Author Communication
Isolated
Simultaneous Bilateral Adie’s Pupil
Summaya
Khan, Muhammad Azeem Khizer, Saleh Khurshied
DOI
10.36351/pjo.v35i4.899 Pak J Ophthalmol 2019, Vol. 35, No. 4
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See end of article for authors affiliations …..……………………….. Correspondence to: Dr. Muhammad Azeem Khizer Armed
Forces Institute of Ophthalmology Rawalpindi, Pakistan Email:
m.azeem7@gmail.com |
Adie’s pupil, an efferent pupillary
defect is caused by injury to parasympathetic pupillomotor nerve supply to
Iris sphincter muscle. More commonly seen in young adult females with up to
80% cases having unilateral involvement. According to literature, Adie’s
Pupil may occur alone or as a part of a systemic condition or may be
associated with a syndromic presentation. Bilateral Adie’s pupil in a female
at initial presentation with mild visual symptoms and no syndromic
association is sparsely reported in literature. Keywords: Adie’s pupil, Anisicoria, tonic pupil. |
Adie’s pupil is a common cause of anisocoria. It is an efferent
pupil defect, which causes loss of reaction to light of iris sphincter muscle,
the reason being the injury to the post-ganglionic parasympathetic nerves. Such
patients may be asymptomatic but suffer from accommodative symptoms or
photophobia with difficulty in focusing. Signs are anisocoria with light near
dissociation and loss of accommodation and abnormal response to light, which
may be missed or misdiagnosed in a busy clinical setting. Reports of tonic
pupils in literature date back to early 20th century, with half of
the patients being females. In later years, Adie, Moore and Holmes reported 46
more cases out of which 42 were females. According to reports, this disease has
a predominance of women with 20-40 years old age group but has also been reported
in children with a possibility of familial incidence1. In 20% of the 220 cases from literature, both
eyes were involved. The incidence of the condition is reported to be 4-7 per
100,000 per year2.
We present a case of a 30 years
old female with bilateral Adie’s tonic pupils.
CASE
REPORT
A 30 years old female presented with a 3-month history of photophobia
and blurring of near vision, both of which had gradually increased over time.
The patient had no other ocular or systemic complaints. No significant ocular
history was present. Her medical history was insignificant and there was no
history of usage of any systemic or topical medication.
On examination her visual
acuity was 6/6 for far and N6 for near in both eyes (OU). Although she was N6
OU, she required 15-20 seconds of constant effort at the near target to see the
line clearly. The pupils showed anisocoria with a pupillary diameter of 7mm on
right side and 8mm on left (Fig. 1) with patient focusing on
a distant target in a moderately lit room with no response to light stimulus
bilaterally. Segmental contractions of iris were not seen in either eye. The
patient had light near dissociation on both sides with a pupillary diameter of
4mm on right side and 5mm on left side when looking at near target for 60
seconds, which reverted to previous pupillary size
Fig 1: Picture showing bilateral
dilated pupils prior to instillation of topical 0.1% Pilocarpine drops.
after 30 seconds of cessation
of fixation to near target. In a dimly lit room, after instillation of 0.1%
pilocarpine, examination after 30 minutes revealed a pupillary diameter of 5 mm
on right side and 4mm on left (Fig. 2). Rest of the examination
was unremarkable and no evidence of iris trauma or pigment dispersion were
found.
Fig 2: Picture showing bilateral
constricted pupils 30 minutes after instillation of topical 0.1% Pilocarpine
drops.
Detailed neurological examination did not reveal any abnormality. Opinion
of the neurologist was sought, radiological and lab investigations were done,
including MRI Brain and Visual Pathways with contrast, with none of them
revealing any abnormality. Based on history, examination and investigations, a
diagnosis of bilateral Adie’s pupil was made.
The patient was counselled
regarding the condition and was offered prepared topical 0.1% pilocarpine eye
drops. The patient perceived resolution of her complaint of photophobia with
the usage of the offered eye drops in both eyes once daily and was satisfied
with the treatment offered. She was also offered hyperopic correction for near
vision, but was not interested in using near glasses.
DISCUSSION
Adie's syndrome is mostly a disease of young adults. Most patients
with Adie's pupil have a variable accommodative paralysis at the onset, which
is the main cause of their complaints. In 1812, Ware3 described a 30
to 40-year-old woman with a right pupil showing light near dissociation.
In 1902, Saenger4 described a 34-year-old woman with bilateral
light-near dissociation, her left pupil was larger than the right and after
accommodation, it took around 10 minutes for left pupil to return to its
original size. Also in 1902, Strasburger5 reported a patient whose both
pupils were not reacting to light, however, both pupils reacted to near target
but very late, and a delay was noted in relaxing accommodation when refocusing
back to a far target. Strasburger also noted other neurological signs and
symptoms with an early stage of multiple sclerosis considered the most likely
diagnosis. Drouet et al. and Millar et al6 reported bilateral Adie's
pupil in a patient during an attack of migraine. The authors discussed postganglionic
dysfunction as a cause of transient mydriasis. Our patient had no history of
migraine and the pupils were not transiently dilated.
Jivraj and Johnson7 reported a case of a young girl who
had acute unilateral tonic pupil, which became bilateral 2 months later and
found a rare association of neurosyphilis with the condition. Our patient reported
with simultaneous bilateral involvement and was found to be seronegative for
syphilis. An association between Bilateral Adie’s pupil with Sjögren's syndrome
has been reported in many cases8 with high prevalence in females.
Our patient was not found to have any associated feature of the syndrome.
Several cases with Bilateral
Adie's pupil along with Autonomic dysfunction and diminished or absent tendon
reflexes have been reported in literature. Holmes G et al.9
described bilateral Adie’s pupil in 3 patients, who had signs of autonomic
dysfunction (known as Holmes-Adie Syndrome). Our Patient had no evidence of any
Autonomic dysfunction and had normal deep tendon reflexes. Presence of bilateral
Adie's pupil is a relatively rare initial presentation and in such bilateral
cases are commonly reported to present unilaterally and proceed to involve the
other pupil after an interval of few weeks to few months. Our case is relatively
rare that our patient had simultaneous bilateral Adie’s pupil on initial
presentation but with mild visual symptoms. On the other hand, majority of the
cases with bilateral involvement occur in females and in a younger age group,
which is similar to our case. A similar case was reported by Indranil and
Shroff10 but their patient was a male with a longer duration of more
severe visual symptoms which is not commonly reported. Patient was not found to
have any other systemic predisposing conditions or associations and was
supported and evidenced by relevant radiological and lab investigations. Keeping
in mind the differential diagnosis and after proper evaluation, a final
diagnosis of bilateral Adie’s pupil was made.
1.
Lambert SR, Yang LL, Stone C. Tonic pupil associated with congenital neuroblastoma,
Hirschsprung disease, and central hypoventilation syndrome. Am J Ophthalmol
2000; 130: 238-240.
2.
Martinelli P.
Holmes-Adie syndrome. Lancet. 2000; 356: 1760-1761.
3.
Ware J.
Observations relative to the near and the distant sight of different persons.
Philos Trans R Soc London, 1812: 31-50.
4.
Saenger A.
Ueber myotonische Pupillenbewegung. Neurol Zentralblatt. 1902; 21: 837-839.
5.
Strasburger J.
Pupillenträgheit bei Accommodation und Convergenz. Neurol Zentralblatt. 1902; 21:
738-740.
6.
Millar E, Habib M, Gnanaraj L. Bilateral tonic pupil secondary to migraine in a child. J Pediatr
Ophthalmol Strabismus, 2010; 47: e1–2.
7.
Jivraj I, Johnson M.
A rare presentation of neurosyphilis mimicking a unilateral Adie's tonic pupil.
Semin Ophthalmol. 2014; 29: 189–91. [PubMed: 23952008].
8.
Bachmeyer C, Zuber M, Dupont S, Blance P, Dhote R, Mas JL. Adie syndrome as the initial sign of primary Sjogren syndrome. Am
J Ophthalmol. 1997; 123: 691-692.
9.
Holmes G.
Partial iridoplegia associated with symptoms of other diseases of the nervous
system. Trans Ophthalmol Soc UK, 1931; 51: 209–28.
10.
Saha I, Shroff CM, Gupta C, Verma R. An interesting case of simultaneous bilateral Adie's tonic pupil.
Oman J Ophthalmol. 2018; 11 (1): 82–84.
Author’s
Affiliation
Dr. Summaya
Khan
Consultant Ophthalmologist
Armed Forces Institute of Ophthalmology
Rawalpindi, Pakistan
Dr. Muhammad
Azeem Khizer
Resident Ophthalmologist
Armed Forces Institute of Ophthalmology
Rawalpindi, Pakistan
Dr. Saleh
Khurshied
Final Year MBBS Student
Army Medical College, Rawalpindi
Author’s
Contribution
Dr. Summaya
Khan
Conception, design, final approval.
Dr. Muhammad
Azeem Khizer
Literature
search, Acquisition of data, drafting, revision.
Dr. Saleh
Khurshied
Manuscript drafting, final review.