Beruflich Dokumente
Kultur Dokumente
Illustration of the principal respiratory muscles and their position in the respiratory system
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Congenital cystic adenomatoid malformation
先天性囊腫腺樣畸形
Characteristic Type 1 Type 2 Type 3
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• Associated anomalies Rare(5%) Common(56%) None
• Respiratory distress D1-4 Ws D1 within
Hs
• Gestational age
Premature/Term 32%/52% 75%/25% 7%/33%
Stillborn 16% 32% 33%
• Gross and micro- Single or Multiple, Large
scopic features multiple small non-
cystic Cystic (>2 cm) (<2 cm)
• Prognosis Good Poor Poor
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Pulmonary sequestration (I)
肺葉隔離
Characteristic Intralobar 肺葉內 Extralobar 肺葉外
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• Incidence ratio 3 1
• Sex Equal Male 80%
• Side 60% left 90% left
• Location Usually in post- Above diaphragm,
basal segment rarely below
• Age at presentation Adolescent, Neonate 60%,< 1Y
& symptoms 50%> 20Y Respiratory
distress Recurrent
pulmonary infections
*connect with foregut Rare Common
Pulmonary sequestration (II)
Characteristic Intralobar Extralobar
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• Associated None
Frequent(50%),e.g. anomalies
CDH(30%)
• Arterial supply Systemic- from Systemic- from
aorta, a single pulmonary or
aorta, large vessel small vessel
• Venous drainage Pulmonary- inf. Systemic- azygos
pulmonary V. or hemiazygos
• Anatomic relations Within or part Separate- visceral
of normal lobe pleura
• Bronchial commu. Present, small None
Congenital diaphragmatic hernias. Diagram of diaphragm from below with areas of
potential herniation indicated. 1. Sternocostal foramina of Morgagni anteriorly; 2.
esophageal hiatus; 3. lumbocostal foramina of Bochdalek posteriorly
Congenital diaphragmatic hernia (CDH)
先天性橫膈膜疝氣
Group1. Group2. Group3.
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• Pulmonary insignificant significant severe,bilateral
hypoplasia
• Symptomatic usually asym. usually always
within 6h of birth
• Blood gas near-normal
Highest possible preductal Pao2 >80mmHg <60 mmHg
Lowest possible preductal Paco2<40mmHg >40mmHg
• Pulmonary insignificant prone to intractable
hypertension R to L shunt episodes
• Mortality <5% about 30% 100%
Congenital diaphragmatic hernia. The tip of the nasogastric tube and
obvious loops of gas-filled bowel are located in the left hemithorax.
Late presentation of a left diaphragmatic hernia in a 3-year-old child. (A) CXR
after a barium swallow shows a part-cystic, part-solid mass in the left chest with
a normal oesophagus and gastric position. (B) The delayed film at 24 hours
shows that the colon is in the left chest, confirming a diaphragmatic hernia
and excluding a lung malformation.
Pathophysiology of congenital diaphragmatic
hernia
先天性橫膈膜疝氣 病理生理學
• Hypoventilation 換氣不足
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Cause Consequence
Reversible
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1. Anatomical hypoplasia Hypoxia, hypercarbia
No 2. Compression from bowel Hypoxia,
hypercarbia Yes 3. Surfactant deficiency
Hypoxia, hypercarbia Yes
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• Hypoperfusion 血液灌流不足
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- 1. Anatomical hypoplasia ↑PVR R→L shunt
No 2. ↑smooth muscle mass ↑PVR R→L shunt
Protocol for postnatal management 出生後治療方
案 congenital diaphragmatic hernia
(A) 氣管
(B) 食道
(C) 胸腺
(D) 肺臟
3. 先天性橫膈疝氣 (congenita1 diaphragmatic hernia) 之
致死原因最主要為:
(A) 肺部被腹腔腸道壓迫
(B) 肺部發育不全
(C) 大動脈被壓迫
(D) 併發其他嚴重先天性異常
4. 患有橫膈疝氣 (diaphragmatic hernia) 的新生兒
在發生呼吸窘
迫急救時,何者為錯誤步驟?
(A)
氣管內插管
(B)
置入胃管
(C)
面罩呼吸 (mask and AMBU bagging)
(D) 動脈血液氣體分析
5. 有關食道氣管廔管 (E-T Fistula) 兒童之描述,何
者錯誤?
(A) 病童口中出現過多的氣泡狀唾液或分泌液
(B) 病童會出現窒息、咳嗽、發紺現象
(D) 病童發生率以男嬰為多,男女比率為三比一