Ординатура / Офтальмология / Английские материалы / Handbook of Pediatric Neuro-Ophthalmology_Wright, Spiegel, Thompson_2006

CHAPTER 8: BRAIN LESIONS WITH OPHTHALMOLOGIC MANIFESTATIONS 281

or cranial nerves, and obstruction of the venous sinuses. The precise symptoms developed by a patient depend on the location of the tumor. For example, a meningioma in the cerebellopontine angle produces nystagmus and ocular motor dysmetria as well as increased intracranial pressure.

The treatment of intracranial meningioma is complete surgical resection wherever possible. Radiation therapy has been attempted for tumors located in inoperable locations with varying degrees of success.

Meningiomas may also occur, primarily associated with the sheath of the optic nerve; they occur in this location about as often in children as in adults.28 A patient with a primary orbital meningioma usually presents with unilateral visual loss. In childhood, the visual loss is usually quite advanced at the time of presentation. The optic disc may be swollen or atrophic, although it is most often atrophic. Optociliary shunt vessels may be present on the surface of the disc. The tumor is readily diagnosed with neuroimaging. Radiologic features include tubular enlargement of the optic nerve associated with calcification. The appearance is often described as a “train track,” with enhancing parallel lines surrounding a lucent space in axial CT sections of the optic nerve.

The appropriate treatment for primary orbital meningiomas is controversial. Alper has said that many of these tumors behave malignantly in children and should be resected completely and immediately.1 In general, resection of the affected optic nerve should be performed before the tumor spreads intracranially. Radiotherapy remains an alternative treatment. There are case reports of improvement after radiotherapy.37

In our center, we prefer to follow these tumors at 3-month intervals in a fashion analogous to that for optic nerve gliomas. Visual acuity is monitored as well as tumor volume and extent by neuroimaging. A patient who demonstrates increasing encroachment of the meningioma toward the intracranial space, tumor enlargement, or complete loss of vision should be considered for a combined craniotomy/orbitotomy resection.1

Tumors of Congenital Origin

Three distinct tumors of congenital origin present during childhood: germinoma, craniopharyngioma, and arachnoid cyst.

Germinomas are relatively rare tumors that arise from germ cells. They most often arise in the region of the pineal gland

FIGURE 8-14. Midsagittal MRI of a 12-year-old boy with a germinoma. His symptoms were difficulty seeing the blackboard. He had bilateral pupillary light–near dissociation, anisocoria, and convergence-retraction nystagmus. The tumor (arrow) is seen compressing the tectal plate.

but contain no cells typical of the pineal gland. They may also appear in the hypothalamic or thalamic region. As in all brain tumors, the presenting signs of germinoma depend on their location. If the tumor arises in the region of the pineal gland, the patient will have signs of increased intracranial pressure from compression of the dorsal midbrain and of the cerebral aqueduct (Fig. 8-14). The ophthalmologic signs of the dorsal midbrain syndrome (Parinaud) are a skew deviation, fourth and sixth cranial nerve pareses, convergence-retraction nystagmus, impaired upgaze, anisocoria, and corectopia. The differential diagnosis of germinoma in the region of the pineal includes pinealoma and teratoma.

If the tumor is in the hypothalamic region, downward extension may cause visual loss and upward extension may cause papilledema from obstruction of the third ventricle. Ger-

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minomas in the region of the thalamus also cause visual loss from infiltration of the lateral geniculate body, optic tracts, or portions of the geniculocalcrine radiations. The diagnosis in each of these locations is made by biopsy. Treatment is radiation therapy. These tumors are extraordinarily sensitive to radiation therapy, with a 10-year survival rate of approximately 72%.40

Craniopharyngioma is the third most common brain tumor of children in some studies, representing about 10% of tumors.20 It often presents to the ophthalmologist as visual loss of unknown etiology or as a sensory strabismus. The physician should be very wary of this tumor when diagnosing amblyopia or functional visual loss. For instance, we saw a 6- year-old with exotropia and 20/200 vision in one eye. Mild optic atrophy was present. An MRI demonstrated a large craniopharyngioma. In childhood, this tumor usually presents after visual symptoms are present because of the delay in diagnosis with children.

Craniopharyngiomas arise from embryonic rests of Rathke’s pouch. The tumor is composed of a capsule, a cystic portion containing the classic “machine oil,” and a solid mass. The tumor may compress the optic pathways, the hypothalamus, the third ventricle, and the pituitary gland (Fig. 8-15). The optic pathway may be compressed from above, below, or from the side, thus causing nearly any visual field defect. We previously reported on the clinical course of 12 children with craniopharyngiomas. Nearly all these children suffered severe visual loss and most presented with profound optic atrophy.33 There was no recovery of function following treatment in children, unlike the improvement seen in adults.

The treatment of craniopharyngioma involves an attempt at total resection when accompanied by an acceptable minimal morbidity. If complete resection is impossible, as is often the case, a partial resection is performed. For older children, teenagers, and adults, postoperative radiotherapy is employed. For young children, radiotherapy is delayed, relying on clinical examination and repeat neuroimaging at 3-month intervals for the first year and at 6-month intervals thereafter to detect recurrences. These studies evaluate the suprasellar space for regrowth and consequent compromise of the visual system. If there is regrowth, repeat resection and/or drainage of the reaccumulated cystic material followed by radiotherapy is performed. In our experience, children with visual loss and especially those with

FIGURE 8-15. Coronal MRI of a craniopharyngioma in a 1-year-old patient that is obliterating the suprasellar cistern.

optic atrophy at the time of their presentation will not have significant visual improvement after decompression. However, they do not seem to suffer further visual loss.

The differential diagnosis of craniopharyngioma includes suprasellar epidermoid and dermoid cysts, Rathke pouch cysts, arachnoid cysts, and pituitary adenoma. Pituitary adenomas are uncommon in children but may present similarly to craniopharyngioma with endocrine dysfunction, hypopituitarism, and/or visual loss.

Arachnoid cysts are collections of CSF contained within a membrane of meningeal tissue. They may arise anywhere there are meninges. In some circumstances, arachnoid cysts may enlarge because of the active production of fluid. Enlargement may cause symptoms of mass effect, increased intracranial pressure, or, if there is a suprasellar cyst, an optic neuropathy. Suprasellar arachnoid cysts most often present in young patients

CHAPTER 8: BRAIN LESIONS WITH OPHTHALMOLOGIC MANIFESTATIONS 285

FIGURE 8-16. Sagittal MRI of a suprasellar arachnoid cyst in a 4-year-old patient. No ophthalmologic defect could be detected.

(Fig. 8-16).15 Pituitary and hypothalamic signs as well as third ventricle compression may occur from expansion of a suprasellar or arachnoid cyst in addition to visual loss. Diagnosis of a cyst is usually incidental but is easily made by MRI. Treatment of symptomatic lesions is drainage with capsule removal. There is only a very low chance of recurrence, even when removal of the capsule is incomplete.

References

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22.Lambert SR, Hoyt CS, Nahara MH. Optic nerve hypoplasia. Surv Ophthalmol 1987;32:1–9.

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TABLE 9-1. Nystagmus Types as Identified by History, Physical Examination, and Ocular Motility Recordings.

1.Acquired

2.Arthrokinetic

3.Associated

4.Audiokinetic

5.Bartel’s

6.Bruns’

7.Centripetal

8.Cervical

9.Circular/elliptic/oblique

10.Congenital/infantile syndrome

11.Convergence

12.Dissociated

13.Downbeat

14.Drug-induced

15.Epileptic

16.Flash-induced

17.Gaze-evoked

18.Horizontal

19.Induced (provoked)

20.Intermittent vertical

21.Jerk

22.Latent/manifest latent

23.Lateral medullary

24.Lid

25.Miner’s (occupational)

26.“Muscle-paretic”

27.Optokinetic

28.Optokinetic after (induced)

29.Pendular

30.Periodic/aperiodic alternating

31.Physiological (endpoint, fatigue)

32.Pursuit (after induced)

33.Rebound

34.Reflex (Baer’s)

35.See-saw

36.Somatosensory induced

37.Spasmus nutans

38.Spontaneous (“voluntary”)

39.Torsional

40.Uniocular

41.Upbeat

42.Vertical

43.Vestibular (central, peripheral, geotropic, ageotropic, galvanic, head shaking, positional, caloric, rotational)