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Case Report

Radiological Category:. Pediatrics. Principal Modality (1): Principal Modality (2):. General Radiography. MRI. Case Report. Submitted by:. Todd Danziger, MS IV. Faculty reviewer:. Sandra Oldham, M.D. Date accepted:. 28 September 2011. Case History.

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Case Report

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  1. Radiological Category: Pediatrics Principal Modality (1): Principal Modality (2): General Radiography MRI Case Report Submitted by: Todd Danziger, MS IV Faculty reviewer: Sandra Oldham, M.D. Date accepted: 28 September 2011

  2. Case History Infant History: 37 week premature girl born at OSH via repeat caesarian section with birth weight 2.645 kg and APGARs 8 @ 1 min and 9 @ 5 min. Developed respiratory distress and hypoxia after birth and was transferred to NICU, where she was initially placed on NCPAP and then eventually intubated. ECHO was obtained at OSH and patient was transferred to MHCH via air transport for higher-level of care. Maternal History: 22 yo G2P1 Prenatal Labs: Blood: O+, Ab neg, RPRNR, HepB neg, HIV neg, GBS neg, rubella equivocal, UDS neg 36 week Ultrasound: AFI 7.2 cm, hydronephrosis of right kidney

  3. Case History P.E.: T 99.6 BP 46/32 MAP 36 P 148 R 19-66 SpO2 86 FiO2 21% Gen: NAD, sedated, under radiant warmer HEENT: Normocephalic, oral mucosa moist, ears normally set and rotated Resp: Lungs clear to auscultation CV: Normal rate, regular rhythm, systolic murmur 2/6 best heard over LUSB, good pulses in all extremities, normal peripheral perfusion GI: soft, non-distended, no organomegaly, anus patent GU: normal female Integument: warm, pink, no rash Neurologic: No focal defects, sedated

  4. Radiological Presentations Chest/Abdomen Pediogram

  5. Radiological Presentations A/P Portable

  6. Test Your Diagnosis Which one of the following is your choice for the appropriate diagnosis? After your selection, go to next page. • Meconium Aspiration • Neonatal Respiratory Distress Syndrome • Transient Tachypnea of the Newborn • Persistent Pulmonary Hypertension of the Newborn • Congenital Heart Disease

  7. Radiological Presentations MRI heart

  8. Radiological Presentations MRI heart

  9. Radiological Presentations MRI heart

  10. Radiological Presentations MRI heart

  11. Initial CXR: normal-sized heart with normal pulmonary vascularity with right descending aortaLater CXR: upturn of the cardiac apex . . . Boot-shaped heartECHO: moderate VSD, moderate aortic override, pulmonary atresia with a hypoplastic main pulmonary artery and RV outflow tract, and mild biventricular hypertrophy. Arch sidedness could not be determined. Repeat ECHO suggested a double aortic arch. Cardiac MRI: TOF (VSD, pulmonary atresia, overriding aorta, RVH); double aortic arch with descending aorta on the right; PDA Findings and Differentials Findings: Differentials: • Congenital Heart Disease (Tetralogy of Fallot) • Meconium Aspiration Syndrome • Neonatal Respiratory Distress Syndrome • Transient tachypnea of the newborn • Persistent Pulmonary Hypertension of the Newborn

  12. Meconium Aspiration Syndrome Clinical History: -Meconium-stained amniotic fluid in a term or post-term infant -Respiratory distress: tachypnea, hypoxia, and hypercapnia -May develop pulmonary hypertension and require positive pressure ventilation Radiographic finding: Air trapping and patchy atelectasis Wiedemann J R, A M Saugstad, L Barnes- Powell, and K Duran. Meconium Aspiration Syndrome. Neonatal Network 2008, 27: 81-87. Discussion

  13. Neonatal Respiratory Distress Syndrome or Hyaline Membrane Disease Clinical history: Premature infant with tachypnea, grunting, nasal flaring, chest wall retractions, and cyanosis in first few hours of life Pathogenesis: -Surfactant deficiency -Poor compliance -Atelectasis, intrapulmonary shunting, hypoxemia, and cyanosis -Hyaline membrane: mechanical ventilation, oxygen exposure, and alveolar capillary leak Radiographic findings: Uniform reticulonodular or ground-glass pattern and air bronchograms Treatment: surfactant Hermansen CL, Lorah KN. Respiratory Distress in the Newborn. American Family Physician 2007, 76: 987-994 Discussion

  14. Transient tachypnea of the newborn Most common etiology of neonatal respiratory distress (>40%) Secondary to residual pulmonary fluid remaining in the fetal lung Risk factors: maternal asthma, maternal diabetes, macrosomia, and cesarean delivery Tachypnea begins soon after birth and can last several hours to days Radiographic findings: -diffuse parenchymal infiltrates -“wet silhouette” -intralobar fluid accumulation and fluid in fissures Hermansen CL, Lorah KN. Respiratory Distress in the Newborn. American Family Physician 2007, 76: 987-994 Discussion

  15. Persistent Pulmonary Hypertension of the Newborn Also called persistent fetal circulation Pathogenesis: -Acute or chronic hypoxia in utero -Failure of pulmonary vascular resistance to fall with postnatal lung expansion and oxygenation -Worsening right-to-left shunt Present with hypoxemia, cyanosis, and acidosis Radiographic findings: depend on cause ECHO: absence of structural heart disease, evidence of increased pulmonary vascular resistance, and right-to-left shunt at foramen ovale and/or ductus arteriosus Treatment: Inhaled nitric oxide; ECMO for most severely ill Marino BS Fine KS. Blueprints Pediatrics, 5th ed. Baltimore: Lippincott Williams & Wilkins, 2009. Discussion

  16. Tetralogy of Fallot Most common cyanotic heart disease Obstruction of right ventricular outflow tract Ventricular septal defect (VSD) Overriding of the aorta above the VSD Right ventricular hypertrophy Right aortic arch in 20-30% “Tet spells”: periodic episodes of cyanosis, rapid and deep breathing, agitation from increase in right ventricular outflow tract resistance Haider EA. The Boot-shaped Heart Sign. Radiology 2008, 246: 328-9 Discussion

  17. Pathogenesis Anterosuperior displacement of the infundibular septum Aortic valve forms superior border of VSD Subpulmonic stenosis and right-to-left shunt Right ventricular hypertrophy Pulmonary arteries become hypoplastic and the aorta grows larger Discussion Kumar et al. Robbins and Cotran Pathologic Basis of Disease, 8th ed. Philadelphia: Saunders, 2010: 542-3. Photos from Yale Medical Group

  18. Boot-shaped heart sign (65%) Sign of decreased pulmonary vasculature Upturned cardiac apex from right ventricular hypertrophy Small or absent main pulmonary artery Accentuated by a small thymus Only seen in infants with moderate to severe cases Upturn of the apex increases with severity of RV outflow tract obstruction RV failure is rare Haider EA. The Boot-shaped Heart Sign. Radiology 2008, 246: 328-9 Discussion

  19. Tetralogy of Fallot Diagnosis

  20. Haider EA. The Boot-shaped Heart Sign. Radiology 2008, 246: 328-9Hermansen CL, Lorah KN. Respiratory Distress in the Newborn. American Family Physician 2007, 76: 987-994.Kumar et al. Robbins and Cotran Pathologic Basis of Disease, 8th ed. Philadelphia: Saunders, 2010: 542-3. Marino BS Fine KS. Blueprints Pediatrics, 5th ed. Baltimore: Lippincott Williams & Wilkins, 2009.Wiedemann J R, A M Saugstad, L Barnes-Powell, and K Duran. Meconium Aspiration Syndrome. Neonatal Network 2008, 27: 81-87. References

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