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).
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
Congenital defect of the ear varying from normal ear features with reduced size to no external ear growth
Classification: Marx/Rogers
Grade I A smaller than normal auricle with all normal features of an ear
Grade II An abnormal auricle with some recognizable normal structures
Grade III An abnormal auricle with some non recognizable normal structures
Grade IV Anotia
Prominent Ear
5%. AD trait.
Features:
Poorly defined antihelical fold
Conchoscaphal angle >90 degrees
Conchal excess (can be determined by placing medial pressure along helical rim)
Types of Vascular Anomalies
Vascular Malformations
Vascular tumours
Types of vascular tumours
Benign:
Infantile haemangiomas
Congenital haemangioma
Malignant:
Kaposi sarcoma
KHE (Kaposiform haemangioendothelioma)
Angiosarcoma
Common Congenital Hand Malformations
Syndactyly: fused/webbed fingers
Polydactyly: extra fingers
Bradydactyly: abnormally short digit
Mirror Hand: symmetrical duplication of the upper limb
Central Hand deficiency: “cleft hand”, missing middle finger/central portion of hand
Symbrachydactyly: short, webbed or missing fingers
Clinodactyly: abnormal curvature of digit
Brachial Plexus Birth injuries
Erb’s Palsy (C5-6): Upper Trunk Injury
Extended Erb’s Palsy (C5-7)
Global/total brachial plexus injury (C5-T1)
Lower Trunk (Klumpke’s) Injury (C8-T1)
Classification of Nerve Injuries
Sunderland Classification
Neurapraxia (Sunderland type I)
Axonotmesis (Sunderland types II to IV)
Neurotmesis (Sunderland type V)
Avulsion
Definition of Toxic Shock Syndrome
Acute, life-threatening condition caused by bacterial toxins.
Most commonly associated with Staphylococcus aureus and Streptococcus pyogenes.
Clinical features of TSS
Cole and Shakespeare Criteria
o Fever >39.0°C
o Rash
o Diarrhoea +/- vomiting
o Irritability
o Lymphopenia
Hyperaemia of conjunctivae, mucous membranes
Multi-organ system involvement:
o Mucous membranes: conjunctival, oropharynx hyperaemia
o CNS: alteration of consciousness level
o Muscular: myalgia
o Haematological dysfunction: thrombocytopenia
o Impaired renal/hepatic function
Paediatric focused history TSS
Onset, duration and progression of symptoms
Systemic symptoms
o Fever, sweats, rashes, D&V, increased work of breathing
Feeding, toileting
PMHx:
o Congenital, birth, neonatal Hx
o Development and growth Hx
o Immunisation
SHx
o Living arrangements
o Schooling
o Social services involvement
Management of TSS
Sepsis 6
o Urine output
o Lactate
o Blood cultures
o O2
o Antibiotics as per trust (Fluclox & Clindamycin)
o Fluids
Clean and dress burn wound
Escalation and involvement of PICU, general Paeds
IVIG/FFP in severe cases
Paediatric maintainance fluids, calculate for a 22kg child
Fluid: 0.9% NaCl + 5% Glucose +/- 20mmol K+
Holliday–Segar formula (bodyweight)
o First 10kg: 100 ml/kg/day
o Next 10kg: 50 ml/kg/day
o >20kg: 20 ml/kg/day
Over a 24‑hour period, males rarely need more than 2,500 ml and females rarely need more than 2,000
22kg child (1000 + 500 + 40 = 1540ml/day)
Causes of TSS
Staphylococcus aureus (TSST-1 toxin production).
Streptococcus pyogenes (exotoxins, e.g., SpeA, SpeC).
Pathophysiology of TSS
Superantigen mechanism: Toxins act as superantigens, leading to:
Massive T-cell activation.
Cytokine storm (IL-1, IL-6, TNF-α release).
Widespread inflammation, vascular leakage, and multiorgan dysfunction.
Complication of TSS
Acute respiratory distress syndrome (ARDS).
Disseminated intravascular coagulation (DIC).
Multiorgan failure.
Long-term sequelae in survivors (e.g., chronic fatigue, cognitive dysfunction).
