Central nervous system tumours (CNS) are the most common solid tumours in children and a leading cause of mortality and morbidity in children worldwide [
Despite advances in neuroimaging, early diagnosis of pediatric CNS tumours remains a dilemma. Contributing factors to this problem are varied presentation and rarity of these tumours in childhood [
Visual manifestations of brain tumours in children include blurring of vision, diplopia, ocular nerve palsy, papilledema, optic atrophy, ptosis, nystagmus, strabismus, proptosis, and photophobia. [
The purpose of this study is to evaluate the neuroophthalmic and clinical characteristics of intracranial-space-occupying lesions in children.
This prospective observational study was carried out for a period of 2.5 years from June 2017 to December 2019 after Ethical Committee Clearance. All children between 0 and 16 years presenting with brain tumours confirmed by Magnetic Resonance Imaging (MRI) and treated surgically in our institute were included in this study. Patients who had received chemotherapy or radiotherapy before surgery or with coexistent primary neoplasm elsewhere were excluded from the study. Patient’s age; gender; neurological symptoms which include history of headache, nausea/vomiting, seizures, and anosmia; and neurological signs which include cerebellar signs, speech involvement, motor deficits, altered sensorium, endocrine involvement, and cranial nerve palsies were evaluated by the neurosurgical team.
Ophthalmic features which include diminution of vision and visual field defects, ocular pain, red eye, proptosis, papilledema, disc pallor, shunting of vessels, ptosis, pupil abnormality, nystagmus, and strabismus were evaluated by the ophthalmologist.
Other parameters which were evaluated were the location of the tumour by contrast-enhanced MRI, type of surgical intervention, and postoperative histopathological diagnosis.
A total of 35 patients were evaluated. Ages ranged from 8 months to 16 years, and the mean age of the patients was 8.28 years. The average duration of symptoms in our study was 6.45 weeks.
According to the location, 16 cases were infratentorial in location and 19 cases were supratentorial in location. Frequency of neurological signs and symptoms according to supra- and infratentorial locations are mentioned in Table
Frequency of neurological signs and symptoms according to supratentorial and infratentorial location of the tumour.
Clinical features | Infratentorial | Supratentorial | Total |
---|---|---|---|
Headache | 16 (45.7%) | 17(%) | 33 (94.3%) |
Nausea/vomiting | 14 (40%) | 10 (28.6%) | 24 (68.6%) |
Ophthalmic involvement | 15 (42.9%) | 15 (42.9%) | 30 (85.7%) |
Cerebellar signs | — | 16 (45.7%) | 16 (45.7%) |
Ophthalmoparesis | 15 (42.8%) | ||
Cranial nerve II | 14 | 15 | 29 (82.9%) |
Cranial nerve III | 2 | 1 | 3 (8.6%) |
Cranial nerve IV | 2 | 1 | 3 (8.6%) |
Cranial nerve V | 1 | 0 | 1 (2.9%) |
Cranial nerve VI | 13 | 2 | 15 (42.9%) |
Cranial nerve VII | 4 | 0 | 4 (11.4%) |
Cranial nerve VIII | 1 | 0 | 1 (2.9%) |
Lower cranial nerves | 2 | — | 2 (5.7%) |
Seizures | 1 (2.9%) | 13 (37.1%) | 14 (40%) |
Altered sensorium | 1 (2.9%) | 4 (11.4%) | 5 (14.3%) |
Ataxia | 4 (11.4%) | 0 | 4 (11.4%) |
Motor deficit | 0 | 3 (8.6%) | 3 (8.6%) |
Endocrine involvement (stunted growth) | 0 | 2 (5.7%) | 2 (5.7%) |
Increased head circumference | — | 1 (2.9%) | 1 (2.9%) |
23 patients presented with ocular symptoms at the time of presentation, whereas 31 patients were found to have abnormal findings after detailed ophthalmic evaluation. The ophthalmic manifestations of the patients according to infratentorial and supratentorial locations are illustrated in Table
Ophthalmic manifestations of the tumours according to infratentorial location and supratentorial location of the tumour.
Infratentorial | Supratentorial | Total | |
---|---|---|---|
Visual acuity involvement | 6 (17.1%) | 7 (20%) | 13 (37.1%) |
Diplopia | 11 | 2 (5.7%) | 13 (37.1%) |
Strabismus | 11 | 1 (2.9%) | 12 (34.3%) |
Ptosis | 2 (5.7%) | 1 (2.9%) | 3 (8.6%) |
Papilledema | 9 (25.7%) | 8 (22.9%) | 17 (48.6%) |
Ophthalmoparesis | 13 (37.1%) | 2 (5.7%) | 15 (42.8%) |
Nystagmus | 13 (37.1%) | 1 (2.9%) | 14 (40%) |
Disc pallor | 5 | 6 (17.1%) | 11 (31.4%) |
Pupillary abnormality | 2 (5.7%) | 2 (5.7%) | 4 (11.4%) |
commonest ophthalmic symptoms were diminution of vision and diplopia, and the most common ophthalmic sign was papilledema followed by ophthalmoparesis and nystagmus.
The location of the tumours according to their age distribution is illustrated in Table
Tumour location according to age distribution.
Tumour location | 0-5 years | 6-10 years | 11-16 years | Total |
---|---|---|---|---|
Cerebellar tumour | 1 | 5 | 5 | 11 (31.4%) |
Sellar region tumours | 0 | 3 | 3 | 6 (17.14%) |
Ventricular tumours | 3 | 1 | 1 | 5 (14.3%) |
Left frontal lobe tumour | 0 | 1 | 2 | 3 (8.6%) |
Pontine tumour | 2 | 1 | 0 | 3 (8.6%) |
Frontoparietal tumour | 2 | 0 | 0 | 2 (5.7%) |
Left basal ganglia tumour | 0 | 0 | 1 | 1 (2.9%) |
Multiple intracranial tumour | 0 | 0 | 1 | 1 (2.9%) |
Pineal gland tumour | 1 | 0 | 0 | 1 (2.9%) |
Prepontine tumour | 1 | 0 | 0 | 1 (2.9%) |
Right frontotemporoparietal tumour | 1 | 0 | 0 | 1 (2.9%) |
Total | 11 | 11 | 13 | 35 |
The type of surgical interventions are mentioned in Table
Type of surgical intervention.
Operative characteristics | Frequency | Percentage |
---|---|---|
Craniectomy with surgical resection | 22 | 62.9% |
CSF diversion | 4 | 11.4% |
Transsphenoidal gross total resection | 3 | 8.6% |
Endoscopic gross total tumour excision | 2 | 5.7% |
Primary radiotherapy | 2 | 5.7% |
Endoscopic biopsy | 1 | 2.9% |
Stereotactic biopsy | 1 | 2.9% |
Total | 35 | 100.0% |
The histopathological diagnosis of all the patients and their distribution according to their location are mentioned in Table
Histopathological diagnosis and their distribution according to their location.
Histopathology | Frequency | Location | Frequency |
---|---|---|---|
Medulloblastoma | 6 (17.1%) | Cerebellum | 6 |
High-grade glioma | 5 (14.3%) | Pons | 3 |
Left frontal lobe | 1 | ||
Right frontoparietal lobe | 1 | ||
Low-grade glioma | 4 (11.4%) | Cerebellum | 2 |
Left frontal lobe | 1 | ||
Left lateral ventricle | 1 | ||
Abscess | 4 (11.4%) | Bilateral frontoparietal lobe | 1 |
Left frontoparietal lobe | 1 | ||
Left basal ganglia | 1 | ||
Multiple intracranial lesions | 1 | ||
Arachnoid cyst | 4 (11.4%) | 4th ventricle | 1 |
Cerebellum | 2 | ||
Sellar region | 1 | ||
Craniopharyngioma | 4 (11.4%) | Sellar region | 3 |
Third ventricle | 1 | ||
Choroid plexus papilloma | 1 (2.9%) | Left lateral ventricle | 1 |
Giant cell tumour | 1 (2.9%) | Cerebellum | 1 |
Infected epidermoid cyst | 1 (2.9%) | Prepontine region | 1 |
Meningioma | 1 (2.9%) | Left lateral ventricle | 1 |
Optic nerve glioma | 1 (2.9%) | Sellar region | 1 |
Pineoblastoma | 1 (2.9%) | Pineal region | 1 |
Pituitary adenoma | 1 (2.9%) | Sellar region | 1 |
Tuberculoma | 1 (2.9%) | Left frontal lobe | 1 |
Total | 35 | 35 |
Pediatric brain tumours are a heterogenous group of neoplasms which vary in their cell of origin, mode of presentation, management protocols, and clinical outcomes [
This study was undertaken to observe a particular cohort of children who were clinically and radiologically diagnosed with intracranial tumours managed by the neurosurgical team along with ophthalmological examination done by the ophthalmology team.
In this study, there were 35 patients with a mean age of 8.28 years which was comparable to the study by Liu et al. [
The road to diagnosis of brain tumours can be a potential challenge as many clinical symptoms can mimic other common childhood ailments. The clinical signs of the tumour can vary based on the location, age, and aggressiveness of the tumour, and a high index of suspicion followed by prompt evaluation and surgical intervention is required, the delay of which can lead to untoward complications [
The commonest neurological symptom was headache followed by nausea and vomiting which confirms the well-known predominance of symptoms of raised intracranial pressure in these cases according to the literature [
The other neurological signs and symptoms seen in our study were cerebellar signs (45.7%), optic nerve involvement (82.9%), other types of cranial nerve involvement (42.8%), seizures (40%), altered sensorium (14.3%), ataxia (11.4%), motor deficits (8.6%), endocrine involvement (5.7%), and increased head circumference (2.9%). These are consistent with various similar studies in the literature on pediatric intracranial tumours [
Brain tumours can alter the normal neuroanatomical structures of the visual system leading to visual impairment and dysfunction which can have a lifelong impact on the quality of life of patients and their families [
Ophthalmic signs and symptoms are a mode of presentation in children with intracranial tumours, and the ophthalmological evaluation must be performed with greater attention as the presentation in children is less specific than that in adults [
Neuroophthalmologic evaluation should be done by protocol for children with brain tumours. However, early assessment of vision may not be done routinely at all hospitals due to various reasons which include (a) priority given to brain tumours, (b) lack of apparent visual symptoms at the time of presentation, (c) inability to clearly describe symptoms which is particularly more common in young children, (d) difficulty in examining young children, and (e) difficult access to a neuroophthalmologist [
Out of the 35 patients in our study, 23 (65.71%) patients presented with ocular symptoms and 31 (88.57%) patients were found to have abnormal findings after detailed ophthalmological examination. Ocular symptoms at the time of presentation are higher than in some studies in the literature like the study by Liu et al. which can again be attributed to the late presentation of these cases in developing countries [
The visual symptoms seen in our study were diminution of vision, diplopia, strabismus, and ptosis. All these visual symptoms can be devastating and lead to long-term effects on the quality of life for the patient and the family. Visual impairment can be caused by affecting both the afferent and the efferent pathways. Direct compression of the visual pathway by the tumour can lead to diminution of vision, visual field defect, and ocular motility defects [
In their study on children diagnosed with intracranial-space-occupying lesions, Alswaina et al. have highlighted the role of an ophthalmologist in these cases. They have reported an incidence of optic atrophy in 46%, diminution of vision in 46%, papilledema in 24%, nystagmus in 24%, sixth nerve palsy in 19%, and third nerve palsy in 12% [
Ophthalmoparesis was seen in 15 (42%) patients in our study. Palsies of III, IV, and VI nerve were more common in children with infratentorial tumours than in children with supratentorial tumours which again correlated with the data available in the literature [
Ophthalmological signs seen in our study were papilledema, nystagmus, disc pallor, visual field defects, and pupillary abnormality. Papilledema or pale disc from pediatric brain tumours can result from tumours intrinsic to the optic nerves or optic chiasm, tumours contiguous to the optic nerve or chiasm, or tumours located elsewhere causing nonlocalizing increased intracranial pressure [
In our study, visual field defects were present clinically and confirmed by perimetry as bitemporal hemianopia in 2 patients of sellar region tumours. However, 4 patients had clinical symptoms of field defects which could not be confirmed by perimetry due to noncompliance of the children. In a study carried out by Harbert et al., the authors reported that 15.2% of children with CNS tumours have visual field defects that go unrecognized and recommended that serial neuroophthalmological evaluation of children with brain tumours is required to diagnose a visual field defect since patient or caregiver reporting may be limited [
Pupillary abnormality was seen in 4 patients (11.4%) out of which 2 patients were cases of pineal region tumours, and these patients presented with features of Perinaud’s syndrome which include upgaze palsy convergence-retraction nystagmus, light-near dissociation of pupils, and lid retraction [
After complete neurological and ophthalmic evaluation of the patient, imaging was done in all patients with suspected intracranial tumours. Contrast-enhanced MRI of the brain is the investigation of choice which was done in all our cases. Additionally, depending on the type and location of the tumour, MRI angiogram to display vasculature alongside the tumour, MR spectroscopy to assess the metabolic activity of the tumour, and functional MRI and tractography to provide additional information on the course of important white matter tracts and their relationship with the tumour were done for planning of surgery. Sedation was required in younger children or children not cooperative to undergo MRI.
In our study, 20% of the cases were located in the cerebral cortex, 31.4% of the cases were located in the cerebellum, 8.6% of the cases were located in the brainstem, 17.1% of the cases were located in the pituitary and suprasellar region, and 14.2% of the cases were intraventricular in location. According to the literature, about 25-30% are in a supratentorial location, followed by cerebellum (15-20%), brainstem (10-12%), pituitary and suprasellar regions (10-15%), and brain ventricles (5-6.4%) which is comparable to our study [
Of the 35 tumours, 19 (54.3%) were supratentorial and 16 (45.7%) were infratentorial. This was comparable to the study carried out by Suresh . [
In older children, infratentorial tumours are more common than supratentorial tumours [
Out of the 16 cases of posterior fossa tumours in our study, medulloblastoma (6 cases) was the commonest tumour followed by astrocytoma (5 cases). This is in contrast to most studies on pediatric posterior fossa tumours which state that the incidence of astrocytoma is higher than that of medulloblastoma in these cases [
Six cases of sellar region tumours were seen in our study, out of which 3 cases were craniopharyngiomas, one case each of pituitary adenoma, arachnoid cyst, and optic nerve glioma. This collaborates with the study of Laws and Sheehan, who have suggested that diagnosis of sellar region tumours in pediatric patients requires a multidisciplinary approach and detailed ophthalmic and neurological tests are critical in these cases [
Surgical resection is the main modality of treatment for most pediatric brain tumours. Depending on the tumour type, the goals of surgical management are to achieve a tissue diagnosis and reestablish normal CSF (cerebrospinal fluid) pathways, CSF diversion, tumour debulking, and complete tumour resection [
Astrocytoma was the most frequent tumour seen in our study followed by medulloblastoma. This is consistent with most studies in the literature [
The frequency of craniopharyngioma (11.4%) in the present study was high in our study but was comparable to various studies from India, Egypt, Japan, and Brazil [
The other tumours present in our study include pineoblastoma, meningioma, choroid plexus papilloma, pituitary adenoma, and tuberculoma which are comparable to various studies in the literature [
Statistical significance was not evaluated because of the very small sample size of the study.
To conclude, diagnosis of pediatric intracranial tumours is complex and requires a multidisciplinary approach for prompt management. Neuroophthalmic manifestations are common but can be easily missed in children with intracranial tumours. With increased survival rates, pediatric intracranial tumours with ophthalmic involvement can cause devastating long-term consequences due to vision loss or ocular motor abnormalities eventually leading to decreased quality of life.
An ophthalmologist should have a high index of suspicion for brain tumours especially in patients presenting with common ocular symptoms like diminution of vision, diplopia, and strabismus without any neurological symptoms. Ophthalmological evaluation and monitoring can also be helpful in these cases for preoperative planning and systematic treatment monitoring and for postoperative prognostication and multidisciplinary care of the patient.
We also conclude that in any child presenting to an ophthalmologist with additional signs and symptoms that could result from a CNS tumour, a thorough neuroophthalmic examination should be done. This can help in early diagnosis and prompt management of pediatric intracranial tumours and also helps in improvement of visual prognosis. Other salient features observed and concluded in our study include the following:
In pediatric brain tumours, male preponderance is seen which collaborates with many studies in the literature. Supratentorial location was more common in general, while in older children, infratentorial tumours are more common than supratentorial tumours Headache, vomiting, and cerebellar signs were the commonest neurological features; diminution of vision, diplopia, and strabismus were the commonest ophthalmic symptoms; while papilledema, ophthalmoparesis, and nystagmus were the most frequent ophthalmological signs Neurological manifestations of seizures, altered sensorium, and motor deficits were more frequently seen in supratentorial tumours, while cranial nerve involvement and ataxia were seen in infratentorial tumours. Ophthalmological manifestations including diplopia, strabismus, ophthalmoparesis, and nystagmus were more frequently seen in infratentorial tumours Astrocytoma was the commonest histopathological diagnosis followed by medulloblastoma, which again collaborated with various studies in the literature
The authors declare that they have no conflicts of interest.