16. 4 clefts and 4 pouches
5th and 6th contribute to the 4th
Clefts provide âcovering to
structures of the corresponding
arch and pouch.
BRANCHIAL CLEFTS AND POUCHES
18. 3rd pouch- (ventral and dorsal wings)
ď§Ventral wing- thymus
ď§Dorsal wings- inferior parathyroid glands
ď§4th pouch
ď§Superior parathyroid gland
ď§Parafollicular C cells of thyroid gland
19.
20. ď§ Involves malformations of eyes, ears,
palate, and mandible
ď§ 2 main manifestations of âFirst Arch
Syndromeâ
⢠Treacher Collins Syndrome
⢠Pierre Robin Syndrome
21. ď§Mandibulofacial dysostosis
ď§Inherited AD
ď§Features
⢠Midface and mandibular hypoplasia
⢠Ear anomalies: microtia, anotia, stenotic
or atresia of EAC, malformation of
malleus and incus (CHL)
⢠Eye anomalies: coloboma of lower lids,
down-slopping palpebral fissures
⢠Cleft palate
22.
23. ď§ 3 Main features
⢠Micrognathia (small mandible)
⢠Glossoptosis (posterior
displacement/retraction of
tongue)
ď§ Cleft palate (U-shaped)
27. ď§Laryngeal stenosis
ď§Laryngoptosis (low position of larynx)
ď§Chondromalacia
ď§Double aortic arch
ď§Pulmonary artery sling
⢠Left pulmonary artery originates from
right pulmonary artery
⢠Slings around right main-stem bronchus
31. ď§Thyroglossal duct cyst
⢠7% of population
⢠Failure of ablation of TGD
⢠Anywhere from base of tongue to
upper mediastinum
⢠Typical finding
⢠Cystic lesion just below hyoid in
midline that moves with
deglutination and tongue protrusion
32. ď§May contain thyroid tissue
⢠Potentially the only functioning
thyroid
ď§Perform U/S or CT to look for thyroid
and to assess lesion
ď§Treatment â surgical
ď§May contain cancer
⢠1%
⢠Papillary carcinoma
33.
34.
35. ď§Lingual Thyroid
⢠Failure of decent of thyroid atopic
⢠90% of cases at the base of tongue
(lingual thyroid)
⢠4:1 = female : male
⢠Usually not noted until teenage or
young adult
⢠Asymptomatic (most cases);
dysphagia, airway compromise
⢠Reddish mass (well vascularized) at
base of tongue
36. ď§Hypothyroidism â 70% of cases
ď§2/3 cases â only functioning thyroid tissue
⢠Thyroid function study prior to treatment
ď§Treatment
o Asymptomatic â Monitor
o Symptomatic
⢠Excise +/- transplant tissue into
muscles of neck
⢠Radioiodine therapy (destroys all
thyroid tissue)
o Usually require lifelong thyroid
replacement
37.
38.
39.
40. ď§DiGeorge Syndrome
ď§Congenital absence of thymus and
parathyroids
ď§Partial deletion of chromosome 22
ď§CATCH -22
⢠Cardiac anomalies
⢠Abnormal facies
⢠Thymic aplasia
⢠Cleft palate
⢠Hypocalcemia
ď§Tetany and impaired cellular immunity (T-
cells)
44. ď§Results from failed obliteration of
branchial clefts
ď§2-3% are bilateral
ď§2nd cleft cyst is the most common type
ď§~95% of cases
45. ď§Contribute to the formation of
ď§Face
ď§mouth
ď§During the 5th weekâthe 2nd pharyngeal
arch enlarges and overgrows the 3rd & 4th
arches,forming the ectodermal depression
called cervical sinus
FATE OF BRANCHIAL ARCHES
46. ď§By the end of 7th week the 2nd to 4th
pharyngeal grooves and cervical sinus
disappeared, giving the neck a smooth
contour
47.
48. ď§ During the 4th & 5th weeks, head âneck
region of the human embryo exhibit
four branchial grooves or clefts on
each side.
ď§ The grooves separate the branchial
arches externally
ď§ Only 1st pair persists as the external
acoustic meatus
ď§ The other grooves normally obliterated
with cervical sinus as the neck
develop
BRANCHIAL GROOVES
49. ď§Branchial membranes appear in the floor of
branchial grooves
ď§These membranes form where the epithelia of
the grooves and pouches approach each other
ď§The endoderm of pouches and ectoderm of the
grooves are soon separated by mesenchyeme
ď§Only 1st branchial membrane becomes the
tympanic membrane, others obliterate
BRANCHIAL MEMBRANES
51. ď§ECTOPIC THYMIC TISSUE-
Thymus gland lies in the neck
ď§ECTOPIC PARATHYROID-
Inferior parathyroid may present at
bifurcation of common carotid artery
52. ď§BRANCHIAL FISTULA is an abnormal tract
opening both on the side of the neck and in the
pharynx (tonsillar fossa)
ď§ It is the result of the persistence of parts of the second
branchial groove and second pharyngeal pouch
ď§ It provides drainage for a lateral cervical cyst
(remnants of the cervical sinus) and is found most often
just below the angle of the jaw
53.
54. ď§BRANCHIAL OR LATERAL CERVICAL SINUSES
------- are relatively rare and almost always
open externally on the side of the neck. They
are due to a failure of the second branchial
cleft or groove to obliterate.
ď§ A blind pit or channel opens on the lower third of the
neck near the sternocleidomastoid muscle
ď§ There may be a periodic mucus discharge
ď§ A branchial sinus opening into the pharynx is rare, but
one may open into the tonsillar fossa as a result of the
persistence of part of the second pharyngeal pouch
55. ď§Branchial cyst -sinus persists as cyst along
anterior border of sternocleidomastoid
muscle.
If ruptures BRANCHIAL SINUS
56. ď§BRANCHIAL VESTIGE is rare; and seen
as cartilaginous or bony remnants of
branchial arch cartilages, which
normally disappear. It is seen on the
side of the neck in front of the lower
third of the sternocleidomastoid muscle
59. 1. Agenesis -congenital hypothyroidism
2. Ectopic thyroid -lingual, sublingual or
thoracic
3. Thyroglossal cyst -cystic remnant of
thyroglossal duct.
ď§ Always near or at the midline,
ď§ 50% are close to or just inferior to body
of hyoid bone.
ď§ Also found-at the base of tongue, close
to thyroid cartilage
4. Thyroglossal fistula -when thyroglossal is
connected to the outside by a fistulous
canal
Congenital anomalies of thyroid gland
& thyroid duct
60.
61.
62. ď§Anterior 2/3rd âformed by
fusion of Tubercular impar
&two lingual swellings
ď§Posterior 1/3rd âfrom the
cranial part of hypobranchial
eminence(copula)
ď§Post most part of the
tongue derived from 4th arch.
TONGUE :
63. ď§ANKYLOGLOSSIA(TONGUE-TIE)-frenulum of
the tongue extends to its tip.
ď§MACROGLOSSIA-
ď§MICROGLOSSIA
ď§CLEFT TONGUE
ď§BIFID TONGUE
ď§CONGENITAL CYST & FISTULA-remnants of
thyroglossal duct
Congenital anomalies of tongue
64.
65. ď§ Median cleft lower lip
ď§ Cleft upper lip
ď§ Oblique facial cleft
ď§ Macrostomia
ď§ Microstomia
FACE ANOMALIES: