Q5
LONG ANSWER
10M
★ High-yield
Describe the development of the face and the palate. Add a note on cleft lip and cleft palate.
The Facial Primordia
The face develops between the 4th and 8th weeks from five primordia surrounding the stomodeum, all containing neural crest mesenchyme.
| Primordium | Number | Derivatives |
|---|---|---|
| Frontonasal prominence | One | Forehead, bridge of the nose |
| Medial nasal prominences | Two | Tip and septum of the nose, philtrum of the upper lip, premaxilla carrying the four incisor teeth, and the primary palate |
| Lateral nasal prominences | Two | Alae (sides) of the nose |
| Maxillary prominences | Two (1st arch) | Cheeks, lateral parts of the upper lip, most of the maxilla, secondary palate |
| Mandibular prominences | Two (1st arch) | Lower lip, chin and mandible |
- The lower lip forms from a single pair fusing in the midline, which is why cleft lower lip is very rare while cleft upper lip is common
- The nasolacrimal duct forms in the groove between the lateral nasal and maxillary prominences, which is why its congenital obstruction is common
Development of the Palate
- The incisive foramen is the landmark separating the primary from the secondary palate, and clefts are classified by their relation to it
- Palatal closure occurs about a week later in the female, which is why isolated cleft palate is commoner in girls while cleft lip is commoner in boys
Classification of Clefts
| Type | Position | Embryological failure |
|---|---|---|
| Cleft lip | Anterior to the incisive foramen | Failure of the maxillary prominence to fuse with the medial nasal prominence |
| Cleft of the primary palate | Anterior to the incisive foramen; involves the alveolus | Same as above, extending back |
| Cleft palate (isolated) | Posterior to the incisive foramen | Failure of the palatine shelves to fuse with each other |
| Combined | Both | Both mechanisms |
| Median cleft lip | Midline of the upper lip — rare | Failure of the two medial nasal prominences to merge; associated with holoprosencephaly |
| Oblique facial cleft | From the lip to the eye | Failure of the maxillary and lateral nasal prominences to fuse |
- Cleft lip is commoner in boys, more often on the left, and more often unilateral
- Incidence about 1 in 700; multifactorial, with phenytoin, valproate, smoking and folate deficiency as risk factors
Development of the Tongue
| Part | Origin | Nerve supply follows |
|---|---|---|
| Anterior two-thirds | Two lingual swellings and the tuberculum impar — first arch | General sensation — lingual (V3); taste — chorda tympani (VII, second arch) |
| Posterior one-third | Copula and hypobranchial eminence — third arch overgrows the second | Glossopharyngeal (IX) |
| Posterior-most part | Fourth arch | Internal laryngeal branch of the vagus |
| Muscles | Occipital myotomes that migrate in | Hypoglossal (XII) — except palatoglossus (vagus) |
- Taste in the anterior two-thirds is carried by the second arch nerve although the mucosa is first arch — a mismatch explained by the second arch contribution being overgrown
- The third arch overgrows the second posteriorly, which is why the vallate papillae lie in front of the sulcus terminalis but are supplied by IX
Development of the Teeth, Salivary Glands and Nose
| Structure | Origin | Note |
|---|---|---|
| Enamel | Ectoderm — ameloblasts of the enamel organ | The only dental tissue not of mesodermal origin; cannot regenerate |
| Dentine, pulp, cementum | Neural crest mesenchyme of the dental papilla and sac | Odontoblasts survive and can lay down secondary dentine |
| Parotid gland | Ectoderm | Encapsulated late, after the lymphatics form — which is why it contains lymph nodes |
| Submandibular and sublingual glands | Endoderm | Encapsulated early; contain no lymph nodes |
| Nasal cavities | Nasal placodes deepen into nasal pits, then sacs | Separated from the mouth by the oronasal membrane, which breaks down |
| Paranasal sinuses | Outgrowths of the nasal cavity | Only the maxillary and ethmoid are present at birth; the frontal and sphenoid develop in childhood |
- Two dentitions develop from the same dental lamina — 20 deciduous teeth from the 6th week and 32 permanent teeth from buds lingual to them
- Choanal atresia — failure of the oronasal membrane to break down; a neonate is an obligate nose-breather, so bilateral atresia causes cyclical cyanosis relieved by crying
- The frontal sinus is absent at birth, which is why frontal sinusitis does not occur in young children
Applied Aspects
- Cleft lip repair at about 3 months, cleft palate at 9 to 12 months — the "rule of tens" (10 weeks, 10 pounds, haemoglobin 10) is a traditional guide to fitness for lip repair
- Feeding difficulty is immediate — the infant cannot generate suction; special teats and positioning are needed, and nutrition must be secured before surgery
- Otitis media with effusion is almost universal in cleft palate, because the tensor veli palatini cannot open the auditory tube; hearing must be monitored
- Speech requires velopharyngeal competence; a cleft causes a nasal escape and needs long-term speech therapy
- Management is by a multidisciplinary team — surgeon, orthodontist, ENT surgeon, speech therapist, audiologist and psychologist — over many years
- Periconceptional folic acid reduces the risk, as it does for neural tube defects
- Dental development is disturbed when the alveolus is involved, and orthodontic care with alveolar bone grafting is needed around 9 to 11 years
- Choanal atresia presents as cyclical cyanosis in the neonate, relieved by crying, because the newborn breathes through the nose
- The nasolacrimal duct forms in the groove between the lateral nasal and maxillary prominences; its lower end is the last to canalise, which is why congenital obstruction with a watering eye is so common and usually resolves spontaneously
- Frontonasal dysplasia and holoprosencephaly represent a spectrum in which failure of the forebrain to divide is mirrored in the face — "the face predicts the brain"
- Antenatal ultrasound detects most clefts of the lip from about 18 weeks, allowing counselling and planned delivery in a centre with the necessary team
All questions in Systemic Embryology
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- Q5Describe the development of the face and the palate. Add a note on cleft lip and cleft palate. ★
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