Paediatrics Flashcards

1
Q

How should a neonate with bowel obstruction be transported? (8)

A

Two sides
Tubes (NG):relieve symptoms, prevent vomiting and aspiration pneumonia, improve ventilation (severely distended abdo splints diaphragm), measure and replace fluid and electrolyte loss, evaluate level of obstruction
Warmth: incubator/space blanket /aluminium foil
Oxygen:combat anaerobic infection, support respiration. not more than 40%! (Eye complications)
Stabilise/prevent sepsis: evaluate and correct 5 Hs - hypoxia, hypotension, hypothermia, hypoglycaemia! (Check for every neonate); hydration
Iv fluid
Documents: referral letter, consent (operation, blood transfusion, contrast) , contact details family
Escort: qualified nurse or doctor
Specimens

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2
Q

Where is level of obstruction If NGT is draining saliva in neonate?

A

Oesophageal atresia

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3
Q

Where is level of obstruction If NGT is draining milk in neonate?

A

Stomach outlet obstruction

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4
Q

Where is level of obstruction If NGT is draining bilious green fluid in neonate?

A

Post ampula of vater
Surgical emergency until proven otherwise!

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5
Q

How and which fluids are given to neonates as a general rule? (5)

A

Day 1: 60 ml/kg/24h of 5% dextrose in water (no na/k) or potassium free neolyte
Day 2: 90 ml/kg/24h of neolyte (10% dextrose)
Day 3: 120 ml/kg/24h of neolyte (10% dextrose)
Day 4-30: 150 ml/kg/24h of neolyte (10% dextrose)
Day 30-1 year: 150 ml/kg/24h of paediatric maintenance fluid

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6
Q

! Differential diagnosis neonatal (congenital) bowel obstruction (6)

A

• Atresias 80%: small bowel atresia, anorectal malformation, oesophageal atresia
• Hirschprung’s disease 10%
• malrotation with (midgut) volvulus (not sigmoid in children!)
• meconium ileus

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7
Q

Prenatal diagnosis of neonatal bowel obstruction? (5)

A

• History pregnancy and mother: cystic fibrosis, diabetes, medication, AMA, intrauterine infection
• polyhydramnios especially in high obstructions
• prenatal ultrasound: distended fluid filled stomach, duodenum and small bowel

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8
Q

Duodenal atresia symptoms and associated malformations? (4)

A

• Early bile stained vomiting hours after birth, can be delayed if incomplete obstruction
• Associated malformations: heart defects 20%, down syndrome 30%, Vacterl association

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9
Q

Definitive Treatment duodenal atresia?

A

Duodenoduodenostomy

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10
Q

Treatment meconium ileus?

A

Wash out with saline to loosen stool. If that did not work, refer- probably Hirschprung’s (present similarly)

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11
Q

Define hirschprung disease

A

Congenital Aganglionosis of distal colon and rectum (can’t develop, present at birth)
Of myenteric layer (Auerbach plexus) and submucosal (meissner)

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12
Q

Embryology of hirchsprung disease? (5)

A

• Neural crest cells migrate into gi tract (5th to 10th week gestation) from cranial to caudal
• In myenteric and submucosal layers in Auerbach and Meissner plexuses respectively
• migration stop before distal colon and rectum

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13
Q

Pathophysiology hirschsprung disease regarding bowel motility ? (5)

A

• Aganglionic distal bowel
• interfere with internal bowel mobility
• hypertrophy of extrinsic innervation (sphincter too tight- on Pr flatus and faesces will run out)
• excitatory innervation predominates
• result: increased muscle tone- can’t pass flatus or stools!

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14
Q

Types and incidence hirschprung disease?

A

• Short segment disease (90%):only rectum to sigmoid or less
• long segment disease (10%): rectum, sigmoid and variable length of descending colon

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15
Q

Clinical presentation infant with hirschprung disease? ( 7)

A

• No meconium or flatus passed within 24 hours of birth!
• resonant abdominal distention
. Early Vomiting (low bowel obstruction) green
• poor feeding
• explosive stool and flatus discharge on rectal exam
• enterocolitis in newborns due to stasis in proximal dilated bowel → septicaemia
• perforation rare 3%

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16
Q

Diagnosis hirschsprung disease? (6)

A

• History no meconium/ flatus, constipation
. Clinical signs eg distended abdomen, explosive stools on Pr
• AXR: dilated bowel loops filled with air. No air-fluid levels.
• contrast study (nb to use water soluble contrast, not barium ): narrow distal segment, dilated prox bowel, retention contrast > 24 hours
• definitive diagnosis = full thickness rectal biopsy
-Histology: no ganglion cells visible in Auerbach (myenteric layer), Meissner plexus (submucosal)

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17
Q

Differential diagnosis infant not passing stool? (5)

A

• Small bowel atresia
• hirschsprung disease
• meconium ileus
• anorectal malformations
• malrotation with midgut! Volvulus

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18
Q

Emergency Treatment hirschsprung disease complications of bowel obstruction, enterocolitis, and low bowel obstruction? (8)

A

Bowel obstruction:
• ng tube suction and NPO
• iv fluid 5% dextrose (day 1 neonate)
• refer to paediatric surgeon for colostomy

Enterocolitis
• iv : bonus ringers then maintenanceas above
• ng tube suction and NPO
• saline enemas to empty colon from debris until fluid returns to clear
• antibiotics

Lower bowel obstruction
• diverting colostomy and full thickness rectal biopsy to establish diagnosis

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19
Q

Definitive Treatment hirschsprung disease? (3)

A

• resect diseased bowel and anastomose normal bowel to anus = pull through operation (usually 3 stage, but can be 1 stage in ideal setup)

3 stages = colostomy with biopsy, pull through procedure or resection diseased bowel, anastomosis

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20
Q

Clinical presentation older children with hirschprung disease? (5)

A

• Severe constipation
• Massive distention
• malnutrition
• rectum may be empty
• enterocolitis rare

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21
Q

Define degenerative visceral myopathy ( 4)

A

Progressive impaired intestinal function and motility in absence of mechanical obstruction due to abnormal smooth muscles! in the bowel. Normal at birth , constipation toddler, die as teenager (no cure)
Also affect muscle urinary tract
Clinically similar to hirschsprung in older child
Normal ganglion cells on rectal biopsy

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22
Q

Type of inguinal hernias that occur in children?

A

Always indirect! And always congenital, not acquired like adults

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23
Q

Embryology inguinal hernia child?

A

• Incomplete closure of outpouching of peritoneum, the processus vaginalis (patent), after descent testes in utero
• round ligament in girls
Abdominal contents through deep inguinal ring into inguinal canal and through superficial inguinal ring into groin

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24
Q

Differential diagnosis bowel obstruction after the neonatal period? (5)

A

• Intussusception
• hypertrophic pyloric stenosis
• malrotation and midgut volvulus
• post-op adhesions- look for scars!
• strangulated or incarcerated inguinal and umbilical hernia

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25
Q

Clinical presentation and history paediatric malrotation with midgut volvulus? (7)

A

• Sudden onset Pr blood
• bilous vomiting (high obstruction)
• abdominal distention
• previously healthy baby
• intermittent bowel obstruction (Ladd’s bands keep it in wrong malrotated position )
• volvulus of whole small bowel can occur anytime but most common in neonatal period → bowel gangrene, shock, death
• bloody mucous from rectum = ominous sign for necrotic bowel

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26
Q

Definitive surgical treatment paediatric volvulus?

A

• Derotate
• cut Ladd’s bands
. Broaden mesentary

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27
Q

Most common paediatric cause haematochezia?

A

Intussusception

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28
Q

Peak age intussusception in paeds?

A

3-18 months, peak 5 months

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29
Q

Define intussusception

A

Invagination one part of the bowel into another
Proximal segment = intussuceptum
Distal = intussucipiens

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30
Q

Most common site intussusception?

A

Ileocolic 90%
Can also be ileo-ileal, colo-colonic

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31
Q

Pathophysiology intussusception paeds? (4)

A

Mostly healthy children with recent viral infection - swallow infected phlegm or gastroenteritis

• lead point pulled into lumen by peristalsis
• age 3-9 months: lead point mostly enlarged bowel lymphoid tissue (Peyer’s plaques ) following viral infection
• older > 9 months: lead point might be Meckel’s diverticula, polyps, lymphoma, worms or other foreign bodies

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32
Q

Symptoms and history paediatric intussusception? (4)

A

• Well fed on history, history recent upper respiratory tract infection or gastro-enteritis
• bloody, slimy “red current jelly” stool
• signs bowel obstruction: vomiting, abdominal distention
• intermittent acutely painful colicky abdominal pain that can’t be soothed

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33
Q

Clinical signs paediatric intussusception? (4)

A

• Abdominal distension and tender
• intussusceptum might protrude through anus mimicking rectal prolapse
• sausage shaped abdominal/rectal palpable mass
• dehydration

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34
Q

Special investigations and signs for paediatric intussusception? (3)

A

• Plain AXR: no air in rectum
• Ultrasound: target sign
• electrolytes

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35
Q

Management paediatric intussusception? (5)

A

• Npo and NGT
• iv rehydration and maintenance
•Pneumatic reduction if: child fully resuscitated, no peritonitis, no free air on xr
• laparotomy if pneumatic reduction contraindicated or failed:manual reduction
• if reduction fail, resect intussusception and primary anastomosis (r hemi- colectomy)

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36
Q

Define Meckel’s diverticulum and origin (4)

A

• Remnant of the vitelline duct - connects developing embryo with yolk sac (omphalomesenteric duct)
• antimesenteric border of small bowel
• true intestinal diverticulum (all normal layers intestinal wall )
• heterotopic mucosa may be found: 67% of symptomatic Meckel’s. 86% gastric mucosa, rest pancreatic

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37
Q

Location Meckel’s diverticulum?

A

Terminal ileum, 60-70 cm proximal to ileocolic junction

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38
Q

Complications Meckel’s diverticulum? (4)

A

Ectopic gastric tissue in diverticulum may cause:
•Ulceration
• perforation
• bowel obstruction

• bleeding most common: painless but can be massive, frequently stops spontaneously, transfusion often necessary

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39
Q

Treatment Meckel’s diverticulum?

A

Laparoscopic resection

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40
Q

Diagnosis Meckel’s diverticulum?

A

Technetium 99m scan
• pertechnate ions carry isotope
• stored and secreted into bowel lumen by gastric mucosal cells

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41
Q

Clinical presentation inguinal hernia children? (5)

A

History
• intermittent! Visible swelling -boys inguino-scrotal, girls inguino-labial

Examination
• palpable swelling inguinal canal/scrotum painless
• can be reduced!

• swelling after crying/ straining
• resolve while sleeping

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42
Q

Treatment uncomplicated paeds inguinal hernia? (5)

A

• Not emergency, book for elective surgery
• don’t postpone, high risk complications incarceration or strangulation
• never close spontaneously, always need op

• inguinal incision region of external meatus. Mobilise hernial sac. Cord structures separated from hernial sac, hernial sac clamped transsected and ligated.
• reconstruction inguinal canal done in adults rarely necessary in children because only indirect

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43
Q

Name 3 indications surgery for umbilical hernia in children

A

• Not closed by age 5
• rarely incarceration -
• very large defect >2cm won’t close spontaneously
. Cosmesis

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44
Q

Define omphalocoele (3)

A

• Congenital abdominal wall defect at umbilical ring
• evisceration internal organs in sac covered by 3 layered membrane of peritoneum, Wharton’s jelly, amnion
• sac usually contains small bowel, liver, spleen, colon, occasionally gonads

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45
Q

Embryology ompholocoele?

A

Failure bowel loops to return to abdominal cavity following physiological herniation of umbilical cord between week 6-10. Small bowel usually malrotated but functional

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46
Q

Name 3 associated malformations of omphalocoele

A

• Vacterl association
. Genetic abnormalities
• beckwith wiedemann syndrome: macrosomia, omphalocoele, macroglossia, prone to hypoglycaemia

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47
Q

Treatment omphalocoele with intact membrane? (6)

A

• Drip: maintenance fluids
• ng tube on free drainage
• treat omphalocele sac with antiseptic eg inadine (iodine) preferred, silver containing ointment
• cover with gauze and crepe bandage

• small <5cm: reduction and primary closure at tertiary hospital in theatre otherwise might splint diaphragm
. Giant bigger than baby’s head : conservative. Paint with antiseptics daily until fully epithelialized. Cosmetic repair at 2-3 years.

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48
Q

Name 6 long-term complications or outcomes omphalocoeles

A

Small have none. Large:
• mortality related to associated abnormalities
• GORD
• feeding difficult and failure to thrive

• midgut volvulus in untreated malrotation
• cosmesis
. Beckwith-wiedemann at risk for Wilms or hepatoblastoma

Often underscended testes, undeveloped abdo cavity,

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49
Q

Define gastroschisis

A

Full thickness defect of abdominal wall usually located to right of abdominal wall
10% have small bowel atresia

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50
Q

Treatment gastroschisis and omphalocoele with ruptured membrane? (8)

A

• Drip with 2x normal maintenance! Excessive fluid loss.
• incubator: excessive heat loss
• ngt on free drainage
• examine opening and bowel to look for strangulation
• cover bowel with plastic: bag, glad wrap, empty Iv fluid bag . not gauze!
• position baby on right lateral side

• surgical reduction bowel and primary closure abdominal wall defect. Put in ICU with ventilation to prevent splinting of diaphragm, abdominal compartment syndrome
• If impossible due to oedema and inflammation because exposed to irritating amnion fluid intra-utero:silo bag and delayed closure of defect

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51
Q

Long term outcomes gastroschisis? (2)

A

• 50% mortality due to poor resus and transport from periphery, prolonged ileus, sepsis
• severe bowel loss:short bowel syndrome- extensive bowel necrosis that occurred during gestation or poor reduction technique

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52
Q

Name 7 differences between omphalocoele and gastroschisis - size, location, membrane, abnormalities, contents, age, heat and fluid loss

A

• 4-12 cm defect vs opening <5 cm
• central, epigastric, hypogastric defect with umbilical cord inserted onto sac vs right of umbilical cord with it in normal position
• membrane intact or perforated vs not covered with membrane
• more associated abnormalities 74% vs less 10%
• can contain small bowel, liver, spleen, colon, gonads vs usually only bowel
• full term often big babies vs premature
• less danger heat and fluid loss vs more

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53
Q

Surgical treatment biliary atresia? (2)

A

Extrahepatic bile ducts : Kasai Porto- enterostomy (attach liver to bowel)
• must be done before 3 months age, not high long term success (30-50%)

If fail or intrahepatic bile ducts, liver transplant
(80 % 5 year survival)

Supplement fat soluble vitamins Kade

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54
Q

Second most common cause of surgical neonatal jaundice?

A

Choledochal cyst

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55
Q

Investigation to diagnose choledochal cyst?

A

Sonar

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56
Q

Treatment choledochal cyst paeds? (3)

A

• Cholecystectomy
• cyst excision
•hepatico - jejunostomy

Excellent prognosis

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57
Q

Aetiology obstructive jaundice later inchildhood? ( 5)

A

• Cholelithiasis: haemolytic disease (childhood), cholesterol stones (adolescence)
• Liver abscess
• ascaridiosis
• tumours (compression )
• traumatic: haemobilia

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58
Q

What is vacterl?

A

An association.

Vertebral defects: scoliosis, hemivertebra, spina bifida
Anorectal malformation, atresias
Cardiac anomalies: ASD, vSD, PDA etc
Tracheddesophageal fistula
Esophageal atresia
Renal: aplasia kidney, hydronephrosis etc
Limb defects: polydactyly, absence radius, club feet

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59
Q

Classification anorectal malformations and differences? (6)

A

High malformation
• more common males
• boys 95% recto-urinary fistula: pass meconium through urethral orifice
• on perineal inspection, no fistulas.

Low
. Females more common
• 95% girls recto-vestibular fistula
• fistula to perineum or vestibulum (girls) on perineal inspection.

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60
Q

Clinical signs anorectal malformations? ( 2)

A

Bowel obstruction! In boys. Not girls.
• girls pass enough stools through recto-vestibular fistula so won’t get obstruction
• boys get bowel obstruction after 1-2 days, tiny or absent recto-urinary fistula doesn’t allow sufficient stool to be passed.
Abdominal distension, vomiting late sign.

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61
Q

Treatment anorectal malformation? (3)

A

• Treat as bowel obstruction: hydration, NPO, NGT on free drainage, keep warm, refer

• high lesion: 3 stage procedure
-Colostomy
-Posterior sagittal anorectoplasty (psarp) 4 weeks later
- anal dilatation if needed, colostomy closure.

• low lesions: anoplasty or ano-rectoplasty without colostomy.

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62
Q

Workup if absent anus neonate? (4)

A

• Clinical evaluation and history, classify high vs low
• babygram (xray)
• screening sonar: renal, heart
•Invertogram: confirm high or low lesion (distance bowel to skin )

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63
Q

Name and describe the 2 types of congenital diaphragmatic hernias (3)

A

Bochdalek
• postero-lateral
• pleuroperitoneal membrane fails to close at posterolateral aspect
• worse prognosis, more common, symptomatic

Morgagni
• retrosternal
. Failure sternal and crural portions diaphragm to fuse retrosternal
• better prognosis, rare, often asymp

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64
Q

What problems do bochdalek hernias cause? (5)

A

• Always malrotation
• associated congenital anomalies 23%
• postnatal physiology: lung hypoplasia, pulmonary hypertension, persistent foetal circulation

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65
Q

Clinical features and diagnosis bochdalek hernia? (6)

A

• Decreased chest movement affected side
• shift cardiac impulse
• bowel sounds in chest
• scaphoid abdomen
• cyanosis, respiratory distress
• X-ray findings bowel in chest

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66
Q

Prognosis and complications bochdalek hernia? (7)

A

• 85% in critical condition at delivery, up to 60% won’t survive past neonatal period.
• lung hypoplasia not changed by operation
• 10% mental retardation
• pulmonary function abnormal 50%
• growth failure 30-50%
• GORD > 50%
• intestinal obstruction 20% (malrotation and volvulus)

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67
Q

Treatment bochdalek hernia? (4)

A

• Ng tube free drainage
• oxygen
• drip 5% dextrose iv if day 1
• delayed surgical repair only after pulmonary hypertension corrected

68
Q

Clinical presentation morgagni hernia?

A

Asymptomatic or recurrent respiratory symptoms

69
Q

Definitive Treatment morgagni hernia?

A

Laparotomy repair to prevent colon obstruction and gangrene

70
Q

Name 2 most common types oesophageal atresia

A
  • 85% proximal atresia with distal fistula to the trachea
  • 10% pure oesophageal atresia without fistula. Will see gassless abdomen on X-ray
71
Q

Symptoms oesophageal atresia? (4)

A

After first feed, after swallow, immediate regurgitation of milk through mouth and nose.
Cause 3 Cs: Choking, coughing, cyanosis

72
Q

Investigations for oesophageal atresia?

A

Chest X-ray (no contrast needed): check oesophageal permeability with feeding tube. NGT will curl up

73
Q

Emergency Treatment oesophageal atresia? (5)

A

• Give oxygen
• treat as bowel obstruction, NGT free drainage, iv.
• examine for life-threatening abnormalities. Proper evaluation with clinical, babygram and screening sonar for vacterl with special attention to the heart
• prepare for transport two sides
• suction saliva with 10 ml syringe

74
Q

Definitive Treatment oesophageal atresia? (7 )

A

• Early operation:
- thoracotomy
- division and suture of tracheo-oesophageal fistula
-Oesophageal anastomosis

• if primary anastomosis fails due to long gap, wait for oesophagus to grow
- gastrostomy to feed
-Oesophagostomy if no other option
-Plan replacement procedure after 1 year age (colon or stomach)

75
Q

Name 8 red flags of paediatric constipation

A

• Not pass meconium within 48 hours birth (cystic fibrosis, hirschsprung)
• neurological signs or symptoms especially lower limbs (cerebral palsy, spinal cord lesion)
• vomiting (intestinal obstruction, hirschsprung)
• ribbon stool (anal stenosis)
• abnormal anus (anal stenosis, IBD, sexual abuse)
• abnormal lower back or buttocks (spina bifida, spinal cord lesion, sacral agenesis)
• failure to thrive (coeliac disease, hypothyroid, safeguarding)
• acute severe abdominal pain and bloating (obstruction, intussusception)

76
Q

Differential diagnosis testicular swelling? (4)

A

• Indirect umbilical hernia (children) or direct /indirect adults
• testicular torsion
• hydrocele
• lymphoedema

77
Q

Symptoms of incarcerated inguinal hernia?

A

Painful swelling in inguinal region (uncomplicated not painful)

78
Q

Examination features of paeds incarcerated inguinal hernia? (3)

A

• Tender firm mass in inguinal canal or scrotum
• child may be fussy, unwilling to feed, crying
• overlying skin oedematous, erythematous, discoloured

79
Q

Examination and investigation features of strangulated inguinal hernia? (4)

A

Same as incarcerated hernia plus signs systemic toxicity:
• tachycardia
•Leucocytosis
• tender distended abdomen
• CRP elevated

80
Q

Treatment paediatric incarcerated hernia? (4)

A

• Attempt reduction. If successful, convert emergency into elective. Sedate child and put in trendelenburg position on mother’s lap
• If reduction not successful within 2 hours, treat as bowel obstruction (drip, NGT ) and refer for surgery
• inguinal incision as for elective uncomplicated hernia. Open hernial sac, evaluate bowel
• if necrotic bowel: resection and primary anastomosis

81
Q

Symptoms hydrocele (4)

A

• Painless
. • non-reducible
• constant swelling not intermittent like hernia
• of scrotum or inguinoscrotal region

82
Q

Investigations for hydrocele?

A

• Ultrasound
Transillumination doesn’t help, fluid filled bowel also transilluminates.

83
Q

Hydrocele treatment paeds (2)

A

. Often congenital, can close spontaneously, so expectant treatment.
• if persist beyond age 1 to 2, operative resection indicated

84
Q

Symptoms umbilical hernias?

A

Majority asymptomatic. Incarceration very rare. Does not cause abdominal pain so look for another cause of this.

85
Q

Most common cause chronic abdominal pain in pre-school children?

A

Constipation.

86
Q

Treatment femoral hernia children?

A

High risk incarceration. Must treat aggressively surgery ASAP even if not strangulated

87
Q

Define physiological neonatal jaundice

A

Occurs in the first week of life, very common. Due to:
• increased red cell breakdown (lots of hb in utero to maximise oxygen delivery to fetus,no longer needed)
• immature liver not able to process high bilirubin concentrations.
Start day 2-3, peak days 5, resolved by day 10.

88
Q

Define pathological neonatal jaundice (3)

A

•Present on day 1 of life
• >14 days
• jaundice and pale stools

89
Q

Name 8 differentials for neonatal jaundice that has an indirect unconjugated hyperbilirubinaemia with haemolysis (high reticulocyte count)

A

Aka pre-hepatic jaundice
Common:
• Abo incompatibility
• Rh factor
• infections

Rare haematological:
• g6pd deficiency
• pyruvate kinase abnormality
•Spherocytosis
• sickle cell disease
• thalassemia (less hb )

90
Q

Name 8 differentials for neonatal jaundice that has an indirect unconjugated hyperbilirubinaemia without haemolysis

A

Common:
• physiologic jaundice
• polycythaemia (high concentration rbc)
• mother with diabetes
• breast milk jaundice

Rare
• hypothyroid
• ITP (immune thrombocytopenia) (platelets low )
• crigler - najjar syndrome
• Gilbert syndrome

91
Q

Name 7 differentials for neonatal jaundice that has an direct conjugated hyperbilirubinaemia with haemolysis (high reticulocyte count)

A

Aka hepatic or post-hepatic jaundice. Common.
• torches infections (toxoplasma, other, rubella, CMV, herpes, syphilis)
• septicaemia
• UTI
• hepatitis B, C
• HIV, Tb
• pathologic neonatal hepatitis: idiopathic
• TPN cholestasis

92
Q

Name 8 differentials for neonatal jaundice that has an direct conjugated hyperbilirubinaemia with out haemolysis

A

Aka hepatic or post hepatic jaundice. Rare.
Structural:
• biliary atresia
• choledochal cyst
• intrahepatic bile duct hypoplasia
• hepatic infarction

Metabolic
• galactosaemia (test stool)
• alpha 1 antitrypsin deficiency
• cystic fibrosis

• alagille syndrome (too few bile ducts)

93
Q

Workup in a jaundiced baby >14 days? (With obstructive suspected) (4)

A

• Torches and screen for infective causes
• metabolic screen
• fasting Ultrasound liver and biliary tree. Look for gallbladder- > 80% with biliary atresia don’t have gallbladder (when eaten: false negative - can’t see gallbladder because contracted)
• LFT s: GGT typical in biliary atresia

94
Q

Define biliary atresia

A

Atresia of extra and intrahepatic bile ducts
Most common cause obstructive jaundice neonates

95
Q

Etiology biliary atresia (2)

A

Unknown, but theorised :
• intra-uterine viral infection
• genetic predisposition

96
Q

Classic bloods in biliary atresia?

A

Conjugated hyperbilirubinaemia
GGt markedly raised.

97
Q

Which medication can be prescribed for pruritis due to jaundice?

A

Phenobarbital

98
Q

Presentation choledochal cyst?

A

Abdominal Mass and pain

99
Q

Differential diagnosis for neck mass in children? (8)

A

• Acute lymphadenitis (infection)
• chronic lymphadenitis (infection)
• tumours eg lymphoma most common
Congenital:
• thyroglossal cyst
• epidermoid cyst
• branchial cyst or fistula
• lymphangioma
• haemangioma and Av malformations

100
Q

Causes acute lymphadenitis paeds?

A

Bacterial infection of oropharynx (tonsillitis, pharyngitis), face or scalp (impetigo )
Staph aureus, streps

101
Q

Clinical presentation acute lymphadenitis paeds?

A

• Multiple tender lymph nodes submandibular or anterior cervical region
• smooth, soft, not matted, mobile
• fever, systemic illness

102
Q

Treatment acute lymphadenitis paeds?

A

• Antibiotics (amoxil/ augmentin )
. Sometimes node may enlarge and become fluctuant → abscess formation. Need surgical drainage.

103
Q

Clinical presentation chronic lymphadenitis paeds?

A

Chronically enlarged non-tender lymph nodes

104
Q

Causes chronic lymphadenitis paeds? (3)

A

• Tb
• atypical mycobacterium infection
• malignancy
• HIV with concurrent Tb or lymphoma

105
Q

Investigations for chronic lymphadenitis paeds?

A

Single, dominant node >2 cm presenting longer than 6-8 weeks which hasn’t responded to antibiotics, should be excised, cultured and submitted for histology.

106
Q

Clinical examination lymphoma? (4)

A

•Rubbery
• non-tender
• fixed
• may enlarge quickly. Within days.

107
Q

Embryology of congenital branchial neck masses?

A

• During week 4-8, branchial arches (ridges) and clefts develop in lateral cervicofacial area of embryo.
• embryonic structures have failed to mature or have persisted in an aberrant fashion.

108
Q

Presentation congenital branchial neck masses? (3)

A

• Fistula or cyst anywhere on anterior border sternocleidomastoid
• cyst present with nontender enlarging swelling
• fistula present with drainage of saliva from the ostium

109
Q

Treatment congenital branchial neck masses?

A

Early excision

110
Q

Complication congenital branchial neck masses?

A

Cysts and fistulas can become infected if not resected early in childhood

111
Q

Embryology thyroglossal cyst?

A

• Foramen caecum = site of development of thyroid, at base of prospective tongue.
• as tongue develops, thyroid diverticulum descends in neck, maintaining its connection to foramen caecum
• can be located anywhere along migratory tract if it fails to become obliterated (midline!)

112
Q

Clinical presentation thyroglossal cyst? (4)

A

• In midline neck! At or Just below hyoid bone
• can become infected due to communication with mouth via foramen caecum
• smooth, soft, non-tender
• does not move upwards when tongue protrudes due to attachment to foramen caecum.

113
Q

Treatment thyroglossal cyst?

A

• Early surgical excision to avoid complications of infection
• complete excision of cyst and its tract upward to the base of the tongue

114
Q

Pathophysiology and etiology epidermoid cysts?

A

• Represent ectoderm elements which were trapped under skin
• contain sebaceous material
• can be anywhere

115
Q

Clinical presentation epidermoid cysts?

A

• Can occur anywhere but most commonly at lateral corner eyebrow
• characteristic swelling
• can be midline of neck and be confused with midline thyroglossal duct cysts. Due to entrapment of epithelium of branchial arch origin at the time of embryologic midline fusion

116
Q

Embryology lymphangioma?

A

• Type of av malform
. Failure of lymph spaces to connect to the rest of the lymphatic system

117
Q

Clinical presentation lymphangioma? (4)

A

• soft, smooth, non-tender mass
• compressible!
• can be transilluminated!
• depending on size and location, may be respiratory compromise and difficulty feeding.

118
Q

Treatment lymphangioma? (4)

A

• Goals - improve cosmetic appearance,relieve impaired breathing and eating
. Big lesions causing respiratory difficulty may need urgent intubation at birth
• Surgery difficult due to infiltrative nature of these lesions
• preferred treatment = us guided infiltration with bleomycin, alcohol or other sclerosing agents.

119
Q

Define haemangioma

A

Benign tumours of the capillary vessels of the skin

120
Q

Clinical presentation haemangioma? (4)

A

• Can occur anywhere but common on face and neck
• typical growth:
- enlarge in first 2 years life
- Stationary phase
-Involution- outgrow blood supply.

121
Q

Treatment options haemangioma? (3)

A

• Conservative watchful waiting!
Regular follow up 3 monthly, measure and photo
• excision or sclerosation (bleomycin) , propanol if complicated eg ulcerated, or impairing function eg around eye or nose
• for ulceration: antiseptic cream and propanol.

122
Q

Define hypertrophic pyloric stenosis

A

Fibromuscular thickening of the pyloric muscle with unknown etiology. Usually present between 3-6 weeks of age.
More common in boys

123
Q

Symptoms hypertrophic pyloric stenosis (5)

A

• Acute or gradual onset projectile vomiting
• non bilious vomit of milk
• shortly or right after feeding
• child losing weight
• still hungry after vomiting

124
Q

Examination findings hypertrophic pyloric stenosis (4)

A

• Signs malnutrition
• otherwise healthy; no signs sepsis, raised ICP or other reason vomiting
• peristaltic waves may be visible in upper abdomen
• Olive - shaped tumour palpable in right hypochondrium
More common in boys

125
Q

Special investigations for hypertrophic pyloric stenosis? (3)

A

• Sonar: thickened pyloric muscle visible with long channel
• plain axr: big dilated stomach. Barium contrast elongated and narrowed pyloric channel, beak and string signs, Chinese umbrella
• bloods: hypokalemic, hyponatremic metabolic alkalosis

126
Q

Treatment hypertrophic pyloric stenosis? (3)

A

• Rehydrate! Correct low k, na, cI with 0.45% Nacl with dextrose and 20-30 mmol/l of Kcl, 6-10 ml/kg/hour for 24-48 hours ( chronic dehydration)
• ramstedt pyloromyotomy
• feeding can be restarted 3-6 hours after operation
Excellent prognosis

127
Q

Define and describe pathophysiology gastro oesophageal reflux in paeds (4)

A

• Common, self limiting, usually resolve by 6-12 months
• functional or physiological process in healthy infant
• involves regurgitation, which is passive return of gastric contents retrograde into oesophagus
• “happy spitter”

128
Q

Define and describe pathophysiology gastro oesophageal reflux disease in paeds

A

• Pathologic process
• scrawny screamer
• sick child: poor weight gain, signs esophagitis, persistent respiratory symptoms, changes in neurobehaviour

129
Q

Name 5 clinical features and symptoms gastro-oesophageal reflux in paeds

A

• regurgitation with normal weight gain , non projectile effortless vomiting at any time
• vomit partially digested milk (sour smell)
• no signs or symptoms œsophagitis, respiratory or neurobehavioural
• uncomfortable baby due to heartburn
• sometimes will feed again after vomiting, sometimes not due to heartburn pain

130
Q

Name 4 clinical features and symptoms gastro-oesophageal reflux disease in paeds

A

• Regurgitation with poor weight gain
• persistent irritability in infants. Pain in children
• apnea and cyanosis, wheezing, aspiration, recurrent pneumonia, chronic cough, stridor
• neurobehaviour: neck tilting in infants (Sandifer’s syndrome)

131
Q

Investigations options for gastro-oesophageal reflux disease paeds? (3)

A

• Barium swallow to evaluate upper anatomical gi structure, but inadequate for GERD
• 24 hour ph probe: gold standard but expensive
• endoscopy with biopsy: evaluate persistent GERD, pud, H pylori infection, allergic enteropathy, Barrett’s esophagus

132
Q

Management gastro-oesophageal reflux paeds? ( 4)

A

• Reassure parents
• thicken feeds (maizena in milk)
• frequent small feeds
• upright position after feeds, tiger on a tree hold

133
Q

Management gastro-oesophageal reflux disease paeds? (4)

A

• Rule out other diseases
• same advice as to reflux babies
• gaviscon, pPI
• surgery only in some cases: Nissen fundoplication

134
Q

Differential diagnosis gastro-oesophageal reflux disease like symptoms or vomiting paeds? (10)

A

Git
• pyloric stenosis
• malrotation
• cow’s milk allergy
• peptic ulcer disease
• hepatitis
• eosinophilic esophagitis

• urinary tract: infection, obstruction

• CNS: hydrocephalus, meningitis

• metabolic disorders: renal tubular acidosis, hypocalcaemia, hypothyroid

• functional: rumination, anorexia

135
Q

Name 5 indications for surgery in gastro-oesophageal reflux disease paeds

A

• Atypical especially respiratory symptoms
• complications: aspiration, stricture, Barrett’s
• apparent life threatening episode (alte) associated with GORD
• patients with neurological impairment and reflux requiring feeding gastrostomy
• symptoms persist in spite of lifestyle changes and medication

136
Q

Identify pathology picture 45

A

Red current jelly stool: intussusception

137
Q

Identify pathology picture 48

A

Indirect inguinal hernia (paeds always indirect)

138
Q

Identify pathology picture 49 - breast ultrasound

A

No pathology. Normal
• upper grey layer = skin
• mixture of fat (hypoechoic dark) and glandular tissue (hyperechoic grey)
• striped layer posterior to breast = pectoral muscle
• post or deep to ribs = posterior shadowing dark area
• deepest layer = lungs - air reflect most of the sound waves.

139
Q

Describe Fibroadenoma appearance on ultrasound (6)

A

• Hypo echoic
• oval or round shape
• circumscribed margin
• horizontal orientation/wider than tall
• sometimes minimal posterior enhancements
• may have gross calcifications

140
Q

Approach to intestinal bleeding in children? (3)

A

• Begin resuscitation early
-Tachycardia according to age most sensitive indicator
-Ng tube
- iv 20ml/kg ringers
- blood products if non-responder, ongoing bleed (also give somatostatin analogue), pre existing heart or lung condition
•Urgent referral for therapeutic gastroscope or colonoscopy
• stabilize for transport

141
Q

Define rectorrhagia

A

Passage of red blood without stool from rectum or anus

142
Q

Name 4 things that can imitate bloody stools

A

• Some antibiotics
• iron supplements
• bismuth
• beetroot

143
Q

Differential diagnosis upper gastrointestinal bleeding neonates?

A

• haemorrhagic disease of the newborn (low vit k)
• swallowed maternal blood
• stress gastritis (ICU)

144
Q

Differential diagnosis lower gastrointestinal bleeding neonate? (3)

A

• necrotising enterocolitis (immature)
• anal fissure
• malrotation with volvulus

145
Q

Differential diagnosis gastrointestinal bleeding infants 1 month to 1 year? (5)

A

Upper
•esophagitis (reflux)
• stress gastritis

Lower
• anal fissure
• intussusception
• milk protein allergy

146
Q

Differential diagnosis gastrointestinal bleeding infants 1-2 year? (4)

A

Upper
• Peptic ulcer disease (possible H pylori)
• gastritis

Lower
• polyps
• Meckel diverticulum

147
Q

Differential diagnosis gastrointestinal bleeding children older than 2 year? (5)

A

Upper
• esophageal varices
• peptic ulcer disease

Lower
• polyps
• IBD
• infectious diarrhoea

148
Q

Define haemorrhagic disease of the newborn

A

•Vitamin K deficiency
• vitamin k dependent clotting factors decline rapidly, lowest point 48-72 hours postnatal

149
Q

Symptoms haemorrhagic disease of the newborn?

A

Upper gi bleed
Coffee ground gastric aspirate or melena

150
Q

What is done to prevent haemorrhagic disease of the newborn?

A

Routine vitamin k administration at birth

151
Q

Diagnosis haemorrhagic disease of the newborn?

A

Coagulation studies

152
Q

Diagnosis upper gi bleeding due to swallowed maternal blood?

A

Apt test ( alkali denaturation test)
• Blood on filter paper with 1% sodium hydroxide
• maternal blood: denatured, appear rusty brown
• fetal blood: no reaction, remain pink/red

153
Q

Symptoms and presentation stress gastritis? (3)

A

• Coffee ground (slow bleed) vomitus
• stressful delivery, needed resus, premature, mechanical ventilation
• negative apt and normal coagulation studies

154
Q

Treatment stress gastritis neonates? (4)

A

• Resuscitate, ventilate
• ng tube
• gastric irrigation
• iv h2 blockers

155
Q

Define necrotising enterocolitis of neonates

A

• Premature newborn
• bowel wall bacterial infection due to immature mucosal barrier

156
Q

Name 2 risk factors for necrotising enterocolitis of neonates

A

• Premature
. Formula fed infants: breast milk immunoglobulins

157
Q

Presentation necrotising enterocolitis of neonates? (6)

A

• Sudden feeding intolerance
• abdominal distention
. Billious vomiting
• abdominal wall erythema or mass
• sepsis/ acidosis/shock
• blood per rectum
• premature

158
Q

Diagnosis and findings necrotising enterocolitis of neonates?

A

AXR:
• pneumatosis intestinalis
• bowel wall thickening
• portal venous gas
• pneumo peritoneum

159
Q

Treatment necrotising enterocolitis of neonates? (3)

A

• npo, NGT, total parental feeds
• antibiotics
• surgery if necrotic or perforation

160
Q

Name 2 causes esophagitis in infants

A

• Peptic esophagitis: GER
• viral esophagitis

161
Q

Name 4 causes gastritis in infants

A

• H pylori infection
• secondary to severe systemic illness
- burns
- head trauma
- NSAIDs

162
Q

Symptoms Anal fissure in infants?

A

• Bright red blood per rectum outside of stool or small drops in diaper (rectorrhagia)
• otherwise healthy

163
Q

Examination features Anal fissure in infants? (3)

A

• Fissure posterior midline of canal
• rectal exam very painful
• may have hard stool

164
Q

Treatment Anal fissure in infants?

A

Stool softeners, wait and see

165
Q

Name 2 types polyps found in children

A

• Hamartoma ( common)
• adenoma (rare)

166
Q

Clinical presentation polyps found in children? (3)

A

• Painless red rectal bleeding
• can prolapse through anus
• usually distal colon

167
Q

Name 4 differences between hamartoma and adenoma polyps found in children

A

• Single sporadic vs multiple associated with FPC familial polyposis coli
• no malignant potential, some hyperplastic vs high cancer risk
• sigmoidoscopy to confirm vs colonoscopy
• no treatment necessary, follow up, vs colectomy in Early adolescence required