ORIGINAL ARTICLE

Delayed diagnosis of subacute angle closure glaucoma in patientspresenting with headaches

Ronit Nesher • Michael D. Mimouni •

Shafik Khoury • Gideon Nesher • Ori Segal

Received: 20 January 2014 / Accepted: 10 March 2014

� Belgian Neurological Society 2014

Abstract Subacute angle closure glaucoma (SACG) may

lead to chronic angle closure glaucoma and irreversible

vision loss. Headaches may be the sole presenting symp-

tom. This study characterizes the medical course and

symptoms of patients with SACG in whom headache was

the major symptom. This retrospective observational study

consisted of 30 consecutive patients, suffering from head-

aches and diagnosed with SACG, collected from the

Glaucoma Service at the Ophthalmology Department of

Meir Medical Center, Kfar Saba, Israel, a tertiary care

referral facility. The primary study outcomes were reasons

for referral, number of specialists visited and number of

imaging studies performed before diagnosing SACG and

headache characteristics. The majority of the patients

experienced headaches once or twice a week. Four patients

suffered a classic SACG pain involving the eye and frontal

or hemicranial area. The mean time from onset of head-

aches to diagnosis was 2.6 years. The main reason for

referral to the glaucoma clinic was consultation (53 %),

and SACG was suspected by the referring physicians in

two patients. Seventy-three percent of the patients were

referred to at least three physicians in various medical

specialties prior to referral to the glaucoma clinic. Patients

usually do not volunteer history regarding headaches and

clinicians often do not associate headaches with SACG in

the absence of ocular symptoms. SACG should be included

in the differential diagnosis in individuals older than

40 years presenting with late onset of headaches. Such

patients should be referred to an ophthalmologist.

Keywords Subacute angle closure glaucoma �Iridocorneal angle closure � Glaucoma � Headaches �Migraine

Introduction

Among the clinical entities of glaucoma associated with a

narrow irido-corneal angle, subacute angle closure glau-

coma (SACG) is one of the less recognized. Although

subacute iridocorneal angle closure might be a more

appropriate term, regarding the lack of glaucomatous visual

field or optic nerve damage in many instances, SACG is

still the term used to describe this symptomatology in most

publications.

The diagnosis is often reached when the classical pre-

sentation occurs. Patients present with periodic unilateral

ocular or periocular pain, associated with the finding of

transiently elevated intraocular pressure in the presence of

narrow irido-corneal angles. However, some patients may

present with headaches in the absence of ocular discomfort.

The typical presentation of SACG is often incomplete and

may erroneously lead to a diagnosis of migraine [1]. The

natural history of SACG patients varies. Some go through

complete resolution of symptoms, others develop severe

acute attacks with highly elevated intraocular pressure,

intense pain, blurring of vision, nausea and vomiting, and

R. Nesher (&) � M. D. Mimouni � O. Segal

Department of Ophthalmology, Meir Medical Center,

the Sackler Medical School, Tel Aviv University, 59

Tchernichovsky St., Kfar Saba, Israel

e-mail: nesher@inter.net.il

S. Khoury

Department of Internal Medicine, Meir Medical Center,

the Sackler Medical School, Tel Aviv University,

Kfar Saba, Israel

G. Nesher

Department of Internal Medicine, Shaare-Zedek Medical Center,

The Hebrew University Medical School, Jerusalem, Israel

123

Acta Neurol Belg

DOI 10.1007/s13760-014-0290-2

one-third slowly progress to close the entire irido-corneal

angle which may result in the development of chronic

angle closure glaucoma [2]. Thus, early diagnosis and

treatment of these patients are of utmost importance. The

aim of this study was to describe the clinical course of

patients with SACG whose major complaint at presentation

was headaches, and to characterize their symptoms and

their work-up prior to referral to the glaucoma clinic. We

hypothesized that SACG presenting as a headache might be

overlooked by physicians in various medical specialties,

leading to a significant delay in diagnosing and treating this

condition.

Methods

All data for the study were collected and analyzed in

accordance with the policies and procedures of the insti-

tutional review board of Meir Medical Center and the

tenets set forth in the Declaration of Helsinki. A retro-

spective consecutive design was used. The patients in this

study were individuals suffering from headaches as the

major symptom and eventually diagnosed as suffering from

SACG. All patients were examined in a glaucoma clinic in

one tertiary care medical center between 1995 and 2009,

and then treated with laser iridotomy (LI) and followed.

Excluded from this study were patients who did not

experience resolution of headaches following treatment

with LI. There were 30 patients: 8 males and 22 females.

Their mean age was 58 years (range 36–75). 23 were

myopic (near-sighted) and 11 were hypermetropic (far-

sighted). The patients’ examinations included a detailed

medical history, complete eye examination and a detailed

description of the nature of the headaches and associated

symptoms. All patients underwent LI, while some required

laser iridoplasty.

Results

The patients were referred by various medical profession-

als. Figure 1 illustrates the numerous pathways that led to

glaucoma consultation. The variety of consultants that

patients have encountered prior to arriving at the glaucoma

clinic included a hospital or community neurologists,

hospital neuro-ophthalmologists, and hospital or commu-

nity ophthalmologists. All patients were examined by a

general ophthalmologist at some point, and 40 % were

examined by a neurologist. All patients were examined by

at least two specialists prior to referral to glaucoma clinic,

73 % were examined by three specialists or more, and

20 % by five specialists or more. Consultation was the

main reason for referral to the glaucoma clinic (16

patients). In 13 of these patients, the referring physicians

were unaware of the patients’ headaches, and patients were

referred for suspected primary open-angle glaucoma

(Fig. 2). In three cases, the referring physicians were aware

of the headaches and referred the patients for comprehen-

sive eye examination. Fourteen patients were diagnosed

with narrow irido-corneal angles, and referred by general

ophthalmologists for LI procedures. However, in only two

cases, a diagnosis of SACG was suspected. Altogether,

although headaches were present in all patients, in 60 % of

the cases the referring physicians were unaware of these

symptoms. Table 1 elaborates the features of the patients’

headaches. Only four patients (13 %) suffered from a

typical SACG pain involving the eye and frontal or

hemicranial area. Eye pain was experienced by only six

patients (20 %), but the main discomfort in these cases

came from concomitant headaches. In 18 patients (60 %),

there was no pain in ocular or periocular area at all. In six

patients (20 %), the pain was mostly periocular. The fre-

quency of headaches ranged from one episode per month to

daily headaches. The majority of patients experienced

headaches once or twice per week. Some patients suffered

from additional symptoms: nausea (six patients), blurred

vision (five patients), vomiting, diarrhea and vertigo (each

in one case). Imaging modalities, such as a head CT scans

or MRI, are often part of the workup of recurrent head-

aches. 13 patients (43 %) underwent at least one imaging

procedure. One patient underwent head CT scans on three

occasions as part of the continuous quest for diagnosis

while another patient had both head CT scan and MRI. The

mean time from onset of headache episodes to the final

diagnosis of SACG was 2.6 ± 1.8 years.

Discussion

Headache is a very common complaint with a wide dif-

ferential diagnosis [3–6]. Many patients feel that mild to

moderate headaches are the inevitable part of the stressful

daily life they are leading. Therefore, patients often fail to

mention it to their treating physicians. Moreover, with the

abundance of over-the-counter analgesics, patients often

turn to self-treatment and do not seek medical assistance

for these complaints. Only when the frequency of head-

aches or their intensity increase in severity and become

unbearable, patients seek medical advice.

Another factor leading to delayed diagnosis of SACG is

the insufficient awareness of this syndrome by the medical

community (including internists, neurologists and even

ophthalmologists). Thus, the fact that in 60 % of our

patients the history of headaches was not revealed prior to

the examination in the glaucoma clinic is probably due to

both lack of patients’ volunteering this information and

Acta Neurol Belg

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suboptimal medical history taking by physicians. In that

context, it was interesting to note that even thorough

reviews on the art of history taking in a headache patient

fail to give in-depth information about SACG [7]. While

the anatomical narrowing of the irido-corneal angle in our

group of patients was clearly detected by the referring

ophthalmologists, many did not question the patients about

the existence of headaches and others did not suspect the

causal relationship between narrow irido-corneal angles

and headaches. The lack of awareness to SACG diagnosis

resulted in multiple physician referrals, unnecessary

investigations, inappropriate treatment and substantial

delay in making the correct diagnosis and applying the

appropriate treatment.

Similar findings were previously reported by us in a

small group of nine cases [8] and by Schindler and col-

leagues [9] in a case series of 11 SACG patients. In that

group, the delay in diagnosis was 2.6 years, during which

45 % of these patients underwent some form of imaging

such as CT or MRI. Recently, Hollands and colleagues [10]

recognized that the role of the family practitioner in

identifying patients with primary open-angle glaucoma was

limited. In contrast, in SACG patients, who often complain

of headaches, an attentive family practitioner may play a

pivotal role in suspecting this diagnosis and referring these

patients to an ophthalmologist for evaluation of the irido-

corneal angles by gonioscopy. In view of the fact that angle

closure glaucoma carries a threefold excess risk of severe,

bilateral visual impairment compared to open-angle glau-

coma [11], recognition of the entity of SACG which may

lead to angle closure glaucoma is critical. Lewis and

Fourman [12] suggested that any patient with new-onset

headache whose pain does not readily conform to a well-

defined headache syndrome should be referred to an

Fig. 1 Routes of referral of

patients with headaches to the

glaucoma clinic. ER emergency

room

Fig. 2 Reasons for referrals to the glaucoma clinic. Patients referred for LI were diagnosed as having narrow irido-corneal angles and referred

for this preventive procedure. ACG angle closure glaucoma, IOP intraocular pressure, LI laser iridotomy, POAG primary open-angle glaucoma

Acta Neurol Belg

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ophthalmologist, specifically for gonioscopy. We fully

embrace this recommendation.

The true incidence of SACG is unknown, since a

number of cases are asymptomatic, especially among

people of Asian origin, and others are misdiagnosed [13–

15]. Friedman et al. [16] recently reviewed the topic of

angle closure and angle closure glaucoma in an attempt to

summarize the current practice in these cases as well as

future approaches. Currently, the treatment of choice is LI.

This procedure creates an opening in the iris, forming a

direct passage of aqueous from the posterior to the anterior

chamber of the eye. Thus, aqueous can reach the irido-

corneal angle at all times, including periods when pupillary

block occurs, preventing intraocular pressure from rising.

Preventive LI in individuals with narrow irido-corneal

angles is important as it prevents the development of an

acute attack of angle closure glaucoma in most cases. In

addition, it prevents the occurrence SACG.

Limitations of our study include its retrospective design

and the relatively small number of participants. In addition,

it lacks data on the incidence of headaches as the main

manifestation of SACG. This can only be achieved by a

prospective study in patients with late onset headaches.

Headaches may be the sole manifestation of SACG. The

diagnosis of SACG is often overlooked. Patients often feel

that headaches are part of the stressful life they are leading

and, therefore, often fail to mention it, and physicians often

do not associate headaches with SACG in the absence of

prominent eye symptoms. SACG should be included in the

differential diagnosis in all individuals over 40 years old

with late onset of headaches. Such patients should be

referred to an ophthalmologist for gonioscopic evaluation

of the anterior chamber angle. In most patients, headaches

disappear completely after performing LI and only a small

percentage require further treatment with laser iridoplasty

to widen the angle.

Conflict of interest All authors declare no conflict of interest.

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Table 1 Location and frequency of headaches

No. of pts. (n = 30)

Location of headache

Frontal 9

Widespread 6

Periocular 6

Temporal 3

Frontal and eye 2

Hemicranial and eye 2

Temporal and eye 2

Frequency of headaches (per month)

Everyday 3

10–12 3

6–8 9

2–4 10

1 5

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