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Related Experiment Video

Updated: Oct 29, 2025

Author Spotlight: Anterior HR-OCT as a Non-Invasive Tool for Characterizing Ocular Surface Squamous Neoplasia

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Septo-optic dysplasia.

Ieva Sataite¹, Simon Cudlip¹, Jayaratnam Jayamohan¹

¹Department of Neurosurgery, Oxford University Hospitals NHS Foundation Trust, Oxford, United Kingdom.

Handbook of Clinical Neurology

|July 9, 2021

Summary

Septo-optic dysplasia (SOD), a rare congenital disorder, involves optic nerve hypoplasia, absent septum pellucidum/corpus callosum, and pituitary issues. Management focuses on a multidisciplinary approach to improve symptoms, as SOD is not curable.

Keywords:

Achiasmia Agenesis of septum pellucidum Hydrocephalus Hypopituitarism Optic nerve hypoplasia Septo-optic dysplasia de Morsier's syndrome

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Area of Science:

Pediatric Endocrinology
Neuro-ophthalmology
Genetics

Background:

Septo-optic dysplasia (SOD), also known as de Morsier's syndrome, is a rare congenital disorder.
It is characterized by a triad of optic nerve hypoplasia, agenesis of the septum pellucidum and corpus callosum, and hypothalamic-pituitary axis hypoplasia.

Purpose of the Study:

To provide comprehensive information on the etiology, epidemiology, diagnosis, and management of Septo-optic dysplasia.
To highlight the importance of a multidisciplinary approach in managing SOD, despite its incurable nature.

Main Methods:

Review of existing literature on Septo-optic dysplasia.
Discussion of diagnostic criteria and differential diagnoses.
Outline of treatment strategies including hormonal replacement, surgical interventions, and supportive therapies.

Main Results:

SOD presents with a distinct set of clinical features affecting vision, brain structure, and endocrine function.
Early diagnosis and intervention are crucial for optimal patient outcomes.
A tailored, multidisciplinary approach is essential for managing the complexities of SOD.

Conclusions:

Septo-optic dysplasia requires a comprehensive management plan involving various medical specialties.
While SOD is not curable, significant improvements in quality of life can be achieved through targeted treatments.
Hormonal replacement, ophthalmological surgery, seizure management, and neuropsychological support are key components of care.