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Incidence:

1:4000 births

Brief history:
1670: W. Durston first description of an EA
1696: T. Gibson first description of an EA with TEF
1939: Ladd i Leven first doctors who treated using

multi-staged surgery and EA with TEF


1941: Cameron Hight first success in treating EA with

TEF using a one-stage procedure

Embryology

4th week:
laringo-tracheal bud;
tracheo-esophageal
septum;

separation of the
diverticulumby the
primitive foregut, in a
cranial-caudal direction

the eso-tracheal separation process stops earlier


tracheo-esophageal fistula = TEF

the absence of this process


laryngo-tracheo-esophageal fistula

esophageal atrezia pathogenesis?


- unclear explanation
- possible: local vascular deficit

Genetic factor
- families known with more members
having EA

- 9% of twins with EA

Esophageal malformations
classification
- Gross EA without TEF (6-8%)

EA with proximal TEF

Esophageal malformations
classification
- Gross EA with distal TEF (85%)

EA with distal
and proximal TEF

Esophageal malformations
classification
- Gross -

TEF without
AE H-type (3
- 5%)

esohageal stenosis
membranous atresia

* there are also described:

complete absence of the esophagus


eso-bronchial fistual
esophageal duplication
laryngo-tracheo-esophageal fistula

Associated anomalies: 50-70%!!!


cardiac 30%: - patent arterial duct
- ventricular septal defect
- atrial septal defect
gastrointestinal 12%:
- anorectal malformations most frequent
- duodenal atresia
- annular pancreas
- pyloric stenosis
neurologic 5%
genitourinary 5%
skeletal 2%
V.A.C.T.E.R.L. association - 25%

EA with distal TEF clinical signs


polihydramnios raises suspicion of digestive tube
atresia more frequent in pure EA
bubbly-like saliva and mucus (foam aspect)
noisy breathing
cianosis
feeding attempt:
- suffocation
- cianosis
- cough
progressive bloating

EA with distal TEF - diagnosis

Prenatally - ultrasonography!
Failure of nasogastric tube insertion
X-ray
- gastric air
- associated pulmonary
malformations
- contrast agent +/ esophagoscopy?
bronchoscopy ?

EA with distal TEF differential diagnosis

Meningeal hemorrhage and neonatal anoxia

swallowing disorders
Functional defects in breathing-swallowing

coordination
Esophageal diverticula

EA+distal TEF preoperatory attitude


Continuous aspiration
Postural treatment

Broad spectrum antibiotic


vit. K (hypoprotrombinemia of the newborn)

Hydro-electrolytic balance
Venous access by central catheter

EA and distal TEF


- surgical procedure -

Approach

- transpleural
- extrapleural

TEF ligation + E-E anastomosis

Critical situations: - gastrostomy


- continuos aspiration
then:
TEF
ligations
+
EE
anastomosis

if the distance between the two


esophageal ends:

The best esophagus is


the patients own esophagus!

miotomy at the superior esophageal end


a) circular LIVADITTI

b) spiral KIMURA

Tubing a flap from the superior end


tension anastomosis and elective palsy with assisted
ventilation

Small gastric curvature


stretching SCHARLI

Delayed primary
anastomosis techniques
Howard bougienage of the
superior end

Bianchi multistaged superior end


stretching

Puri spontaneous esophagus growth !!!

Esophageal substitution procedures


gastric tube GAVRILIU

gastric transposition - SPITZ

Esophageal substitution procedures


jejunal interposition

colon esophagoplasty

Postoperative complications
Anastomotic fistula

Anastomotic stenosis
TEF recurrence
Swallow disorders
Gastroesophageal reflux
Esophageal motility disorders, dysphagia

Thoracic wall deformities

Prognosis
Waterston - 1962
I

II

III

> 2500 gr
pneumonia (-)
Associatied anomalies (-)
1800 2500 gr
Moderate pneumonia OR
Associated anomalies
< 1800 gr OR
> 1800 gr
Severe pneumonia
Severe anomalies

survival
95%

68%

6%

Prognostic

Spitz - 1994

survival

1500 gr
major CCM (-)

97%

II

< 1500 gr or
Major CCM (+)

59%

III

< 1500 gr and


Major CCM (+)

22%

Congenital diaphragmatic hernia


(CDH)

Definition

congenital diaphratic defect >>

migration of the abdominal viscera in the


thorax

Incidence:
1:4000 births

Brief history:
1679 Riverius first description of a CDH
1796 Morgagni describes the anterior defect in CDH
1848 Bochdalek describes the postero-lateral defect in CDH

1946 Gross first successful treatment of CDH at a neonate


1971 Rowe i Uribe persistent pulmonary hypertension at
newbord is associated with CDH

1976 Barlett first CDH survivors successfully treated with ecmo


ECMO

Embriology
4 components are responsible of diaphragmatic development:
1. Septum transversum
2. Pleuroperitoneal membranes
3. Dorsal mesoesophagus
4. Thoracic wall

Week V-X: pleuroperitoneal canal closure defect


10th week: reintegration of the intestine finds the defect
thoracic migration

Possible herniation spots:


1. retrosternal
- right: foramen MORGAGNI
- left: slit LAREY

2. posterolateral BOCHDALEK
3. esophageal hiatus

!: herniation stage overlaps a very important stage in respiratory


tree development when bronchic tree and pulmonary arteries
development (Week IV-XVI)

90% of the cases LEFT defect.

Causes:
- Latent closure of the pleuroperitoneal duct
- Hepatic protection on the right

10-20% of the cases: peritoneal hernia sac existence

Physiopathology
Lung mass
Surfactant

Pulmonary compliance

Oxigenation
CO2 discharge

Pulmonary a. hypoplasia
Hypoxia
Acidosis

Pulmonary a. pressure

Left-right severe shunt

Physiopathology
Vasoactive substances:

prostaglandins
tromboxanes

leucotrienes
Cardiac function and cardiac outpout worsens

the clinical presentation

Fiziopatologie - 3
Ventilation in
pressures

Iatrogenic barotrauma

Fragile alveola
breaking

Pulmonary
tension
Emphysema

Without surgical intervention:


1. Pulmonary hypoplasia ISNT REVERSIBLE on short
term.
2. Pulmonary compliance gets better in the first week of
life.
3. The lack of surfactant can be managed by aggressive

treatment
4. Pulmonary hypertension can be reversible in the

first week of life the pressure in pulmonary a. can


decrease up to 50%

Clinical presentation. Diagnosis


3 classic situations:

1.Severe respiratory failure after birth,


requiring intensive support.
2.Minimal signs and symptoms.
3.Different levels of respiratory failure in
the first day of life.

Varied levels of dyspnea and cyanosis


Excavated abdomen
Vomiting
No lung sounds on the affected hemithorax
Abnormal sounds of the lung hydro-aeric
Abnormal cardiac projection area

Radiologic examination
Thoraco-abdominal plain x-ray

hydroaeric images
deviation of the heart and
trachea
grey abdomen, opaque

X-ray examination
Contrast subtance use:

Differential diagnosis for pulmonary congenital malformations

Associated anomalies 30%


1.
2.
3.
4.
5.

Cardiac (hypoplasia) most frequent


Genitourinary
Gastrointestinal
CNS
Skeletal

CDH can associate pulmonary sequestration in

the hernia sac wall

Tratament
-

Initially, surgical treatment was thought to be the priority.

Postoperative, it was observed that after a short period of good


evolution (honey-moon) severe respiratory imbalance

Overall mortality: 50%!

First of all pulmonary hyoplasia and


pulmonary hypertension treatment

Medical care in pulmonary hypertension:


- tolazolin

- nitroglycerin
- nitroprusside
- prostaglandines
wanted side effects (pulmonary HYPO tension).
High frequency with low pressures ventilations
Extracorporeal membrane oxygenation (ECMO)
- cardiopulmonary by-pass, most frequently venous-arterial, carotid a.
and jugulary v.
- ECMO using in severe cases of superior digestive bleeding can
decrease mortality from 80% to 60%

Stabilization of the CDH patient


1. Nasogastric decompression
2. Sedation and paralysis
- air swallowing
- compliance
- sympathetic vasoconstriction
3. Ventilation as gentle as possible
4. Surfactant unclear restults
5. Pulmonary vasodilators
- unselective unclear
- selective NO (nitric oxide)

Observations
Surgery must wait as long as possible in order to
decrease pulmonary hypertensions and raise
pulmonary compliance

Therapeutic alternative: lung transplant


chirurgia fetal percutaneous trachea occlusion

Surgical treatment

abdominal approach

Large defect:
- prosthetics

- lambou inversat de latissimus dorsi

Prognostic:
- rezervat

Complicaii:
- gastroesophageal reflux
- pulmonary complications
- mechanical occlusion,

volvulus, relapse

Retrosternal hernia
dimension
Discreet clinical signs respiratory and/or digestive
Herniated gut strangulation
- pain
- vomiting
- bloating
- hiccups
- no stools or gas
- retrosternal or prehepatic sounds

Retrosternal hernias
Anterior-posterior position and profile plain X-ray

Surgical treatment, good prognosis.

Hiatal hernias

AKERLUND classification:
1. brachioesophagus

2. paraesophageal hernias
3. sliding hernias:
most frequent in children
they also gather cardioesophageal relaxation
mobile cardia

Tablou clinic
Vomiting with blood secondary anemia

Retrosternal pain
Hiccupe
Dyspnea
Cough
Aspiration pneumopathy

Peptic esophagitis cardioesophageal stenosis

Radiologic examination
Gastroesophageal reflux
Easy to see dilation on plain x-ray
Contrast substance

Treatment
1. Medical and postural
- good results, tried first before surgery
2. Surgery
Objectives:
1` abdominal reduction hernia
2` diaphragm reconstruction
3` anti-reflux procedure
- NISSEN fundoplication wrapping the gastric
fundus around the terminal esophagus reinforcing
the closing function of the lower esophageal
sphincter