Paediatric Plastics Flashcards
What is Craniosynostosis?
Premature fusion of one or more cranial sutures
What is Virchow’s law in relation to craniosynostosis?
Premature suture fusion results in cranial growth predominately parallel to sutures (rather than perpendicular).
Craniofacial Clefts
Definition of Cleft Lip
Cleft lip is a congenital abnormality of the primary palate involving the lip, alveolus, and hard palate anterior to the incisive foramen. If extending posterior to the incisive foramen, it is termed cleft lip and palate (CLP).
Definition of Cleft Palate
Cleft palate is aetiologically and embryologically distinct from CL and CLP, and represents a cleft of the secondary palate involving the hard palate (HP), posterior to the incisive foramen, and/or the soft palate (SP).
Classification of Cleft Lip
General
Unilateral or bilateral: based on the whether the vomer is attached to one of the palatal shelves (i.e. unilateral CP) or neither of them (i.e. bilateral CP).
Complete or incomplete.
Based on supposed inheritance pattern:
- Non-syndromic C +/- CP.
- Non-syndromic CP.
- Syndromic CL with or without CP.
- Syndromic CP.
Veau’s classification (1931)
A Incomplete cleft of secondary palate.
B Complete cleft of secondary palate.
C Complete unilateral cleft lip and palate.
D Bilateral cleft lip and palate.
Cleft lip/palate epidemiology
Most prevalent facial abnormality in the world. Incidence of 0.2–2.3 per 1000 births.
High: Asian 1:450
Intermediate: Caucasian 1:1000
Low: Black: 0.5:1000
Males (2:1)
Cleft lip congenital associations?
Trisomy 13 (Patau’s syndrome) and trisomy 21.
Waardenburg’s syndrome.
Van der Woude syndrome (associated with lip pits).
Environmental factors:
- Alcohol.
- Anticonvulsants (e.g. phenytoin).
- Folic acid deficiency.
- 13-cis-retinoic acid.
- Tobacco.
Normal facial development/ embryology
Face forms from five facial primordia: 1. Frontonasal prominence
2. Bilateral maxillary prominences
3. Bilateral mandibular prominences
Frontonasal prominence: Forehead, nose, and top of the mouth
Maxillary prominences: Lateral sides of the mouth
Lip formation occurs during weeks 4–7 of gestation
Cleft Lip Embryology
CL results from failure of fusion of the medial and lateral nasal prominences (or processes) with the maxillary processes during week 5 of gestation. If associated with impaired palatal shelf fusion, CLP will result.
Microtia
Prominent Ear
Types of Vascular Anomalies
Vascular Malformations
Vascular tumours
Types of vascular tumours