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International Journal of Biological & Medical Research

Int J Biol Med Res. 2011; 2(4): 1181 – 1183

Arnold- chiari malformation Type-1 with Syringomyelia- a serial imaging study

A R T I C L E I N F O A B S T R A C T

Keywords:

Case Report

Arnold-chiai malformation Type-1, Magnetic Resonance Imaging, Surgical decompression, Syringomyelia.

1. Introduction

Arnold-chiai malformation Type-1 is hind brain herniation through the foramen magnum.

Syringomyelia is a hydromyelia, the commonest cause of which is Arnold-chiari malformation.

The present study aimed at a continuous follow up of a patient over a period of 25 years to

document the rate of progression and assess the result of surgery. The case was followed up by

Magnetic Resonance Imaging at regular intervals. Surgical decompression did not halt the

progression of symptoms though imaging is stationary.

Arnold chiari malformation Type 1 is a congenital malformation,

generally asymptomatic during childhood, often manifests with

headaches and cerebellar symptoms herniation of cerebellar

tonsils in MRI scan of cervical spine[1,2]. Symptoms are headaches

aggravated by valsalva manoeuvres such as yawning, laughing,

crying, coughing, sneezing or straining, tinnitus, dizziness, vertigo,

nausea, nystagmus, facial pain, muscle weakness, impaired gag

reflex, restless leg syndrome, sleep apnoea, dysphagia, impaired

coordination, pupillary dilatation, dysautonomia, tachycardia,

syncope and chronic fatigue. The blockage of Cerebro-Spinal Fluid

(CSF) flow may also cause a syrinx to form, eventually leading to

Syringomyelia. Central cord symptoms such as hand weakness,

dissociated sensory loss, and, in severe cases, paralysis may occur.

Diagnosis is made through a combination of patient history,

neurological examination, and Magnetic Resonance Imaging

(MRI). The radiographic criterion for diagnosing a congenital

Chiari I Malformation is a downward herniation of the cerebellar

tonsils greater than 5 mm below the foramen magnum. Other

imaging techniques involve the use of 3 Dimensional

Computerized axial Tomography (3-D CT) imaging of the brain and

cine imaging can be used to determine if the brainstem is being

compressed by the pulsating arteries that surround it. Some

authors support conservative treatment [3]. Once symptomatic

onset occurs, a common treatment is decompression surgery, [4]

in which a neurosurgeon usually removes the lamina of the first

and sometimes the second or even third cervical vertebrae and

part of the occipital bone of the skull to relieve pressure. The flow

of spinal fluid may be accompanied by a shunt. Since this surgery

usually involves the opening of the dura and the expansion of the

space beneath, a dural graft is usually applied to cover the

expanded posterior fossa (duraplasty). Bony decompression of the

foramen magnum together with some form of dural

decompression is all that is required to treat this type of

Syringomyelia [5-8]. Detethering the spinal cord as an alternate

approach relieves the compression of the brain against the skull

opening (foramen magnum), obviating the need for

decompression surgery and associated trauma. However, this

approach is significantly less documented in the medical

literature, with reports on only a handful of patients. Patients who

have Syringomyelia present with a variety of symptoms ranging

from neck and arm pain through to fairly severe disability, with

muscle weakness and paralysis. Once the diagnosis of

Syringomyelia is suspected, it is readily confirmed by an MRI scan.

If the cavity in the spinal cord is enlarging and threatens to cause

significant disability, there may be a place for surgical intervention.

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a Assistant professor of Biochemistry,Maharajah's Intitute Of Medical SciencesNellimarla, VizianagaramPIN-535 217,Andhra pradesh,Indiab Physician & Pulmonologist,Visakhapatnam,PIN-530 017,Andhra pradesh, India

a bDr. Padmasree Dantu , Dr. Srinivas Pusuluri

* Corresponding Author : Dr. Padmasree Dantu

Assistant professor of BiochemistryMaharajah's Intitute Of Medical SciencesNellimarla, Vizianagaram - 535 217Andhra pradeshIndiaE.mail: docpadmaamc@yahoo.co.in

Copyright 2010 BioMedSciDirect Publications. All rights reserved.c

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Padmasree Dantu & Srinivas Pusuluri / Int J Biol Med Res. 2011; 2(4): 1181 – 1183

2. Case report

2.1.Case history

A 20-year-old male presented with Symptoms (first noticed

sometime in 1985- 86). He developed Pain with parasthesia in the

left cubital fossa, latter pain and parasthesia spread to the whole of

left upper limb, Pain nape, difficulty in performing fine moments

like buttoning, fine dissections, giddiness on extension of neck,

heaviness of the left upper limb. Findings at that time were no

sensory loss, Weakness of the left thenar and hypothenar muscles

and also the lumbricals, hyperasthesia and burning sensation of

left arm, forearm and left half of the chest, upper limbs deep

tendon reflexes absent, lower limbs deep tendon reflexes

exaggerated, bowels and bladder normal.

Investigations Complete Blood Picture (CBP), CSF analysis,

Sensory Evoked Potential (SEP), Electromyogram (EMG), CT scan

brain was taken. As there were no neurological deficits a

conservative approach was advised. There was relatively no

progression of the symptoms until 2003. He was reviewed

regularly. MRI service, dorso lumbar spine was taken in 1992. MRI

cervical spine taken in 1993. In 2003 there was sudden change in

the presentation, with a nasal quality and hoarseness of voice,

unsteady gait, and giddiness after a cough/sneeze, cough syncope,

easy fatigability for which advised emergency MRI. Patient

underwent surgery in April 2003. The procedure done was “

Posterior foramen magnum decompression, excision of posterior

arch of C1 and duraplasty”. The postoperative period was

uneventful. He continued to have the same symptoms as before

with the exception of absence of cough syncope and giddiness after

cough. Follow up MRI scans were taken 4years and 8 years after

surgery. 8 years post surgery, noticed an increased nasal quality of

his voice and that some of his words were slurred. There was

reduction in the volume of his voice, snoring. Findings were absent

gag reflex, anesthesia of posterior pharyngeal wall and left vocal

cord was paralyzed. Patient continues to suffer physically and

psychologically inspite of surgery.

Figure 2. Post surgery (4 years later), MRI showing syrinx

from C2-D3. Outcome after decompression was not

satisfactory as there was no clinical improvement and

imaging showed a persisting cavity.

Figure 3. Imaging status 8 years after surgery showing

retrolisthesis of C4 over C5 and the extent of syrinx remained

the same. This is one of the few cases where surgery could not

help the patient.

Post surgery (4 years later), MRI showing syrinx from C2-D3

MRI 8 years after surgery showing retrolisthesis of C4 over C5

4.Conclusion

CBP, CSF analysis, SEP, EMG were normal at the time of diagnosis

and CT scan brain showed early Syringomyelia with basilar

impression.

MRI cervico, dorso lumbar spine showed Syringomyelia of cord

extending from C2 to D3 disc level with evidence of tonsillar

herniation extending up to base of odontoid process. In MRI

cervical spine after one year, there is evidence of Arnold chiari

malformation Type 1 with Syringomyelia.

Before any surgery is undertaken, detailed discussions need to

take place between the patient and the neurosurgeon, as to the

potential benefits of surgery as well as the inconveniences,

discomforts and risks that go with an operation on the brain or

spinal cord. Surgery has often little or no benefit with substantial

risk. Decisions regarding surgery are best based on patient

cost/benefit. The cost of brain surgery is substantial - medical risk,

monetary cost, and time cost. The main benefit to Chiari surgery is

prevention of progression of a condition. Chiari surgery is nearly

always best avoided in persons who have no neurological signs

referable to the posterior fossa. Operated for progressive physical

signs that are unequivocally due to the Chiari. Decisions regarding

whether or not surgery is indicated are best made by non-

surgeons (i.e. neurologists), as surgeons are often biased towards

surgery due to their training, inclination towards active

treatments, and other factors. An unbiased second opinion could

avoid a lifetime of neurological injury. Paresthesia or anesthesia

symptoms were less likely to improve with hindbrain

decompression. These findings may help guide surgical decision-

making and aid in patient education.

3.Discussion

Figure 1. Follow up MRI, before surgery showing extension of the lesion up to D5.

MRI, before surgery showing extension of the lesion up to D5

1183

5.Acknowledgements

6.References

Thankful to the co-author cum the patient to bear with, in

pressing times of tedious study. High regards for the department

of Radiodiagnosis, Maharajah's Intitute Of Medical Sciences,

Vizianagaram for their timely help.

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ISSN: 0976:6685.c

Padmasree Dantu & Srinivas Pusuluri / Int J Biol Med Res. 2011; 2(4): 1181 – 1183