neonatal respiratory distress- surgical perspective

L/O/G/O

Neonatal Respiratory Distress

“Surgical Perspective”

Neonatal Respiratory Distress

“Surgical Perspective”

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Dr.B.SELVARAJ

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Neonatal Respiratory DistressNeonatal Respiratory DistressNeonatal Respiratory DistressNeonatal Respiratory Distress “Surgical Perspective”“Surgical Perspective”“Surgical Perspective”“Surgical Perspective”

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Dr.B.SELVARAJ MS;Mch;FICS;

• PEDIATRIC SURGEON

• ASSOCIATE PROFESSOR

• MELAKA MANIPAL MEDICAL COLLEGE

• MELAKA- 75150

• MALAYSIA

Neonatal Respiratory Distress

Recognise various conditions

Make early& accurate diagnosis

Prompt Life Saving treatment

Immediate surgical referral 4

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Surgical Perspective

Objectives

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A NeonateA NeonateA NeonateA Neonate’s request to Surgeons request to Surgeons request to Surgeons request to Surgeon

“Please exercise the greatest gentleness with my

diminutive tissues and try to correct the deformity at first operation; give me blood and proper amount of fluid and electrolytes; add

plenty of oxygen to anesthesia, and I will show you that I can tolerate a terrific amount of

surgery. You will be surprised at the speed of my recovery, and I shall be grateful to you”

--Dr. Willis Potts

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Neonatal Respiratory DistressNeonatal Respiratory DistressNeonatal Respiratory DistressNeonatal Respiratory Distress— Surgical CausesSurgical CausesSurgical CausesSurgical Causes

Causes

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E

C

D

A Esophageal

Atresia

Diaphragmatic Hernia

Congenital

Lobar

Emphysema

Posterior

Choanal

Atresia

Pierre

Robin

Sequence

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Embryology Of Esophageal Atresia

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Esophageal AtresiaEsophageal AtresiaEsophageal AtresiaEsophageal Atresia

EA

Challenging&

Fascinating Problem

Team Work

Approach

Post op Ventilator

Care

VACTERL

Anomaly

Incidence 1 in 3500

livebirths

Epitome of Modern Surgery

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Esophageal AtresiaEsophageal AtresiaEsophageal AtresiaEsophageal Atresia TypesTypesTypesTypes

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Esophageal AtresiaEsophageal AtresiaEsophageal AtresiaEsophageal Atresia---- Associated AnomaliesAssociated AnomaliesAssociated AnomaliesAssociated Anomalies

Vertebral

Anorectal

Cardiac- commonest

Tracheo

Esophageal Fistula

Renal

Limb

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Esophageal AtresiaEsophageal AtresiaEsophageal AtresiaEsophageal Atresia Clinical FeaturesClinical FeaturesClinical FeaturesClinical Features

Clinical

Features

Drooling of saliva

Maternal Polyhydramnios

Inability to pass NGT

into Stomach

In atresia with TEF���� Aspiration

of gastric contents

����Chemical Pneumonitis

Feeding ���� Cough,

choking &

Cyanosis

In pure atresia����

Gasless Abdomen

���� Scaphoid Abd

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Esophageal AtresiaEsophageal AtresiaEsophageal AtresiaEsophageal Atresia----Drooling of salivaDrooling of salivaDrooling of salivaDrooling of saliva

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Physiological Effect of Distal TEF

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• 1. Hyaline membrane disease may necessitate higher ventilator pressures, which encourage air to pass through the distal fistula.

• 2. A distended abdomen elevates and "splints" the diaphragm.

• 3. Gastric distension may result in gastric rupture and pneumoperitoneum.

• 4. Passage of air through a distal tracheoesophageal fistula diminishes the effective tidal volume.

(B) 1. Aspiration of gastric juices leads to soiling of the lungs and pneumonia

• 2. Gastroesophageal reflux

• 3. Direction of gastric fluid proximally through distal fistula.

• 4. Overflow of secretions or inadvertent feeding may contribute to aspiration and contamination of the airway.

Esophageal Atresia

Imaging Studies

AXR����Gasless in pure Atresia

CXR����Atelectasis&Pneumonia Antenatal MRI of Fetus

USG Abd����to R/O Urogenital anomaly

Echo to R/O cardiac

anomaly&Rt Aortic

arch

AXR &CXR����Curledup NGT in blind upper pouch

Imaging

Studies S

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Esophageal Atresia

Antenatal MRI

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Esophageal Atresia

Clinical Diagnosis

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• . (A) Diagnosis of

esophageal atresia is

confirmed when a 10-

gauge (French)

catheter cannot be

passed beyond 10 cm

from the gums. (B) A

smaller-caliber tube is

not used because it

may curl up in the

upper esophageal

segment, giving a false

impression of

esophageal atresia.

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Esophageal Atresia

CXR

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Esophageal Atresia With

TE Fistula- Bronchoscopy

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TE Fistula

Rt Bronchus

Esophageal Atresia

Pre op Management

Pre op Proximal pouch

Decompression

NPO

If for staged repair����

Do Gastrostomy

Head up position

In pure atresia����

Stretch proximal pouch

daily

I V Antibiotics S

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Esophageal Atresia

Pre op Management

Pre op Proximal pouch

Decompression

NPO

If for staged repair����

Do Gastrostomy

Head up position

In pure atresia����

Stretch proximal pouch

daily

I V Antibiotics S

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Esophageal Atresia

Waterston’s Risk Categories

●Birth weight >2.5 Kgs

●No Anomalies

●No Pneumonitis

●Primary Repair����100%survival

●Birth weight 1.8 to 2.5 Kgs

●Non life threatening anomalies

●Mild Pneumonitis

●Delayed Primary Repair����80%survival

●Birth weight < 1.8 Kgs

●Life threatening anomalies

●Severe Pneumonitis

●Staged Repair����40%survival

Risk

Categories

Category

A

Category B

Category C

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Esophageal Atresia

Operative Management

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3

Lanman’s Rt posterolateral retropleural

thoracotomy

Ligation & division of Azygos vein

Disconnect TEF; Repair tracheal defect

4 Liberally mobilise the upper pouch for tension

free anastomosis

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Esophageal Atresia

Operative Management

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In wide gap����Livaditi’s circular myotomies

Never mobilise distal pouch much

Extra pleural drain

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8

Transanastomotic feeding tube for early gavage

feeding

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Esophageal Atresia

Operative Management

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Normal Mediastinum- Rt side

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Esophageal Atresia

Immediate Primary Repair

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Esophageal Atresia

VATS Repair

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Esophageal atresia

Post op Management in NICU

Gastrograffin swallow on 7th POD; If no leak����oral

Feeding & remove chest drain

Feeding through transanastomotic feeding

Tube from 2nd POD

Regular chest Physio&Nasopharyngeal

suction

Otherwise exubate in 1st POD

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Electively paralyse&mechanically ventilate

For 3 to 5 days in tension anastomosis

Esophageal Atresia

Complications

LATE

Tracheomalacia

GE Reflux

EARLY

Anastomotic Leakage

Anastomotic Stricture

Recurrent TEF Esophageal Dysmotility

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Clinical

Features

Operation

Preop

Trt

•VACTERL

•Maternal Poly Hydramnios

•Drooling of saliva in baby

•Inability to pass NGT into stomach

•NPO

•Headup position

•IV Antibiotics

•Upper pouch suction

Complica

tions

Associ

Anomaly EA&

TEF

Esophageal Atresia

TE Fistula���� Recap

Imaging

CXR •Curledup NGT in blind

upper pouch

•Echo to R/O cardiac

Anomaly

• USG Abd to R/O

Urogenital anomaly

•Immediate primary

Repair

•Delayed primary

Repair

•Staged Repair

•Anastomotic leak

•Anastomotic stricture

•Tracheomalacia

•GE Reflux

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Congenital Diaphragmatic Hernia

& Eventration of diaphragm

Herniation of abdominal contents into the thorax through a defect in diaphragm

In Eventration, diaphragm is replaced by a thin membrane

Incidence 1 in 4000 live births

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Congenital Diaphragmatic Hernia

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Congenital Diaphragmatic Hernia

Pathology

Posterolateral Bochdalek Hernia: Commonest; Lt side 80% Rtside 19% Bilateral 1%

Anteromedial Morgagni’s Hernia: 2%

Hypoplastic ipsilateral lung due to compression by herniated viscera

Persistent pulmonary hypertension(PPHN) due to abnormal pulmonary vasculature

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Congenital Diaphragmatic Hernia

Associated Anomalies

Neural tube defects

PDA,VSD,Coarctation of Aorta

Midgut Malrotation

Cleft palate

Exomphalos

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Congenital Diaphragmatic

Hernia����Clinical Features

Respiratory distress����Tachypnea, Cyanosis,Tachycardia,Etc

Scaphoid Abdomen

Mediastinal shift to opposite side

In Lt sided CDH: Pseudodextrocardia

Absence of breath sounds & presence of bowel sounds in ipsilateral hemithorax

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Congenital Diaphragmatic Hernia—

Imaging Studies

Antenatal USG & MRI Chest of the fetus

Postnatal CXR����Presence of bowel shadows in the hemithorax; Mediastinal shift to the opposite side;Diaphragmatic margin is absent

In Eventration����Frontal&lateral CXR show elevated diaphragm with smooth unbroken outline

Flouroscopy:Paradoxical movement of diaphragm in eventration

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Congenital Diaphragmatic Hernia—

Antenatal Fetal USG Chest

Congenital Diaphragmatic Hernia—

Antenatal Fetal MRI

Congenital Diaphragmatic Hernia—

Imaging Studies

Congenital Diaphragmatic Hernia—

Imaging Studies

Diaphragmatic Hernia

Pre op Management

In Utero transfer to a maternity unit close to Pediatric surgical centre����Electively deliver, promptly resuscitate & operate

NGT aspiration to decompress GIT

ET tube ventilation; “No face mask ventilation”

“EXIT”���� Extrauterine Intrapartum Treatment

Correct acidosis with NaHCO3 & Tromethamine

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Diaphragmatic Hernia

Pre op Management

Delayed Repair after preop stabilisation aimed at correcting hypoxia, hypoperfusion& PPHN

Correct PPHN with Tolazoline or Nitric oxide

Pre&post ductal O2 saturation to assess the degree of arterial oxygenation &ductal shunting

In intractable PPHN����ECMO may be tried

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Diaphragmatic Hernia

Bad Prognostic Indicators

Infants born to mothers with Polyhydramnios

Stomach in thorax

Lung Head ratio: LHR < 1

Presentation within first 6Hrs of birth

Very low blood pH & high pCo2

Rt sided hernia

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Diaphragmatic Hernia

Prognostic Indicators

Alveolar-arterial O2 tension difference (AaDo2) :If>500mmHg bad prognosis

Ventilatory Index: VI=RRxMAPxPaco2;When Paco2 is <40mmHg & VI is <1000 all babies survive

O2Index:OI=MAPXFiO2X100/Pao2 OI<6 Survival rate 98% OI>17 No Survivors

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Diaphragmatic Hernia

Operative Management

Diaphragmatic Hernia

Operative Management

Eventration of Diaphragm

Plication

Diaphragmatic Hernia

Post op Management in NICU

Ventilatory support to maintain postductal Po2 80 to 100mmHg;Pco2 < 30mmHg & pH > 7.4

This can be achieved by high frequency ventilation with low airway pressures & low tidal volume

Weaning from ventilator should be meticulous & slow as small variation in pH, Po2& Pco2 will lead to PPHN

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CDH is a congenital anomaly with a high mortality. Usually associated with pulmonary hypoplasia and pulmonary hypertension.

Surgical repair is the only treatment. Delayed surgery until the patient is stable is associated with better outcomes.

Congenital cardiac and renal diseases, hypoxemia and hypercapnia increases mortality.

HFOV, ECMO, iNO has improved the survival of CDH.

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Congenital Diaphragmatic Hernia

Recap

Permissive hypercapnia with acceptable pO2 has shown to improve survival.

Long term follow up is necessary to detect

complications.

Tracheal occlusion in utero, keeps lung expanded

but it is an abnormal lung.

Primary repair if small defect, patch if large defect, to prevent tension

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Congenital Diaphragmatic Hernia

Recap

Congenital Lobar Emphysema

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•Congenital lobar overinflation

–Normal architecture with overdistension of the alveoli

–In true emphysema permanent distension of airspaces distal to terminal bronchiole with

destruction of their walls

•Etiology

–Not found in up to 50%

–Bronchial obstruction found in ~25%

•Allows collapse on exhalation (ball-valve mechanism)

•Air trapping leads to alveolar overinflation

•Intrinsic obstruction (more common)

–Intramural: Defect in the bronchial wall

•Defective quantity or quality of cartilage

–Intraluminal: Lesion in the lumen of the bronchus

•Redundant bronchial folds, mucous plugs

•Extrinsic obstruction

–Compression of the bronchus from a lesion outside the bronchial wall

•Cardiovascular: PDA, vascular sling

•Mass: Lymph node, bronchogenic cyst, oncologic mass

Congenital Lobar Emphysema

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•Males:Females= 3:1

•Upper lobes are predominantly involved

–LUL: 42%

–RML: 35%

–RUL: 21%

–Lower lobes: <1%

–Bilateral involvement: <20%

•Associated congenital malformations: 14-21%

–Congenital heart disease

•PDA

•VSD

–Of those with CHD, >10% have additional anomalies

•Rib cage defects

•Renal defects

Congenital Lobar Emphysema

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•Presentation

–Age

•At birth: 33%

•By 1 m.o.: 50%

•After 6 m.o.: 5%

–Symptoms (in order of decreasing frequency)

•Moderate respiratory distress (most)

•Cyanosis (half)

•Mild respiratory distress (less than half)

•Asymptomatic (infrequent)

•Severe life-threatening distress (least common)

Congenital Lobar Emphysema

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•Radiographic evaluation

–CXR: PA and lateral

•Large, space-occupying air-filled lobe

•Collapse of ipsilateral lung

•Mediastinal shift

•Atelectasis of contralateral lung

•Clear anterior mediastinum

–CT

•May identify point of obstruction, intraluminal or extrinsic

–Ventilation-Perfusion scan

•Decreased perfusion of affected lobe (secondary to vessel

compression)

•Increased perfusion of unaffected lobe (secondary to shunting)

Congenital Lobar Emphysema

Imaging Studies

Congenital Lobar Emphysema

Segmental Resection

Lobectomy

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Pierre-Robin Sequence

Micrognathia/ Retrognathia

Glossoptosis

High arched Palate and/or Cleft Palate

Occur sporadic or syndromic���� Stickler’s & Velocardiofacial syndromes

Respiratory distress aggravated during feeding

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Pierre-Robin Sequence- Grading

Grade1: Eats & breaths well in supine position

Grade 2: Breaths well but obstructs when fed by mouth

Grade 3: Cannot breathe or eat without obstruction and desaturation

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Pierre-Robin Sequence

Most neonates responds to 2-3 months of conservative treatment

Nursing the baby in prone position with head tilted to one side prevent tongue fall back

Feeding the baby in lateral position with jaw pushed forward

Rarely in severe cases may require anterior fixation of the tongue����Anterior Glossopexy

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Pierre-Robin Sequence

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Posterior Choanal Atresia

Absence of posterior nasal openings; Atresia can be a membrane or bony overgrowth

Common in Rt nostril

Neonates are obligatory nasal breathers; in bilateral cases���� “ Cyclical Cyanosis”relieved by crying

Respiratory distress aggravated during feeding

Difficulty in feeding & failure to thrive

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Posterior Choanal Atresia

Failure to pass NGT through nostrils

CT Skull ����Reveal bony block unilateral or bilateral

Respiration is not a problem in unilateral cases but it is in bilateral cases���� oral airway

Feeding by a spoon or a long nipple directly into the pharynx

Transpalatal or transnasal surgical correction

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Posterior Choanal Atresia

Associaed Anomaly

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Coloboma and/or CNS abnormalities

Heart abnormality

Atresia choanae

Retardation of growth

Genital defects (males)

Ear anomalies/deafness

Posterior Choanal Atresia

Posterior Choanal Atresia

Posterior Choanal Atresia

Posterior Choanal Atresia

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With the advent of miniaturized endoscopic equipment and powered instrumentation, the most popular and successful method over the past decade has been the endoscopic transnasal technique.

NEONATAL RESPIRATORY

DISTRESS����Surgical Perspective Sl No Symptoms Signs Workup Diagnosis Treatment

1

Attacks of

choking,

coughing and

cyanosis during

feeding

Drooling of

saliva

Failure to pass

NGT into the

stomach

CXR:Curled

up NGT in

upper

esophagus

AXR:Gasless

abdomen in

pure atresia

Esophageal

Atresia with or

without TEF

•Primary

repair

•Delayed

Primary repair

•Staged repair

2

Tachypnea

Tachycardia

Cyanosis

Scaphoid

abdomen

Mediastinal

shift

Bowel sound in

the hemithorax

CXR:Bowel

loops in

hemithorax

Mediastinal

shift

Congenital

diaphragmatic

hernia/

Eventration

Repair/

Plication of

Diaphragm

NEONATAL RESPIRATORY

DISTRESS����Surgical Perspective Sl No Symptoms Signs Workup Diagnosis Treatment

3

Cough,

tachypnea and

breathlessness

Shift of trachea/

Mediastinum

Area of hyper

resonance

Decreased

breath sounds

CXR:Mediastin

al shift

Hyperlucent

area of lung

Adjacent

normal lung

Congenital

Lobar

Emphysema

Bronchoscopy

if needed

Lobectomy/

Segmental

resection

4

Respiratory

distress

aggravated

during feeding

Micronathia

Glossoptosis

Cleft palate

-----

Pierre-Robin

sequence

Nurse baby in

prone position

Anterior

glossopexy

NEONATAL RESPIRATORY

DISTRESS����Surgical Perspective Sl No Symptoms Signs Workup Diagnosis Treatment

5

Respiratory

distress

aggravated

during feeding

“ Cyclical

cyanosis”

Failure to pass

a catheter

through the

nostril

CT Skull�

Bony block

unilateral or

bilateral

Posterior

Choanal

Atresia

Surgical

correction

Transnasal or

Transpalatal

approach

“ Practice without theory is blind”

“Theory without practice is sterile”

CARRY HOME MESSAGE

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“EYES CANNOT SEE WHAT

THE MIND DOES NOT KNOW”

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