呼吸系統

Illustration of the principal respiratory muscles and their position in the respiratory system

Illustration of the principal respiratory muscles and their position in

the respiratory system
Anatomic compartments of the mediastinum. The anterior compartment is
bound posteriorly by the pericardium, ascending aorta, and brachiocephalic
vessels and anteriorly by the sternum. The middle compartment extends from
the posterior limits of the anterior compartment to the posterior pericardial line.
The posterior compartment extends from the pericardial line to the dorsal chest
 Anterior mediastinum 前縱膈
腔
< 2Y > 2Y
• Benign teratoma • Malignant germ cell
• Thymic hyperplsia tumor
• Cystic granuloma • Thymoma
• lymphoma
Middle mediastinum 中縱膈腔
< 2Y > 2Y
• Brochogenic cyst • Lymphoma
• Granuloma
Posterior mediastinum 後縱膈腔
< 2Y > 2Y
• Neuroblastoma • Ganglioneuroma
• Enterogenous cyst • sarcoma
 Developmental anomalies of lung
肺臟發育畸形
• Primary anomalies
Agenesis of the lung
Lobar underdevelopment
• Secondary anomalies
Congenital diaphragmatic hernia 先天性橫膈膜疝氣
Accessory fissures and lobes
. Congenital (cystic) masses 先天性肺臟囊腫
Pulmonary Sequestration 肺隔離
Congenital cystic adenomatoid malformation (CCAM)
Congenital lobar emphysema 先天性大葉肺氣腫
Brochogenic cysts 支氣管囊腫
Neurenteric cysts
Pulmonary arteriovenous malformation
 Pulmonary developmental anomalies 肺臟發育畸
形 in relation to pulmonary vasculature
development
Lung anatomy & Resultant condition
vasculature
--------------------------------------------------------------------------
• Normal lung Aplastic or hypoplastic lung
Abnormal vasculature Arteriovenous malformation
• Abnormal lung intra- or extra-lobar sequestration
Abnormal vasculature (cystic) adenomatoid malformation
• Abnormal lung Brochogenic cyst
Normal vasculature Congenital lobar emphysema

---------------------------------------------------------------------------
Congenital cystic adenomatoid malformation
先天性囊腫腺樣畸形
Characteristic Type 1 Type 2 Type 3
----------------------------------------------------------------------------------------
--
• 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

--------------------------------------------------------------------------
 Pulmonary sequestration (I)
肺葉隔離
Characteristic Intralobar 肺葉內 Extralobar 肺葉外
--------------------------------------------------------------------------
• 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
--------------------------------------------------------------------------
• 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.
--------------------------------------------------------------------------------------------
-
• 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

------------------------------------------------------------------------------------------
Cause Consequence
Reversible
------------------------------------------------------------------------------------------
1. Anatomical hypoplasia Hypoxia, hypercarbia
No 2. Compression from bowel Hypoxia,
hypercarbia Yes 3. Surfactant deficiency
Hypoxia, hypercarbia Yes
----------------------------------------------------------------------------------------
• Hypoperfusion 血液灌流不足

------------------------------------------------------------------------------------------
- 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

Delayed CDH surgery protocol 延遲手術方案

• High risk CDH 高風險
↓ ↓
• Unstable Stable
↓ ↓
• Preop ECMO CDH repair >24 hrs
↓
• Off ECMO within 7 days ?
↓ ↓
• Yes- CDH repair No- CDH repair → Lung
post ECMO on ECMO transplantation?
Algorithm for the management of the
fetus with CDH
先天性橫膈膜疝氣 胎兒治療流程
• Ultrasound, Amnio Pubs, Cardiac Echo
↓ ↓
• Other anomalies Isolated defect
↓ Prognostic evaluation-
↓ Gestation age, Polyhydramnios,
↓ Volume (stomach), Lung thorax ratio
↓ ↓ ↓ ↓
• Counsel ←←← Severe-early Equivocal Mild-late
<24 weeks 24-32 weeks >32weeks
↓ ↓ ↓ ↓ ↓
• Abort or no Rx Repair in utero Counsel follow
↓ ↓
• Monitor tocolysis Deliver (vaginal) after

↓ lung maturation (L/S)

• Deliver (C/S) near term repair, ECMO, etc
 Pathophysiology of the deterioration seen
following the honeymoon period 蜜月期 in infants
with congenital diaphragmatic hernia

Pulmonary hypoplasia 肺臟發育不全

↓
Hypoxia-Hypercarbia 低血氧 - 高二氧化碳血
症
↑ ↓
Poor gas exchange Pulmonary
Right to left shunt vasoconstriction
↑ ↓
Pulmonary hypertension 肺動脈高壓
肺動脈高壓
 Congenital diaphragmatic hernia
先天性橫膈膜疝氣

* Lung Hypoplasia 肺臟發育不全

= Volume + Timing of Herniation
* Fetal CDH appears to be a dynamic process,
and early herniation of a large volume of viscera
through a large defect leads to severe lung
hypoplasia and neonatal death.
Indications for neonatal ECMO
葉克膜 適應症
1. Oxygenation index* > 25
2. No congenital anomaly incompatible with
normal life
3. Gestational age > 35 weeks
4. Mechanical ventilation less than 7-10 days
5. No evidence of intracranial hemorrhage
6. In CDH, evidence of a honeymoon period
(i.e. Pao2 > 60)
*Oxygenation index=Mean airway pressure x Fio2 x 100 /
postductal Po2
Congenital diaphragmatic hernia 先天性橫膈膜疝
氣 associated anomalies 合併畸形
in 153 cases(excluding pulmonary
hypoplasia)
• Malrotation 腸道旋轉不全　 63
• Congenital heart lesions 　先天性心臟病 8
(PDA:3; Hypoplastic LT. heart:2; VSD:1; ASD+
coarctation of aorta:1; Pulmonary stenosis:1)
• Undescended testes 8
• Meckel’s diverticulum 7
• Skeletal anomalies 7
• Myelomeningocele 3
• Renal anomalies 3
• Extralobar pul. sequestration 2
• CCAM 1
• Duodenal atresia 1
• Hirschsprung’s disease 1
Morgagni hernia

A, A mass is noted in the

right chest on a
posteroanterior view chest
radiograph.
B, Lateral chest radiograph
shows that the mass is in
the anterior chest.

C, Barium enema shows

that a portion of the
transverse colon is the
hernia.
D, Computed tomography
shows a contrast-filled
colon in the right anterior
chest.
Large hiatal hernia. Hiatal hernias can sometimes be seen on a plain chest x-
ray. On an upright posteroanterior (PA) chest x-ray (A), a mass with an air/fluid
level within it can be seen behind the heart (large arrows). This also can be
easily seen on the lateral chest x-ray (B).
Eventration of the diaphragm.
(B)PA film showing dome-like configuration to anterior aspect of right
diaphragm. Note the importance of the lateral film
(C)demonstrating the normal position of the right posterior costophrenic recess.
A, Paralysis of right hemidiaphragm. Frontal radiograph shows elevation of
the right diaphragm associated with right basilar atelectasis. Note that the
frontal view of the chest shows that the diaphragm has a smooth, rounded
contour with its peak (arrow) in the center of the right hemithorax. Opposite
diaphragm is in normal position (arrowheads).
B, Subpulmonic effusion. In subpulmonic effusion, the elevated right
hemidiaphragm has a peak that is more lateral (arrow), a feature that helps to
distinguish subpulmonic effusion from diaphragmatic paralysis.
Traumatic rupture of the
diaphragm diagnosed 1
year after the trauma.
The supine chest
radiograph(A) immediately
after the trauma shows
bilateral pleural effusions
and a poorly-defined
opacity at the right lung
base. 　　　　　　　　　
Two months after the
trauma the chest
radiograph is normal (B)
but 1 year later a focal
bulge on the right
hemidiaphragmatic contour
appears suggesting rupture
and herniation (C). 　　
MRI (D) confirmed the
diagnosis of diaphragmatic
rupture and shows the
interrupted diaphragm
(arrows) and the herniated
liver tissue (L).
Oesophageal atresia. Supine chest radiograph shows orogastric tube curled
in the proximal oesophageal pouch.
食道閉鎖手術後併發症
H-type TOF. Upper GI contrast study shows the
fistula running obliquely at the level of the thoracic
inlet.
Technique of tracheostomy
氣管切開手術
A, With the patient’s neck extended and
centered in the midline, a short horizontal
incision is made over the second or third
tracheal ring after the level of the cricoid
cartilage has been carefully palpated.
B, After horizontal division of the platysma,
the strap muscles are separated in the
midline, the cricoid is identified, and the
thyroid isthmus usually is divided and
sutured to allow easy access to the second
and third tracheal rings. The second and
third rings are incised vertically.
C, Smooth thyroid pole retractors are used
to spread the opening in the trachea. The
endotracheal tube is withdrawn to a point
just above the incision. The tracheostomy
tube is introduced with a small amount of
water-soluble lubricant and with its large-
volume cuff collapsed. The endotracheal
airway is not removed until it is
demonstrated that the tracheostomy tube is
properly seated and permits suitable gas
exchange. The flange of the tracheostomy
tube is both sutured to the skin and tied
with the usual tapes around the neck.
Tracheostomy tube (top) with inner cannula and attached ambu bag adaptor
(middle) and obturator (bottom)
A, Commercial device used in performing a cricothyrotomy with a modified
Seldinger technique. The adult Pertrach percutaneous emergency airway
provides a means of rapidly inserting a tube into a patient's trachea (through
either the cricothyroid membrane or trachea). It provides an adequate airway
that can, by its standard 15-mm adapter, be used to allow the patient to
breathe. The pediatric device is provided uncuffed in three sizes for infants
and children of various sizes. (Tracheostomy only is recommended in children
and infants.)
B, Method of establishing an airway with the Pertrach cricothyrotomy kit: (1)
The 14-ga needle is inserted into the trachea through a cricothyroid membrane
or upper trachea. Placement in the airway is verified by drawing air. (2) The
dilator is placed to its hilt into the tracheostomy tube, and both are inserted as
a single unit. The leader of the dilator is inserted into the trachea through the
needle, which is then split and removed. The dilator and trachea tube are
inserted into the trachea. (3) The dilator is removed. The cuff can then be
inflated and a respirator applied.
Pleural effusion in a supine patient. In this supine patient a right-sided
effusion produces a veil-like opacity in the lower chest through which preserved
lung vessels can be seen. In addition the diaphragm is ill-defined, the
costophrenic angle blunted and there is an apical cap (arrow).
Sonographic appearance (A) and
schematic representation (B) of a pleural
effusion. Fluid will appear as an anechoic
area over the brightly echogenic diaphragm.
The collapsed lung can usually be seen as
a wedge-shaped echogenic structure
moving with respirations within the fluid.
Note that the sonographic image has been
rotated 90° clockwise to match the
schematic drawing.
To insert a chest tube, the patient is placed semi-erect with the ipsilateral
shoulder abducted and preferably restrained. Supplemental oxygen is
recommended.
Technique for chest tube thoracotomy
2. 下列何者不位於縱膈腔 (mediastinum ）內？

　　 (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

(C) 病童仰臥並將頭部抬高至少 30 度，可改善胃液逆流至

氣管

(D) 病童發生率以男嬰為多，男女比率為三比一