Unilateral progressive proptosis in neonate.
ABSTRACT Orbital teratomas are congenital germ cell tumors that present at birth or within the first few months of life with unilateral proptosis. Primary orbital teratomas produce diffuse enlargement of the orbit. Orbital teratoma contains a wide diversity of tissues ranging from undifferentiated mass of tissue to a fully differentiated fetus in fetus. The tumor components are derived from two or more germinal layers. Most teratomas are cytnlogically benign, having both cystic and solid areas and on occasions may undergo a malignant change. Most patients in the past have been treated by orbital exenteration. We report a case of orbital teratoma that rapidly progressed over a course of days.
UNILATERAL PROGRESSIVE PROPTOSIS IN NEONATE
Gilbert Paragache1, Naresh K Panda2, Kusum Joshi3
Key Words :Teratoma, proptosis, exenteration, orbit.
The word "teratoma" means "monstrous growth" in
Greek. Teratomas are rare congenital tumors. They
originate in totipotential cells and are now considered to
be choristomas rather than true neoplasms.
An one month old male child had presented to Nehru
Hospital, Postgraduate Institute for Medial Education and
Research, Chandigarh India, with rapidly progressive
proptosis of right eye since birth. The prenatal period was
uneventful and the baby weighed 3.2 Kg at birth.
Physical examination revealed marked proptosis in the right
eye. The eyeball was embedded within the mass that could
be seen all around the globe. The cornea showed exposure
keratitic changes and was opacified (Fig.I).
The mass had a cystic consistency. Transillumination
was present in the swelling. Ocular mobility was markedly
restricted in all direction, without any vision. The bony
orbit was enlarged. Left eye was normal. Laboratory
studies showed an elevated Serum Alpha Fetoprotein
Radiology showed a large rounded mass occupying the
right orbit with heterogenous attenuation and stippled
calcification. Expansion of the bony orbital wall was seen
without obvious intracranial extension. Various
components of tumor like fat, fluid, and calcifications could
be confirmed. The extraocular muscles and optic nerve
could not be identified (Fig.II).
Presence of frozen globe without any vision prompted us
to do a complete orbital exenteration. The mass was well
Fig. I : Clinical photograph of the patient showing massive proptosis
with globe embedded within the mass and opacified cornea
Fig. II : T1 weighted MRI image coronal view showing the mass
occupying the right orbit with cystic and solid areas.
1,2 Department of Otolaryngology, Head, and Neck Surgery, 3Department of Pathology, Post Graduate Institute for Medical Education
and Research, Chandigarh, India.
Unilateral Progressive Proptosis in Neonate
Fig.IV : High power view showing nests of squamous epithelium adjacent
to mature glial component. (H&E X480).
encapsulated. The bony orbital wall was expanded. The
Optic foramen and superior orbital fissures were normal.
Gross examination showed a globular, 6 x 5 x 4 cms mass
attached to the posterior surface of the globe. Eyeball was
small and shrunken (Fig. III). Cut section revealed pale
yellow thinly encapsulated tumor that was greasy and soft
in consistency. It had few gray white areas, resembling
tooth with hair shafts, gelatinous, cartilaginous and bony
Microscopy revealed tumor components to be consistent
with a mature teratoma, with derivatives from all three
germinal layers. The tumor was largely lipomatous but
had cystic spaces containing keratin flakes lined by
stratified squamous epithelium and dermal appendages.
Areas with glandular, cartilaginous, bony and neuroglial
differentiation were seen. All components were mature
and there were no dedifferentiated areas. The eyeball was
distorted and free of tumor (Fig. IV).
The first case of orbital teratoma was reported by Holmes1
in 1863. Hoyt and Joe2 had reviewed 30 cases from the
literature. Barber et al3 in 1974 reviewed the literature and
compiled a total of 54 cases.
Congenital orbital teratoma is an extremely rare tumor of
the orbit that may be considered in the diagnosis when a
newborn presents with unilateral proptosis. The other
differential diagnoses must include micro opthalmos with
cyst, congenital cystic eyeball, unilateral congenital
glaucoma, dermoid cyst of the orbit, neuroblastoma,
neurofibroma, nasofrontal or sphenoidal meningocele,
orbital haematoma, hemangioma and lymphangioma. A
diagonosis of primary orbital teratoma was considered
due to the presence of unilateral proptosis, expansion of
the orbit, fluctuation and trans-illumination in the mass.
Rapid growth potential and risk for malignant
transformation4 was the reason for a radical approach in
treatment in the past. Instances of preservation of
proptotic eye, either for possible vision or to act as a
'growing orbital implant' to achieve facial and orbital
symmetry has been seen in the literature.5 Our case had
pronounced proptosis with a frozen globe and absent
vision. Hence, we opted to treat the patient by orbital
exenteration. Prognosis of such tumors is extremely good
at present if preoperative diagnosis is correct.
1. Holmes, T. (1863) : Congenital tumor removed from the orbit.
Trans. Pathol. Soc. London. 14: 248.
2. Hoyt WF, Joe S. (1961) : Congenital Teratoid Cyst. Arch
3. Barber JC, Bareber LF, Dupont G et al. (1974) : Congenital
orbital teratoma. Arch, opthalmol. 91: 45-48.
4. Saradarian AU. (1947) : Malignant teratoma of the orbit. Six
and one half years observation. Arch, opthalmol. 37: 253 -
5. Jones ST. (1963) : Dermoid and teratoid tumors of the eye and
orbit with a case of the orbit. J Arkansas Med Soc. 60 : 66 -73.
Address for correspondence:
Dr. Naresh K. Panda
Additional Professor and Head
Department of Otolaryngology, Head, and Neck Surgery
PGIMER, Chandigarh, India.
Indian Journal of Otolaryngology and Head and Neck Surgery Vol. 56 No. 1, January - March 2004
Fig.III : Gross photograph showing the compressed eyeball and a pale
gray white, partly cystic mass in the retro orbital space.