Pedia 2 Flashcards
What is congenital umbilical hernia? Why does it happen
It occurs due to failure of the mid gut to return to the abdominal cavity
Not covered with skin, contents are visualized
What are the 2 types of congenital umbilical hernia?
Exomphalos major and exomphalos minor
What is the difference between Exomphalos Major and exomphalos minor?
[size of defect? Covering? Contents? Treatment?]
Defect:
Exomphalos major: Large (>5cm)
Exomphalos minor: Small (<5cm)
Sac:
Exomphalos major: Ambiotic membrane
Exomphalos minor: Amniotic membrane & Wartons jelly
Contents:
Exomphalos major: Any abdominal viscera (up to liver)
Exomphalos minor: Usually small loop of intestine
Treatment:
Exomphalos major: Complex and needs staged repair
Exomphalos minor: Simple, reduction, herniotomy and herniorraphy
Cause of infantile umbilical hernia?
Imperfect closure of the umbilical scar
What is the covering of the infantile umbilical hernia?
Skin
Treatment of infantile umbilical hernia?
-Spontaneous closure
-Surgical repair may be indicated if persists for more than 2 years or with defect more than 2 fingers
What is gastrochisis?
Congenital defect of the anterior abdominal wall with intact peritoneum
May occur at umbilicus or away from it
(Picture of baby with intestines poking out)
What is Prune belly syndrome
Congenital defect of the anterior abdominal wall with lack of abdominal musculature so skin is wrinkled
Treatment of prune belly syndrome?
Surgical reconstruction and repair of the abdominal wall + management of the associated anomalies
What medical conditions are associated with prune belly syndrome?
Undescended testis
Urinary tract malformations (especially mega-ureter) with vesico-ureteric reflux
What happens in Ectopia vesica?
Absent lower abdominal wall
Absent anterior bladder wall
Wide separation of the symphysis pubis
Other urinary anomalies especially episadius
(Picture of absent wall over the bladder)
Complications of ectopia vesica?
Severe UTI & metaplasia (precancerous)
Treatment of ectopia vesica?
Temporary closure of defect
Then> definitive reconstruction of the bladder and pelvic wall
From where can congenital diaphragmatic hernia herniate?
Posterior hernia: foramen of bochdalek (most common)
Anterior hernia: foramen of morgagni
Clinical presentation of diaphragmatic hernia?
Maternal polyhydraminos
Neonatal cyanosis & respiratory distress
Signs: scaphoid abdomen
Diminished air entry with audible intestinal sounds on the chest
Heart sounds heard better on the right side
What investigations would you do for Congenital diaphragmatic hernia
Plain chest x ray (diagnostic)
-presence of bowel in the chest
-shift of the mediastinum to the contra lateral side
-Apparent dextrocardia
Gastrograffin meal
Blood gases: hypoxia hypercapnia &acidosis
Treatment of congenital diaphragmatic hernia (pre-operative and operative)
Pre-operative:
•NGT & IV fluids: to deflate the stomach→decrease the lung compression & the mediastinal shift.
•Endotracheal intubation
•Extracorporeal membrane oxygenation (ECMO).
•Pulmonary vasodilators:
o Nitric oxide (NO) inhalation.
o Non-selective α-adrenergic blockers.
o Phosphodiestrase inhibitors (Sildenafil).
Operative repair:
• Open (through subcostal incision).
• Thoracoscopic.
✓ Principles:
• Reduction of the abdominal contents.
• Repair of the diaphragmatic defect either by sutures or by application of Gortex patch.
• Closure should be performed without tension.
Which type of biliary atresia is correctable?
Portion of the extrahepatic tree only is occluded
Which type of biliary atresia is non-correctable?
When it’s both Intrahepatic and extrahepatic atresia
Presentation of biliary atresia?
Neonatal obstructive jaundice (since birth)
Treatment for correctable and non correctable biliary atresia?
- Correctable: Kasai operation (Roux en Y hepatico-jejunostomy)
- Non-correctable: liver transplantation
What investigations would you do for biliary atresia
US (initial)
HIDA scan (confirmatory)
ERCP
Definition & clinical presentation of choledocal cyst?
Congenital cystic dilatation of the intra &/or extra hepatic biliary system
Presentation:
Triad of:
- Childhood obstructive jaundice. (Not since birth)
- Rt. hypochondrial swelling.
- +/- Pain.
What can predispose you to cholangiocarcinoma?
Choledocal cyst
Treatment for choledocal cyst?
▪Treatment:
- Extra-hepatic only: Excision of the cyst with Roux-en-Y Choledocho jejunostomy.
- Intra-hepatic affection (Caroli’s disease): liver transplantation.
Investigations for Choledocal cyst?
-US (initial)
-CT
-HIDA scan (confirmatory)
-ERCP
What are the complications of congenital diaphragmatic hernia?
Ipsilateral pulmonary hypoplasia
Contralateral lung compression
Strangulation (of the bowel)
Cause of congenital diaphragmatic hernia?
Normal embryology:
Fusion of:
• septum transversum
• pleuroperitoneal folds
Rt. fuses before Lt. making Lt. weaker
