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Congenital II Flashcards

1
Q

What are the main reasons for surgery on a symptomatic congenital cystic lung disease (CTM)?

A

Surgery is indicated due to:
* Size
* Compression of nearby structures
* Complications, such as infection

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2
Q

What is considered the best and definitive treatment for symptomatic CTM?

A

Surgery, specifically lobectomy, is considered the best and definitive treatment.

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3
Q

What are some complications associated with lobectomy?

A

Complications can include:
* Tension pneumothorax
* Near-exsanguination
* Chest wall fibromatosis

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4
Q

What imaging is advised for asymptomatic CTM discovered on antenatal ultrasound?

A

A chest radiograph should be obtained postnatally, but HRCT is advised to delineate the abnormality.

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5
Q

What pulmonary function test results are typically seen in patients with CTM?

A

Respiratory rates (RR) are elevated and tidal volumes are reduced.

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6
Q

List reasons for operating on an asymptomatic CTM.

A

Reasons include:
* Preventing nonmalignant complications
* Allowing optimal lung growth
* Preventing malignant transformation

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7
Q

True or False: The optimal timing for surgery on asymptomatic CTM is well established.

A

False

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8
Q

What is the risk of complications for asymptomatic congenital cystic disease?

A

There is about a 3% risk of complications such as infection, bleeding, and air leak.

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9
Q

What is the risk of malignancy associated with asymptomatic CTM?

A

There is about a 4% risk of malignancy.

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10
Q

What is the classical definition of pulmonary sequestration?

A

Pulmonary tissue that is isolated from normal functioning lung and nourished by systemic arteries.

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11
Q

What are the two types of pulmonary sequestration?

A

The two types are:
* Intrapulmonary sequestration
* Extralobar sequestration

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12
Q

What is the age of diagnosis for intralobar sequestration?

A

More than half are diagnosed after adolescence.

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13
Q

What percentage of extralobar sequestration cases are detected in infancy?

A

Generally detected in infancy.

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14
Q

What type of symptoms are commonly presented in patients with pulmonary sequestration?

A

Nonspecific symptoms such as cough, fever, and chest pain.

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15
Q

What are the common organisms cultured from resected specimens of pulmonary sequestration?

A

Aspergillus fumigatus and Pseudomonas aeruginosa.

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16
Q

What is the treatment for pulmonary sequestration?

A

Surgical excision (open or thoracoscopic) or embolization.

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17
Q

What is congenital lobar emphysema?

A

A rare condition where the affected lobe cannot deflate and overdistends, displacing adjacent lobes.

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18
Q

What are the common locations affected by congenital lobar emphysema?

A

Left upper lobe (42%), right middle lobe (35%), and right upper lobe (21%).

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19
Q

What are the clinical features of infantile lobar emphysema?

A

Features include hyperresonance of the affected hemithorax and diminished breath sounds.

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20
Q

What is bilateral pulmonary agenesis?

A

A rare malformation that may occur in anencephaly.

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21
Q

What are the associated malformations with unilateral pulmonary agenesis?

A

Cardiac, gastrointestinal, skeletal, vascular, genitourinary, and craniofacial abnormalities.

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22
Q

What is the prevalence of pulmonary agenesis?

A

0.34 per 100,000 live births.

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23
Q

What is the most common variant of pulmonary aplasia?

A

Unilateral absence of the distal lung with a carina and main-stem bronchial stump.

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24
Q

Fill in the blank: Pulmonary hypoplasia is characterized by _______.

A

[incompletely developed lung parenchyma connected to bronchi that may also be underdeveloped]

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25
What is the consequence of bronchial stump with unilateral absence of the distal lung?
Secretions can pool in the stump, become infected, and possibly spill over to infect the sole lung.
26
What is lobar agenesis and aplasia?
Rarer than complete absence of one lung, usually affect the right upper and middle lobes together.
27
Define pulmonary hypoplasia (CSLs).
Incompletely developed lung parenchyma connected to bronchi that may also be underdeveloped.
28
What is the lung-to-body weight ratio considered for pulmonary hypoplasia in term babies?
< 0.012
29
What is the incidence of associated malformations with pulmonary hypoplasia?
Around 50%.
30
What may permit the growth of alveoli in pulmonary hypoplasia?
Correction of any underlying abnormality, if feasible.
31
At what week is airway branching complete?
16 weeks.
32
What is ectopia in relation to lung tissue?
Growth of normal tissue in the incorrect anatomic position.
33
Name a common ectopic tissue found in the lung.
Ectopic glial tissue.
34
What are alternative terms for ectopic lung tissue?
* Congenital pneumatocele * Congenital pulmonary hernia
35
What is a tracheoesophageal fistula?
An abnormal connection between the trachea and esophagus.
36
What is the most common associated condition with esophageal atresia?
Tracheoesophageal fistula.
37
What genetic factors are implicated in esophageal atresia?
* CHD7 in familial CHARGE syndrome * FOXF1 * 16q24.1 * ZIC3 gene
38
What is the presentation of pure esophageal atresia?
Commonly frothing, choking, and cyanotic episodes at birth.
39
What is the common diagnosis method for tracheoesophageal fistula?
Plain radiograph.
40
What percentage of infants with tracheoesophageal fistula have associated anomalies?
> 50%.
41
What is VATER association?
Vertebral, anal, tracheoesophageal, radial, and renal anomalies.
42
What is the most common type of esophageal atresia?
Type C esophageal atresia with distal TEF
43
What is the role of continuous suction in preoperative care for tracheoesophageal fistula?
To reduce the risk of aspiration and protect the airway.
44
What surgical technique is most common for esophageal atresia and tracheoesophageal fistula?
Right thoracotomy with an extrapleural approach and end-to-end single-layer anastomosis.
45
What is the prognosis for early surgical repair of esophageal atresia?
Good.
46
What is scimitar syndrome?
Characterized by a small right lung and abnormal venous drainage to systemic veins.
47
What is the prognosis for infants with scimitar syndrome presenting in heart failure?
Worse prognosis due to associated abnormalities.
48
What is the effect of congenital absence of the pulmonary veins?
Results in pulmonary venous obstruction and severe hypertensive changes.
49
What is congenital alveolar capillary dysplasia?
[Definition not provided in the text]
50
What condition results in pulmonary venous obstruction?
Veins ## Footnote May also be associated with partial anomalous pulmonary venous drainage
51
What is the prognosis for children with severe pulmonary venous obstruction?
Very poor prognosis; few survive longer than 1 year ## Footnote Stenting open the ostia via cardiac catheterization has been reported
52
What does congenital alveolar capillary dysplasia represent?
A misalignment of lung vessels due to a failure of capillaries to grow in appropriate number and location ## Footnote It is an unusual cause of congenital pulmonary hypertension
53
What are the clinical features of pulmonary arteriovenous malformations (PAVMs)?
Cyanosis, hemoptysis, chest pain, neurologic complications ## Footnote Breathlessness is rare, and patients can exercise at a high level
54
What is the prevalence of hereditary hemorrhagic telangiectasia (HHT)?
1 in 5000–8000 ## Footnote HHT is associated with pulmonary arteriovenous fistulae
55
What mutations are associated with HHT1 and HHT2?
HHT1 - mutations in endoglin (ENG, 9q34); HHT2 - mutations in ACVRL1 (ALK-1, 12q-13) ## Footnote A third locus has been described on chromosome 5
56
What is the primary diagnostic method for PAVMs?
Echocardiography ## Footnote It has excellent positive and negative predictive value
57
What is a common complication of PAVMs?
Systemic, particularly cerebral, abscess, or thrombosis ## Footnote Iron deficiency may increase the risk of ischemic stroke
58
What are capillary malformations associated with?
Syndromes such as Sturge-Weber, Klippel-Trenaunay, and Parkes-Weber ## Footnote They may also be associated with macrocephaly and diffuse overgrowth
59
What is the presentation of congenital chylothorax?
Severe respiratory distress, may be fatal in the neonatal period ## Footnote Milder cases with prolonged survival have been described
60
What is the most common cause of death in congenital diaphragmatic hernia (CDH)?
Pulmonary hypoplasia ## Footnote Due to herniation of abdominal contents into the chest
61
What imaging is used to diagnose congenital diaphragmatic hernia?
Plain radiograph of the chest and abdomen ## Footnote Ultrasound may demonstrate intrathoracic intestinal peristalsis
62
What is the management approach for congenital diaphragmatic hernia?
Stabilize the baby and optimize respiratory function; delayed surgical repair ## Footnote Lower mortality rates have been noted with this approach
63
What is the survival rate of neonates after repair of congenital diaphragmatic hernia?
39% - 95% ## Footnote Variation reflects the severity of pulmonary hypoplasia
64
What is the definition of anterior diaphragmatic hernia?
Occurs through the foramen of Morgagni, associated with the internal mammary artery ## Footnote Tend not to present in the neonatal period
65
What can be a complication following repair of a diaphragmatic hernia?
Chylothorax ## Footnote Octreotide may be used as treatment
66
What are potential long-term outcomes after repair of congenital diaphragmatic hernia?
Neurologic abnormalities, motor and cognitive anomalies, gastroesophageal reflux ## Footnote Lung function may vary from normal to obstructive or restrictive disease
67
What is diaphragmatic eventration?
May be congenital or acquired; results from incomplete development of the diaphragm ## Footnote Can also occur due to injury to the phrenic nerve
68
What is diaphragmatic eventration?
May be congenital or acquired.
69
What is a congenital lesion in diaphragmatic eventration?
Rarer, results from incomplete development of the muscular portion of the diaphragm or its innervation.
70
What conditions have been described in neonatal cases of congenital diaphragmatic eventration?
Fetal rubella and CMV infection.
71
What are common causes of acquired diaphragmatic eventration?
* Injury to the phrenic nerve * Difficult instrumental delivery * Insertion of a chest drain for pneumothorax * Cardiac surgery * Difficult breech delivery (associated with Erb palsy)
72
Which side is the defect in diaphragmatic eventration more common?
Left.
73
How does diaphragmatic eventration usually present?
Moderate to complete thinning of the diaphragmatic muscle fibers.
74
Are bilateral lesions in diaphragmatic eventration common?
Rare.
75
What associated anomalies are linked with diaphragmatic eventration?
* Rib anomalies * Cardiac anomalies * Renal ectopia * Exomphalos
76
How is diaphragmatic eventration typically diagnosed?
Confirmed by ultrasound examination demonstrating paradoxical movement of the diaphragm.
77
What is the usual management for diaphragmatic eventration?
Usually no treatment is needed; radiologic appearance improves with time.
78
When may bilateral large eventrations need repair?
When significant.
79
What is a common cardiac malformation associated with congenital lung abnormalities?
Atrial or ventricular septal defect.
80
What should be a routine part of the work-up for suspected congenital lung abnormalities?
Echocardiography.
81
What is the significance of micronodular type II pneumocyte hyperplasia?
It is largely confined to patients with tuberous sclerosis and is a rare manifestation.
82
What are the characteristics of the hyperplastic cells in type II pneumocyte hyperplasia?
Show no atypia and appear to be devoid of any malignant potential.
83
What are the key investigations for diagnosing congenital lung malformations?
* Chest radiography * Ventilation-perfusion scanning * Ultrasound scanning * Computed tomography * Magnetic resonance imaging * Barium swallow * Esophageal tube injection * Angiography * Bronchoscopy
84
What does a normal chest radiograph not exclude?
A significant congenital thoracic malformation (CTM).
85
What does ventilation-perfusion scanning provide?
Functional information.
86
What is a key limitation of ventilation-perfusion scanning?
Its use is declining as CT and MRI give better anatomical and functional images.
87
What imaging technique is often the first investigation of choice for suspected CTM in adults?
Computed tomography.
88
What is the role of barium swallow in diagnosing vascular rings?
To confirm esophageal compression and hold-up of contrast.
89
What is a potential complication of bronchoscopy?
Missed laryngeal clefts.
90
What does low contrast volume bronchography delineate?
The major airways.
91
True or False: Barium swallow can rule out an H-type tracheoesophageal fistula.
False.
92
What is the significance of embolization in congenital disorders of the lung?
Controversial, particularly other than for pulmonary arteriovenous fistula.

Kendigs Respiratory Diseases (38 decks)

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