15. CLEFT LIP
• Hypoplasia of mesenchyme -> failure of fusion of
medial nasal and maxillary processes
• Incidence - 1 in 750
• M>F
• Sporadic/genetic
• Maternal
smoking/alcohol/anticonvulsants/antihypertensives
• Small notch in the vermilion border to complete
separation involving skin,muscle,mucosa.
• Unilateral/bilateral
16.
17. CLEFT PALATE
• Failure of palatal shelves to fuse
• Incidence – 1 in 2500
• Isolated or in addition to cleft lip
• Involving only uvula/soft and hard palates
• Unilateral / bilateral
• Recurrent otitis media,subsequent hearing
loss,misarticulated speech
18.
19.
20.
21. Cleft lip Mx :
• Problem with feeding
• Treatment – by team approach
• Surgical closure by 3mths of age,when infant has
achieved sufficient weight gain,and is free of
oral/respiratory/systemic infection
• Modified Millard rotation advancement
technique
• Revised repair may be required at 4-5yrs of age
22. Cleft palate Mx :
• Correction depends on degree of
deformity,adequacy of existing palatal segments
• Surgery - with a goal of uniting cleft
segments,comprehensible speech,reduction of
nasal regurgitation
• Usually performed before 1 yr of age
• Associated missing/malformed teeth replaced by
prostheses
24. ESOPHAGEAL ATRESIA and TEF
• Esophageal atresia is the M/C congenital
anomaly of esophagus
• >90 % have associated TEF
• Most common type – upper esophagus ending
in a blind pouch and TEF connected to distal
esophagus
26. • Advanced maternal age,smoking,low
socioeconomic status
• Sporadic/genetic
• 50 % are a part of syndromes
( VACTERL/CHARGE/VEINGOLD)
• Present with frothing and bubbling at mouth and
nose
• Recurrent pneumonias due to regurgitation and
aspiration
27. • Dx by inability to pass an NG tube (coiled tube on
XRAY)
• Plain Xray showing airless scaphoid abdomen if no
TEF / air distended stomach if TEF +
• Orifice seen on bronchoscopy / endoscopy
28. • Mx : prone positioning, esophageal suctioning
to prevent aspiration of secretions
• Definitive Rx by surgical ligation of TEF and end to
end anastomosis of esophagus
• If gap between ends
>3-4cms,gastric/jejunal/colonic segment as
neoesophagus
• If LBW, gastrostomy tube placement
29. HYPERTROPHIC PYLORIC STENOSIS
• Incidence of 1-3/1000
• First born males ++
• Blood groups O and B
• >> risk if maternal h/o pyloric stenosis and
maternal intake of macrolides
• Asso with apert / zellweger syndromes
30.
31. • At around 3 weeks of age,present with non
bilious vomiting following feeding
• Loss of fluid,H+ ions and Cl- ions
Hypochloremic metabolic alkalosis
Severe dehydration,chronic malnutrition
• Ictero-pyloric syndrome -> associated with
unconjugated hyperbilirubinemia
32. • Dx : palpable olive shaped,firm,movable
mass,located above and to right of umbilicus(mid
epigastrium),after vomiting
• Visible gastric peristaltic wave across abdomen
• Confirmed by USG (>> length,>>thickness of wall,<<
luminal diameter)
35. • C/F : early satiety,upper abdominal pain
• Dx : by contrast studies,endoscopy
• Mx : Medical treatment of gastro esophageal
reflux
Nissen’s fundoplication
36. DUODENAL ATRESIA
• Most common cause of congenital duodenal
obstruction
• Most common among all intestinal atresias
• Incidence of 1 in 10000 live births
• Failure of recanalization of intestinal lumen
• Asso with preterm delivery and
polyhydramnios
• Trisomy 21 in 1/3rd of patients
38. • 30 % have concomitant anomalies like
CHD,malrotation,annular pancreas etc
• Present on 1st day of life with bilious vomiting
• Peristaltic wave may be seen
• Jaundice in 1/3rd
• Plain X Ray abdomen shows ‘double bubble sign’
• Prenatal diagnosis by fetal sonographic double
bubble
39. • Mx : Nasogastric decompression
Correct fluid and electrolyte imbalance
Definitive Rx by duodenoduodenostomy
40. MECKEL DIVERTICULUM
• Most common congenital GI anomaly
• Remnant of omphalomesenteric duct
• Initially connects the yolksac with the intestine
• Attenuates and separates by 7th week POG
41. • Rule of 2 –
2% of all infants
2nd year of life presentation
2% of those are symptomatic
2 inches long
2 feet proximal to ileocaecal valve (along the
antimesenteric border)
43. HIRSCHSPRUNG DISEASE
• Congenital aganglionic megacolon
• Most common cause of lower GI obstruction in
neonates (1 in 5000)
• Developmental disorder of enteric nervous
system
• Absence of ganglion cells in the submucosal and
myenteric plexus
• Sporadic/genetic,associated with Down etc
• Not seen in preterm infants
44.
45. • Short segment (80%) or long segment
• Present with failure to pass
meconium,distended abdomen,sometimes
bilious vomiting
• Older children present with chronic
constipation since infancy,not responding to
medical Rx
• Cannot propel faeces out -> proximal segment
dilated -> stasis -> enterocolitis,sepsis
• Compression leading to urinary retention and
hydronephrosis
46. • Faecal mass palpable in left lower abdomen
• Empty rectum on PR
• Dx by rectal biopsy (gold standard)
• >> acetylcholinesterase on staining
• Anorectal manometry,Contrast studies
47. • Rx – surgical
1. SWENSON – excise and anastomose
2. DUHAMEL – bringing down normal segment
behind aganglionic segment (neorectum)
3. SOAVE – stripping the mucosa of abnormal
segment and bypassing the abnormal bowel
from within
48. NONROTATION/MALROTATION
• Failure of bowel to rotate after it returns to the
abdominal cavity
• Asso with diaphragmatic
hernia,omphalocoele,gastroschisis
• Small intestine (except 1st and 2nd parts of duo)
occupies the right side of abdomen,colon on the
left side
• M/C is when caecum fails to move to right lower
quadrant
49. • Bands of tissue called LADD BANDS connecting
caecum to right upper quadrant ->cross
duodenum and cause obstruction,can produce
volvulus (around SMA)
50. • 50 % present within 1st month of life,rest within
1st year
• With bilious vomiting,tender abdomen
• Bacterial overgrowth can later lead to
malabsorption
• Contrast studies are diagnostic
• USG shows inversion of SMA and vein (vein on
left of artery)
• Rx : surgery- reduction of volvulus,bands
dissected
51. GERD
• Passage of gastric contents into esophagus
with/without regurgitation,vomiting
• Normal physiologic phenomenon- but if
causing troublesome symptoms -> GERD
• >> risk : obesity,repaired esophageal
atresia,cystic fibrosis,hiatal
hernia,preterms,CP and family history +
52. • Present with :
recurrent regurgitation with/without vomiting
poor weight gain or weight loss
irritability
heart burn in older children
hematemesis,dysphagia and odynophagia(esophagitis)
• Dx :
Detailed history
24hr esophageal ph monitoring
24hr combined intraluminal impedance &pH monitoring
Upper gi endoscopy (esophagitis/barrett’s/strictures)
Contrast studies to rule out other causes of obstruction
53. Gastric emptying (GE) scintigraphy (milk scan) –
radiolabelled milk ingested and series of images
recorded up to 60 minutes after ingestion.To
diagnose and quantiate reflux and gastric
emptying time.Images showing isotope in the
lungs indicate pulmonary aspiration.
54. • Mx : Depends on severity of symptoms,response
to pharmacologic therapy
• Left lateral position with head end elevation by
30 deg postprandially
• Formula fed infants given extensively hydrolyzed
protein formula
• Add thickening agents like rice cereal to formula
• >> energy density of formula to promote weight
gain
• Avoiding caffeine,chocolate,spicy foods
55. • Pharmacologic therapies : buffer/suppress acid
secretion
• H2 receptor blockers – ranitidine
• PPI – omeprazole (0.7-3-3mg/kg/day)
lansoprazole(0.6-1.6mg/kg/day)
esomeprazole(<20kg - 5 to 10mg,
>20kg - 10 to 20 mg od)
2 to 4 weeks
3months if endoscopically diagnosed reflux +
A/E include headache,diarrhoea,nausea,constipation
