Chiari 1 Malformation presenting as - PowerPoint PPT Presentation

About This Presentation
Title:

Chiari 1 Malformation presenting as

Description:

... of obscure cause probably caused by C1M 5/12 aged 10-20 _at_ presentation 10/12 had headache Esotropia was the usual squint (10/12) 4/12 : divergence ... – PowerPoint PPT presentation

Number of Views:172
Avg rating:3.0/5.0
Slides: 18
Provided by: yaha
Category:

less

Transcript and Presenter's Notes

Title: Chiari 1 Malformation presenting as


1
Chiari 1 Malformation presenting as
Strabismus of obscure cause
  •  Kowal, L Yahalom, C
  • OMC CERARVEEH, Melbourne

2
Chiari 1 malformation (C1M)
  • Tonsillar herniation 3 - 5 mm below foramen
    magnum
  • Unlike many congenital CNS malformations, C1M
    patients usually asymptomatic until late
    childhood or early adulthood

3
Symptoms Signs of C1M
  • Symptoms nonspecific - headache, dizziness,
    neck pain, extremity weakness, numbness .
  • Neurologic signs ataxia, dysarthria, nystagmus,
    cranial nerve deficit .

4
THIS SERIES 12 CASES
  • 12 cases of acquired strabismus mostly
    convergent esotropia as the presenting sign of
    C1M.
  • No other credible explanation for the strabismus
  • Isolated acquired esotropia has been previously
    described as a rare presenting sign of C1M in
    several case reports - this will be the largest
    series so far.

5
NON- STRABISMIC FEATURES
  • 10/12 minor neurological symptoms esp.
    headache
  • 2/12 CHIARI PLUS - more serious neurological
    signs/symptoms (10 11)

6
10 /12 patients with esotropia
  • 4/10 DgtN divergence insuff
  • Some may be bilateral 6ths
  • 2/10 NgtD convergence Xs
  • 1/10 ND
  • 1/10 6th nerve palsy
  • 2/10 ET vertical deviation

7
4 patients ET DgtN
Follow up y Treatment ET ? Presenting signs/ symptoms. Duration. Age at presentation
3 Glasses N6-22 D14-26 i/mitt diplopia 1y 7 1
0.1 Glasses N9 D16 i/mitt diplopia 8y 20 4
0.2 Glasses N35 D40 diplopia 1y 18 7
1 NSD (age 6 ½) N14 - 30 D25 - 35 i/mitt ET 1mo 6 9
  • All Headache, no other neurological
    symptoms/signs.
  • All Refraction -1.50 to 1 DS
  • 9 ET N30, D35 at age 7 yrs. Strab surgery
    planned
  • NSD Neurosurgical decompression
  • Range of measurements at different examinations

8
2 patients ET NgtD
Follow up yrs Treatment ET ? Presenting signs symptoms Age at presentation yrs
1.2 Glasses N6-12 D1-4 i/mitt near diplopia 11y 19 6
4 BMR in 10/04. Early orthotropia Increasing to constant ET N42, D36 Infrequent ET 0.5 10
10 Chiari plus. She has developmental
delay, and early closure of fontanelles. MRI
7 mm C1M, stable mild ventriculomegaly.
9
Other presentations
F/up yrs Other Refrn Manifest deviation ? Presenting symptoms Age at presentation
1.2 L IO L SO- Mild L/R R1.25 L1.50 ET 40 ND Recent onset diplopia 14 12
1 R LR- RL -9 ET PP 0-14. R gaze 12-22 Diplopia on R gaze 15 mo 16 5
0.5 Convergence insufficiency R-1.50 L-0.50 XT 18N OD Near Diplopia 18y. Pixilated vision 28 2
  • Patient 12 NSD 6 mo s/p dx of CM1. Little
    improvement in ET ? Bimedial recession 2/04.
    Ortho 8 mo later
  • The other patients manage with glasses.
  • Patients 12 and 2 presented with headache as
    well as diplopia

10
Other presentations
F/u (yrs) Other Signs and findings Pres. symptoms Age presentation
1.3 Myasthenia and thyroid r/u poor motor fusion H -2 to 1 V/-0.5 Esophoria / hyperphoria I/mitt H V diplopia 45 3
1.6 Upper limb paresthesia poor motor fusion RIO , LSO-. Tilt R AHP diplopia 9 8
0.2 Nystagmus ET 16 DN Constant H diplopia 10 y Oscillopsia 60 11
  • 11 is the second Chiari plus. His symptoms
    began with diplopia and balance problems.
    Diplopia persisted s/p 2 neurosurgical
    procedures.
  • 3 and 8 manage well with glasses.

11
Our patients who had surgery
  • 10 Squint sx with good early results (f/u 4w)
  • 9 NSD. Strab persists. BMR planned.
  • 11 NSD. Strab persists. Prism working.
  • 12 NSD. Strab persists. Successful squint sx
    (f/u 8 mo).

NSD neurosurgical decompression
12
Age at presentation
Most of the patients presented outside normal age
range for strabismus
13
ET as only manifestation of C1MSummary of
published literature
Neurosurgery Squint Sx Age y Nunber of cases
Yes with resolution of ET Before C1M Dx Recurrence 6 mo later 13 1 Bixenman J Ped Ophthal Strab 1987
Yes. ET no better PT in borderline C1M. Recurrence 6 mo later. Second sx successful s/p neuro-sx. 13 1 Pokharel JAAPOS 2004
PT2 with resolution of ET. F/u 2-3 y PT in 2. One successful. 2nd recurrence s/p 2 sx. 5 to 37 4 Biousse AJO 2000
Yes with resolution of ET Recurrence 12 mo after surgery 24 1 Passo J Clin N-Ophthal 1984
Yes with resolution of ET Recurrence after surgery 2 Weeks Hamed Ophthalmology1999
Yes. PT4 patients with resolution of ET. gt 4y f/u No 17 to 37 5 Lewis J N-ophthal 1996
PT with resolution of ET No 9 1 Defoort-Dhellemmes Amer Jnl Ophthal 2002
PT - ET no better Botox to LMR (successful after 6 mo f/u) 14 1 Imes -Ophthalmology 2001
One patient from Lewiss series did not get any
sx treatment / PT Primary treatment
14
Summary of published literature ET as the ONLY
manifestation in C1M
  • 16 patients ages 5 - 37
  • 7/16 strabismus sx as primary treatment
  • 6/7 recurrence of strabismus
  • 4/5 subsequent NSD with resolution of strab
  • 8 patients NSD as primary treatment
  • 7/8 had resolution all signs/symptoms
  • Conclusion Strab Sx alone usually ineffective.
    NSD usually necessary effective.

15
Summary
  • We describe 12 cases of acquired strabismus of
    obscure cause probably caused by C1M
  • 5/12 aged 10-20 _at_ presentation
  • 10/12 had headache
  • Esotropia was the usual squint (10/12)
  • 4/12 divergence insufficiency ET, D gt N
  • 1/12 apparent sixth nerve palsy
  • 1/12 unexplained head tilt.
  • 2/12 i/mitt diplopia with poor motor fusion

16
Summary
  • Most patients were referred for neurosurgical
    evaluation. 3/12 had NSD. It is generally felt
    by neurosurgeons that strabismus alone is an
    inadequate reason for NSD.
  • Strabismus did not resolve in these 3 cases,
    with subsequent successful squint surgery in
    1case.
  • Other patients Most manage well with prism
    glasses.

17
Conclusions
  • Isolated acquired strabismus is not a rare
    presenting sign of C1M, and there might be a good
    number of patients being mis / under- diagnosed.
  • Appropriate primary management of C1M with
    strabismus alone whether strabismus surgery or
    NSD is unclear.
Write a Comment
User Comments (0)
About PowerShow.com