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Neurology · Septo-Optic Dysplasia

Understanding Septo-Optic Dysplasia (SOD)

At a Glance

Septo-Optic Dysplasia (SOD) is a rare, non-progressive condition involving underdeveloped optic nerves, pituitary hormone deficiencies, and midline brain differences. While brain structure won't worsen over time, evolving hormone needs and development can be effectively managed with a medical team.

Receiving a diagnosis of Septo-Optic Dysplasia (SOD) can feel overwhelming, but it is important to remember that this diagnosis is a starting point, not a final destination. SOD is a rare condition that occurs during early brain development [1][2]. While the name may sound complex, understanding the basics of the condition can help you feel more empowered as you navigate your child’s care.

What is Septo-Optic Dysplasia?

Septo-Optic Dysplasia, also known as de Morsier syndrome, is a congenital (present at birth) condition involving the development of the brain’s midline structures [1][3]. Because it is a “spectrum disorder,” it affects every child differently [4][5]. Some children may have very mild symptoms, while others require more intensive medical support [6].

Doctors typically diagnose SOD when a child has at least two of the following three features, known as the classic triad. You can learn more about these in The Three Pillars: Symptoms and the SOD Triad:

  1. Optic Nerve Hypoplasia (ONH): The optic nerves (which carry visual information from the eyes to the brain) are smaller than usual, which can affect vision [1].
  2. Pituitary Hormone Abnormalities: The pituitary gland (the “master gland” at the base of the brain) may not produce enough of certain hormones, such as growth hormone or thyroid-stimulating hormone [7].
  3. Midline Brain Defects: Structural differences in the center of the brain, most commonly the absence of the septum pellucidum (a thin membrane in the middle of the brain) [2].

It is important to note that many children do not have all three features; in fact, only about 38% of patients present with the full triad [8].

Understanding the SOD Spectrum

The most important thing to know is that SOD is a non-progressive condition [9]. This means that the structural brain differences and the underdevelopment of the optic nerves are fixed—they will not get worse over time [10].

However, because the body grows and changes, your child’s medical needs may evolve. For example:

Orienting Facts for Parents

While the diagnosis is a lot to process, these facts can help ground you as you move forward:

By focusing on your child’s specific needs rather than the label of the diagnosis, you can provide them with the specialized care they need to thrive.

Common questions in this guide

What is the classic triad of Septo-Optic Dysplasia?
The classic triad includes optic nerve hypoplasia, pituitary hormone abnormalities, and midline brain defects. A child typically needs at least two of these three features to receive an SOD diagnosis.
Will my child's Septo-Optic Dysplasia get worse over time?
No. SOD is a non-progressive condition, meaning the structural brain differences and underdeveloped optic nerves will not worsen. However, as your child grows, their hormone levels and developmental needs may change and require ongoing monitoring.
Did I do something during pregnancy to cause my child's SOD?
No, there is nothing you did or didn't do to cause this condition. SOD occurs very early in pregnancy during brain development and is not your fault.
How is Septo-Optic Dysplasia treated?
While the structural differences in the brain cannot be reversed, many symptoms are highly treatable. For example, pituitary hormone deficiencies can be effectively managed with specialized medications prescribed by an endocrinologist.
Which specialists should be on my child's SOD care team?
Because SOD affects multiple systems, your child will likely need a multidisciplinary team. The core care team typically includes a neurologist for brain development, an endocrinologist to monitor hormones, and an ophthalmologist to track vision.

Questions for Your Doctor

5 questions

  • Based on the MRI results, which specific midline brain structures are affected in my child?
  • Has a full pituitary hormone panel been completed to screen for deficiencies like growth hormone or adrenal insufficiency?
  • What degree of optic nerve hypoplasia was found, and how might this affect my child's visual development?
  • How often will we need to repeat hormone testing as my child grows?
  • Which specialists (endocrinology, ophthalmology, neurology) should be part of my child's core care team?

Questions for You

4 questions

  • What symptoms or signs first led us to seek a medical evaluation (e.g., eye movements, growth concerns, low blood sugar)?
  • What are my child's current strengths and developmental milestones?
  • Do I have a clear system for tracking my child's appointments, test results, and specialist recommendations?
  • What are my biggest fears right now, and what information would help me feel more prepared to manage my child's care?

References

References (12)
  1. 1

    Septo-optic dysplasia plus diagnosed in adulthood.

    Infante-Valenzuela A, Camara-Lemarroy CR, Reyes-Mondragon AL, et al.

    Neurological sciences : official journal of the Italian Neurological Society and of the Italian Society of Clinical Neurophysiology 2017; (38(9)):1705-1707 doi:10.1007/s10072-017-2985-7.

    PMID: 28474147
  2. 2

    Prenatal diagnosis of isolated agenesis of the septum pellucidum with ultrasound and magnetic resonance imaging

    Nemcsik-Bencze Z, Várbíró S, Rudas G, Nemcsik J

    Orvosi hetilap 2020; (161(52)):2195-2200 doi:10.1556/650.2020.31912.

    PMID: 33361505
  3. 3

    Septo-optic dysplasia associated with chromosome 15q13.3 duplication: a case report.

    Ham JA, Kim SH, Park D

    Journal of Yeungnam medical science 2023; (40(4)):419-422 doi:10.12701/jyms.2022.00493.

    PMID: 36458369
  4. 4

    The clinical aspects of septo-optic dysplasia: A narrative review with illustrative case report.

    Al-Salihi MM, Qassim T, Aji N, et al.

    International journal of surgery case reports 2023; (109()):108575 doi:10.1016/j.ijscr.2023.108575.

    PMID: 37524018
  5. 5

    Clinical and Radiologic Spectrum of Septo-optic Dysplasia: Review of 17 Cases.

    Alt C, Shevell MI, Poulin C, et al.

    Journal of child neurology 2017; (32(9)):797-803 doi:10.1177/0883073817707300.

    PMID: 28482731
  6. 6

    Review of the MRI brain findings of septo-optic dysplasia.

    Ward DJ, Connolly DJA, Griffiths PD

    Clinical radiology 2021; (76(2)):160.e1-160.e14 doi:10.1016/j.crad.2020.09.007.

    PMID: 33019967
  7. 7

    Endocrine Dysfunction in Children with Zika-Related Microcephaly Who Were Born during the 2015 Epidemic in the State of Pernambuco, Brazil.

    Veras Gonçalves A, Miranda-Filho DB, Rocha Vilela LC, et al.

    Viruses 2020; (13(1)) doi:10.3390/v13010001.

    PMID: 33374895
  8. 8

    Clinical characteristics of septo-optic dysplasia accompanied by congenital central hypothyroidism in Japan.

    Nagasaki K, Kubota T, Kobayashi H, et al.

    Clinical pediatric endocrinology : case reports and clinical investigations : official journal of the Japanese Society for Pediatric Endocrinology 2017; (26(4)):207-213 doi:10.1297/cpe.26.207.

    PMID: 29026269
  9. 9

    Visual Acuity Outcomes in Children With Optic Nerve Hypoplasia and Septo-Optic-Pituitary Dysplasia.

    Salman MS, Hossain S, Carson E, et al.

    Pediatric neurology 2023; (149()):167-175 doi:10.1016/j.pediatrneurol.2023.09.018.

    PMID: 38557645
  10. 10

    Patients with septo-optic dysplasia: General ophthalmologic assessment and retinal imaging.

    Eibenberger K, Rezar-Dreindl S, Briem J, et al.

    European journal of ophthalmology 2023; (33(5)):NP11-NP20 doi:10.1177/11206721221128865.

    PMID: 36163692
  11. 11

    Recurrent Hypoglycaemia Leading to Early Diagnosis of Septo-Optic Dysplasia in a Small-for-Gestational-Age Infant-A Case Report.

    Tan YRL, Dong X, Lek N, et al.

    Clinical case reports 2026; (14(2)):e71985 doi:10.1002/ccr3.71985.

    PMID: 41674888
  12. 12

    The central diabetes insipidus associated with septo-optic dysplasia (de Morsier syndrome).

    Hetman M, Fułek M, Zajączkowska K, et al.

    Pediatric endocrinology, diabetes, and metabolism 2018; (24(4)):197-203 doi:10.5114/pedm.2018.83367.

    PMID: 30963758

This page provides a general overview of Septo-Optic Dysplasia for educational purposes. Always consult your child's pediatric neurologist or endocrinologist for specific medical advice.

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