Paediatrics Flashcards
How should a neonate with bowel obstruction be transported? (8)
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
Where is level of obstruction If NGT is draining saliva in neonate?
Oesophageal atresia
Where is level of obstruction If NGT is draining milk in neonate?
Stomach outlet obstruction
Where is level of obstruction If NGT is draining bilious green fluid in neonate?
Post ampula of vater
Surgical emergency until proven otherwise!
How and which fluids are given to neonates as a general rule? (5)
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
! Differential diagnosis neonatal (congenital) bowel obstruction (6)
• Atresias 80%: small bowel atresia, anorectal malformation, oesophageal atresia
• Hirschprung’s disease 10%
• malrotation with (midgut) volvulus (not sigmoid in children!)
• meconium ileus
Prenatal diagnosis of neonatal bowel obstruction? (5)
• 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
Duodenal atresia symptoms and associated malformations? (4)
• Early bile stained vomiting hours after birth, can be delayed if incomplete obstruction
• Associated malformations: heart defects 20%, down syndrome 30%, Vacterl association
Definitive Treatment duodenal atresia?
Duodenoduodenostomy
Treatment meconium ileus?
Wash out with saline to loosen stool. If that did not work, refer- probably Hirschprung’s (present similarly)
Define hirschprung disease
Congenital Aganglionosis of distal colon and rectum (can’t develop, present at birth)
Of myenteric layer (Auerbach plexus) and submucosal (meissner)
Embryology of hirchsprung disease? (5)
• 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
Pathophysiology hirschsprung disease regarding bowel motility ? (5)
• 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!
Types and incidence hirschprung disease?
• Short segment disease (90%):only rectum to sigmoid or less
• long segment disease (10%): rectum, sigmoid and variable length of descending colon
Clinical presentation infant with hirschprung disease? ( 7)
• 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%
Diagnosis hirschsprung disease? (6)
• 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)
Differential diagnosis infant not passing stool? (5)
• Small bowel atresia
• hirschsprung disease
• meconium ileus
• anorectal malformations
• malrotation with midgut! Volvulus
Emergency Treatment hirschsprung disease complications of bowel obstruction, enterocolitis, and low bowel obstruction? (8)
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
Definitive Treatment hirschsprung disease? (3)
• 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
Clinical presentation older children with hirschprung disease? (5)
• Severe constipation
• Massive distention
• malnutrition
• rectum may be empty
• enterocolitis rare
Define degenerative visceral myopathy ( 4)
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
Type of inguinal hernias that occur in children?
Always indirect! And always congenital, not acquired like adults
Embryology inguinal hernia child?
• 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
Differential diagnosis bowel obstruction after the neonatal period? (5)
• Intussusception
• hypertrophic pyloric stenosis
• malrotation and midgut volvulus
• post-op adhesions- look for scars!
• strangulated or incarcerated inguinal and umbilical hernia
Clinical presentation and history paediatric malrotation with midgut volvulus? (7)
• 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
Definitive surgical treatment paediatric volvulus?
• Derotate
• cut Ladd’s bands
. Broaden mesentary
Most common paediatric cause haematochezia?
Intussusception
Peak age intussusception in paeds?
3-18 months, peak 5 months
Define intussusception
Invagination one part of the bowel into another
Proximal segment = intussuceptum
Distal = intussucipiens
Most common site intussusception?
Ileocolic 90%
Can also be ileo-ileal, colo-colonic
Pathophysiology intussusception paeds? (4)
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
Symptoms and history paediatric intussusception? (4)
• 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
Clinical signs paediatric intussusception? (4)
• Abdominal distension and tender
• intussusceptum might protrude through anus mimicking rectal prolapse
• sausage shaped abdominal/rectal palpable mass
• dehydration
Special investigations and signs for paediatric intussusception? (3)
• Plain AXR: no air in rectum
• Ultrasound: target sign
• electrolytes
Management paediatric intussusception? (5)
• 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)
Define Meckel’s diverticulum and origin (4)
• 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
Location Meckel’s diverticulum?
Terminal ileum, 60-70 cm proximal to ileocolic junction
Complications Meckel’s diverticulum? (4)
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
Treatment Meckel’s diverticulum?
Laparoscopic resection
Diagnosis Meckel’s diverticulum?
Technetium 99m scan
• pertechnate ions carry isotope
• stored and secreted into bowel lumen by gastric mucosal cells
Clinical presentation inguinal hernia children? (5)
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
Treatment uncomplicated paeds inguinal hernia? (5)
• 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
Name 3 indications surgery for umbilical hernia in children
• Not closed by age 5
• rarely incarceration -
• very large defect >2cm won’t close spontaneously
. Cosmesis
Define omphalocoele (3)
• 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
Embryology ompholocoele?
Failure bowel loops to return to abdominal cavity following physiological herniation of umbilical cord between week 6-10. Small bowel usually malrotated but functional
Name 3 associated malformations of omphalocoele
• Vacterl association
. Genetic abnormalities
• beckwith wiedemann syndrome: macrosomia, omphalocoele, macroglossia, prone to hypoglycaemia
Treatment omphalocoele with intact membrane? (6)
• 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.
Name 6 long-term complications or outcomes omphalocoeles
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,
Define gastroschisis
Full thickness defect of abdominal wall usually located to right of abdominal wall
10% have small bowel atresia
Treatment gastroschisis and omphalocoele with ruptured membrane? (8)
• 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
Long term outcomes gastroschisis? (2)
• 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
Name 7 differences between omphalocoele and gastroschisis - size, location, membrane, abnormalities, contents, age, heat and fluid loss
• 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
Surgical treatment biliary atresia? (2)
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
Second most common cause of surgical neonatal jaundice?
Choledochal cyst
Investigation to diagnose choledochal cyst?
Sonar
Treatment choledochal cyst paeds? (3)
• Cholecystectomy
• cyst excision
•hepatico - jejunostomy
Excellent prognosis
Aetiology obstructive jaundice later inchildhood? ( 5)
• Cholelithiasis: haemolytic disease (childhood), cholesterol stones (adolescence)
• Liver abscess
• ascaridiosis
• tumours (compression )
• traumatic: haemobilia
What is vacterl?
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
Classification anorectal malformations and differences? (6)
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.
Clinical signs anorectal malformations? ( 2)
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.
Treatment anorectal malformation? (3)
• 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.
Workup if absent anus neonate? (4)
• Clinical evaluation and history, classify high vs low
• babygram (xray)
• screening sonar: renal, heart
•Invertogram: confirm high or low lesion (distance bowel to skin )
Name and describe the 2 types of congenital diaphragmatic hernias (3)
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
What problems do bochdalek hernias cause? (5)
• Always malrotation
• associated congenital anomalies 23%
• postnatal physiology: lung hypoplasia, pulmonary hypertension, persistent foetal circulation
Clinical features and diagnosis bochdalek hernia? (6)
• Decreased chest movement affected side
• shift cardiac impulse
• bowel sounds in chest
• scaphoid abdomen
• cyanosis, respiratory distress
• X-ray findings bowel in chest
Prognosis and complications bochdalek hernia? (7)
• 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)