Coupon Accepted Successfully!


Paediatric Diseases

Pulmonary sequestration
  1. QSequestrated lung is defined as “a congenital mass of aberrant pulmonary tissue that has no normal connection with the bronchial tree or with the pulmonary arteries.
  2. QIt is usually located in one of the basal segments of lower lobe.

In 98% cases intralobar sequestration involves medial parts of left lower lobe.

  1. About 98% of extralobar sequestrations are left sided.
  2. Two varieties of pulmonary sequestration are known: Intra and Extralobar type.
  3. Extralobar (ELS)  it is most common variety in neonates and infants, is located between the lower lobe and diaphragm, has its own pleural covering, and other anomalies are common (65%) in ELS (like pulmonary hypoplasia, horseshoe lung, CCAM, bronchogenic cysts, diaphragmatic hernia and cardiac anomalies).
  4. Intralobar sequestration (ILS)  diagnosed after adolescence, contained within lung with no separate pleural covering and is intimately connected to adjacent lung.
  5. It is usually supplied by an anomalous artery arising from aorta and its venous drainage is via the azygos system, the pulmonary vein, or IVC. Hence angiography is useful for confirmation of the diagnosis, which shows one or more systemic vessel entering the mass, usually arising from aorta at or below diaphragm.

The preop imaging modality of choice for pulmonary sequestration is angiography. Q

  1. Congenital Diaphragmatic Hernia

  1. It is most common intrathoracic fetal anomaly due to absence of closure of pleuroperitoneal canal by 9th week of gestation
  2. Presents as respiratory distress in neonatal period due to life threatening deficiency of small airways and alveoli, and scaphoid abdomen.
  3. QCXR:
    1. Bowel loops in chest.
    2. Contralateral mediastinal shift.
    3. Complete/partial absence of diaphragm.
    4. Absence of stomach/small bowel in abdomen.
  4. QBochdalek's hernia usually causes severe respiratory distress in neonate and is one of the commonest congenital anomalies of thorax.
  5. QIn Morgagni hernia, omentum and colon are the most frequent hernial contents. It is known to be associated with trisomy syndromes.

QOn USG, congenital diaphragmatic hernia can be detected as early as at 17th week of gestation.

Absolute diagnostic ultrasonographic sign is visualization of peristalsis in chest and paradoxical motion of the abdominal contents on fetal inspiration.

QThirteen pairs of ribs and not vertebral anomalies occur in association with a Bochdalek's congenital diaphragmatic hernia.

  1. Congenital Lobar Emphysema (CLE)
    1. Progressive over inflation of one or multiple lobes, usually of the upper lobes or right middle lobe (left upper lobe → 43%, right middle 32%, right upper 20% and lower lobe 5%)
    2. QLocation: Left upper lobe (LUL) most common (43%)
    3. The word emphysema is misnomer as there is no alveolar wall destruction
    4. Male to female ratio is 3:1
    5. Associated congenital anomalies (cardiovascular) are seen in 50% cases
    6. QCXR:
      1. Hazy mass like opacity immediately following birth
      2. Air trapping
      3. Hyperlucent expanded lobe after clearing of fluid
      4. Contralateral mediastinal shift
      5. Widely separated/ reduced vascular markings
    7. Treatment is by lung resection
    8. QCT is very useful in indeterminate cases.
  2. Bronchogenic cysts and esophageal duplication cysts:
    1. Mediastinal bronchogenic cysts and esophageal duplications are often considered part of the spectrum of bronchopulmonary foregut malformations.
    2. Esophageal duplication cyst is round or tubular lesion occurring in the lower posterior mediastinum, which often distort esophagus but only rarely communicate with the esophageal lumen. Proximal esophageal duplication cysts may be associated with tracheal compression and present with upper airway obstruction. While distal esophageal cysts are often asymptomatic and found as an incidental chest X-ray finding.
    3. Barium meal will confirm the presence of a smooth extrinsic esophageal filling defect and CT/MRI will demonstrate its cystic nature.

Cystic Mediastinal mass and associated vertebral anomalies like hemivertebra, block vertebra, butterfly vertebra or spina bifida suggest the diagnosis of Neuroenteric Cyst.Q

Test Your Skills Now!
Take a Quiz now
Reviewer Name