---
title: "Optic nerve hypoplasia (includes septo-optic dysplasia)"
description: "Optic nerve hypoplasia is underdevelopment of the optic nerve present from birth. When combined with midline brain abnormalities and pituitary dysfunction, it is termed septo-optic dysplasia. ## Features and diagnosis Severity ranges from mild visual reduction to profound impairment. -…"
url: "https://bluefinvision.com/knowledge-base/topics/conditions-diseases/optic-nerve-hypoplasia-includes-septo-optic-dysplasia/"
updated: "2025-12-16"
category: "Conditions & Diseases"
---

# Optic nerve hypoplasia (includes septo-optic dysplasia)

Optic nerve hypoplasia is underdevelopment of the optic nerve present from birth. When combined with midline brain abnormalities and pituitary dysfunction, it is termed septo-optic dysplasia.

## Features and diagnosis

Severity ranges from mild visual reduction to profound impairment.

- Small, pale optic discs often with a double-ring sign
- Nystagmus and reduced visual acuity in infancy
- Neuroimaging may show absent septum pellucidum or other midline defects
- Endocrine abnormalities such as growth hormone deficiency in septo-optic dysplasia

## Management

Care is multidisciplinary and long term.

- Regular ophthalmic monitoring and low-vision support
- Endocrine evaluation and hormone replacement where needed
- Early developmental and educational interventions
- Genetic counselling in selected cases