Child with Brainstem Glioma - History taking in Pediatrics

This is a very brief case presentation on how to approach a child with an Intracranial tumor. 

This is followed by a discussion on Brainstem glioma including common presentation, neurological manifestation, investigations including imaging findings, and management which can be helpful for viva questions.

Chief complaints

XYZ, is 10 years old child who presented with complaints of

  1. Gradual loss of weight since 6 mths 
  2. Cough, cold on and off since 6 mths 
  3. Loss of appetite since 3 mths 
  4. Vomiting since 3 mths Convulsions in the last 7 days

On General examination

pediatric general examination

The child is conscious and oriented, but apathic.  
Looks Grossly emaciated.
His current weight is just 13 kg 
HR-88/min 
RR-24/min 
BP-90/60 mm Hg

Systemic Examination

Eye examination - Positive finding only

pediatric eye examination

Ptosis is present. 
Squint in the left eye.

CNS examination - Positive findings only


Cranial Nerves

Lt eye medial squint, Ptosis is present nystagmus horizontal.

Tone

Hypertonia all 4 limbs.

Reflexes

DTR brisk Plantar equivocal B/L
Neck rigidity is present.

Other systemic examination

The systemic examination, of respiratory, cardiac, abdominal, and musculoskeletal examination is normal. ( except for gross wasting)

Investigation

Hematology and Basic labs

Normal CBS with lymphocytosis. 
Serum Sodium normal ( Rule out hyponatremia due to SIADH)

Fundus Examination

Disc margins appear blurred.

Lumbar Puncture (link1)

Guarded Lumbar puncture is done. 
The CSF shows predominant Lymphocytosis, with few RBCs. CSF protein and Sugar were normal

Imaging

An Emergency CT scan was ordered.
CT Findings of the child -

Axial CT shows hyperdense Space occupying leison involving brainstem (supratentorial) encroaching bilateral thalamus. The frontal horn of both lateral ventricles was dilated. There were no signs of midline shift.

The plan for MRI for detailed examination was made.

Discussion on Brain stem glioma - Speace occupying leison

Let us briefly review Brainstem Gliomas.  

These tumors occur in the region between the aqueduct of Sylvius and the fourth ventricle (brainstem) 

These tumors have a predilection to originate from the left side. Most are located in the pons; Brainstem gliomas are highly aggressive brain tumors.

Histopathology findings in Brainstem glioma

Histopathologically, brainstem gliomas can range from WHO Grade 1 to 4. 

  1. Grade 1, juvenile pilocytic astrocytoma, 
  2. Grade 2, diffuse astrocytoma, 
  3. Grade 3, anaplastic astrocytoma, and 
  4. Grade 4, glioblastoma multiforme. 

The grading is based on the presence of nuclear atypia, vascular proliferation, mitoses, and necrosis. Typically, the necrosis is seen in Grade 4.

Epidemiology

Brainstem gliomas have been reported to make up 9.4% of intracranial tumors in children. Brainstem gliomas account for approximately 10-20% of all childhood brain tumors.

Sex and Age distribution

Some reports have suggested a slight male preponderance. There is a  bimodal age distribution, peak incidence in the first decade, the second peak in the fourth decade. 


3 of 4 patients are younger than 20 years. Identified in children as young as 1 year.

Clinical features

The anatomic location of the SOL determines the neurological manifestations.

  1. Common presenting symptoms include failure to thrive, double vision, weakness, unsteady gait, difficulty in swallowing, dysarthria, headache, drowsiness, nausea, and vomiting. Rarely, behavioral changes or seizures. Older children may have deterioration of handwriting and speech.
  2. Pontine lesions usually present with any or all of the above signs and symptoms, depending on location and extension.
  3. Midbrain and lower brainstem - upper spinal cord signs and symptoms may be seen with the extension of the neoplasm to involve these structures.
  4. Tectal lesions typically present with headache, nausea, and vomiting.
  5. Hydrocephalus is a common presentation, especially for tumors in periaqueductal or fourth ventricle outflow locations.

Physical examination

  1. Common clinical findings constituting a triad of cranial nerve deficits, long tract signs and ataxia of trunk and limbs. 
  2.  Papilledema may be seen. 6th and 7th cranial nerves are involved commonly. 
  3. Facial sensory loss and a primary position, up beating nystagmus may be seen. 
  4. Involvement of cranial nerve III or IV suggests a mesencephalic component. 
  5. Tectal lesions may present with diplopia reflecting an internuclear ophthalmoplegia.

Differential diagnosis of brainstem glioma

  1. Arteriovenous Malformations 
  2. Ependymoma 
  3. Medulloblastoma 
  4. Metastatic Disease to the Brain 
  5. Tolosa-Hunt Syndrome

Investigations and Diagnosis

Laboratory Studies

Blood chemistry is not as helpful as a rule. Cerebrospinal fluid (CSF) examination is often important for differential diagnosis. 

The protein content of CSF may be elevated. Because of the risk of increased intracranial pressure due to obstructive hydrocephalus, caution in clinical and imaging assessment prior to lumbar puncture is stressed.

CT scan findings

CT imaging is an appropriate choice when MRI is not available, sedation for the procedure may not be feasible, or in emergencies. 

CT identifies calcifications, cystic changes, and displacement of the ventricular system.

MRI findings

MRI is a diagnostic test of choice. MRI can differentiate vascular malformations and other processes that can be misdiagnosed as a brainstem glioma on a CT scan

The typical MRI appearance of a brainstem glioma is an expansile, infiltrative process with low-to-normal signal intensity on T1-weighted images and heterogeneous high-signal intensity on T2-weighted images, with or without contrast enhancement. 

MR spectroscopy has been used to help distinguish between tumor and nontumor lesions in the brain. An elevated choline peak suggests neoplasm.

Treatment

Treatment of brainstem gliomas has been frustrating; at this point, new therapies have yielded little benefit over conventional treatment with radiotherapy alone. 

Adjuvant chemotherapy is not used in children because efficacy has not been proven. Data have suggested that pre radiation chemotherapy may improve survival in pediatric diffuse intrinsic brainstem gliomas. 

Bevacizumab is a VEGF receptor inhibitor, approved as monotherapy for recurrent glioblastoma multiforme in May 2009. Focal radiotherapy is the cornerstone of the treatment of brainstem gliomas and can improve or stabilize the patient's condition. 

Response to radiotherapy, in addition to the dose of radiation, depends on several variables such as tumor location, histologic type, and response to early treatment.

More on history taking

  1. A child with Global developmental delay
  2. An Infant with Jaundice
  3. A Child with Chronic lung disease
  4. A child with hepatosplenomegaly
  5. Brain Abscess in Children - LAQ

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