Lithium heart and great vessel abnormalities in utero

Transcription

1 This PowerPoint is going to cover a large amount of information. The material, along with my notes, will be available online through the Student Pathology Association ( students/student- pathology- association.html). For this material, I used a combination of resources: 1. First Aid for the USMLE Step 1 (2015; 2016) 2. Pathoma (2014 Edition) 3. First Aid Q&A for the USMLE Step 1 (3 rd Edition) 4. Gray s Anatomy Review (2 nd Edition) 5. Gray s Anatomy for Students (3 rd Edition) 6. Langman s Medical Embryology (12 th Edition) 7. Katzung and Trevor: Basic & Clinical Pharmacology (13 th Edition) 8. Katzung and Trevor Pharmacology: Examination & Board Review (11 th Edition) 9. Current Diagnosis & Treatment: Obstetrics and Gynecology (11 th Edition) 10.Board Buster Step 1 (2007) 11.UpToDate 12.Junqueira s Basic Histology Text & Atlas (14 th Edition) 13.Embryology of the Head and Neck (Rebecca Hartley, 2014) 1

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3 Diethylstilbestrol (DES) à synthetic nonsteroidal estrogen once commonly used in pregnant women to prevent breast engorgement, among many other uses. Prenatal exposure increases risk of multiple conditions vaginal clear cell adenocarcinoma reproductive tract malformations Fetal alcohol syndrome from maternal alcoholism mental and growth retardation morphogenetic disturbances Lithium heart and great vessel abnormalities in utero Nicotine premature delivery conotruncal defects urinary tract abnormalities 3

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5 The black bars show the periods during development when severe structural malformations can occur. The white bars to the right of the black ones show periods when damage can consist of minor structural malformations, growth restrictions, or functional deficiency. 5

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7 I was trying to figure out some clever way to split these meds into body systems, or by trimester, or by effect. However, I actually think the simplest means of learning these effects is just to look at the First Aid chart (p 560 in FA2015) and sort of memorize it. Once you get all your pharmacology down, however, maybe take a stab at thinking a bit more about WHY each of these drugs cause certain defects (i.e., warfarin can cause fetal hemorrhage due to its role in inhibiting vitamin K- dependent clotting factors.) 7

8 The only mnemonic I could come up with was LIMP FAT CAT VW AD for teratogenic medications sorry. 8

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11 Because Harry Potter. That s why. 11

12 Another partof the First Aid chart 12

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14 Be sure to know your heart abnormalities. The Heart morphogenesis section of First Aid (p 268 in FA2015) is gold. Also related to this slide, at least as far as cardiac anomalies are concerned, is Congenital cardiac defect associations (p 290 of FA2015). 14

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17 This is actually assuming you are still in touch with Dr. Hickey s lectures from last block. Make sure you study all teratogens, including maternal illnesses! 17

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28 The left sixth aortic arch is responsible for the development both of the pulmonary arteries and the ductus arteriosus. Without regression of the ductus arteriosus, a patent connection remains between aorta and the pulmonary trunk. The ductus arteriosus often reaches functional closure within 24 hours after birth, whereas anatomic closure and subsequent formation of the ligamentum arteriosum often occur by the twelfth postnatal week. 28

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32 Pages 189 & 190 in Langman s have a great overviewof the derivatives. Cool site for mnemonics: arch- derivatives- mnemonic- images.html Remember: 1 à maxillary artery 2 à stapedial artery; hyoid artery 3 à common carotid artery; proximal portion of internal carotid artery 4 à (L) aortic arch; (R) proximal portion of right subclavian artery 6 à Proximal part of pulmonary arteries; (L) ductus arteriosus 32

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34 The fourth aortic arch develops into the aortic arch on the left side and the brachiocephalic and subclavian arteries on the right side of the embryo. Improper development of the arch of the aorta will cause an increased pressure in the subclavian artery and, subsequently, the brachial artery. Similarly, decreased flow through the aorta will lead to a decreased pressure in the femoral artery. The second aortic arch, specifically the dorsal aspect, develops into aspects of the small stapedial artery. The proximal part of the third aortic arch gives rise to the common carotid arteries, which supply the head. The fifth aortic arch is said not to usually develop in human embryos. The proximal part of the sixth aortic arch develops into the left pulmonary artery. 34

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36 During embryonic development the left dorsal aorta gives rise to the thoracic aorta. The aortic arch is formed by the aortic sac and the fourth pharyngeal artery. The third pharyngeal artery will give rise to the common carotid artery. The fifth pharyngeal arch artery will disappear bilaterally, and the sixth will form the ductus arteriosus on the left and part of the pulmonary trunk. In this case the region of the arch of the aorta between the subclavian artery and the left common carotid artery is formed by the fourth aortic arch. 36

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39 Dr. Hartley: Fish. Branchial is fish. Because, you know, they have no pharynx. 39

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42 Pharyngeal (branchial) cleft cysts are the most common congenital cause of a neck mass. They are epithelial cysts that arise anterior to the superior third of the sternocleidomastoid muscle from a failure of obliteration of the second branchial cleft in embryonic development. The second arch grows caudally and, ultimately, covers the third and fourth arches. The buried clefts become ectoderm- lined cavities that normally involute. Occasionally this process is arrested and the entrapped remnant forms an epithelium- lined cyst, in some cases with a sinus tract to the overlying skin. Many branchial cleft cysts are asymptomatic; others may become tender, enlarged, or inflamed, or they may develop abscesses that rupture, resulting in a purulent draining sinus to the skin or pharynx. Surgery is indicated in these cases. 42

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47 Thyroglossal duct cysts occur due to retention of a remnant of the thyroglossal duct along the path followed by the descending thyroid gland during development. The path begins at the foramen cecum of the tongue and descends in the midline to the final position of the thyroid. The sixth pharyngeal arch provides origin to muscles and cartilage of the neck and would produce a midline mass connected to the tongue. A branchial cyst or fistula would not be present in the midline. The first pharyngeal arch gives rise to muscles of mastication and the malleus and incus. The third pharyngeal arch provides origin to the stylopharyngeus muscle and hyoid bone. 47

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50 The two arytenoid cartilages are pyramid- shaped cartilages with three surfaces, a base of arytenoid cartilage and an apex of arytenoid cartilage 50

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54 The largest part of the palate is formed by the secondary palate, which is embryologically derived from the lateral palatine processes. The median palatine process gives rise to the smaller primary palate, located anteriorly. The intermaxillary segment gives rise to the middle upper lip, premaxillary part of the maxilla, and the primary palate. The median nasal prominences merge with each other and the maxillary prominences to give rise to the intermaxillary segment. The frontonasal eminence gives rise to parts of the forehead, nose, and eyes. 54

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87 Chart made from a mix of First Aid and UpToDate 87

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90 Junqueira explains the process a bit better than FA, I think, if you re like me and have issues keeping your ploidies and whatnot straight. The book is available online through AccessMedicine. 90

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103 ClinicalKey: Ferri's Color Atlas and Text of Clinical Medicine, First Edition 103

104 I think watching Pathomais really helpful for these tumors. This is just an overview to help you classify them. 104

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