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 Table of Contents    
Year : 2014  |  Volume : 7  |  Issue : 1  |  Page : 13-15  

Huge congenital teratoma containing tooth in a three-day-old neonate

1 Regional Institute of Ophthalmology, Kolkata, West Bengal, India
2 Department of Ophthalmology, North Bengal Medical College, Darjeeling, West Bengal, India
3 Department of Pediatrics, North Bengal Medical College, Darjeeling, West Bengal, India
4 Department of Pathology, Medical College, Kolkata, West Bengal, India

Date of Web Publication1-Mar-2014

Correspondence Address:
Krishna Pada Baidya
8A, Abinash Chandra Banerjee Lane, Beliaghata, Kolkata - 700 010, West Bengal
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/0974-620X.127912

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Congenital teratoma is a rare cause of congenital proptosis. A full term three-day-old male child was presented with massive unilateral swelling in the left orbit, which was noted since birth. Diagnosis of orbital teratoma without intracranial extension was made based on clinical examination and imaging. Lid-sparing exenteration of the left orbit was done. Histopathological examination confirmed the diagnosis of cystic teratoma. Congenital orbital teratoma with a tooth and mature elements of all the three germ cell layers is reported due to its extreme rarity.

Keywords: Congenital, orbit, proptosis, teratoma, tooth

How to cite this article:
Baidya KP, Ghosh S, Datta A, Mukhopadhyay S, Bhaduri G. Huge congenital teratoma containing tooth in a three-day-old neonate. Oman J Ophthalmol 2014;7:13-5

How to cite this URL:
Baidya KP, Ghosh S, Datta A, Mukhopadhyay S, Bhaduri G. Huge congenital teratoma containing tooth in a three-day-old neonate. Oman J Ophthalmol [serial online] 2014 [cited 2023 Jan 30];7:13-5. Available from: https://www.ojoonline.org/text.asp?2014/7/1/13/127912

   Introduction Top

Proptosis is very rare at birth. Congenital teratoma is a rare cause of proptosis. [1] Teratomas are true tumors composed of a variety of mature or immature tissues derived from the three germinal layers. Orbital teratoma usually grows rapidly. Orbital teratoma may have unusual presentation like recurrent orbital cellulitis. [2] Although rare, malignant teratoma can occur in orbit. [3] Orbital teratoma may endanger the eye as well as the bony walls and may extend intra-cranially. Congenital orbital teratomas are more common in females. [4] We report a case of massive congenital orbital teratoma in a three-day-old male child with complete loss of vision in the affected side.

   Case Report Top

A three-day-old child presented with massive unilateral swelling in the left orbit, which was present since birth. The full term male child was born by normal vaginal delivery after an uneventful pregnancy. Left-sided non-axial proptosis was noted. A reddish tense cystic mass measuring about 5 × 5 cm protruding through left palpebral fissure was observed. There was marked stretching of the eyelids over the mass. Transillumination of the mass was noted. Left nostril was obliterated due to the compression from the mass. Downward displacement of the left angle of mouth was noted [Figure 1]. The eye ball could not be separately recognized, and its position was recognized from the position of cornea which was seen displaced upwards and outwards. The mass was non-pulsatile, non-reducible. No bruit was present. The corneal diameter was 10 mm. Cornea was hazy. The right eye was normal. The child did not have any other systemic abnormality. Computerized tomography (CT) scan demonstrated cystic lesion with solid components including dental element occupying the left orbit. There was no intracranial extension. The globe was not separate from the cystic lesion. Expansion of bony orbit was noted [Figure 2]. Flash visually evoked potential (VEP) of the left eye generated no amplitude signifying non-salvageable vision. We planned to remove the mass and preserve the eye. However, during surgery the mass could not be separated from the wall of the eye ball and management planning was changed to a modified lid-sparing exenteration of the left orbit. Macroscopically the specimen was globular in shape, fleshy, with lobulated outer surface; compressed eyeball being incorporated in the mass at its upper outer quadrant. The hazy cornea and the sclera adjacent to cornea could be identified, but other part of the globe was inseparable from the main mass. Histopathological examination of the excised tissue showed a benign cystic teratoma composed of ectodermal, mesodermal, and endodermal elements. Stratified squamous epithelium, glial tissue, neuronal element, fibroadipose tissue, cartilage, and colonic mucosa were present. All the elements were mature [Figure 3]. The patient was followed-up for next one year without any evidence of recurrence [Figure 4]. The informed consent of patient's parents was obtained and institutional review board permitted the study.
Figure 1: Massive left-sided proptosis in a three-day-old child

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Figure 2: Computerized tomography scan of left orbit showing tooth (solid arrow) in a cystic cavity

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Figure 3: Photomicrograph of neoplasm involving eyeball (choroid shown by arrow pointing left) and composed of primitive neural element in the form of round cells in small sheets (thick arrow pointing right), glial cells, and fibers (arrow pointing upwards), well-formed gland with lumen lined by tall columnar cells (thin arrow pointing right) (Haematoxylene Eosin, ×40)

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Figure 4: One year follow-up of exenterated left orbit

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   Discussion Top

Our case was a mature teratoma in a very uncommon location like orbit. Teratomas are commonly found in ovary, testis, mediastinum, and sacrococcygeal area. They are true neoplasm originating from pleuripotent germ cells that undergo abnormal migration to abnormal locations of the body. The migrated germ cells acquire tumorogenic potential and grow into a teratoma. An important differential diagnosis was dermoid cyst, which is more common in orbit. Dermoid cysts are congenital tumors thought to arise in areas of ectoderm pinched off along bone suture lines. They are lined by non-keratinized stratified squamous epithelium and contain skin appendages. Gunalp I et al., studied 128 orbital cystic lesions of which only one case (0.8%) was teratoma, whereas the most common (29.7%) lesion was dermoid. [5] Malignant transformation of an orbital teratoma is rare. [3],[6]

Other differential diagnoses that have been considered were microphthalmos with cyst, neuroblastoma, encephalocele, lymphangioma, rhabdomyosarcoma, retinoblastoma, dermoid cyst, retrobulbar hemorrhage, and vascular malformation. Microphthalmos was excluded as cornea was of normal diameter. Neuroblastoma and rhabdomyosarcoma usually show evidence of bone destruction and do not present at birth. CT scan did not show intraocular mass lesion or calcification and hence retinoblastoma was excluded. Encephaloceles are usually located in the midline with an associated bony defect. Any history of birth trauma that might lead to retrobulbar hemorrhage was absent. Simple clinical tests including trans-illumination test along with CT findings helped us to provisionally diagnose the case as teratoma. CT scan plays important role in diagnosis as well as to find tumor extension. Calcification and fluid level are infrequent. In a study involving 160 cases of orbital teratoma in adults, 69% had the lesion that was most commonly located in the lateral aspect of the orbit, 85% having adjacent bone changes, 46% with lower CT attenuation than the water in the vitreous body, 14% with calcification, 5% with fluid levels, and 73% with a visible wall. [7] Fine needle aspiration cytology is not helpful in the diagnosis of teratoma. Definitive diagnosis can only be made by histopathological study of excised mass. [3] Generally the eye is normally developed in orbital teratoma, as was seen in histology of our case. Eyeball may be displaced or compressed due to rapid growth of the tumor leading to vision loss as the result of perforation, collapse, exposure keratopathy, and secondary optic atrophy. Though rapid enlargement is the usual presented, it may take a slower course. In our case corneal opacity interfered with fundus examination. However, zero amplitude flash VEP was suggestive of compressive optic neuropathy. The objective in the management is removal of the tumor as early as possible with preservation of the globe, even in a blind eye, to encourage orbito-facial development. Per-operatively, we had to change our plan of preserving the eye to a modified exenteration, for reason explained. Successful removal of massive congenital orbital teratoma by an eyelid-sparing exenteration technique has been reported in literature. [8] In this case, the mass contained a tooth, which is a rare finding in orbital teratoma. [2],[9]

Orbital exenteration is a disfiguring procedure. Reconstructive procedure for cosmetic rehabilitation depends on the extent of resected orbital tissue, nature of the lesion, and age of the patient. Temporalis muscle transposition, midline forehead flaps rotations, dermis fat graft, orbito-facial prosthesis, spontaneous granulation tissue, and rectus abdominis free flap with rib cartilage transplantation are options for extensive bony defect. We performed eyelid-sparing procedure and succeeded in preserving large amount of tarsal plate with bulbar conjunctiva. The temporalis muscle transfer with dermis fat graft is a useful technique for such benign lesion. But, we aborted this idea because of smallness of temporalis muscle size as patient was only three-day-old. Instead we allowed spontaneous tissue to grow inside the orbital cavity. Customized silicone-made orbital implant may be used in future.

We report this case of orbital teratoma, an unusual cause of proptosis in new born, present since birth with a tooth and mature elements of all the three germ cell layers, due to its extreme rarity.

   Acknowledgments Top

We acknowledge Dr. Anup Sadhu and Dr. Ruby Sen for their help in diagnosis of the case.

   References Top

1.Levin ML, Leone CR Jr, Kincaid MC. Congenital orbital teratomas. Am J Opthalmol 1986;102:476-81.  Back to cited text no. 1
2.Hassan HM, Mc Andrew PT, Yagan A, Jacques TS, Hayward R. Mature orbital teratoma presenting as a recurrent orbital cellulitis with an ectopic tooth and sphenoid malformation-a case report. Orbit 2008;27:309-12.  Back to cited text no. 2
3.Mahesh L, Krishnakumar S, Subramanian N, Babu K, Biswas J. Malignant teratoma of the orbit: A clinicopathological study of a case. Orbit 2003;22:305-9.  Back to cited text no. 3
4.Alkemade PP. Congenital teratoma of the orbit. Opthalmologica 1976;173:274-85.  Back to cited text no. 4
5.Günalp I, Gündüz K. Cystic lesions of the orbit. Int Ophthalmol 1996;20:273-7.  Back to cited text no. 5
6.Gardon JW, McManis JC. Congenital orbital intracranial teratoma with subsequent malignancy: Case report. Br J Ophthalmol 1986;70:111-3.  Back to cited text no. 6
7.Chawda SJ, Moseley IF. Computed tomography of orbital dermoids: A 20-year review. Clin Radiol 1999;54:821-5.  Back to cited text no. 7
8.Gnanaraj L, Skibell BC, Coret-Simon J, Halliday W, Forrest C, DeAngelis DD. Massive congenital orbital teratoma. Ophthal Plast Reconstr Surg 2005;21:445-7.  Back to cited text no. 8
9.Stan C, Szabo I, Breazu G. Orbital teratoma- tooth in the orbit. Case report. Oftalmologia 2002;52:34-7.  Back to cited text no. 9


  [Figure 1], [Figure 2], [Figure 3], [Figure 4]

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