Abstract
The nevus sebaceous of Jadassohn (NSJ) syndrome is a not uncommon pediatric dermatosis, with malignant potential. It is the cutaneous manifestation of another phakomatosis, characterized by neurologic, ophthalmic, cardiovascular, skeletal, and urogenital involvement. The features of this syndrome overlap those of the oculo-auriculo-vertebral dysplasia of Goldenhar and tuberous sclerosis. The extent of system involvement suggests a developmental insult during the first few weeks of gestation. A clearly genetic basis has not been established. An infant with NSJ syndrome is described who had associated optic nerve hypoplasia. His clinical, pathologic, and radiologic findings, including computed tomography (CT) and magnetic resonance imaging, are presented.
| Original language | English (US) |
|---|---|
| Pages (from-to) | 1570-1576 |
| Number of pages | 7 |
| Journal | Ophthalmology |
| Volume | 94 |
| Issue number | 12 |
| State | Published - 1987 |
| Externally published | Yes |
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ASJC Scopus subject areas
- Ophthalmology
Cite this
Optic nerve hypoplasia and the syndrome of nevus sebaceous of Jadassohn : A new association. / Katz, Barrett; Wiley, C. A.; Lee, V. W.
In: Ophthalmology, Vol. 94, No. 12, 1987, p. 1570-1576.Research output: Contribution to journal › Article
}
TY - JOUR
T1 - Optic nerve hypoplasia and the syndrome of nevus sebaceous of Jadassohn
T2 - A new association
AU - Katz, Barrett
AU - Wiley, C. A.
AU - Lee, V. W.
PY - 1987
Y1 - 1987
N2 - The nevus sebaceous of Jadassohn (NSJ) syndrome is a not uncommon pediatric dermatosis, with malignant potential. It is the cutaneous manifestation of another phakomatosis, characterized by neurologic, ophthalmic, cardiovascular, skeletal, and urogenital involvement. The features of this syndrome overlap those of the oculo-auriculo-vertebral dysplasia of Goldenhar and tuberous sclerosis. The extent of system involvement suggests a developmental insult during the first few weeks of gestation. A clearly genetic basis has not been established. An infant with NSJ syndrome is described who had associated optic nerve hypoplasia. His clinical, pathologic, and radiologic findings, including computed tomography (CT) and magnetic resonance imaging, are presented.
AB - The nevus sebaceous of Jadassohn (NSJ) syndrome is a not uncommon pediatric dermatosis, with malignant potential. It is the cutaneous manifestation of another phakomatosis, characterized by neurologic, ophthalmic, cardiovascular, skeletal, and urogenital involvement. The features of this syndrome overlap those of the oculo-auriculo-vertebral dysplasia of Goldenhar and tuberous sclerosis. The extent of system involvement suggests a developmental insult during the first few weeks of gestation. A clearly genetic basis has not been established. An infant with NSJ syndrome is described who had associated optic nerve hypoplasia. His clinical, pathologic, and radiologic findings, including computed tomography (CT) and magnetic resonance imaging, are presented.
UR - http://www.scopus.com/inward/record.url?scp=0023492541&partnerID=8YFLogxK
UR - http://www.scopus.com/inward/citedby.url?scp=0023492541&partnerID=8YFLogxK
M3 - Article
C2 - 3431829
AN - SCOPUS:0023492541
VL - 94
SP - 1570
EP - 1576
JO - Ophthalmology
JF - Ophthalmology
SN - 0161-6420
IS - 12
ER -