonsils, b. Rounded morphology of tonsils, c. and d.

intermediate morphology of tonsils

Figure 1 Morphology of herniated tonsil in patients with Chiari malformations

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Changing Concepts, From Tonsillar Herniation to CSF Flow Abnormality to

Craniocervical Instability

The pathogenesis of Chiari malformations remains elusive.

In 1978, Nyland and Krogness, noted relatively small size of the posterior fossa in patients

with Chiari I malformation (39). In 1995, Batzdorf and his colleagues found, that compared

to normal persons, the ratio of the posterior fossa volume to the supratentorial volume was

diminished in many patients (40).

Both Milhorat (4) and Brandon W. Smith (37) have cited that Chairi 1 malformation was not

just mere tonsillar herniation but more of crowding at foramen magnum, obliterating

retrocerebellar spaces, and thus hindering normal CSF flow.

Oldfield, divided Chiari 1 patients into 2 distinct groups, “crowded” and “spacious” groups,

with tonsil impaction in both sets of patients (41). He noted that significantly more volume is

occupied by hindbrain tissue in the subset that appears to have a crowded posterior fossa as

compared to spacious group. He suggested that the pathogenesis of Chiari 1 malformation

cannot be explained only by crowded posterior fossa and there must be some other

mechanism, currently unrecognized, resulting in impaction of the tonsils in the foramen

magnum (5). Based on his clinical study of 48 patients with a typical Chiari 1 malformation,

he noticed that in postoperative patients, the peg-shaped tonsils become round along with

disappearance of the cervicomedullary protuberance and diminution of tonsillar ectopia by

51%. He told that this happens in essentially all patients. After successful surgery, the

subarachnoid space opens to provide normal pulsatile CSF flow across the foramen magnum.

According to him, ‘the Chiari malformation’ is a result of systolic impaction of the tonsils in

the foramen magnum with each pulse, which occurs over 120 000 times per day with a pulse

of 85. It is not a ‘malformation’, as it is an acquired disease, not congenital.’

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Chiari 0 malformations is an alteration, characterized by compromised posterior fossa with,

low tip of the obex and normal position of the tonsils (42–46). Tubbs et al (43), found that

location of the brainstem was caudally displaced more than 3 standard deviations below

normal. Chiari 1 malformation and Chiari 0 malformation are spectrum of same disease

(8,47). They both are result of CSF flow abnormality which occurs at foramen magnum due

to tonsillar herniation or due to compromised posterior fossa or any other factors

(8,42,43,48,49). The concept of CSF flow abnormality instead of tonsillar herniation being

the cause, explains the occurrence of symptoms irrespective of magnitude of tonsillar

descent.

Figure 2 Chairi 0 and Chiari 1 malformation

Atul Goel(50), based on his analysis of outcome of 65 patients treated by atlantoaxial fixation

proposed that the pathogenesis of Chiari 1 malformation, with or without associated basilar

invagination and/or syringomyelia, is primarily related to atlantoaxial instability. He also

13
proposed that development of tonsillar herniation is a natural protective phenomenon against

instability .

Syringomyelia

Around 32-100% of Chiari 1 malformation patients have syringomyelia (1,4,51).

The prevalence of syringomyelia ranges, in different countries, between 2 and 13 per 100,000

inhabitants (52). Gender distribution of syringomyelia have been reported from female

preponderance to equal for both sexes (53,54). The prevalence of syringomyelia varies from

place to place and changes with economic development (52).

Various theories have been put forth to explain the occurrence of syringomyelia with Chiari 1

malformation.

Gardner et al (3) had put forth hydrodynamic theory in 1965. He proposed that there is a

blockage of the foramen of Magendie which results in a "water hammer"-like transmission of

pulsatile CSF pressure from a communication between the fourth ventricle to the central

canal of the spinal cord via obex. The closed foramina direct an intraventricular CSF into

central canal and herniating structures through foramen magnum act as a one-way pump and

valve, creating hydromyelia which then expands and forms syringomyelia.

Williams et al (9,10,55) criticized Gardner’s theory. He argued that failure of the fourth

ventricular outlets to open in a timely manner should lead to intrauterine hydrocephalus, yet

only the minority of patients with syringomyelia and Chiari 1 malformation have

hydrocephalus. According to Williams, due to blockage of foramen magnum there is

craniospinal pressure dissociation. During Valsalva-like manoeuvres (coughing, sneezing,

etc.), in Chiari patients, there is CSF flow through a “non-physiological” route i.e. from the

obex into the central canal, termed as “suck” by Williams. The syrinx is maintained by

longitudinal fluid motions inside the cavity itself, which Williams termed “slosh”.

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du Boulay et al (11) told that there are two successive intracranial CSF pulse waves instead of

one as proposed by Gardner, a ventricular one and a cisternal one. The cisternal CSF pulse

wave propagates into the central canal in Chiari 1 malformation. The cerebellar tonsils

function as a one-way valve, maintaining the syrinx and enlarging it.

The above theories were based on existence of communication between central canal and

fourth ventricle. However, this communication is found only in minority of cases (2,38).

Ball and Dayan (56) rejected the above hypotheses because CSF pulse wave were not of

enough magnitude so as to open central canal and form syrinx. They proposed that during

Valsalva-like manoeuvre there is increased spinal subarachnoidal pressure, due to congestion

of the epidural venous plexus, which forces CSF via perivascular space (Virchow-Robin

space) into the spinal parenchyma, and by accumulation of CSF from these perivascular

entrances to the spinal parenchyma, a syrinx is formed. The syrinx may secondarily rupture

the ependyma, thus dilating the rudimentary central canal.

Oldfield et al (2) also favoured transmedullary CSF infiltration through the perivascular

spaces. He states that instead of Valsalva manoeuvre, it is the regular systolic congestion of

the brain that creates a cranial CSF pulse, which then cause rhythmic piston like motion of

cerebellar tonsils, at the obstructed foramen magnum, creating spinal CSF pulse wave. This

spinal CSF pulse wave in time forces CSF into the spinal parenchyma via Virchow robin

spaces.

However, theories based on transmedullary seepage were contradicted by pressure studies,

showing equal or even higher pressure in syrinx as compared to spinal subarachnoid space

(12,57,58).

Klekamp et al (57) suggested that syrinx formation is because of altered equilibrium

between CSF and the intramedullary extracellular fluid (ECF). Whenever there is CSF flow

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abnormality, an interstitial oedema is created and, depending on local flow resistance and

ECF accumulates into the parenchyma or the central canal. This concept favoured by

presence of presyrinx state seen in many patients.

Levine et al (12) advanced theory based on effect of craniospinal pressure dissociation on

transmural pressure of venous system. The transmural pressure differential, varies with

physical activity. It causes mechanical stress on the vessels, eventually destroying the blood

spinal cord barrier resulting in the leakage of an ultrafiltrate of the blood, eventually forming

a syrinx.

Koyanagi and Houkin (58) et al proposed that there is reduced compliance of posterior spinal

veins due to reduced compliance of subarachnoid space. These vessels lack pial covering

around it. During diastole, there is disruption of Starling’s equilibrium at these vessels and

leading to intramedullary oedema, and finally syrinx formation either in the parenchyma or in

the central canal.

All the above theories partially explain syrinx formation, which indicates that syrinx

formation may be multifactorial (59–62).

Clinical features and Natural History

Clinical manifestations of Chiari 1 malformation vary according to the age at which they first

appear (63–65). Symptoms are often due to compression of the brainstem, and in patients

under 2 years of age, this often manifests with stridor, apnoea, cyanosis, increased muscle

tone and life-threatening respiratory problems. In older children, there may be development

of scoliosis secondary to syringomyelia and an ataxic gait. In both children and adults,

chronic brainstem compression may manifest as occipital headache, nystagmus, neck pain,

neck tilt, dysphagia, diplopia, change in voice etc. Myelopathic symptoms like stiffness of

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limbs, clumsiness of hands, dysesthesia in hand, repeated falls, decreased sensation may also

be present. Yet again, patients with Chiari 1 malformation can be asymptomatic (33,66–69).

The frequency of asymptomatic syringomyelia has been reported as 23 % (54). The natural

history is not uniform, with deterioration in 20–51 %, 10–80 % remain unchanged and11 %

improves (72). Spontaneous reduction of tonsillar herniation in Chiari 1 malformation is

uncommon, but may occur, in 11–18 % of cases (70). Spontaneous resolution of

syringomyelia in adult patients with cerebellar ectopia is rare, although cases have been

reported (73–76). Probable mechanisms include spontaneous drainage between the syrinx

andthe subarachnoid space or restoration of normal CSF dynamics at the craniovertebral

junction(73,74).

Treatment

Foramen magnum decompression

Cornelis Joachimus van Houweninge Graftdijk (24,77) in 1930 first described posterior fossa

decompression, followed by description by Penfield and Coburn in 1938(78). However, they

described it in patients of Arnold Chiari malformation and both of their patients died. In

1938, McConnell and Parker published results for posterior fossa decompression in five

patients of Chiari I malformation, two of whom had successful outcomes (79). In 1950

Gardner and Goodall(80), published their series of 17 patients with Chiari 1 malformation

and syringomyelia managed with posterior fossa decompression. This series resulted in the

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widespread adoption of posterior fossa decompression for Chiari 1 malformation with larger

reports following over the next few decades (81–84).

Variations of foramen magnum decompression has been described, with no consensus on the

best available way of doing the surgery. These variations revolve around, splitting the dura,

opening the dura, opening the arachnoid, adhesiolysis and exploration of fourth ventricle,

plugging of obex, tonsillar resection, and lax duraplasty (80,85–91).

Gardner (92) did foramen magnum decompression with opening of the fourth ventricle and

plugging of the obex. Williams (55) modified this technique by suturing muscle to the obex.

These obex plugging procedures were associated with a high risk of intraoperative

hemodynamic instability along with high failure rate.

Foramen magnum decompression, with opening of fourth ventricle to drain into cisterna

magna was then advocated by Hankinson (93) and later Peerless and Durward (94). Rhoton

(95) added upper cervical laminectomy, and myelotomy to above. Subpial excision of the

tonsils was described by Bertrand (96).

Due to complication of cerebrospinal fluid leak postoperatively, foramen magnum

decompression with opening of dura leaving arachnoid intact was described by Logue and

Edwards (97). Isu, (98) described resection of outer layer of dura along with foramen

magnum decompression. Foramen magnum decompression with a lax duraplasty has gained

popularity after Oldfield proposed his theory of formation of syrinx in Chiari malformation

(2).

In addition, there remains controversy over the concept of cerebellar slumping due to overly

extensive posterior fossa decompression that can lead to further neurologic deterioration (99).

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These variations represent the quest to find best possible way to treat patient avoiding

complication. However, independent of surgical method used, the main goal of these

surgeries is to establish CSF flow across the foramen magnum (100).

Fixation

In 1997, Goel et al published their experience of surgically treated 190 patients of basilar

invagination (101), where they divided basilar invagination into 2 groups- group I, basilar

invagination without syringomyelia and group II, basilar invagination and Chiari

malformation. Goel then in 2009, reclassified basilar invagination into group A, those with

incompetent atlantoaxial joint and group B, those with competent atlantoaxial joint. Group A

patients had associated Chiari malformation and syrinx. He postulated that group A patient

have acquired instability and requires decompression as well as stabilisation of the region,

while group B patients can be managed alone with decompression (16,50,102). In 2014, Goel

A, published his study of 65 patients, where he postulated that the pathogenesis of CM with

or without associated basilar invagination and/or syringomyelia is primarily related to

atlantoaxial instability. He suggested that Chiari malformation should be treated with

atlantoaxial stabilization and segmental arthrodesis, with or without inclusion of occiput (50).

He also cautioned that foramen magnum decompression is not necessary and may be counter-

effective in the long run (50).

Other surgeries

Syringosubarachnoid shunt has been described for refractory syringomyelia (103). However,

there was apprehension the syringosubarachnoid shunt leads to spinal cord injury, in form of

myelotomy and insertion of the catheter, and that shunt dysfunction may occur (98).

However, there is a renewed interest in syringosubarachnoid shunt for patient with refractory

syringomyelia with authors reporting promising results (104–107).

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Outcome studies for Chiari 1 malformation patients

The outcome of surgery is variable with many patients improving post-surgery and some

deteriorating. Various studies have been done studying outcome of the patients after surgery

and making observation about surgical technique (1,18,21,30,50,84,102,108). There are many

scales, both validated and non-validated, for assessing outcome of patient after surgery (109).

However, very few authors have studied predictors which were present before surgery and

which affect the outcome.

Furtado et al (20) studied 20 paediatric patients for clinical, radiological and demographic

factors and radiological changes in cord associated with syringomyelia. They found that age

at presentation, duration and type of symptoms, cranial and foramen magnum morphometry,

and syrinx-related changes have no bearing on outcome at short-term follow-up. He also

found that spinal cord diameter differs significantly in patients with and without functional

improvement (20). However, this study was only limited to paediatric population.

Hekman et al (19) in study about 245 patients found that peripheral nerve symptoms, either

neuropathy (including dysesthesia, paraesthesia, and hyperesthesia) or loss of sensitivity to

pinprick, correlated with a poor outcome after surgery. She also found that patients

presenting with syringomyelia were significantly more likely to achieve a better outcome

score. The degree of tonsillar herniation did not have correlation with outcome. However,

overall effect of factors on patient outcome was not analysed.

Greenberg (71) et al developed the Chiari severity index, to evaluate Chiari 1 malformation

patients considering surgery. He integrated both clinical and neuroimaging features, to

identify patients that are most likely to improve after surgical treatment. However, CSI was

developed using paediatric population. Also, it has not considered bulbar symptoms or basilar

invagination.

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Lacunae in literature.

The main motivating factor for patients is the expected improvement in quality of life

following surgery. Various studies have been done and metrics have been published for

assessing outcome following surgery (50,108,110–112). However, there are not many studies

which stratify the patient preoperatively, so that they can be informed about their expected

outcome. We studied preoperative factors which affect the outcome, to develop a tool to help

stratify patients in accordance with their disease severity.

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 AIMS AND OBJECTIVE

Aims

To study factors affecting the outcome in Chiari malformations patients undergoing surgery

Objectives

In symptomatic patients with Chiari 0/1 malformation:

1. CSF flow dynamics across the foramen magnum in patients with chiari malformation

before and after the foramen magnum decompression surgery.

2. Correlating the above finding with the pre and post operative clinical status.

MATERIALS AND METHODS:

Study design

Hospital based prospective cohort study of various clinical and radiological and CSF flow

findings affecting outcome in patient with Chiari malformation undergoing treatment.

Study population

Patients between age group 18 to 70 years, having Chiari malformations who will present

to Department of Neurosurgery, AIIMS, Saket Nagar, Bhopal, between July 2019 and Jan

2021 for evaluation and treatment and were willing to participate in study.

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 Inclusion criteria:-

1. Age between 18 years to 70 years

2. Symptomatic patients with Chiari 1 malformation with or without syringomyelia

3. Patients with Chiari 0 malformation and syringomyelia.

4. chiari malformation 2 patient without brainstem decent.

Exclusion Criteria:-

1. Chiari 2, 3 or 4 malformations

2. Patients with tonsillar descent having no symptoms and syringomyelia.

3. Syringomyelia related with tumour

4. Post traumatic syringomyelia

5. Syringomyelia associated with spinal dysraphism

6. Skull base anomaly

Sample collection-The sampling methodology employed in current study is universal

sampling. All consenting adults fulfilling the inclusion and exclusion criteria undergoing

surgery for Chiari malformation are considered as study participants. I tried to take a

sample size of minimum 30 patients in study duration.

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Methodology-

I collected all of the prospective clinical data, including pre- and post-operative symptoms,
determine the functional grades and the change in clinical improvement based on clinical
examination notes, calculate the absolute and relative differences in posterior fossa area (PFA,
mm2), cisterna magna area (CMA, mm2), and tonsillar herniation, calculate the change in functional
grade, and analyzed the radiologic data according to the degree of clinical improvement.

Neurologic assessment
Symptoms and clinical exams obtained from a clinical examination of surgeon. Functional grade is
determined using a grading scale previously reported in the literature48.
( Functional grading system from Noudel et al., 2011).(tab1)

Patients received an initial post-operative follow-up visit in the clinic between 5-40 days (mean =
16.27), with continual follow-up at several month intervals thereafter. Clinical improvement was
determined by a clinician who only assessed clinical symptoms and blinded to the radiologic results.
Patients were determined to have improved symptoms if it was clearly documented in any post-
operative clinic notes. Patients were categorized as having no, partial, or complete resolution of their
symptoms based on their last documented visit.

Radiologic studies

MRI Protocol
A useful MRI protocol for preoperative and postoperative evaluation of Chiari I malformation will
include axial and sagittal T1- and T2-weighted fast spin-echo sequences, sagittal cardiacgated phase
contrast cinemode images, sagittal cardiac-gated cine true fast imaging with steady-state precession
(true FISP), and sagittal high-spatial-resolution cisternographysequences. In addition,DW images and
FLAIR will be useful for suspected acute infarcts, and contrast-enhancedT1-weighted sequences will
be included if infection is suspected. The midsagittalcraniocervicaljunction region phase contrast
cine-mode images will provide quasi– real-time dynamic assessment of the CSF flow characteristics.
The sequence will derive signal contrast between flowing and stationary nuclei by sensitizing the
phase of the transverse magnetization to the velocity of motion and enables measurement of flow
velocity and flow pulsation magnitude or simply a qualitative assessment of CSF flow. Heavily T2-
weighted MR cisternography– type sequences—such as 3D driven equilibrium (DRIVE), fast imaging
employing steadystate acquisition (FIESTA), or constructive interference in steady state (CISS)—will
providen exquisite delineation of parenchyma- CSF interfaces, thereby providing detailed assessment
of cerebellar morphology
The diagnosis of Chiari I malformation will be made using sagittal T1-weighted MRI brain studies, and
will be defined as tonsillar herniation of at least 5 mm below the level of the foramen magnum. The
change in tonsillar herniation will be calculated as the post-operative tonsillar herniation minus the
pre-operative value. PFA and CMA will be measured using T2-weighted imaging at the mid-sagittal
plane. The method for determining the PFA and CMA will based on previously reported methods of
measuring posterior fossa and cisterna magna volumes; the contents of the posterior fossa will be
measured as the neural structures along with the surrounding subarachnoid space, while excluding
the thickness of the skull. The CMA will be measured as the height of the cisterna magna multiplied
24
by the depth of the cisterna magna perpendicular to the occipital dura divided by two (which
assumes that the area of the cisterna magna is approximately a triangle). The increase in PFA will be
calculated as the post-operative PFA subtracted by the pre-operative PFA, whereas the relative PFA
increase will be calculated as the PFA increase divided by the preoperative
PFA. The relative CMA increase will be calculated as CMA increase divided by the pre-operative
CMA. Narrowing of the CSF spaces anterior and posterior to the spinal cord will be assessed on
T2-weighted imaging. Post-operative improvement in CSF flow will be demonstrated by
comparing T2-signal on pre- and post-operative images and by Looking for differences in
signaling in the posterior fossa and around the spinal cord.
Phase-contrast cine flow imaging will be compared pre- and post-operatively, when Available, and
assessed on all patients post-operatively to confirm adequate CSF flow.
Post-operative MRI with cine will be obtained approximately three months after surgery.

Surgical Technique
All patients undergoing surgical Chiari I decompression will be in the prone position. A standard
suboccipitalcraniectomy will be performed in order to ensure a wide decompression of the cerebellar
hemispheres, brainstem, and midline structures. For all patients, a C1 laminectomy will also
performed to decompress the cervical spinal cord. The atlanto-occipital ligament will be divided and
the underlying outer dura leaflet will be incised and reflected radially, further procedure will be
addressed for pial coagulation/shrinkage. The patients will be then closed in a standard multi-layer
fashion, similar to intradural procedures to guard against any potential CSF leak not noted
intraoperative.

The goals of surgery are to –

1) Decompress the brainstem
2) Restore pulsatile flow of CSF at the cervicomedullary junction.

Decompression of the brainstem is performed via the removal of the sub occipital bone and the
dorsal arch of C1 and/or C2. After bony removal, the intradural contents are explored. Any adhesions
observed are lysed, thus restoring the egress of CSF from the fourth ventricle and across the
cervicomedullary junction. The placement of a patulous dural graft also aids in the flow of CSF across
the junction.54 Surgical steps for foramen magnum decompression: Position: Patients are intubated
in the supine position. An arterial line and indwelling Foley catheter are placed. Antibiotics with a
spectrum against common skin flora are given at least 30 minutes prior to skin incision. After
intubation, a 3-point Mayfield head-holder is placed. The patient is then turned into the prone
position onto blanket rolls (37). The head is fixed in a slightly flexed posture. This facilitates exposure
and decompression. Flexion is avoided in the presence of associated bony CV junction anomalies like
basilar invagination. The head of the table is kept above heart level. All pressure points are carefully
padded. With the arms at the patient‘s side, the shoulders may be taped back with care taken to
avoid injury to the brachial plexus Figure. 655 Exposure: The incision is begun immediately below the
inion and is carried to and past the spinous process of C2. The incision should not be carried over the
inion because pressure over this bony prominence when the patient is supine may increase the
incidence of wound dehiscence. After the skin is incised, Bovie cautery is used to carry the incision

25
through the subcutaneous fat to the fascia. The muscles are divided in the midline fascial sulcus. Care
should be taken not to Bovie into the muscle itself. This may lead to muscle shrinkage and increased
postoperative pain. The dissection is carried down to the sub occipital bone and over the dorsal
tubercle of C1 and the C2 spinous process. The muscles are first stripped laterally off the sub occipital
bone. This may be done with a periostial elevator or Bovie cautery. A ―V‖ of muscle insertion should
be left in the midline immediately below the inion. The muscle dissection continues until about 3cm
of bone is exposed on each side of midline. The foramen magnum is fully identified. Care should be
made when using the Bovie near this region; the authors prefer to use an angled curette to clear any
muscle off the edge of the foramen magnum. Angled cerebellar retractors are used to maintain the
exposure. Attention should then be placed on the C1 lamina. The Bovie may be used for the56 most
medial dissection, but sharp dissection should be used for the lateral aspect. This decreases the risk
of vascular injury (eg, vertebral artery). The vertebral venous plexus may be encountered during this
part of the operation. Bleeding may be profuse. If a definite vessel is visualized it may be contr olled
with bipolar cautery, but the bleeding is usually controlled with a piece of thrombin-soaked Gelfoam
and gentle pressure. If the cerebellar ectopia is above the C2 lamina, the authors do not strip the
muscles off C2, but if the preoperative MRI demonstrates herniation below C2, the spinous process
and lamina are exposed for laminectomy or partial laminectomy. A second cerebellar retractor is
used to maintain the spinal exposure.
Figure. 757

Bone Removal: A craniectomy should be planned about 2cm above the foramen magnum in children
and 3cm in adults. Too generous of a craniectomy will encourage cerebellar sagging or ptosis. The
appropriate extent of bone removal assessed by the sagittal MRI and removing enough occipital bone
to decompress the tonsils. The craniectomy is performed with a high-speed drill and cutting burr. The
drill is used to thin the bone first over the cerebellar hemispheres. Next, the thick bony keel should
be thinned. This bone is often vascular and bleeding is easily controlled with bone wax. The drilling
should proceed rostral to caudal. Drilling near the foramen magnum is performed last. Once the bone
is adequately thinned, a curette is used to expose the dura mater over one of the cerebellar
hemispheres. Leksell or large Kerrison ronguers are then used to remove the remaining bone, thus
fully exposing the dura over the cerebellar hemispheres and the cervicomedullary junction. The
decompression should be performed until just medial to the occipital condyles and the ring of the
foramen magnum begins to turn ventrally. Venous bleeding may be encountered during this
widening, but may be easily controlled with thrombin-soaked Gelfoam. A C1 laminectomy is
performed next, either with the aid of the drill or the Leksell ronguer. 58 The laminectomy should
only be as wide as the underlying dura mater. An excessively wide decompression places the
vertebral artery at risk. If the tonsillar herniation is below the C1 lamina, partial C2 laminectomy will
also be required. Dural Opening: There is often a tight ring of redundant tissue at the
cervicomedullary junction. This should be incised and reflected laterally before the dural Figure. 859
opening. The dura mater is opened in a ―Y‖ fashion with a 15 scalpel blade, beginning in the midline
over the cervical spinal cord. Small scalpel strokes are made until the arachnoid is encountered but
not violated. A dural guide is then placed subdurally, but above the arachnoid, and the dura is
opened with the blade cutting immediately upon the advancing dural guide. As the cerebellar dura is
approached at the cervicomedullary junction, bleeding from the circular or occipital sinuses may be
encountered. If bleeding is encountered, it should be controlled with bipolar cautery or via the
placement of small metal clips. The final flap of dura is sutured rostral to fully expose the cerebellum,
26
lower brain stem, and upper cervical spinal cord. The arachnoid should be intact. Figure. 960
Intradural Exploration: Some surgeons do not open the arachnoid and proceed immediately to the
placement of the dural graft (65). Some examine the flow of CSF and movement of the cerebellar
tonsils with ultrasound and do not open the dura mater or arachnoid if adequate movement and flow
of CSF is established after the craniectomy (50). Jorg Klekamp et al, (25) intraoperative findings, the
arachnoid pathology was graded according to the following criteria : Grade 0 = no arachnoid
pathology detectable Grade 1 = slight arachnoid adhesions to cerebellum or spinal cord, arachnoid
translucent Figure. 1061 Grade 2 = severe arachnoid scarring, arachnoid not translucent, dense
adhesions to cerebellum, brainstem, or spinal cord. If the arachnoid is to be opened, it is incised
sharply and is attached to the dura mater with suture or metalclips. Using loupe magnification or the
operating microscope, the tonsils and the foramen of Magendie are examined. A number of points
need to be stressed in this respect. Keeping the subarachnoid space clear from any contamination
with blood is mandatory to limit postoperative arachnoid scar formation. Arachnoid dissection should
be restricted to the midline, avoiding cranial nerves and perforating vessels using sharp dissection
only to avoid any tearing on these structures (25). Often the tonsils are adherent to each other or the
brainstem by thickened arachnoid. A Penfield 4 is used to separate the 2 tonsils from each other and
identify PICA. The goal is to separate the tonsils to permit the egress of CSF from the fourth ventricle.
Care should be taken to not damage PICA or try to dissect adherent tonsils from the brainstem. If the
tonsils are not easily separated, bipolar cautery may be used to shrink the arachnoid and hence the
tonsils, which should permit the flow of CSF from the fourth ventricle. If the scarring is too dense, a
more aggressive strategy is used.62 The tonsils may be removed via an endopial resection. PICA
should be unequivocally identified prior to the subpial resection. Dural Grafting: Multiple choices of
grafting materials are available. Autologous choices include pericranium, ligamentum nuchae, and
fascia lata. Nonautologous options include cadaveric dura, lypholized dura or fascia, bovine
pericardium, human pericardium, and Gor-Tex. Autologous graft material is preferred whenever
possible. The wound should be copiously irrigated prior to the placement of the final sutures, to
ensure the removal of blood products and verify that there is not any active bleeding. The
anesthesiologist should perform a Valsalva maneuver to check the integrity of the suture line. If any
sites of leaking are visualized, simple sutures are placed. A small piece of muscle may also be sutured
at the site of a leak. Figure. 1163 Muscle and Skin Closure: The muscle should be closed in layers with
absorbable suture. The fascia is closed and reattached to the muscle and fascia along the ligamentum
nuchae. The midline is attached to the cuff of muscle that was spared during the initial exposure. The
dermis is closed and the skin closed with sutures or staples. POSTOPERATIVE CARE: All patients are
closely observed for 24 hours postoperatively. The patients should have full cardiovascular and
respiratory monitoring and close observation for neurologic changes. On postoperative day 1 patients
are fully mobilized. Their diet is advanced, but any signs of aspiration should be carefully sought. A
soft collar may be prescribed for comfort only. Patients return to clinic at 6 weeks and 3 months with
an MRI of the craniovertebral junction and cine-MRI flow study. This is to ensure adequate flow of
CSF at the cervicomedullary junction and a decrease in the size of any syrinx that was present
preoperatively. It serves as a baseline study for future care.64 POSTOPERATIVE COMPLICATIONS Early
Complications:  CSF leak and /or pseudomeningocele  Meningitis o Infective o Chemical 
Hematoma Late Complications:  New or enlarging syrinx  Obstruction of CSF flow across the
cervicomedullary junction due to scarring  Cerebellar ptosis. Early Complications By far the most
common early complication is CSF leak and/or pseudomeningocele. Meticulous surgical technique
should minimize the incidence of this complication. CSF leak should first be treated by over sewing
27
the wound. If this fails, placement of a lumbar subarachnoid drain is appropriate. If the lumbar drain
fails, re-exploration may be necessary. 65 Meningitis has been reported to occur following Chiari
decompression. Contamination usually occurs at the time of surgery, and may be minimized by
attention to detail (such as - preoperative antibiotics, careful skin preparation, etc.). If non-
autologous graft material was used, removal of the graft may be required if the infection cannot be
cleared with antibiotics alone. Chemical meningitis also frequently occurs following this procedure. It
may be due to blood in the CSF or immune reaction to nonautologous graft material. Diagnosis is
made by spinal tap with negative cultures. Often eosinophils are present in the CSF. Patients may
have temporary or permanent lower cranial nerve paresis. This may lead to aspiration or minor
problems with respiration. These complications may be minimized by not trying to aggressively
dissect the cerebellar tonsils off the brain stem. If symptoms are severe, tracheotomy and/or
gastrostomy tubes may be required. The most feared complication is hematoma. Clinical
deterioration may be rapid and is the main reason for close neurologic observation postoperatively. If
clinical deterioration is observed following surgery, the patient should be taken for emergent CT scan
and then to the OR for hematoma evacuation. If rapid deterioration occurs, emergent 66
decompression at the bedside (with closure in the operating room) may rarely be required. Late
Complications Clinical deterioration may occur after initial improvement following surgery. Risk
Factors of a Neurological Recurrence (25)  Arachnoid pathology  Less experienced surgeon 
Arachnoid not opened  Basilar invagination An MRI of the brain and cervical spinal cord and a cine-
MRI flow study are indicated under these circumstances. Common etiologies include new or
enlarging syrinx, obstruction of CSF flow across the cervicomedullary junction due to scarring, and
cerebellar ptosis.
Statistical analysis
A univariate analysis will be used to compare groups, and p < 0.05 will be considered significant.
Microsoft Excel 2011 will be used to organize the data in a de-identified encrypted file. Statistical
analyses will be performed using a TI-87 graphing calculator.We will use Microsoft Excel for data
entry and EPI-info 7 for data analysis. Descriptive statistics measures that is mean and standard
deviation will be used to summarize numerical data and count and percentage for summarizing
nominal data. Study period will be of 18 months. All patients meeting the inclusion criteria will be
enrolled for the study. Comparison of categorical data will be done using unpaired t-test. The
continuous data will be compared by student test/ Mann whitney test as applicable. Further, p<0.05
will be considered significant.

Ethical Issues

Ethical clearance will be taken from ‘AIIMS Ethics Committee’ for conducting this study. New patient
presenting to outpatient department or in ward who fulfilled the inclusion criteria will be directly
contacted by researcher and explained in detail about the study, then invited to take part in study
after duly creating their doubts. The standard of care will be remained same for patients irrespective
of their willingness or not to take part in study. Confidentiality will be strictly observed in collecting
data of patients. The final surgical approach for a patient will be decided after their thorough
inpatient evaluation, pre-operative discussion among the team members, keeping in mind the best
interest and preference of patients by team of senior consultant neurosurgeons.

28
STUDY PROFORMA

29
 DATA COLLECTION

PATIENT PRESENTING TO PATIENT WITH SYRINX WHO

OPD HAVING SYRINX UNDERWEN SURGERY

PREOPERATIVE

IMAGING FINDING CLINICAL FINDINGS

Intra operative findings

POST- OPERATIVE

IMAGING FINDING CLINICAL FINDINGS

Follow up

30
Tools - Clinical outcome

Chicago Chiari Outcome Score (CCOS), originally provided by Aliaga et al (111), is a score

for post-operative outcome assessment in Chiari patients.

1 2 3 4

Characteristic/score

Pain Worse Unchanged & Improved or Resolved/ no

refractory to controlled new symptom

medications with

medications

Non-pain symptoms Worse Unchanged or Improved and Resolved/ no

improved but unimpaired new symptom

impaired

Functional status Unable to Moderate Mild Fully functional

attend work or impairment impairment

school (<50% (>50%

attendance) attendance)

Complications Persistent Persistent Transient Uncomplicated

complication, complication, complication course

poorly well controlled

controlled

Table 2 Chicago Chiari Outcome Score (CCOS)

31
However, some components of CCOS scores have poor inter-rater reliability, particularly

functional status sub-score of CCOS (112). Also, CCOS does not consider headache and

involvement of lower cranial nerve in scoring. Given the above limitations we have modified

CCOS, dividing pain and non-pain symptoms- into sensory symptoms, motor symptoms and

headache -and used in the study. We have also included involvement of lower cranial nerve

in CCOS.

This modified CCOS was used to assess patients postoperatively during out-patient follow up

in prospective patients and at the time of telephonic contact for retrospective patients.

Characteristic/score 1 2 3 4

Sensory symptom Worse Unchanged Improved or Resolved/ no

& refractory controlled new symptom

to with

medications medications

Motor symptoms Worse Unchanged Improved Resolved/ no

or improved and new symptom

but impaired unimpaired

Lower cranial Worse Unchanged Improved Resolved/ no

nerves or improved and new symptom

but impaired unimpaired

Functional state Unable to do Able to do Able to do Able to do

routine routine routine routine activity

activity activity and activity and and >50%

32
 not able to <50% office/school

attend school office/school attendance

or office attendance

Headache Worse Unchanged Improved or Resolved/ no

& refractory controlled new symptom

to meds with meds

Complications Persistent Persistent Transient Uncomplicated

complication, complication, complication course

poorly well

controlled controlled

Based on modified CCOS, patients were divided into 3 groups-

6-14 Poor Outcome

15-20 Impaired Outcome

21-24 Good Outcome

Table 3 Modified Chicago Chiari Outcome Score (mCCOS) [modified from Aliaga et al.
(111)]

All the patients were interviewed for their functional status, changes in sensory symptoms,

motor symptoms, lower cranial nerves symptoms and headache. Surgical complications, if

any, were enquired and noted.

To evaluate functional status, both preoperatively and postoperatively, functional status

questionnaire was used (113). It was modified, to assess the ability of person in three spheres

- basic daily activity, intermediate daily activity and social/role function.

33
Functional Status Questionnaire

Physical Function (Activities of Daily Living, or ADL)

Basic ADL: During the past month have you had difficulty with

(1) Taking care of yourself, that is, eating, dressing or bathing?

(2) Moving in or out of a bed or chair?

(3) Walking indoors, such as around your home?

Response Points

usually did with no difficulty = 4

some difficulty = 3

much difficulty = 2

usually did not do because of health = 1

Those people who were able to get score of 9 and above were then asked next set of

questions.

If they got less than 9, then they were grouped into functional status 1

These patients were generally unable to do routine activity.

Intermediate ADL: During the past month have you had difficulty with

(1) Walking several blocks?

(2) Walking one block or climbing one flight of stairs?

(3) Doing work around the house, such as cleaning, light yard work or home maintenance?

(4) Doing errands such as grocery shopping?

(5) Driving a car or using public transportation?

Response Points

usually did with no difficulty = 4

some difficulty = 3

34
much difficulty = 2

usually did not do because of health = 1

Those people who were able to get score of 13 and above were then asked next set of

questions.

If they got less than 13, then they were grouped into functional status 2 .

These patients were generally, able to do routine activity but unable to do attend work or

office.

Social/Role Function

If you were employed/going school during the past month, how was your work

performance?

(1) Done as much work as others in similar jobs/ other students?

(2) Worked/studied for short periods of time or taken frequent rests because of your health?

(3) Worked/studied your regular number of hours?

(4) Done your job/study as carefully and accurately as others with similar jobs?

(5) Worked/studied at your usual job/period, but with some changes because of your

health?

(6) Feared losing your job/school because of your health?

Response to 2, 5 and 6 Points

all of the time = 1

most of the time = 2

some of the time = 3

none of the time = 4

Response to 1, 3 and 4 Points

all of the time = 4

most of the time = 3

35
 some of the time = 2

none of the time = 1

Those patients who were able to get points more than 20 were grouped as functional status

4 . These patients were generally able to do routine activity and >50% of their office/school

work.

Those patients who were able to get points less than 20 were grouped as functional status

3. These patients were generally able to do routine activity but <50% of their office/school

work

Table 4 Functional status questionnaire

Patients were asked about the presence of sensory, motor, lower cranial nerve symptoms

(from the list of symptoms) and headache preoperatively and interviewed on whether their

sensory, motor and lower cranial nerve symptoms are same, improved with partial resolution,

totally resolved or has worsened. To decrease recall bias in retrospective cases, only those

cases were taken whose response were in accordance of their medical records

Sensory symptoms Motor Symptoms Lower cranial symptoms Headache

Paraesthesia Weakness of limbs Difficulty in swallowing

Dysesthesia Stiffness of body Double vision

Ataxia Difficulty while Facial pain/numbness

Walking

Blisters Clumsiness of hands Tinnitus

Swaying while Tippling over small Dysarthria

walking objects

36
 Unable to stand Buckling of knee Stridor

straight

Shortness of breath Difficulty in Regurgitation

lifting/holding object

Neck pain Bed ridden

Back pain

Urinary retention /

incontinence

Table 5 List of sensory, motor and lower cranial nerve symptoms in Chiari malformations

pateints

Headache presence was asked separately and then evaluated for whether it has worsened,

resolved, controlled with medications or not controlled with medications.

Patients were asked from the list of complications and were divided into 4 groups based on

their responses - Persistent complication poorly controlled, Persistent complication well

controlled, Transient complication, Uncomplicated course.

List of complications

Wound site CSF leak

Difficulty in breathing

Tracheostomy

Dysphagia, requiring Ryle’s tube feed.

Increased weakness – paraplegia, quadriplegia

Any other complications

Table 6 List of complications

37
The responses of functional status, sensory symptoms, motor symptoms, lower cranial nerve

symptoms, headache and complications were then transferred to modified CCOS and scores

were calculated accordingly.

Tools- Radiological Outcome

Patient’s Radiological imaging were examined, and data were collected about the following

preoperatively.

Radiological Factors Response

Presence of tonsillar descent Yes/no

Descend of tonsil below McRae’s line

Extent of tonsillar descent No herniation, above C1, till C1, Below C1

Presence of Syringomyelia Yes/no

Syringomyelia starts at vertebral level, SS CVJ to L1/L2

Syringomyelia ends at level, SE CVJ to L1/L2

Extent of syringomyelia Difference between vertebral level SS and

SE+ 1

Type of extent Cervical, cervicodorsal, dorsal, holocord

Shape of syrinx Tubular (ratio of maximum : minimum

diameter ≤1.1)- yes/no

Fusiform (ratio of maximum : minimum

diameter >1.1)- yes/no

Basilar invagination BI Wackenheim’s line was used

Present/absent

Platybasia Clivus canal angle <150⁰ = platybasia

Present/absent

38
 Occipitoatlantal assimilation Present/absent

AAD Atlantodental interval

AAD is present if ADI >3mm

Response:

Present/absent

Hydrocephalus Yes/ no

Scoliosis Yes/ no

Table 7 Preoperative Radiological assessment

Post-operative imaging and reports done after 3 months were collected and analysed.

Postoperatively Response

Syrinx maximum diameter Stable, resolving, worsened

CSF flow at foramen magnum Yes/ No

Table 8 Radiological factors assessment chart

Radiological outcome was defined as

Worse- if syrinx increased in maximum diameter
Stable- if maximum diameter remained the same
Improved - if maximum diameter decreased in size
The data collected were transferred to IBM SPSS statistics software ver. 25.0.

The data were summarized in the form of mean and standard deviation for numerical data, and

frequencies and percentages for categorical data. Presentation of summarized data was done

39
using tables and figures. Non-Parametric tests used for the inferential statistics of those

variables which failed to achieve normal distribution. Mann Whitney U test was used for

comparison of the mean values between two groups. Kruskal Walli’s test was used for

comparison of the mean values between three or more groups. Spearman’s rank correlation

was used to assess correlation between two factors. The distribution of the categorical variables

among the different age groups and gender was compared using Chi-square test. Following

analysis confidence level and p-value were checked to find out statistical significance of data.

Chiari Outcome Predictability Index (COPI)

Based on the above data obtained from surgical patients, we devised a Chiari Outcome

Predictability Index (COPI), giving weightage points to various clinical and radiological

factors, having correlation with outcome and syrinx improvement.

Index

Factors -2 -1 0 1 2

Presence of Sensory Symptoms -1

Presence of Motor symptoms -1

Lower cranial nerve symptoms -2

Duration of first symptoms (<9months) 1

On presentation functional statusa -2 0 1 2

Tonsillar descent -1

Syringomyelia Absent 1

Syrinx diameter less than 6 mm 1

Basilar Invagination -1

a. Unable to do routine activity points = -2 ,

Able to do routine activity and not able to attend school or office points = 0,

40
 Able to do routine activity and <50% office/school attendance points = 1,

Able to do routine activity and >50% office/school attendance points = 2

Table 9 Chiari Outcome Predictability Index (COPI)

Those patients based on the score has been divided into three groups –

Score Expected outcome

Less than or equal to -2 Less likely good outcome

-1 to 0 More likely good outcome

>0 Most likely good outcome

Table 10 Groups subdivided into Chiari Outcome Predictability Index (COPI)

41
data of patients. The final surgical approach for a patient is decided after their thorough

inpatient evaluation, pre-operative discussion among the team members, keeping in mind the

best interest and preference of patients by team of senior consultant neurosurgeons.

42
RESULTS

A total of 72 patients fulfilling selection criteria were selected for the study. Out of 72, 6

patients did not go surgery. The number of operated patient studied during the period of study

was 66, which correlated well with calculated sample size. The median duration of follow up

was 2 years.

Demographic data

GENDER

Figure 3 Gender Distribution

AGE

43
44
DISCUSSION

Patients who present to hospital with Chiari malformations are often in dilemma about their

quality of life after surgery. This gets further accentuated when they are explained about the

pathology and unclear natural history of the disease, along with various surgical options. This

apprehension is more evident in patients with concomitant syringomyelia. During this study ,

by studying various preoperative clinical and radiological factors, we have developed a

clinical tool which can help to predict the outcome of patient.

Age

Chiari malformations are disease which affects all age groups from child to old. The

minimum age of patient in our study was 25 years and the maximum age was 65 years.

Overall occurrence of Chiari follows a unimodal distribution with age , with most 75

percentiles of patients presenting before age of 40. This age distribution is similar to that

observed by Arnautovic et al (1).

The reported incidence of syringomyelia with Chairi malformation ranges from 32% to 100%

45
46
SHORTCOMINGS AND FUTURE DIRECTIONS

The median clinical follow-up in our study was close to 2 years. Our results are, therefore,

only applicable to short term outcomes of these patients. We reviewed cases spanning a 10 -

year period and included all patients with Chiari malformations, whom we could contact, and

have access to their radiological imaging and/or report. Inclusion of retrospective patient data

along with prospective cohort is a limitation of our study.

Further prospective studies with larger sample size will ascertain the usefulness of copi

This study does not predict the type of surgery which may be appropriate for the patient.

Factors which can help in deciding the type of surgical intervention required for the patients,

can be further studied.

47
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63
 ANNEXURES

PROFORMA

Tools –

Table 1. Functional grading system from Noudel et al., 2011.

Grade Clinical examination Functional Assessment
0 Normal Asymptomatic or complete recovery
I Normal Slight impairment
II Objective signs Slight impairment
III Objective signs Personal &socioprofessional
Disturbances
IV Disabled Persistence of autonomy
V Disabled Loss of autonomy
Clinical outcome
Chicago Chiari Outcome Score (CCOS), originally provided by Aliaga et al

(111), is a score for post-operative outcome assessment in Chiari patients.

1 2 3 4
Characteristic/score

Pain Worse Unchanged & Improved or Resolved/ no

controlled with newsymptom
refractory to medications
medications

Non-pain symptoms Worse Unchanged or Improved and Resolved/ no new

improved but unimpaired symptom
impaired

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 Functional status Unable to attend Moderate Mild impairment Fully functional
work or school
impairment( (>50% attendance)

<50%
attendance)

Complications Persistent Persistent Transient Uncomplicated course

complication, complication, complication
poorly controlled well controlled

Table 2 Chicago Chiari Outcome Score (CCOS)

This modified CCOS will be used to assess patients postoperatively during

outpatient follow up in prospective patients and at the time of telephonic

contact for study patients.

Characteristic/score 1 2 3 4

Sensory symptom Worse Unchanged & Improved or Resolved/ no new

controlled with symptom
refractory to medications
medications

Motor symptoms Worse Unchanged or Improved and Resolved/ no new

improved but unimpaired symptom
impaired

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Lower cranial nerves Worse Unchanged or Improved and Resolved/ no new
improved but unimpaired symptom
impaired

Functional state Unable to do Able to do routine Able to do routine Able to do routine

routine activity activity and activity and activity and >50%

66
 not able to <50% office/school
attend school attendance
or office office/school
attendance

Headache Worse Unchanged & Improved or Resolved/ no new

refractory to controlled with symptom
meds meds

Complications Persistent Persistent Transient Uncomplicated

complication, complication, complication course
poorly well controlled
controlled

Based on modified CCOS, patients were divided into 3 groups-

6-14 PoorOutcome

15-20 Impaired Outcome

21-24 Good Outcome

Table 3 Modified Chicago Chiari Outcome Score (mCCOS) [modified from Aliaga et al. (111)]

All the patients will be interviewed for their functional status, changes in sensory symptoms,

motor symptoms, lower cranial nerves symptoms and headache. Surgical complications, if any,

will be enquired and noted.

To evaluate functional status, both preoperatively and postoperatively, functional status questionnaire

will used (113). It was modified, to assess the ability of person in three spheres

67
- basic daily activity, intermediate daily activity and social/role function.

Functional Status Questionnaire

Physical Function (Activities of Daily Living, or ADL)

Basic ADL: During the past month have you had difficulty with

(1) Taking care of yourself, that is, eating, dressing orbathing?

(2) Moving in or out of a bed orchair?

(3) Walking indoors, such as around yourhome?

Response Points

usually did with no difficulty =4 some

difficulty =3

much difficulty =2

usually did not do because of health = 1

Those people who were able to get score of 9 and above were then asked next set of

questions.

If they got less than 9, then they were grouped into functional status 1

These patients were generally unable to do routine activity.

Intermediate ADL: During the past month have you had difficulty with

(1) Walking severalblocks?

(2) Walking one block or climbing one flight ofstairs?

(3) Doing work around the house, such as cleaning, light yard work or homemaintenance?

68
 (4) Doing errands such as groceryshopping?

(5) Driving a car or using publictransportation?

Response Points usually did with no

difficulty = 4 some difficulty = 3

much difficulty = 2

usually did not do because of health = 1

Those people who were able to get score of 13 and above were then asked next set of

questions.

If they got less than 13, then they were grouped into functional status 2 .

These patients were generally, able to do routine activity but unable to do attend work or office.

69
 Social/Role Function

If you were employed/going school during the past month, how was your work

performance?

(1) Done as much work as others in similar jobs/ otherstudents?

(2) Worked/studied for short periods of time or taken frequent rests because of
yourhealth?

(3) Worked/studied your regular number of hours?

(4) Done your job/study as carefully and accurately as others with similarjobs?

(5) Worked/studied at your usual job/period, but with some changes because ofyour

health?

(6) Feared losing your job/school because of yourhealth?

Response to 2, 5 and 6 Points all of the time = 1 most of the time = 2 some of the time = 3 none of the time = 4

Response to 1, 3 and 4 Points all of the time = 4 most of the time = 3

70
 some of the time = 2

none of the time = 1

Those patients who were able to get points more than 20 were grouped as functional status

. These patients were generally able to do routine activity and >50% of their office/school work.

Those patients who were able to get points less than 20 were grouped as functional status

3. These patients were generally able to do routine activity but <50% of their office/school work

Table 4 Functional status questionnaire

Patients will be asked about the presence of sensory, motor, lower cranial nerve symptoms

(from the list of symptoms) and headache preoperatively and interviewed on whether their

sensory, motor and lower cranial nerve symptoms are same, improved with partial resolution,

totally resolved or has worsened. To decrease recall bias in retrospective cases, only those cases

will be taken whose response were in accordance of their medical records

Sensory symptoms Motor Symptoms Lower cranial symptoms Headache

Paraesthesia Weakness of limbs Difficulty in swallowing

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 Dysesthesia Stiffness of body Double vision

Ataxia Difficulty while Facial pain/numbness

Walking

Blisters Clumsiness of hands Tinnitus

Swaying while Tippling over small Dysarthria

walking Objects
Unable to stand Buckling of knee Stridor

straight
Shortness of breath Difficulty in Regurgitation

lifting/holding object
Neck pain Bed ridden
Back pain

Urinary retention /

incontinence

Table 5 List of sensory, motor and lower cranial nerve symptoms in Chiari malformations

pateints

Headache presence will be asked separately and then evaluated for whether it has

worsened, resolved, controlled with medications or not controlled with medications.

Patients will be asked from the list of complications and will be divided into 4 groups based on

their responses - Persistent complication poorly controlled, Persistent complication well

controlled, Transient complication, Uncomplicated course.

72
 List of complications

Wound site CSF leak

Difficulty in breathing Tracheostomy

Dysphagia, requiring Ryle’s tube feed.

Increased weakness – paraplegia, quadriplegia

Any other complications

Table 6 List of complications the responses of functional status, sensory symptoms, motor symptoms, lower
cranial nerve symptoms, headache and complications will be then transferred to modify CCOS and scores will
be calculated accordingly.

Tools- Radiological Outcome

Patient’s Radiological imaging will be examined, and data will be collected about the following preoperatively.

Radiological Factors Response

Presence of tonsillar descent Yes/no

Descend of tonsil below McRae’s line

Extent of tonsillar descent No herniation, above C1, till C1, Below C1

Presence of Syringomyelia Yes/no

Syringomyelia starts at vertebral level, SS CVJ to L1/L2

Syringomyelia ends at level, SE CVJ to L1/L2

Extent of syringomyelia Difference between vertebral level SSand

SE+ 1

Type of extent Cervical, cervicodorsal, dorsal, holocord

73
Occipitoatlantal assimilation Present/absent

AAD Atlantodental interval If

age ≤ 8 years

AAD is present if ADI >5mm If

age >8 years

AAD is present if ADI >3mm

Response:

Present/absent

Hydrocephalus Yes/ no

Scoliosis Yes/ no

Shape of syrinx Tubular (ratio of maximum : minimum

diameter ≤1.1)- yes/no

Fusiform (ratio of maximum : minimum

diameter >1.1)- yes/no

Basilar invagination BI Wackenheim’s line was used

Present/absent

Platybasia Clivus canal angle <150⁰ = platybasia

Present/absent

74
Table 7 Preoperative Radiological assessment

Post-operative imaging and reports done after 3 months will be collected and analysed.

Postoperatively Response

Syrinx maximum diameter Stable, resolving, worsened

CSF flow at foramen magnum Yes/ No

Table 8 Radiological factors assessment chart

Radiological outcome was defined as Worse- if

syrinx increased in maximum diameter Stable- if

maximum diameter remained the same

Improved - if maximum diameter

decreased in size

The data collected will be transferred to IBM SPSS statistics software ver. 25.0.

The data will be summarized in the form of mean and standard deviation for numerical

data, and frequencies and percentages for categorical data.

Presentation of summarized data will be done using tables and figures. Non-Parametric tests will be
used for the inferential statistics of those variables which failed to achieve normal distribution. Mann
Whitney U test will be used for comparison of the mean values between two groups. KruskalWalli’s
test will be used for comparison of the mean values between three or more groups. Spearman’s rank
correlation will be used to assess correlation between two factors. The distribution of the categorical
variables among the different age groups and gender will be compared using Chisquare test.
Following analysis confidence level and p-value will be checked to find out statistical significance of
data.

75
PROFORMA: CASE RECORD

IDENTIFICATION AND PATIENT DEMOGRAPHICS

1. NAME: UHID NUMBER

2. ADDRESS
76
3. OCCUPATION

4. RELIGION

5. AGE:

6. GENDER: M F

7. HANDEDNESS

NEW/FOLLOW UP:

HISTORY AND EXAMINATION

1. PRESENTING COMPLAINTS

2. HISTORY OF PRESENT ILLNESS

3. MEDICAL COMORBIDITIES AND PREVIOUS TREATMENT

4. BLADDER/ BOWEL INVOLVEMENT

5. HISTORY OF TRAUMA

6. LOCAL EXAMINATION

WORK IMPAIRMENT

1 2 3 4 5 6 7 8 9 10

BED RIDDEN NORMAL

NURICK GRADE

0 1 2 3 4 5

CENTRAL NERVOUS SYSTEM EXAMINATION

77
7. HIGHER MENTAL FUNCTIONS

8. CRANIAL NERVE DEFICITS

9. MOTOR AND SENSORY SYSTEM EXAMINATION

10. REFLEXES

11. CEREBELLAR FUNCTIONS

12. SKULL SND SPINE EXAMINATION

RADIOLOGICAL ASSESSMENT SYRINX

o >=6CM

o<6CM

CRANIOMETRIC MEASUREMENTS:

COMPUTATIONAL FLOW DYNAMICS ANALYSIS:

HYDROCEPHALUS

o YES o

NO

INSTABILITY

o AAD

o CERVICAL o

LUMBAR

CORD SIGNAL CHANGES

78
o NORMAL

o T1 o T2 o BOTH

o INFORMED CONSENT

Full Study Title: Prospective Study For Evaluation Of CSF Flow Dynamics Across Foramen
Magnum In Adult Chiari Malformation/ Syringomyelia Complex Patients And Its Clinical Correlation
With Outcomes After Surgery

You have been requested to participate in a research study.

You have been informed about the study by Dr. Manas Prakash, you can contact anytime if you
have any queries regarding the research study.

Please initial
box.

(Subject)

1. I confirm that I have read and understood the information sheet for The study. I [ ]
confirm that the study has been explained to me and I have had ample time and
opportunity to ask questions.

79
 2. I understand that my participation in the study is voluntary and that I am free to [ ]
withdraw at any time, without giving any reason, without my medical care or legal
rights being affected.

3. I agree not to restrict the use of any data or results that arise from this study [ ]
provided such a use is only for scientific purpose(s)

I confirm that I have fully explained all aspects of the trial to the subject (or legally acceptable
Representative)

Signature of the person conducting the Informed Consent;

Manas Prakash ________________ ____/ __ /______

Signature of the Date Month Year

Study Investigator Study Investigator

________________ ___________________ ____/ _/______

Name of the person Signature of the person Date Month Year

(To be completed by the Patient)

________________ ___________________ ____/___/___

Name of the Witness Signature of the witness Date Month Year

(To be completed by the

witness)

80
सूचित सहमचत
पूर्ण अध्ययन शीर्णक: सीएसएफ (मस्तिष्कमेरु द्रव) फ्लो डायनामिक्स के व्यापक िू ल्ाां कन के मिए सांभामित
अध्ययन िैग्नि इन एडल्ट मियरी िािफॉिेशन / सीररां गोिीमिया कॉम्प्लेक्स पेशेंट्स एां ड सर्जरी के बाद इसके
क्लिमनकि सहसांबांध आपसे एक शोध अध्ययन िें भाग िेने का अनुरोध मकया गया है

कृपया
प्रारां मभक
बॉक्स।
(मिषय)
[ ]
1. मैं पुचि करता हूं चक मैंने अध्ययन के चिए सूिना पत्र पढा और समझा है । मैं पुचि

करता हूं चक अध्ययन ने मुझे समझाया है और मेरे पास सवाि पूछने के चिए पयाा प्त
समय और अवसर है ।

[ ]
2. मैं समझता हूं चक अध्ययन में मे री भागीदारी स्वैस्तिक है और मैं चिना चकसी कारण
के, चिना चकसी चिचकत्सीय दे खभाि या कानूनी अचिकार ूं के प्रभाचवत हुए चिना चकसी

भी समय वापस िेने के चिए स्वतूंत्र हूं ।

81
 [ ]
3. िैं इस अध्ययन से उत्पन्न मकसी भी डे टा या पररणािोां के उपयोग को प्रमतबांमधत नही ां
करने के मिए सहित हां , बशते ऐसा उपयोग केिि िैज्ञामनक उद्दे श्य के मिए हो

मैं पु चि करता हूं चक मैं ने परीक्षण के सभी पहिु ओूं (या कानूनी रूप से स्वीकाया प्रचतचनचि) क पू री तरह से समझाया है .

cerebrospinal fluid (CSF) - मस्तिष्कमे रु द्रव (सीएसएफ)

सू चित सहमचत का सूं िािन करने वािे व्यस्ति का हिाक्षर;

मानस प्रकाश का हस्ताक्षर _____________ __ दिनाांक / माह / वर्ण

अध्ययन अन्वेर्क

82
________________ ___________________ ____/ /__
व्यक्लि का नाि व्यक्लि का हस्ताक्षर मदनाां क/ िहीना /िषज

(रोगी द्वारा पूरा मकया र्ाना है )

________________ ___________________ ____/ /__

व्यक्लि का नाि व्यक्लि का हस्ताक्षर मदनाां क /िहीना /िषज

(गवाह द्वारा पूरा चकया जाना)

83
Patient Information Sheet
Dear Patient/guardian of ....................................................

1. We are conducting an observational study at the All India Institute of Medical Sciences,
Bhopal. Patient/guardian is invited to take part in the study. You are requested to go through the
following paragraphs: CSF flow dynamics across the foramen magnum in patients with chiari
malformation before and after the foramen magnum decompression surgery.
Correlating the above finding with the pre and post operative clinical status.

i) Title: Prospective Study For Evaluation Of CSF Flow Dynamics Across Foramen Magnum In Adult
Chiari Malformation/Syringomyelia Complex Patients And Its Clinical Correlation With Outcomes After
Surgery
ii) Purpose of the Study: The aim of our study is to evaluate the change in neurological,
functional ,radiological and CSF flow across foramen magnum in adult chiari malformation patients
undergoing for surgical treatment.
iii) Methodology: If patient will agree to participate in this study, he will be This will be a
prospective observational study in which the patients (>18years age) who need to undergo elective
foramen decompression surgery. Patient who is planned to undergo elective surgery will be
examined with MRI scan of the CVJ before the surgery and in post-operative after 3 months of
surgery.
MRI scan is a non invasive, painless technique. During the procedure and study period patient will be
monitored according to the standard guidelines of the institute. Any side effects will be treated
accordingly.

iv) Eligibility to participate in the study: Any patient who undergo elective Foramen Magnum
Decompression surgery for adult chiari malformation type 0, 1 or 1.5 with no other co-morbidities
and aged above 18 years is eligible to participate in the study.

v) Any risk to the subject associated with the study: As MRI is non-invasive method, so risk
associated with my study is very unlikely.

vi) Confidentiality: All the records will be confidential and the patient identity would be known
to the chief investigator and would not be released to anybody else.

vii) Provision of free treatment for research related injury: No research related injury is
expected.

viii) Freedom of individual to participate and to withdraw from research at any time without
penalty or loss of benefits to which the subject would otherwise entitled: Participation in the study
is voluntary. Refusal to participate will not influence care of the participants in this hospital in any
way.

ix) Costs and Source of investigations and drugs: You will not be charged anything for anything
else related to this study project.

84
x) Available information sources: The study is approved by the ‘Institutional Human Ethics
Committee’ AIIMS Bhopal. Any query regarding the study can be clarified by contacting Dr. Manas
Prakash (8887718651), investigator of the study.

Contact details of the ethics committee:

Dr.AshwinKotnis, 21
Member secretary,
IHEC,
AIIMS, Bhopal
Email:ihec@aiimsbhopal.edu.in
Contact no. 07773000869

85
रोगी सूिना पत्र

मप्रय रोगी / अमभभािक ……………………………………….

1. हम अस्तखि भारतीय आयु चवाज्ञान सूं स्थान, भ पाि में एक पया वेक्षणीय अध्ययन कर रहे हैं । अध्ययन में भाग िे ने के चिए र गी
/ अचभभावक क आमूं चत्रत चकया जाता है । आपसे चनम्नचिस्तखत पै राग्राफ ूं के माध्यम से जाने का अनु र ि चकया जाता है : फ रै म
मै ग्नम डीकम्प्रे शन सजा री के पहिे और िाद में िीररयार चवकृचत के र चगय ूं में सीएसएफ प्रवाह की गचत।

पू वा और प स्ट ऑपरे चिव नै दाचनक स्तस्थचत के साथ उपर ि ख ज क सहसूं िूंचित करना।

i) शीषजक: प्रौढ़ मियारी मिरूपण / सीररां गोिीमिया र्मटि रोमगयोां िें सीएसएफ (मस्तिष्कमेरु द्रव) फ्लो डायनामिक्स
के व्यापक िूल्ाां कन के मिए सांभामित अध्ययन / सर्जरी के बाद पररणािोां के साथ इसके नैदामनक सहसांबांध

ii) अध्ययन का उद्दे श्य: हमारे अध्ययन का उद्दे श्य शल्यचिचकत्सा उपिार के चिए आने वािे वयस्क िीरी चवकृचत वािे
र चगय ूं में मस्तिष्क के वृ क्क में न्यू र िॉचजकि, कायाा त्मक, रे चडय िॉचजकि और सीएसएफ प्रवाह में पररवता न का मूल्याूं कन
करना है ।

iii) काया प्रणािी: यचद र गी इस अध्ययन में भाग िे ने के चिए सहमत ह जाएगा, त वह यह एक सूं भाचवत अवि कन अध्ययन
ह गा चजसमें उन र चगय ूं (> 18 वर्ा की आयु ) क ऐस्तिक फ रामे न डीकम्प्रेशन सजा री से गु जरना ह गा। र गी ज ऐस्तिक
सजा री से गु जरने की य जना िना रहा है , उसकी सजा री से पहिे और सजा री के िाद के ऑपरे शन में सीवीजे के एमआरआई
स्कैन के साथ जाूं ि की जाएगी।

एमआरआई स्कैन एक गै र इनवे चसव, ददा रचहत तकनीक है । प्रचिया और अध्ययन की अवचि के दौरान र गी की चनगरानी
सूं स्थान के मानक चदशाचनदे श ूं के अनु सार की जाएगी। चकसी भी पक्ष प्रभाव के अनु सार इिाज चकया जाएगा।

iv) अध्ययन में भाग िे ने की पात्रता: क ई भी र गी ज वयस्क चियारी चवकृचत के चिए वैकस्तिक फ रामे न मै ग्नम चडकम्प्रे सन
सजा री से गु जरता है , 0, 1 या 1.5 चजसमें क ई अन्य सह-रुग्णता नहीूं है और 18 वर्ा से अचिक आयु के व्यस्ति अध्ययन में भाग
िे ने के चिए पात्र हैं ।

v) अध्ययन से जु डे चवर्य के चिए क ई भी ज स्तखम: िूूं चक एमआरआई गै र-इनवे चसव पद्धचत है , इसचिए मे रे अध्ययन से जु डा
ज स्तखम िहुत कम है ।

vi) ग पनीयता: सभी ररकॉडा ग पनीय ह ग

ूं े और र गी की पहिान मुख्य जाूं िकताा क पता ह गी और चकसी अन्य क जारी
नहीूं की जाएगी।

vii) अनु सूंिान से सूं िूंचित ि ि के चिए मुफ्त उपिार का प्राविान: अनु सूंिान से सूं िूंचित चकसी भी ि ि की उम्मीद नहीूं है ।

viii) चकसी क चकसी भी समय दूं ड या िाभ के नु कसान के चिना चकसी भी समय अनु सूंिान से पीछे हिने की स्वतूं त्रता,
चजसका चवर्य अन्यथा हकदार ह गा: अध्ययन में भागीदारी स्वैस्तिक है । भाग िे ने से इनकार करना चकसी भी तरह से इस
अस्पताि में प्रचतभाचगय ूं की दे खभाि क प्रभाचवत नहीूं करे गा।

ix) िागत और जाूं ि और दवाओूं का स्र त: इस अध्ययन पररय जना से सूं िूंचित चकसी भी िीज के चिए आपसे कुछ भी
शु ल्क नहीूं चिया जाएगा।

86
x) उपिब्ध सू िना स्र त: अध्ययन क सूं स्थागत मानव आिार सचमचत ’एम्स भ पाि द्वारा अनु म चदत चकया गया है । अध्ययन
के िारे में चकसी भी प्रश्न क अध्ययन के अन्वेर्क डॉ। मानस प्रकाश (8887718651) से सूं पका करके स्पि चकया जा
सकता है ।

आिार सचमचत के सूंपका चववरण:

डॉ.अचिन क िचनस, २१

सदस्य सचिव,

IHEC,

एम्स, भ पाि

ई-मे ि: ihec@aiimsbhopal.edu.in

सूं पका नूं िर। 07773000869

87
88