Term
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Definition
| "Hidden" or undescended testes; may be due to defective gubernaculum or hormone (androgen) deficiency. The testes remain within or just outside abdominal cavity, usually they remain suspended in inguinal canal. |
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Term
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Definition
| When testis are seen in abnormal position. Example is thigh, abdominal wall, perineum, or pubis. Can be due to abnormal tails of gubernaculum. |
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Term
| Congenital Indirect Inguinal Hernia |
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Definition
| Normally, processus vaginalis is closed and tunica vaginalis cavity separated from peritoneal cavity. Not so with a hernia–the intestine protrudes into cavity of tunica vaginalis via unclosed processus vaginalis. |
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Term
| Encysted Hydrocele of Spermatic Cord |
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Definition
| Fluid collects in middle unclosed part of processus vaginalis testis. |
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Term
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Definition
| Fluid collects in tunica vaginalis testis via unclosed processus vaginalis; fluid will approach testes and thus is not defined as a cyst (the other hydrocele was) |
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Term
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Definition
| Urethra opens onto dorsal side of penis; results from not enough ectodermal-mesenchymal interactions during development of genital tubercle; urine expelled at root of malformed penis |
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Term
| Androgen Insensitivity Syndrome |
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Definition
| Cells of genital tubercle, labioscrotal, urogenital folds resist action of testosterone; individual is XY but will not develop testes; instead secondary female characteristics will occur; individual is a normal but sterile female. |
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Term
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Definition
| External urethral orifice is on ventral surface of body of penis |
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Term
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Definition
| External urethral orifice is on ventral side of body of penis |
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Term
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Definition
| Urethral orifice is at junction of penis and scrotum |
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Term
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Definition
| External Urethral Orifice is between unfused halves of scrotum. Hypospadias in general result from inadequate production of androgens or inadequate receptor types for androgens. |
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Term
| Atrial Septal Defect: Ostium Secondum, Volume Load Defect |
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Definition
| Common Defect; occurs in the area of the fossa ovalis; there is abnormal resorption of the septum primum during formation of the foramen secundum; this means that the valve of the foramen ovale will be perforated or abnormally large. Left to Right shunting results. Just remember that this abnormality is due to excessive resorption of septum primum or defective development of the septum secondum |
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Term
| Atrial Septal Defect: Endocardial Cushion Defect/Volume Load Problem |
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Definition
| Septum Primum won't fuse with the endocardial cushions; there is a patent foramen primum and blood flows from left to right. Less Common |
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Term
| Atrial Septal Defect: Sinus venosus/Volume Load Defect |
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Definition
| There is incomplete absorption of the sinus venosus into the right atrium, abnormal development of the septum secundum, or both. Result of this is that the superior part of the interatrial septum is missing. Left to right shunting occurs. |
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Term
| Atrial Septal Defect: Common atrium/Volume Load Defect |
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Definition
| A patient has all three types of atrial defects! This means there is no interatrial septum! Left to Right shunt. |
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Term
| Ventricular Septal Defect |
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Definition
| Most commmon defect; occurs more frequently in males. Volume load defect. Truncus arteriosus doesn't separate correctly, the interventricular septum (membranous part) does not fuse with the truncus arteriosus as a result. Left to right shunting of blood results. Can be detected by cardiac auscultation. |
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Term
| Aortic Valve Stenosis: Pressure Load Defect |
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Definition
| Aortic valve is fused and the valve is narrower; causes extra pressure and work for the heart; hypertrophy of left ventricle and heart murmurs are observed. There is obstructed ventricular outflow. |
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Term
| Coarctation of Aorta: Pressure Load Defect |
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Definition
| Constriction of aorta; most are juxtaductal. This means they occur at the origin of left subclavian artery and entrance of ductus arteriosus. However, coarctation can also occur at a preductal (proximal to ductus arteriosus) or postductal (distal to ductus arteriosus) site as well. More common in males. |
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Term
| Patent Ductus Arteriosus: Volume Load Defect |
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Definition
The ductus arteriosus (originating from the 6th pharyngeal arch) does not close at birth. Blood from aorta flows into pulmonary trunk, resulting in pulmonary hypertension. Another type of left to right shunt.
Can occur because of maternal rubella infection in early pregnancy, this infection results in failture of TGF–Beta induction after birth |
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Term
| Transposition of Great Vessels |
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Definition
Switching of aorta and pulmonary trunk; this means that blood can't become oxygenated!
Lethal, Incompatible with Life.
Caused from failure of bulbar and truncal ridges to twist around in development.
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Term
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Definition
| Aorticopulmonary septum doesn't develop normally; commonly, there will be a single arterial trunk that branches to form the pulmonary trunk and ascending aorta. A ventricular septal defect is always present when the aorta and pulmonary trunks fail to separate; results in increased pulmonary flow. |
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Term
| Unequal division of truncus arteriosus |
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Definition
| Partitioning of truncus is unequal, which produces one large great artery and a small one. This means the aorticopulmonary septum isn't aligned; ventricular septal defect results. |
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Term
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Definition
| Cusps of pulmonary valve are fused and this narrows the pulmonary trunk opening. |
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Term
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Definition
| Conus arteriosus of right ventricle is underdeveloped; hypertrophy of right ventricle. Note: conus arteriosus is a conical pouch of RV from which the pulmononary trunk develops. |
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Term
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Definition
| Origin of this is malalignment of the subpulmonary infundibulum; the conus arteriosus is anteriorly displaced and 4 symptoms result: Pulmonary Stenosis, Ventricular Septal Defect, Overriding Aorta, Right Ventricular Hypertrophy |
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Term
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Definition
| More common in females; ductus arteriosus does not close and transform into the ligamentum arteriosus |
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Term
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Definition
| Also called double pharyngeal arch artery; a vascular ring around the trachea and esophagus shows up. Results from failure of the distal part of the right dorsal aorta to regress |
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Term
| Right arch of aorta w/out retroesophageal component. |
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Definition
| The entire right part of the aorta does not regress. This means that the aorta does not have a retroesophageal component. |
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Term
| Right Arch of Aorta with a retroesophageal component |
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Definition
| The right arch of the dorsal aorta persists; the ductus arteriosus or ligamentum arteriosum attaches from the right arch to the distal part of the aorta and this forms a ring that will constrict trachea and esophagus. |
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Term
| Anomalous Right Subclavian Artery |
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Definition
| The right 4th pharyngeal arch artery + dorsal aorta disappears cranial to 7th intersegmental artery. This means that the right subclavian develops adjacent to the left SC (instead of adjacent to R common carotid)! |
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Term
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Definition
| Peritoneal Sac enters medial end of inguinal canal directly through a weakened posterior wall; develops when abdominal musculature has been weakened. Bulge ocurs medial to the inferior epigastric vessels in inguinal triangle. |
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Term
| Boundaries of Hesselbach's/Inguinal Triangle |
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Definition
1. Laterally by inferior epigastric artery
2. Medially by rectus abdominis muscle
3. Inferiorly by inguinal ligament |
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Term
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Definition
| Congenital in origin. Peritoneal sac enters inguinal canal by passing through deep inguinal ring, just lateral to inferior epigastric vessels; occurs because some/all of embryonic processus vaginalis is open (patent). |
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Term
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Definition
| Abnormal fluid within peritoneal cavity; between visceral and parietal layers |
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Term
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Definition
| Obstruction of fetal airway. Airways dilate, lungs enlarge and become echogenic, diaphragm flattens, fetal ascites can develope |
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Term
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Definition
| Atresia (premature and blunt end of trachea) of trachea, often accompanied by tracheoesophageal fistula. |
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Term
1. Lung hypoplasia
2. Polyhydramnios
3. Oligohydramnios |
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Definition
1. Reduced lung volume
2. Excess Fluid in amniotic cavity; associated with tracheoesophageal fistula
3. Decrease in amniotic fluid; associated with stunted lung development and/or pulmonary hypoplasia |
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Term
| Congenital Hypertrophic Pyloric Stenosis |
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Definition
Hypertrophy of muscles in pyloric region
Stenosis of lumen of pyloric canal
Stomach is distended and contents expelled–vomitting |
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Term
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Definition
| partial occlusion of lumen of duodenum due to incomplete recanalization; leads to bile-stained vomit if stenosis is distal to bile duct opening |
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Term
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Definition
| Complete occlusion of lumen (i.e. failure to recanalize); vomiting begins immediately after birth |
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Term
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Definition
| From bifid ventral pancreatic bud; ring of pancreas encircles 2nd part of duodenum, causing obstruction |
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Term
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Definition
| Persistence of abdominal herniation; size of hernia depends on the contents; if the SI protrudes, amnion and peritoneum will cover the herniated bowel |
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Term
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Definition
Protruding mass covered by skin and subcutaneous tissue
Signifies previous herniation that has protruded back into abdominal cavity–however, there is still a small defect in the umbilical area. Thus, we see an umbilical hernia. |
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Term
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Definition
| Due to defect near median plane of abdominal wall. Viscera protrudes into amniotic cavity. Cause is incomplete closure of lateral folds during 4th week of development |
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Term
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Definition
| Caudal limb returns first from umbilical cord during development; this means that small intestine lies to the right; very benign condition |
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Term
| Reversed Rotation of Midgut |
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Definition
| midgut loop rotates in a clockwise direction instead of counterclockwise; thus duodenum lies anterior to the transverse colon; the superior mesenteric artery is anterior to T colon and posterior to duodenum; superior mesenteric artery compresses transverse colon |
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Term
| Subhepatic cecum and appendix |
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Definition
| Cecum is adherent to liver, doesn't descend to iliac fossa; this can cause difficulty in diagnosis of appendix |
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Term
Volvulus
causes...duodenal obstruction |
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Definition
Twisting of small intestine
Mixed rotation + volvulus cuases cicum to lie inferior to pylorus and become fixed to posterior ab wall by peritoneal bands; this can obstruct the duodenum |
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Term
| Ileal/Meckel's diverticulum |
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Definition
When the umbilical vesicle/vitelline duct persists
Located ~2'' from ileocecal valve on anti-mesenteric border of ileum
If it contains gastric or pancreatic tissue, it gets infected and closely resembles appendicitis |
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Term
Umbilical Fistula
or
Vitelline Cyst |
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Definition
When vitelline duct does not regress from anterior ab wall; cycst can form due to this abnormal connection
The fistula occurs when the connection persists all the way from peritoneal sac to anterior ab wall (vitelline duct is completely patent) |
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Term
| Portal-systemic (porto-caval) anastomosis |
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Definition
When portal vein tributaries communicate with caval tributaries. Where does it occur?
1. Lower end of esophagus-Esophageal tributaries of left gastric vein & esophageal tributaries of azygos/hemiazygos veins protrude into esophagus–––>hemetemesis
2. Lower end of anal canal-superior, middle, and inferior rectal vein join w/ internal iliac vein and from varicosities––>leads to hemorrhoids
3. Umbilicus-veins along ligamentum teres-paraumbilical veins; tributaries of superior and inferior epigastric veins–––>form caput medussae or dilated tortuous veins radiating from umbilicus
4. Retroperiotoneal organs-veins of colon, duodenum, pancreas, spleen w/ renal, lumbar, azygos veins–––>dilated veins around posterior ab wall
5. Bare area of liver: Venous sinusoids of liver +Diaphragmatic veins (intercostal and phrenic) |
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Term
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Definition
| Sudden, forceful compression of abdomen; may tear the ducts leading to leakage of secretions from adj tissues |
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Term
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Definition
| When growth is in head of pancreas...can cause obstructive jaundice, retention of bile pigments, enlargement of gall bladder. Growth will compress portal vein and lead to portal hypertension |
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Term
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Definition
| Right Side of 4th Pharyngeal Arch (distal end) does not involute; the arch of the aorta will pass by the trachea and esophagus posteriorly AND anteriorly. The trachea and esophagus become constricted, causing dyspnea and dysphagia. |
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Term
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Definition
| When right-side of aortic arch and ligamentum arteriosum form constricting ring around esophagus and trachea |
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Term
| Retroesophageal Right Subclavian Artery |
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Definition
| Occurs when the right 4th pharyngeal arch artery do not involute as they should. The right 4th PAA and right dorsal aorta disappears cranial to the 7th intersegmental artery. Thus, the right subclavian artery forms from the right seventh intersegmental artery and distal R dorsal aorta. Differential growth will shift the origin of the R subclavian cranially. |
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Term
| Treacher-Collins Syndrome |
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Definition
| Haploinufficiency of the Treacle protein leads to a depletion of neural crest cell precursor; this leads to abnormally high # neural crest cells migrating to 1st and 2nd pharyngeal arches. The crest cells in the 1/2 P. arches play an important role in face development (i.e. maxillary, stapedial, and hyoid vasculature). Thus, abnormalities in the arches lead to craniofacial defects and growth retardation. |
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Term
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Definition
| Developmental defects due to (this is the current hypothesis) dysregulation of genes SOX9 and KCNJ2. The jaw bones don't develop normally (could be another defect in pharyngeal arch 1&2 development). The patient presents with a small mandible, posterior displacement of the tongue, upper airway obstruction, and cleft palate. |
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Term
| DiGeorge/22q11 Deletion Syndrome |
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Definition
Salient Features: Use CATCH-22 pneumonic
Cardiac Abnormality (especially tetrology of Fallot)
Abnormal facies
Thymic aplasia
Cleft palate
Hypocalcemia |
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Term
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Definition
Blood in Pericardial Cavity; can be diagnosed by Beck's Triad which is 1. Diminished BP 2. Muffled Heart Sounds 3. Distended Neck Veins
Common Causes: Stab Wound, Myocardial Infarction
Treatment: Pericardiocentesis |
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Term
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Definition
| Heart is outside pericardial cavity; occurs due to faulty development of sternum/pericardium; also there is no fusion of lateral folds in forming thoracic wall |
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Term
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Definition
| Formation of lesions in wall of t. intima; these lesions are fatty plaques or atheroscleromas; made of FA's, cholesterol, and Calcium; can tear vessel wall and/or decrease blood flow and also makes flow turbulent; weakens t. media |
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Term
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Definition
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Term
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Definition
1. Hypertrophy of t. media
2. Thickening/reduplication of internal elastic lamina
3. Thickening of fibrous tissue in t. intima
Causes lumen diameter to decrease and accelerates development of atherosclerosis |
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Term
| Sequential Histopathogenesis Underlying Atherosclerosis |
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Definition
1. Excessive LDL in blood
2. Free endothelial radicals oxidize LDL
3. Oxidized LDL invites monocyte migration into T. intima, monocytes become macrophages there
4. Smooth Muscle Cells (SMC) migrate from T. Media to subendothelial CT of T. intima, causes Macrophage activation
5. Foam Cell formation: SMC + macrophages + oxidized LDL
6. Fatty Streak Formation: SMC divide, secrete collagen/ECM to thicken up Foam Cells
7. Plaque Formation: Cytokines of SMC convert streak into fibrofatty plaque
8. Luminal obstruction + weakened vascular walls! |
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Term
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Definition
Due to luminal obstruction of coronary artery; causes "ischemic" changes in myocardial tissue.
Striated, branching pattern of tissue is no longer apparent and eventually macrophages engulf myocytes. |
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Term
| What changes occur in elastic fibers of tunica media that lead to formation of aneurysm (e.g. Marfan's syndrome)? |
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Definition
| Defect in Fibrillin-1 gene leads to weakened E-fibers in T. Media. This in turn leads to a weakened arterial wall aortic aneurysm and dissection will likely occur. |
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Term
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Definition
Bleeding into and along the wall of the aorta; associated with hypertension or connective tissue disorders.
Laceration of tunica intima leads to blood flow in tunica media; the laceration plane usually between middle and outer thirds of media.
Medial hematoma will often develop and can burst through tunica adventitia-this has disastrous consequences. |
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Term
| Peripheral Artery Disease |
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Definition
Symptoms are 1. pain + numbness
2. Decreased wound healing 3. Tissue Death
Major Cause of PAS? Atherosclerosis |
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Term
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Definition
Instrinsic weakness of T. media of veins
Weakness of valves in veins; blood flow to heart gets backed up; common in legs (women), anorectal region (hemorroids), and spermatic cord (varicocele). |
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Term
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Definition
1. Trauma
2. Post-surgical
3. Post-radiation
4. Inflammation
5. Parasitic Obstruction
6. Obstruction due to Metastasis |
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Term
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Definition
| Narrowed anal canal; urorectal septum, as it grows towards the cloacal membrane, deviates dorsally. This creates the narrowness-uncomfortable! |
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Term
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Definition
| Anal membrane does not perforate through the anus. This leads to a closed anus-not good. |
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Term
| Anal agenesis with Perineal Fistula |
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Definition
| Anal Canal ends blindly and there often is some type of fistula; ano-perineal or ano-vaginal or ano-urethral. |
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