Fetal abdominal wall and abdomen

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

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

Tracheoesophageal fistula (abnormal connection between the esophagus and trachea.

90 % the cause of esophageal atresia in fetuses is

Elevated

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

Ascites

Abnormal fluid collection in the peritoneal cavity

Exomphalos

Another name for the omphalocele

1. Bowel perforation 2. Fetal hydrops 3. Infection

Ascites may result from:

Cryptorchidism, omphalocele and hernia

Associated abnormalities that can be seen with Bladder exstrophy are:

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

Associated findings with duodenal atresia are:

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

Associated findings with gastroschisis are:

Males

Bladder exstrophy is commonly seen in males or females:

Kidney cancer called Wilms tumor and liver cancer called hepatoblastoma

Children born with Beckwith-Wiedman Syndrome are at an increased risk of developing:

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

Differential diagnosis for duodenal atresia includes:

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

Duodenal atresia defect identified by sonography

24 weeks of gestation

Duodenal atresia is identified by us after

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

Duodenal atresia usually results from

18 & 21

Esophageal atresia is associated with trisomy

Males

Esophageal atresia is mostly common in males or females?

Males

Gastroschis is been found more frequently in males or females:

Gastroschis is not associated with trisomies

Gastroschis less serious than omphalocele because

Ascites outlines the umbilical vein, fetal bladder, it moves with changing positionPsedoascites does not outline organs 2. Psedoascites does not change position with fetal movement

How can you differentiate between ascites and psedoascites by u/s?

The brain anatomy is normal in case of sacrococcygeal teratoma.

How can you differentiate between sacrococcygeal teratoma and myelomeningoceles?

Meconium ileus

Impacted meconium is called

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

Infants with cystic fibrosis have multiple medical problems, including:

to connect the stomach to the jejunum, thus bypassing the obstruction

Infants with duodenal atresia require immediate surgery after birth. Why?

may be benign or malignant

Is Sacrococcygeal teratoma always benign or malignant?

Bladder exstrophy

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

Esophageal atresia

Is a congenital narrowing or obstruction of the esophagus

Tracheoesophageal fistula

Is a connection or hole between the lower esophagus and the trachea

VACTERL association

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

Lethal Disorder

Is body-stalk syndrome a lethal disorder or non-lethal disorder?.

Situs inversus

Is reversal of organs.

Gastroschisis

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

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

The body-stalk syndrome

Limb- Body Wall Complex is also known as:

Maternal cocaine use

Limb- Body Wall Complex is associated with:

Cystic fibrosis

Most cases of meconium ileus occur in newborns is:

3rd trimester

Normal colon is usually not seen until

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

OEIS Syndrome stands for:

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

Omphalocele is Associated with

midline defect

Omphalocele is a

of the umbilical cord, & contained by a membrane

Omphalocele is central, at the base

5

Pentalogy of Cantrell/ syndrome includes how many defects:

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)

Possible causes of obstruction are:

Anorectal atresia

Presents as a complex disorder of the bowel and genitourinary tract

Prominent hypoechoic abdominal muscles appear almost anechoic in b/n the subcutaneous & peritoneal fat layers

Pseudoasctes is caused by:

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

The 2 Causes of non-visualization of the stomach are

16-17 weeks

The amniotic membrane supposed to fuse with the chorions membrane around

8th menstrual week

The bowel normally herniates into the proximal umbilical cord in the

Deficiency in development of the lower abdominal wall musculature

The cause of bladder exstrophy is:

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

The classic features of Beckwith-Wiedman Syndrome are

13th weeks gestation

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

Omphalocele or gastroschisis

The differential diagnosis for bladder exstrophy includes:

11-12th week

The herniated bowel returns to the abdomen during the

Meconium ileus

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

Heart defects

The most common abnormality that is associated with omphalocele

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

The most commonly observed anomaly associated with esophageal atresia is

Bowel diameter

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

18 mm

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

5 mm

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

complete Situs inversus

The partial situs inversus is more severe than

Associated (VECTRAL) anomalies

The prognosis of anorectal atresia is poor with anorectal atresia because of

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

The prognosis of omphalocele is worse than gastroschisis due to:

It permits development of the intra-abdominal organs

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

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

The two major defects under Pentalogy of Cantrell/ syndrome are:

The two most common abdominal wall defect are the:

The two most common abdominal wall defect are the:

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

U/S appearance of Amniotic Band Syndrome is:

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

U/S appearance of Beckwith-Wiedman Syndrome includes:

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

U/S appearance of Bladder exstrophy is

Dilated colon and calcified meconium

U/S appearance of anorectal atresia is

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

U/S appearance of meconium ileus is

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

U/S appearance of meconium peritonitis is:

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

US appearances of Limb- Body Wall Complex is

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

US appearances of Pentalogy of Cantrell/ syndrome are:

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

US appearances of esophageal atresia are

1. Ectopia cordis (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)

What are the 5 features of Pentalogy of Cantrell?

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

What are the u/s appearances of gastroschisis?

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

What are the u/s appearances of omphalocele?

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

What is Amniotic Band Syndrome?

Both the Bladder and distal colon

are located outside of the lower abdominal wall

to the right of the umbilicus

in case of gastroschisis the defect is located

Meconium peritonitis

is a common cause of peritoneal calcification.

Duodenal atresia

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

Imperforate anus (anorectal atresia )

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

Beckwith-Wiedman Syndrome

is classified as an overgrowth syndrome

Meconium peritonitis

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

Meconium

is the earliest stools of an infant.

The cloaca

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

Maternal serum AFP

levels are higher in gastroschisis than omphalocele.

Sacrococcygeal teratoma

most common tumor in neonates.