2. Respiratory Distress
• Respiratory distress is characterized by a child’s
response to inadequate gas exchange in the lungs
resulting from any condition that compromises
oxygenation and ventilation.
3. Signs of respiratory distress
• ↑ Respiratory rate
• ↑ Work of breathing
• Chest indrawing
• Use of accessory m/s of breathing like nasal flaring
• Noisy breathing (granting, wheezing, stridor)
4. Investigations
• Depend on the cause and on available facilities
- Blood glucose
- Arterial Blood Gases
- Chest X ray
- Full septic screen
- ECG and Echo
6. • Checking baselines
• Blood culture
• Fluids and nutrition (60ml/kg/d of 10% dextrose)
• Antibiotics
• Blood glucose level (target >2.6 mmol/L)
• Maintain temperature and humidity
7. • Respiratory support
- Supplemental O2
- CPAP
- Mechanical ventilation
Neonates who cannot be oxygenated by any of these means may require
a full cardiac evaluation to exclude congenital heart disease
16. Principles
• When respiratory failure is present already, urgent treatment is
required, regardless of the underlying cause.
• Accurate diagnosis of the cause is made on the clinical signs and
imaging.
• The degree of respiratory or metabolic acidosis must be
determined as a guide to the resuscitation required.
• Where applicable, surgery is undertaken to correct the cause,
usually after correction of the physiological disturbances.
23. • The most common laryngeal anomaly and cause of stridor in infancy.
• inspiratory stridor that is worse with feeding, agitation, and supine position.
• present at birth or shortly thereafter.
• Symptoms peak at 6 to 8 months and usually resolve between 18 and 24
months of age.
• Difficult for infants to coordinate the "suck-swallow-breathe”.
LARYANGOMALACIA
24. • Self-limiting disease
• Infants with severe laryngomalacia experience life-threatening complications
of airway obstruction.
• Indications for surgery
- Failure to thrive
- Chronic hypoxia
- Pulmonary hypertension
- Cor-pulmonale
LARYANGOMALACIA
25. • Surgery
- Tracheotomy was the treatment of choice until the mid-1980s.
- Now supraglottoplasty was treatment of choice.
LARYANGOMALACIA
27. • The tracheal wall cartilage is soft and pliable
• a common cause of stridor in infancy
• Primary - Unknown etiology
• Secondary- extrinsic compression of a vascular anomaly
- tracheoesophageal fistula
- long-term ventilator support
TRACHEOMALACIA
28. • Bypassing and stenting is the treatment of choice.
• endotracheal stenting with angioplasty
• Palmaz stents
TRACHEOMALACIA
29. • During intra-uterine development, the small bowel,
stomach, spleen and left lobe of the liver pass through
the defect in the diaphragm into the chest, limiting the
space available for the developing lung.
• This causes lung hypoplasia, which in many infants is
severe enough to produce severe respiratory distress
within minutes of birth, and may not be compatible with
life.
CONGENITAL DIAPHRAGMATIC HERNIA
ASSOCIATED PATHOLOGY
30. • Most congenital diaphragmatic hernias are diagnosed well before birth, on
antenatal ultrasonography.
• Factors that may indicate a worse prognosis on antenatal scanning are -
- Early gestational age at diagnosis on ultrasonography
- Lung-to-head ratio (LHR) at 24–26-weeks’ gestation <1.0
- Small fetal lung volume on 3D ultrasonography and MRI
- >50% liver in chest on right side
- Liver in chest on left side
- Stomach and spleen in chest on left side
- No hernial ‘sac’
CONGENITAL DIAPHRAGMATIC HERNIA
ANTENATAL DIAGNOSIS
31. • The majority of infants born with a Bochdalek (posterolateral) diaphragmatic
hernia become symptomatic at or shortly after birth.
• Where pulmonary hypoplasia is severe, the infant becomes cyanosed with
severe respiratory distress within minutes of birth.
• 85% of posterolateral hernias involve the left hemidiaphragm.
CONGENITAL DIAPHRAGMATIC HERNIA
POSTNATAL DIAGNOSIS
32. • The remainder are right-sided (12%) or bilateral (3%), associated anomalies
occur in up to 40%.
• The most common serious abnormalities are heart defects.
• Most anterior (retrosternal) hernias are symptomless unless strangulation
occurs.
CONGENITAL DIAPHRAGMATIC HERNIA
POSTNATAL DIAGNOSIS
33. • CXR
- loops of bowel can be seen in the left chest.
- The heart is deviated to the right.
- Little room is left for the lungs, particularly the left lung which is
markedly compressed.
- the tip of NG tube can be seen in the chest.
• Ba Study
- bowel within the thoracic cavity.
CONGENITAL DIAPHRAGMATIC HERNIA
INVESTIGATION
34. • Diagnosed on antenatally => the infant is transferred to a tertiary paediatric
surgical centre prior to birth.
• This is because these infants may develop severe pulmonary distress very
quickly after birth, making subsequent transfer difficult and potentially
dangerous.
CONGENITAL DIAPHRAGMATIC HERNIA
TREATMENT FOR POSTEROLATERAL (BOCHDALEK)
HERNIA
35. • Initial treatment involves intensive cardiorespiratory support and insertion of
a nasogastric tube to prevent bowel dilatation within the chest.
• Ventilation with a face mask (‘bagging’) should be avoided as this may force
air into the stomach, increasing its volume at the expense of the already
compromised lungs.
• Vigorous endotracheal ventilation should also be avoided because of the risk
of causing barotrauma and a tension pneumothorax
CONGENITAL DIAPHRAGMATIC HERNIA
TREATMENT FOR POSTEROLATERAL (BOCHDALEK)
HERNIA
36. • Definitive surgery is performed when the infant’s condition is stable and this
may be between 12 h and 7 or more days after birth.
• In left-sided defects, a left transverse or subcostal abdominal incision is
used.
• The major cause of death remains pulmonary hypoplasia and pulmonary
hypertension.
• Survival rates about 80%.
CONGENITAL DIAPHRAGMATIC HERNIA
TREATMENT FOR POSTEROLATERAL (BOCHDALEK)
HERNIA
37. • Anterior diaphragmatic (Morgagni) hernias are often diagnosed on an
incidental x-ray of the chest in a symptomless patient, but repair is still
advisable because of the risk of strangulation of the bowel.
• This is usually performed as a laparoscopic procedure.
• The results are excellent.
CONGENITAL DIAPHRAGMATIC HERNIA
TREATMENT FOR ANTERIOR DIAPHRAGMATIC HERNIA
39. • Congenital Cystic Adenomatoid Malformation (CCAM) aka as congenital pulmonary airway
malformation (CPAM)(A spectrum of cystic and solid lesions of the lung identified
histologically)
• there is an overgrowth of terminal bronchiolar-type tubular structures and a lack of mature
alveoli
• Composed of cartilage, smooth muscle, and bronchial glands containing columnar and
cuboidal epithelial cells
• 25% of all congenital lung anomalies (second only to congenital lobar emphysema)
40. • Normal vascular development
• affected area communicates with the normal airway
• Usually only one lobe (a solitary lesion)
42. Cystic adenomatoid malformation
Computed tomography image of the chest shows multicystic
lesions in the same region. Excision and histologic
examination showed cystic adenomatoid malformation
45. • The underlying abnormality is bronchomalacia from congenital deficiency of
the cartilage.
• It results in expiratory obstruction and trapping of air in the affected lobe,
leading to massive distension of a pulmonary lobe.
• The mediastinum is displaced and the chest wall over the affected area is
prominent.
• Present with tachypnea, dry cough, stridor. Cyanosis is usually an indication
for urgent treatment.
CONGENITAL LOBAR EMPHYSEMA
46. • X-rays
- an area of increased radiolucency in
which there are some broncho-vascular
markings.
- There is also downward displacement of
the diaphragm on the affected side, and the
over-distended lung may herniate across the
midline.
• Treatment - Lobectomy
49. • ANTENATAL DIAGNOSIS
- Sometimes oesophageal atresia is diagnosed on antenatal
ultrasonography by the observation of polyhydramnios, a small
stomach or abnormal oesophageal contraction with swallowing.
• EARLY DIAGNOSIS
- Early diagnosis is important as delay may lead to aspiration and
progressive pulmonary complications.
- confirmed when an oro-gastric catheter cannot be passed
through the mouth and oesophagus into the stomach
DIAGNOSIS
50. • SYMPTOMS SOON AFTER BIRTH
- excessive salivation or of being ‘excessively mucousy’ is highly
suggestive of oesophageal atresia
- repeated suction & rattling respirations, tachypnoea or fine frothy
white bubbles of mucus in the nostrils or on the lips.
- history of maternal polyhydramnios
DIAGNOSIS
51. • DIAGNOSIS BEFORE FEEDING
- oesophageal atresia should be diagnosed before the infant is
fed, because feeding will cause an acute episode of spluttering, coughing
and cyanosis, with aspiration of milk into the lungs.
- A firm 10 French catheter should be introduced through the `
mouth and passed carefully down the oesophagus; if it becomes
arrested at about10 cm from the lips, the diagnosis of oesophageal atresia
has been established.
- A small catheter may curl up in the upper oesophagus and give a
false impression of oesophageal continuity.
DIAGNOSIS
52. • If the infant has dysmorphic facies and other features suggestive of a major
chromosomal abnormality, early genetic consultation is mandatory.
• The two most common chromosomal abnormalities are Trisomy 18 and
Trisomy 21.
• There are a number of malformation clusters, such as the VATER (or
VACTERL) and CHARGE associations, which are well known to occur with
oesophageal atresia
GENETIC CONSIDERATION
54. • X-ray
An x-ray of the thorax and abdomen
is taken to demonstrate the presence of air
in the stomach and small bowel, which
indicates that there is a fistula between the
trachea and the lower segment of the
oesophagus.
PRE-OP PREPARATIONS
55. • Echocardiography
- Nearly 25% of infants with oesophageal atresia have congenital heart
disease.
- It is important to identify cardiac lesions preoperatively because a
prostaglandin E1 infusion will need in ‘duct-dependent’ cases and to identify a
right aortic arch and influence the surgical approach to the oesophagus.
- Butthe cardiac defect does not delay the oesophageal surgery, and
oesophageal repair takes precedence over surgery to the heart.
PRE-OP PREPARATIONS
56. • Renal ultrasonography
- If the infant has not passed urine, a renal ultrasound examination
must be performed to exclude bilateral renal agenesis.
- If the infant has no kidneys or has severely dysplastic kidneys, as it
occurs in 3% of the cases, no surgery is justified.
PRE-OP PREPARATIONS
57. • The operation is performed usually within 12 h of admission to hospital.
• Frequent suction to upper pouch
• placed in an incubator or under an overhead heater to avoid heat loss
• Vitamin K is given intramuscularly
• Intravenous fluids
• Antibiotics
TREATMENT
58. • The fistula is closed through a right posterolateral extrapleural thoracotomy.
• division of the tracheo-oesophageal fistula and reconstruction of an end-to-
end oesophageal anastomosis.
• In some centres, thoracoscopic repair of oesophageal atresia is performed.
• Postoperatively, oral feeds can be commenced after 3 or 4 days.
TREATMENT
60. • Can occur in term neonates without any risk factors.
• Primary spontaneous pneumothorax is defined as a pneumothorax occurring
secondary to apical blebs or bullae without evidence of other lung pathology.
• Secondary spontaneous pneumothorax occur in the context of underlying
lung disease, such as cystic fibrosis, lung infections, bronchiolitis, asthma
(even mild), connective tissue disorders & CCAM.
PNEUMOTHORAX
61. • supplemental oxygen
• Intravenous access
• A pneumothorax of less than 15% can often be managed by observation with
or without supplemental oxygen
MANAGEMENT
62. • The indications for surgical management include recurrence, persistent air
leak, bilateral disease, and possibly the presence of large bullae.
• VATS appears to be superior to standard thoracotomy with respect to
postoperative pain and complications.
• Transaxillary mini-thoracotomy is a viable alternative to VATS and apical
pleurectomy can be easily performed through it.
• For pleurodesis, thoracoscopic pleural abrasion and talc poudrage are
effective techniques.
MANAGEMENT
63. • The indications for surgical management include recurrence, persistent air
leak, bilateral disease, and possibly the presence of large bullae.
• VATS appears to be superior to standard thoracotomy with respect to
postoperative pain and complications.
• Transaxillary mini-thoracotomy is a viable alternative to VATS and apical
pleurectomy can be easily performed through it.
• For pleurodesis, thoracoscopic pleural abrasion and talc poudrage are
effective techniques.
MANAGEMENT
65. • Potential life threatening airway emergency
• Retropharyngeal space: potential space between posterior pharyngeal wall
and prevertebral fascia extend from base of skull to level of T2
• Tx-need +/- intubation +/- surgical drainage