1. CASE MANAGEMENT JUNE 16, 2014 Dr. Lulubel F. Ilagan
Add approach to a child with Vascular birthmark
Dr. Lulubel F. Ilagan
Second year resident

2. General Data: JC 5 months old, female Filipino Catholic
Hagonoy, Bulacan

3. Enlarging Mass on Upper Lip
Vascular birthmark since birth?

4. History of Present Illness
At birth
- reddish,elevated skin lesion, less than 5mm with irregular borders on the lip, lateral to the philtrum, left
At 1 month
- size: 1 x 1 cm with elevation and thickening
-pediatrician: hemangioma.
3 months old
- 3.5 x 3 cm, violet-red, causing slight eversion of the left side of the upper lip.
- referred to a hematologist
If may time pa ka te bhey, suggest to have time line na emphasizng the size/change in character/color..para maskita anu nangyari after treatment..
Sa slides ma to para kasi cyang masyadong wordy..

5. Example* PREDNISONE Upon birth 1 month 2month 4month size color
character

6. History of Present Illness
- Doppler UTZ
- CBC (normal)
- prednisone at 2 mg/kg/day
4 months old
- Followed up
- Decrease in size to 2.5 x 2.0 cm, lighter in color
5 months
- Prednisone stopped due to ulceration
- Increasing in size

7. History of Present Illness
Advised to start propranolol
Day of Admission
2d Echo done

8. Review of Systems General: No weakness, no fever, no weight loss
Skin: No pruritus, no jaundice, no easy bruisability
HEENT: No headache, no dizziness, no hearing loss, no epistaxis, no
hoarseness, no excessive salivation, no blurring of vision
Neck: No pain, no limitation of movement
Respiratory: No cough, no colds, no difficulty of breathing
Cardiovascular: No palpitations, no easy fatiguability, no chest pain
Gastrointestinal:, no diarrhea, no hematemesis, no melena, no
hematochezia
Endocrine: No polyuria, polydipsia, polyphagia
Genitourinary: No discharge, no incontinence, no dysuria
Musculoskeletal: No limitation of movement, no joint pains
Nervous system: No behavioral changes, no seizure
Hematologic: No bleeding, No bruising

9. Birth and Maternal History
29-year old, G1P1 (1001)
Prenatal checkup - 2 months AOG; health center
Nonsmoker, nonalcoholic beverage drinker
Ultrasound – 3rd trimester: normal
Second trimester – cough for 5 days
Delivered term, spontaneous vaginal delivery at a
lying-in clinic
Newborn screening - normal

10. Nutritional History Mixed feeding for < 1 month  bottlefed
Complementary feeding - 4 months, cereals
At present: Bonna, 1:2 dilution, 4 oz q3h
Served daily with mashed potato, banana, or cereals at least twice/day
Given multivitamins and ascorbic acid daily.

11. Immunization History
BCG: 1 dose

12. Past Medical History No previous admission
No previous surgical procedure
No allergies

13. Growth and Developmental History
Gross Motor: good head control at 3 months; rolls over at present
Fine motor: Grasped objects at 4 months
Adaptive: social smile at 2 months of age.
Language: cooed at 3 months
Spacing paayus po..

14. Family History Father: fish retailer Mother: housewife29
Father: fish retailer
Mother: housewife
(+) DM – maternal grandmother
(+) HPN – paternal grandfather
(-) vascular lesions
(-) bronchial asthma 27

15. Environmental History
2-bedroom, well lit, well ventilated house
3 other household members
Purified drinking water
Garbage collected 3x times/day
No nearby factories
With exposure to cigarette smoke

16. Physical Examination
General Survey: Awake, not in cardiorespiratory distress
Vital Signs: BP: 90/60 CR: RR: 24
Temp 36.2 degrees Celsius
Anthropometrics: Weight: 6.4 kg (z score 0) Height:65 (z score 0)
Kulang HC, CC, AC

17. Physical Examination
Skin: Violet-red mass, nontender, 4.5 x 4 cm, with irregular borders on the upper lip, lateral to the philtrum
Head: Open anterior fontanel about 1 x1 cm, soft and flat, pink palpebral conjunctivae, anicteric sclerae, no alar flaring, violet-red mass, nontender,5 x 4 cm, with irregular borders on the upper lip, lateral to the philtum, no cervical lymphadenopathy
Paayus po spacing

18. Physical Examination
Chest: Symmetrical chest expansion, no retractions, clear breath sounds
Heart: Adynamic precordium, normal rate and rhythm, no murmurs
Abdomen: Globular, normoactive bowel sounds, soft, non palpable liver and spleen
Extremities: Warm, pulses full and equal, crt<2 sec
Spacing…

19. Physical Examination Neurological Examination
MSE: Calm, appropriately dressed
Cranial Nerves: pupils 2-3mm equally reactive to light, extra ocular muscles are full and equal, good masseter tone, can smile with no facial asymmetry. Patient turns to sound, can turn head side to side with good gag.
Motor: symmetric movement of all extremities
Reflexes: Deep tendon reflex 2+ on all extremities
Meningeals: No Babinski, no nuchal rigidity
Autonomics: No excessive sweating
Spacing and font please..

20. Salient Features 5 months old, Female
Violet-red mass on upper lip, increasing in size
Treated with hydrocortisone
Diba salient din na present cya since birth?
Hydrocort?or prednisone?

21. Working Impression
Hemangioma, left upper lip

22. Benign acquired disorders Genetic disorders Vascular birthmarks
lesions
Cutaneous
Ang color contrast ay delikadong hindi mkkikita sa maganda nating projector..
Nevus
Café-au-lait

23. Vascular lesions
Benign Acquired disorders
Pyogenic granuloma, angiokeratoma of mibelli, spider angioma
Genetic disorders
Blue-rubber bleb, Malfucci syndrome, Osler-Weber-Rendu disease
Vascular birthmarks
Malformation
Tumors
When you discuss this, huwag masyado magdwell sa mga specific dses na i-rurule out mo lang nmn..

24. Vascular Birthmarks Vascular tumors most common is hemangioma Vascular
Malformation
developmental error in blood vessel formation
do not regress but slowly enlarges
Vascular
Birthmarks

25. Please retype.  di talaga to kita. Yung pertinent lang namn
Please retype.  di talaga to kita.. Yung pertinent lang namn.. Tapos emphasize wat is present in the patient

26. MAJOR DIFFERENCES BETWEEN HEMANGIOMA AND VASCULAR MALFORMATIONS
CLINICAL
Variably present at birth
subsequent rapid growth
slow spontaneous involution

28. hemangioendothelioma
Vascular Tumors
Hemangioma
Tufted Angioma
Kaposiform
hemangioendothelioma
Diffuse Hemangioma

29. hemangioendothelioma Diffuse Hemangiomatosis
Vascular Tumors
Hemangioma
Tufted Angioma
Kaposiform
hemangioendothelioma
Diffuse Hemangiomatosis
Numerous hemangiomas are widely distributed

30. hemangioendothelioma
Vascular Tumors
Hemangioma
Tufted Angioma
Kaposiform
hemangioendothelioma
Diffuse Hemangioma
Very aggressive locally
Solitary, firm and deep purple
Do not regress spontaneously
Associated with Kasabach-Meritt syndrome

31. hemangioendothelioma
Vascular Tumors
Hemangioma
Tufted Angioma
Kaposiform
hemangioendothelioma
Diffuse Hemangioma
Histologically: “cannonball-like tufts of blood vessels
Slowly expanding dusky reddish-blue plaque with satellite
lesions (regression not expected) or solitary vascular nodule

32. hemangioendothelioma
Vascular Tumors
Hemangioma
Tufted Angioma
Kaposiform
hemangioendothelioma
Diffuse Hemangioma
Most common tumor of infancy (5% of all infants)
Proliferation of the vascular endothelium

33. J.C.
Precursor lesion at birth
Rapid growth
F>M

34. Classification SUPERFICIAL
- bright red, protuberant, compressible, sharply demarcated
DEEP
- more diffuse, less defined; cystic, firm overlying skin may appear normal or with bluish hue

35. PHASE Rapid expansion/ Proliferative phase Stationary phase Involution
- not correlated with size or site
- lesions on the lip seem to persist most of the time

37. Reminder:
If may literature sources ka, don’t forget to acknowldege author at the bottom..
Eg.
Ilagan et al 2010

38. PATHOPHYSIOLOGY Not elucidated
Proliferation of benign endothelial like cells that possess histochemical markers (GLUT-1, Lewis Yantigen, FcyRII and merosin - present also in placental BV

39. PATHOPHYSIOLOGY
PLACENTAL THEORY - explains programmed life cycle - genetic similarity ENDOTHELIAL PROGENITOR CELL (EPC) THEORY - increased circulating EPC - Human IH EPC injected to mice

40. SYNDROMES ASSOCIATED WITH HEMANGIOMA
PHACE SYNDROME GORHAM BANNAYAN-RILEY-RUVALCABA
posterior fossa brain defects, hemangioma, arterial malformations, cardiac, eye abnormalities (sternal raphe defects/ supraumbilical raphe)
cutaneous hemangiomas with massive osteolysis
macrocephaly lipomas, hemangiomas of autosomal dominant inheritance
Paayos ng spacing..

41. TREATMENT OBSERVE sign of regression: blanched or pale gray
60% involutes at 5 years old
90-95% - 9 year old
INDIVIDUALIZE
- Cochrane analysis “lack of well-designed clinical trials and the absence of US FDA-approved medications IH, limits ability to clearly identify the single best option”.
Paayos ng spacing..

42. HIGH RISK INFANTILE HEMANGIOMA
Location
Type
Growth Phase
Periorificial (eyes, nose, mouth *)
Segmental
Maximal Proliferation phase (usually 3-6 months)
Central Facial
Multiple
Lumbosacral
Rapidly proliferating
Genital

43. RATIONALE FOR TREATMENT
1. To prevent or improve functional impairment or pain
2. Prevent or improve scarring and/or disfigurement*
3. To avoid life-threatening complications

44. TREATMENT CORTICOSTEROIDS 1960s Prednisone 2-3 mg/kg/day
Regression evident after 2-4 weeks *
Response obtained: taper
Mechanism/pathophysio
When do u give? Is this first line?

45. TREATMENT INTRALESIONAL CORTICOSTEROID INJECTION Triamcinolone
1-2 mg/kg
Bleeding, skin atrophy, skin necrosis, infection, anaphylaxis, adrenal suppression

46. TREATMENT VINCRISTINE Vinca alkaloid microtubule inhibitor
1.0 to 1.5 mg/m2 weekly
Immunosuppression, neuropathy, alopecia
When do u give na? Mechanism of action?

47. TREATMENT INTERFERON antiangiogenic properties effective
- Spatstic diplegia (20%), neurotoxicity
When do u give na? Mechanism of action?

48. TREATMENT PULSED DYE LASER Very superficial lesions
Small (<4-5 cm), ulcerated hemangioma *
Ulceration, scarring

49. TREATMENT TOPICAL THERAPY Timolol 0.5% gel Superficial IH
No large clinical trials

50. TREATMENT PROPRANOLOL 2mg/kg/day * Excellent results
“exact indications, dosage, length of treatment, and long-term sequelae have not been thoroughly investigated”
Complications
- Bradycardia, Hypotension, Hypoglycemia
Last option mo na ba to? Or eto yung una after Pred? if yes, kindly rearrange the order.. You can put vincristine and intralesional chemo under”OTHER OPTIONS FOR TREATMENT”..

51. Hindi po kita ito.  please change font and color
Hindi po kita ito..  please change font and color.. Please correlate with patient

52. Hindi po kita ito.  please change font and color
Hindi po kita ito..  please change font and color.. Please correlate with patient

53. After propranolol, ano nangyari
After propranolol, ano nangyari? If u plan to do the time line as seen earlier sa HPI, you can show it again here to emphasize if size has decreased..

54. TREATMENT
SURGICAL
Pedunculated or exophytic hemangiomas in which scarring is highly probable
Chronically ulcerated hemangiomas causing pain
Will lead to disfigurement, bleeding
Ayusin ng font.. Indications for surgery?

55. RECOMMENDATIONS Facial hemangioma >/= 5cm
>/= 5 cutaneous lesions
Evaluate for PHACE
Hepatic ultrasound

56. You may want to add:
Ano nangyari after propranolol?nagtachy ba cya? Lumiit ba yung mass?
When do we rrefer hemangiomas to a hematologist?
When does it become alarming? VS saying to Mom na “observe” lng?

57. Pls do not forget to remind your moderator/reactor
Be early tom kasi may flagcem.. We cant help u na if mag8am na..
Good Luck!

58. THANK YOU