Year : 1973 | Volume
: 21 | Issue : 4 | Page : 166--170
Primary tumours of the orbit causing unilateral proptosis- Part II-diagnosis and management
Hari Mohan, DK Sen
Department of Ophthalmology, Irwin Hospital, New Delhi, India
Department of Ophthalmology, Irwin Hospital, New Delhi
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Mohan H, Sen D K. Primary tumours of the orbit causing unilateral proptosis- Part II-diagnosis and management.Indian J Ophthalmol 1973;21:166-170
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Mohan H, Sen D K. Primary tumours of the orbit causing unilateral proptosis- Part II-diagnosis and management. Indian J Ophthalmol [serial online] 1973 [cited 2022 Oct 7 ];21:166-170
Available from: https://www.ijo.in/text.asp?1973/21/4/166/34623
An appreciation of the numerous and diverse causes of unilateral proptosis is essential to the diagnosis of primary tumours of the orbit.
This report is concerned with our experiences in diagnosing and treating 54 consecutive cases of primary orbital tumours. The great majority of them have been followed over a number of years to see the reaction of therapy.
The diagnosis of an expanding lesion of the orbit may be made on the basis of clinical evalution, X-ray examination or surgery with or without histopathological examination of the tissue removed [Table 1].
The diagnosis by clinical methods is frequently impossible. Symptoms and signs are more general rather than specific for a particular tumour. The amount of proptosis of the globe and the direction of its displacement indicate only the size and position of the lesion within the orbit. Therefore, the diagnosis by clinical evaluation remains largely presumptive. Compressibility of the tumour and non-interference of ocular motility suggested a diagnosis of haemangioma in 3 cases. In 3 more cases associated haemangioma in other parts of the body was strongly suggestive of such a diagnosis. Generalized lymphomatous lesions indicated the cause of proptosis in 5 cases. Site of lesion and nature of displacement of globe suggested the origin of tumour in the lacrimal gland in 8 cases. Fungating, lesions of carcinoma were typical in all the 4 cases. Early and profound diminution of vision was suggestive of glioma of the optic nerve in 3 cases. However, a case of lymphosarcoma and another case of rhabdomyosarcoma simulated glioma of optic nerve in this respect.
X-ray examination is one of the important means which discloses the cause of proptosis in a good number of cases. In this series there were positive X-ray findings in 25 cases and of this number, the findings were indicative or diagnostic in 11 cases. In 4 cases of hacmangiomas, X-ray showed expansion of the orbit, a nonspecific finding indicating long-standing increased intra-orbital pressure. In rest of the cases X-ray findings were negative. One case of lymphosarcoma and another case of rhabdomyosarcoma showed enlargement of the optic foramen on the involved side. Otherwise the only finding seen in intraorbital sarcomas was the presentation of a soft tissue mass. The tendency of lacrimal gland epithelial tumour to invade bone gives X-ray examination an important role in their diagnosis. JONES AND PFEIFFER  reported 18 positive X-ray reports out of 20 cases of proven lacrimal gland epithelial tumours. In the present series, findings included enlargement of the lacrimal gland fossa in 5 cases and hyperostosis in 3 cases.
Though neurofibroma can cause marked deformities of the bony orbit (Moss  ) roentgenogram failed to show any disturbance in the bony orbit in either of the 2 cases. Two of the cases of optic nerve gliomas showed no roentgenographic findings whatsoever. Three cases showed concentric enlargement of the optic foramen.
Confirmation by biopsy of any etiologic diagnosis is essential. Many a time nature of the lesion is known only when the tissue removed is examined histopathologically. Of the 8 haemangiomas examined histopathologically, 2 were capillary or angioblastic in cytology and 6 were cavernous. The fibroma was pure in type which is a rarity (DUKE-ELDER  ).
It was composed of dense collagenous mass. All the 3 cases of reticuloendothelial tumours localized in the orbit were diagnosed histopathologically. The 5 cases diagnosed by clinical evaluation had widespread systemic disease and therefore no orbital biopsy was undertaken. However, lymph node biopsy was done in all these cases. Lymphoid tissue around the eye is most commonly encountered sub-conjunctivally and in the lacrimal gland. It is at these sites, therefore, the lymphomatous tumours are most likely to appear. Such tumours deep in the orbit are usually in the nature of reticulum cell sarcoma (REESE  ). It is of great interest to note that we came across a case of lymphoblastic lymphoma situated deep in the orbit where there is normally no lymphoid tissue. Such occurrence is ascribed to the presence of foetal remnants and pre-existing lymphoid elements (COATS  ). Nine of the 11 lacrimal gland epithelial tumours were labelled as benign histopathologically, but 2 of them turned malignant at a later date. So even histological diagnosis of a benign tumour is not a sure guide to the prognosis and all such cases should be taken as potentially malignant and should be kept under observation for a long time. Both the cases of isolated neurofibromas were diagnosed at operation as they were seen to arise in association with peripheral nerves. The diagnosis was confirmed by histopathological examination. One of the cases of optic nerve gliomas was very interesting. As is known gliomas of the optic nerve belong essentially to the least malignant type (Grade I). However, in this case histopathological examination proved the tumour to be of grade II type (MOHAN AND SEN ).
Accessibility of the tumour and its nature (encapsulated or diffuse) require different approaches for their management.
Radiation to the orbit is more often employed for malignant tumours. Usually the entire orbit needs irradiation and therefore an important part of the technique is to prevent ocular complication by protecting the cornea and the lens.
Surgical exposure of the orbital contents is not without its danger. Such explorations, therefore, are undertaken only when it is absolutely essential. Whenever an orbit is opened every effort should be made to remove the whole tumour with as little trauma as possible and save all ocular functions as far as possible.
The orbit may be explored by several methods of approach-superiotemporal route (BENEDICT  ), superionasal route (CALLAHAN  ), through the lower lid (DAVIS  ), transconjunctival approach (CALLAHAN  ) lateral route (KRONLEIN  ), and transfrontal route  -without much risk to life or vision and with little or no resulting deformity. These should be adopted to each individual case depending on the location of the tumour within the orbit and the extension of the growth. However one has to decide when surgery is to be undertaken. REESE  has advocated certain criteria which have proved to be quite useful:
Proptosis with a palpable mass:
Mass well defined-appropriate surgery.Mass ill defined-modified Kronlein's approach of Reese.
Proptosis without a palpable mass:
Visual function impaired modified Kronlein's approach. Retinal striae present-Kronlein's approach.No pertinent findings temporize and observe.
If a well defined, encapsulated tumour mass is found, complete excision is definitely indicated. However, when the mass is illdefined, friable or I invasive, a biopsy should be taken.
Direct approach through the conjunctiva or skin of the eye lid (Anterior orbitotomies) was utilized when the tumour was confined to the anterior third of the orbit. In two cases where the tumour was situated on the medial side of the globe difficulties were encountered and more room for manoeuvre was obtained by pushing the globe laterally after doing a lateral orbitotomy. BENEDICT'S  brow approach and KRONLEIN'S  procedure were quite satisfactory when the tumour was confined to the middle third of the orbit. But for tumours at the apical portion of the orbit transfrontal route  was preferred. Though KRONLETN'S procedure may be entirely adequate even for tumours in this situation, one seldom can be certain that the tumour in such a situation has not extended into the optic canal or cranial cavity. With the transfrontal route, the tumour could be reached more directly and deroofing of the optic canal can be undertaken if necessary.
Six of the patients with haemangioma were operated upon, one by anterior approach and four by Kronlein's route and one by combined anterior and lateral approach. At operation the location and size of the tumour were quite variable. Only 4 were within the muscle cone close to the optic nerve and the rest were outside the muscle cone. In 5 of these patients the tumours were apparently removed completely and all of them were living and well at last reports. In one case the tumour was so diffuse that complete excision was not possible. The patient was later on put on radiotherapy. Another case was subjected to radiotherapy from the beginning. In both of them there was definite improvement. Sclerosing fluid was injected in 3 cases with good results. In all the cases the tumour showed regression.
Exenteration was done in two cases of rhabdomyosarcoma but both of them died within the first year of onset of symptoms. The remaining 3 cases refused exenteration and therefore put on radiotherapy which failed in two cases and caused some improvement in the third case. However, all of them have been lost to follow up after irradiation.
All the cases of undifferentiated sarcoma were put on radiotherapy. Two of them did not respond well and the patients died within one-and-a half years; the third case improved and remained symptom free when last reported after 5 years.
Local biopsy was done in 3 cases of reticulo-endothelial tumours confined to the orbit. Lymph gland biopsy was done in rest of the cases where the disease was generalised. Radiotherapy was given in every instance. Five patients could be followed up. Of them the 2 patients with generalised disease died within 2 years. Irradiation, however, gave dramatic response in 3 cases where the lesion was clinically confined to the orbit.
Lacrimal gland epithelial tumours are not very sensitive to irradiation. Operation is the treatment of choice. Such tumours are anteriorly situated and, therefore, are readily accessible by anterior route. However, if the tumour is not entirely removed at the first operation, it becomes progressively malignant and since complete removal of such tumours by anterior approach is often difficult due to backward extension of the tumours, lateral orbitotomy is often preferred. In the present series 6 cases were operated upon by Kronlein's approach and 3 cases by anterior approach. Surgery included all of the capsule and adjacent periosteum, plus underlying bone when it was involved. In the remaining two cases where the histopathological report was carcinoma, exenteration of the orbit was done with the excision of the bone of the lacrimal gland fossa and adjacent orbital wall. LANE  reported 95 cases from the literature with 12.6 percent mortality, 20% recurrences and 6.4% distant metastases. In the follow up in this series, 2 out of 9 benign lacrimal gland tumours showed recurrence. One of them died within 3 years. The other case was symptom free 6 years after deep X-ray therapy had been instituted.
Both the cases diagnosed initially as malignant tumour died due to extension. The outcome in the cases of carcinoma was poor. In two cases exenteration was done which was followed by deep X-ray. The remaining two cases reported very late and therefore were subjected only to radiotherapy.
Both the neurofibromas were excised by anterior approach and there was no recurrence after a long follow up. Two of the neurilemmomas were removed by Kronlein's procedure, one by anterior orbitotomy and the other by transfrontal route. Three of the optic nerve gliomas were successfully excised by Kronlein's approach. In one case where the tumour extended backwards unroofing of the optic canal and excision of the tumour was accomplished by the transfrontal approach. In the 5th case the tumour could not be removed completely even by transfrontal route. The patient was, therefore, put on radiotherapy with good results.
Exact diagnosis in cases of unilateral proptosis is always a leading problem to the ophthalmologists. Clinical diagnosis is often presumptive. X-ray findings may be diagnostic or suggestive. In this series there were positive roentgenographic finding in 25 (46.3%) and of this number the findings were indicative or diagnostic in 11 (20.4%) of all the cases. In an additional 6 (11.1%) cases the findings considered in the light of all the clinical and other laboratory evidence suggested the diagnosis.
It is incumbent on the ophthalmologist to determine when surgical treatment is indicated and what type of approach can be used to best advantage. (Surgery was performed in 34 cases comprising 63% of all cases. Fourteen cases (25.9%) had Kronlein's procedure, 9 (16.6%) cases had anterior orbitotomies, 3 (5.5%) had transfrontal approach, 6 (11.1 %) had exenteration done and 2 (3.7%) had combined procedures). Tumours of the anterior third of the orbit were most satisfactorily removed by way of anterior orbitotomies or by a combination of anterior and lateral orbitotomies. For tumours in the middle third of orbit Kronlein's procedure was found most suitable while transfrontal approach gave better accessibility to tumours situated at the apical portion of orbit or extending intra cranially.
Deep X-ray therapy was most useful in cases of reticulo-endothelial tumours clinically confined to the orbit.
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