Congenital Disorders In The New Born And Childhood

Question 1

All congenital disorders manifest as structural abnormalities.

True or False?

Answer 1

False

Can manifest either as structural or functional abnormalities.

Question 2

Thalidomide is teratogenic

True or false

Answer 2

True

Causes loss of limbs in newborns

Question 3

List some of the predisposing factors for congenital disorders in newborns

Answer 3

Alcohol
Smoking
Drugs
Irradiation
Infections (TORCHES)
Nutritional
Insults during first trimester

Question 4

List the congenital anomalies of the head, neck and spine

Answer 4

Hydrocephalus
Spina bifida
Encephalocoele

Question 5

List the congenital anomalies of the thoracic region

Answer 5

Oesophageal atresia + - TOF
Diaphragmatic hernias
Cardiac anomalies
Pulmonary malformations

Question 6

List the congenital anomalies of the urological system

Answer 6

Hypospadias
Bladder exstrophy
Posterior urethral valve
Disorders of sexual development

Question 7

List the congenital anomalies of the gastrointestinal tract

Answer 7

Hydrocoeles & Hernia
Prune belly syndrome
Gastrochisis
Exomphalos (omphalocele)
Hirschsprung’s disease
Malrotation
Intestinal atresias
Anorectal malformations
Biliary atresia
Choledochal cyst

Question 8

List the congenital anomalies of the limbs and musculoskeletal system

Answer 8

Syndactyl
Polydactyl
Hemimelia
Sirenomelia
Conjoint twins

Question 9

What is the principle of management for congenital disorders in the new born

Answer 9

1) Genetic counseling
2) Prenatal diagnosis (anomaly scan @20 weeks and amniocentesis)
3) Foetal intervention (sacrococcygeal teratoma, amniotic fluid augmentation)
4) Elective termination of pregnancy
5) Delivery at a facility that can handle neonatal and pediatric surgical care
6) Multidisciplinary team approach

Question 10

In intrauterine life physiologic herniation occurs at what age?

Answer 10

6th to 10th week GA

Question 11

The membrane that covers the intestine in omphalocoele consists of

Answer 11

Visceral peritoneum
Wharton’s jelly
Amniotic membrane

Question 12

What are the contents of the omphalocoele sac

Answer 12

Intestine
Stomach
Liver (in 50% of cases)

Question 13

How do you classify omphalocoele

Answer 13

There are two ways of classification
A)
i. Syndromic
ii. Non-syndromic

B)
i. Major
ii. Minor

Question 14

What are the forms of syndromic omphalocoele

Answer 14

1) Upper midline (pentalogy of Cantrell)
cardiac anomaly
ectopia cordis
posteriolateral diaphragmatic hernia
sternal cleft
omphalocoele

2) Central midline (beckwith-wiedemann) syndrome
omphalocoele
macroglosia
hypoglycemia

3) Lower midline syndrome
omphalocoele
coacal extrophy
anorectal malformation

Question 15

What is the management of omphalocoele

Answer 15

1) Care of sac (with or without antibiotics)

2) Assessment for size and other anomalies

3) Surgical management
i. Initial management with late closure
ii. Primary closure
iii. Staged closure using skin or silo

Question 16

Where is the site that gastroschisis occurs?

Answer 16

At the site of involution of the 2nd umbilical vein

Question 17

At what age does gastroschisis occur?

Answer 17

4th week of gestation

Question 18

Gastroschisis is usually associated with other congenital anomalies

True or False

Answer 18

False

Question 19

What are the differences between omphalocoele and gastroschisis based on
Sac
Site of defect
Peritoneal cavity
Maternal age
Other congenital anomalies
State of bowel
Time to operate
Return of bowel function

Answer 19

Know this for OSCE, possible theory and MCQ