Septo-Optic Dysplasia Complicated by Infantile Spasms and Bilateral Choroidal Fissure Arachnoid Cysts

Emir Tas MD

From the Division of Epilepsy and Clinical Neurophysiology, Department of Neurology, Children's Hospital Boston, Boston, MA (ET, MT, DPS, YZE, TL); Department of Pediatric Radiology, Division of Neuroradiology, Children's Hospital Boston, Boston, MA (SPP); and Department of Neurology, Harvard Medical School, Boston, MA (DPS, YZE, TL).

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Molly Tracy MD,

Molly Tracy MD

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Dean P. Sarco MD,

Dean P. Sarco MD

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Yaman Z. Eksioglu MD, PhD,

Yaman Z. Eksioglu MD, PhD

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Sanjay P. Prabhu MD,

Sanjay P. Prabhu MD

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Tobias Loddenkemper MD,

Tobias Loddenkemper MD

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Emir Tas MD,

Emir Tas MD

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Molly Tracy MD,

Molly Tracy MD

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Dean P. Sarco MD,

Dean P. Sarco MD

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Yaman Z. Eksioglu MD, PhD,

Yaman Z. Eksioglu MD, PhD

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Sanjay P. Prabhu MD,

Sanjay P. Prabhu MD

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Tobias Loddenkemper MD,

Tobias Loddenkemper MD

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First published: 28 December 2010

https://doi.org/10.1111/j.1552-6569.2009.00453.x

Citations: 12

Correspondence: Address correspondence to Tobias Loddenkemper, MD, Department of Neurology, Division of Epilepsy and Clinical Neurophysiology, Children's Hospital Boston, 300 Longwood Avenue, Fegan 9, Boston, 02115, MA. E-mail: [email protected]

J Neuroimaging 2011;21:89-91.

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Abstract

ABSTRACT

BACKGROUND AND PURPOSE

Septo-optic dysplasia (SOD) is the triad of optic nerve hypoplasia, panhypopituitarism, and agenesis of septum pellucidum, and has been described previously to be associated with heterotopias and midline interhemispheric cyst. We describe a case of SOD with arachnoid cysts, persistent primary hyperplastic vitreous, and malformations of cortical development.

METHODS

Case report and review of literature.

RESULTS

Our patient was found to have SOD, bilateral ventriculomegaly, pachygyria, gray matter heterotopia, bilateral choroidal cysts near the brainstem, and persistent primary hyperplastic vitreous. She later developed infantile spasms and required enucleation of the abnormal eye and cyst fenestration.

CONCLUSION

Coincidence of seizures, SOD, bilateral choroid fissure cysts, heterotopias, and persistent primary hyperplastic vitreous is a unique constellation. It is unclear whether this represents a new syndrome or SOD spectrum variation. Patients with SOD and arachnoid cysts should be monitored for signs of herniation.

References

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Volume21, Issue1

January 2011

Pages 89-91

Septo-Optic Dysplasia Complicated by Infantile Spasms and Bilateral Choroidal Fissure Arachnoid Cysts

Abstract

ABSTRACT

BACKGROUND AND PURPOSE

METHODS

RESULTS

CONCLUSION

References

Citing Literature

References

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Septo-Optic Dysplasia Complicated by Infantile Spasms and Bilateral Choroidal Fissure Arachnoid Cysts

Abstract

ABSTRACT

BACKGROUND AND PURPOSE

METHODS

RESULTS

CONCLUSION

References

Citing Literature

References

Related

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