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CHAPTER 1

DEVELOPMENT OF THE HEAD AND NECK

Overview

Pharyngeal Arches

Pharyngeal Pouches, Membranes, and Clefts

Skull

Face

Palate

Tongue

Thyroid Gland

Clinical Correlates

Questions

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Overview

GENERAL INFORMATION

3 major germ layers form the initial developing embryo:

Ectoderm

Mesoderm

Endoderm

Mesoderm differentiates into:

Paraxial mesoderm

Intermediate mesoderm

Lateral plate mesoderm

Ectoderm gives rise to 2 layers:

Neuroectoderm

Neural crest

The head and neck are formed by:

Paraxial mesoderm

Lateral plate mesoderm

Neural crest

Ectodermal placodes

Most of the head and neck is formed from the pharyngeal arches

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Pharyngeal Arches

GENERAL INFORMATION

Start forming in the 4th week of development

Develop as blocks separated by pharyngeal clefts

Initially, 6 arches develop, but the 5th regresses

Arising from the endoderm are compartments called pharyngeal pouches that extend toward the pharyngeal clefts

Help form 4 of the 5 swellings of the face:

2 mandibular processes (pharyngeal arch)

2 maxillary processes (pharyngeal arch)

1 frontonasal prominence

Composed of:

External surface–ectoderm

Internal surface–endoderm

Central part–lateral plate mesoderm, paraxial mesoderm, neural crest

Skeletal components develop from the neural crest tissue

Muscular structures develop collectively from the mesoderm

Each arch is innervated by a cranial nerve that migrates with the muscles

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DERIVATIVES OF THE PHARYNGEAL ARCHES

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Pharyngeal Pouches, Membranes, and Clefts

GENERAL INFORMATION

Pharyngeal pouches—4 develop from endoderm

Pharyngeal clefts—each is a groove formed from ectoderm

Pharyngeal membranes—each is composed of tissue located between a pharyngeal pouch and a pharyngeal cleft; composed of external ectoderm, mesoderm and neural crest in the core, and an internal endoderm lining

PHARYNGEAL POUCHES

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PHARYNGEAL MEMBRANES

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PHARYNGEAL CLEFTS

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Skull

GENERAL INFORMATION

Skull is formed from:

Lateral plate mesoderm (neck region)

Paraxial mesoderm

Neural crest

Bony skull is formed by either of 2 mechanisms:

Intramembranous ossification

Endochondral ossification

Skull development is divided into 2 parts:

Viscerocranium—forms the bones of the face

Neurocranium—forms the bones of the cranial base and cranial vault and can be divided into membranous neurocranium and cartilaginous neurocranium

VISCEROCRANIUM

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SKULL FONTANELLES

Fontanelle

Time of Closure

Anterior fontanelle (bregma)

4–26 months

Posterior fontanelle (lambda)

1–2 months

Sphenoidal fontanelle (pterion)

2–3 months

Mastoid fontanelle (asterion)

12–18 months

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MEMBRANOUS NEUROCRANIUM

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CARTILAGINOUS NEUROCRANIUM

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Face

GENERAL INFORMATION

The face is formed mainly from neural crest, which makes 3 swellings that surround the stomodeum:

Frontonasal prominence

Maxillary prominence (from the 1st pharyngeal arch)

Mandibular prominence (from the 1st pharyngeal arch)

Lateral to the frontonasal prominence, 2 additional areas of ectoderm form the 2 nasal placodes that invaginate in the center to form nasal pits, creating ridges of tissue on either side of the pits:

Lateral nasal prominence

Medial nasal prominence

Fusion of the medial nasal prominences at the midline results in formation of the intermaxillary segment

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Palate

GENERAL INFORMATION

Formed by the:

Primary palate (intermaxillary segment)

Secondary palate (protrusions from the maxillary prominences)

Intermaxillary segment: the initial portion of the palate in development; contains the central and lateral incisors

Swellings of the maxillary prominence form shelves that project medially and are separated by the tongue

When the tongue no longer occupies the space between the palatal shelves, these processes fuse together to form the secondary palate

The primary and secondary palatal tissues all meet at the incisive foramen

Primary and secondary palates and the nasal septum fuse to form the definitive palate

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Tongue

GENERAL INFORMATION

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MUSCLES

Mesoderm from the occipital somites migrates anteriorly with the hypoglossal nerve to give rise to the extrinsic and intrinsic muscles of the tongue

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Thyroid Gland

GENERAL INFORMATION

Begins as an invagination at the foramen cecum

Descends inferiorly to its final position alongside the larynx

May be connected to the foramen cecum by the thyroglossal duct

Divided into 2 lateral lobes connected by an isthmus, from which a pyramidal lobe sometimes develops

Follicular cells are derived from the endoderm; parafollicular cells are derived from the ultimobranchial body

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Clinical Correlate

PHARYNGEAL POUCH ABNORMALITIES

ECTOPIC THYROID

Thyroid tissue in an aberrant location

Often the only thyroid tissue in the affected person

Susceptible to thyroid diseases like normal thyroid tissue

May occur anywhere along the migratory pathway of the thyroid gland beginning at the foramen cecum

Usually located at the base of the tongue (lingual thyroid)

Common locations include:

Lingual thyroid

Sublingual thyroid

Thyroglossal duct remnant

Anterior mediastinum

Prelaryngeal

Intralingual

Intratracheal

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PHARYNGEAL ARCH ABNORMALITIES

PIERRE ROBIN

First reported as a condition characterized by micrognathia, cleft palate, and glossoptosis

Now includes any condition with a series of anomalies caused by events initiated by a single malformation

In this micrognathia, the inferior dental arch is posterior to the superior arch

The cleft palate may affect the hard and the soft palate

Glossoptosis (posterior displacement of the tongue) may cause airway obstruction or apnea

The mandible usually grows fairly quickly during childhood

Multiple surgeries typically needed to correct the cleft palate and to aid speech development in children

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TREACHER COLLINS

A hereditary condition affecting the head and neck

Thought to be caused by a defect in the gene or chromosome 5

Children of an affected parent have a 50% risk of having the syndrome

Clinical manifestations include:

Downslanting eyes

Notching of the lower eyelids

Hypoplastic mandible

Hypoplastic zygomatic bones (zygomas)

Underdeveloped or malformed ears or “sideburns,” or both, are prominent

Common associated problems include:

Hearing loss

Eating/breathing difficulties

Cleft palate

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DIGEORGE SYNDROME

A rare condition caused by a deletion on chromosome 22, characterized by a wide array of clinical manifestations

Possible explanation: proper development is dependent on migration of neural crest cells to the area of the pharyngeal pouches

Although researchers described the syndrome as abnormal development of the 3rd and 4th pharyngeal pouches, defects involving the 1st to the 6th pouches have been observed

Possible associated problems include:

Congenital heart defects (such as tetralogy of Fallot, right infundibular stenosis, truncus arteriosus, aberrant left subclavian artery, and ventricular septal defect)

Facial defects (such as cleft palate, microstomia, downslanting eyes, low-set ears, or hypertelorism)

Increased vulnerability to infections (due to impaired immune system from the loss of T cells associated with absence or hypoplasia of the thymus)

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CLEFT LIP AND PALATE

Cleft lip: a gap in the upper lip

Cleft palate: a gap in the palate

Classification of the developmental defect is with reference to the incisive foramen:

Primary cleft

Secondary cleft

Complete cleft

Both cleft lip and cleft palate often cause difficulty with feeding and eventually speech

Surgery is the most common form of treatment for both

PRIMARY

Occurs anterior to the incisive foramen and results from a failure of the mesenchyme in the lateral palatine process to fuse with the intermaxillary segment (primary palate)

Common types of primary cleft:

Unilateral cleft lip

Unilateral cleft alveolus

Unilateral cleft lip and primary palate

Bilateral cleft lip and primary palate

SECONDARY

Occurs posterior to the incisive foramen; results from failure of the lateral palatine process to fuse together

Common types of secondary cleft:

Cleft in soft palate

Unilateral cleft in hard and soft palate

Bilateral cleft of hard and soft palate

COMPLETE

Extends through the lip, the primary palate, and the lateral palatine process; results from a failure of the lateral palatine process to fuse together with each other, as well as with the nasal septum and primary palate

Common types of complete cleft:

Unilateral cleft lip and cleft palate

Bilateral cleft lip and cleft palate

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