“ Your face, my thane is as a book …” Macbeth

1. “Your face, my thane is as a book…”Macbeth Promoting multiprofessional education and development in Scottish maternity care

2. Objective: to detect the commonest abnormalities of the • face • eye • ear and hearing • mouth • to review their significance in the newborn and refer appropriately.

3. Structure of the talk • Face size vs Head • Symmetry and Position • Abnormal Eyes • Abnormal Ears • Nose and Lips • Abnormal Mouth • Abnormal Palate. The Face

4. The Face: Whole Picture

5. Symmetry and Position • Facial Palsy: Commoner afterforceps delivery Compression effect of the forceps on the branch of the facial nerve is often transient, and total recovery is usually anticipated Look for other injuries.

6. Symmetry and Position Asymmetric crying faces: Congenital lack of normal innervation to mid face Association with cardiac disease.

7. The Face: Trauma • Petechiae and bruising Congestion common in face to pubes presentation This bruising from bleeding under deep layers of scalp.

8. The Eye • Normal Lids • Overall size • Symmetry/position • Round iris • Pupil/lens • Red light reflex/retina. Normal eye

9. The Eyelids Swelling from delivery Bruising Sub-aponeurotic Infection.

10. The Eye • Coloboma: Is a defect in the closing of the globe Ranges in severity from a notch in the lower part of the iris, making the pupil pear–shaped, to defects behind the iris in the fundus.

11. The Eye With your ophthalmoscope set to +3 dioptres examine the cornea, iris and red reflex from a distance of approx 20 cm.

12. Red reflex An abnormal red reflex requires an urgent ophthalmologic opinion as it may represent a cataract or a mass of abnormal tissue.

13. The White Eye/Leukocoria Cataract • The lens does not allow light through to illuminate the retina • May be tiny central dots. Familial e.g. Galactokinase deficiency. retinoblastoma Retinoblastoma

14. The White Eye Primary persistent vitreous The solid tissue which formed the globe of the eye has persisted leaving permanent blindness and glaucoma.

15. The Eye Congenital glaucoma Obstruction to outflow of fluid from the eye Large hazy eye.

16. The Eye Blocked Lacrimal Duct Resolve <12 months >12 months refer to ophthalmologist for possible probing.

17. Dacrocystoceles

18. Squashed nose

19. The Nose Choanal Atresia: This can be one or both sides, bony or membranous Usually presents with cyanosis when not crying.

20. The Nose Snuffles Noses can be partially blocked by vernix, blood, milk, etc. Later low grade bacterial sepsis can be a problem.

21. The Ear • Size • Shape • Position • Abnormalities • Extra bits.

22. The Ear Position Normal Low set

23. Low Set Ears

24. The Ear Shape Branchial arch anomalies with unusual ears, small mandibles Oto-renal syndromes with unusual ears, fistulae in neck and renal problems.

25. The Ear Size Trisomy 18 with small abnormal and low set ears Potter’ syndrome: Renal problems leading to oligohydramnios and a squashed baby.

26. Ear position and shape As in CHARGE syndrome.

27. The Ear Pre-auricular pits or small fistulae. This baby’s mother had a similar pit but on the other side. There were no associated problems in this case.

28. The Ear Skin tags Simple tags are usually only cosmetic. Multiple or large tags can indicate hearing abnormalities or syndromes. Formal excision is usually recommended.

29. The Mouth Gums Tongue Palate Hard Soft.

30. Tongue tie Common Not usually divided unless interfering with feeding Possible late association with speech problems.

31. Ranulae Mucous inclusion cysts in the sublingual salivary glands on the floor of the infants mouth. They are usually small and will spontaneously regress. Larger ones need surgical removal.

32. Epstein's Pearls Nodules of epithelial cells resembling pearls just lateral to the midline on the hard palate (with a cluster at the junction of the hard and soft palate) are a normal finding.

33. Usually superficial in the gum and are removed to avoid maternal nipple problems. Neonatal teeth

34. The Mouth

35. Cleft Lip and or palate = 1 / 700 births 53% Isolated Cleft Soft (2°) Palate Cleft palate 80% Isolated deformity Familial 12% Syndromic 8% Affected sib = x 3.5 Two sibs = x 13.

36. Cleft PalateHigh Risk Family History Maternal exposure to teratrogens incl. Anti-convulsants, steroids, alcohol Syndromes, e.g.Trisomy13,18 Infant of diabetic mother (Χ2) Milk coming down nose during feeds.

37. Cleft palate

38. Cleft palate

39. Cleft uvula A small posterior cleft palate is often missed. The bifid uvula seen here points towards a sub mucous cleft.

40. Cleft lip and palateUnilateral.

41. Cleft lip and palate Bilateral.

42. Cleft Palate - Examination Spontaneous visualisation best Palpation with little finger of hard and soft palate Visualisation during gag HIGH RISK group only - use laryngoscope.

43. Any questions?

44. Summary Follow your instincts about a funny-looking face, but it may be familial! One abnormality may follow another – remember associations esp. hearing. If there is an increased risk of a cleft palate – formally view the soft palate. Kind thanks to Janelle Aby, MD  for many of the photographs