Post on 31-May-2015
description
Congenital diaphragmatic herniaEMERGENCY PEDIATRIC SURGERY Topic
Reviewed and presented byPatinya YutchawitTritraporn sawantranon
There are two ways to live:
you can live as if nothing is a miracle;
you can live as if everything is a miracle.
Albert Einstein
Objective
Describe the clinical features and the principles of treatment of a neonate with congenital diaphragmatic hernia.
Describe the clinical features of a diaphragmatic hernia in the newborn.
Describe the surgical anatomy of a diaphragm. Indicate the sites of its potential pathological defects.
Part I : Introductionconceptsanatomy ,pathophysiology Dx (Pre-peri-postnatal) ,DDx
Mr.Patinya Yutchawit
CDH ?
Congenital diaphragmatic hernia (CDH) is a developmental defect of the diaphragm that allows abdominal viscera to herniate into the chest.
Incidence
1 : 2000-5000 live birth
8 % of all major congenital anomalies
mortality rate nearing 70 percent
CDH accounts > 1% of total infant mortality in USA
Cost per new case CDH = 250 000 $
Embryology
Pleuroperitoneal membranes
Embryology
Diaphragm เจริญมาจาก 4 ส่วน คือ
1. septum transversum เจริญไปเป็นส่วน ventral ( wk 3-5 )
2. mesentery of foregut เจริญไปเป็นส่วน posterior ( wk 8 )
3. กลา้มเน้ือของผนงัทรวงอกไปเป็นส่วน lateral
4. pleuroperitoneal canal ปิด wk 9 เป็นส่วน posterolateral
Anatomy of diaphragm
ประกอบด้วย fibromuscular
4 ส่วน
- central tendon
- sternal compartment
- costal compartment
- lumbar compartment
Type of CDH
1. ผา่นรูเปิดปกติ เช่น esophageal hiatus hernia
2. ผา่นรูเปิดผดิปกติ เช่น Morgagni hernia และ Bochdalek
Bochdalek henia >>> pleural cavity
Bochdalek hernia
Morgagni hernia
LEFT POSTERO-LATERAL DIAPHRAGMATIC FORAMEN
Department of Surgery
MD. KKU. 2012
MORGAGNI FORAMENDepartment of Surgery
MD. KKU. 2012
Causes
The cause of CDH is largely unknown
CDH can occur as part of a multiple malformation syndrome in up to 40% of infants (cardiovascular, genitourinary, and gastrointestinal malformations)
Karyotype abnormalities have been reported in 4% of infants with CDH, and CDH may be found in a variety of chromosomal anomalies including trisomy 13, trisomy 18, and tetrasomy 12p mosaicism
Pathology of CDH
Failure of closure of pleuro-peritoneal canal
Most common area is a postero-lateral defect
( Bochdalek )
Left side more common (8:1)
Pathology of CDH
Diaphragmatic defect
Abdominal viscera fill the chest cavity
Abdomen small & poorly developed
Pathology of CDH
Both lungs hypoplastic
More so on the ipsilateral side
Pulmonary vessels hypoplastic Persistent Pulmonary Hypertension of the Newborn ; PPHN
Pathology of CDH
Following delivery
Bowels fill with air
Compression of ipsilateral lung
Mediastinal shift
Compression of contralateral lung
mechanical compressionof lung
Pathology of CDH
Lung hypoplasia
PPHN Mechanical compression
Respiratory distress
Lung development in CDH
No. of bronchial branches – greatly reduced
Alveolar development severely affected
Increased muscle mass in the conducting airways
Seen in contra lateral lung too
Pulmonary vasculature in CDH
Reduction in the total no. of branches
Both in ipsilateral and contra lateral lungs
Significant adventitial and medial wall thickening
Increased susceptibility to PPHN
hypoxia, acidosis, hypothermia, stress
Persistent fetal circulation
pulmonary
artery
resistance
pulmonary
artery
pressures
pulmonary
vascular flowRight to left shunting
Hypoxia &Progressive
desaturation
Respiratory failure
Clinical presentation
NEW BORN• Respiratory distress• Scaphoid abdomen• Diminished breath sound• Shift mediastinum• Gurgling sound in thorax
CHILD• Abdominal pain• Vomiting• Bowel complication :
- gut obstruction - bowel ischemia
20% of cases may have antenatal Hx. of polyhydramnios
Diagnosis
Prenatal diagnosis
Sensitivity is higher when there are
associated abnormalities, higher GA, and
experienced ultrasonographer
Mean GA at diagnosis: 24 weeks
Diagnosis—prenatal DxUltrasound Findings
Left CDH: heterogeneous mass in chest,
right mediastinal shift, fluid filled stomach
in chest, peristalsis of fluid filled bowel,
may also have homogeneous mass
continuous with intra-abdominal liver
Right CDH: homogeneous mass (liver) in
the right chest left mediastinal shift,
pleural fluid, bowel present, gallbladder in
chest is diagnostic of right side CDH
Prognostic factors
Karyotype
Liver herniation
Lung area to head circumference ratio
Lung volume
Associated anomalies (25-50%/95%)
Diagnosis
Peri-Postnatal diagnosis
ARDS in few hours with absent of breath sound
+ Clinical features (Remember???)
>> Child ? / New born?
Differential diagnosis
Diaphragmatic eventration : less severe effects
on lung, rare
Congenital cystic adenomatoid malformation
Bronchopulmonary sequestration
Bronchogenic cysts
Bronchial atresia
Enteric cycts
Teratomas
Part II : Investigation and management Miss.Tritraporn sawantranon
Investigation
Chest x-ray
Investigation
Ultrasound
Investigation
CT scan of thorax
Investigation
UGI study
Treatment
เป็นภาวะฉุกเฉินทางกมุารศลัยศาสตร์
การผา่ตดัเร่งด่วนไม่สามารถเพิ่มอตัราการรอดชีวติของทารกได้
การเร่งรีบผา่ตดัท าใหอ้ตัราตายสูงข้ึน
เม่ือทารกท่ีเป็น CDH จะเขา้การผา่ตดัตอ้งไดรั้บการฟ้ืนฟแูละแกไ้ขภาวะ hypoxia และ cardiovascular instability ก่อน (pre-op stabilization)
Pre-op stabilization
OG or NG tube
Exogenous surfactant
Inhale NO
Pre-op stabilization
Machanical ventilator High frequency ventilator : 60 – 120 คร้ังต่อนาทีO2 sat > 90% PaCO2 < 60 mmHg
ECMO ( extracorporeal membrane oxygenator )
Pre-op stabilization
Maintain reference range of glucose and calcium
Support BP : inotropic drugs , adequate circulating volume
Operational treatment
Trans abdominal approach
Reduction of visceral hernia
Closure diaphragmatic defect
Prepare for refer
Respiratory support
OG tube decompression
Semi-upright position
Intravenous fluid
Keep warm
Part III : Wrap up
Mr.Patinya Yutchawit
Miss.Tritraporn sawantranon
Case Male, 36 week GA, 30 yr. old G1P0
SVD
Apgars 31,75,910, ETT in DR
BW – 2,700 g
Normal pregnancyU/S – 28 week CDH
Amnio – Normal, 46XY
On examScaphoid abdomen
Bowel sounds over left chest
CXR
What’s next to do?
Summary and take home key points1. Congenital diaphragmatic hernia is a developmental defect in the diaphragm that allows abdominal viscera to herniate into the chest, thereby interfering with normal lung development. Pulmonary hypoplasia and pulmonary arterial hypertension can result in life-threatening respiratory compromise. Barotrauma and hypoxia should be avoided.
2. Prenatal diagnosis is based upon ultrasound examination. The principal findings include a chest mass (which may exhibit peristalsis) and mediastinal shift, in the setting of malposition of the stomach and/or liver.
3. Chromosomal abnormalities and structural malformations in other major organ systems (eg, congenital heart disease, neural tube defects) are often associated with congenital diaphragmatic hernia.
Summary and take home key points4. Prognosis is worse in the setting of an abnormal karyotype, severe associated anomalies, right-sided defect, liver herniation, and lower fetal lung volume. [Prognostic factors] Absence of liver herniation is a strong predictor of postnatal survival.
5. The prenatal assessment of patients with suspected congenital diaphragmatic hernia should include: fetal karyotype, ultrafast fetal magnetic resonance imaging to look for liver herniation, echocardiography, serial ultrasound examinations, and antepartum fetal testing.
6. Fetal endoscopic tracheal occlusion is an investigational procedure for treatment of isolated severe congenital diaphragmatic hernia. It obstructs the normal egress of lung fluid during pulmonary development, resulting in increased transpulmonic pressure and large fluid-filled lungs, which can prevent the abnormal development of lung parenchyma and pulmonary vasculature.
Reference
The developing human ,8th Edition
Schwartz's Principles of Surgery, 9e
UpToDate : Congenital diaphragmatic hernia in the neonate
BMJ Best practice : Hiatal hernia
Slides : Department of Surgery KKU – CDH
Ass. Prof. Dr Osama Bawazir – CDH
Weili Chang M.D. – CDH
T h a n k y o u ! ! Any question ???