Case of the Year

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

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

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

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

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

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 StainComplications • If over forehead and upper lip Sturge Weber Syndrome must be excluded • Spine abnormalities • Varicose veins • Psychological • Eye problems is on eyelid or next to eye

23. Port Wine StainTreatment • 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

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 3 - 5 yrs of age • Almost certainly by puberty • Important to document on record • Prevalent among • Mongolians • Asians • Malay • Polynesians • East African • Latin Americans • Turkish

25. Questions/Discussion