DMS 223 OB Week 3

In VACTERL association V, A, C, T, E, R & L stands for:

(V) = vertebral abnormalities (A) = anal atresia (C) = cardiac (heart) defects (T) = tracheal anomalies including tracheoesophageal fistula (E) = esophageal atresia (R) = renal (kidney) and radial abnormalities (L) = (other) limb abnormalities

U/S appearance of amniotic band syndrome is:

1. Abnormal sheet or band (echogenic structure) that attaches to fetus or flaps with fetal movement 2. Restriction of fetal mobility 3. Fetal deformities & malformations 4. Extremity amputation, edema , body wall , facial defects

Possible causes of colon obstruction are:

1. Anal atresia or imperforated anus 2. Hirschprung disease--no relaxation of the anal sphincter (is caused by the absence of nerves within the bowel wall)

Ascites may result from:

1. Bowel perforation 2. Fetal hydrops 3. Infection

Differential diagnosis for duodenal atresia includes:

1. Choledochal cyst (cystic dilatation of the common bile duct) 2. Enlarged GB 3.Hepatic cyst 4. Renal cyst 5. Bowel duplication

U/S appearance of bladder exstrophy is:

1. Cystic mass anterior to lower abdominal wall 2. Lack of visualization of bladder

U/S appearance of meconium ileus is:

1. Echogenic bowel which can be dilated and thick walled 2. Polyhydramnios 3. Fetal ascites 4. Intraabdominal cysts

The two major defects under Pentalogy of Cantrell syndrome are:

1. Ectopia cordis (through the sternum defect) 2. An abdominal wall defect (most commonly an omphalocele)

What are the 5 features of Pentalogy of Cantrell?

1. Ectopiacordis (through the sternum defect) 2. An abdominal wall defect (most commonly an omphalocele) 3. Diaphragmatic hernia 4. Distal sternum cleft (defect) 5. Intracardiac defect (a ventricular septal defect)

U/S appearances of Pentalogy of Cantrell syndrome are:

1. Ectopic heart 2. Omphalocele 3. Distal sternum defect 4. Diaphragmatic hernia 5. Pericardial or pleural effusion

The 2 Causes of non-visualization of the stomach are:

1. Esophageal atresia 2. Diaphragmatic hernia (the stomach could be in the thoracic cavity)

U/S appearances of Limb-body wall complex is:

1. Exencephaly or encephalocele 2. Large abdominal and thorax defects 3. Scoliosis 4. Limb defects 5. The umbilical cord is short

U/S appearances of esophageal atresia are:

1. Failure to visualize stomach or small stomach, growth restriction is present in 40% of the cases 2. Polyhydraminos

U/S appearance of Beckwith-Wiedemann syndrome includes:

1. Growth acceleration, enlargement of organs (macrosomia, macroglossia ) 2. Polyhydramnios 3. Enlarged placenta 4. Omphalocele

What are the U/S appearances of omphalocele?

1. Insertion of the umbilical cord into the mass 2. A covering membrane produces a smooth, well-defined surface to the mass 3. Polyhydramnios presents in one third of the cases 4. Ascites is common 5. Ectopic heart

Associated findings with gastroschisis are:

1. Intra uterine growth retardation (77%) 2. Prematurity (55%) 3. Cardiac problems 4. Minor malformations in small bowel

The 3 important things to demonstrate to differentiate between the gastroschisis and omphalocele are:

1. It is very important to image the cord insertion site and the fetal anterior abdominal wall to evaluate for the presence of defects. 2. Determine which organs are eviscerated 3. Look for other anomalies

The classic features of Beckwith-Wiedemann syndrome are:

1. Macrosomia (nephromegaly, hepatomegaly) 2. Macroglossia (which may interfere with breathing, swallowing, and speaking) 3. Omphalocele is usually noted

OEIS Syndrome stands for:

1. O: Omphalocele 2. E: Exstrophy of the bladder and rectum 3. I: Imperforate anus 4. S: Spinal defects: Spina bifida

The two most common abdominal wall defects are the:

1. Omphalocele 2. Gastroschisis

U/S appearance of meconium peritonitis is:

1. Peritoneal calcification (calcification in the peritoneal organs like liver, spleen) 2. The ascitic fluid also may be echogenic. 3. Hydramnios (polyhydramnios) is present in 65% of fetuses with meconium peritonitis

Duodenal atresia defect identified by sonography how?

1. The "double-bubble" (fluid-filled stomach and duodenum) 2. Polyhydramnios

What are the U/S appearances of gastroschisis?

1. The cord insertion is normal 2. No covering membrane present 3. Herniation occurs to the right of the cord insertion 4. The defect in the wall may be seen & eviscerated bowel or organs may be noted in the amniotic fluid 5. Small AC 6. Bowel in the fluid may appear large 7. Large defects may contain liver, bladder, & adnexa

Duodenal atresia usually results from:

1. The interruption of the duodenal lumen by a membrane 2. Annular pancreas 3. Duodenal stenosis

Associated findings with duodenal atresia are:

1. Trisomy 21 2. Polyhydramnios 3. Symmetric IUGR 4. Bowel malrotation 5. Cardiac anomalies

The herniated bowel returns to the abdomen during the _____ week.

11-12th

The diagnosis of omphalocele / gastroschisis cannot be made with certainty before _____ gestation.

13 week's

Omphalocele is associated with _____ trisomy's.

13, 18, Turner's syndrome & Beckwith-Wiedemann syndrome

The amniotic membrane supposed to fuse with the chorions membrane around _____ weeks.

16-17

Esophageal atresia is associated with trisomy _____ & _____.

18 & 21

The normal diameter of the fetal colon is less than or equal to:

18 mm

Duodenal atresia is identified by U/S after:

24 weeks of gestation

Normal colon is usually not seen until _____ trimester.

3rd

Pentalogy of Cantrell syndrome includes how many defects:

5

The normal diameter of the fetal small bowel is less than or equal to:

5 mm

The bowel normally herniates into the proximal umbilical cord in the _____ menstrual week.

8th

_____ presents as a complex disorder of the bowel and genitourinary tract.

Anorectal atresia

The most commonly observed anomaly associated with esophageal atresia is:

Anorectal atresia (congenital malformation where the anal or rectal opening is obstructed)

Abnormal fluid collection in the peritoneal cavity is called:

Ascites

How can you differentiate between ascites and psedoascites by U/S?

Ascites outlines the umbilical vein, fetal bladder, it moves with changing position Psedoascites does not outline organs and does not change position with fetal movement

The prognosis of anorectal atresiais poor with anorectal atresia because of:

Associated (VECTRAL) anomalies

_____ is classified as an overgrowth syndrome.

Beckwith-Wiedemann syndrome

In the case of cloaca, both the _____ & _____ are located outside of the lower abdominal wall.

Bladder and distal colon

_____ is a congenital abnormality in which part of the urinary bladder is present outside the body.

Bladder exstrophy

The most reliable criterion for diagnosing dilated bowel is an increase in:

Bowel diameter

Associated abnormalities that can be seen with bladder exstrophy are:

Cryptorchidism, omphalocele and hernia

The cause of bladder exstrophy is:

Deficiency in development of the lower abdominal wall musculature

U/S appearance of anorectal atresia is:

Dilated colon and calcified meconium

_____ is a condition in which the duodenum has not developed properly .It is not open and cannot allow the passage of stomach contents.

Duodenal atresia

AFP values in case of duodenal atresia are commonly normal, elevated or reduced?

Elevated

_____ is a congenital narrowing or obstruction of the esophagus.

Esophageal atresia

Another name for omphalocele is:

Exomphalos

Omphalocele is a congenital malformation in which variable amounts of abdominal contents protrude to the right of the umbilical cord.

False, is a midline defect

Sacrococcygeal teratoma is always benign. T/F?

False, may be benign or malignant

Gastroschisis less serious than omphalocele because:

Gastroschis is not associated with trisomies

_____ is the herniation of abdominal contents through a right-sided, periumbilical abdominal wall defect with no peritoneal covering over the bowel or other contents.

Gastroschisis

The most common abnormality that is associated with omphalocele is:

Heart defects

_____ is a disorder that occurs when a membrane covers the anus completely or partially closed.

Imperforate anus (anorectal atresia)

The 3 causes of meconium ileus are? Most cases of meconium ileus occur in newborns is?

Is the third most common form of neonatal bowel obstruction after atresia and malrotation. Most cases of meconium ileus occur in newborns is: Cystic fibrosis.

The purpose of the normal embryologic herniation of the bowel is:

It permits development of the intra-abdominal organs

Children born with Beckwith-Wiedemann syndrome are at an increased risk of developing:

Kidney cancer called Wilms tumor and liver cancer called hepatoblastoma

_____is a congenital condition that is characterized by abnormalities in the anterior body wall (chest and abdomen) and/or limbs (arms and legs).

Limb-body wall complex

Bladder exstrophy is commonly seen in males or females:

Males

Esophageal atresia is mostly common in:

Males

Gastroschisis has been found more frequently in males or females?

Males

Limb-body wall complex is associated with:

Maternal cocaine use

_____ is the earliest stools of an infant.

Meconium

Impacted meconium is called:

Meconium ileus

The meconium sometimes becomes thickened and congested in the ileum, a condition known as _____.

Meconium ileus

____ is a common cause of peritoneal calcification.

Meconium peritonitis

_____ is peritonitis resulting from perforation of the bowel into the peritoneal cavity in utero.

Meconium peritonitis

What is amniotic band syndrome?

Occurs when the fetus becomes entangled in fibrous string-like amniotic bands (membrane) in the uterus, restricting blood flow and affecting the fetal development. Is the rupture of the amnion, which leads to entrapment or entanglement of the fetal parts

The prognosis of omphalocele is worse than gastroschis is due to:

Omphalocele is associated with 13, 18, Turner's syndrome & Beckwith-Wiedemann syndrome

The differential diagnosis for bladder exstrophy includes:

Omphalocele or gastroschisis

Infants with cystic fibrosis have multiple medical problems, including:

Pancreatic disease and respiratory problems resulting from long-standing lung disease

Pseudoascites is caused by:

Prominent hypoechoic abdominal muscles appear almost anechoic in between the subcutaneous & peritoneal fat layers

In the case of gastroschisis, the defect is located to the _____ of the umbilicus.

Right

_____ most common tumor in neonates.

Sacrococcygeal teratoma

_____ is reversal of organs.

Situs inversus

Limb-body wall complex is also known as:

The body-stalk syndrome

How can you differentiate between sacrococcygeal teratoma and myelomeningoceles?

The brain anatomy is normal in case of sacrococcygeal teratoma.

_____ is the embryonic structure that develops into the rectum and urogenital sinus (reproductive and urinary system).

The cloaca

_____ is a connection or hole between the lower esophagus and the trachea.

Tracheoesophageal fistula

90% of the cause of esophageal atresia in fetuses is:

Tracheoesophageal fistula (abnormal connection between the esophagus and trachea.

Infants with duodenal atresia require immediate surgery after birth to connect the stomach to the jejunum, thus bypassing the obstruction. T/F?

True

Maternal serum AFP levels are higher in gastroschisis than omphalocele. T/F?

True

Omphalocele is central, at the base of the umbilical cord, & contained by a membrane. T/F?

True

The body-stalk syndrome is a lethal disorder. T/F?

True

The partial situs inversus is more severe than the complete situs inversus. T/F?

True

_____ is a nonrandom association of birth defects that affects multiple organ systems.

VACTERL association