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

呼吸系統

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 wall.

Anterior mediastinum前縱膈腔

< 2Y• Benign teratoma• Thymic hyperplsia• Cystic granuloma

> 2Y• Malignant germ cell

tumor• Thymoma• lymphoma

Middle mediastinum中縱膈腔

< 2Y• Brochogenic cyst

> 2Y• Lymphoma• Granuloma

Posterior mediastinum後縱膈腔

< 2Y• Neuroblastoma• Enterogenous cyst

> 2Y• Ganglioneuroma• sarcoma

Developmental anomalies of lung肺臟發育畸形

• Primary anomaliesAgenesis of the lungLobar underdevelopment

• Secondary anomaliesCongenital 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 cystsPulmonary 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 Maybe 3. Cardiac dysfunction ↑PVP;↓SP Yes 4. Hypoxia, hypercarbia ↑PVR R→L shunt Yes ----------------------------------------------------------------------------------------- PVR: pul. vascular resistance; PVP: pul. venous pressure; SP: sys. pressure

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. (A)PA film showing dome-like configuration to anterior aspect of right

diaphragm. Note the importance of the lateral film (B)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