APPROACH TO NEONATE WITH ACUTE RESPIRATORY DISTRESS

ABNORMAL LUNGS BY CXRAY

YES NO

COMMON UNCOMMON AIRWAY Perfusion Neuromuscular

FINDINGS BP CVS findings
RDS HCT Abdominal findings
TTN DIAPHRAGMATIC HERNIA
PULMONARY ASPIRATION SYNDROMES TEF CHF
PNEUMOTHORAX CYSTS AND TUMORS Anemia Persistant fetal circulation
Upper Airway CCHD
PULMONARY EDEMA CONGENITAL LOBAR EMPHYSEMA Polycythemia
PLEURAL EFFUSION PULMONARY HYPOPLASIA Laryngeal Hypotension ASPHYXIA
PULMONARY HAEMORRHAGE Lower Airway Hypovolemia ICH
SEPSIS
ACIDOSIS
HYPERTHERMIA
HYPOTHERMIA
 ANATOMY OF RESPIRATORY TRACT

ANATOMICAL FUNCTIONAL

URT LRT CONDUCTING PART RESPIRATORY PART

• NOSTRILS • TRACHEA • URT • RESPIRATORY

NASAL CAVITY • BRONCHI BRONCHIOLES
• • +
• TERMINAL BRONCHIOLES
• PNS • UPTO TERMINAL • ALVEOLAR DUCTS
• RESPIRATORY
• PHARYNX BRONCHIOLES BRONCHIOLES • ALVEOLI
• LARYNX • ALVEOLI

CLEANING AND
WARMING AND EXCHANGE
MOISTENING OF
AIR
 DEVELOPMENT OF RESPIRATORY SYSTEM

A laryngotracheal groove is developed at 4th week of IUL.

By 5th week extrapulmonary bronchi develops by the division

of laryngotracheal groove

LARYNX is the cranial most part of LTG.

Lung buds when gets in contact with the respective

pericardioperitoneal canal , filled with gradually expanding
bronchi/ lung buds

The right and the left bronchi are divided into lobar bronchi.

By 7th week ,each lobe is divided into segmental bronchi

A ventral outpouching from the The conducting airways continue to branch and the
endodermal epithelium of bronchial bud resembles a gland .
VIABLE
primitive foregut (bronchial bud) Trachea and primitive foregut are seperated by
interacts with tissue of progressive fusion of epithelial ridges and similar MAY
mesodermal origin process occurs between central tendon and SURVIVE
pleuroperitoneal folds
Non viable
 ALARMING SIGNS

RESPIRATORY DISTRESS AT BIRTH

RESPIRATORY DISTRESS NOT SETTLING DOWN

EXCESSIVE FROTHING

CYANOSIS AFTER FEEDS

COUGHING

CHOKING

COILING OF NGT IN MOUTH

UNABLE TO PASS NGT THROUGH NOSTRILS

When a common respiratory problem in newborn is not responding to usual line of management , there is a possibility of
underlying congenital respiratory lesion , and it should be corrected.

Majority of the lesions are of lower respiratory tract and may present as respiratory infection or pneumonia, any time in
childhood.

Congenital Malformations may be related to that of airway or that of lung.

Stage of lung developmement Congenital lesion of lung

Embryonic(3-6 wks) Atresia: Tracheal,Laryngeal,Esophageal
Pulmonary agenesis/tracheal stenosis
TEF
Bronchogenic cyst
Pulmonary sequestration(extralobar)
Pseudoglandular(6-16wks) CPAM
Diaphragmatic Hernia
Pulmonary sequestration(intralobar)
Canalicular(16-26wks) Pulmonary hypoplasia
Saccular(26-36 wks) RDS
Alveolar (upto 6 yrs) Congenital lobar emphysema
Bronchopulmonary dysplasia
The diagnostic evaluation of a child with suspected congenital anomalies of lower respiratory tract should begin with :

1) Detailed antenatal history

2) Physical examination
a) symmetry of chest
b)change in resonance
c)air entry

Scaphoid chest- may suggest CDH

Excessive frothing should alert thr possibility of EA

3)Detailed examination to rule out other defects

4) Plain chest radiographs may give a clue about congenital lesions

Findings like a) opacification
b)mass lesion
c)cystic lesions
d)unusual air collection
e)mediastinal shift

Other diagnostic imaging studies such as barium study , CT, MRI can confirm the diagnosis
 TOPIC OF DISCUSSION

1)CHOANALATRESIA

2)TRACHEOESOPHAGEALFISTULA

3)LARYNGEALWEB

4)CongenitalCystadenoidMalformation

5)CongenitalDiaphragmaticHernia
 CHOANAL ATRESIA

It is a narrowing or blockage of the nasal passageway that results in difficulty breathing.

The blockage is due to tissue (10%) or bone (90%) in the nasal airway at birth.

It may affect one or both sides of the nose.

If only one side is blocked , symptoms may go un noticed and surface later in childhood.

FEMALE >MALE
May be associated with CHARGE syndrome

Trouble breathing, cyanosis and may have breathing failure.

1)Pre natal usg

2)CT
3)NASAL ENDOSCOPY

Neonates are obligate nasal breathers.

Making the baby cry will initiate mouth breathing and temporarily improves respiratory status.
Surgery is the only long term treatment.
Endoscopic choanal atresia repair is the most common approach
 IS CHOANAL ATRESIA AN EMERGENCY??

YES
If bilateral immediate surgery might be needed as it is a life threatening condition,

CHOANAL ATRESIA REPAIR

During the repair, surgeon creates a hole through the bone or tissue thats blocking the nasal airway.

This is done through the nose (trans nasal ) or through mouth( transpalatal)

In some cases , a stent is placed inside the babys nasal passage to keep the airway open during
healing.Stents are removed surgically a few months after placement.

PERFORMED UNDER GA
An esophageal atresia is a congenital condition of esophageal discontinuity that results in proximal esophageal obstruction.

A tracheo esophageal fistula is an abnormal fistulous connection between trachea and esophagus

EA/ TEF can occur alone or in combination.

Approximately 1/3rd of infants are born with LBW and 60-70 % have associated anomalies .

During 4th week the esophageotracheal diverticulum fails to completely divide trachea and esophagus.
 TRACHEO ESOPHAGEAL FISTULA
First noticed in the year 1697

INCIDENCE: 1 IN 3000 LB

ETIOLOGY:

Division of foregut happens at 4th and 5th week of IUL

Imperfect division results in a fistula

ASSOCIATIONS : VACTERL

CLINICAL PRESENTATION:
EARLY INDICATORS:

POLYHYDARAMNIOS
COILING OF NGT
CHOKING,CYANOSIS, AND COUGHING ON ORAL FEEDS

BREATHING LEADING TO ABDOMINAL DISTENSION

CLINICAL PRESENTATION:
DEHYDRATION .( Proximal esophagus donot communicate with stomach)
ASPIRATION.(reflux)
Most common type is TYPE C (85%)

TYPE A “Pure” esophageal atresia implies EA without TEF

GROSS ( TYPE A,B,C,D,E)
VOGT (TYPE 2,3A,3B,3C AND 4)
The prenatal diagnosis of EA/TEF relies in principle, on two nonspecific signs: polyhydramnios and absent or
small stomach bubble. Polyhydramnios is associated with a wide range of fetal abnormalities, but most
commonly it pursues a benign course. Similarly the sonographic absence of a stomach bubble may point to
a variety of fetal anomalies.

DIAGNOSIS:
A NGT that cannot be passed beyond 10-12 cm from nares
Chest xray shows the tube ending in thoracic inlet
The upper pouch can be better visualized by insufflating 20-30 ml air into the tubes as the radiograph is
being taken
The radiograph should also be examined for skeletal anomalies, pulmonary infiltrates,cardiac size and
shape, abnormal bowel gas patterns
 MANAGEMENT:
A) PRE OPERATIVE TREATMENT:
Protecting lungs by evacuating the proximal esophageal pouch with an indwelling Replogle tube or frequent
suctioning.

Placing the baby in an upright position lessens the likelihood of reflux of gastric contents upto distal esophagus into
trachea.

Broad spevtrum antibiotics

Routine ETT is avoided because positive pressure ventilation may be inadequate to inflate lungs as air may be
directed into TEF

2d echo to rule out VACTERL

TEF REPAIR
1) CONVENTIONAL
2)THORACOSCOPIC METHOD

The surgical approach for most common type of Proximal EA with distal TEF is extrapleural dissection via open thoracotomy.

In infants with pure esophageal atresia , a primary anastomosis is not feasible in the newborn period , therefore a gastrostomy tube is placed initially for enteral
feeding and then a cervical esophagostomy is performed for drainage of oral secretions, and then a esophageal replacement operation is performed at 1 year of
age using right or left colon.

1) Involves surgical division of fistula and esophageal anastomosis via right pleural thoracotomy with patient in left lateral position.
 DEVELOPMENT OF LARYNX

Larynx develops from the cranial most part of LTG

 LARYNGEAL WEB

LARYNX is developed from the superior most part of laryngotracheal groove

Due to incomplete recanalization of larynx , the condition occurs.

Mostly anterior with subglottic involvement

75% at glottic level

PRESENTATION:
Airway obstruction, weak cry, aphonia since birth.

SIGNS AND SYMPTOMS:

Frequent shortness of birth, stridor, poor feading

DIAGNOSIS:
FLEXIBLE LARYNGOSCOPY( microlaryngoscopy)

WEB can be of two types : THICK AND THIN

THIN: C02 laser or cut with knife

THICK: excision via laryngofissure and placement of silicon keel and subsequent dilatation..
Congenital Pulmonary Airway Malformation( CPAM) previously known as Congenital Cystadenoid Malformation (CCAM) is a rare pulmonary disorder,

The pathogenesis of CPAM is uncertain.

It may be a result of abnormality of branching morphogenesis of lung.

The abnormal hamartomatous CPAM proliferation usually retains its communication with normal bronchiolar tree but doesnot participate in normal gas
exchange.

CPAM is almost always unilateral(85-95%)

CPAM type Features Prognosis

0 Involves all lobes Incompatible with life

1 Macrocystic( 2cm and above) lined by pseudostratified columnar 50% of postnatal cases with
favourable prognosis

2 Microcystic( 2cm and less)lined by cuboidal or columanar higher congenital anomalies are
associated
( 40%)

3 predominantly small lesiosn lined by cuboidal epithelium poor prognosis

4 large air filled cysts lined by flattened epithelial cells

 CCAM

CCAM is a benign lung lesion that appears before birth as a cyst or mass in the chest.

It is made up of abnormal lung tissue that doesnot function properly, but continues to grow.

TWO types of lesions are present MICROCYSTIC and MACROCYSTIC.

Some of the lesions are life threatening , if not treated ,

Some of CCAM are small enough that they will not cause any problem to the baby during pregnancy, and can be removed after birth.

HOWEVER, some large lesions can cause severe fatal complications like hydrops
These conditions may require treatment before birth
 MANAGEMENT OF CCAM DURING PREGNANCY

In rare circumstances , the lung mass grows, and take up the valuable space in the chest. This can restrict normal lung growth and can lead to
underdeveloped lungs which will not function adequately at birth.
Large lesions can also shift the heart and leads to hydrops fetalis.

:
We have a large number of treabefore birth to decrease the size of ccam or cpam and reduce the chance of fetal heart failure.

, to prevent the growth of the lesion.: The steroids may be used to interrupt the growth of the lesion. Using steroids to slow down growth
of ccam can prevent the need for fetal surgery.

2 fluid from the cyst: Fluid filled macrocystic lesions can be drained prenatally to remove fluid and reduce the size of mass
the fluid may accumulate , in which case a shunt has to be placed .
The goal is to divert fluid from cyst to amniotic sac . The shunt remains until delivery

If the baby not responding to other prenatal treatments and showing early signs of failure . A fter surgery , the fetus is returned to the womb where the
lung can continue to grow.

4) :
Special procedure which allows fetal surgeons to establish an airway and remove the CCAM. After the mass is removed and baby can breathe , they
will be delivered .fully. This technique is required when the lungs are compressed and baby cant breathe on their own.
 TYPES OF CCAM
DEVELOPMENTAL DEVELOPMENTAL EVENT STOCKER CLASSIFICATION
STAGAE
EMBRYONIC Formation of tracheal bud and branching to segmental bronchi O- TRACHEOBRONCHIAL
1-BRONCHIAL/BRONCHIOLAR
PSEUDOGLANDULAR completion of airway branching to terminal bronchioles 2-BRONCHIOLAR

CANALICULAR formation of respiratory bronchioles to prealveolar structures 3-BRONCHIOLAR/ALVEOLAR DUCT

SACCULAR formation of secondary septae 4-DISTAL ACINAR

CPAM type Features Prognosis

0 Involves all lobes Incompatible with life

1 Macrocystic( 2cm and above) lined by pseudostratified columnar 50% of postnatal cases with
favourable prognosis

2 Microcystic( 2cm and less)lined by cuboidal or columanar higher congenital anomalies are
associated
( 40%)
3 predominantly small lesiosn lined by cuboidal epithelium poor prognosis
4 large air filled cysts lined by flattened epithelial cells

TYPE 1CCAM is most common lesion in postnatal series of airway malformation

. Cysts are larger than 2cm and usually limitted in number. There are limitted to single lobe in 95% of the cases
 SURGERY OF CCAM AFTER BIRTH

Most common presentation of CCAM is after birth in the post natal period as progressive respiratory distress
with grunting, retractions, and cyanosis.

Most lesions can be successfully treated with surgery after birth, even in asymptomatic individuals to prevent infection and
avoid potential malignant transformation of lesion.
CT with contrast will be performed to confirm the diagnosis and location of the lung lesion.
 CONGENITAL DIAPHRAGMATIC HERNIA

CDH is a life threatening anomaly that can be diagnosed prenatally.

The physiological derangements associated with CDH in the form of pulmonary hypoplasia and pulmonary hypertension need expert
management by skilled neonatologist both before and after surgery.

ETIOLOGY: Exact etiology is unknown, It is believed to be multifactorial with interplay of genetic as well as environmental factors.

Occurs when the diaphragm has a defect and the contents from the abdomen goes to the chest, impacting the growth and
development of lungs,

The lungs will be smaller than expected ( pulmonary hypoplasia) and will have less developed blood vesels. This causes pulmonary
hypertension.

INCIDENCE: 1 in 2500-3000 LB

L>R

SIGNS & SYMPTOMS

Typically discovered during a routine prenatal ultrasound.
one may typically notice , stomach, intestine, or liver in the chest where the lungs should be
The babys heart may be pushed to one side by extra organs in chest.

HIGH RESOLUTION FETAL ULTRASOUND

U;LTRAFAST FETAL MRI
FETAL ECHOCARDIOGRAM
 DEVELOPMENT OF DIAPHRAGM
Fetal diaphragm develops in 7th -10th week of gestation by fusion of pleuroperitoneal membranes with septum
transversum of dorsal mesentery of esophagus thus closing the pleuroperitoneal canal.

Any interfernce in this process results in CDH.

The nitrofen model speculates CDH is an embryopathy caused by toxin exposure in early gestation.

The diaphragm seperates the pleural cavities from the peritoneal cavity.

The diaphragm is formed through the fusion of tissue from four different sources:

1) SEPTUM TRANSVERSUM - MESODERM- PRIMORDIUM OF CENTRAL TENDON OF DIAPHRAGM in adult

2)PAIRED PLEUROPERITONEAL MEMBRANE - SOMATIC MESODERM- DORSAL AND DORSOLATERAL BODY WALL

3) DORSAL MESENTERY OF ESOPHAGUS- CRURA OF DIAPHRAGM

4)BODY WALL PERIPHERAL PORTIONS OF DEFINITIVE DIAPHRAGM

 PATHOPHYSIOLOGY
A)PRENATAL:
a) Abnormal communication between the peritoneal and pleural cavities allows herniation of intestines into the pleural space as the
developing gastrointestinal tract returns from its extracoelomic phase at 10-12 weeks of gestation.
b)The heart and the mediastinum are shifted to opposite side thus compressing the contralateral lung.
c)Dimnished intrathoracic space affects the pseudoglandular phase of lung development during first trimester and results in bilateral
pulmonary hypopalsia, which is more severe on ipsilateral side .
d) There is inadequate surface area for gaseous exchange , decreased croos sectional area of pulmonary vessels and probable
surfactant deficiency.
e) Raised level of circulating vasoactive mediators , along with an increased sensitivity to these mediators , further causes pulmonary
hypertension.

B)POST NATAL:

With the initiation of newborns first breath , fetal circulation is converted to adult type due to fall in PVR.
Failure of this process occurs in CDH patients leads to persistant fetal circulation, with increased right to left shunt
This causes fall in saturation of systemic blood circulation and hypoxia , which further increases PVR .
A vicious cycle develops and leads to respiratory failure
 CLINICAL PRESENTATION:

It depends on degree of pulmonary hypoplasia and degree of PPHN .

Most patients develop respiratory distress either at birth or within first 24 hrs

Milder forms may present by 5-7 days.

Other Presentations:
Antenatally diagnosed CDH
Respiratory distress at birth associated with cyanosis , hypoxia , tachycardia , abnormal bulge of hemithorax on affected sides,
mediastinal shift to opposite sides as seen by abnormal cardiac impusle, bowel sounds heard over hemithorax instead of
breath sounds or scaphoid abdomen

An infant with Morgagni or right sided hernia may be asymptomatic at birth and may present later with respiratory or GI
symptoms
ASSOCIATED ANOMALIES :

In about 40% of the cases other anomalies are present

Most common is Congenital Heart Defects .
Other anomalies include _ TOF , HLHS , COA ,etc

DD:
CCAM
 TYPES OF CDH
TYPE BOCHDALEK MORGAGNI

DEFECT Posterolateral Anterior

% 95 2

Defect on left side Defect on right side

Left lobe of liver, spleen , stomach , Right lobe of liver along with intestines
varying lengths of small and large occupy the hemithorax
intestine and rarely left kidney
obstruction of GE junction due to Abnormal drainage of hepatic veins
displacement of stomach leading to directly into right atrium may cause
polyhydramnios. difficulty in surgical repair.
 ANTENATAL DIAGNOSIS:

The prescence of stomach bubble or fluid filled intestinal loops in the fetal hemithorax at the same cross sectional level as the heart is
suggestive of CDH

Mediastinal shift to opposite side and prescence of liver in thorax also helps in diagnosing CDH.

Associated POlyhydramnios is seen in 80% of cases.

These features are evident around 18 weeks of gestation at the level of Morphology scan,

However mean age of detection of CDH is around 24 weeks.

FETAL ECHO :

To confirm and evaluate concurrent heart disease .

3d US has been used to confirm antenatal diagnosis and accurately assess perinatal outcome

POST NATAL DIAGNOSIS:

Plain xray of chest and abdomen shows abscence of diaphragmatic dome on affected side with herniation of bowel loops into hemithorax in addition
to mediastinal shift

Post natal 2d echo to rule out other cardiac anomalies and PPHn.

Measurement of Preductal ABG is a corner stone for attempting to establish clinical predictive criteria in CDH.
 DETERMINING SEVERITY OF CDH BEFORE DELIVERY

The strongest predictor of severity is the location of liver., when the liver is more up in the chest, there is more pulmonary hypoplasia

The degree of hypoplasia can be predicted by measurengts obtained during the fetal ultrasound and MRI .

1) Lung area to head circumference ratio ( LHR)

2)Lung area to head circumference observed to expected ( LHR O/E)
3)TOTAL LUNG VOLUME observed to expected (MRI O/E)

PERINATAL STABILIZATION

The indicators for fetal stabilization are LTR of less than 0.2 in the abscence of any severe associated anomalies in near term fetus of atleast 36 weeks of
gestation

PROTOCOL

1) Monitoring of fetal respiratory movement and heart beat by USG

2)Administration of morphine and diazepam to mother
3)Confirmation of interruption of any fetal movements is followed by CS
4)Administration of muscle relaxant through umbilical vessels
5)Intubation before clamping the umbilical cord
6)HFOV without bagging
 POST NATAL RESUSCITATION

A neonate with suscpected or antenatally diagnosed CDH should be delivered at a tertiary care centre where facilities of HFVO , iNO , ECMO are
readily available and neonatal surgical expertise is at hand.

Bag and Mask ventilation is contraindicated during resuscitation of neonates as it can cause distension of the stomach and intestines with air and
cause further respiratoty compromise.

The new born should be intubated in the delivery room

NG TUBE: Should be placed to lessen the gaseous distension of stomach and intestine .
Arterial and venous catheters should be placed , and baseline investigations including preductal ABG, CXRAY ,ECG shoud be organized
Different ventilator modalities to maintain a Pao2 > 85 and Pco2 < 50 mmhg should be done.

D)SURGICAL CORRECTION:
Reduction of intrathoracic intestine and closure of diapghramatic defect.
E)ECMO: Used in treatment of neonates with severe respiratory failure.. Exposure of venous blood to ECMO circuit allows correction of pc02 and
po2 abnormalities as the lungs recover from trauma associated with ppv.

PROGNOSIS
Mortality rates for infants with CDHstill in range of 50%

FETAL SURGERY: In utero intervention will lessen the risk for development of pulmonary hypoplasia

MEDICATIONS: SILDENAFIL
 SURGICAL CORRECTION:

TIMING OF SURGERY:

Possibilities of surgery are :

1) In Utero
2)Emergent ( within 2-4 hrs after birth or arrival of patient)
3)Urgent (within 24 hrs of birth or arrival)
4)Delayed

OPERATIVE TECHNIQUE:

Infant with CDH not requiring ECMO can safely undergo operative repair soon after birth
Laproscopic repair of CDH has gained popularity in recent years , but overall benefits and effects on long term outcome remain uncertain.

A recent report suggested that CDH repair after ECMO therapy is associated with improved survival compared to repair while on ECMO .

The preferred approach for a posterolateral CDH is through subcoastal abdominal incision.
The defect may be very large sometimes a , prosthetic material like Gore-Tex patch is used because of lesser operative time and tension -free repair.
A chest tube is kept insitu and post operative radiographs shows immediate mediastinal shift towards the centre .
The chest cavity quickly fills with serous drainage , which later gets absorbed as fluid status is returning back to normal.
COMPLICATIONS:

Recurence of CDH is seen in 10-50% of cases with agenesis of diaphragm requiring prosthetic patches and is difficult to manage surgically.

Long term complications:

FTT
CPD
NDD
Esophageal Dysmotility and Reflux is seen in 50% of patients.

OUTCOME AND PROGNOSIS:

Mortality rate is still high despite of great advancements ( 30-70%)

POOR PROGNOSTIC FACTORS:

 Prenatal diagnosis < 25 weeks
 Coexisting CHD
 Fetal LHR <1.0
 Fetal LTR <0.2
 NAKATA INDEX: Right lung area/ Thoracic Area less than 0.11
 Prescence of polyhydramnios
 Diaphragmatic Agenesis requiring prosthetic Mesh
 Herniation of stomach into Hemithorax
 Right sided CDH
 Prematurity
 LBW
 Need for ECMO
HONEYMOON PERIOD :

Transient stable interval during which adequate oxygenation (Pa02 > 100 mmhg + PCO2 < 50mmhg ) is maintained with maximal conventional
ventilatory and supportive therapy.

This phenomenon is usually seen in first 48hrs of life and has been known to last for 24 hrs after which the subjects deteriorate because of PPHN and left
to right shunt.

DIFFERENT MODES OF VENTILATION USED IN CDH

Mode of ventilation Characteristics Advantagaes Disadvantages

CMV(conventional High PIP <25cm H20, high rates , target pa02 > simple routine principles High PIP associated with VILI
mechanical ventilation) 85% can be done in smaller setups barotrauma , pneumothorax

Permissive hypercapnea Maintain Pac02 of 40-50 mmhg Less VILI(ventilator induced lung Respiratory Acidosis
injury)

HFOV(high frequency oscillations at frequency of 3-15hz Maintains PEEP without VILI Needs speciliazed equipment
oscillary ventilation)
ITPV(intratracheal Constant flow of humidified gas through reverse Reduces physiological dead space , Needs speciliazed equipment
pulmonary ventilation) venturi catheter kept at distal end of ETT facilitates expiration and improves
elimination of C02

iNO used in conjunction with HFOV Vasodilator of pulmonary vasculature May not be available in smaller
setups
ECMO( Extra corporeal VA or VV bypass(veno arterial / veno venal) Used when no response to all other May not be available in smaller
membrane oxygenation) modes setups
CONDITION CHOANAL ATRESIA TEF/EA LARYNGEAL WEB CCAM CDH
INCIDENCE 1 IN 5000- 1IN 8000 LB 1 IN 3500 TO 1 IN 4500 <1 IN 10000 1 in 10,000LB 1 in4000lb
SEX DISTRIBUTION F>M (2;1) M>F M=F M>F

ASSOCIATED CHARGE VACTERL 22q11 deletion Congenital heart defects Musculoskeletal and cardiac
SYNDROMES/ANOMALIES cardiac anomalies trisomy 18

PATHOPHYSIOLOGY BLOCKAGE OF POSTERIOR Failure of division of esophagus and Anamalous embryological cystic intrathoracic masses caused the embryonic
NARES BY BONE OR TISSUE trachea development of larynx by proliferative terminal respiratory pleuroperitoneal fold which
bronchioles and reduction in number should be fused at 6-8
of alveoli
weeks .this failed fusion leads
to defect.

CLINICAL PRESENTATION IF U/L SYMPTOMS WILL BE copious secretions Altered cry ,stridor,aphonia and Respiratory distress, pre and post
LATE’ respiratory distress ductal sp02 difference ,
severe airway obstruction
IF B/L SEVERE RESPIRATORY cyanosis or aspiration after feeds
DISTRESS OR CYANOSIS

DIAGNOSIS(PRENATAL) Transient enlargement of the polyhydramnios in 1/3rd of - Mediastinal shift due to mass effect mediastinal shift away from
nasal cavity in early pregnancy with EA polyhydramnios hernia side is seen
pregnancy appears to be a US usg showing the same level of fetal mri
stomach and heart.
sign of choanal atresia.

DIAGNOSIS(POST NATAL) Ct is the definite dx Isolated EA without TEF ( gasless cry will be weak or aphonic xray imaging, respiratory
unable to pass the ngt through abdomen) sometimes distress,cyanosis,decreased
nostril Coiling of ngt visual bronchoscopy bronchial breath
sounds,scaphoid abdomen

IMMEDIATE TREATMENT serious cases- oropharyngeal continuous suction Endoscopic approaches with intra uterine and extra uterine decompress abdomen
airway antacids C02 laser treatment o2
endotracheal intubation antibiotics open laryngotracheal ventilation, surgery as soon as ppv
ppv possible
McGovern nipple to overcome reconstruction.
the seal between palate and
tongue

DEFINITIVE TREATMENT Resection of soft tissue or Primary repair of atresia with ligation Resection of web Resection-symptomatic surgery
bony septum(transnasal of fistula
approach)
transpalatal approach

POST OP MANAGEMENT Respiratory support, antibiotics, treatment of pphn

dye study to confirm fistula closure