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Surgery Board Review

March 29, 2011. Surgery Board Review. Respiratory distress. Attempt to pass NG tube Esophageal atresia Choanal atresia Half have other congenital anomaly CHARGE Syndrome Coloboma Heart Atresia of choanae Retarded development Genital hypoplasia Ear. Upper Airway. Membranous 90%

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Surgery Board Review

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  1. March 29, 2011 Surgery Board Review

  2. Respiratory distress

  3. Attempt to pass NG tube • Esophageal atresia • Choanalatresia • Half have other congenital anomaly • CHARGE Syndrome • Coloboma • Heart • Atresia of choanae • Retarded development • Genital hypoplasia • Ear Upper Airway

  4. Membranous 90% Bony 10% Gavage feed until repair Maintain oral airway Choanalatresia

  5. True enlargement of tongue • Vs. Glossoptosis in which normal tongue protrudes from abnormally small oral cavity • Focal (pebbly, vescicle-like blebs) • Congenital tumors (lymphangioma, hemangioma) • One-half • Hemihypertrophy Macroglossia

  6. Generalized (smooth surface) • Beckwith-Wiedemann, hypothyroidism • Generalized (papillary, fissured) • Down syndrome • Also poor tone • Progressive • Mucopolysaccharidoses Macroglossia

  7. This child was most likely to have which of the following metabolic abnormalities as a newborn? A Hyperkalemia B Hypokalemia C Hyponatremia D Hypoglycemia E Metabolic Acidosis Question 1

  8. Features • Macroglossia • Macrosomia • Hypoglycemia (islet cell hyperplasia) • Hemihypertrophy • Hypospadias • Omphalocele • 18 month old with B.W. presents with gross hematuria: • Wilms Tumor Beckwith WiedemannSyn

  9. Unusually small mandible (micrognathia) • Posteriorly displaced tongue • Upper airway obstruction • U-shaped cleft palate • May be part of a larger syndrome Pierre Robin Sequence

  10. Hoarseness Faint cry Aphonia Associated dyspnea Laryngeal lesions

  11. Thoracic Lesions

  12. Which is the most common abnormality? Question 2 A B C D E

  13. TE fistulae: Most common types… • 1 Esophageal atresia and distal fistula • 2 Esophageal atresia and no fistula • 3 H-Type (only one without atresia) • Manifestation may not be as severe 2 1 3

  14. Inability to pass NG tube • Use to drain secretions • Resp distress secondary • Fistula • Reflux of pouch contents • Inability to tolerate initial feed • Excessive drooling • May see distension of stomach (air fistula) • Avoid positive pressure ventilation • Antenatal U/S: Microgastria, polyhydramnios TE Fistula

  15. Dilated proximal pouch • Gasless abdomen • Scaphoid abdomen Pure esophageal atresia

  16. Bronchopulmonary foregut malformation

  17. Congenital Lobar Emphysema Congenital cystic adenomatoid malformation Pulmonary sequestration Bronchogenic cyst Bronchopulmonary foregut malformation

  18. Portion of lungs perfused by systemic arteries • Lacks normal connection with tracheobronchial tree • Intralobar: • Most common lower lobes • Anywhere in thorax • Extralobar: • Subdiaphragmatic or retroperitoneal • Associated with other anomalies • Dx: MRI or angiogram Pulmonary sequestration

  19. Pulmonary sequestration

  20. Occur any point along the tracheobronchial tree • Centrally located • Does not communicate, fluid fill with wall composed of tissue resembling large airways • Typically present 2nd decade of life with recurrent wheezing, cough, pneumonia Bronchogenic Cyst

  21. Bronchogenic Cyst

  22. Previously known as Congenital Cystic Adenomatoid Malformation (CCAM) • Hamartomatous lesion • Cystic and adenomatous elements • Resp distress, recurrent PNA, asymptomatic • Severe forms can be lethal after birth • Surgical resection if symptomatic • Controversial if asymptomatic Congenital Pulmonary Airway Malformation (CPAM)

  23. Congenital Pulmonary Airway Malformation (CPAM)

  24. Results from area of poorly developed or absent cartilage • Check-valve effect • Air trapping • Lung hyperexpansion • Possible acute decompensation with positive pressure ventilation Congenital Lobar Emphysema

  25. Gi obstruction

  26. Bilious emesis is always considered a red flag for mechanical obstruction. Surgical emergency until proven otherwise GI Obstruction

  27. Most common cause is overfeeding Second most common: GE Reflux Nonbilious emesis in infants

  28. Fairly common 1:300 • Unknown genetic link • Vomiting starts by 2wks old • Progressively projectile • Age at presentation • Most commonly b/f 1 month • Remember: 2wks-2months • Male predominance • Firstborn Hypertrophic Pyloric Stenosis

  29. Clinical Manifestations: • Abdomen probably will not be distended • Can feed them to look for signs • Distended over stomach immediately after feeding • Peristaltic waves • Palpable olive • After vomiting • Epigastric b/w midline and R midclavicular line Pyloric Stenosis

  30. Diagnosis • Palpable Olive (no imaging required) • Hypochloremic, hypokalemic metabolic alkalosis • Ultrasonography • Pyloric muscle • Thickness 3-4mm • Length 15-16mm Pyloric Stenosis

  31. Upper GI • the "string" sign • the "double-track" sign • the "beak" sign • the "shoulder" sign Diagnosis

  32. Surgical treatment (pyloromyotomy) • Fluid and electrolyte correction are most important • Surgery after these are corrected Pyloric Stenosis

  33. You are working up a 4 month old infant with bilious vomiting. Findings on barium enema reveal cecum located in left upper abdomen. The most likely diagnosis is: • A situsinversus • B malrotation • C intussusception • D intestinal duplication Question 3

  34. Malrotation with midgutvolvulus Bilious Vomiting In Infant

  35. Proximal obstruction at distal duodenum/ proximal jejunum • Bilious emesis • May not have significant abdominal distention until late • Late finding: hematemesis, hematochezia • Bowel ischemia leading to perforation and death Malrotation with midgutvolvulus

  36. Diagnosis • Suspect in setting of: • Bilious vomiting in infant (or older child) • Abdominal films: • Gastric distention and duodenal dilatation • Otherwise gasless abdomen Malrotation with midgutvolvulus

  37. Surgical correction • Ladd Procedure • Division of Ladd bands • Appendectomy • Widening of mesentery • Securing intestines in position of nonrotation Malrotation

  38. Bilious vomiting shortly after birth • May occur later • Annular pancreas • Duodenal stenosis • Duodenal web • Ladd bands • Associated with Down Syn and congenital heart disease in 30-50% Duodenal Atresia

  39. 20% of patients with CF Meconiumileus is CF until proven otherwise Intraluminal obstruction by thickened meconium Distal ileum is small, may have microcolon Possible proximal bowel dilitation “soap bubble” appearance MeconiumIleus

  40. Contrast enema (may be temporarily therapeutic) • Microcolon • Inspissatedmeconium “rabbit pellets” • May perforate • Intraperitoneal calcifications and ascites prenatally MeconiumIleus

  41. You are seeing a new 9 month old who has had difficultly stooling since birth. You see in the records that this imaging has recently been performed Question 4 • Which information is FALSE? • A Rectal suction biopsy should be done • B Identification of transition zone is helpful • C Development of explosive diarrhea fits with diagnosis • D Barium enema should be performed following GI cleanout

  42. Absence of ganglion cells in distal rectum • Bilious emesis and abdominal distention • BE: proximally dilated bowel (normal) • Transition zone • Distal decompressed segment (abnormal) • Barium study should preceed rectal exam or enemas • Definitive: rectal suction biopsy Hirschsprung disease

  43. May be diagnosed in older child with chronic constipation • Requiring rectal stimulation to evacuate • Lifethreateninghirschsprung-related enterocolitis • Explosive diarrhea • Toxic megacolon • Sepsis Hirschsprung disease

  44. 6mos-2yrs • Older children more likely to have lead point • Meckel • Polyp • Lymphoma • Intestinal duplication cyst • Hematoma/inflammation • Hemophilia, HSP Intussusception

  45. Classic location: ileocolic • (may be ileo-ileal if there is a lead point) • Presentation • Paroxysmal bouts of severe colicky abdominal pain • Drawing up legs • Period of somnolence • Normal activity between episodes • Unexplained lethargy or seizure-like episode Intussusception

  46. Emesis may turn bilious late in course Current jelly stool: late finding “sausage-shaped mass” RUQ Intussusception

  47. Evaluation: • Start with plain films • Crescent sign, target sign • If suspicion high, can go straight to air contrast or barium enema • Diagnostic and therpeutic • Emergent surgery if enema unsuccessful • May recur in first 12 to 24 hrs Intussusception

  48. GI bleeding

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