13.10  Paediatric Retinal Detachment

Pediatric retinal detachments are complex in part due to their characteristics. When compared to adults, they are more likely to present with worse initial visual acuity, chronic duration and the macula detached.[1] Proliferative vitreoretinopathy (PVR) is more common, reported to be present in 20-60% of cases, partially due to chronicity as well as increased levels of inflammatory mediators. Various predisposing factors to these retinal detachments that must be elucidated with history, imaging and testing (Figure 13.10.1).

Read SP, Aziz HA, Kuriyan A, et al. RETINAL DETACHMENT SURGERY IN A PEDIATRIC POPULATION: Visual and Anatomic Outcomes. Retina. 2018;38(7):1393-1402.

Common Conditions to Consider

  1. Rhegmatogenous Retinal Detachment
    1. Trauma
      1. External trauma/accident
      2. Self-induced
      3. Self-injurious
      4. Rubbing
    2. Pathologic myopia
      1. Stickler Syndrome
      2. Marfan Syndrome
      3. Wagner Syndrome
      4. Knobloch Syndrome
    3. X-linked Retinoschisis
    4. History of prematurity
      1. Treated Retinopathy of Prematurity (ROP)
      2. Spontaneously regressed ROP
  2. Traction Retinal Detachment
    1. Retinopathy of Prematurity
    2. Familial Exudative Vitreoretinopathy (FEVR)
    3. XLRS
    4. Persistent Fetal Vasculature
    5. Toxocara
    6. Norrie’s Disease
  3. Exudative Retinal Detachment
    1. Coats disease
    2. Retinoblastoma

Preoperative Planning and Diagnostics

  1. Pregnancy and birth history
  2. Genetics referral in the event of abnormal facies or body habitus where genetic disease is suspected
    1. TFamily History
    2. Retinal Detachments
    3. Autoimmune disorders
    4. Joint disorders including hyperflexibility and early onset arthritis
    5. Extensible skin
    6. Pets
  3. Ancillary testing for documentation, diagnosis and follow up
    1. TFundus photography
    2. Fluorescein Angiography
    3. OCT or OCT-A
    4. B-Scan Ultrasound
    5. A-Scan Ultrasound
    6. Anterior segment ultrasonography
  4. Examine family members if necessary
  5. Examination under anesthesia
    1. TOften needed for appropriate diagnosis and management

Surgery

Pre-Incision

Retinal detachment repair is performed with general anesthesia monitored ideally by a pediatric anesthesiologist. Ancillary testing of both eyes as well as blood samples for genetic testing is facilitated at this time. In specific cases where repeated general anesthesia may be undesirable due to social challenges, or systemic morbidity, immediate bilateral surgery is an option.[2]

Yonekawa Y, Wu WC, Kusaka S, et al. Immediate Sequential Bilateral Pediatric Vitreoretinal Surgery: An International Multicenter Study. Ophthalmology. 2016;123(8):1802-1808.

Wound Placement

Anatomical considerations of surgical landmarks must be taken into account. Exposure to the eye is of utmost importance, lateral canthotomy might be necessary for exposure. The sclerotomies are placed according to age and pathology (Table 13.10.1). Pediatric sclera tends to be very tough and trocar entry may be facilitated by holding adjacent sclera or Tenon’s capsule and providing counter traction. If there is a need to remove the lens, or there is concern for iatrogenic retinal breaks with pars plana or pars plicata entry, limbal approach is preferred. Of note, a short trocar system is available from DORC and is especially useful in baby eyes.

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