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DEVELOPMENT OF GIT AND CONGENITAL ANOMALIES OF GIT

GASTROINTESTINAL TRACT DEVELOPMENT , CONGENITAL MALFORMATIONS , ANOMALIES , GERD , NELSON , PEDIATRICS , NELSON

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DEVELOPMENT OF GIT AND CONGENITAL ANOMALIES OF GIT

  1. 1. DEVELOPMENT AND CONGENITAL ANOMALIES OF GIT - Dr.Apoorva.E PG,DCMS
  2. 2. DEVELOPMENT OF GIT
  3. 3. Liver
  4. 4. Urorectal septum
  5. 5. CONGENITAL ANOMALIES OF GIT
  6. 6. 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
  7. 7. 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
  8. 8. 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
  9. 9. 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
  10. 10. CLEFT PALATE CLEFT LIP
  11. 11. 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
  12. 12. • TYPES :
  13. 13. • 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
  14. 14. • 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
  15. 15. • 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
  16. 16. 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
  17. 17. • 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
  18. 18. • 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)
  19. 19. • Contrast shows string sign,shoulder sign,double tract sign • Rx : Correct fluid electrolyte acidbase imbalance Ramstedt ‘s pyloromyotomy Endoscopic balloon dilatation Atropine
  20. 20. HIATAL HERNIA • Herniation of upper part of stomach through esophageal hiatus
  21. 21. • C/F : early satiety,upper abdominal pain • Dx : by contrast studies,endoscopy • Mx : Medical treatment of gastro esophageal reflux Nissen’s fundoplication
  22. 22. 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
  23. 23. • TYPES : Membrane near ampulla of Vater
  24. 24. • 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
  25. 25. • Mx : Nasogastric decompression Correct fluid and electrolyte imbalance Definitive Rx by duodenoduodenostomy
  26. 26. 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
  27. 27. • 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)
  28. 28. • Majority have an ectopic mucosa (acid secreting gastric mucosa M/C) -> ulceration -> painless rectal bleeding (malena / brick colored stool) • Other complications : Intussusception,volvulus,obstruction,diverticulitis, perforation, peritonitis • Dx : Technetium-99m pertechnate scan,USG,CT • Rx : surgical excision
  29. 29. 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
  30. 30. • 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
  31. 31. • Faecal mass palpable in left lower abdomen • Empty rectum on PR • Dx by rectal biopsy (gold standard) • >> acetylcholinesterase on staining • Anorectal manometry,Contrast studies
  32. 32. • 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
  33. 33. 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
  34. 34. • Bands of tissue called LADD BANDS connecting caecum to right upper quadrant ->cross duodenum and cause obstruction,can produce volvulus (around SMA)
  35. 35. • 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
  36. 36. 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 +
  37. 37. • 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
  38. 38. 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.
  39. 39. • 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
  40. 40. • 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
  41. 41. • ? Prokinetics – metoclopramide,domperidone etc • Surgery – Fundoplication (>>LES tone)
  42. 42. ACHALASIA CARDIA
  43. 43. THANK YOU

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