Help
Options
Didn't know it?
click below
click below
Knew it?
click below
click below
Don't Know
Remaining cards (0)
Know
retry
shuffle
restart
0:00
Embed Code - If you would like this activity on your web page, copy the script below and paste it into your web page.
Normal Size Small Size show me how
Normal Size Small Size show me how
CAS 14 FC Topic 1
CAS 14 Topic 1
| Question | Answer |
|---|---|
| How does DiGeorge syndrome present clinically? | CATCH-22: Cleft palate, abnormal facial features, thymic aplasia → T-cell dysfunction/deficiency, cardiac defects: tetralogy of Fallot, VSD, many others, hypocalcemia: due to hypoparathyroidism, 22q11.2 describes the genetic microdeletion |
| What is the embryological defect implicated in 22q11.2 deletion syndrome? | A developmental defect in the third and fourth pharyngeal pouches. |
| What embryonic structures fail to fuse in patients with an anterior cleft palate? | The lateral palatine shelves and the primary palate. |
| What embryonic structures fail to fuse in cleft palate? | Lateral palatine shelves, nasal septum. and medial palatine processes |
| What embryonic structures fail to fuse in a congenital cleft lip? | Maxillary prominence and medial nasal prominence |
| What embryonic structures fail to fuse in patients with a posterior cleft palate? | Lateral palatine shleves do not fuse with each other and the nasal septum |
| What teratogens are associated with cleft lip and palate development? | Anti-epileptic medications (e.g. phenytoin and carbamazepine), maternal ethanol consumption, excessive or inadequate levels of retinoic acid |
| What is the anatomic landmark that separates anterior cleft palate defects from posterior cleft palate defects? | Incisive foramen |
| What is the most common malformation of the head and neck? | Unilateral cleft lips |
| What is an anteroposterior cleft palate? | A combination of both anterior and posterior palatal defects. |
| Cleft lip and palate are associated with which trisomy syndrome? | Trisomy 13 (Patau syndrome) |
| Can you name four features of velocardiofacial syndrome? | Facial dysmorphism, cleft palate, cardiovascular anomalies, learning disabilities |
| What is the most common cause of death in DiGeorge syndrome? | Congenital heart disease |
| If a teratogen is responsible for the development of DiGeorge syndrome, during what period of embryogenesis did the exposure likely occur? | Weeks 4-6 of fetal development |
| Which type of immune system cell do patients with DiGeorge syndrome lack? | T cells |
| Patients with DiGeorge syndrome classically present with infections caused by which 2 pathogens? | PCP and Candida |
| What is the outcome of parathyroid gland hypoplasia in DiGeorge syndrome? | Hypoparathyroidism and hypocalcemia; the latter may present as tetany. |
| Which 2 organs do patients with DiGeorge syndrome lack? | Thymus and parathyroid glands |
| In addition to cardiac defects, chest X-ray of patients with DiGeorge syndrome classically shows what unique feature? | Absent thymic shadow |
| Why is perinatal mortality thought to increase in post-term infants? | Feto-placental insufficiency, asphyxia (with and without meconium), intrauterine infection |
| What neonatal complications are associated with post-term infants? | Shoulder dystocia, neurologic birth injury, meconium aspiration, persistent pulmonary hypertension, perinatal asphyxia |
| How does a post-term infant with fetal growth restriction present? | A long, thin, small for gestational age infant with dry, peeling skin. |
| Why does fetal growth restriction occur in post-term infants? | Can occur due to a poorly functioning placenta that cannot provide adequate nutrition. |
| What is the most common physical presentation of post-term infants? | Macrosomia |
| How is post-term birth defined? | A gestational age > 42 weeks |
| What is the optimal intervention for post-term births? | Induction of labor at 41 weeks gestation |
| What are two general ways in which post-term infants can present? | Macrosomia or fetal growth restriction |
| What five structures are derived from neural crest cells of pharyngeal arch 2? | Reichert's cartilage, which gives rise to the following structures: stapes, temporal styloid process, stylohyoid ligament, lesser horn and upper body of hyoid bone |
| Which muscles are derived from the mesoderm of pharyngeal arch 4? | Cricothyroid muscle, soft palate muscles (except tensor veli palatini), pharyngeal muscles (except stylopharyngeus) |
| Which muscles are derived from the mesoderm of pharyngeal arch 6? | Intrinsic muscles of larynx (except cricothyroid), and upper muscles of the esophagus |
| Which muscles are derived from the mesoderm of pharyngeal arch 1? | Muscles of mastication (temporalis, masseter, medial and lateral pterygoids), mylohyoid, anterior belly of digastric, tensor veli palatini, and tensor tympani |
| What are the teratogenic effects of isotretinoin? | Hydrocephalus, facial nerve palsies, facial abnormalities (e.g. cleft palate, small ears), cardiac abnormalities (e.g. transposition of the great vessels, tetralogy of Fallot), thymic & parathyroid abnormalities, and spontaneous abortion |
| Which bones are derived from the neural crest cells of pharyngeal arch 1? | Midface bones (e.g. maxilla, zygomatic bone, palatine bone, vomer), malleus, incus, and Meckel's cartilage (i.e. mandible, incus, malleus, sphenomandibular ligament) |
| Which muscles are derived from the mesoderm of pharyngeal arch 2? | Muscles of facial expression, posterior belly of the digastric, stylohyoid, and stapedius |
| Which arteries are derived from the mesoderm of pharyngeal arch 4? | The proximal portion of the right subclavian artery and the arch of the aorta |
| What structures are derived from the neural crest cells of pharyngeal arch 3? | Greater horn and lower body of the hyoid bone |
| Which muscle is derived from the mesoderm of pharyngeal arch 3? | Stylopharyngeus muscle |
| What germ cell layers are contained within the pharyngeal arches? | Mesoderm and neural crest cells |
| What structures are derived from the neural crest cells of pharyngeal arch 6? | Laryngeal cartilages (i.e., cricoid, arytenoid, corniculate) |
| Which 2 arteries are derived from the mesoderm of pharyngeal arch 2? | Stapedial and hyoid arteries |
| Which artery are derived from the mesoderm of pharyngeal arch 1? | Maxillary artery |
| Which nerves supply the derivatives of pharyngeal arch 1? | V2 (sensory branch only) and V3 of the trigeminal nerve |
| Which arteries are derived from the mesoderm of pharyngeal arch 6? | Ductus arteriosus and the pulmonary arteries |
| What cranial nerve supplies pharyngeal arch 6? | Recurrent laryngeal branch of cranial nerve X |
| What structure is derived from the neural crest cells of pharyngeal arch 4? | Laryngeal cartilages (i.e., epiglottic and thyroid cartilages) |
| Which cranial nerve supplies the derivatives of pharyngeal arch 2? | Cranial nerve VII |
| Which arteries are derived from the mesoderm of pharyngeal arch 3? | Internal and common carotid arteries |
| What are the four embryonic components of the pharyngeal apparatus? | Arches, pouches, grooves or clefts, and membranes |
| What is the Pierre Robin sequence? | A development abnormality of the first pharyngeal arch that is caused by mandibular hypoplasia that occurs before the ninth week of development. |
| What are the clinical manifestations of Pierre Robin sequence? | Micrognathia (small mandible), glossoptosis (posteriorly displaced tongue), cleft palate (posteriorly displaced tongue prevents palatal closure), airway obstruction (due to prolapse of posteriorly displaced tongue) |
| What is Treacher Collins syndrome? | Occurs secondary to a lack of neural crest cell migration into the 1st pharyngeal arch, resulting in facial malformations and mandibular hypoplasia. |
| What cranial nerve supplies the structures that develop from pharyngeal arch 4? | Superior laryngeal branch of cranial nerve X |
| What germ cell layers are the linings of the pharyngeal apparatus composed of? | Lined by endoderm internally (aka pharyngeal pouches) and ectoderm externally. |
| Which pharyngeal arch degenerates during fetal development? | Pharyngeal arch 5 |
| What cranial nerve supplies the derivatives of pharyngeal arch 3? | Cranial nerve IX |
| Which family of transcription factors regulates segmentation of the embryo craniocaudally, limb formation patterning, neural crest cell migration & differentiation, and branchial (pharyngeal) arch patterning? | Homeobox (HOX) genes |
| Which vitamin A metabolite participates in HOX signaling pathways during embryonic development? | Retinoic acid (vitamin A) |
| How and where do branchial cleft cysts typically form? | From the incomplete obliteration of the pharyngeal grooves/clefts 2, 3, 4 leaving a remnant at the angle of the mandible. |
| What two structures are derived from pharyngeal pouch 4? | Superior parathyroid glands (dorsal wing) and ultimobranchial body |
| What structure is derived from pharyngeal membrane 1? | Tympanic membrane |
| Where are pharyngeal fistulas typically found? | Along the anterior border of the sternocleidomastoid muscle. |
| What structure is derived from pharyngeal pouch 2? | Epithelial lining of the palatine tonsil |
| Pharyngeal fistulas commonly result from persistence of which embryonic structure? | Pharyngeal groove/cleft 2 persists, forming a patent opening from the internal tonsillar area to the external neck |
| What is the developmental fate of pharyngeal grooves/clefts 2, 3, and 4? | Become the temporary cervical sinus and are eventually obliterated due to expansion of mesenchyme of the 2nd arch. |
| What is the embryological fate of pharyngeal membranes 2, 3, and 4? | Are obliterated |
| What three structures are derived from pharyngeal pouch 1? | Eustachian tube, middle ear cavity, and mastoid air cells |
| What structure arises from pharyngeal groove/cleft 1? | Epithelial lining of the external auditory canal |
| Where are ectopic thymic and parathyroid tissue usually found in an adult? | Lateral regions of the neck |
| Which embryonic germ layers give rise to pharyngeal clefts, arches, and pouches, respectively? | Ectoderm, mesoderm, and endoderm |
| What two structures are derived from pharyngeal pouch 3? | Inferior parathyroid glands (dorsal wing) and thymus (ventral wing) |
| The palate receives sensory innervation from branches of which cranial nerve? | Maxillary nerve (CN V2) |
| The tensor veli palatini is innervated by a branch of which cranial nerve? | Mandibular nerve (CN V3) |
| Which muscle forms the bulge in the lateral wall of the nasopharynx known as the torus levatorius? | Levator veli palatini |
| Which muscles both compose and have action on the soft palate? | Levator veli palatini, tensor veli palatini, and musculus uvulae |
| What composes the anterior 2/3 of the palate? | Hard palate |
| What is the action and innervation of the musculus uvulae? | Inserts into the uvula and acts to elevate and laterally deviate the uvula. It is innervated by the vagus nerve (CN X). |
| The greater palatine nerve relays sensory information from which region of the palate? | Hard palate |
| What is the function of the tensor veli palatini? | Tenses the palate |
| What is the function and innervation of the levator veli palatini? | Acts to elevate the soft palate, effectively closing the oropharynx from the nasopharynx. It is innervated by the vagus nerve (CN X). |
| What structures does the palate separate? | The oral and nasal cavities |
| The lesser palatine nerve relays sensory information from which region of the palate? | Soft palate |
| What congenital condition results from failed fusion of the palatine processes of the maxillae during development? | Cleft palate |
| What plexus of arteries is a frequent site of epistaxis (nose bleeds)? | Kiesselbach's plexus |
| What is the location of the Kiesselbach's plexus? | Anterior/inferior quadrant of the nasal septum |
| What arteries make up Kiesselbach's plexus? | Anterior and posterior ethmoidal artery (from the ophthalmic artery), sphenopalatine artery (a terminal branch of the maxillary artery), superior labial artery (from the facial artery), greater palatine artery (a terminal branch of the maxillary artery) |
| What part of the nasal cavity contains the afferent limb of cranial nerve 1, which is the olfactory epithelium? | The superior border of the nasal septum (roof) |
| What is found on the lateral wall of the nasal cavity? | Superior, middle, and inferior nasal conchae |
| What is the function of the nasal conchae | Warm and humidify air during inhalation |
| Is anterior or posterior epistaxis the most common site of severe hemorrhage? | Posterior epistaxis |
| From which artery does posterior epistaxis most commonly arise? | Sphenopalatine artery |
| Nasal polyps in adults are associated with respiratory disease that is exacerbated by which agent? | Aspirin |
| What genetic condition are nasal polyps associated with in children? | Cystic fibrosis |
Created by:
jofreayala
Popular Medical sets