组织学与胚胎学：颜面部的发生

1、正常胚胎发生过程正常胚胎发生过程畸形畸形 (异常发育异常发育)胚期胚期胎期胎期第第八八周周p4w in embryonic stage：内胚层 中胚层 外胚层branchial arch: 22-29dNeural crest cells migrating into the facial region beneath ectoderm.背腹走向，左右对称，6对branchial arch: 22-29d第1鳃弓： 上颌突-上颌骨,颧骨, 颞骨鳞部 下颌突-下颌骨,砧骨,锤骨第2对鳃弓镫骨,颞骨茎突,舌骨小角,舌骨体上段第3对鳃弓舌骨大角,舌骨体的下段第4,6对鳃弓甲状软骨,环状软骨, 勺状软

2、骨,角状软骨,楔状软骨 branchial cleft：5对 Pharyngeal pounchp1 咽囊咽囊: 外侧份 鼓室 内侧份 咽鼓管p2 咽囊咽囊: 腭扁桃体上皮p3 咽囊咽囊: 腹侧份 胸腺 背侧份 下一对甲状旁腺p4 咽囊咽囊: 腹侧份 退化 背侧份 上一对甲状旁腺p5 咽囊咽囊: 后鳃体 滤泡旁细胞鳃弓鳃沟咽囊鳃膜鳃器branchial apparatus：生物进化过程的重演 (4-5w)p 奇结节奇结节 Tuberculum impar ：1鳃弓腹内侧p 侧舌膨大侧舌膨大 Lateral lingual swelling：1鳃弓腹内侧p 联合突联合突 Copula：2,3,4

3、鳃弓（舌根）腹内侧p 界沟界沟 Terminal sulcus：舌体与舌根融合处留有的“V”形沟 p 盲孔盲孔 Foramen cecum：甲状舌管的起始端4w末末舌体上皮来自口凹外胚层口凹外胚层，舌根上皮来自咽咽壁内胚层壁内胚层，舌内结缔组织来自鳃弓间充质。口腔底的外胚层口腔底的外胚层咽壁的咽壁的中胚层中胚层奇结节与联合突之间，内胚层细胞增生形成的细胞团奇结节与联合突之间，内胚层细胞增生形成的细胞团 甲状舌管甲状舌管thyroglossal duct：向咽腹侧深部生长，形成一盲管向咽腹侧深部生长，形成一盲管 第第11-12周，甲状腺滤泡出现，分泌甲状腺激素。周，甲状腺滤泡出现，分泌甲状腺激素

4、。奇结节奇结节甲状腺原基甲状腺原基4w末末舌盲孔舌盲孔6wpPrimordia： 5 prominences额鼻突 frontonasal prominence 上颌突 maxillary prominences下颌突 mandibular prominences口凹 stomodeum 口咽膜 oropharyngeal membrane: 24d下颌下颌突突4w额鼻突额鼻突上颌突上颌突 下颌突下颌突 4w上颌突下颌突5-6w6w鼻板2m额鼻隆起上颌隆起下颌隆起融合的内侧鼻隆起Fused median nasan prominence: 人中Philtrum 正中的上颌骨及牙龈Premaxi

5、llary part of the maxilla and its associated gingiva 原发腭The primary palatepPrimordia： 正中腭突正中腭突Median palatine process primary palate 外侧腭突外侧腭突Lateral palatine processes secondary palate 6w6-7w切齿孔切齿孔 Incisive foramen硬腭硬腭 Hard palate软腭软腭 Soft palate 左右上颌突内侧面间充质增生，向原始口腔内长出的一对扁平突起内侧鼻突融合处内侧面的间充质增生，形成突向原始口

6、腔的突起 nasal septum额鼻突和内侧鼻突的外胚层和中胚层组织增生p第第2,3,4,6 对鳃弓对鳃弓 心上嵴Epicardial ridgep颈窦颈窦 Cervical sinusp颈囊肿颈囊肿 Cervical cyst 颈瘘颈瘘 Cervical fistula4-5w肢芽肢芽Limb buds：4w末中胚层芯和外胚层帽手板/足板 指/趾放线 蹼状手6w7-8w凋亡凋亡 Apoptosis describes the controlled dying of cells which contrast with necrosis. Die at predictable time and

7、 places during development.6w5w手手/足板足板指指/趾放线趾放线体壁中胚层体壁中胚层 软骨内成骨 四肢骨体节中胚层体节中胚层 成肌节 肌肉脊神经长入肢体脊神经长入肢体Rotation ( 7w)Upper limb: 90laterallyLower limb: 90medially6wp唇裂唇裂 Cleft lip 上颌突未与同侧的内侧鼻突融合p正中唇裂正中唇裂 median cleft lip: 两侧的内侧鼻隆起融合异常 双鼻 bifid noseSonic hedgehog gene (SHH gene)p面斜裂面斜裂 Oblique facial clef

8、t 上颌突与同侧的外侧鼻突未融合p腭裂腭裂 Cleft palate 因外侧腭突未与正中腭突融合前腭裂 或左右外侧腭突未能在中线融合正中腭裂 全腭裂p甲状舌管囊肿及瘘甲状舌管囊肿及瘘 Thyroglossal duct cysts and sinuses 甲状舌管残留，舌骨下方、颈部正中p颈囊肿和颈瘘颈囊肿和颈瘘 Cervical cyst and fistula: 颈窦未完全闭锁所致p四肢畸形四肢畸形 limb abnormalities 短肢 海豹肢 (反应停)澳大利亚：尼克胡哲四肢畸形四肢畸形 limb abnormalities 多指/趾畸形多指畸形多趾畸形四肢畸形四肢畸形 limb

9、abnormalities 并指/趾畸形Sympus人鱼Case 1：The mother of a two-year-old boy consulted her pediatrician about an intermittent discharge of mucoid material from a small opening in the side of his neck. There was also extensive redness and swelling in the inferior of the neck, just anterior to the sternocleido

10、mastoid muscle.p What is the most likely diagnosis?p What is the probable embryological basis of this intermitten mucoid discharge?p Discuss the etiology of this congenital anomaly?Case 2：A young woman consulted her physician about a swelling in the anterior part of her neck, just inferior to the hyoid bone.p What kind of a cyst might be present?p Are they always in the median plane?p Discuss the embryological basis of the these cy