1. Case of the Year Lyn Callaghan Advanced Neonatal Nurse Practitioner
Wishaw General Hospital

2. Background Baby boy born by SVD at 40+2 weeks
Spontaneous onset of labour
No Infection risks
Crash Call at delivery due to Shoulder Dystocia
Baby born in good condition
Apgars 9/1 9/5
Mum’s second baby
Healthy pregnancy
Blood Group 0 Negative
Anti D during pregnancy

3. Newborn Examination Baby now 15 hours of age Mother – no concerns
Breast feeding well
Passed urine and meconium
Mother – no concerns
Midwife – feels baby intermittently tachypnoeic
On examination
Pale
No respiratory distress
Normal tone
Normal examination
Note baby becomes tachypnoeic on handling
Heart rate +/- 150 when settled

4. Do you think anything is wrong with the baby?
What do you think it could be?
What do you do next?

5. What happened next? Chased Cord bloods Repeated FBC and bilirubin
Informed mum of rationale
At Birth
Hb 15.5 HCT 0.56, WCC 19.0, Plat 204, Retics 5.5%
Bili 24.
Blood Group O pos
DCT Negative
At 15 hrs of age
Hb 8.2 HCT 0.23, WCC 20.1, Plat 143, Retics 7.1%.
Bili 28

7. Repeat FBC, Partial sepsis screen, DCT, Coagulation
Admit to NNU
Repeat FBC, Partial sepsis screen, DCT, Coagulation
Chest x-ray – normal
Echo – normal structure
Baby examines well
Pale
Tachypnoeic
Tachycardia
Mean BP normal
At 17 hrs of age
Hb 5.1, HCT 0.19, WCC 22, Plat 98. Retics 8.2%
Bili 29
CRP <6
Coag mildly deranged
Blood Group O pos
DCT Neg

8. By 21hrs of age baby developed abdominal tenderness and guarding
Stabalisation
Progress
TUT
Platlet transfusion
FFP
CRUSS
Abdominal X-ray
Abdominal Scan
By 21hrs of age baby developed abdominal tenderness and guarding
Differential diagnosis
Haemorrhage
? Bowel
?Liver
Transferred to Surgical Centre
MRI Confirmed Diagnosis

9. Haemangioma of Liver Can cause Treatment Heart failure Anaemia
Thrombocytopenia
Corticosteroids
Embolization
Hepatic Artery Ligation
Liver Resection

10. Congenital Hepatic Haemangioma
Hepatic tumours account for 1-5% of all Paediatric tumours
Hepatic Haemangioma is the third most commonest tumour of childhood
Occasionally diagnosed on antenatal scan
Often never detected
Rarely present as large abdominal mass
Cardiac failure due to massive atreriovenous shunting
Jaundice from compression of bile ducts
GI Bleeding
Fever/illness resembling systemic inflammatory process

11. What is a haemangioma?
A vascular birthmark caused by abnormal blood vessels in or under the skin
Most common benign tumour of the vascular endothelium in infancy
Can occur anywhere outside and inside of the body

12. What are the common types found on the newborn examination?
What do we tell the parents?

13. Salmon Patch
Stork Bite/Mark
Angels Kiss

14. Salmon Patch Pink maculae Dilated superficial capillaries
Commonly seen at nape of the neck, mid forehead and upper eyelids
Most common vascular malformation
Usually fades within a year

15. Strawberry Haemangioma
Bright red and sharply demarcated

16. Strawberry Haemangioma
Bright red vascular tumour
Dilated mass of capillaries
Usually protrudes above the skin
Can appear anywhere on the body
Can start as a flat red superficial lesion
Can increase in size over next year

17. Strawberry Haemangioma
Usually, growth is complete and involution has commenced by twelve months.
Half of all infantile haemangioma have completed involution by age five.
70% by age seven, and most of the remainder by age twelve
In more severe cases haemangioma may leave residual tissue damage.

18. Strawberry Haemangioma Complications
Psychological
Haemangioma near the eyes, nose, mouth, or on throat may interfere with vital functions and therefore require removal
Some may
break down and ulcerate
Bleed
Rarely can cause heart failure if large and blood being diverted into the excess blood vessels
Lesions next to bone can also cause erosion of the bone
The vast majority of hemangiomas are not associated with complications. Hemangiomas may break down on the surface, called ulceration. If the ulceration is deep, significant bleeding may occur in rare occasions. Ulceration on the deeper area can be painful and problematic. If a hemangioma develops in thelarynx, breathing can be compromised. A hemangioma can grow and block one of the eyes, causing an occlusionamblyopia. Very rarely, extremely large hemangiomas can cause high-output heart failure due to the amount of blood that must be pumped to excess blood vessels. Lesions adjacent to bone can also cause erosion of the bone

19. Strawberry Haemangioma Treatment
Oral corticosteriods
Smaller lesions sometimes injected
Propranalol
Timolol gel
Interferon
Vincristine
Surgical removal
Pulsed dye laser
For very early flat lesions
 Hogeling M et al. (August 2011). "A randomized controlled trial of propranolol for infantile hemangiomas".Pediatrics 128 (2): e259–e266. doi: /peds  PMID 

20. Port Wine Stain Nevus Flammeus
Usually present at birth and on face, but can occur anywhere
Permanent capillary angioma
Does not blanch with pressure
A vascular lesion – capillary malformation in the skin
Does not disappear over time
Also associated more rarely with Klipper Trenaunay Syndrome

21. Port Wine Stain Grows in proportion to general growth
Early stains are usually flat and pink – as child matures colour can deepen to a dark red or purple colour
In adulthood can become bumpy and raised

22. Port Wine Stain Complications
Psychological
Eye problems is on eyelid or next to eye
If over forehead and upper lip Sturge Weber Syndrome must be excluded
Spine abnormalities
Varicose veins
If the port-wine stain is around the eye or on the eyelid, a referral may be made to an optometrist or ophthalmologist for a test of the ocular pressures in that eye. If swelling occurs in the port-wine stain, it may cause vision problems,glaucoma, or blindness.
1 in 10 babies born with a port wine stain on the face will have problems with the eye or the brain
The majority of children have no complications
Brain abnormalities: are an uncommon association with port-wine stains of the face. This is due to extensive blood vessel abnormalities in the brain (the Sturge-Weber syndrome). Epilepsy and other problems may then develop.

23. Port Wine Stain Treatment
Some may improve over time
Laser treatment
variable results depending on skin colour
Works best in young children
Works best on smaller stains
Skin camouflage still common treatment
Some provided free on NHS
Support Groups
Birthmark Support Group
British Association of Skin Camouflage
Changing Faces
Generally, the paler the port-wine stain, the greater the chance of excellent results.
The laser can cause an unpleasant stinging. Therefore, younger children usually have laser treatment under a general anaesthetic or with sedation. Local anaesthetic is normally sufficient for older children and adults.
Up to ten treatments are needed depending on the size. Treatments are given about eight weeks apart. It is best to have completed all sessions before a child reaches the age of 5 years.
There may be pain, bruising and swelling over the treated area for a while after each session.
If lasers don't work, surgery is sometimes required

24. Mongolian Blue Spot Congenital Dermal Melanocytosis
Benign , flat birthmark
Irregular borders and shape
Commonest colour is blue, but can also be blue-grey, blue-black or deep brown
Normally disappears by yrs of age
Almost certainly by puberty
Important to document on record
Prevalent among
Mongolians
Asians
Malay
Polynesians
East African
Latin Americans
Turkish
The Mongolian spot is a congenital developmental condition exclusively involving the skin. The blue colour is caused by melanocytes, melanin-containing cells, that are usually located in the epidermis but are in the deeper region of the skin known as the dermis in the location of the spot.[6] Usually, as multiple spots or one large patch, it covers one or more of the lumbosacral area (lower back), the buttocks, sides, and shoulders.[6] It results from the entrapment ofmelanocytes in the lower half to two-thirds of the dermis during their migration from the neural crest to the epidermis during embryonic development.[6]
The condition is unrelated to sex; male and female infants are equally predisposed to Mongolian spot
The Mongolian spot is referred to in the Japanese idiom shiri ga aoi (尻が青い), meaning "to have a blue butt",[12][13] which is a reference to immaturity or inexperience. In Mexico, where its name is the "green butt" (Spanish: rabo verde) it is referred to as la patada de Cuauhtémoc, meaning "Cuauhtémoc's kick". In Korea, it is thought that the Mongolian spot is the bruise formed when Samshin halmi (Korean: 삼신할미), a shaman spirit to whom people pray around child birth, has beaten in order for a baby to go out from his or her mother.
In the comedy manga series Joshiraku written by Kōji Kumeta, as well as in its anime adaptation, the character Marii Buratei is known to have a Mongolian spot. In one sketch, a westerner notices the spot and mistakes it for child abuse, blaming "barbarian Japanese" and taking Marii to "safety" abroad, against her wishes.

25. Questions/Discussion