Pulmonary Mass Pulmonary Mass in a Neonatein a Neonate

Filomena Hazel R. Villa, MDFilomena Hazel R. Villa, MD

PL 1- PediatricsPL 1- Pediatrics

ObjectiveObjective

To present a case of a neonate with To present a case of a neonate with pulmonary mass, its management and pulmonary mass, its management and differential diagnosesdifferential diagnoses

HistoryHistory

Boy M, 5 hours oldBoy M, 5 hours old Born term (40 weeks), LGA (3732 g)Born term (40 weeks), LGA (3732 g) 21 y/o G1P1 21 y/o G1P1 Prenatal History: unremarkablePrenatal History: unremarkable Natal History: Born at CDNNatal History: Born at CDN

SROM - clear amniotic fluid, unknown amountSROM - clear amniotic fluid, unknown amountNSVD, vertex presentation, APGAR 9,9NSVD, vertex presentation, APGAR 9,9

HistoryHistory

22ndnd hour: feeding was started, + vomiting, hour: feeding was started, + vomiting, tachypneatachypnea

55thth hour of life: persistence of tachypnea hour of life: persistence of tachypnea

Transferred to Thomason via EMSTransferred to Thomason via EMS

Physical ExaminationPhysical Examination VS: T- 99.4; HR- 140; RR-89 VS: T- 99.4; HR- 140; RR-89

BP - 57/33 (MAP 43-47) O2 Sat: 82-92%BP - 57/33 (MAP 43-47) O2 Sat: 82-92%

Alert, and activeAlert, and active

No icterus, no rashesNo icterus, no rashes

Open flat fontanelleOpen flat fontanelle

Eyes, normal shape and size with equal red Eyes, normal shape and size with equal red reflexreflex

Physical ExaminationPhysical Examination

Ears: normally set, no gross anomaliesEars: normally set, no gross anomalies

Nose: nares are patent, intact palateNose: nares are patent, intact palate

Lungs and Chest: anteriorly - clear and equal Lungs and Chest: anteriorly - clear and equal breath sounds; breath sounds; posteriorly - slightly posteriorly - slightly decreased breath sounds on the left decreased breath sounds on the left hemithorax, ICS retractions, mild tachypneahemithorax, ICS retractions, mild tachypnea

Chest: normal rate and rhythm, pulses equal Chest: normal rate and rhythm, pulses equal on all extremities, no murmuron all extremities, no murmur

Abdomen: umbilical stump: clean, with 3 Abdomen: umbilical stump: clean, with 3 vessels, soft, non tender, no organomegaly, vessels, soft, non tender, no organomegaly, no masses, normal bowel soundsno masses, normal bowel sounds

Genitalia: normal male genitaliaGenitalia: normal male genitalia

Physical ExaminationPhysical Examination

Back and spine: no gross defectsBack and spine: no gross defects

Limbs and hips: no hip clicks, 10 fingers and Limbs and hips: no hip clicks, 10 fingers and toes, symmetric movements, capillary refill < toes, symmetric movements, capillary refill < 3 seconds3 seconds

Neurologic: normal suck, tone, grasp, cry, Neurologic: normal suck, tone, grasp, cry, Moro, rootMoro, root

Physical ExaminationPhysical Examination

Assessment and PlanAssessment and Plan

Term male infantTerm male infant Suspected sepsis secondary to respiratory Suspected sepsis secondary to respiratory

distress and desaturations distress and desaturations TTN vs PneumoniaTTN vs Pneumonia

Plan: Sepsis work up, CXRPlan: Sepsis work up, CXR NPO, Isolette, OxygenNPO, Isolette, Oxygen Ampicillin and GentamicinAmpicillin and Gentamicin

Course in the NICUCourse in the NICU

Initial CXR: density in the left retrocardiac areaInitial CXR: density in the left retrocardiac area

Unlikely pneumonia or atelectasisUnlikely pneumonia or atelectasis

Differential diagnoses: Differential diagnoses: 1.1. Intra or Extra lobar sequestrationIntra or Extra lobar sequestration2.2. Mediastinal neuroblastomaMediastinal neuroblastoma3.3. Broncho foregut malformationBroncho foregut malformation4.4. Neuroenteric malformationNeuroenteric malformation5.5. Intrathoracic kidneys Intrathoracic kidneys

Case discussed with RadiologistCase discussed with Radiologist

CT scan of the thoraxCT scan of the thorax

Ultrasound of abdomen and thoraxUltrasound of abdomen and thorax

Echocardiogram: normalEchocardiogram: normal

Course in the NICUCourse in the NICU

CT scan of the thorax:CT scan of the thorax:

Soft tissue mass with no calcificationsSoft tissue mass with no calcifications No diaphragmatic eventration, no evidence of No diaphragmatic eventration, no evidence of

herniahernia Differentials: Teratoma, NeuroblastomaDifferentials: Teratoma, Neuroblastoma

Course in the NICUCourse in the NICU

Sonogram of the abdomen: normal gas pattern, Sonogram of the abdomen: normal gas pattern, liver pancreas and kidneys-within the cavityliver pancreas and kidneys-within the cavity

Sonogram of the thorax: Sonogram of the thorax: Left hemithorax mass Left hemithorax mass Arterial supply from the aortaArterial supply from the aorta Vein drainage-origin not establishedVein drainage-origin not established

Consider: Consider: Extralobar SequestrationExtralobar Sequestration

Course in the NICUCourse in the NICU

44thth hospital day: referral to pediatric hospital day: referral to pediatric surgerysurgery

Images were reviewedImages were reviewed

Plan: removal of the pulmonary massPlan: removal of the pulmonary mass

Course in the NICUCourse in the NICU

55thth hospital day: Surgery (Thoracotomy) hospital day: Surgery (Thoracotomy)

Intra-op findings: Intra-op findings: Pulmonary sequestrumPulmonary sequestrum Arterial aspect supplying the mass emerges from the Arterial aspect supplying the mass emerges from the

thoracic aortathoracic aorta Venous drainage into the intercostal veinVenous drainage into the intercostal vein Resection of pulmonary sequestrationResection of pulmonary sequestration

Course in the NICUCourse in the NICU

Chext X-ray Post-op

Uncomplicated recoveryUncomplicated recovery

Patient was discharged on 6Patient was discharged on 6thth post-op day post-op day

Post- operative CoursePost- operative Course

Pathological FindingsPathological Findings

It consists of a 6.5 x 4.5 x 3.0 cm lower lobe of lung It consists of a 6.5 x 4.5 x 3.0 cm lower lobe of lung tissue. It has an attached purple tan cystic structure tissue. It has an attached purple tan cystic structure filled with hemorrhagic serous fluid and air. This filled with hemorrhagic serous fluid and air. This cystic structure measures 1.5 cm in greatest cystic structure measures 1.5 cm in greatest diameter.diameter.

Diagnosis: pulmonary sequestration with minimal Diagnosis: pulmonary sequestration with minimal interstitial lymphocytic inflammation dilated interstitial lymphocytic inflammation dilated congested blood vessels and focal parenchymal congested blood vessels and focal parenchymal hemorrhage.hemorrhage.

Differential diagnosesDifferential diagnoses

PneumoniaPneumonia Diaphragmatic herniaDiaphragmatic hernia TeratomaTeratoma NeuroblastomaNeuroblastoma CCAMCCAM

Extralobar Pulmonary SequestrationExtralobar Pulmonary Sequestration

Non-functioning lung tissueNon-functioning lung tissue Aberrant blood supply- systemic circulationAberrant blood supply- systemic circulation Has its own pleuraHas its own pleura Associated with other anomaliesAssociated with other anomalies CongenitalCongenital

EXTRALOBAREXTRALOBAR CongenitalCongenital Systemic blood supplySystemic blood supply Systemic venous drainageSystemic venous drainage Own pleuraOwn pleura

INTRALOBARINTRALOBAR Acquired/ congenitalAcquired/ congenital Systemic blood supplySystemic blood supply Pulmonary venous drainagePulmonary venous drainage Pleura shared with adjacent Pleura shared with adjacent

normal lungnormal lung

Extra vs IntralobarExtra vs Intralobar

Extra vs IntralobarExtra vs Intralobar

EpidemiologyEpidemiology

Incidence: 0.15-1.7%Incidence: 0.15-1.7% 15-25%- extralobar15-25%- extralobar 75-85%- intralobar75-85%- intralobar 4:1 male to female 4:1 male to female 60%- first 6 months of life60%- first 6 months of life

EmbryologyEmbryology

Prenatal Diagnosis and TreatmentPrenatal Diagnosis and Treatment

No hydrops- follow closely with ultrasoundNo hydrops- follow closely with ultrasound

With hydrops- thoracoamniotic shuntWith hydrops- thoracoamniotic shunt

Postnatally- resectionPostnatally- resection

DiagnosisDiagnosis

Plain x-ray- triangular or oval shape opacityPlain x-ray- triangular or oval shape opacity CT- cystic componentCT- cystic component Color Doppler ultrasound- anomalous Color Doppler ultrasound- anomalous

vesselsvessels MRI- venous drainageMRI- venous drainage

TreatmentTreatment

Surgical resectionSurgical resection

ThoracoscopyThoracoscopy

Arterial embolizationArterial embolization

ComplicationsComplications

HemorrhageHemorrhage

EmpyemaEmpyema

HemothoraxHemothorax

HemoperitoneumHemoperitoneum

Air leakAir leak

Bronchopleural fistulaBronchopleural fistula

Wound infectionWound infection

AtelectasisAtelectasis

InfectionsInfections

Secretions Secretions managementmanagement

Respiratory failureRespiratory failure

PrognosisPrognosis

With hydrops fetalis- dismalWith hydrops fetalis- dismal

Without hydrops - excellentWithout hydrops - excellent

Baby boy M is presently being followed in Baby boy M is presently being followed in our high risk clinic, growing and our high risk clinic, growing and developing without significant pulmonary developing without significant pulmonary residuals.residuals.