• History & Exam 
    - A 5 year old boy presents to the ED with a 1 week history of vomiting and lethargy. 
    - Past medical history is non-contributory. 
    - Physical examination: 
       (+) Parinaud's syndrome 
       (+) Papilledema

    Figure 1. A non contrast CT Head reveals non-communicating hydrocephalus.

    Figure 2. Post-contrast T1-weighted MR-imaging of the brain reveals a large pineal region mass.

    1. Parinaud’s syndrome is characterized by all of the following EXCEPT:

    2. In managing this patient’s hydrocephalus, I would prefer to:

    3. You have decided to proceed with surgery to resect this lesion. Which approach would you utilize?

    4. Which of the following describes you?

     5. I practice in one of the following locations.

    6. Please add any suggestions or comments regarding this case:

    • ETV+endoscopic tumor biopsy first, then, think about wether the tumor should be resected directly based on pathology of the tumor.
    • Good case, I would get a biopsy first to r/o germinoma
    • This is a pineal tumor and the cleavage more apropiate for the localitation is the transtentorial occipital aproach, without shunt because surely after surgery the hydrocephalus remited
    • First, I'd solve the hydrocephalus with an endoscopic third ventriculostomy and biopsy of the lesion through the same approach; depending on the histological report I'd proceed with resection or radiosurgery.
    • tough case
    • case should have included some vascular studies.
    • Immediate VP shunt is what is currently available. And due to the large patients load with limited resources, almost all the patients undergoe vp shunt prior to definitive treatment. In most cases the patients end up with empirical radiotherapy.
    • programmable shunt
    • I would like to see the results of tumoral marks.
    • Primero tomaría marcadores tumorales en sangre y liquido y según sus resultados decidiría procedimiento a seguir es decir si biopsia o resección de tumor si es un germinal tomaría biopsia y dejaría una vetriculostomia si sangra en la toma de biopsiá endoscopios esperando limpie liquido cera lo raquídeo aclare para intentar una 3er vetriculostomia eventualmente si no funciona una válvula presión media programable. Sino es un germinal mi abordaje seria supra cerebeloso infra tentorial
    • No
    • I would get CSF and serum BHCG, AFP at time of EVD placement.
    • totall resection is my objective
    • this is a good case for discussion & revisiting the ideas in the today's context
    • Some interesting questions were not asked: role of tumor markers/ endoscopic biopsy during ETV/ role of radiotherapy without resection in cases of germinoma and non-germinomatous malignant germ cell tumors/ staging with spinal MRI...
    • After ETV IF PATIENT shows significant improvement, blood test should be donwe to acertain nature of tumor.
    • If the tumor markers are positive, chemotheraphy and radiation therapy are instituted following endoscopic third ventriculostomy. If the tumor markers are negative, endoscopic tumor biopsy is done in addition to ETV.
    • The first order of management should be to manage this patients hydrocephalus. My approach would be to perform an ETV and at the same time obtain an endoscopic biopsy. If definitive surgical management is warranted following the biopsy results, then an occipital transtentorial approach would allow for good access to the tumor. I prefer to approach pineal tumors just behind the motor cortex to try and avoid a visual field cut from retraction of the occipital lobes
    • Endoscopic biopsy is feasible, however would be difficult to obtain a complete resection endoscopically. Therefore open resection best (Occipital transtentorial)
    • Good subject for a review.
    • High presión shunt
    • I think that management should be EVD for immediate symptoms. Get blood and CSF labs for pineal region tumors (AFP, HCG), then ETV and endoscopic biopsy. If the biopsy shows pathology that would benefit from resection then I would take a supracerebellar infratentorial approach because the internal cerebrals look elevated on MR.
    • I would use an anterior trans-choroidal approach.
    • I would use an anterior trans-choroidal approach.
    • Supracerebellar, infratentorial approach is also one alternative
    • The options in Question 3 seems to be a big leap! I would do a 3rd ventriculostomy & get serum & CSF markers while doing the 3rd ventriculostomy. Then via a 2nd more anterior burr hole, can get a biopsy with the endoscope. Then depending on the markers or biopsy results, patient may just need radiation (if Germinoma). If all the preceding is non-diagnostic or for example Teratoma, then open surgical resection would be appropriate in my (non-expert!) opinion.
    • Need serum and CSS markers for HCG and afp
    • The questions seem to oversimplify the options a bit: in my world (full-time pediatric NS in private/academic setting)the most common first procedure is ETV with biopsy. We would also start with blood markers and add CSF markers when doing the ETV. Sequence is markers first, then ETV/BX. If germinoma no surgery (just chemo. If malignant non-germinomatous germ cell tumor then chemo and consider surgery if partial/no response. Avoid shunts in germ cell tumors: they are the tumor most likely to implant in the abdomen successfully.
    • It is a cystic non invasive mas it could be removed by any of the mentioned ways
    • if hydrocephallus does not resolve vp shunt. ref to oncologist depending on histopathology.
    • I prefer to do csf study and EVD.Do immediate surgery-tumour excision/tumero-reduction by supracerebellar-infraten approach using microscope.In my establishment in Private practice I am yet to have endoscope equipments.After bx report I will decide radiation/CT as per advise of radiation oncologist/oncologist.
    • I prefer to do csf study and EVD.Do immediate surgery-tumour excision/tumero-reduction by supracerebellar-infraten approach using microscope.In my establishment in Private practice I am yet to have endoscope equipments.After bx report I will decide radiation/CT as per advise of radiation oncologist/oncologist.If followup demands Shunt i will do it before removal of EVD.
    • An excellent way to examine our knowledge about some topics with this kind of exercise. Congratulations.
    • I will place a medium pressure shunt tube, since it may be Pinealoblastoma, i will exercise restraint will biosing the lesion as to the haemastasis
    • I will approach this tumour transcollosally and follow for hydrocephalus....if develops medium pressure VP shunt will be placed...
    • Nice exercise for all
    • thanks
    • What about tumor marker issues?
    • During endoscopic third ventriculostomy it's possible to perform a biopsy and a cyst punction and analyze CSF pineal tumor's markers
    • Actually, I would like to choose transcollosal transfornix approach since the tumor located completely in 3rd ventricle.
    • I prefer to place external drainage and perform surgery at the same session.
    • Thank you for your nice presentation for education.
    • the need of pre surgical endoscopic third ventriculostomy depends on the possibility of the institution of performing an early surgery
    • In-line Codman-Hakim Programable set at 10 cm of H20.
    • this case is intesting and i have operated few such cases. you can see one theses cases on www.drnazirahmad.com
    • In the process of doing VPS with filter i will do CSF study for malignant cells and tumour marker.If It proves radio-sensitive lesion I do command Radiation therapy/RS.
    • Would appreciate expert reviewer to discuss case for learning purposes.
    • For question 2 would do the endoscopic third but would also leave a drain. If planning to do surgery it is better to have control and know that you can remove or add CSF during the procedure and keep the ICP where you want it. Drain can be clamped and then eventually removed post-op.
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