2. CLEFT LIP AND PALATE
Are the second most frequently occurring major
congenital anomalies (1:750-1:1000) ,club foot
being the most common.
More common in Asians and less frequent in
Africans.
Demographics
1. Male:female = 2:1
2. Left:right:bilateral = 6:3:1
3. a. Fetal exposure to substances including
phenytoin, steroids, phenobarbital,
diazepam, and isotretinoin.
b. Maternal smoking.
c. Parental age, especially advanced paternal age
d. Family history of clefting: the risk increase
with increased number of involved family
members.
4. Continued ...
e.Most cases are sporadic.
f. CLP is syndromic in <15% of cases.
NB:
*Critical developmental period of Lip and
palate : 4 to 6 weeks of gestation. An insult
during this period may affect fusion on one or 2
sides and result in clefting.
5. Classification of cleft lip and palate
A-According to the extent of cleft lip:
1-Microform cleft lip
2-Incomplete cleft lip : intact nasal sill.
3-Complete cleft lip: nasal sill and alveolus affected.
B-According to location of the cleft:
1-Unilateral cleft lip.
2-Bilateral cleft lip.
both of the above may be complete or incomplete or
microform.
6.
7. Continued ..
Cleft palate may be complete or incomplete.
It may be unilateral or bilateral.
It may occurs in assocaition with cleft lip(CLP) or
as an isolated finding(CP). Isolated cleft palate
are more likely to be syndromic.
8. Anatomic,functional and aesthetic
impact of clefts
CL
1-Disruption of continuity, orientation and quality of muscles.
2-The alveolus(dentition) and nostril floor are open in complete
cleft lip.
3- Nasal deformity(septal deviation and nasal tip distortion).
CP
1-Recurrent otitis media
2-Nasal regurgitation of foods
3-bad oral hygiene and poor feeding
4-Hypernasal speech and palatal fistula( late complication)
9. Management
1-History and examination( family history,associated
anomalies).
2-Explain surgical goals and timing of interventions.
3- Feeding/nutrition
i. Monitor for appropriate weight gain
ii. May require Haberman bottle or cross-cut nipple to reduce
the work of feeding,especially with CP.
4-Otolaryngology
i. Eustachian tube dysfunction often requires myringotomy
tubes.
ii. Repeated otitis media affects hearing and speech
development.
11. Timing of repair
Cleft lip:
1. 3 months of age, generally accepted
2. “Rule of Tens” (historical criteria) for suitability for surgery
a. 10 weeks old
b. 10 pounds
c. Hemoglobin10 mg/dL
3. May delay in syndromic patients with systemic concerns.
Cleft palate : Perform repair at 1 year of age,
speech milestone (first words).
May be delayed up to 18 months.
12. Repair technique
Goals of CL repair (Millard, Mohler, Randall..etc):
1.Lengthen medial lip element.
2. Reconstitute orbicularis oris.
3. Restore Cupid’s bow, aligning white roll and wet–dry
vermilion.
4. Correct nasal deformity.
5. Symmetry in bilateral
cases.
14. Continued ...
Goals of cleft palate repair (Veau Wardil,Von
Langenbeck)
1- cleft closure and oronasal separation.
2-Normal speech development.
3-Normal hearing development.
4-Normal facial growth.
15. Congenital melanocytic nevi
*Lesion must be present at birth to be classified as
congenital.
*Equal in males and females
*After birth, CMN grow in proportion to overall
increase in body size.
*Classified into:
A-small: less than 1.5 cm2
B- medium: between 1.5 and 20 cm2
C- Giant : > 20 cm2
17. History
1-appear initially as a hairless, pale brown flat lesion.
2-become hyperpigmented with time.
3-At 1-2 years: dark coarse hair
4-At 10 years: elevated and verrucous texture with
hyperkeratosis and hypertrichosis.
location : trunk then limbs then head and neck.
DDX: Other congenital pigmented lesions such as
epidermal nevus, nevus sebaceous, cafe au lait spot,
and Mongolian spot.
18. Risk of Malignant Transformation
Risk of Melanoma increase with increased size of the
lesion.
Small CMN: 1-5%
Giant CMN: up to 42 %
Remember: ABCD Screenig tool.
19. Management
*Consider malignant risk,cosmetic appearance ,possible scarring,
and patient psychology.
*non-excisional treatment like lasers, curettage, and
dermabrasion remove only the superficial part of the lesion.
Excisional options include:
primary excision and closure, serial excision, skin grafting, tissue
expansion and skin substitutes.
NB:To address the malignant potential, only complete
excision of the nevus can be recommended as a solution.
20. Vascular Anomalies
The currently accepted classification:
A. Hemangioma ( IH,congenital)
B. Vascular malformations
1. Arterial malformation
2. Venous malformation
3. Capillary malformation
4. Lymphatic malformation
5-Mixed malformation
23. Clinical stages of development of IH
1.Proliferating phase: 0-12 months:
Bright red , ,elevated, noncompressible plaque.
2. Involuting phase (12 months to 10 years):
The crimson color fades to dull purplish hue with
increased pallor of the skin.
3- Involuted phase (>10 years): Loose fibrofatty
tissue replaces previous parenchymal tissue.
24. Complications
1- Ulceration: The most common.
2-Infection: Usually intraoral or perianal HA.
3-Bleeding: respond to pressure.
4- Kasabach-Merritt syndrome: due to platelets
trapping.
5- Emotional distress
6- Skeletal distortion: due to pressure from large HA
7- High-output heart failure: large hepatic HA.
25. Treatment
1-Conservative management
2- Propranolol
3- Steroids
4- Laser: Pulsed dye LASER for pain reduction in
ulcerated HA and for residual color after
involution.
5-surgery: for 1- visaul obstruction
2-nasopharyngeal obstruction
3- auditory canal obstruction.
26. Vascular malformation
Common features:
1- grow proportionately with the child.
2- Always present at birth (distinguishing factor from
hemangiomas, which grow after birth
and are not often seen at birth).
3- male = females.
27. Capillary malformations ( portwine
stains)
# present at birth as pink or
red intradermal discolorations
that may involve small areas or
involve an entire limb or face.
# most commonly seen in the
face.
# May be part of Sturge–Weber syndrome( CM,DD,
retinal detachment, leptomeningeal malformation).
28. Treatment
Treatment options
a. Observation
i. Lesions do not regress (VS HA).
ii. Can progress to “cobblestone” appearance.
b. Pulsed dye laser: usually multiple session.
C-Surgical excision: For contour deformity.
29. Venous malformation
1-Bluish, soft, compressible lesion which swells on
dependent positioning.
2-Changes in hormone levels can cause
enlargement(puberty,pregnancy).
3- Location: Most common site is head and neck.
30. Treatment
a-Observation
b. Compression therapy
i. Useful for pain and edema
ii. Minimizes phlebothrombosis
c. Sclerotherapy: Most effective if performed on small
cutaneous lesions using ethanol.
i. Side effects: Blistering, full thickness necrosis, neural deficits
ii. Make sure no important neural structures are nearby if
using ethanol sclerotherapy.
d. Surgical excision: usually performed after sclerotherapy to
improve appearance or function.
