newborn respiratory distress 11.28.2011
TRANSCRIPT
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Morning Report 11/28/2011
By Elizabeth Parsons, MDUniversity of Utah, Department of Pediatrics
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Case Presentation
CC: Term female presents by transfer fromTimpanogos Regional Medical Center forrespiratory distress , cyanosis and hypoxia
Term female delivered by NSVD at 41 6/7weeks gestation to a 32 yo G7P5015 mother.Mother had limited PNC with lay midwife and
was planned home delivery. Admitted tohospital after failure to progress. Membranesruptured 9 hours prior to delivery and had clear
amniotic fluid.
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Case Presentation
Initially vigorous, but at 2 min of life appeared“dusky” and tachypneic with increased work of breathing. Sats were 47% on room air. Received
CPAP x 5 minutes and then placed in head-boxFiO2 45-50% with improvement of sats > 90%.APGARS were 8 at 1 minute and 7 at 5 minutes.
Prenatal History: Uncomplicated pregnancy.No prenatal ultrasound. Maternal blood type Apositive/Antibody negative. No other labs or
prenatal care obtained.
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Case Presentation
FHx: Patient has older sibling that died after surgeryfor congenital heart disease (double aortic arch?)Patient has 5 other healthy siblings.
SH: Polygamy family. Neg illicits, ETOH, tobaccoduring pregnancy
Meds: No medications during pregnancy. Received
vitamin K; Erythromycin and Hep B vaccine refused
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Physical Exam
Growth Parameters: BW 4610 g, (98%) L = 58 cm ( 100%), OFC
37.5 cm (92%)
VS: T: 36.5, HR 148, RR 49-55, BP 83/65 (71)
GENERAL: Term female infant in moderate respiratory distress.
HEENT: AFOSF, EOMI. PERRL. RR present b/l. Normally
formed ears without pits or tags. Palate intact.
NECK: Supple with no masses. Clavicles are intact.
HEART: Regular rate and rhythm. Her PMI is displaced to the left.
No murmur is heard. Capillary refill is 3-4 seconds.
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Physical Exam
LUNGS: Tachypneic with suprasternal and subcostal retractions.
Diminished BS on left and right lung base.
ABDOMEN: Soft, ND, NT. + BS. Liver at RCM. UAC/UVC in
place. No masses.
GENITOURINARY: Nml female external genitalia. Patent anus
BACK: Spine is straight with no sacral dimple or tuft noted.
EXTREMITIES: Nml ROM. Normal hips. Normally formed
hands/feet/digits.
NEUROLOGIC:Vigorous suck, palmar grasp, Moro. Normal tone
and strength.
SKIN: Warm and pink. No rashes, jaundice or petechiae.
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Differential Diagnosis
PULM: RDS, TTN, Pulm hypoplasia,pneumothorax, diaphragmatic hernia, aspiration(meconium), pneumomediastium, congenital lobar
emphysema, CCAM, TE fistula, thoracic mass, pulmsequestration, Pulm AVM, Pulmonary interstitialedema, Pulm edema, congenital alveolar proteinosis,
pulmonary agenesis, chylothorax.
CV: Acyanotic/cyanotic CHD, pulmonaryhypertension, arrythmias, vascular ring,
cardiomyopathy
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Differential Diagnosis
Metabolic: IDM/hypoglycemia, inborn errors,acidosis
Neuro: Birth trauma, hemorrhage, maternalsedation, HIE, seizure d/o, hydrocephalus, spinalcord injury.
ID: Sepsis (GBS), pneumonia
Upper Airway Obstruction: (Choanal atresia,
pierre robin, vocal cord paralysis, cleft palate)
Thoracic cage deformities
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Objective
CXR
ABG: 7.32/42/125/22/-4 iSTAT Glu = 98
CBC: WBC = 21 (11%B, 59% P; I/T=0.16)HCT =56 PLT: 196
CRP < 0.1
Blood culture sent
RFP: Normal
COAGS: Normal
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Diagnosis
CT angiography
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Diagnosis
Congenital Lobar Emphysema
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Congenital Lung Abnormalities
Congenital Lobar Emphysema
Diaphragmatic Hernia
Congenital Pulmonary Airway Malformation
Pulmonary Sequestrations
Bronchogenic Cyst
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Congenital Lobar Emphysema
Occurs secondary to bronchial obstructionresulting in massive overinflation of one or more
pulmonary lobes (extrensic vs intrinsic)
- Airway cartilage deficiency (intrinsic)
- Aberrant pulmonary vessel compression
- Bronchial stenosis/ kinking/redundant bronchialmucosal flaps
LUL > RML> RUL.
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CLE: Clinical Manifestations
Can be diagnosed on antenatal ultrasound
Sx range from asymptomatic(rare), wheeze,
tachypnea, cyanosis or mild/mod/severe respiratory
distress
Affected lung is overdistended and nonfunctional
with compression/atelectasis of ipsilateral normal
lung
Can cause mediastinal shift with compression of
contralateral lung
15-20% associated with congenital heart disease
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CLE: Diagnosis
CXR:• Space-occupyingair-filled lobe
• Collapse of ipsilateralLung
• Mediastinal shift
• Atelectasis of contralateral lung
• Often misdiagnosedas tension ptx
Roxas, Ronato. Detecting Congenital Lung Abnormalities in Children and Adults. http://reference.medscape.com. Medscape Pediatrics. 11/19/201
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Gross/Histology CLE
Gross specimen: Photograph taken by Elizabeth Parsons, MD courtesy of Michael Rollins, MD, Pediatric Surgery. Micro Path
:Roxas,
Ronato. Detecting Congenital Lung Abnormalities in Children and Adults. http://reference.medscape.com. Medscape Pediatrics. 11/19/201
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CLE: Diagnosis
CT Angiography: Can identify source of obstruction(instrinsic vs extrinsic)
Ventilation-Perfusion Scan: ↓ perfusion of affectedlobe and ↑ perfusion of unaffected lobe
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CLE: Treatment
Figure 384-2 Algorithm for evaluation and treatment of congenital lobar emphysema (CLE) Nelson's Textbook of Pediatrics
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Congenital Diaphragmatic Hernia
Defect in diaphragm allowingcommunication between thoracicand abdominal cavities.
Hiatal, Paraesophageal, Retro-sternal (Morgagni), orposteriorlateral (Bochdalek)location.
1/2,000-5,000 live births
Female: Male = 2:1
80-90% are left sided.
Maheswari, Akhil et al. “Diaphragmatic Hernia” in Nelson Textbook of Pediatrics, 19 th Ed. Kleigman et al. Philadelphia; Elsevier. 2011.
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Congenital Diaphragmatic Hernia
Usually spontaneous; rarely familial
Associated with Trisomy 21, 18 & 13, Turner,
Pallister Killian, Fryns, & Brachmann de Lange 30% have associated congenital anomalies (CNS
lesions, CHD, esophageal atresia, omphalocele)
Survival dependent on severity of pulmonaryhypoplasia (average ~ 67%)
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Congenital Diaphragmatic Hernia
Respiratory Distress (may not present until 48 hrs)
PE: ↑ chest wall diameter, scaphoid abdomen,
displaced PMI, diminished BS, Bowel sounds inchest wall.
Intestinal obstruction (vomiting, sepsis, shock)
Cardiovascular collapse (compression)
TX: Resp support/careful ventilation, CV support,
and surgery when stable.
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Congenital Pulmonary Airway
Malformation (aka CCAM)
Developmental hamartomatous abnormality of thelung, with adenomatoid proliferation of cystsresembling bronchioles
Blood supply from pulmonary circulation andcommunicates with tracheobronchial tree.
1-4 cases/ 100,000
Insult usually occurs early < 35 days of gestation
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CCAM Types
Type 1 (50%): MACROcyst(s) (2-10 cm indiameter). Ciliated pseudostratified epithelium thatsometimes secrete mucin (1/3). Best prognosis.
Type 2 (40%): MICROcyst(s) (0.5-2cm). Similarhistology to type 1. Strong association with othercongenital abnormalities.
Type 3 (<10%): Multiple microscopic cyst create aSOLID mass without obvious cyst. Bronchiole likestructures with cuboidal ciliated epithelium.
POOREST prognosis.
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CPAM/CCAM
Prenatal Ultrasound (defined as micro/macro cystic
May show fetal hydrops (bad prognosis!), pleural
effusion, ascites, polyhydramnios Neonate: respiratory distress/failure, pneumothorax,
mediastinal shift, pulmonary hypoplasia
Older Child: Recurrent Infections, chest pain,dyspnea, hemoptysis, FTT
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CCAM/CPAM Diagnosis
CT scan usually with angiography
MRI
C i l P l Ai
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Congenital Pulmonary Airway
Malformation (aka CCAM)
Lakhoo K: Management of congenital cystic adenomatous malformations of the lung, Arch Dis Child Fetal Neonatal Ed 94:F73 –F76, 2009.)
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CPAM/ CCAM
Mandell, Gerald, MD. Imaging in Congenital Cystadenomatous Malformation. http://emedicine.medscape.com. Medscape Reference. 2011
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CCAM/CPAM Treatment
Symptomatic neonates- surgical resection
Asymptomatic: Surgery < 1 year of age
Unresected CCAM has potential for sarcomatous/ carcinomatous malignant differentiation andspontaneous pneumothoraces.
Rule out pleuropulmonary blastoma (differentialdiagnosis)
Mortality 10-30%. Higher with hydrops,polyhydramnios, or type 3 CCAM.
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Pulmonary Sequestration
Cystic/Solid mass composed of embryonic tissuethat DOES NOT communicate with thetracheobronchial tree AND has anomalous
SYSTEMIC blood supply.
15-25% have multiple systemic feeding vessels
Thought to be due to an accessory lung bud that
develops from the ventral primitive foregut.
Does NOT participate in gas exchange or result in aL→ R shunt.
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Pulmonary Sequestration
INTRAPULMONARY
75% of cases
Males = Females
Does not have its OWN pleura (lieswithin pleura of lung lobe)
Systemic blood supply, but PULM
VENOUS DRAINAGE!!!
Posterior basal segment of lung(L>R)
Often delayed diagnosis with
presentation of recurrent infectionsor hemoptysis.
EXTRAPULMONARY
25% of cases
Males: Females = 4:1
Has OWN visceral pleura
Thoracic/Subdiaphragmatic All leftsided.
Systemic blood supply AND
SYSTEMIC venous drainage Presents earlier as abdominal mass,
heart failure, respiratory distress.
Associated with CDH, colonic
duplication, vertebral anomalies,
etc
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Pulmonary Sequestration
Berrocal, Teresa et al. Congenital Anomalies of the Tracheobroncial Tree, Lung, and Mediastinum; Embryology, Radiology, and Pathology. Jan
2004. Radiographics. 24. e17.
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Pulmonary Sequestration Diagnosis
CXR with recurrent pneumonia that has slow orincomplete resolution.
Prental doppler US showing systemic arterial supplyto fetal lung lesion
CT/ MR angiography
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Intrapulmonary Sequestration
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Extrapulmonary Sequestration
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Pulmonary Sequestration Treatment
Balloon occlusion of systemic arterial supply
Surgical resection is curative
Intrapulmonary- often poorly defined margins sooften needs lobectomy
Prognosis is good.
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Bronchogenic Cyst
Congenital cysts that originate from primitiveforegut
Mediastinal (2/3), intrapulmonary (1/3), or neck (rare)
Paratracheal, hilar, carinal
Do not initially communicate with tracheobronchialtree, but may if instrumented or have recurrent
infection
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Bronchogenic Cyst
Most asymptomatic, but in infancy can result inrespiratory distress depending on location(subcarinal)
Symptoms related to compression: cough, stridor,dyspnea, dysphagia, recurrent pneumonia
Rarely detected prenatally; usually dx late
Usually contain mucoid material lined by ciliated
columnar/cuboidal epithelium. May develop PUD if gastric mucosa or may have cartilage.
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Bronchogenic Cyst Diagnosis
CXR
Esophogram
CT/ MRI
Roxas, Ronato. Detecting Congenital Lung Abnormalities in Children and Adults. http://reference.medscape.com. Medscape Pediatrics.11/19/201
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Bronchogenic Cyst
Roxas, Ronato. Detecting Congenital Lung Abnormalities in Children and Adults http://reference.medscape.com.Medscape Pediatrics. 11/19/201
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Bronchogenic Cyst Treatment
Surgical Resection of ALL bronchogenic cyst- highpredilection for infection.
Asymptomatic newborns: Resect at 3-6 months toallow normal compensatory lung growth
Prognosis is Excellent.
f
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References
Behrman, Richard E., Hal B. Jenson, and Robert M. Kliegman. Nelson's Textbook of
Pediatrics. London: W.B. Saunders, 2011.
Berrocal, Teresa et al. Congential Anomalies of the Tracheobronchial Tree, Lung and
Mediastinum; Embryology, Radiology, and Pathology. Jan 2001. Radiographics. 24 e17.
Lakhoo K: Management of Congenital Cystic Adenomatous Malformations of the Lung,
Arch Dis Child Fetal Neonatal Ed 94:F73 – F76, 2009.)
Mandell, Gerald, MD. Imaging in Congenital Cystadenomatous Malformation.
http://emedicine.medscape.com. Medscape Reference. 2011
Roxas, Ronato. Detecting Congenital Lung Abnormalities in Children and Adults.http://reference.medscape.com. Medscape Pediatrics. 11/19/2011