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September 20-22, 2017 | Toronto, Canada
10
TH
AMERICAN PEDIATRICS HEALTHCARE &
PEDIATRIC INFECTIOUS DISEASES CONGRESS
Pediatric Healthcare & Pediatric Infections 2017
Introduction:
Septo-optic dysplasia (SOD) or De Morsier’s syndrome is
diagnosedwhenopticnervehypoplasiaisseeninconjunctionwithdysgenesis
of the septum pellucidum. Its diagnosis is mainly neuroradiologic with an
incidence of 1:50,000. This paper presents four cases of SOD diagnosed via
magnetic resonance imaging in less than a year in our institution. The cases
represent the classic SOD as well as an unusual blend of the neuroradiologic
features of the different SOD subsets with unique associations (aqueductal
stenosis and posterior cervical myelomeningocoele).
Case Presentation:
1) An 18-month Filipino female with seizure,
developmental delay and hydrocephalus revealed an absent septum
pellucidum and corpus callosum, small optic nerves, dilated ventricles,
aqueductal stenosis, grey matter heterotopia and a posterior cervical
myelomeningocoele; 2) A 15-year old male with recurrent seizure showed
dysplastic optic nerves, dilated ventricles with box-shaped frontal horns,
absent septum pellucidum and a thinned-out corpus callosum; 3) A
17-month old male with blurred vision demonstrated cerebral atrophy,
open-lip schizencephaly, absent septum pellucidum with dilated ventricles
and atrophic optic nerves; 4) A three-week old male with seizures since
birth showed absent septum pellucidum with box-like appearance of the
frontal horns of the lateral ventricles.
Conclusion:
Coincidence of seizures, developmental delay, calloso-septo-
optic-dysplasia plus, aqueductal stenosis, hydrocephalus and cervical
myelomeningocoele is a unique constellation of the neuroradiologic
features of the different subsets of SOD with indefinite prognosis. Patients
with classic SOD or calloso-septo-optic dysplasia plus with rare associations
should both be closely followed up for re-assessment, further evaluation
and management of neurologic and non-neurologic deficits.
Speaker Biography
Joy A David has completed her Doctor of Medicine from the University of Santo Tomas,
Philippines. She has worked in the field of Public and Community Health for nine years
before she entered Residency Training in Radiology. She is in her second year of training
at Bicol Medical Center (BMC) Naga City, Camarines Sur, Philippines and is currently the
Chief Resident of the BMC Department of Radiology. She has presented her case paper
in both local and international gathering/congress. She is also a Certified Women and
Child Protection Specialist.
e:
queendavid30@ymail.comCalloso-septo-optic dysplasia-plus (De Morsier’s syndrome) with aqueductal stenosis and posterior
cervical myelomeningocoele: Magnetic resonance imaging: A case report/series of De Morsier’s
syndrome
Joy A David and Mahgn R Collao
Bicol Medical Center, Philippines