Ophthalmic Surgery, Lasers and Imaging Retina

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Case Report 

Idiopathic Infantile Chronic Rhegmatogenous Retinal Detachment

Hassan A. Aziz, MD; Audina M. Berrocal, MD; Robert A. Sisk, MD; Timothy G. Murray, MD

Abstract

The authors describe a 3-month-old female infant with infantile rhegmatogenous retinal detachment with no associated congenital or developmental abnormalities. The unique presentation of this patient indicates that the detachment was a result of an early developmental defect.

Abstract

The authors describe a 3-month-old female infant with infantile rhegmatogenous retinal detachment with no associated congenital or developmental abnormalities. The unique presentation of this patient indicates that the detachment was a result of an early developmental defect.

Idiopathic Infantile Chronic Rhegmatogenous Retinal Detachment

From the Department of Ophthalmology, Bascom Palmer Eye Institute, University of Miami Miller School of Medicine, Miami, Florida.

The authors have no financial or proprietary interest in the materials presented herein.

Address correspondence to Hassan A. Aziz, MD, Bascom Palmer Eye Institute, 900 NW 17th Street, Miami, FL 33136. E-mail: haaziz@med.miami.edu

Received: August 01, 2009
Accepted: March 01, 2011
Posted Online: July 14, 2011

Introduction

Rhegmatogenous retinal detachment (RRD) is an uncommon pathology in the pediatric population, accounting for 3% to 7% of all patients with the disease.1 It differs from RRD in adults because the child’s visual system is still developing and achieving good visual outcomes poses a great challenge to the treating ophthalmologist.

The most common etiologies of RRD in the pediatric population include high myopia, trauma, developmental anomaly, previous intraocular surgery, and uveitis (Table 1). Surgical buckling and/or pars plana vitrectomy with the use of silicone oil in refractive cases are the mainstay in management of pediatric RRD. Factors contributing to poor visual outcomes include delayed diagnosis, poor vision preoperatively, non-traumatic etiologies, presence of proliferative vitreoretinopathy, macular involvement, and use of silicone oil.1–4

Predisposing Factors for Rhegmatogenous Retinal Detachment in the Pediatric Population

Table 1: Predisposing Factors for Rhegmatogenous Retinal Detachment in the Pediatric Population

In this unique case, we present a 3-month-old infant with an idiopathic chronic RRD likely secondary to retinal developmental abnormality.

Case Report

A 3-month-old female infant was referred to the Bascom Palmer Eye Institute with asymmetric ocular red reflex. The infant was the full-term product of an uncomplicated pregnancy and delivery. There was no significant medical or family history of eye disease or maldevelopment. No developmental or syndromic anomalies were noted. The patient’s right eye was unremarkable. The left eye was found to have an RRD secondary to a retinal hole adjacent to the superior ora serrata, resulting in a macula involving retinal detachment. Proliferative vitreoretinopathy with a demarcation line documented the chronicity of the detachment. The vitreous body was intact with no tractional component noted. Ultrasound examination noted a shallow total retinal detachment. Axial length was 17 mm (within normal limits).

The patient underwent surgical repair with scleral buckling (placed for 14 weeks) and cryotherapy that was later supplemented with 360° of laser photocoagulation at the time the buckle was removed (Figure). Three years later, her visual acuity was 20/30 in the right eye and 20/100 in the left eye, with an otherwise unremarkable examination.

(Left) Montage fundus photograph with retinal hole at 12°. (Right) Montage photograph showing chorioretinal scar at the site of the retinal hole (12°) and retinal indentation from the scleral buckle.

Figure. (Left) Montage fundus photograph with retinal hole at 12°. (Right) Montage photograph showing chorioretinal scar at the site of the retinal hole (12°) and retinal indentation from the scleral buckle.

Discussion

Discerning the etiology or associated risk factor in the development of pediatric RRD is of great importance. It is necessary to perform a thorough systemic evaluation to rule out developmental anomalies (Table 2) or trauma and a full ophthalmic examination with axial length measurement to rule out myopia and uveitis.

Causes and Incidence of Congenital or Developmental Abnormalities Leading to Rhegmatogenous Retinal Detachment

Table 2: Causes and Incidence of Congenital or Developmental Abnormalities Leading to Rhegmatogenous Retinal Detachment

This child’s unique presentation of a chronic RRD at 3 months of age with a normal axial length and no associated systemic disorder is unusual. The retinal hole is unlikely to be due to trauma for the following reasons: (1) the mother had an uneventful, vaginal, quick delivery of her second child; (2) the vitreous was intact over the hole and there was no evidence of hyaloidal separation or retinal traction; and (3) there was no reported history or signs of trauma.

To our knowledge, this is the first documented case of infantile chronic RRD presenting within the first 3 months of life. The normal phenotypic presentation of the patient and the lack of associated anomalies are suggestive that this may represent a developmental defect. Clinical documentation with wide-field imaging and serial evaluation was useful in documenting the pathology and response to surgical repair.

References

  1. Chang PY, Yang CM, Yang CH, et al. Clinical characteristics and surgical outcomes of pediatric rhegmatogenous retinal detachment in Taiwan. Am J Ophthalmol. 2005;139:1067–1072. doi:10.1016/j.ajo.2005.01.027 [CrossRef]
  2. Wang NK, Tsai CH, Chen YP, et al. Pediatric rhegmatogenous retinal detachment in East Asians. Ophthalmology. 2005;112:1890–1895. doi:10.1016/j.ophtha.2005.06.019 [CrossRef]
  3. Wadhwa N, Venkatesh P, Sampangi R, Garg S. Rhegmatogenous retinal detachments in children in India: clinical characteristics, risk factors, and surgical outcomes. J AAPOS. 2008;12:551–554. doi:10.1016/j.jaapos.2008.05.002 [CrossRef]
  4. Weinberg DV, Lyon AT, Greenwald MJ, Mets MB. Rhegmatogenous retinal detachments in children: risk factors and surgical outcomes. Ophthalmology. 2003;110:1708–1713. doi:10.1016/S0161-6420(03)00569-4 [CrossRef]

Predisposing Factors for Rhegmatogenous Retinal Detachment in the Pediatric Population

Factor% of Occurrence
Myopia79.1%
High myopiaa38%
Non-surgical trauma30.7%
Congenital or developmental abnormality16.6%
Previous intraocular surgery5.1%

Causes and Incidence of Congenital or Developmental Abnormalities Leading to Rhegmatogenous Retinal Detachment

CauseIncidence
Congenital cataract24.5%
Marfan syndrome20.4%
Cicatricial retinopathy of prematurity12.3%
Retinoschisis10.3%
Vitreoretinal degeneration6.2%
Coloboma4.1%
Macular degeneration4.1%
Familial exudative vitreoretinopathy4.1%
Stickler syndrome2.0%
Microphthalmos2.0%
Buphthalmos2.0%
Persistent fetal vasculature2.0%
Morning glory syndrome2.0%
Prader–Willi syndrome2.0%
Authors

From the Department of Ophthalmology, Bascom Palmer Eye Institute, University of Miami Miller School of Medicine, Miami, Florida.

The authors have no financial or proprietary interest in the materials presented herein.

Address correspondence to Hassan A. Aziz, MD, Bascom Palmer Eye Institute, 900 NW 17th Street, Miami, FL 33136. E-mail: haaziz@med.miami.edu

10.3928/15428877-20110707-02

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