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Year : 2022  |  Volume : 2  |  Issue : 1  |  Page : 109-111

Glaucoma management dilemma in a patient with Allgrove syndrome

SV Aravind Eye Hospital, Tirupati, Andhra Pradesh, India

Date of Submission17-Dec-2020
Date of Acceptance01-Jul-2021
Date of Web Publication07-Jan-2022

Correspondence Address:
Dr. Maninee Suman
SV Aravind Eye Hospital, Alipiri to Zoo Park Road, Beside NCC Campus, Chittoor District, Tirupati, Andhra Pradesh - 517 505
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/ijo.IJO_3680_20

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AAA syndrome or Allgrove syndrome is a rare multisystem disorder. The ocular manifestations reported in the literature include alacrima, keratoconjunctivitis sicca, corneal melts, lacrimal gland atrophy, pupillary abnormalities, accommodative dysregulation, amblyopia, and optic atrophy. We report here the occurrence of glaucoma in a patient with AAA syndrome, which has not been reported earlier, and also discuss the multiple difficulties in managing glaucoma in the presence of the underlying ocular and systemic conditions.

Keywords: AAA syndrome, glaucoma, management

How to cite this article:
Suman M, Ravilla ST, Keerthi, Vardhan S A. Glaucoma management dilemma in a patient with Allgrove syndrome. Indian J Ophthalmol Case Rep 2022;2:109-11

How to cite this URL:
Suman M, Ravilla ST, Keerthi, Vardhan S A. Glaucoma management dilemma in a patient with Allgrove syndrome. Indian J Ophthalmol Case Rep [serial online] 2022 [cited 2022 Aug 14];2:109-11. Available from: https://www.ijoreports.in/text.asp?2022/2/1/109/334946

AAA syndrome, also known as Allgrove syndrome, is characterized by alacrima, achalasia of the esophageal cardia, and adrenocorticotrophic hormone–resistant adrenal insufficiency.[1],[2],[3] Alacrima is the first and most consistent feature of this syndrome.[4] It is likely due to mutations in the AAAS gene whose predicted product is ALADIN (alacrima–achalasia–adrenal insufficiency neurologic disorder); located on chromosome 12q13.[1],[2]

In our literature search, glaucoma has never been reported in this condition. Our patient presented with advanced glaucoma, and we discuss the multiple hurdles in controlling the intraocular pressure (IOP) in AAA syndrome.

  Case Report Top

A 16-year-old girl diagnosed previously with AAA syndrome presented to us with defective vision and grittiness of both eyes (BE). She had a history of absence of tears and difficulty in swallowing solid food since birth and was using lubricating eye drops. She was diagnosed with primary adrenal insufficiency and achalasia cardia of the esophagus at 4 years and underwent pneumatic dilatation of esophagus. She had been on oral hydrocortisone 15 mg and fludrocortisone 100 mcg daily since then.

On ocular examination, her best-corrected visual acuity was 20/40 in BE. Slit-lamp evaluation revealed dry ocular surface, anisocoria, and sluggish pupillary reflex. A cup disc ratio of 0.6 with an early bipolar notch in the right eye (RE) and 0.8 with bipolar thinning in the left eye (LE) was noted, macula was normal in BE. IOP was 34 and 38 mmHg in RE and LE, respectively. On gonioscopy, a ciliary body band was seen in BE. Optic coherence tomography showed retinal nerve fiber layer thinning in BE. Automated perimetry was not reliable. She was diagnosed with severe dry eye in BE, and a provisional diagnosis of juvenile glaucoma or steroid-induced glaucoma was made.

She was advised to continue lubricating eye drops and was initially started on latanoprost eye drops. Timolol was added 2 weeks later as IOP was uncontrolled. Two months later despite good compliance, her IOP was uncontrolled (32 and 40 mmHg in RE and LE, respectively). She was then started on a combination eye drop (brimonidine + brinzolamide) in addition to eye drop Xalacom (latanoprost and timolol).

The child had not been on regular follow-up for over a year because of financial crisis and the COVID-19 (coronavirus disease 2019) lockdown. We were able to contact the family and get them to review after counseling on the urgency of the condition. She had stopped using all antiglaucoma medication (AGM), had developed early cataract (polychromatic luster on the posterior surface), and had uncontrolled IOP over 30 mmHg with cup disc ratio of 0.9, bipolar thinning, and notching in BE [Figure 1] and [Figure 2]. She was started on maximum medical therapy in BE and advised to come after 1 month. After 1 month, IOP was still high in BE and Humphrey Field analyzer 24-2 and 10-2 showing advanced field defect without involving fixation in BE [Figure 3] and [Figure 4]. Due to unsatisfactory IOP control despite maximum medical therapy, surgical options were considered after improving the ocular surface with frequent lubricants. Cataract surgery with gonioscopy-assisted transluminal trabeculotomy (GATT) was planned for LE and eye drop Ripatec was added to RE. Intraoperatively, GATT was aborted because of bleeding from the angle. Limited bent ab interno needle goniectomy (BANG) was done instead. On the first postoperative day, IOP was 33 and 20 mmHg in RE and LE, respectively. Meanwhile, the endocrinologist's opinion was sought to reduce the systemic steroid dosage to 50 mcg fludrocortisone and 10 mg hydrocortisone per day.
Figure 1: Right eye fundus showing glaucomatous optic atrophy

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Figure 2: Left eye fundus showing glaucomatous optic atrophy

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Figure 3: Humphrey Field analyzer 10-2 of right eye

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Figure 4: Humphrey Field analyzer 10-2 of left eye

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IOP was uncontrolled and remained over 30 mmHg in BE at 15 and 30 days postop. We now need to consider the option of a glaucoma drainage device (GDD).

  Discussion Top

There are a few case reports in the literature describing ocular manifestations of AAA syndrome.[1],[2],[3],[4],[5],[6],[7] Till now, glaucoma has not been reported. We postulate that the presence of glaucoma in our patient may be due to long-term use of oral steroids or due to angle abnormalities associated with juvenile glaucoma.

The dilemma in treating the glaucoma in this patient is that we cannot stop oral steroids because her systemic condition will worsen. Also, she cannot be given oral AGM because of achalasia and history of allergy to many oral medications. There are limited options for topical AGM as preservatives can aggravate the ocular surface disease (OSD).[8] Since AAA patients are hypersensitive to cholinergic agents, pilocarpine would have been an option, but then again it would not have benefited long term.[9] There are high chances of trabeculectomy failure because of the dry ocular surface. On the contrary, microtrauma during glaucoma surgery can aggravate OSD.[10]

Weighing the risks and benefits, GATT was considered as the first option as it is a conjunctival sparing surgery in which trabecular meshwork (TM) is circumferentially bypassed via a suture or catheter. Intraoperatively, it was noted that the angle structure was abnormal with more pigmentation of TM. GATT was aborted due to uncontrolled bleeding. BANG was done instead, wherein a 27G bent needle was used to incise the TM and then passed circumferentially along Schlemm's canal for about 90°.

Intraoperative findings suggest that this may be a presentation of juvenile glaucoma. However, the possibility of steroid-induced etiology cannot be ruled out. As the patient is very young, has advanced glaucoma and high IOP despite maximum AGM, and has no improvement with minimally invasive glaucoma surgery, we are considering GDD as our next option. This case report highlights the importance of early diagnosis, multidisciplinary approach in syndromic patients, and the importance of counseling.

  Conclusion Top

This case report gives an insight of the importance of early diagnosis, multidisciplinary approach, timely referral to the appropriate specialists as well as screening for new signs and symptoms at each encounter of patients with AAA syndrome.

Declaration of patient consent

The authors certify that they have obtained all appropriate patient consent forms. In the form the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.

Financial support and sponsorship


Conflicts of interest

There are no conflicts of interest.

  References Top

Mullaney PB, Weatherhead R, Millar L, Ayyash II, Ayberk H, Cai F, et al. Keratoconjunctivitis sicca associated with achalasia of the cardia, adrenal cortical insufficiency and lacrimal gland degeneration: Keratoconjunctivitis sicca secondary to lacrimal gland degeneration may parallel degenerative changes in esophageal and adrenocortical function. Ophthalmology 1998;105:643-50.  Back to cited text no. 1
Brooks BP, Kleta R, Caruso RC, Stuart C, Ludlow J, Stratakis CA. Triple A syndrome with prominent ophthalmic features and a novel mutation in the AAAS gene: A case report. BMC Ophthalmol 2004;4,7.  Back to cited text no. 2
Singh A, Shah A. Esophageal achalasia and alacrimia in siblings. Dementia Indian Paediatr 2006;43:161-3.   Back to cited text no. 3
Moore PS, Couch RM, Perry YS, Shuckett EP, Winter JS. Allgrove syndrome: An autosomal recessive syndrome of ACTH insensitivity, achalasia and alacrima. Clin Endocrinol (Oxf) 1991;34:107-14.  Back to cited text no. 4
Pedreira CC, Zacharin MR. Allgrove syndrome: When a recognizable paediatric disorder occurs in adulthood. Med J Aust 2004;180:74-5.  Back to cited text no. 5
Kimber J, McLean BN, Prevett M, Hammans SR. Allgrove or 4 “A” syndrome: An autosomal recessive syndrome causing multisystem neurological disease. J Neurol Neurosurg Psychiatry 2003;74:654-7.  Back to cited text no. 6
Gazarian M, Cowell CT, Bonney M, Grigor WG. The “4A” syndrome: Adrenocortical insufficiency associated with achalasia, alacrima, autonomic and other neurological abnormalities. Eur J Pediatr 1995;154:18-23.  Back to cited text no. 7
Zhang X, Vadoothker S, Munir WM, Saeedi O. Ocular surface disease and glaucoma medications: A clinical approach. Eye Contact Lens 2019;45:11-8.  Back to cited text no. 8
Chu ML, Berlin D, Axelrod FB. Allgrove syndrome: Documenting cholinergic dysfunction by autonomic tests. J Pediatr 1996;129:156-9.  Back to cited text no. 9
Lam J, Wong T, Tong L. Ocular surface disease in posttrabeculectomy/mitomycin C patients. Clin Ophthalmol 2015;9:187-91.  Back to cited text no. 10


  [Figure 1], [Figure 2], [Figure 3], [Figure 4]


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