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)
Treatment bochdalek hernia? (4)
• Ng tube free drainage
• oxygen
• drip 5% dextrose iv if day 1
• delayed surgical repair only after pulmonary hypertension corrected
Clinical presentation morgagni hernia?
Asymptomatic or recurrent respiratory symptoms
Definitive Treatment morgagni hernia?
Laparotomy repair to prevent colon obstruction and gangrene
Name 2 most common types oesophageal atresia
- 85% proximal atresia with distal fistula to the trachea
- 10% pure oesophageal atresia without fistula. Will see gassless abdomen on X-ray
Symptoms oesophageal atresia? (4)
After first feed, after swallow, immediate regurgitation of milk through mouth and nose.
Cause 3 Cs: Choking, coughing, cyanosis
Investigations for oesophageal atresia?
Chest X-ray (no contrast needed): check oesophageal permeability with feeding tube. NGT will curl up
Emergency Treatment oesophageal atresia? (5)
• 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
Definitive Treatment oesophageal atresia? (7 )
• 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)
Name 8 red flags of paediatric constipation
• 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)
Differential diagnosis testicular swelling? (4)
• Indirect umbilical hernia (children) or direct /indirect adults
• testicular torsion
• hydrocele
• lymphoedema
Symptoms of incarcerated inguinal hernia?
Painful swelling in inguinal region (uncomplicated not painful)
Examination features of paeds incarcerated inguinal hernia? (3)
• Tender firm mass in inguinal canal or scrotum
• child may be fussy, unwilling to feed, crying
• overlying skin oedematous, erythematous, discoloured
Examination and investigation features of strangulated inguinal hernia? (4)
Same as incarcerated hernia plus signs systemic toxicity:
• tachycardia
•Leucocytosis
• tender distended abdomen
• CRP elevated
Treatment paediatric incarcerated hernia? (4)
• 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
Symptoms hydrocele (4)
• Painless
. • non-reducible
• constant swelling not intermittent like hernia
• of scrotum or inguinoscrotal region
Investigations for hydrocele?
• Ultrasound
Transillumination doesn’t help, fluid filled bowel also transilluminates.
Hydrocele treatment paeds (2)
. Often congenital, can close spontaneously, so expectant treatment.
• if persist beyond age 1 to 2, operative resection indicated
Symptoms umbilical hernias?
Majority asymptomatic. Incarceration very rare. Does not cause abdominal pain so look for another cause of this.
Most common cause chronic abdominal pain in pre-school children?
Constipation.
Treatment femoral hernia children?
High risk incarceration. Must treat aggressively surgery ASAP even if not strangulated
Define physiological neonatal jaundice
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.
Define pathological neonatal jaundice (3)
•Present on day 1 of life
• >14 days
• jaundice and pale stools
Name 8 differentials for neonatal jaundice that has an indirect unconjugated hyperbilirubinaemia with haemolysis (high reticulocyte count)
Aka pre-hepatic jaundice
Common:
• Abo incompatibility
• Rh factor
• infections
Rare haematological:
• g6pd deficiency
• pyruvate kinase abnormality
•Spherocytosis
• sickle cell disease
• thalassemia (less hb )
Name 8 differentials for neonatal jaundice that has an indirect unconjugated hyperbilirubinaemia without haemolysis
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
Name 7 differentials for neonatal jaundice that has an direct conjugated hyperbilirubinaemia with haemolysis (high reticulocyte count)
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
Name 8 differentials for neonatal jaundice that has an direct conjugated hyperbilirubinaemia with out haemolysis
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)
Workup in a jaundiced baby >14 days? (With obstructive suspected) (4)
• 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
Define biliary atresia
Atresia of extra and intrahepatic bile ducts
Most common cause obstructive jaundice neonates
Etiology biliary atresia (2)
Unknown, but theorised :
• intra-uterine viral infection
• genetic predisposition
Classic bloods in biliary atresia?
Conjugated hyperbilirubinaemia
GGt markedly raised.
Which medication can be prescribed for pruritis due to jaundice?
Phenobarbital
Presentation choledochal cyst?
Abdominal Mass and pain
Differential diagnosis for neck mass in children? (8)
• 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
Causes acute lymphadenitis paeds?
Bacterial infection of oropharynx (tonsillitis, pharyngitis), face or scalp (impetigo )
Staph aureus, streps
Clinical presentation acute lymphadenitis paeds?
• Multiple tender lymph nodes submandibular or anterior cervical region
• smooth, soft, not matted, mobile
• fever, systemic illness
Treatment acute lymphadenitis paeds?
• Antibiotics (amoxil/ augmentin )
. Sometimes node may enlarge and become fluctuant → abscess formation. Need surgical drainage.
Clinical presentation chronic lymphadenitis paeds?
Chronically enlarged non-tender lymph nodes
Causes chronic lymphadenitis paeds? (3)
• Tb
• atypical mycobacterium infection
• malignancy
• HIV with concurrent Tb or lymphoma
Investigations for chronic lymphadenitis paeds?
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.
Clinical examination lymphoma? (4)
•Rubbery
• non-tender
• fixed
• may enlarge quickly. Within days.
Embryology of congenital branchial neck masses?
• 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.
Presentation congenital branchial neck masses? (3)
• Fistula or cyst anywhere on anterior border sternocleidomastoid
• cyst present with nontender enlarging swelling
• fistula present with drainage of saliva from the ostium
Treatment congenital branchial neck masses?
Early excision
Complication congenital branchial neck masses?
Cysts and fistulas can become infected if not resected early in childhood
Embryology thyroglossal cyst?
• 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!)
Clinical presentation thyroglossal cyst? (4)
• 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.
Treatment thyroglossal cyst?
• Early surgical excision to avoid complications of infection
• complete excision of cyst and its tract upward to the base of the tongue
Pathophysiology and etiology epidermoid cysts?
• Represent ectoderm elements which were trapped under skin
• contain sebaceous material
• can be anywhere
Clinical presentation epidermoid cysts?
• 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
Embryology lymphangioma?
• Type of av malform
. Failure of lymph spaces to connect to the rest of the lymphatic system
Clinical presentation lymphangioma? (4)
• soft, smooth, non-tender mass
• compressible!
• can be transilluminated!
• depending on size and location, may be respiratory compromise and difficulty feeding.
Treatment lymphangioma? (4)
• 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.
Define haemangioma
Benign tumours of the capillary vessels of the skin
Clinical presentation haemangioma? (4)
• Can occur anywhere but common on face and neck
• typical growth:
- enlarge in first 2 years life
- Stationary phase
-Involution- outgrow blood supply.
Treatment options haemangioma? (3)
• 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.
Define hypertrophic pyloric stenosis
Fibromuscular thickening of the pyloric muscle with unknown etiology. Usually present between 3-6 weeks of age.
More common in boys
Symptoms hypertrophic pyloric stenosis (5)
• Acute or gradual onset projectile vomiting
• non bilious vomit of milk
• shortly or right after feeding
• child losing weight
• still hungry after vomiting
Examination findings hypertrophic pyloric stenosis (4)
• 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
Special investigations for hypertrophic pyloric stenosis? (3)
• 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
Treatment hypertrophic pyloric stenosis? (3)
• 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
Define and describe pathophysiology gastro oesophageal reflux in paeds (4)
• 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”
Define and describe pathophysiology gastro oesophageal reflux disease in paeds
• Pathologic process
• scrawny screamer
• sick child: poor weight gain, signs esophagitis, persistent respiratory symptoms, changes in neurobehaviour
Name 5 clinical features and symptoms gastro-oesophageal reflux in paeds
• 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
Name 4 clinical features and symptoms gastro-oesophageal reflux disease in paeds
• 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)
Investigations options for gastro-oesophageal reflux disease paeds? (3)
• 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
Management gastro-oesophageal reflux paeds? ( 4)
• Reassure parents
• thicken feeds (maizena in milk)
• frequent small feeds
• upright position after feeds, tiger on a tree hold
Management gastro-oesophageal reflux disease paeds? (4)
• Rule out other diseases
• same advice as to reflux babies
• gaviscon, pPI
• surgery only in some cases: Nissen fundoplication
Differential diagnosis gastro-oesophageal reflux disease like symptoms or vomiting paeds? (10)
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
Name 5 indications for surgery in gastro-oesophageal reflux disease paeds
• 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
Identify pathology picture 45
Red current jelly stool: intussusception
Identify pathology picture 48
Indirect inguinal hernia (paeds always indirect)
Identify pathology picture 49 - breast ultrasound
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.
Describe Fibroadenoma appearance on ultrasound (6)
• Hypo echoic
• oval or round shape
• circumscribed margin
• horizontal orientation/wider than tall
• sometimes minimal posterior enhancements
• may have gross calcifications
Approach to intestinal bleeding in children? (3)
• 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
Define rectorrhagia
Passage of red blood without stool from rectum or anus
Name 4 things that can imitate bloody stools
• Some antibiotics
• iron supplements
• bismuth
• beetroot
Differential diagnosis upper gastrointestinal bleeding neonates?
• haemorrhagic disease of the newborn (low vit k)
• swallowed maternal blood
• stress gastritis (ICU)
Differential diagnosis lower gastrointestinal bleeding neonate? (3)
• necrotising enterocolitis (immature)
• anal fissure
• malrotation with volvulus
Differential diagnosis gastrointestinal bleeding infants 1 month to 1 year? (5)
Upper
•esophagitis (reflux)
• stress gastritis
Lower
• anal fissure
• intussusception
• milk protein allergy
Differential diagnosis gastrointestinal bleeding infants 1-2 year? (4)
Upper
• Peptic ulcer disease (possible H pylori)
• gastritis
Lower
• polyps
• Meckel diverticulum
Differential diagnosis gastrointestinal bleeding children older than 2 year? (5)
Upper
• esophageal varices
• peptic ulcer disease
Lower
• polyps
• IBD
• infectious diarrhoea
Define haemorrhagic disease of the newborn
•Vitamin K deficiency
• vitamin k dependent clotting factors decline rapidly, lowest point 48-72 hours postnatal
Symptoms haemorrhagic disease of the newborn?
Upper gi bleed
Coffee ground gastric aspirate or melena
What is done to prevent haemorrhagic disease of the newborn?
Routine vitamin k administration at birth
Diagnosis haemorrhagic disease of the newborn?
Coagulation studies
Diagnosis upper gi bleeding due to swallowed maternal blood?
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
Symptoms and presentation stress gastritis? (3)
• Coffee ground (slow bleed) vomitus
• stressful delivery, needed resus, premature, mechanical ventilation
• negative apt and normal coagulation studies
Treatment stress gastritis neonates? (4)
• Resuscitate, ventilate
• ng tube
• gastric irrigation
• iv h2 blockers
Define necrotising enterocolitis of neonates
• Premature newborn
• bowel wall bacterial infection due to immature mucosal barrier
Name 2 risk factors for necrotising enterocolitis of neonates
• Premature
. Formula fed infants: breast milk immunoglobulins
Presentation necrotising enterocolitis of neonates? (6)
• Sudden feeding intolerance
• abdominal distention
. Billious vomiting
• abdominal wall erythema or mass
• sepsis/ acidosis/shock
• blood per rectum
• premature
Diagnosis and findings necrotising enterocolitis of neonates?
AXR:
• pneumatosis intestinalis
• bowel wall thickening
• portal venous gas
• pneumo peritoneum
Treatment necrotising enterocolitis of neonates? (3)
• npo, NGT, total parental feeds
• antibiotics
• surgery if necrotic or perforation
Name 2 causes esophagitis in infants
• Peptic esophagitis: GER
• viral esophagitis
Name 4 causes gastritis in infants
• H pylori infection
• secondary to severe systemic illness
- burns
- head trauma
- NSAIDs
Symptoms Anal fissure in infants?
• Bright red blood per rectum outside of stool or small drops in diaper (rectorrhagia)
• otherwise healthy
Examination features Anal fissure in infants? (3)
• Fissure posterior midline of canal
• rectal exam very painful
• may have hard stool
Treatment Anal fissure in infants?
Stool softeners, wait and see
Name 2 types polyps found in children
• Hamartoma ( common)
• adenoma (rare)
Clinical presentation polyps found in children? (3)
• Painless red rectal bleeding
• can prolapse through anus
• usually distal colon
Name 4 differences between hamartoma and adenoma polyps found in children
• 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
