9B71.3

ICD-11 MMS 2026-01

Retinopathy of prematurity

Description

Retinopathy of prematurity is a vasoproliferative disorder that affects extremely premature infants potentially leading to severe visual impairment or blindness. Exposure of newborn premature infants to hyperoxia down regulates retinal vascular endothelial growth factor. Blood vessels constrict and can become obliterated, resulting in delays of normal retinal vascular development. Low birth weight, young gestational age, and severity of illness (e.g. respiratory distress syndrome, bronchopulmonary dysplasia, sepsis) are associated factors. It primarily occurs in extremely low birth weight infants because of the cessation of normal retinal vascular maturation.

Index terms

  • Retinopathy of prematurity
  • RLF- [retrolental fibroplasia]
  • ROP - [retinopathy of prematurity]
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  • Retrolental fibroplasia
  • Terry syndrome
  • retinopathy of prematurity NOS
  • Retinopathy of prematurity, Stage 1
  • Stage 1, Demarcation Line
  • Retinopathy of prematurity, Stage 2
  • Stage 2, Ridge
  • Retinopathy of prematurity, Stage 3
  • Stage 3, Extraretinal Fibrovascular Proliferation
  • Plus disease
  • Retinopathy of prematurity, Stage 4
  • Stage 4, Partial Retinal Detachment
  • Retinopathy of prematurity, Stage 5
  • Stage 5, Total Retinal Detachment
  • Retinopathy of prematurity, Zone 1
  • Retinopathy of prematurity, Posterior Zone 2
  • Retinopathy of prematurity, Zone 3

Inclusions

  • Retrolental fibroplasia

Exclusions from above levels

  • 01 – Certain infectious or parasitic diseases
  • 05 – Endocrine, nutritional or metabolic diseases
  • 18 – Complications of pregnancy, childbirth and the puerperium
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  • 19 – Certain conditions originating in the perinatal period
  • 22 – Injury, poisoning or certain other consequences of external causes
  • 8A21.0 – Posterior cortical atrophy

Sibling codes

  • 9B71.0 – Diabetic retinopathy
  • 9B71.1 – Hypertensive retinopathy
  • 9B71.2 – Radiation retinopathy
  • 9B71.4 – Paraneoplastic retinopathy
  • 9B71.5 – Autoimmune retinopathy
  • 9B71.Y – Other specified retinopathy
  • 9B71.Z – Retinopathy, unspecified

Foundation entity

Retinopathy of prematurity – http://id.who.int/icd/entity/947283385

Allowed postcoordination axes

Part of: 9B71: Retinopathy › 09: Diseases of the visual system