DisPONnect

1. DisPONnect A Case of Pontine Glioma The Medical City | Department of Pediatrics ASMPH Interns – Group 2

2. Outline • Patient Information and Data • Approach to Diagnosis • Course in the Wards • Diagnostics • Therapeutics • Prognosis and Complications • Biopsychosocial Aspect: Palliative Care

3. DisPONnect Patient History A Case of Pontine Glioma

4. Identifying Information Patient Name: SA Age: 8 years old Nationality: Filipino Religion: Roman Catholic Handedness: Right Admitted: November 15, 2013 Information: EC and RA, patient’s parents Good Reliability

5. Chief Complaint Inarticulation (Nabubulol at Nagba-babytalk)

6. Patient History

7. Patient History

8. Patient History

9. Patient History

10. Review of Systems

11. Past Medical History • Past Illnesses • No previous history of cancer, stroke, seizures, eye correction, pneumonia, PTB, cardiac disease, hypertension, diabetes, asthma, kidney or thyroid disease • Hospitalizations • Previously admitted for Dengue Fever in 2012 • Surgeries • No previous surgical procedures • Trauma • No history of trauma • Allergies • No allergies to food or medications • Medication • No current medication use

12. Family History • Patient is of Filipino descent from Maybunga, Pasig City • Bronchial Asthma in the maternal aunt • No family history of cancer, stroke, seizures, diabetes, hypertension, heart disease, allergies • Household Members: • Patient • Patient’s Siblings • Patient’s Parents • Patient’s maternal Aunt

13. History of Birth and Infancy Birth History Nutritional History Not breastfed Due to low maternal milk production Formula Milk: NAN HA, Gain, Lactum Weaned at 6mo of age Current Diet: meats, vegetables and fruits Preferences: sour foods (e.g. sinigangisda) • Born full term via normal spontaneous delivery to a 37-year old G3P3 (3003) • Birth Weight: 3.08kg • Good Activity, Good Cry • Attended by: OB-GYN • No perinatal or neonatal complications

14. 24-Hour Food Recall

15. Immunization History

16. History of Childhood Developmental History Personal and Social History Grade 3 Student Above average performance (6th honor) Favorite Subject: Science and English Hobbies: Spend time with friends, singing and dancing Has shown interest in the opposite sex, but has no crushes • Gross Motor • Able to do backward heel to toe walk • Fine Motor • Able to draw a complete person • Can write fairly well • Language • Can add and subtract • Can distinguish between left and right • Personal/Social • Can dress self completely

17. Environmental History • Residence: 1-story cement structure • Maybunga, Pasig City • Electricity: Meralco • Water: Manila Water Company, Inc. • Near to major roads, but not near any factory • No exposure to tobacco, toxins or environmental hazards • Waste: Daily, not segregated

18. Stakeholder Analysis

19. DisPONnect Physical Examination A Case of Pontine Glioma

20. Physical Examination Anthropometrics Weight: 34.5kg Z-score (0,2) Height: 138cm Z-score (0,2) BMI: 18.11kg/m2 Vital Signs BP: 118/76mmHg HR: 82bpm RR: 20cpm Temperature: 36.5C Pain: 0/10 General Survey Awake, Alert Not in CardioRespiratory Distress GCS 15

21. Physical Examination • Eyes: • Anicteric sclerae, pink palpebral conjunctivae, no cataracts or discharge • Skin: • Fair color, no rashes, good skin turgor, hair evenly distributed, nails with no clubbing • Ears: • No visible mass or lesion, no discharge, no auricular tenderness, patent canal, intact tympanic membrane with cone of light

22. Physical Examination • Nose • No deformities, no nasal discharge, no nasal congestion • Throat • Lips moist and pink, no cleft lip or palate, no tonsillopharyngeal congestion • Neck • Flat neck veins, no cervical lymphadenopathy

23. Physical Examination • Chest/Lungs • Symmetric chest expansion, no retractions, clear breath sounds, no rales, no wheezes • Cardiovascular • Adynamic precordium, normal rate, regular rhythm, good S1/S2, no murmurs, heaves or thrills • Abdomen • Flat, no previous surgical scars, normoactive bowel sounds, no masses palpated, no organomegaly, no tenderness

24. Physical Examination • Genitalia • Grossly female genitalia, no discharge • Extremities • Full and equal pulses, no edema, no cyanosis, CRT <2 seconds

25. Neurologic Examination

26. Neurologic Examination

27. Neurologic Examination

28. Neurologic Examination ++ ++ ++ ++ ++ ++ No Flaccidity or Rigidity No Atrophy or Hypertrophy ++ ++

29. Neurologic Examination • Cerebellar • Dragging gait on the left • Dysdiadochokinesia: Left • Dysmetria: Left • Babinski: Bilateral • Meningeal signs • Negative Kernig’s and Brudzinski’s sign • No neck rigidity

30. Salient Features SUBJECTIVE OBJECTIVE Stable VS, GCS 15 Shallow nasolabialfold, right Dysarthria Absent gag reflex Left-sided motor weakness (4/5) (+) Dysdiadochokinesia, dysmetria, left (+) Dragging gait (+) Babinski, bilateral • 8-year old female • No history of neurologic disease • 3 week history of right-sided facial weakness • 6 day history of drooling, dysphagia and slurred speech • Left-sided weakness • Unstable gait

31. DisPONnect Approach to Diagnosis A Case of Pontine Glioma

32. Neurologic Diagnosis

34. Stroke in the Young What is the Lesion?

35. Arteriovenous Malformation What is the Lesion? • Abnormal shunting of blood  expansion of vessels and a space-occupying effect or rupture of a vein and intracerebral bleeding • May remain asymptomatic throughout life but can rupture and bleed any time • History of ipsilateral seizures and migraine-like headaches • Ruptured AV malformation: severe headache, vomiting, nuchal rigidity, progressive hemiparesis, and seizure

36. Aneurysm What is the Lesion? • Usually asymptomatic • Located at the carotid bifurcation or on the anterior and posterior cerebral arteries rather than the circle of Willis. • Results from a congenital weakness of the vessel • Ruptured aneurysms: intense headache, nuchal rigidity, coma, intracerebral hemorrhage and progressive hemiparesis

37. Meningitis What is the Lesion? • Acute infection of the central nervous system (CNS) • May present acutely, subacutely and chronically (>1week) • Often preceded by fever, respiratory or gastrointestinal symptoms, followed by nonspecific signs of CNS infection such as increasing lethargy and irritability • Systemic infection + meningeal symptoms, seizures and altered mental status

38. Brain Lesion What is the Lesion? • Most common in children 4 -8 years old • Causes: emboli, meningitis, chronic otitis media and mastoiditis, sinusitis, soft tissue infection of the face or scalp, orbital cellulitis, dental infections, penetrating head injuries, immunodeficiency states, and infection of ventriculoperitoneal shunts • 80% of abscesses are found in the frontal, parietal and temporal lobes • Clinical presentation: low grade fever, headache and lethargy  vomiting, severe headache, seizures, papilledema, focal neurologic signs (hemiparesis), coma • Cerebellar abscess: nystagmus, ipsilateral ataxia and dysmetria, vomiting, and headache

39. Primary CNS Lesion • Metastatic Lesion What is the Lesion? • 2nd most frequent malignancy in childhood • Higher incidence in children >7 years • Progressive Symptoms • Brainstem tumor effects: motor weakness, cranial nerve deficits, cerebellar deficits, and/or signs of increased intracranial pressure • Uncommon • Primary neoplasia: ALL, lymphoma, neuroblastoma, rhabdomyosarcoma, Ewing sarcoma, osteosarcoma, and clear cell sarcoma of the kidney

40. Is There a Lesion? • Yes!

41. Baby Talk and Slurring of Speech Dysarthria • Dysarthria: disorders in articulating speech sounds • Vs. Dysphonia • Vs. Dysprosody • Vs. Dysphasia • Motor paralysis of organs of articulation

42. Dysarthria Dysarthria Cause of Dysarthria • Drooling + Dysphagia • Swallowing Problem • Absent Gag Reflex CRANIAL NERVE IX and X Palsy Dysarthria: http://trialx.com/curetalk/wp-content/blogs.dir/7/files/2011/05/diseases/Dysarthria-1.jpg

44. Central Facial Nerve Palsy, Right • Possible Location of Lesion: • Left Corticobulbar Tract • Above the Facial Nucleus (located at the Pons)

45. Left-Sided Weakness • Corticospinal Tract • Cerebral Cortex • Mesencephalon • Pons • Medulla • Spinal Cord • Contralateral lesion above decussation

46. Cerebellar Signs • Unsteady gait • Dysmetria, Left • Dysdiadochokinesia, Left • Possible Locations: • Cerebrum • Cerebellum • Midbrain • Pons • Midbrain http://www.asn.org/neurographics/3/2/1/2.shtml

47. Cranial Nerves and the Brainstem • CN involvement • Above Nucleus: Contralateral • At Nucleus and Below: Ipsilateral Manifestations • Corticospinal Tract • Contralateral weakness

48. Pontine Lesions • Cranial Nerve Nuclei • Abducens nerve (CN VI) • Trigeminal nerve (CN V) • Cochlear and the lateral and superior vestibular (CN VIII) • The superior and inferior salivatory nuclei and the lacrimal nucleus (cranial nerves VII and IX) • Fiber Tracts • Corticospinal, corticobulbar, and corticopontine, spinocerebellar, spinothalamic, lateral tectospinal, rubrospinal, and corticopontocerebellartracts

49. Localization Brainstem Lesion, Possibly Pontine

50. DisPONnect Course in the Wards: Diagnostic A Case of Pontine Glioma