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Pediatrics > Congenital chest > Flashcards

Congenital chest Flashcards

1
Q

Pumonary hypoplasia

A

Reduced thoracic circumference

Fetal lung: head ratio <1

Associations include:

  • Fryns syndrome
  • Meckel Gruber syndrome
  • Neu-Laxova syndrome
  • Pena-Shokeir syndrome

Adults:
Swyer-James Syndrome
Poland syndrome
Scimitar syndrome

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2
Q

Jeune syndrome

A

Asphyxiating thoracic dysplasia

Long narrow thoracic cavity, cystic renal dysplasia, and characteristic skeletal features. It is also sometimes classified as one of the short rib polydactyly syndromes

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3
Q

Congenital diaphragmatic hernias

A

Most common: Left and posterior (Bochdaleck)

Rare: Morgagni (anterior)

Right: GBS Pneumonia

45% malrotation

Mortality: Pulmonary hypoplasia

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4
Q

Bronchogenic cyst

A

Most common foregut duplication cyst

Most commonly in mediastinal location

Extreme rare malignant conversion (pleuropulmonary blastoma, rhabdomyo, anaplastic, leyomio, adenoc)

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4
Q

Bronchogenic cyst

A

Most common foregut duplication cyst

Most commonly in mediastinal location

Extreme rare malignant conversion (pleuropulmonary blastoma, rhabdomyo, anaplastic, leyomio, adenoc)

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5
Q

Pulmonary sequestration

A

Aberrant formation of segmental lung tissue that has no connection with the bronchial tree or pulmonary arteries. It is a bronchopulmonary foregut malformation (BPFM).

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6
Q

Extralobar pulmonary sequestration

A

Pleural surface (low chance of infection), draining to systemic veins, associated with congenital anomalies, presents during infancy, predominantly in the left lower lobe, however 10% subdiaphragmatic.

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7
Q

Intralobar pulmonary sequestration

A

Most common pulmonary sequestration (75%)
No pleural surface (young patient with recurrent “pneumonia”), no congenital anomalies, draining to the pulmonary veins.

Draining via the pulmonary veins but can occur through the azygos-hemiazygos system, portal vein, right atrium or inferior vena cava

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8
Q

Cogenital lobar emphysema (overinflation)

A

Males
Aberrant left pulmonary artery
ASD, VSD, ToF
LUL: Most common

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9
Q

Congenital cystic adenomatoid malformation (CPAM)

A

Congenital pulmonary airway malformation

Lung malignancy:
~10% of pediatric lung cancers have a history of CPAM
bronchoalveolar carcinoma associated with type 1 CPAM
pleuropulmonary blastoma associated with type 4 CPAM

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10
Q

Normal second trimester ultrasound + new lung mass

A

Primary lung tumor

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11
Q

Pleuropulmonary blastoma

A

Most common lung malignancy in children

Classified under sarcomatoid carcinomas of the lungs

Solid: worse
Cystic: better (type 1, similar to CPAM 1/4).

CPAM 4 is associated with PPB

0-2 years of age.

DICER 1

Multilocular cystic nephroma, rarely Wilms.

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12
Q

Inflammatory myofibroblastic tumor (IMT)

A

Most common benign tumor in chidren

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13
Q

UVC

A

Left portal vein>ductus venosum>left liver vein

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14
Q

UAC

A

T8-T10
L3-L5

Omphalocele is contraindications

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15
Q

Askin tumor

A

Ewing sarcoma of the chest wall

16
Q

DICER 1 syndrome

A

Annual chest CT to age 18 years, biannual abdominal/pelvic US to age 40 years, annual brain MR to age 25 years, and annual history and physical exam emphasizing thyroid palpation

pleuropulmonary blastoma
cystic nephroma
rhabdomyosarcoma
multinodular goiter
ovarian Sertoli-Leydig cell tumor
differentiated thyroid carcinoma
pineoblastoma
Wilms tumor
chondromesenchymal hamartoma
pituitary blastoma

https://www.researchgate.net/publication/336596856_Imaging_of_DICER1_syndrome

17
Q

Pediatric cystic nephroma (multilocular cystic nephroma)

A

Genetic analysis shows a high prevalence (86%) of DICER1 gene mutations in pediatric cases, as compared to ~10% of adult cystic nephroma.

Differential diagnosis:

  • Cystic partially differentiated nephroblastoma: Impossible to differentiate by imaging alone similar age-group no DICER 1 mutation
  • Cystic Wilms tumor from pediatric cystic nephroma: solid nodular components in a tumor should arouse suspicion of Wilms tumor. Pediatric cystic nephroma tends to occur in younger children than Wilms tumors
  • Multicystic dysplastic kidney (MCDK): Entire kidney is replaced by non-communicating cysts. Usually diagnosed prenatally or at birth, while multiloculated cystic renal tumors do not occur in the perinatal period. Enhanced compressed renal parenchyma is seen to surround cystic nephroma on contrast-enhanced cross-sectional studies, while this tissue is absent in MCDK
18
Q

Swayer James syndrome

A

Postinfectious bronchiolitis

Diminutive hyperlucent lung

19
Q

Poland syndrome

A

Pec major and minor
Hyperlucent lung
Ipsilateral syndactily

Differential diagnosis:
For hypoplastic breast/absent tissue on one side on mammography, consider:

  • amazia: absence of breast parenchyma
  • amastia: absence of breast tissue
    can occur from an excision of a pre-puberal breast bud or from chest wall irradiation during childhood
20
Q

Scimitar syndrome (hypogenetic lung syndrome)

A

PAPVR/Congenital venopulmonar syndrome

Left to right shunt

21
Q

VV ECMO

A
22
Q

Round pneumonia/neonatal peripheral atelectasia

A

Five years and 90% of patients who present with round pneumonia are younger than twelve.

Round pneumonia is uncommon after the age of eight because collateral airways tend to be well developed by this age

S pneumo> H i.

5% progress to lobar pneumonia

23
Q

Recurrent respiratory papillomatosis

A

Langerhan cell histiocytosis-like

HPV-6 and 11 are most commonly implicated (90% total cases), although HPV-16, 18, 31, and 33 also implicated

HPV-11 is associated with more aggressive papillomatosis

HPV-16 and 18 are more associated with malignant transformation, although “low risk” sub-types (e.g. 6, 11) have also resulted in malignant degeneration

24
Q

Acute chest syndrome

A

Leading cause of death in SCD

25
Q

Cystic fibrosis

A

Absent vas deferens (infertility)

Finger in glove sign

Apical predominance bronchiectasis (cylindrical»>varicoid)

Finger in gloves (Mucus plugging)

Fatty replaced pancreas

26
Q

Primary ciliary dyskinesis

A

Spermatic tail dont move (infertility in males).

Ovum is not taking by the fallopian tubes (subferitlity in females)

Apical bronchiectasis

50% of patients demonstrating situs inversus

27
Q

Situs inversus

A 
Situs inversus (totalis): Chest and abdominal aorta. Associated with IVC absence.
Situs solitus: Chest aorta
Situs ambiguus (heterotaxy): Indeterminate configuration.

“When situs solitus and situs inversus occur, the atrial situs always corresponds to the visceral situs. In situs ambiguus, the bronchial anatomy always corresponds to the atrial situs”.

  1. Most with dextrocardia (true mirror image)
    Only 3-5% incidence of congenital heart disease (transposition of the great vessels).
    - 80% have a right-sided aortic arch.
  2. Situs inversus with levocardia (which is much rarer: 0.00005%) congenital heart disease is found in 95% of patients

When situs solitus and situs inversus occur, the atrial situs always corresponds to the visceral situs.

28
Q

Right atrial situs

A

Crista terminalis: it is a smooth thickened muscular structure which represents junction between sinus venosus and developing heart
Right atrial appendages: triangular or pyramidal shaped and broad-based; it contains pectinate muscles extending towards the atrioventricular valve-

29
Q

Left atrium situs

A

Crista terminalis: absent in left atrium
Left atrial appendages: long slender finger-like; contains pectinate muscles
Coronary sinus always runs inferoposteriorly to the left atrium

30
Q

Situs ambigous (heterotaxy)

A

Right isomerism: Cyanotic congenital cardiac anomalies

Left isomerism: Less complex congenital heart disease

Intestinal malrotation and midgut volvulus

Bronchial anatomy accurately reflects atrial situs.
hyparterial bronchus (below artery): supplies the bilobed left lung

eparterial bronchus (alongside the artery): supplies the trilobed right lung

31
Q

Left isomerism

A

Known as polysplenia syndrome:

multiple splenules without a parent spleen
azygos or hemiazygos continuation of the inferior vena cava
bilateral hyparterial bronchi
bilateral bilobed lungs
bilateral pulmonary/left atria
midline/transverse liver
intestinal malrotation

32
Q

Right isomerism

A

Asplenia syndrome (Ivemark syndrome)

Cyanotic congenital heart disease

Duplicated SVC

Absent coronary sinus

Patients die before 1-year-old because of severe/complex congenital heart disease.

Most patients are immunocompromised due to absent spleen.

Pediatrics (10 decks)

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