Gastro N-Z Flashcards

1
Q

What is the definition and epidemiology of Necrotising enterocolitis NEC?

A

Severe gastrointestinal disease characterized by massive epithelial destruction leading to intestinal barrier failure.

0.5% births.

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
2
Q

What is the aetiology of Necrotising enterocolitis NEC?

A

Unknown, likely multifactorial. Hypothesis: immature intestinal battier and mucosal immune system -> bacterial translocation and epithelial inflammation. Localised injury leads to increased infiltration in that area leading to more infection and more inflammation -> further damage.

Eventually, intestinal necrosis and sepsis.

Bell’s classification I (suspect, non septic signs) II (definite, blood investigation and XR changes), III (advanced shock, peritonitis or pneumoperitoneum)

Associated with prematurity, low BW, formula feeding, stress, bacterial colonization. UVA/C insertion, CHD, maternal cocaine nuse, RDS.

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
3
Q

What are the history and exam findings of Necrotising enterocolitis NEC?

A

Stage I: temperature instability, bradycardia, aopnea, lethargy, poor feeding, bilious aspirates, GI bleeding, ileus on XR.

StageII: + metabolic acidosis, thrombocytopenia, GI bleeding, tender abdomen, wall erythema, mass, AXR signs

Stage III: + shock, marked peritonitis, abdominal distension, neutropenia, DIC, ionotropic support, pneumoperitoneum on AXR.

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
4
Q

What investigations would you do for Necrotising enterocolitis NEC?

A

Bloods: depend on stage, but usually low Hb, high WCC, metabolic acidosis and electrolyte imbalances

AXR: Football sign of air collecting in anterior abdomen when supine, may see fxed dialted loops of bowel, outlined falciform ligament, pneumoperitoneum.

USS: operator dependent, may see intramural gas.

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
5
Q

What is the management of Necrotising enterocolitis NEC?

A

Conservative: Stages I/II, NBM, IV feed and fluids, NGT decompression, IV Abx, Blood/platelet transfusion and electrolyte monitoring.

Surgical: failure of conservative management, Laparotomy via supraumbilical transverse incision with resection of necrosed bowel and formation of stoma/mucous fistula.

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
6
Q

What are the complications and prognosis of Necrotising enterocolitis NEC?

A

Stricture, fistula, recurrence, short gut syndrome, malabsorption, TPN associated cholethiasis.

20-40% of NEC require surgery. Mortality 50%.

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
7
Q

What is Neonatal jaundice?

A

Excess bilirubin leading to yellow discoloration of skin and sclera.

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
8
Q

What is the background of Neonatal jaundice?

A

50% of neonates to different degrees.

Unconjugated (pre hepatic)

· Physiological

o Unstable fetal Hb: high turnover

o Defective BR metabolism: low hepatic metabolism in neonates.

o Defective excretion: underdeveloped gut flora.

o Breast milk jaundice: 2nd week to 3rd month, due to UPDGR inhibition

· Pathological

o Haemolytic

§ AIHA

§ HDN

§ Hereditary spherocytosis, G6PD

§ Congenital infection, bacterial sepsis.

o Non-haemolytic

§ High Hb load: haemorrage or polycythaemia

§ Galactosaemia and hypothyroidism

Conjugated (hepatic / post hepatic)

· Neonatal hepatitis: TPN related, congenital infection, a1ATD, CF.

· Bile duct obstruction: biliary atresia, coledochal cyst

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
9
Q

What is the history and exam findings of Neonatal jaundice?

A

General: May be asymptomatic (phys) or unwell (poor feeding, vomiting, fevers confusion, seizures, behavioral etc)

Age of onset: <24h pathological, >24h probably physiological, >2/52 breast milk

Clinical jaundice starts at BR level 80-120 umol/l. Look in sclera.

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
10
Q

What investigations would you use for Neonatal jaundice?

A

Early(<24h): FBc, film, maternal/infant ABO, maternal TORCH screen, DAT

> 24h: Blood + film, examine

Persistent (>2wk) Total serum BR and conj/unconj fraction. Then bloods or USS.

Conjugated: requires urgent investigation, USS biliary tree of biopsy isotope scanning.

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
11
Q

What is the management of Neonatal jaundice?

A

General: tx cause if present.

Tx jaundice: prevent bilirubin encephalopathy (kernicterus)

Phototherapy: Place neuonate under 450nm wavelength lights on warm bed. Converts stereoisomer rendering it soluble and leading to renal excretion.

Exchange transfusion: if intensive phototherapy fails, or extreme cases. Must have hearing screening.

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
12
Q

What is the complications and prognosis of Neonatal jaundice?

A

BR has neurotoxic effects: seizures, cerebral palsy, sensorineural deafness, LDs.

Good if physiological, spontaneous resolvement.

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
13
Q

What is Oesophageal atresia / trachea esophageal fistulae?

A

Congenital malformation of the oesophagus leading to loss of continuity in the lumen, leading to an upper or lower oesophageal pouch. TOFs are abnormal communication between oesophagus and trachea and are related to OA.

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
14
Q

What is the background of Oesophageal atresia / trachea esophageal fistulae?

A

1/4500.

General: Aetiology unclear. Spritx classification involves judgement of presence/absence of cardiac abnormalities and low BW extent.

Classification:

· Proximal OA with distal/proximal TOF (or both)

· Isolated OA

· H type TOF without OA

Associated with VACTERL (vertebral, anorectal, cardiac, tracheal, oesophageal, renal, limb) / CHARGE (coloboma, heart defects, atresia choanal, retarded G&D, genital hypoplasia, ear deformities). SCHISIS (Exhomphalos, cleft lip and palate, genital hypoplasia). Potter syndrome (bilateral renal agenesis) and Trisomy 18.

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
15
Q

What is the history and exam findings of Oesophageal atresia / trachea esophageal fistulae?

A

Prenatal: polyhydramnios due to intestinal obstruction. Small or absent stomach bubble in scan.

Postnatal: all infants with hx of polyhydramnios should have NG tube passed at birth. May present with coughing and choking at first feed.

Abdominal distension in TOF, or scaphoid abdomen. Examine other anomalies.

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
16
Q

What investigations would you use for Oesophageal atresia / trachea esophageal fistulae?

A

Plain XR: CXR confirms dx with coiled NGT in upper pouch. Distal bowel gas shows distal TOF.

Specific: ECHO for other abnormalities.

17
Q

What is the management of Oesophageal atresia / trachea esophageal fistulae?

A

Initial: immediate transfer to neonatal surgical centre. Head up and prone, preventing regurgitation. Suciton and irrigation to upper pouch. NBM.

Surgical: (short gap) Single stage correction with anastomosis of upper and lower ends with TOF ligation. Chest drain may be left at anastomotic site.

Surgical (long gap): Placement of Peg and ligation of TOF then allow child growth. Secondary surgery with oesophageal replacement with gastric transposition. Thoracotomy or thoracoscopic.

18
Q

What is the complications and prognosis of Oesophageal atresia / trachea esophageal fistulae?

A

Surgical: anastomosis leak, dysmotility, GORD, strictures, recurrence of fistula. Tracheomalacia, failure to thrive and other complicaitons too

Survival rates 90%. RF are low BW, and other abnormalities. Long gap OA require the most operations.

19
Q

What is Pyloric stenosis?

A

Hypertrophy and hyperplasia of the pyloric sphincter muscle leading to gastric outflow obstruction. AKA HPF.

20
Q

What is the background of Pyloric stenosis?

A

1-500 live births.

Multifactorial – genetic and environmental. May be due to deficiency of NOS containing neurons at pylorus, abnormal innervation, hypergastrinaemia, hyperacidity.

Due to hypertrophy or hyperplasia of the clls in the circular/longitudinal muscle wall muscles in gastric pylorus.

21
Q

What is the history and exam findings of Pyloric stenosis?

A

Presents at 2-6/52, with progressive non bilious projectile vomiting after prolonged feeds, which can be projectile. May have coffeeground vomiting from Mallory Weiss tear.

Persistently hungry after vomit, constipated, failure to thrive.

Systemic: weight loss, dehydration, CRT high , low skin turgor, sunken fontanelle, low urine output.

GI: visible peristalsis from left to right LUQ during feed, Palpation of pyloris feels like olive mass and aided by use of test feed.

22
Q

What investigations would you use for Pyloric stenosis?

A

Bloods: ABG shows classic: low K, metabolic alkalosis, hypochloraemia.

USS abdomen used to aid diagnois. Pyloric muscle parameter of >4mm

23
Q

What is the management of Pyloric stenosis?

A

Preoperative: electrolyte imbalance correction.

Operation: Ramstedt pyloromyotomy – longitudinal incision through serosa with splitting of sphincter muscle. Can be supraumbilical incision or laporoscopic.

24
Q

What is the complications and prognosis of Pyloric stenosis?

A

Recurrence uncommon, <1% morbidity.

Post operative vomiting common but settles in 25h. .

25
Q

What is Small bowel atresia?

A

Congenital malformation in the GI tract (duodenum, JJ etc) leading to absence or closure of a part of the lumen.

26
Q

What is the background of Small bowel atresia?

A

DD atresia: Duodenum ends in blind pouch past Ampulla of Vater. Type I: duodenal web or wind sock. Type II: complete obstruction with fibrous cord between prox/dist pouches. Type III: complete gap.

JJ or IL atresia: Type I membranous, Type II (intact mesentery but cord between pouches) type III (mesenteric defect with gap between pouches), Type IV (Multiple JJ atresia.

General: Cause unknown.

DD 1/ 5k , JJ 1 /2 K

27
Q

What is the history and exam findings of Small bowel atresia?

A

Depends on level and extent. USS dx before birth. NNU admission at birth, AXR.

Vomiting may be seen, may be bile stained or non bilious depending on atresia site.

28
Q

What investigations would you use for Small bowel atresia?

A

AXR: dilated bowel loops, double bubble (absent/reduced gas post obstruction). Contrast studies may be warranted. Examine for other potential malformations.

29
Q

What is the management of Small bowel atresia?

A

General: NGT and stabilize. IV fluids and feeds. Explore other anomalies.

Surgical: transverse supraumbilical incisions, primary anastomosis between pouch ends,

30
Q

What is the complications and prognosis of Small bowel atresia?

A

Aspiration, anastomotic complications, stenosis or leak, abnormal peristalsis in proximal bowel, SBS.

Mortality related to severity of associated abnormalities, not this one itself. Dependent on prematurity.

31
Q

What is a retinoblastoma?

A
  • The most common intraocular tumour in children

* Malignant tumour of retinal cells

32
Q

What is the epidemiology of retinoblastoma?

A
  • Accounts for 5% of severe visual impairment in children
  • Can be unilateral or bilateral
  • All bilateral tumours are hereditary, approx. 20% of unilateral cases are hereditary
33
Q

What is the aetiology of retinoblastoma?

A

• Affects retinoblastoma susceptibility gene on chromosome 13
• Dominant inheritance with incomplete penetrance
• Most children present within first 3 years of life
• Children from families with hereditary form of disease should be screened regularly from
birth

34
Q

What are the clinical features of retinoblastoma?

A
  • White pupillary reflex replaces normal red reflex (usually seen incidentally)
  • Squint
35
Q

What investigations do you do for retinoblastoma?

A
  • MRI
  • Examination under anaesthetic
  • Tumours are often multifocal
36
Q

What is the management for retinoblastoma?

A

• Aim to cure but preserve vision
• Chemotherapy is used to shrink tumours
• Laser treatment of the retina
• Radiotherapy may be used in more advanced cases
• MOST PATIENTS ARE CURED
• However, many will be visually impaired
• Some patients require enucleation (removal of the eye)
• Significant risk of second malignancy (especially sarcoma) among survivors of hereditary
retinoblastoma