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 ???