|
Goldenhar's syndrome: case report.East
Afr Med J. 2002 Sep;79(9):502-4.
A case of
Goldenhar's Syndrome (oculoauriculovertebral dysplasia) in a
3-day-old Nigerian neonate with right anophthalmos and lipodermoid,
left limbal dermoid, bilateral preauricular appendages and
mandibular hypoplasia is presented. The lipodermoid was attached to
the tarsal conjunctiva of the lower lid of the anophthalmic side.
The causative factor was presumably maternal drug (traditional
medicine) ingestion at three months gestation. No similar case has
been reported previously. The literature on Goldenhar's Syndrome is
briefly reviewed.
A caruncular
dermoid with contiguous eyelid involvement: embryologic
implications.
Ophthal Plast Reconstr Surg.
1998 Sep;14(5):375-7.
An
11-year-old girl with a mass arising from the caruncle and the upper
eyelid was examined for spontaneous epibulbar bleeding. Excision of
the lesion involved reconstruction of the proximal nasolacrimal
system, lid margin, and conjunctiva. The histopathologic diagnosis
was consistent with a dermoid of the caruncle. This case is notable
not only for a dermoid accompanied by epibulbar bleeding but for its
caruncular origin and attachment to the upper eyelid. This case
suggests that the caruncle and the upper eyelid arise from similar
embryologic structures.
Epibulbar choristomas containing lacrimal tissue. Clinical
distinction from dermoids and histologic evidence of an origin from
the palpebral lobe.
Ophthalmology. 1987
Oct;94(10):1249-57.
Three new
cases of epibulbar choristomas, all containing lacrimal tissue, are
presented and compared with those reported previously in the
literature. The first and second cases were unilateral, complex
choristomas, consisting of ectopic lacrimal tissue, smooth muscle,
and cartilage; the first case was also remarkable for the presence
of lesional tissue in the peripheral cornea and evidence of
progressive growth during puberty. The third case was a bilateral,
complex choristoma, manifesting epibulbar lacrimal tissue,
cartilage, and smooth muscle; also associated were bilateral optic
nervehead colobomas and a uniocular focus of posterior scleral
cartilage demonstrated by computed tomography (CT) scanning. On
clinical examination, all three lesions displayed highly
vascularized thickenings of the conjunctiva with diagnostically
useful gelatinous elevations, which corresponded microscopically to
lobules of lacrimal tissue. In addition, all three cases had the
interesting diagnostic feature of superficial corneal scarring or
sclerosis, with a fine vascularity adjacent to the conjunctival
lesional tissue. Electron microscopy of the third case demonstrated
completely normal cytoarchitecture of the lacrimal tissue. Because
of the frequent presence of Müller's smooth muscle in epibulbar
lacrimal choristomas, the authors propose that these lesions
represent embryologic ectopias or overly extensive field effects of
the palpebral lobe of the lacrimal gland.
Transconjunctival resection of an orbital dermoid tumour.J
Maxillofac Surg. 1985 Oct;13(5):239-42.
An unusual
case of an orbital dermoid tumour containing a well differentiated
tooth-like structure is documented. A previously unreported surgical
approach to a maxillofacial dermoid tumour is introduced. Using a
conjunctival incision, orbital floor exploration was undertaken with
excellent visibility and access. A large dermoid tumour was removed
without the need for an external skin incision. The surgical
approach and pathology of this unusual lesion are reviewed.
Ring dermoid
syndrome. A new syndrome of autosomal dominantly inherited,
bilateral, annular limbal dermoids with corneal and conjunctival
extension.Arch
Ophthalmol. 1980 Jun;98(6):1059-61.
A new
hereditary syndrome of bilateral ocular dermoids is described in
five patients from three generations of a single family. There were
no associated extraocular anomalies. The choristomas involved the
limbus for 360 degrees, extended anteriorly onto the cornea, and
extended posteriorly about 5 mm within the conjunctiva for 360
degrees. Additional clinical findings of diagnostic value were
conjunctival plaques of keratinization, hairs, and corneal lipid
deposition. An irregular corneal astigmatism, amblyopia, and
concomitant strabismus were secondary features. The unique
bilateral, annular configuration of the dermoids is pathognomonic of
this syndrome. Early surgical intervention is indicated to try to
improve the visual prognosis and cosmesis. At surgery, the
conjunctival portion of one case was easily resected in toto, but
the limbal portion required partial excision. Histopathological
examination confirmed the clinical diagnosis of dermoid choristoma.
|