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  • Case report
  • Open Access

Idiopathic full thickness macular hole in a 10-year-old girl

International Journal of Retina and Vitreous20184:25

https://doi.org/10.1186/s40942-018-0128-9

  • Received: 21 February 2018
  • Accepted: 3 July 2018
  • Published:

Abstract

Background

Macular holes in children are generally associated with trauma.

Case presentation

We report the first case of an idiopathic full thickness macular hole in a 10-year-old girl. 23-gauge transconjunctival pars plana vitrectomy, induction of a posterior vitreous detachment, ILM blue-assisted internal limiting membrane peel, fluid–air exchange and air-26% sulfur hexafluoride (SF6) exchange was performed with subsequent macular hole closure.

Conclusion

This is the first reported case of an idiopathic full thickness macular hole in a child. Treatment with pars plana vitrectomy with peeling of the ILM resulted in significant anatomic and functional improvement.

Keywords

  • Macular hole
  • Children

Background

A macular hole is a full thickness defect in the neural retina at the fovea. It is thought to occur as a result of pathological changes at the foveal vitreoretinal interface [1]. Idiopathic macular holes most commonly occur in adults in the 6th to 7th decade [2]. Macular holes in children are rare and are generally associated with trauma [3]. We present a case of an idiopathic full thickness macular hole (FTMH) in a child.

Case presentation

A 10-year-old girl presented with reduced vision in the right eye. The vision had deteriorated from 20/17 1 year previously, to 20/60. She was otherwise well, with no history of trauma or inflammation of either eye and no other significant medical or drug history. Her grandfather, and grandfather’s brother had a history of retinal detachment.

Visual acuity (VA) was 20/60 in the right eye and 20/20 in the left eye. Ocular examination was unremarkable except for the presence of a FTMH in the right eye (Fig. 1). There was no evidence of trauma, inflammation or signs of retinal dystrophy. Optical coherence tomography (OCT) showed a 365 μm FTMH with no vitreomacular traction or posterior vitreous detachment (Fig. 2).
Fig. 1
Fig. 1

Colour fundus photograph of the right eye shows a full thickness macular hole

Fig. 2
Fig. 2

Horizontal high definition spectral domain OCT of the right eye shows a full thickness macular hole

Following informed consent of her parents, we carried out a 23-gauge transconjunctival pars plana vitrectomy. Induction of a posterior vitreous detachment (PVD) was completed using triamcinolone, followed by ILM blue-assisted internal limiting membrane (ILM) peel, fluid–air exchange and air-26% sulfur hexafluoride (SF6) exchange. She was positioned face down for 3 days postoperatively. At postoperative week 1, VA was 20/60 with OCT evidence of hole closure (Fig. 3). At postoperative month 1, VA was 20/20, and at 4 months postoperative, the macular hole remained closed with remodeling of the outer retina on OCT (Fig. 4). There was still a small defect at the photoreceptor level.
Fig. 3
Fig. 3

Horizontal high definition spectral domain OCT of the right eye 1 week post operatively shows closure of the macular hole

Fig. 4
Fig. 4

Horizontal high definition spectral domain OCT of the right eye 1 month post operatively shows further improvement in macular architecture

Discussion

There is only one previous report in the current literature of an idiopathic FTMH in a child. This case however, had features of cavitary maculopathy and did not have a full thickness defect [4].

In our case, the patient and her family were very reliable historians, strongly denying any possibility of antecedent trauma. Excellent vision of 20/17 was documented just 1 year prior by her optometrist. However, despite this, we must acknowledge, that given the active nature of children it is possible that an unreported trauma may have occurred in our case. Children often do not immediately report their recent behaviors or visual symptoms, possibly out of fear of retribution or simply a lack of understanding. In addition, the possibility of non-accidental injury should always be considered as other life-threatening injuries and or situations may also be present.

In the setting of blunt trauma, the most common cause of macular hole in children, a contrecoup mechanism as a result of axial globe compression is thought to increase vitreomacular traction forces [5]. Our case illustrates an idiopathic FTMH in a child, in which neither vitreomacular separation or vitreofoveal traction could be visualized clinically or on OCT. The mechanism of idiopathic FTMH formation in a child remains unclear.

Review of the English language literature identified 15 pediatric eyes with a non-traumatic macular hole (Table 1). In contrast to our case, all had clinical or historical features of a secondary non-traumatic cause including: vascular (retinopathy of prematurity (ROP) [6], and Coats’ disease [7, 8]), infective (Bartonella neuroretinitis [911]), and congenital (choroidal coloboma [12], regressed Bergmeister papilla [13]), and juvenile idiopathic epiretinal membrane [14]) entities. A case of accidental Nd:YAG laser induced macular hole in a child has also been reported, photothermal and photomechanical disruption of the retina occurring as a result of energy absorbed by the retinal pigment epithelium (RPE) [15]. The patients with ROP all had vitreoretinal surgery prior to discovery of the macular hole, and all had an associated retinal detachment. They were treated with a combination of open and closed vitrectomy in addition to a radial scleral buckle [6]. Of the 15 eyes, surgical hole closure was attempted in 9 cases. Apart from the patients with ROP, the other 5 cases were treated with vitrectomy, ILM peel and gas tamponade. Anatomic closure of the hole was reported in all 5 cases [4, 7, 1315].
Table 1

Summary of reported non-traumatic full-thickness macular hole in paediatric patients

Author, case year

Associated cause of macular hole

No. of patients

Pt no.

Patient age (months, yrs), gender

Presenting BCVA

Size of hole (μm)

Surgery

Outcome

Last exam BCVA

Ahmad 2005

Retinopathy of Prematurity

5

1

10 months

F

Not reported

Not reported

Yes

Scleral buckle

PPV + FAX

Large macular hole

20/3270

   

2

3 years 2 months

F

Not reported

Not reported

Yes

PPV + ILM peel +FAX

Cyanoacrylate glue applied to the hole

Radial scleral buckle

Cyanoacrylate glue present

Retina attached

20/760

   

3

1 year 3 months

F

Not reported

Not reported

Yes

Radial sponge × 2

Retina attached

CF 2 feet

   

4

1 year 2 months

F

Not reported

Not reported

No

Total retinal detachment

NLP

   

5

2 years

M

Not reported

Not reported

Yes

Radial sponge

PPV + ILM peel + silicone oil

Retina attached

20/360

Albini 2005

Bartonella Neuroretinitis

1

1

10 years

F

CF 1 foot

750 × 500 μm

No

Not reported

Not reported

Yokoyama 2005

Juvenile Idiopathic Epiretinal Membrane

1

1

3 years

F

20/125

Not reported

Yes

PPV + ILM Peel

Macular hole closed

20/80

Nakano 2005

Incomplete regression of a Bergmeister Papilla

1

1

10 years

F

20/25

Not reported

Yes

PPV + ICG ILM Peel + SF6

Post operative prone position 1 week

Macular hole closed

20/60

Donnio 2008

Bartonella Neuroretinitis

1

1

11 years

M

20/200

Not reported

No

Not reported

Not reported

Kumar 2010

Coats’ Disease

1

1

9 years

M

20/400

Not reported

No

Not reported

Not reported

Wong 2012

Coats’ Disease

1

1

10 years

M

20/150

Not reported

Yes

PPV + Autologous plasmin enzyme injection

PPV + ICG ILM peel + C3F8 tamponade

Macular hole closed

20/60

Park 2012

Idiopathic

Cavitary Maculopathy

1

1

8 years

F

20/40

Not reported

Yes

PPV + ILM peel + C3F8

Post operative prone position 2 weeks

Macular hole closed

20/40

Fernandez 2013

Accidental Nd:YAG laser

1

1

11 years

M

20/100

1077 μm

Yes

PPV + ILM Peel + C3F8

Post operative prone position 1 week

Macular hole closed

20/25

Seth 2015

Bartonella Neuroretinitis

1

1

11 years

F

CF 1 foot

Not reported

No

Not reported

Not reported

Bansal 2017

Choroidal Coloboma

1

1

10 years

F

20/60

Not reported

Not reported

Not reported

Not reported

BCVA best corrected visual acuity, PPV pars plana vitrectomy, FAX fluid air exchange, ILM internal limiting membrane, CF count fingers, NLP no light perception, ICG indocyanine green, C3F8 Perfluoropropane, SF6 sulfur hexafluoride

Macular surgery in the pediatric population has unique management and technical challenges. As previously mentioned, it may be difficult to accurately and reliably date how long the macular hole has been present. This poses a challenge when attempting to predict the presence of any contributory amblyopia to the presenting vision, and the potential visual benefit that can be expected from surgery. Induction of a PVD is difficult in children, and use of triamcinolone as in our case, may augment visualization of the posterior hyaloid. Fortunately for this patient, induction of the PVD was similar to that in an adult. Secondary complications including iatrogenic retinal tears and vitreous hemorrhage as a result of a young, adherent posterior hyaloid, in addition to late complications including vitrectomy induced cataract, should be considered carefully.

Finally, the ability to comply with post-operative care and practices including possible face down positioning are important in the pre-operative assessment of a child for macular surgery. Interestingly 3 cases specifically reported instructing their patient to position prone in the post-operative period [4, 13, 15]. These patients were of similar age to our patient, and also achieved macular hole closure.

Conclusion

This is the first reported case of an idiopathic FTMH in a child. Treatment with pars plana vitrectomy with peeling of the ILM resulted in significant anatomic and functional improvement.

Abbreviations

FTMH: 

full thickness macula hole

VA: 

visual acuity

OCT: 

optical coherence tomography

PVD: 

posterior vitreous detachment

ILM: 

internal limiting membrane

SF6: 

sulfur hexafluoride

ROP: 

retinopathy of prematurity

RPE: 

retinal pigment epithelium

Declarations

Authors’ contributions

JG reviewed the patient at first presentation. AH and GF performed surgery and post-operative care. LL and SL collated and prepared the patient data. LL was a major contributor in writing the manuscript. All authors read and approved the final manuscript.

Acknowledgements

None.

Competing interests

The authors declare that they have no competing interests.

Availability of data and materials

The data in the current case report are available from the Sydney Eye Hospital and Save Sight Institute medical records. The data is available from the corresponding author on reasonable request.

Consent for publication

Consent for publication was obtained.

Ethics approval and consent to participate

Ethics approval for this research was obtained (South Eastern Sydney Local Health District Human Research Ethics Committee, HREC Ref Number: 17/232 LNR/17/POWH/529) SSA Ref 18/G/019). Consent for participation was obtained.

Funding

Drs Fernandez and Levasseur received funding from the Sydney Eye Hospital Foundation.

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Authors’ Affiliations

(1)
Sydney Hospital and Sydney Eye Hospital, Macquarie Street, Sydney, NSW, Australia
(2)
Discipline of Ophthalmology, Sydney Eye Hospital Campus, University of Sydney, Macquarie Street, Sydney, NSW, Australia

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Copyright

© The Author(s) 2018

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