newborn respiratory distress 11.28.2011

8/3/2019 Newborn Respiratory Distress 11.28.2011

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Morning Report 11/28/2011

By Elizabeth Parsons, MDUniversity of Utah, Department of Pediatrics



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.



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.



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



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.



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.



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



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



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



Diagnosis

CT angiography



Diagnosis

Congenital Lobar Emphysema



Congenital Lung Abnormalities


Diaphragmatic Hernia

Congenital Pulmonary Airway Malformation

Pulmonary Sequestrations

Bronchogenic Cyst




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.



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



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

http://reference.medscape.com/




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





CLE: Diagnosis

CT Angiography: Can identify source of obstruction(instrinsic vs extrinsic)

Ventilation-Perfusion Scan: ↓ perfusion of affectedlobe and ↑ perfusion of unaffected lobe



CLE: Treatment

Figure 384-2 Algorithm for evaluation and treatment of congenital lobar emphysema (CLE) Nelson's Textbook of Pediatrics



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.




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%)




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.



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



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.



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



CCAM/CPAM Diagnosis

CT scan usually with angiography

MRI

C i l P l Ai



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.)



CPAM/ CCAM

Mandell, Gerald, MD. Imaging in Congenital Cystadenomatous Malformation. http://emedicine.medscape.com. Medscape Reference. 2011

http://emedicine.medscape.com/




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.



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.




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




Berrocal, Teresa et al. Congenital Anomalies of the Tracheobroncial Tree, Lung, and Mediastinum; Embryology, Radiology, and Pathology. Jan

2004. Radiographics. 24. e17.



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



Intrapulmonary Sequestration



Extrapulmonary Sequestration



Pulmonary Sequestration Treatment

Balloon occlusion of systemic arterial supply

Surgical resection is curative

Intrapulmonary- often poorly defined margins sooften needs lobectomy

Prognosis is good.



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



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.



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





Bronchogenic Cyst

Roxas, Ronato. Detecting Congenital Lung Abnormalities in Children and Adults http://reference.medscape.com.Medscape Pediatrics. 11/19/201





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



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





newborn respiratory distress 11.28.2011

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