Development of the Pharyngeal

Pouches and Clefts

Dr J.O Ezugworie
MBBS, MSc, FWACS
Pharyngeal gut
 Pharyngeal Pouches
Introduction
• The primordial pharynx or pharyngeal gut widens
rostrally to join the stomodeum or primordial
mouth
• The pharyngeal gut narrows caudally to join the
oesophagus
• The endoderm of the pharynx lines the internal
aspect of the pharyngeal arches
• In between the pharyngeal arches, the pharyngeal
gut passes into a balloon-like diverticula – the
pharyngeal pouches
Pharyngeal Pouches and the Derivatives
 Introduction
• The pharyngeal pouches form by the 4th and
5th weeks of development
• There are 5 pairs of the pharyngeal pouches,
but the 5th is rudimentary
• The endodermal epithelial lining of the
pouches gives rise to a number of important
organs
 Derivatives of the Pharyngeal Pouches

 First pharyngeal pouch

 Tympanic (middle ear) cavity ; Auditory (Eustachian) tube
; Part of ear drum
• The distal portion of the first pharyngeal pouch widens
into a sack-like structure; the primitive tympanic or
middle ear
• The proximal portion remains narrow to become the
auditory (Eustachian) tube
• The endothelial lining of the tympanic cavity participate
in the formation of the ear drum or tympanic
membrane
Pharyngeal Pouch Derivatives
Derivatives of the Pharyngeal Pouches
Second pharyngeal pouch
 Palatine tonsil ; Tonsillar fossa
• The second pharyngeal pouch forms buds that
penetrate into the surrounding mesenchyme
• Mesodermal tissue later invade the buds to form the
primordium of the palatine tonsils
• The tonsil is infiltrated by lymphatic tissue during the
3rd and 5th months of development
• Part of the pouch remains in the adult as the tonsillar
fossa
Derivatives of the Pharyngeal Pouches
Third pharyngeal pouch
 Inferior parathyroid gland
 Thymus
• The distal end of the 3rd pharyngeal pouch has a
dorsal and a ventral wing
• The epithelium of the dorsal wing forms inferior
parathyroid gland by the 5th week of development
• Inferior parathyroid gland moves to rest finally on
the dorsal surface of the thyroid gland
Derivatives of the Pharyngeal Pouches
• The ventral wing forms the thymus
• The thymus migrates in a caudal and a medial
direction, pulling the inferior parathyroid with it
• The main portion of the thymus quickly moves
to the anterior part of the thorax (the final
portion) fusing with the opposite side
• The tail portion of the thymus sometimes
persists, embedded in the thyroid gland or as
isolated thymic nests
Derivatives of the Pharyngeal Pouches
• Growth and development of the thymus
continue until puberty.
• Thymus occupies a considerable space in the
thorax.
• It lies behind the sternum, anterior to the
pericardium and great vessels.
• It is atrophied and replaced by fatty tissues in
older persons.
Derivatives of the Pharyngeal Pouches
Fourth pharyngeal pouch
 Superior parathyroid gland
 Ultimobranchial body
 Parafollicular cells or C cells
• The distal end has a dorsal and a ventral wing
(like the 3rd pouch)
• The dorsal region of the 4th pharyngeal pouch
forms the superior parathyroid gland
Derivatives of the Pharyngeal Pouches
• The ventral wing gives rise to the
ultimobranchial body which later gets
incorporated into the thyroid gland.
• Cells of the ultimobranchial body give rise to
the parafollicular cells or C cells of the thyroid
gland.
• The parafollicular cells or C cells secrete
Calcitonin, a hormone regulating the calcium
level in the blood.
 Pharyngeal Clefts
• The ectodermal lined grooves or recesses separating the
pharyngeal arches externally
• 5th week embryo is characterized by the presence of four
pharyngeal clefts
• It was previously thought that the first pharyngeal cleft
contributed to the formation of the auditory meatus
(EAM).
• This has been disproved.
• Rather the external auditory meatus is formed by the
invagination of the surface ectoderm from the first
pharyngeal arch
Pharyngeal Clefts
 Pharyngeal Clefts
• First pharyngeal cleft disappears due to the
overgrowth of the second pharyngeal arch (which
forms most of the external ear).
• Second pharyngeal arch overlaps the 3rd and 4th
pharyngeal arches due to active proliferation of the
mesenchymal tissue.
• The 2nd, 3rd and 4th pharyngeal clefts lose contact
with the outside.
• This is because the 2nd pharyngeal arch merges with
the epicardial ridge in the lower part of the neck.
 Pharyngeal Clefts
• The 2nd, 3rd and 4th pharyngeal clefts form a
cavity lined by ectodermal epithelium called
the cervical sinus.
• The cervical sinus disappears with further
development.
• Thus all the clefts disappeared.
 Clinical Correlates
Ectopic thymic and parathyroid tissues.
• Glandular tissues from the pharyngeal arches
undergo migration.
• Accessory glands or remnants of the tissue
may persist along the pathway.
• Like thymic tissue may remain in the neck
 Clinical Correlates
Branchial fistulas
• The remnants of the 2nd, 3rd and 4th pharyngeal
clefts may remain in contact with the surface by a
narrow canal.
• This results from failure of the 2 nd pharyngeal arch
to grow caudally over the 3rd and 4th arches to
obliterate the clefts.
• Such fistula on the lateral aspect of the neck usually
provides drainage for a lateral cervical cyst.
 Clinical Correlates
Internal brachial fistulas
• These are rare – a small canal may connect the
cervical sinus to the lumen of the pharynx.
• This usually opens into the tonsillar region.
• Such a fistula results from rupture of the
membrane between the 2nd pharyngeal cleft
and pouch during development.
Lateral Cervical Cysts And Fistulas
 Clinical Correlates
• DiGeorge sequence (3rd and 4th pharyngeal
pouch syndrome)
• This includes hypoplasia or absence of the
thymus and or parathyroid glands with or
without CNS defects, abnormal external ears,
micrognathia (small jaws), and hypertelorism
(widely spaced eyes)
NOTE: Read up Neural crest cells and
craniofacial defects.
Lateral Cervical Cyst