Neonatal Surgery (TOF; intussusception; CDH) Flashcards
What is the incidence of TOF?
1:3000 - 1:4500
What are the anomalies often associated with TOF?
Associated anomalies in 50%: VACTERL association
What is VACTERL?
Disorder that affects many body systems. Stands for: - Vertebral defects - Anal atresia - Cardiac defects - TOF - Renal abnormalities - Limb abnormalities Generally 3+ for VACTERL diagnosis
What are the CFx of TOF?
VARIABLE with fistula type. KEY = FROTHY - Hx Maternal polyhydramnios Several months (no atresia): - Non - bilious vomiting - Cyanosis with feeds - Coughing - Respiratory distress - Recurrent pneumonia - Frothy bubbles of mucous in mouth and nose that return after suctioning
Rx TOF?
- Call PIPER
- Transfer out with films
- Check for other abnormalities (VACTERL)
- Early repair with surgical ligation to prevent lung damage and maintain nutrition and growth
What are the complications of TOF?
- Pneumonia
- Sepsis
- Reactive airways disease
What are the complications of TOF post repair?
- Oesophageal stenosis and strictures at site
- GORD
- Poor swallowing (dysphagia, regurg)
What is the most common cause of bowel obstruction between 6-36mo?
Intussusception
What are the pathologic lead points of intussusception?
- Enlarged Peyer’s patches due to viral infections of GIT
- Polyps
- Meckel’s diverticulum
- Lymphoma
What is the pathophysiology of intusussception?
Usually idiopathic.
- Often starts at ileocaecal junction
- telescoping of bowel into itself causing obstruction and vascular compromise
CFx intussusception?
- Acute onset abdo pain
- Episodic, “colicky”
- Vomiting +/- bilious
- Abdo mass
- Red currant jelly stools suggests mucosal necrosis and sloughing
PEx features intussusception?
Abdo exam:
- palpate for masses (esp SAUSAGE shaped upper abdo mass) and tenderness
- Signs BO: distension
- Look for localised peritonitis = transmural ischaemia
Ix intussusception?
- AXR for BO or perforation
- US if suspect pathology
Rx intussusception?
- Peritonitis = operative
- No peritonitis = air enema reduction
Prongosis intussusception?
- 10% recurrence (more likely non - idiopathic)
What is the incidence of intestinal atresia?
2 - 14%
What is the pathophysiology of duodenal atresia?
Failure of bowel to recanalize after endodermal epithelium proliferation (W8 - 10)
What is the pathophysiology of jejunal / ileal atresia?
Acquired as result of vascular disruption -> ischaemic necrosis -> resorption of necrotic tissue -> blind distal and proximal ends
CFx duodenal atresia?
- Gastric distension and vomiting (usu Bilious)
- VACTERL association
- 24% have DS
PEx approach to intestinal atresia?
- Complete physical (esp abdo, anus)
- Evaluate for resp distress
- Evaluate volume status
- Congenital anomalies (VACTERL)
- Jaudice
Ix intestinal atresia?
- Contrast enema +/- UGI with small bowel follow through
- Group and screen
- INR and PTT if for surgery
Rx intestinal atresia?
- NBM
- NG tube decompression
- Fluid resuscitation
- TPN
- Broad spec ABx
- Surgery
What is the incidence of hypertrophic pyloric stenosis?
0.03 - 1% live births
When do infants with hypertrophic pyloric stenosis generally present?
1 - 20w; most common = 6-8w
What is the gender spread of hypertrophic pyloric stenosis?
4M:1F
Which ABx represents a RFx for HPS?
Early erythromycin exposure (<13d)
What is the pathophysiology of HPS?
- Acquired pyloric circular muscle hypertrophy resulting in gastric outlet obstruction.
- Hypovolemia due to vomiting of gastric contents ==> hypochloremic hypokalemic metabolic alkalosis
CFx HPS?
- Projectile non bilious vomiting 30 - 60min after feeds.
- Hungry after vomiting
- Dehydration
PEx features HPS?
“Olive”: 1-2cm smooth palpable mass above umbilicus.
- visible L - R gastric contraction “waves” after feeding
Ix HPS?
-UEC: ELECTROLYTES
- US: pyloric length 14mm+ and thickness 4mm+
Upper GI series only when US unavailable or not diagnostic: will show “string sign”
Mx HPS?
- Fluid resuscitation with NSal
- Correct acid/base anomalies with D5, 1/2NSal + KCl maintenance
- NG tube decompression not required
- Pyloromyotomy (Ramstedt vs transumbilical or lap approach)
What are the types of congenital diaphragmatic hernia?
3 types of CDH:
- Posterolateral (Bochdalek; 85% on LHS)
- Anterior (Morgagni)
- Hiatus
When does CDH present
Within hours of life
What is the pathophysiology of LHS CDH?
- SB and LB
- Stomach
- Solid viscera (spleen, L lobe of liver)
herniate into thorax
PLUS: - pulmonary hypoplasia
- pulmonary HTN
What is the pathophysiology of RHS CDH?
-Liver
- LB
Herniate into thorax
PLUS:
- pulmonary hypoplasia
- pulmonary HTN
What are the CFx of CDH?
- Early respiratory distress
- Cyanosis
- Scaphoid abdomen
- Prenatal diagnosis
PEx features of CDH?
- Decreased air entry +/- bowel sounds in the chest
- Displaced heart sounds (R as most hernias on L)
Ix in CDH?
- Prenatal US / MRI
- ABG
- CXR (bowel loops in hemithorax, shifted heart)
- Echo
- Genetic consultation if warranted
Mx CDH?
- Intubate
- Orogastric suction
- KEY: tertiary transfer
- Period of respiratory stabilisation due to associated pulmonary hypoplasia (may need ECMO)
- Surgical repair when stable
What is malrotation?
Failure of to normally rotate around superior mesenteric artery with associated abnormal intestinal attachments and anatomic positions. Spectrum of rotation abnormalities
Age of presentation of malrotation?
- 1/3 by 1w
- 3/4 by 1mo
- 90% by 1y
What is the cardinal sign of malrotation?
Bilious vomiting (esp if non-distended abdomen)
What are the PEx features of malrotation?
- Bilious drainage from NGT
- Tachycardic, pale
- Diaphoretic
- Flat abdomen
- Tenderness
AXR features of malrotation?
- obstruction of proximal small bowel
- “double bubble” sign
- intestinal wall thickened
Mx malrotation?
- IV ABx
- Fluid resuscitation
- Emergent laparotomy:
LADD procedure
What is Ladd procedure?
- Couterclockwise reduction of midgut volvulus
- division of Ladd’s bands
- division of peritoneal attachments b/w caecum and abdo wall that obstruct duodenum
- broadening of mesentery
- +/- appendicectomy
What is gastroschisis?
Defect of abdominal wall with free extrusion of intestine in amniotic cavity
mx gastroschisis?
- NG decompression
- IVF
- IVABx
- Call PIPER
- Put in humidiheat
- Keep viscera moist and protected (GLAD WRAP) until surgical reduction with primary abdo closure OR staged closure with silo
- May have bowel dysmotility and require motility medications
What is gastroschisis associated with?
- younger maternal age
- IUGR
What is an omphalocele?
- defect of abdominal wall with extrusion of sac covered viscera
What is the rule of 2s for Meckel’s diverticulum?
- 2% of population
- 2M : 1F
- 2% symptomatic
- Within 2ft of IC valve
- 2 inches in diameter
- 2 inches in length
- 2 types of tissue (gastric, pancreatic)
- Often presents by 2y of age
What does bilious vomiting represent in an infant?
Life threatening emergency secondary to midgut volvulus until proven otherwise
What is the pathophysiology of Hirschsprung’s disease?
Defect in migration of neurocrest cells to intestine resulting in:
- aganglionic bowel that fails to peristalse and
- internal sphincter that fails to relax (internal anal sphincter achalasia)
CFx of Hirschsprung’s disease?
- Failure to pass meconium in first 48h
- Sx of BO: distension, constipation, bilious emesis
Ix Hirschsprung’s disease?
- Rectal biopsy (aganglionosis and neural hypertrophy)
- AXR
- Contrast enema (narrow rectum and transition zone)
What is the first Ix to perform in ?intussusception?
AXR to exclude perforation or obstruction
What should be completed after AXR in intussusception work up?
US
