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Congenital Anomalies

Congenital Anomalies. Fred Hill, MA, RRT. Abdominal Wall Defects. Omphalocele - central defect in umbilicus, covered by a membrane Gastroschisis - cleft in abdominal wall to right of umbilicus. Not protected by membrane. External loops of bowel are thickened, covered by a fibrinous peel.

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Congenital Anomalies

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  1. Congenital Anomalies Fred Hill, MA, RRT

  2. Abdominal Wall Defects • Omphalocele - central defect in umbilicus, covered by a membrane • Gastroschisis - cleft in abdominal wall to right of umbilicus. Not protected by membrane. External loops of bowel are thickened, covered by a fibrinous peel

  3. Omphalocele

  4. Gastroschisis

  5. Abdominal Wall Defects Interventions • Protection and support of viscera are most important. • Nasogastric tube for decompression of bowel • Thermal regulation • Fluids and electrolytes • Prevention of infection - prophylactic antibiotics • Surgical interventions

  6. Abdominal Wall Defects Problems • Associated defects • Trisomy 13 or 18 • Urinary tract abnormalities • Beckwith-Wiedemann syndrome: includes macrosomia, macroglossia, omphalocele, and hypoglycemia • Congenital heart defects • Pentalogy of Cantrell: omphalocele, as well as defects in diaphragm, sternum, heart, and pericardium

  7. Abdominal Wall Defects Problems • Reduced abdominal cavity • Malrotation of bowel (omphalocele) • Bowel atresias,strictures, adhesions, stenoses (gastroschisis) • Difficulty in ventilation when bowel is compressed surgically into abdomen

  8. Congenital Diaphragmatic Hernia • Occurrence: 1 in 3000 births • Description: displacement of abdominal contents through diaphragm into thoracic cavity - most often left-sided

  9. Congenital Diaphragmatic Hernia

  10. Congenital Diaphragmatic HerniaRecognition • Respiratory distress • Scaphoid abdomen • Presence of bowel sound and/or absence of breath sounds in all or portion of chest • Displaced heart sounds - away from affected side - most often, dextrocardia

  11. Congenital Diaphragmatic HerniaInterventions • Do notbag-and-mask ventilate • Give 100% oxygen • Intubate if respiratory distress is profound • Ventilate with small tidal volumes/ minimize peak airway pressures (which will tend to be high) • Watch for pneumothorax • Decompress stomach with orogastric tube (if possible) • Transport with affected side down • Surgical intervention

  12. Congenital Diaphragmatic HerniaProblems • Pulmonary hypoplasia • Pneumothorax/barotrauma • Persistent fetal circulation

  13. Tracheo-Esophageal Fistula/ Esophageal Atresia • Occurrence: 1 in 4500 births • Description: various interruptions in esophagus and abnormal connections to the trachea

  14. Tracheo-Esophageal Fistula/ Esophageal Atresia

  15. Tracheo-Esophageal Fistula/ Esophageal Atresia • Esophageal atresia without fistula (5-7%) • Esophageal atresia with distal fistula (85%) • Esophageal atresia with proximal fistula • Esophageal atresia with proximal and distal fistula • T-E fistula without esophageal atresia (H-type) (5%)

  16. Tracheo-Esophageal Fistula/ Esophageal Atresia Recognition • Polyhydramnios • Excess salivation and drooling • Episodes of choking, gagging, and dyspnea, especially with feeding • Crying or coughing leads to distended abdomen • Chest X-ray may reveal pneumonia, pneunonitis, atelectasis, elevated diaphragm. Dilated esophageal pouch. Presence or absence of air in abdomen • Inability to pass a large catheter into esophagus

  17. Tracheo-Esophageal Fistula/ Esophageal Atresia Interventions • Maintain in 30 degree, upright position to minimize chances of gastric reflux • Insert nasogastric tube into esophageal pouch and suction to remove excess, pooled secretions • Humidification, CPT, oxygen, and antibiotics may be added in the treatment of aspiration pneumonitis • Feeding can be accomplished via gastrostomy tube when surgical correction is delayed • Surgical intervention

  18. Tracheo-Esophageal Fistula/ Esophageal Atresia Problems • Cardiac (37%): most common (1) VSD, (2) PDA, (3) Tetrology of Fallot • Gastrointestinal (21%) • VACTERL syndrome (7%): vertebral, anal, cardiac, trachea, esophageal, renal, and limb anomalies

  19. Choanal Atresia Descriptions • Choanae: two openings in the posterior portion of the nasal cavity that allow airflow from the nose to pharynx • Choanal atresia: blockage of these openings from choanal stenosis, a bony septum, or membranous obstruction

  20. Choanal Atresia

  21. Choanal AtresiaRecognition • Newborns are “obligate nasal breathers” first two months of life • Respiratory distress - cyanosis and retractions • resolves when the baby cries • worsens when the baby sucks • Failure to pass a 6 Fr suction catheter through nares • Visualization of region by nasopharyngoscope • Unilateral choanal atresia may have less severe to nonexistent respiratory distress, inspiratory stridor may be heard

  22. Choanal AtresiaInterventions • Placement of oral airway • Topical decongestant in case obstruction caused by nasal edema rather than choanal atresia

  23. Choanal AtresiaProblems • 20 to 50% have assciated defects • CHARGE syndrome • Colobomata of the eyes • Heart defects • Atresia of the choanae • Renal anomaly • Growth and mental retardation, gastresophageal reflux • Ear deficits

  24. Pierre-Robin Syndrome • Description: Glossoptosis and micrognathia. Tongue is large in comparison to mandible, reduced oropharynx. Often includes cleft palate. Tongue is more posterior and falls back in hypopharynx to cause airway obstruction. • Recognition: reduced mandible. Mild-to-severe respiratory distress to complete obstruction

  25. Pierre-Robin Syndrome

  26. Pierre-Robin SyndromeInterventions • Prone position • Nasopharyngeal airway • Nasotracheal airway • Surgical suturing of tongue to lower lip to button attached to skin of chin • Tracheostomy • Gastrostomy or nasogastric tube for feedings

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