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Long case marfan syndrome
1. Long case presentation
Chairman:
Professor Shakwat Ara Shakoor
NIO&H.
Moderator:
Dr. Zahidul Ahsan Menon
Assistant professor, NIO&H.
Presenter:
Maj. (Dr.) A.K.M. Rashed-Ul-Hasan
FCPS Part- 2(Student), NIO&H.
2. Particulars of the patient
1. Name Mr. Hridoy
2. Father’s name Shakwat hossain
3. Age 20 yrs
4. Gender Male
5. Present address Joyram pur, Chuadanga
sadar, Chuadanga.
6. Permanent address -Do-
7 .Occupation Student
8. Date of Examination 01 august 2016
4. History of Present ocular illness
Patient states that he has been suffering from
dimness of vision in left eyes for nine years
which was gradually worsening day by day
but was not associated with pain, redness,
watering, photophobia or haloes around light.
He has no complaints of hearing difficulty.
5. Patient has history of trauma over left
orbital area 09 years back by bamboo
stick. With these problems he visited local
ophthalmologist and was referred to
NIO&H for better management.
6. Past ocular illness
• No history of surgery.
• No history of using spectacles.
General medical & surgical history
• Nothing conributory.
8. Ocular examination
1. Visual
acuity
OD OS
Unaided -
Distant
6/6 6/60
Near N-6 Unable to see
With PH - No improvement
With aid - No improvement
2. Color vision Trichromatic Trichromatic
3. Field of
vision
(confrontation )
Normal Normal
10. Slit lamp examination
OD OS
1. Eye lid &
eye lashes
Normal Normal
2. Conjunctiva
3. Cornea
4. Anterior
chamber
Normal in
depth
Shallow
infero-nasally
Deep
Supero-
temporally
5. Iris Normal Iridodonesis
11. Cont…
6. Pupil 3 mm,
briskly reacting
3 mm,
briskly reacting
7. Crystalline
Lens
Normal Subluxation
infero-nasally
Phacodonesis
Lental opacity
8. IOP (GAT) 14 mm of Hg 16 mm of Hg
9. Gonioscopy
CB
CB
CB
CB CB CB
CB
CB
13. Examination of the posterior
segment
OD OS
1. Media Clear Clear
2. Optic disc Normal
C:D- 0.3
Normal
C:D- 0.3
3.Vascular
arcades
Normally
arranged
Normally
arranged
14. 4. Peripheral
retina
Normal White crescentic
streak along the
infero-temporal
vascular arcade &
“Y” shaped white
streak just below the
macula with some
pigmentary change
in periphery
5. Macula F. Reflex present F. Reflex present
15. Systemic examination
Musculoskeletal features
Tall & thin stature
Disproportionately long limbs.
Arm span > height : 177.5 cm > 172.5 cm
High- arched palate
Chest appearance- normal.
19. Cardio-vascular system
Pulse : 72/min.
BP: 110/70 mmHg
S1 & S2 - Audible
Murmur – absent
Apex beat – palpable in left 5th intercostal space
20. Respiratory system
Genito-urinary system
Nervous system
Gastro-intestinal system
The skin and nails
No Abnormality
Detected
21. Salient feature
Mr. Hridoy , 20 years of age, from
Chuadanga, with the complaints of dimness
of vision in left eye for nine years which was
worsening day by day but was not associated
with pain, redness, watering, photophobia or
haloes around light or deafness. Patient has
history of trauma over left orbital area 09
years back by bamboo stick. No h/o surgery
or spectacle use.
22. Cont…
O/E: VA in RE 6/6 and in LE 6/60. No
improvement of vision with pinhole or
refraction in L/E. Pupillary reaction to light is
brisk in B/E.
On SLE: A/C is shallow infero-nasally & deep
supero-temporally with iridodonesis, infero-
nasally subluxated crystalline lens,
phacodonesis present in L/E.
23. On fundoscopy :
Media clear in B/E with normal right eye
In left eye white crescentic streak along
the infero-temporal vascular arcade &
“Y” shaped white streak just below the
macula with few pigmentary changes in
periphery.
24. Cont…
On Systemic examination:
Extremities are long, arm span
(177.5 cm) > height (172.5cm),
High arched palate.
Thumb sign ( Steinberg’s sign) &
Wrist sign ( walker-Murdoch sign) : +ve
Flat feet