Embryology GastrointesInal System
Transcription
Embryology GastrointesInal System
Gastrointes*nal System Embryology Gastrointestinal System: Embryology 1. The primordial gut at the beginning of the 4th week is closed a. At the cranial end by the oropharyngeal membrane b. At the caudal end by the cloacal membrane 2. The embryonic layers of the primordial gut give rise to a. Epithelia and glands – endoderm b. Stroma – LP splanchnic mesoderm 3. The epithelium within the stomodeum (cranial to the oropharyngeal membrane) and epithelium of the proctodeum (caudal to the cloacal membrane) is derived from surface ectoderm 4. The primordial gut is divided into three regions: a. Foregut – vascularized by celiac trunk b. Midgut – vascularized by superior mesenteric artery c. Hindgut – vascularized by inferior mesenteric artery Gastrointestinal System: Embryology 4a. Foregut – 1. derivatives are: a. Primordial pharynx 1. Oral cavity 2. Teeth 3. Pharynx 4. Tongue 5. Tonsils 6. Salivary glands (submaxillary, submandibular, sublingual) b. Upper respiratory system (nasal cavities, nasopharynx) c. Lower respiratory system (larynx, trachea, bronchi, lungs) d. Esophagus e. Stomach f. Duodenum g. Liver h. Extrahepatic biliary system (including gallbladder) i. Pancreas All foregut derivatives except pharynx, respiratory system, and most of esophagus, are supplied by the celiac trunk, the artery of the foregut. Development of Oral Cavity Oral Cavity The pharyngeal apparatus consists of: 1. Pharyngeal arches 2. Pharyngeal membranes 3. Pharyngeal pouches 4. Pharyngeal grooves These embryonic structures contribute to formation of the head and neck. 3 4 Pharyngeal Arches: The pharyngeal arches begin to develop early in the fourth week as neural crest cells (cranial ectomesenchyme) migrate into the future head and neck regions. Pharyngeal Arches: The primordial jaws – first pair of pharyngeal arches – appear as surface elevaAons lateral to the developing pharynx. 1st pair Developing Pharynx Primordial Jaws Primordial Jaws = Maxillary Prominence (green) & Mandibular Prominence (red) Pharyngeal Arches: By the end of the fourth week, four pairs of pharyngeal arches are visible externally. Pharyngeal Arches: The forming pharyngeal arches are separated from each other by fissures – pharyngeal grooves. Pharyngeal grooves Fate of Pharyngeal Arches Contribute extensively to the formation of the face, nasal cavities, mouth, larynx, pharynx, and neck. A typical pharyngeal arch contains: 1. An aortic arch – an artery that arises from the truncus ateriosus of the primordial heart. 2. A cartilaginous rod – that forms the skeleton of the arch 3. A muscular component – that differentiates into the muscles in the head and neck 4. A nerve – that supplies the mucosa (epithelial lining) and muscles derived from the arch Pharyngeal Arches Fate of Pharyngeal Arches The pharyngeal arches contribute extensively to the formaAon of the face, nasal caviAes, mouth, larynx, pharynx, and neck. A typical pharyngeal arch contains: 1. An aor*c arch – an artery that arises from the aorAc sac and connects with the truncus ateriosus of the primordial heart. Pharyngeal Arch: Aor*c Arches 1st AorAc Arch Maxillary artery 2nd AorAc Arch Stapedial artery 3rd AorAc Arch CaroAd arteries: Common, Internal & External 4th leS AorAc Arch Arch of the Aorta 4th right AorAc Arch Right proximal Subclavian artery 5th AorAc Arch Degenerates 6th AorAc Arch, proximal porAon Pulmonary arteries 6th AorAc Arch, distal porAons leS side Ligamentum arteriosum right side Degenerates Pharyngeal Arches Fate of Pharyngeal Arches A typical pharyngeal arch contains: 2. A car*laginous rod – that forms the skeletal structures of the arch Pharyngeal Arch: Car*laginous Rod 1st Arch Meckel’s carAlage (maxillary & mandibular prominences, malleus, and incus 2nd Arch Stapes, Styloid process, Lesser cornu (horn) of hyoid bone, Superior part of body of hyoid bone 3rd Arch Greater cornu (horn) of hyoid, Inferior body of hyoid bone 4th Arch Thyroid carAlage, Cricoid carAlage, Arytenoid carAlages, Corniculate carAlages, Cuneiform carAlages, TriAceal carAlages Pharyngeal Arches Fate of Pharyngeal Arches A typical pharyngeal arch contains: 3. A muscular component – that differenAates into the muscles in the head and neck Pharyngeal Arches: Muscular Component 1st Arch Muscles of masAcaAon: temporalis, masseter, medial and lateral pterygoids; mylohyoid; anterior belly of digastric muscle 2nd Arch Muscles of facial expression: buccinator, auricularis, frontalis, platysma, orbiculais oris, obicularis occuli; stapedius, stylohyoid, posterior belly of digastric 3rd Arch Stylopharyngeus 4th & 6th Cricothyroid, Levator veli palaAni, Constrictors of pharynx (superior, middle, inferior), Intrinsic muscles of larynx (oblique arytenoid, transverse arytenoid, posterior crico-‐arytenoid, lateral crico-‐ arytenoid, thyro-‐arytenoid), Striated muscle of esophagus Pharyngeal Arches Fate of Pharyngeal Arches A typical pharyngeal arch contains: 4. A nerve – that supplies the mucosa (epithelial lining) and muscles derived from the arch Pharyngeal Arches: Nerve Supply 1st Arch Trigeminal nerve (V1, V2, V3) 2nd Arch Facial nerve (VII) 3rd Arch Glossopharyngeal nerve (IX) 4th Arch Vagus nerve (X): Superior laryngeal branch of vagus (CN X) [External laryngeal nerve & Internal laryngeal nerve], Recurrent laryngeal branch of vagus nerve (CN X) First Arch – Mandibular Arch 1. First aortic arch – maxillary artery. 2. Skeletal structures – a. Maxillary prominence – gives rise to the maxilla (upper jaw), zygomatic bone, and squamous part of temporal bone b. Mandibular prominence – forms the mandible (lower jaw) c. Malleus d. Incus e. Anterior ligament of malleus f. Sphenomandibular ligament 3. Muscles – muscles of mastication: temporalis, masseter, medial and later pterygoids; mylohyoid; anterior belly of digastric muscle 4. Nerve – trigeminal nerve (CN V) The first pair of pharyngeal arches plays a major role in facial development. Second Arch – Hyoid Arch 1. Second aortic arch – stapedial artery. 2. Skeletal structures a. Stapes b. Styloid process c. Lesser cornu (horn) of hyoid bone d. Superior part of body of hyoid bone e. Stylohyoid ligament 3. Muscles – muscles of facial expression: buccinator, auricularis, frontalis, platysma, orbiculais oris, obicularis occuli; stapedius, stylohyoid, posterior belly of digastric 4. Nerve – facial nerve (CN VII) Third Arch 1. Third aortic arch – common carotid artery. 2. Skeletal structures – a. Greater cornu (horn) of hyoid b. Inferior body of hyoid bone 3. Muscles – stylopharyngeus 4. Nerve – glossopharyngeal nerve (CN IX) Fourth & Sixth Arches 1. A. Fourth Aortic Arch 1. Left 4th – arch of the aorta 2. Right 4th – proximal portion of right subclavian artery 1. B. Sixth Aortic Arch • Left 6th distal – ductus arteriorsus • Left 6th proximal – left pulmonary artery • Right 6th distal – degenerates • Right 6th proximal – right pulmonary artery 2. Skeletal structures a. Thyroid cartilage b. Cricoid cartilage c. Triticeal cartilages (paired) d. Arytenoid cartilages (paired) e. Corniculate cartilages (paired) f. Cuneiform cartilages (paired) Fourth & Sixth Arch 3. Muscles – a. Cricothyroid b. Levator veli palatini c. Constrictors of pharynx (superior, middle, inferior) d. Intrinsic muscles of larynx (oblique arytenoid, transverse arytenoid, posterior arytenoid, latreal arytenoid, thyroarytenoid) e. Striated muscle of esophagus 4. Nerves a. Superior laryngeal branch of vagus (CN X) (a & b, above) b. Recurrent laryngeal branch of vagus nerve (CN X) (c - e, above) Development of the Face 1. Five facial primordia appear early in the fourth week a. Around the large primordial stomodeum 1. A single frontonasal prominence (1) 2. Paired maxillary prominences (2) 3. Paired mandibular prominences (2) Frontonasal Prominence Maxillary Prominence Primordial Stomodeum Mandibular Prominence Development of the Face 1. Five facial primordia appear early in the fourth week a. Five facial primordia are derivatives of the first pharyngeal arch b. Form cartilage, bone, and ligaments in facial and oral regions 1. Are derived from cranial ectomesenchyme: a. Maxillary prominence – gives rise to the maxilla (upper jaw), b. Zygomatic bone, c. Squamous part of temporal bone d. Mandibular prominence – forms the mandible (lower jaw) e. Malleus f. Incus g. Anterior ligament of malleus h. Sphenomandibular ligament c. Form the craniofacial voluntary muscles 1. Are derived from paraxial and prechordal mesoderm: a. temporalis, masseter, medial and later pterygoids; mylohyoid; anterior belly of digastric muscle Development of the Face 10. Each lateral nasal prominence is separated from the maxillary prominence by a cleft, called the nasolacrimal groove. Lateral Nasal Prominence Nasolacrimal groove Maxillary Prominence Development of the Face 12. By the end of the sixth week, each maxillary prominence has begun to merge with the lateral prominence along the line of the nasolacrimal groove. a. Establishes continuity between the side of the nose (formed by the lateral nasal prominence) and the cheek region (formed by the maxillary prominence) Development of the Face 11. By the end of the 5th week, the primordia of the auricles of the ears have begun to develop. a. Six auricular hillocks (ectomesenchymal swellings) form around: 1. The 1st pharyngeal groove (three on each side) a. The primordia of the auricle b. The external auditory meatus (canal). External Auditory Meatus Auricular hillocks Development of the Face 14. Between the 7th and 10th weeks the medial nasal prominences merge with each other. Similarly, the maxillary prominences and lateral nasal prominences merge with each other. Development of the Face 15. Merging of medial nasal prominence and maxillary prominence results in a. Continuity of the upper jaw b. Continuity of the upper lip c. Separation of nasal pits from mouth Development of the Face 16. As medial nasal prominences merge they form an intermaxillary segment F Intermaxillary segment E Development of the Face 17. Intermaxillary segment forms a. Philtrum (middle part) of upper lip b. Premaxillary part of the maxilla and associated gingiva (gum) c. The primary palate Primary palate Philtrum of lip Premaxillary part of maxilla & associated gingiva Development of Palate Palate Development of the Palate 2. Palatogenesis a. Begins at the end of the 5th week b. Ends at the 12th week c. Critical period for malformations is 6th to 9th week Primary palate Developing maxilla Primordium of premaxillary part of maxilla Development of the Palate – Primary Palate 3. Early in 6th week the primary palate – median palatine process – begins to develop from deep part of intermaxillary segment of the maxilla a. Initially formed by merging of medial nasal prominences Medial nasal prominences merging with each other Development of the Palate – Primary Palate 3. Early in 6th week the primary palate – median palatine process – begins to develop from deep part of intermaxillary segment of the maxilla b. A wedge-shaped mass of ectomesenchyme between internal surfaces of maxillary prominences of developing maxilla Developing maxilla Intermaxillary segment of maxilla Development of the Palate – Primary Palate 3. Early in 6th week the primary palate – median palatine process – begins to develop from deep part of intermaxillary segment of the maxilla c. Primary palate forms premaxillary part of maxilla 1. Represents small part of adult hard palate 2. Located anterior to incisive foramen Developing maxilla Primordium of premaxillary part of maxilla c. Primary palate forms premaxillary part of maxilla 1. Represents small part of adult hard palate 2. Located anterior to incisive foramen Premaxillary part of maxilla Incisive foramen Development of the Palate – Secondary Palate 4. The secondary palate is the primordium of the hard and soft parts of the palate Secondary palate Hard palate Soft palate Development of the Palate – Secondary Palate 4. The secondary palate is the primordium of the hard and soft parts of the palate a. Begin developing early in 6th week b. Forms from two ectomesenchymal projections – lateral palatine processes (palatal shelves) Median palatine process Median palatine process Lateral palatine processes Development of the Palate – Secondary Palate The secondary palate b. Lateral palatine processes (palatal shelves) 1. Extend from internal aspects of maxillary prominences 2. Initially project inferomedially on each side of tongue 3. During 7th to 8th week, processes elongate and ascend to horizontal position superior to tongue Median palatine process Nasal septum Maxillary prominence Lateral palatine process Nasal septum Lateral palatine process Development of the Palate – Secondary Palate 4. The secondary palate b. Lateral palatine processes (palatal shelves) 4. Gradually processes approach each other and a. fuse with posterior part of primary palate b. fuse in the median plane c. fuse with median nasal septum Primary palate Posterior part of primary palate Lateral palatine process Medial nasal septum Development of the Palate – Secondary Palate 4. The secondary palate b. Lateral palatine processes (palatal shelves) 4. Gradually processes approach each other and a. fuse with posterior part of primary palate b. fuse in the median plane c. fuse with median nasal septum Primary palate Fuse in the median plane Lateral palatine process Median nasal septum Development of the Palate – Secondary Palate 4. The secondary palate b. Lateral palatine processes (palatal shelves) 4. Gradually processes approach each other and a. fuse with posterior part of primary palate b. fuse in the median plane c. fuse with median nasal septum Primary palate Lateral palatine process Fuse in the median plane Median nasal septum Development of the Palate – Secondary Palate 4. The secondary palate b. Lateral palatine processes (palatal shelves) 4. Gradually processes approach each other and a. fuse with posterior part of primary palate b. fuse in the median plane c. fuse with median nasal septum Nasal septum Lateral palatine process Fuse in the median plane with median nasal septum Development of the Palate – Secondary Palate 5. The nasal septum develops as a down growth from internal parts of merged medial nasal prominences a. Fusion between nasal septum & lateral palatine processes progresses in an anterior to posterior direction from the 9th week to the 12th week Nasal septum Lateral palatine process Fusion in the median plane with median nasal septum Development of the Palate – Secondary Palate 5. Bone gradually develops in primary palate a. Forms premaxillary part of maxilla b. Lodges the incisor teeth Premaxillary part of maxilla Incisor tooth Development of the Palate – Secondary Palate 6. Concurrently, bone extends from maxilla and palatine bones into lateral palatine process to form the hard palate Incisor teeth Incisive foramen Suture between premaxillary part of maxilla and lateral palatine process of maxilla Lateral palatine process of maxilla Horizontal plate of palatine bone Premaxillary part of maxilla Development of the Palate – Secondary Palate 7. The posterior parts of processes do not ossify a. They extend posteriorly beyond the nasal septum b. Fuse to form the soft palate, including uvula c. The median palatine raphe indicates line of fusion of lateral palatine processes Frenulum of lip Incisive papillae (deep = i. foramen) Upper lip Hard palate Gum Median palatine raphe Soft palate Uvula Development of the Palate – Secondary Palate 8. A small nasopalatine canal persists in median plane a. Occurs between premaxillary part of maxilla and palatine processes of maxilla b. Represented in adult by incisive foramen (Fig. 10-36) Incisor teeth Incisive foramen Suture between premaxillary part of maxilla and lateral palatine process of maxilla Lateral palatine process of maxilla Horizontal plate of palatine bone Premaxillary part of maxilla Development of Teeth Development of Teeth Teeth Teeth: Embryological Development Ectoderm Ameloblasts Neural Crest Odontoblasts Mesenchyme Cementocytes Enamel • Moves down • No longer produced after adulthood Predentin • Type I collagen Dentin • Moves outward • Type I collagen • Hydroxyapatite • Fluoroapatite Cementum • Covers root Teeth: Developmental Stages 1. Bud stage • Neuroectodermal cells sAmulate overlying ectodermic epithelial cells to proliferate • Forms the epithelial tooth bud Ectoderm 2. Early cap stage NE cells Mesoderm • Cells of the tooth bud proliferate & invaginate into underlying mesoderm = early cap • BMP-‐4 & AcAvin βA sAmulate formaAon Teeth: Developmental Stages (cont.) 3. Late Cap Stage Bud of Permanent Tooth • Develops from dental lamina • Remains dormant Outer Dental Epithelium • Neural crest Inner Dental Epithelium • Neuroectoderm NC neuroectodermal cells lining the epithelial tooth bud (“cap”) Secrete: • FGF-4 • BMP-2,4,7 Enamel Knot • Signals tooth development Regulate tooth shape Teeth: Developemental Stages (cont.) 4. Bell Stage • Mesenchyme adjacent to developing enamel organ proliferates to form cellular mass as the enamel organ becomes cap shaped Inner Dental Epithelium Layer of the enamel knot Preameoloblasts • Ameoloblasts • Secrete enamel downward Outermost Cells (apical) of the dental papilla Primitive Dental Papilla Preodontoblasts • Odontoblasts • Secrete non-mineralized predentin which later calcifies into dentin Teeth: Developmental Stages (cont.) 5. Tooth Erup*on Enamel Dentin Cementoblasts • Secretes a layer of cementum to cover root(s) of tooth Blood vessel in dental pulp Odontoblasts Periodontal Ligament • Holds tooth to alveolus Dental Sac gives rise to: • Cementoblasts • Periodontal ligament SecAons of Teeth • Crown: projects from the gingiva • Root: fixed in tooth socket by periodon6um – Number of roots varies • Most of the tooth is composed of den*n – Covered by enamel over the crown – Covered by cement over the root • Set in tooth sockets: alveolar process of maxillae & mandible – Adjacent sockets are separated by interalveolar septa Sections of Teeth: A) An incisor tooth B) A molar tooth In living people, the pulp cavity is a hollow space within the crown & neck containing connective tissue, blood vessels, & nerves. The cavity narrows down to the root canal in a single-rooted tooth or to one canal per root of a multirooted tooth. The vessels & nerves enter or leave through the apical foramen. Development of the Tongue Development of Tongue Tongue Development of the Tongue 1. Tuberculum Impar (median tongue bud) appears near end of 4th week 2. It is a median triangular elevation in the floor of the primordial pharynx 3. It is the first indication of tongue development 4. Soon, two distal tongue buds (lateral lingual swellings) develop on either side of the median tongue bud 5. The three lingual buds develop from proliferation of the prechordal mesoderm in the first pair of pharyngeal arches 6. The distal tongue buds rapidly increase in size and fuse (median sulcus of the tongue) to form the anterior 2/3’s of the tongue Development of the Tongue 7. The posterior 1/3 of the tongue (pharyngeal part) develops from two elevations that develop caudal to the foramen cecum a. The copula forms by fusion of the ventromedial parts of the second pair of pharyngeal arches b. The hypopharyngeal eminence develops caudal to the copula from prechordal mesoderm of the third and fourth pharyngeal arches 8. As the tongue develops the copula is gradually overgrown by the hypopharyngeal eminence and disappears 9. The posterior 1/3 of the tongue develops from the rostral part of the hypopharyngeal eminence 10. The terminal sulcus is the line of fusion between the anterior 2/3 and posterior 1/3 of the tongue Development of the Tongue 11. Cranial ectomesenchyme forms the connective tissue and vasculature of the tongue 12. Most tongue muscles are derived from myoblasts that migrate from occipital myotomes 13. The hypoglossal nerve (CN XII) accompanies the myoblasts during their migration and innervate the tongue muscles as they develop, except for the palatoglossus muscle which is innervated by the vagus nerve (CN X) 14. The sensory supply to the anterior 2/3 of the tongue is from the lingual branch of the mandibular division of the trigeminal nerve (CN V), the nerve of the first pharyngeal arch 15. The facial nerve supplies the taste buds in the anterior 2/3 of the tongue, except for the circumvallate papillae 16. The circumvallate papillae are innervated by the glossopharyngeal nerve (CN IX) Development of the Tongue 17. Clinical Correlates a. Anomalies of the tongue are uncommon, except for 1. Fissuring of the tongue – infants with Down syndrome 2. Hypertrophy of the lingual papillae – infants with Down syndrome b. Congenital Lingual Cysts and Fistulas 1. Cysts may be derived from remnants of thyroglossal duct 2. May emerge to produce symptoms of discomfort and/or dysphagia (difficulty swallowing) 3. Fissures are also derived from persistance of lingual parts of thyroglossal duct a. Open through foramen cecum into oral cavity Mouth: Embryology Development of the Tongue 17. Clinical Correlates c. Ankyloglossia (tongue-tied) 1. The lingual frenulum normally connects the inferior surface of the tongue to the floor of the mouth 2. Sometimes the frenulum is short and entends to the tip of the tongue a. Interferes with free protrusion of the tongue b. May make breast feeding difficult 3. Occurs in about 1:300 live births in North American infants Mouth: Embryology Development of the Tongue 17. Clinical Correlates d. Macroglossia 1. An excessively large tongue 2. Results from generalized hypertrophy of the tongue 3. Usually results from lymphangioma (a lymph tumor) 4. May result from muscular hypertrophy Mouth: Embryology Development of the Tongue 17. Clinical Correlates e. Microglossia 1. An abnormally small tongue 2. Usually associated with micrognathia (underdeveloped mandible and recession of the chin) 3. May be associated with certain limb defects, Hanhart syndrome Mouth: Embryology Development of the Tongue 17. Clinical Correlates f. Glossoschisis (bifid or cleft tongue) 1. Very uncommon anomaly 2. Results from incomplete fusion of the distal tongue buds 3. Exhibits as a deep median groove in the tongue 4. Usually a cleft tongue does not extend to tip of tongue Salivary Glands Salivary Glands: Embryology 1. Salivary glands begin developing during the 6th and 8th weeks 2. They begin as solid epithelial buds from primordial oral cavity 3. The club-shaped ends of the buds grow into the underlying cranial ectomesenchyme 4. The connective tissue stroma of the glands is derived from cranial ectomesenchyme (neural crest) 5. All parenchyma arises by proliferation of oral epithelium a. Surface ectoderm – parotid b. Endoderm – submandibular (submaxillary) gland and sublingual gland Salivary Glands: Embryology 6. Parotid glands a. Are the first to appear – early in the 6th week b. They develop from buds of oral ectoderm near the angles of the stomodeum c. The buds grow towards the ears and branch to form solid cords with rounded ends d. Later the cords canalize – develop lumina – and become ducts by 10 weeks e. The rounded ends develop into acini f. Secretion begins at 18 weeks g. The capsule and connective tissue develops from cranial ectomesenchyme (neural crest) Salivary Glands: Embryology 7. Submandibular (Submaxillary) glands a. Appear late in the 6th week b. Develop from endodermal buds in floor of stomodeum c. Solid cellular processes grow posteriorly, lateral to developing tongue 1. Later they branch and differentiate d. Acini begin to form at 12 weeks e. Secretory activity begins at 16 weeks f. Growth of glands continues after birth with formation of mucus acini g. Lateral to tongue, a linear groove forms that soon closes to form sumandibular duct Salivary Glands: Embryology 8. Sublingual glands a. Appear in the 8th week, about 2 weeks later than other salivary glands b. Develop from multiple endoderm buds in the paralingual sulcus c. Buds branch and canalize to form 10 – 12 ducts that open independently into the floor of the mouth Development of Larynx Larynx Development of Larynx 1. The epithelium of larynx develops from the endoderm of the cranial end of the laryngotracheal tube. 2. The cartilages of the larynx develop from the cartilages in the 4th and 6th pharyngeal arches. 3. The cartilages are derived from neural crest cells (cranial ectomesenchyme). Development of Larynx 4. The ectomesenchyme are the cranial end of the laryngotracheal tube proliferates rapidly and produces the paired arytenoid swellings. a. Swellings grow towards tongue, converting the slit-like opening – the primordial glottis – into a T-shaped laryngeal inlet. b. Reduces the developing laryngeal lumen to a narrow slit. Epiglottic swelling Primitive glottis Arytenoid swellings Development of Larynx 4. The ectomesenchyme are the cranial end of the laryngotracheal tube proliferates rapidly and produces the paired arytenoid swellings. a. Swellings grow towards tongue, converting the slit-like opening – the primordial glottis – into a T-shaped laryngeal inlet. b. Reduces the developing laryngeal lumen to a narrow slit. Epiglottis Laryngeal inlet Arytenoid swellings Cuneiform tubercle Development of Larynx 5. The laryngeal epithelium proliferates rapidly, resulting in a temporary occlusion of the laryngeal lumen. 6. Recanalization of the larynx usually occurs by the 10th week. Endoderm Esophagus LP Splanchnic mesoderm Laryngotracheal tube Epithelium LP Splanchnic mesoderm Developing cartilages Development of Larynx 7. The laryngeal ventricles form during the recanalization process. 8. The recesses are bounded by folds of mucous membrane that become the a. The vestibular folds (cat purring). b. The vocal folds (human phonation) Development of Larynx 9. The epiglottis develops from the caudal part of the hypopharyngeal eminence. a. A prominence produced by the ventral ends of the 3rd and 4th pharyngeal arches. b. The rostral part of the eminence forms the posterior third of the pharyngeal part of the tongue. Hypopharyngeal Eminence Development of Larynx 10. The laryngeal muscles develop from myoblasts of the 4th and 6th pharyngeal arches and therefore innervated by branches of the vagus (CN X) nerve. 11. Growth of larynx and epiglottis is rapid during first three years after birth. By this time the epiglottis has reached its adult form. Esophagus Development of Esophagus Esophagus 4a. Foregut – 1d. Esophagus 1. Development a. Develops from foregut immediately caudal to pharynx b. The esophagus is partitioned from the trachea by the tracheoesophageal septum c. Initially, the esophagus is short, but it elongates rapidly Endoderm Esophagus Laryngotracheal tube LP Splanchnic mesoderm Epithelium LP Splanchnic mesoderm Developing cartilages Esophagus: Embryology 4a. Foregut – 1d. Esophagus 1. Development d. It reaches its final length by the 7th week e. The epithelium and glands are derived from endoderm 1. The epithelium proliferates and completely obliterates the lumen; recanalization of the esophagus occurs by the end of the 8th week (programmed apoptosis) f. The skeletal muscle in the upper 2/3 of the esophagus (overlap in the middle 1/3 with smooth muscle) is derived from prechordal mesoderm of the pharyngeal arches g. The smooth muscle in the lower 2/3 of the esophagus (overlap in the middle 1/3 with skeletal muscle) is derived from LP splanchnic mesoderm h. Both types of muscle are innervated by branches of the vagus nerve (CN X) Esophagus: Embryology 4a. Foregut – 1d. Esophagus 2. Clinical correlates a. Esophageal atresia 1. Blockage of the esophagus occurs with an incidence of 1:3000 to 1:4500 live births 2. About 1/3 of affected infants are born prematurely 3. Associated with tracheoesophageal fistula in more than 85% of cases Esophagus: Embryology 4a. Foregut – 1d. Esophagus 2. Clinical correlates b. Tracheoesophageal Fistula (TEF) 1. A fistula (abnormal passage) between trachea and esophagus. 2. Results from incomplete division of cranial part of foregut into respiratory and esophageal parts during 4th week. 3. Incomplete fusion of tracheoesophageal folds results in a defective tracheoesophageal septum, resulting in a tracheoesophageal fistula. 4. Most common anomaly of lower respiratory tract 5. Occurs in 1/3000 – 4500 births 6. Males most affected 7. In more than 85% cases the fistula is associated with esophageal atresia Esophagus: Embryology b. Tracheoesophageal Fistula (TEF) 8. Four main varieties of tracheoesophageal fistula. a. Superior part of esophagus ends blindly (esophageal atresia), the inferior part of esophagus joins trachea near its bifurcation (gastric contents may enter trachea and lungs) b. Fistula between patent esophagus and trachea (esophageal contents and gastric contents may enter trachea and lungs) c. Fistula between superior part of esophagus and trachea, inferior part of esophagus ends blindly (esophageal contents enter the trachea and lungs). d. Fistula between superior part of esophagus and trachea, fistula between inferior part of esophagus and trachea near its bifurcation (esophageal and gastric contents may enter trachea and lungs) Polyhydramnios – is often associated with esophageal atresia and tracheoesophageal fistula. The excess amniotic fluid cannot pass to the stomach and intestines for absorption and subsequent transfer through the placenta to the mother’s blood for disposal. Varieties of Tracheoesophageal Fistulas 1. Superior part of esophagus ends blindly (esophageal atresia) • Inferior part of esophagus joins trachea near its bifurcation (gastric contents may enter trachea and lungs – aspiration) Esophagus Varieties of Tracheoesophageal Fistulas 2. Fistula between superior part of esophagus and trachea, inferior part of esophagus ends blindly (esophageal contents enter the trachea and lungs). Varieties of Tracheoesophageal Fistulas 3. Fistula between patent esophagus and trachea (esophageal contents and gastric contents may enter trachea and lungs) Esophagus Varieties of Tracheoesophageal Fistulas 4. Fistula between superior part of esophagus and trachea, fistula between inferior part of esophagus and trachea near its bifurcation (esophageal and gastric contents may enter trachea and lungs) 4a. Foregut – 1d. Esophagus 2. Clinical correlates c. Esophageal stenosis 1. Narrowing of the lumen 2. Usually occurs in distal 1/3 3. Occurs as web or long segment with threadlike opening 4. Results from incomplete canalization during 8th week 5. May also result from failure of esophageal blood vessels to develop in affected area – atrophy of segment occurs 4a. Foregut – 1d. Esophagus 2. Clinical correlates d. Short esophagus 1. Normally esophagus is very short 2. Esophagus fails to elongate as neck and thorax develop 3. Part of stomach may be displaced superiorly through esophageal hiatus into thorax 4. Congenital hiatal hernia 5. Most hiatal hernias occur after birth\ 6. Usually in middle-aged people 7. Result from weakening and widening of esophageal hiatus in diaphragm 4. a. Foregut 1d. Esophagus 2. Clinical Correlates e. Laryngotracheoesophageal Cleft 1. Uncommon 2. The larynx and trachea fail to separate completely from the esophagus 3. Symptoms similar to tracheoesophageal fistula, EXCEPT aphonia (absence of voice) is a distinguishing feature Stomach Stomach: Embryology 4a. Foregut 1e. Stomach 1. The distal part of foregut is initially a tubular structure 2. ~ middle of 4th week, a slight dilation indicates site of stomach primordium 3. First appears as fusiform enlargement of caudal part of foregut and oriented in median plane 4. Primordium soon enlarges and broadens ventrodorsally 5. During next two weeks the dorsal border of stomach grows faster than the ventral border and demarcates a. the greater curvature of stomach – dorsal border b. The lesser curvature of stomach – ventral border Stomach: Embryology 4a. Foregut 1e. Stomach 6. Rotation of the stomach: a. as the stomach enlarges it acquires its adult shape, b. It slowly rotates 90 degrees in a clockwise direction around its longitudinal axis 1. The ventral border (lesser curvature) moves to the right 2. The dorsal border (greater curvature) moves to the left 3. The original left side becomes the ventral surface 4. The original right side becomes the dorsal surface Stomach: Embryology 4a. Foregut 1e. Stomach 6. Rotation of the stomach: a. as the stomach enlarges it acquires its adult shape, b. It slowly rotates 90 degrees in a clockwise direction around its longitudinal axis 5. Before rotation, the cranial and caudal ends are in the median plane 6. During rotation and growth, its cranial end moves to the left and slightly inferiorly 7. Its caudal end moves to the right and superiorly 8. After rotation, the stomach assumes its final position – its long axis is almost transverse to the long axis of the body 9. Explains why left vagus supplies anterior (ventral) wall and right vagus supplies posterior (dorsal) wall of adult stomach Stomach: Embryology 4a. Foregut 1e. Stomach 7. Mesenteries a. Stomach is suspended from dorsal wall by a dorsal mesentery = dorsal mesogastrium 1. Originally in median plane but carried to left during rotation and formation of omental bursa (lesser sac of peritoneum) b. Stomach is suspended from ventral wall by a ventral mesentery = ventral mesogastrium 1. Attaches stomach to liver (hepatogastric ligament) and duodenum to liver (hepatoduodenual ligament) 2. Forms anterior wall of lesser sac 3. Opening into sac is epiploic foramen of Winslow Stomach: Embryology 4a. Foregut 1e. Stomach 7. Mesenteries c. Omental bursa 1. Isolated clefts (cavities) develop in the LP splanchnic mesoderm forming the dorsal mesentery 2. The clefts soon coalesce to form a single cavity, the omental bursa or lesser peritoneal sac 3. As the stomach enlarges, the omental bursa expands and acquires an inferior recess of the omental bursa between layers of the dorsal mesentery – the greater omentum 4. This membranes over hangs the developing intestines. 5. The inferior recess disappears as the layers of the greater omentum fuse 6. The omental bursa communicates with the peritoneal cavity through the omental (epiploic) foramen of Winslow Stomach: Embryology 4a. Foregut 1e. Stomach 8. Clinical correlates a. Congenital hypertrophic pyloric stenosis 1. Anomalies of stomach are uncommon except for this disorder 2. Affects 1:150 males and 1:750 females 3. Marked thickening of the pyloric sphincter 4. The circular, and to a lesser extent, the longitudinal muscle are markedly hypertrophied – LP splanchnic mesoderm 5. Results in severe stenosis of the pyloric canal and obstruction to the passage of food 6. As a result, the stomach becomes markedly distended 7. The infant expels the stomach’s contents with considerable force = projectile vomiting Liver Liver: Embryology 1. The liver, gallbladder, and biliary duct system arise a. Early in the 4th week b. As a ventral outgrowth from the caudal part of the foregut 1. Hepatic diverticulum c. Develops from embryonic endoderm d. Hepatic diverticulum 1. Extends into septum transversum a. A mass of LP splanchnic mesoderm b. Between developing heart and midgut c. Forms ventral mesentery Liver: Embryology 1. The liver, gallbladder, and biliary duct system arise d. Hepatic diverticulum 2. Rapidly divides into two parts as it grows between layers of ventral mesentery a. Cranial portion 1. Is larger 2. Forms primordium of liver 3. Proliferating endodermal cells give rise to a. Interlacing cords of hepatic cells b. Epithelial lining of intraheptic part of biliary apparatus 4. Hepatic cords a. Anastomose around endothelial-lined spaces – primordia of hepatic sinusoids b. Stroma, hematopoietic tissue, and Kupffer cells are derived from LP splanchnic mesoderm of septum transversum Liver: Embryology 1. The liver, gallbladder, and biliary duct system arise d. Hepatic diverticulum 2.a. Cranial portion 4. Liver grows rapidly a. From 5th to 10th weeks fills a large part of the upper abdominal cavity b. The quantity of oxygenated blood from the umbilical vein into the liver determines the development and functional segmentation c. Initially, right and left lobes are about the same d. Soon, right lobe becomes larger e. Hematopoiesis begins during the 6th week f. By 9th week liver accounts for ~ 10% of total weight of fetus g. Bile formation by the hepatic cells begins during the 12th week 3. Bile entering the duodenum through the bile duct after the 13th week gives meconium (intestinal contents) a dark green color 4. The left umbilical vein passes in the free border of the falciform ligament on its way from the umbilical cord to the liver (becomes ligamentum teres hepatis in the adult) Liver: Embryology 2. Clinical Correlates a. Anomalies of the liver 1. Minor variations of liver lobulation are common 2. Congenital anomalies of the liver are rare 3. Variations of the hepatic ducts, bile duct, and cystic duct a. Are common b. Clinically significant c. Accessory hepatic ducts may be present 1. Are narrow channels running from right lobe of liver to anterior surface of body of gallbladder 2. Cystic duct may open into accessory hepatic duct rather than into common bile duct Development of the Gall Bladder Gallbladder: Embryology 1. The liver, gallbladder, and biliary duct system arise d. Hepatic diverticulum 2. Rapidly divides into two parts as it grows between layers of ventral mesentery b. Caudal portion 1. Becomes the gallbladder 2. The stalk of the hepatic diverticulum becomes the cystic duct 3. The extrahepatic biliary apparatus a. Initially, is occluded with epithelial cells b. Is later canalized due to programmed apoptosis 4. The stalk connecting the hepatic and cystic ducts to the duodenum becomes the bile duct 3. Bile entering the duodenum through the bile duct after the 13th week gives meconium (intestinal contents) a dark green color Gallbladder: Embryology 2. Clinical Correlates a. Extrahepatic biliary atresia 1. Most serious anomaly of extrahepatic biliary system 2. Occurs in 1:10,000 to 1:15,000 live births 3. Most common form a. ~ 85% cases b. Obstruction of ducts at or superior to porta hepatis (gateway to the liver) 1. Deep transverse fissure on the visceral surface of the liver c. Caused by failure of ducts to canalize d. Could also result from liver infection during late fetal period 4. Jaundice occurs soon after birth a. If not corrected surgically, child may die if a liver transplant is not performed Exocrine Pancreas Pancreas: Embryology 1. Pancreas develops between layers of the mesentery from a. dorsal pancreatic bud of endodermal cells b. ventral pancreatic bud of endodermal cells c. Arises from caudal part of foregut b. Most of pancreas is derived from dorsal pancreatic bud 2. The larger dorsal pancreatic bud appears first and develops a slight distance cranial to the ventral bud a. It grows rapidly between layers of the dorsal mesentery 3. The ventral pancreatic bud develops near the entry of the bile duct into the duodenum a. It grows between layers of the ventral mesentery b. As the duodenum rotates to the right and becomes “C”-shaped the ventral pancreatic bud is carried dorsally with the bile duct. 1. It soon lies posterior to the dorsal pancreatic bud and fuses with it c. Forms the uncinate process and part of the head of the pancreas Pancreas: Embryology 4. As the stomach, duodenum and ventral mesentery rotate, the pancreas comes to lie on the dorsal abdominal wall. 5. As the pancreatic buds fuse, their ducts anastomose. a. The main pancreatic duct forms from 1. The duct of the ventral bud 2. The distal part of the duct of the dorsal bud b. The accessory pancreatic duct, if present, 1. Forms from the proximal part of the duct of the dorsal bud 2. Opens into the minor duodenal papilla a. Located about 2 cm cranial to the main duct c. The main pancreatic duct and the accessory pancreatic duct often communicated with each other d. In about 10% of the people the pancreatic ducts fail to fuse Pancreas: Embryology 6. Histogenesis: a. The parenchyma of the pancreas is derived from endoderm of the pancreatic buds 1. Form a network of tubules 2. Early in the fetal period (9 weeks), acini begin to develop from cell clusters around the ends of these tubules (primordial ducts) b. The stroma of the pancreas develops from LP splanchnic mesoderm Development of the Duodenum Duodenum: Embryology 1. Early in 4th week the duodenum begins to develop from the caudal part of the foregut and the cranial part of the midgut 2. The parenchyma develops from endoderm and the stroma develops from LP splanchnic mesoderm 3. The junction of the two parts of the duodenum is just distal to the origin of the bile duct 4. The developing duodenum grows rapidly, forming a C-shaped loop that projects ventrally 5. As the stomach rotates, the duodenal loop rotates to the right and comes to lie retroperitoneally 6. Because of its dual origin (foregut and midgut), it is vascularized by both the celiac trunk (foregut) and superior mesenteric artery (midgut) 7. During 5th and 6th weeks the lumen is obliterated due to epithelial proliferation 8. It recanalizes (programmed apoptosis) by the end of the 8th week Duodenum: Embryology 9. Clinical correlates a. Duodenal stenosis 1. Partial occlusion of the lumen results from incomplete recanalization 2. Due to defective programmed apoptosis 3. Most stenoses involve the horizontal (third) and/or ascending (fourth) parts of the duodenum 4. Because of the occlusion, the stomach’s contents (usually containing bile and therefore green in color) are often vomited Duodenum: Embryology 9. Clinical correlates b. Duodenal atresia 1. Complete occlusion of the lumen 2. Not common 3. 20-30% of infants have Down syndrome 4. An additional 20% are premature 5. In 20% of cases, the bile duct enters the duodenum just distal to the opening of the hepatopancreatic ampulla 6. The lumen is completely obliterated with epithelial cells 7. Recanalization fails to occur 8. Most atresias involve descending (2nd) and horizontal (3rd) parts 9. Located distal to opening of bile duct 10. Vomiting usually begins a few hours after birth 11. Vomitus always contains bile 12. Often there is extension of the epigastrium – upper central area of abdomen a. Resulting from an overfilled stomach and superior part of duodenum Duodenum: Embryology 9. Clinical correlates c. Polyhydramnios 1. Occurs because of duodenal atresia 2. Atresia prevents normal absorption of amniotic fluid by the intestines 3. Diagnosis of duodenal atresia is suggested by the “double bubble sign” on plain radiographs or ultrasound scans a. The “double bubble sign” is caused by a distended, gas-filled stomach and proximal duodenum Midgut: Embryology 1. The derivatives are: a. Distal ½ of duodenum (segments 3 & 4) b. Uncinate process of Pancreas (ventral Pancreatic Bud) c. Jejunum d. Ileum e. Cecum f. Appendix g. Ascending colon h. Proximal ½ - 2/3 of transverse colon 2. All derivatives are supplied by the superior mesenteric artery 3. The midgut loops are suspended from the dorsal abdominal wall by an elongated mesentery 4. As the midgut elongates it forms a ventral, U-shaped loop of gut – the midgut loop – projects into the proximal part of the umbilical cord 5. Designated as the physiological umbilical herniation a. Occurs because there is not enough room in the abdomen for the rapidly growing midgut Midgut: Embryology 6. Occurs at beginning of 6th week 7. The midgut communicates with the yolk sac through the narrow yolk stalk (vitelline duct) until the 10th week 8. The midgut loop has a cranial limb and a caudal limb 9. The yolk stalk is attached to the apex of the midgut loop where the two limbs join 10. The cranial limb grows rapidly and forms the small intestines 11. The caudal loop grows more slowly and forms the cecal diverticulum, the primordia of the cecum and appendix Midgut: Embryology 12. Rotation of the midgut a. While in the umbilical cord, the midgut loop rotates 90 degrees counterclockwise around the axis of the superior mesenteric artery b. This brings the cranial limb of the midgut loop to the right and the caudal limb to the left c. During rotation, the midgut elongates and forms the intestinal loops, e.g., jejunum and ileum Midgut: Embryology 13. Return of Midgut to Abdomen a. During the 10th week the intestines return to the abdomen 1. Unknown cause 2. Proposed decrease in size of liver and kidneys 3. Process called reduction of physiological midgut hernia 4. The small intestine returns first a. Passes posterior and superior to superior mesenteric artery b. Occupies central part of abdomen 5. As large intestine returns, it undergoes a further 180-degree counterclockwise rotation a. Later it comes to lie on the right side of the abdomen b. Ascending colon becomes recognizable as the posterior abdominal wall progressively elongates Midgut: Embryology 14. Fixation of intestines a. Rotation of stomach and duodenum causes the duodenum and pancreas to fall to the right 1. They are pressed against the posterior abdominal wall by the colon 2. Adjacent layers of peritoneum fuse and subsequently disappear 3. Consequently most of the duodenum (2nd and 3rd segments) and the pancreas become retroperitoneal Midgut: Embryology 14. Fixation of intestines b. The attachment of the dorsal mesentery to the posterior wall of the abdominal wall is greatly modified after the intestines return to the abdominal cavity 1. At first, the dorsal mesentery is in the median plane 2. As the intestines enlarge, lengthen, and assume their final positions, their mesenteries are pressed against the posterior abdominal wall. 3. The mesentery of the ascending colon fuses with the parietal peritoneum on the wall and disappears 4. Consequently, ascending colon becomes retroperitoneal Midgut: Embryology 14. Fixation of intestines c. The enlarged colon presses the duodenum against the posterior abdominal wall 1. As a result, most of the duodenal mesentery is absorbed 2. Consequently, the duodenum, except for about the first 2.5 cm (derived from foregut), has no mesentery and lies retroperitoneally d. Other derivatives of the midgut loop (e.g., jejunum and ileum) retain their mesenteries 1. The mesentery is at first attached to the median plane of the posterior abdominal wall 2. After the mesentery of the ascending colon disappears, the fanshaped mesentery of the jejunum and ileum acquires a new line of attachment a. Passes from the duodenal-jejunal junction to the ileocecal junction Midgut: Embryology 15. Cecum and Appendix a. Cecal diverticulum 1. Primordium of cecum and vermiform (wormlike) appendix 2. Appears in 6th week 3. A swelling on the antimesenteric border of the caudal limb of the midgut loop 4. The apex does not grow as rapidly as the rest of the diverticulum a. Thus the appendix is initially a small diverticulum of the cecum b. The appendix increases rapidly in length c. At birth it is a relatively long tube arising from the distal end of the cecum d. After birth, the wall of the cecum grows unequally 1. Results in the appendix comes to enter its medial side Midgut: Embryology 15. Cecum and Appendix a. Cecal diverticulum 4. The apex does not grow as rapidly as the rest of the diverticulum e. The appendix is subject to considerable variation in position 1. As ascending colon elongates, the appendix may pass posterior to cecum – retrocecal appendix (~64% of people) 2. The appendix may pass posterior to the colon – retrocolic appendix 3. The appendix may descend over the brim of the pelvis – pelvic appendix Midgut: Embryology 16. Clinical correlates a. Anomalies of the Midgut – are common, most are anomalies of gut rotation 1. Malrotation of the gut a. Result from incomplete rotation and/or fixation of the intestines 2. Congenital Omphalocoele a. Persistence of the herniation of abdominal contents into the proximal part of the umbilical cord b. Results from failure of the intestines to return to the abdominal cavity during the 10th week c. Herniation of intestines into cord occurs in 1:5,000 births d. Herniation of liver and intestines occur in 1:10,000 births e. Size of hernia depends on contents f. The abdominal cavity is proportionately small when an omphalocoele is present, due to impetus to grow being absent g. Immediate surgical repair is required h. Covering of hernia sac is epithelium of umbilical cord – a derivative of the amnion Midgut: Embryology 16. Clinical correlates a. Anomalies of the Midgut 3. Umbilical hernia a. When the intestines enter the abdominal cavity during the 10th week and then herniate through an imperfectly closed umbilicus b. Different from an omphalocoele c. In umbilical hernia the protruding mass (usually greater omentum and some small intestines) are covered by subcutaneous tissue and skin d. Hernia does not reach its maximum size until a month after birth e. Size ranges from 1 to 5 cm f. Defect through which the hernia occurs is the linea alba g. Hernia protrudes during increased abdominal pressure, e.g., crying, straining, coughing h. Can be easily reduced through fibrous ring at umbilicus i. Surgery is not usually performed unless hernia persists to the age of 3 to 5 years Midgut: Embryology 16. Clinical correlates a. Anomalies of the Midgut 4. Gastroschisis a. Relatively uncommon b. Gastroschisis is a misnomer, actually the anterior abdominal wall is split open, not the stomach c. Results from a defect lateral to the median plane of the anterior abdominal wall d. The linear defect permits extrusion of the abdominal viscera without involving the umbilical cord e. The viscera protrude into the amniotic cavity and are bathed by amniotic fluid f. Usually occurs on the right side lateral to the umbilicus g. More common in males than females h. Results from incomplete closure of the lateral folds during the 4th week Midgut: Embryology 16. Clinical correlates a. Anomalies of the Midgut 5. Nonrotation of Midgut a. Relatively common condition b. Sometimes called left-sided colon c. Is generally asymptomatic, but volvulus (twisting) of the intestines may occur d. Occurs when the midgut loop fails to rotate as it reenters the abdomen e. As a result, 1. The caudal limb of the loop returns to the abdomen first 2. The small intestines lie of the right side of the abdomen 3. The entire large intestine lies on the left side of the abdomen f. When volvulus occurs, the superior mesenteric artery may be obstructed 1. Results in infarction and gangrene of the intestines supplied by the artery Midgut: Embryology 16. Clinical correlates a. Anomalies of the Midgut 6. Mixed Rotation and Volvulus a. The cecum lies just inferior to the pyloric region of the stomach and is fixed to the posterior abdominal wall by peritoneal bands that pass over the duodenum b. Duodenal obstruction, caused by 1. The peritoneal bands 2. Intestinal volvulus c. This type of malrotation results from failure of the midgut loop to complete the final 90 degree rotation d. Consequently, the terminal part of the ileum returns to the abdomen first Midgut: Embryology 16. Clinical correlates a. Anomalies of the Midgut 7. Reversed Rotation a. Very unusual b. Midgut loop rotates in a clockwise rather then counterclockwise direction c. As a result, 1. The duodenum lies anterior to the superior mesenteric artery rather than posterior to it 2. The transverse colon lies posterior to the superior mesenteric artery instead of anterior to it d. In more unusual cases 1. The small intestine lies on the left side of the abdomen 2. The large intestine lies of the right side of the abdomen 3. The cecum is in the center 4. Results from malrotation of the midgut followed by failure of fixation of the intestines Midgut: Embryology 16. Clinical correlates a. Anomalies of the Midgut 8. Subhepatic Cecum and Appendix a. Occurs in about 6% of all fetuses b. More common in males c. Results if the cecum adheres to the inferior surface of the liver when it returns to the abdomen 1. It will be drawn superiorly as the liver diminishes in size 2. The cecum remains in its fetal position d. Subhepatic cecum is not common in adults 1. When it occurs, it may create a problem in diagnosis of appendicitis and during appendectomy (surgical removal of the appendix) Midgut: Embryology 16. Clinical correlates a. Anomalies of the Midgut 9. Mobile cecum a. Occurs in ~ 10% of people b. Cecum has an abnormal amount of freedom c. In very unusual cases it may herniate into right inguinal canal d. Results from incomplete fixation of the ascending colon e. Clinically important 1. Possible variations in position of appendix 2. Because volvulus (twisting) of the cecum may occur Midgut: Embryology 16. Clinical correlates a. Anomalies of the Midgut 10. Internal Hernia a. Very uncommon condition b. The small intestine passes into the mesentery of the midgut loop during the return of the intestines to the abdomen c. As a result, a hernia-like sac forms d. Usually does not produce symptoms e. Often detected at autopsy or during anatomical dissection Midgut: Embryology 16. Clinical correlates a. Anomalies of the Midgut 11. Midgut Volvulus a. The small intestine fails to enter the abdominal cavity normally b. The mesenteries fail to undergo normal fixation c. As a result, volvulus (twisting) of the intestines occurs d. Only two parts of the intestines are attached to the posterior abdominal wall, the duodenum and the proximal colon e. The small intestine hangs by a narrow stalk that contains the superior mesenteric artery and vein 1. Vessels are usually twisted in the stalk and become obstructed at or near the duodenal-jejunal junction 2. The circulation to the twisted intestine is often restricted 3. If vessels are completely obstructed, gangrene develops Midgut: Embryology 16. Clinical correlates a. Anomalies of the Midgut 12. Stenosis and Atresia of the Intestine a. Partial occlusion (stenosis) and complete occlusion (atresia) of the intestinal lumen account for about 1/3 of the cases of intestinal obstruction 1. Occurs most often in ileum – 50% and duodenum – 25% 2. Length of area affected varies b. May result from failure of an adequate number of vacuoles to form during recanalization of intestine 1. In some cases, a transverse diaphragm forms, forming a diaphragmatic atresia Midgut: Embryology 16. Clinical correlates a. Anomalies of the Midgut 12. Stenosis and Atresia of the Intestine c. May occur by interruption of blood supply to a loop of fetal intestine resulting from a fetal vascular accident 1. An excessively mobile loop of intestine may twist, interrupting its blood supply 2. Leads to necrosis of section of bowel involved 3. The necrotic segment later becomes a fibrous cord connecting patent ends of the intestine 4. Most atresias of ileum are caused by infarction of fetal bowel resulting from impairment of its blood supply due to volvulus 5. Impairment most likely occurs during 10th week as intestines return to abdomen 6. Malfixation of the gut predisposes it to volvulus, strangulation, and impairment of its blood supply Midgut: Embryology 16. Clinical correlates a. Anomalies of the Midgut 13. Ileal (Meckel’s) Diverticulum (“rule of 2’s”) a. This out pouching is one of the most common anomalies of the digestive tract b. Congenital ileal diverticulum (Meckel’s diverticulum) occurs in 2% to 4% of all people c. Is 3 to 5 times more likely in males than females d. Clinically significant 1. Can become inflamed 2. Mimics symptoms of appendicitis e. Wall of diverticulum contains all layers of ileum 1. May also contain small patches of gastric and pancreatic tissues 2. Gastric mucosa often secretes acid, producing ulceration and bleeding f. Is the remnant of the proximal portion of the yolk stalk g. Typically appears as a finger-like pouch about 3 to 6 cm (2”) long h. Arises from antimesenteric border of ileum ~ 40-50 cm (2’) from ileocecal junction i. May be connected to umbilicus by fibrous cord, omphaloenteric fistula Midgut: Embryology 16. Clinical correlates a. Anomalies of the Midgut 14. Duplication of the Intestine a. Most intestinal duplications are cystic duplications or tubular duplications b. Cystic duplications are more common c. Tubular duplications usually communicate with intestinal lumen d. Almost all duplications result from failure of normal recanalization of the lumen e. As a result, two lumen form f. The duplicated segment of the bowel lies on the mesenteric side of the intestine Development of the Hindgut Hindgut: Embryology 5. Cloaca a. The terminal part of the hindgut is an endodermal-lined chamber that is in contact with surface ectoderm at the cloacal membrane b. This membrane is composed of endoderm of the cloaca and ectoderm of the protodeum, or anal pit c. The cloaca, the expanded part of the hindgut, receives the allantois (a fingerlike diverticulum) ventrally Hindgut: Embryology 5. Cloaca d. Partitioning of Cloaca 1. The cloaca is divided into dorsal and ventral parts by a wedge of mesoderm – the urorectal septum 2. Develops in the angle between the allantois and the hindgut 3. As the septum grows towards the cloacal membrane, it develops forklike extensions that produce infoldings of the lateral walls of the cloaca 4. These folds grow towards each other and fuse, forming a partition that divides the cloaca into two parts a. Dorsally - the rectum and cranial part of the anal canal b. Ventrally – the urogenital sinus Hindgut: Embryology 5. Cloaca d. Partitioning of Cloaca 5. By the 7th week, the urorectal septum has fused with the cloacal membrane, dividing it into a. A dorsal anal membrane b. A larger ventral urogenital membrane 6. Perineal body in the adult represents the area of fusion of the urorectal septum with the cloacal membrane a. The tendinous center of the perineum b. This fibromuscular node is the landmark of the perineum c. Location for convergence and attachment of several muscles Hindgut: Embryology 5. Cloaca d. Partitioning of Cloaca 7. The urorectal septum also divides the cloacal sphincter into a. Anterior part 1. Develops into superficial perineal, bulbospongiosus, and ischiocavernosus muscles 2. Reason why pudendal nerve innervates these muscles b. Posterior part 1. Becomes external anal sphincter 8. Mesodermal proliferations produce elevations of the surface ectoderm around the anal membrane a. As a result, this membrane is soon located at the bottom of the ectodermal depression – the proctodeum (anal pit) b. The anal membrane usually ruptures at the end of the 8th week 1. Brings distal part of digestive tract (anal canal) into communication with amniotic cavity Hindgut: Embryology 6. Anal Canal a. The superior 2/3s (about 25 mm) of the adult anal canal are derived from the hindgut b. Inferior 1/3 develops from the proctodeum c. Pectinate line indicates the junction of the epithelium from the endoderm of the hindgut and the surface ectoderm of the protodeum 1. Located at inferior limits of anal valves 2. Former site of the anal membrane d. About 2 cm superior to the anus is the anocutaneous line = white line 1. Position where epithelium changes from stratified columnar epithelium (recturm) to non-keratinized stratified squamous epithelium (anal canal) 2. At the anus the skin is keratinized and continuous with the skin around the (natal cleft) anus Hindgut: Embryology 6. Anal Canal e. The other layers of the wall of the anal canal is derived from LP splanchnic mesoderm f. Superior 2/3 of anal canal 1. Supplied by the superior rectal artery, a branch of the inferior mesenteric artery 2. Venous drainage via the superior rectal vein, a tributary of the inferior mesenteric vein 3. Lymphatic drainage eventually flows into the inferior mesenteric lymph nodes 4. Innervation from autonomic nervous system Hindgut: Embryology 6. Anal Canal e. The other layers of the wall of the anal canal is derived from LP splanchnic mesoderm g. Inferior 1/3 of anal canal 1. Supplied by the inferior rectal arteries, branches of the internal pudendal artery 2. Venous drainage via the inferior rectal vein, a tributary of the internal pudendal veins 3. Lymphatic drainage eventually flows into the superficial inguinal lymph nodes 4. Innervation from inferior rectal nerve a. Sensitive to pain, temperature, touch, and pressure Hindgut: Embryology 6. Anal Canal e. The other layers of the wall of the anal canal is derived from LP splanchnic mesoderm h. Clinically important 1. Differences of blood supply, nerve supply, and venous and lymphatic drainage of the anal canal are important, especially when considering the metastasis (spread) of cancer cells a. Tumors in the superior part are painless b. Tumors arise from simple columnar epithelium c. Tumors in the inferior part are painful d. Tumors arise from stratified squamous epithelium Hindgut: Embryology 7. Clinical correlates a. Anomalies of hindgut 1. Most anomalies are located in the anorectal region and result from abnormal development of the urorectal system 2. Clinically, they are divided into high and low anomalies depending on whether the rectum terminates superior or inferior to the puborectal sling, formed by puborectalis muscle, a part of the levator ani musculature Hindgut: Embryology 7. Clinical correlates b. Congenital Megacolon – Hirschsprung Disease (neural crest) 1. Most common cause of neonatal obstructions 2. Males affected more than females (4:1) 3. Results from failure of neural crest cells to migrate into colon during 5th to 7th weeks a. Failure of parasympathetic ganglion cells to develop into Auerbach’s and Meissner’s plexuses 4. The dilation results from failure of peristalsis in the aganglionic segment a. Prevents movement of the intestinal contents 5. A part of the colon is dilated because of absence of autonomic ganglion cells in the myenteric plexus distal to the dilated colon segment 6. The enlarged colon – megacolon – has the normal number of ganglion cells 7. In most cases only the rectum and sigmoid colon are involved Hindgut: Embryology 7. Clinical correlates c. Imperforate Anus and Anorectal Anomalies 1. Occurs in 1:5,000 newborns 2. More common in males 3. Abnormal development of urorectal segment, resulting in incomplete separation of the cloaca into urogenital and anorectal protions 4. Lesions are classified as low or high depending on whether the rectum ends superior or inferior to the puborectalis muscle Hindgut: Embryology 7. Clinical correlates c. Imperforate Anus and Anorectal Anomalies 5. Low Anorectal anomalies a. Anal agenesis with or without fistula 1. The anal canal may end blindly, may be an ectopic anus, or an anoperineal fistula that opens into the perineum 2. May open into the vagina in females or the urethra in males 3. More than 90% of low anorectal anomalies are associated with an external fistula 4. Anal agenesis with a fistula results from incomplete separation of the cloaca by the urorectal septum Hindgut: Embryology 7. Clinical correlates c. Imperforate Anus and Anorectal Anomalies 5. Low Anorectal anomalies b. Anal Stenosis 1. The anus is in the normal position, but the anus and the anal canal are narrow 2. Is probably caused by a slight dorsal deviation of the urorectal septum as it grows caudally to fuse with the cloacal membrane 3. As a result, the anal canal and the anal membrane are small 4. Sometimes only a small probe can be inserted into the anal canal Hindgut: Embryology 7. Clinical correlates c. Imperforate Anus and Anorectal Anomalies 5. Low Anorectal anomalies c. Membranous Atresia of Anus 1. The anus is in the normal position, but a thin layer of tissue separates the anal canal from the exterior 2. The anal membrane is thin enough to bulge during straining 3. The anal membrane appears blue due to the presence of meconium superior to it 4. Results from failure of the anal membrane to perforate during the 8th week Hindgut: Embryology 7. Clinical correlates c. Imperforate Anus and Anorectal Anomalies 6. High Anorectal anomalies a. Anal agenesis with or without fistula 1. Anorectal agenesis with a fistula is the result of incomplete separation of the cloaca by the urorectal septum 2. The rectum ends superior to the puborectalis muscle when there is anorectal agenesis 3. Most common type of anorectal anomaly, accounting for 2/3 of anorectal defects 4. Although rectum ends blindly, there is a fistula to the bladder (rectovesical fistula) or urethra (rectourethral fistula) or to the vestibule of the vagina (rectovestibular fistula) in females Hindgut: Embryology 7. Clinical correlates c. Imperforate Anus and Anorectal Anomalies 6. High Anorectal anomalies a. Anal agenesis with or without fistula 5. Passage of meconium or flatus (gas) in the urine is diagnostic of a rectourinary fistula 6. In newborn males, meconium (feces) may be observed in the urine 7. In newborn females, meconium may be observed in the vestibule of the vagina Hindgut: Embryology 7. Clinical correlates c. Imperforate Anus and Anorectal Anomalies 6. High Anorectal anomalies b. Rectal Atresia 1. The anal canal and rectum are present, but separated by a fibrous cord 2. Cause may be abnormal recanalization of the rectum 3. Cause may be defective blood supply