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| REVIEW ARTICLE |
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| Year : 1989 | Volume
: 37
| Issue : 4 | Page : 166-170 |
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Uniocular proptosis in children
A Marthanda Pillai, M Sambasivan
Prof. of Neuro surgery, Medical College Hospital, Trivandrum - 695 011, India
Correspondence Address:
A Marthanda Pillai
Prof. of Neuro surgery, Medical College Hospital, Trivandrum - 695 011
India
 Source of Support: None, Conflict of Interest: None  | Check |
PMID: 2638302 
A retrospective study on 80 children with uniocular proptosis has been done. Of these 57 had orbital lesions, 19 had periorbital lesions and 4 had intracranial lesions. The clinical, radiological and pathological aspects as well as the management of these conditions are discussed.
How to cite this article:
Pillai A M, Sambasivan M. Uniocular proptosis in children. Indian J Ophthalmol 1989;37:166-70 |
| Introduction | |  |
A hetrogeneous group of conditions present with uniocular proptosis, the management of which fall in the realm of the Neurosurgeon, Ophthalmologist, ENT Surgeon and others. Some of the cases are so rare that few Neurosurgeons see enough cases to acquire sufficient knowledge and experience to undertake their management with confidence. Clinically, the situation is worsened by the fact that many varieties of lesions both malignant and non malignant produce similar signs and symptoms.
| Material and methods | | |
80 children with uniocular proptosis were treated in our institution since 1966.
| Investigations | | |
As a routine, X-ray of the skull, orbit, paranasal sinuses, optic foramina and chest were done. A complete Haemogram was done. Bone marrow examination was done as appropriate. Orbital venography used to be the main investigative procedure before the introduction of C. T. Scan. Recently C. T. Scan has been employed routinely. Cartoid Angiography was done in two patients who had orbital varix to exclude intracranial arteriovenous malformation. In a case of angiofibroma of the nasopharynx with proptosis selective external cartoid angiogram was done which showed the tumour in the nasopharynx and orbit.
| Management | | |
Surgical management consisted of Naffzigers operation in 6 cases, anterior orbitotomy in 2 cases, lateral orbitotomy in 26 cases, exenteration in 5 cases and temporal craniotomy in 2 cases. 32 cases had biopsy and radiotherapy and or chemotherapy. 4 cases of psuedotumour were treated with steroids without histopathology on the basis of C. T. Scan appearence. The details of the management are given in [Table - 1].
| Results | | |
The patients with uniocular proptosis could be broadly divided into three groups. Orbital lesions were present in 57 cases, periorbital lesions in 19 cases and intracranial lesions in 4 cases. The type of lesions in each group is given in [Table - 2]. The age of the children ranged from 3 months to 14 years. 26 patients were between 5 years and 8 years and 37 patients between 9 years and 14 years. Of these 47 were males and 33 females.
Apart from proptosis, visual disturbance (54 cases) diplopia (40 cases) and pain (10 cases) were the most important symptoms. In general, lesions of the muscle cone, optic nerve and orbital apex pushed the globe forward along the axis of the orbit, ocular mobility as well as acuity of vision was also affected early, whereas large extraorbital tumours such as fibrous dysplasia and tumours in the anterior part of the orbit tended to displace the globe away from the mass and the tumour mass could be palpated [1].
Chemois indicated severe increased pressure in the veins of the orbit, produced by venous obstruction, due to inflammation or orbital tumour. An early sign of orbital varix was the appearence of dilated episcleral vessels that extend upto the corneal limbus [Figure - 1], The left orbital venogram of the same patient showed orbital varix communicating to the left superior opthalmic vein [Figure - 2]
In cases with venous engorgement unilateral papilloedema was seen. Unilateral papilloedema signified venous engorgement. Actual invation of the nerve head by a glioma of the optic nerve was noted in one case of optic nerve glioma and the other showed optic atrophy.
There was no operative mortality in this group. Patients with benign lesions had good recovery. Patients with retinoblastoma invariably come late and the prognosis was very poor. For example a 2 1/2 year old boy with retinoblastoma who had gross proptosis and intracranial metastases died in 1 month. Results were equally poor in patients with neuroblastoma, a 3 1/2 year old girl had fracture femur, deposits in both sides of the mandible and orbit by the time she presented to us and died in 6 weeks [Figure - 3]. A 6 months old child with Yolk sac tumour also had recurrence of the tumour in the nasopharynx within one month inspite of chemotherapy [Figure - 4].
| Discussion | | |
A variety of lesions present with uniocular proptosis. In addition to local examination a proper history about the duration of proptosis, it's rate of increase and the presence of variations in its degree are very important. A review of the patients general health to exclude primary tumour elsewhere in the body, neurological examination and otorhinological examination go a long way in clinical diagnosis.
As for investigation, the C. T. Scan has replaced other modes of investigation. Computerised tomography is the greatest break through in modern times for the diagnosis of proptosis. It can show the position and size of any abnormal mass, demonstrate abnormally enlarged extraocular muscles, and contrast scan indicate the vascularity of the mass and help in diagnosis vascular abnormalities. Coronal cuts and high resolution C. T. enhance the diagnostic accuracy. In the C. T. benign lesions appear as round or oval with high density and nearly occupy the whole orbit. Pseudotumour has more irregular border and a higher density than benign lesions and produce no destruction of orbit (2).
However X-ray of the skull, orbit and paranasal sinuses, tomogram, orbital venogram, angiogram and ultrasonogram continue to have a complementary role in the investigation of uniocular proptosis. Orbital venography shows displacement, dilatation or non filling of veins which are indirect evidence of the tumour. An orbital venogram is still needed for orbital vascular lesions. All orbital varices should be investigated by carotid angiograms to exclude intracranial arteriovenous malformations. Similarly selective external carotid angiogram is useful in Angiofibroma especially when one is planning to do embolisation. Ultrasonography is undoubtedly excellent in intraocular lesions but its value in localising an orbital lesion is limited.
The number of cases in some of the groups are too small to give statistical significance. However some of the rare and interesting lesions need brief discussion. The group under pseudotumour is far from uniform. Psuedotumours are space filling lesions but have the characteristics of a chronic infiltrating inflammatory process. The dominant cell types vary from one case to another.
Usually lymphocytic cells are dominant. If these are arranged in follicles the lesion is called Psuedolymphoma. Sometimes the inflammatory character of the lymphoid infiltration is dominant as in myositis, sclerotenonitis and infiltrative Graves disease. These conditions are clearly related to immunological process [3].
Although the orbit does not have any lymphatics, lymphcysts and primary lymphomas do occur. In children lymphomas are rare hence every attempt should be done to exclude lymphocytic leukemia. Psuedolymphomas and lymphomas should be followed up since the clinical course could become malignant. (4). However immunocytological studies have been very helpful in differentiating the malignant lesions [5],[6].
Two cases of orbital abcess, the preoperative diagnosis of which will perhaps go up with the increased use of C. T. scan, were excised though transcranial and lateral approach [Figure - 5][Figure - 6]. They had antibiotics for one month with full recovery. Both the cases of orbital abscess gave history of septic focus in the face although at the time of presentation there were no signs of inflammatory lesion and the primary focus of infection had healed. There was one case of chronic granuloma, which presented with 5 month history of proptosis, temporal fullness and pain. Lateral orbitotomy and excision of the lesion was done. The aetiology has not been established yet.
The arachnoid cyst was excised after temporal craniotomy. The cyst had thinned out the orbital roof and lateral wall, the direct pressure and the venous obstruction probably accounted for the proptosis. Post operatively the proptosis subsided completely.
A one year old girl presented with 3 months history of left sided proptosis, papilloedema and left sided facial weakness. She had a seventh nerve neuroma which was removed by temporal craniotomy. [Figure - 7].
The patient with congential cystic eye had excision of the dysgenetic lesion so that a prosthesis could be fitted at a later stage. This is a rare condition recognised at birth as a large orbital mass in place of the normal eye. The upper eye lid was characteristically ballooned up and the dysgenetic tissue had protruded out between the eye lids. An arrest in the invagination of the optic vesicle early in embryogeneses is the most likely explanation. Disorganised proliferation of the neuro ectodermal and glial elements are prominent findings on light microscopy examination. This is perhaps the first case of congential cystic eye where a C. T. scan was done. C. T. showed a predominently hypodense mass with a cystic component anteriorly. The eye ball or any vestiges of normal orbital contents such as orbital muscles or optic nerve could not be made out. The right orbit was larger than the left. [Figure - 8].
Endodermal sinus tumour or Yolk sac tumour occurs in extragonadal locations, and in the orbit it is extremely rare. It is highly malignant. Histologically it is charecterised by laced pattern of vacuolated cells, perivascular glomarulus like structures, undifferentiated embryonal cells and hyaline globules in the meshes [7],[8]. Exenteration was done in the present case followed by chemotherapy. Neurofibramatosis is yet another rare lesion. A 11 year old boy had excision of neurofibroma of the orbit at the age of 4 years, he was readmitted for plexiform neurofibroma of the eye-lids with macrophthalmia [Figure - 9] for which he had plastic repair.
The common ophthalmic manifestation of Tuberous sclerosis is phakomatosis of the retina. In the present series, a 7 year old girl with tuberous sclerosis presented with unilateral proptosis, without any demonstrable orbital lesion.
| Conclusion | | |
This heterogeneous group of conditions presenting with uniocular proptosis needs multidisciplinary management including the involvement of the endocrinologist, immunologist, and physician.
Computerised tomography has gone a long way in making an accurate preoperative diagnosis and also in knowing the extent of the lesion, so that the appropriate surgical approach can be made and in some cases unnecessary operation avoided.
| References | | |
| 1. | Sambasivan M. Uniocular proptosis Experta Medica International Congress Series No. 293.  |
| 2. | H. Oshino M, 109 Cases of Orbital Tumours, Clinical and Diagnostic Studies, Folia Opthalmology, Japan - 635-647, 31/4, 1980. |
| 3. | Peelers HIF, Van Heede P, Felt Kamp, Vroom M, Psuedo Tumours, a clinical study, Doc Opthalmology 387-391, 52/4, 1982. |
| 4. | Tost M. Herde J and Wessel H, Lymphomata and Pseudo Lymphomata in the region of eyelid and orbital activity, Archives Geschwvistforsch, 17-24, 50/1, 1980. |
| 5. | Astarita RW, Minckler D, Taylor C. R. Orbital and adenexal Lymphomas American Journal of Clinical Pathology 615-621, 73/5, 1980. |
| 6. | Harris N L, Harman D C, Pitch B L, Immunohistological diagnosis of Orbital Lymphoid infiltrates American Journal of Surgical Pathology, 83-91, 8/2,1984. |
| 7. | Katz N N K, Ruymann F B, Margo C E, Endodermal Sinus Tumour of the Orbit Journal of Paediatric Opthalmology, 270-274,19/5,1982. |
| 8. | Margo C E, Folberg R, Zimmerman L E, Sesterhann I A, Endodermal Sinus Tumour of Orbit Opthalmology, 1426-1432, 91/92,1983. |
[Figure - 1], [Figure - 2], [Figure - 3], [Figure - 4], [Figure - 5], [Figure - 6], [Figure - 7], [Figure - 8], [Figure - 9]
[Table - 1], [Table - 2]
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