|Year : 1985 | Volume
| Issue : 3 | Page : 171-173
Intraorbital oculomotor schwannoma in Von Recklinhausen's disease
Madhumati Misra, Sanatan Rath
Department of Neurosurgery S.C. B. Medical College, India
S.C.B. Medical College
Source of Support: None, Conflict of Interest: None
|How to cite this article:|
Misra M, Rath S. Intraorbital oculomotor schwannoma in Von Recklinhausen's disease. Indian J Ophthalmol 1985;33:171-3
Von Recklinhausen's disease or multiple neurofibromatosis was first described by Von Recklinhausen in 1882,. Patients with this disease are prone to develop peripheral nerve tumours,. The incidence of neurofibroma amongst all orbital tumours is about 2-3%,,. To the best of our knowledge its association with Von Recklinghausen's disease has not been reported though Duke-Elders does mention of such a possibility.
The present case of an intraorbital oculomotor schwannoma in a girl of Von Recklinhausen's disease presenting as proptosis of right eye is therefore worth reporting. The available literature has been reviewed at length.
| Case report|| |
Is, a female aged 13 years reported with progressive protrusion and increasing diminution of vision of right eye for the last 6 years. [Figure - 1]A. There was no history of headache, fever, vomiting, seizure or injury to head and orbit. She had cafe au lait spots and multiple cutaneous neurofibromata in the body. Examination of right eye revealed partial ptosis and a proptosis of 15 mm with outward tilt. All movements of eye ball except adduction were limited. Proptosis was nonpulsatile and irreducible. Orbital margins were normal and no mass could be felt between the globe and the orbit. Funduscopy revealed secondary optic atrophy in the right eye with vision reduced to finger counting at 1 foot and peripheral visual field grossly constricted. The preauricular and submandibular lymphnodes were not enlarged on the rightside. The left eye was clinically normal.
| Investigations|| |
Laboratory examinations including blood count and urine analysis were within normal limits. X-rays of orbit, skull and opticformina were normal. Computed scanning demonstrated a right sided intraorbital space occupying lesion between the medical orbital wall & the globe which was circumscribed, pyriform and hyperdense [Figure - 1] B 2 Intraorbital structures like opticnerve, globe, recti muscles and orbital apex were found free No intracranial or extra orbital involvement was noted.
On right frontal cranieetomy, a well circumscribed relatively avascular capsulated tumour was found arising from the rootlets of oculomotor nerve between the medical orbital wall and the globe. The tumour could be removed in piecemeal. The postoperative period was uneventful; the eye ball receded and unaided visual acuity improved to 3/60. Histopathological study revealed picture of neurofibrama [Figure - 1]C
| Discussion|| |
Primary tumours in the orbit form a small part of neuro-ophtalmic work, a factor which may account for inadequacies in their investigation and treatment. Since the introduction of computed orbital scanning, primary orbital tumours are being increasingly recognised in relation to their exact anatomical sitution.
The incidence of peripheral nerve tumour amongst all orbital tumours is about 3%,, . In our study, a single case of intraorbital neurofibroma was encountered from a series of 40 primary orbital tumours. The incidence of 2.5% in this study falls close to that reported by other workers. However, a low incidence of this tumour has been reported by some authors.,,
Generalised neurofibromatosis (Von Recklinhausen's disease) was first described by Von Recklinbausen in 1882 patients with such disease often develop benign, encapsulated, slow growing neurofibromata of peripheral nerves,,. Sarcomatous transformation in this tumour is also reported, though rarely,,,. The disease is frequently familial and commonly manifest in childhood; no specific relationship with sex has been observed. The symptoms and signs of intraorbital neurofibroma are more general than specific. Proptosis of long duration, failing vision, ophthalmoplegia, papilloedema or optic atrophy are common features due to intraorbital mass effect, as noted in our patient,,. In advanced cases, intracranial pulsations may be transmitted to the orbit due to destruction of the orbital bones.
Presence of intraorbital schwannoma in patients with Von Recklinhausen's disease is rarely reported. Neurofibramas arise as a result of diffuse proliferation of schwan cells of the peripheral nerve and involve fibroblastic elements of the endoneurium and perineurium. Later, some proliferations occur within the sheath leading to enlargment and tortuosity of the nerve,.
In the present case, there was right sided proptosis of gradual onset and secondary optic atrophy of 6 years duration. Presence of generalised neurofibromatosis and demonstration of an intraorbital well-circumscribed mass lesion in the C.T. Scan suggested the posibility of intraorbital neurofibroma. At surgery its origin from oculomotor nerve was confirmed. Biopsy after surgical excision confirmed the diagnosis of schwannoma. The patient is asymptomatic till the day of last report. However, her vision has improved to 4/60 only, because of longstanding opticatrophy.
| Summary|| |
A rare case of intraorbital oculomotor schwannoma in a 13 year old girl of Von Recklinhausen's disease, diagnosed by computed orbital scanning is reported. It is important to recognise these lesions in early stages as most of them are benign and timely surgery will save both life and vision.
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[Figure - 1]