Year : 1985 | Volume
: 33 | Issue : 2 | Page : 135--137
Leukaemic infiltration of the optic nerves demonstration by computerized orbital tomography
Madhumati Misra, Sanatan Rath, Koneti Rao
Department of Neurosurgery, S.C.B. Medical College, Cuttack, India
Department of Neuro Surgery, S.C.B. Medical College, Cuttack -753 037
|How to cite this article:|
Misra M, Rath S, Rao K. Leukaemic infiltration of the optic nerves demonstration by computerized orbital tomography.Indian J Ophthalmol 1985;33:135-137
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Misra M, Rath S, Rao K. Leukaemic infiltration of the optic nerves demonstration by computerized orbital tomography. Indian J Ophthalmol [serial online] 1985 [cited 2021 Apr 20 ];33:135-137
Available from: https://www.ijo.in/text.asp?1985/33/2/135/30840
The facility to evaluate the orbital anatomy "in vivo" by computed tomographic (CT) scanning has opened discussion on the pathogenetic mechanism of various orbitopathies. In investigating patients with proptotic eyes, CT evidence of the simultaneous presence of ocular and orbital infiltrative lesions should suggest orbital extension of intraocular tumours (retinoblastoma or choroidal melanoma), metastatic tumour to the eye and orbit or infiltrative lymphomatous or leukaemic deposits. In bilateral cases, however the latter is the strongest probability.
It is extremely rare for a patient with systemic lymphoproliferative disorder to seek initial ophthalmic consultation. Only 3 of 1269 patients in Rosenberg series did so, although another 13 patients developed proptosis during the course of their disease. Moreover, proptosis as an initial presentation of acute myelogenous leukaemia is a chance finding in ophthalmic practice,,,,,,. We report the case of a 8 year old girl who was investigated by us for bilateral proptosis. Demonstration of bilateral concurrent ocular and orbital affections with characteristic infiltration of the optic nerves suggested the possibility of biorbital leukaemic infiltration. The diagnosis was confirmed by haematological studies. CT evidence of diffuse optic nerve infiltration warranted urgent orbito-cranial irradiation in combination with systemic chemotherapy for protecting vision. In post therapy check scans, CT evidence of improvement preceded the clinical amelioration and regression of proptosis indicating excellent therapeutic response. The role of CT in evaluating patients with infiltrative orbitopathy and in monitoring the results of therapy is discussed at length with the review of available literature.
LP, an 8 year old girl presented with bilateral progressive proptosis since one month. She gave no history of headache, vomiting, fever or episodic bleeding and had not sustained trauma to-head in the recent past. Physical examination was unremarkable except for puffiness of the face and bilateral proptosis eyes [Figure 1]. Abdominal examination revealed no visceromegaly, bone tenderness elicited on sternal pressure was questionable. Ophthalmic evaluation revealed bilateral, axial, noninflammatory proptosis with passive and uniform restriction of ocular mobility, examination of the external ocular segments revealed no abnormality.
Both eyes had visual acuity reduced to 6/24, intact peripheral visual fields and papilloedema on fundoscopy.
Skull radiographs including lateral, Waters and Caldwell view were unremarkable. Computed tomographs demonstrated bilateral diffuse ocular and orbital infiltrations within the intraconal area with masses of soft tissue attenuation obliterating the global anatomy. Both the optic nerves appeared dense, expanded with increased attenuation and showed no change with contrast enhancement [Figure 2] Bilateral secondary infiltrative orbitopathy was suggested and acute myeloid leukaemia was diagnosed by haematological studies.
Systemic manifestations of 'the disease however, developed subsequently. Biorbital irradiation was combined with the conventional chemotherapy for infiltrative optic neuropathy. Regression of proptosis was marked within 10 days [Figure 3] however, CT evidence of clearing of infiltrations preceded the clinical recovery [Figure 4].
Patients with acute myeloid leukaemia presenting with proptotic eyes as the initial systemic manifestation of the disease are rarely encountered in Ophthalmic practice. Computed orbital tomography in such cases not only defines the anatomical extent and nature of the lesion but often gives a clue to search for an offending systemic pathology which has not yet declared itself clinically, as noted in the present case.
The common orbital disorders which show concurrent involvement of the eye and orbit on a CT display include invasive intra global tumours (retinoblastoma and choroidal melanomal,,,(11),(12)), metastases especially from the breast or bronchus and haematological disorders including lymphoid hyperplasia, lymphomas and leukaemias., Other neoplasms capable of concurrent presentation within the eye and orbit are rare but include medulloepithelioma, glioma of optic nerve, carcinoma of the nonpigmented ciliary epithelium and squamous cell carcinoma of the conjunctiva.
In patients with orbitopathy showing CT evidence of ocular and periocular affection, systemic evaluation must be carried out for lymphoma or leukaemia and to differentiate distance orbital metastases from invasive primary ocular tumours.
CT demonstration of global infiltrations with expansion of the intraorbital optic nerves has been described to be characteristic of leukaemic orbitopathy.
In the presence of papilloedema or visual failure, demonstration of optic nerve infiltration in CT warrant the need for aggressive management with chemotherapy and orbitocranial irradiation. Such combined therapy is popular in the management of concurrent ocular and periocular infiltrative orbitopathies.
However, visualization of an expanded optic nerve at CT in a patient with failing vision and atrophic optic disc prognosticates poor post therapy visual recovery.
Follow up CT studies can be dictated by the clinical course, but in view of the possible risk of radiation exposure as few scans should be done as appears practical. Check scanning should be reserved for patients who show' poor post therapy recovery in whom visual deterioration progresses in spite of aggressive therapy or when orbital recurrence is suspected during follow up period.
The case of an 8-year old girl developing bilateral proptosis as the initial manifestation of acute myeloid leukaemia is presented and the characteristic computed tomographic finding of infiltrative orbitopathy is discussed. CT has the advantage of imaging both the bone and soft tissue structures within the orbit noninvasively. Demonstration of the nature and extent of orbital invasion significantly alters the differential diagnosis of various orbital disorders and changes the management and prognosis as exemplified in our patient with biorbital leukaemic infiltration.
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