Esophageal atresia Flashcards
congenital malformation of the esophagus
Incidence: 1:4000 births
Embryology
4th week:
laringo-tracheal bud; -> tracheo-esophageal septum; -> separation of the diverticulum by 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
EA 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
▪ EA with distal TEF (85%)
▪ EA with distal and proximal TEF
▪ TEF without AE – H-type (3 - 5%)
▪ membranous atresia
▪ esohageal stenosis
* 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
1▪ polihydramnios – raises suspicion of digestive tube atresia – more frequent in pure EA 2▪ bubbly-like saliva and mucus (foam aspect) 3▪ noisy breathing 4▪ cianosis 5▪ feeding attempt: - suffocation - cianosis - cough 6▪ 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
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 patient’s 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 – multi-staged
superior end stretching
▪ Puri – spontaneous esophagus growth !!!
Esophageal substitution procedures
▪ gastric tube - GAVRILIU
▪ gastric transposition - SPITZ
▪ jejunal interposition
▪colon esophagoplasty
SOS SOS SOS
Esophageal substitution procedures
Postoperative complications
- Anastomotic fistula
- Anastomotic stenosis
- TEF recurrence
- Swallow disorders
- Gastroesophageal reflux
- Esophageal motility disorders, dysphagia
- Thoracic wall deformities
Prognosis
Waterston - 1962
I > 2500 gr, pneumonia (-) ,Associatied anomalies (-),survival 95%
II 1800 – 2500 gr,Moderate pneumonia OR,Associated anomalies 68%
III < 1800 gr OR > 1800 gr , survival 6%
Severe pneumonia
Severe anomalies
Prognostic
Spitz - 1994
I ≥ 1500 gr, major CCM (-) , survival 97%
II < 1500 gr or Major CCM (+) , survival 59%
III <1500 gr and Major CCM (+) , survival 22%
Congenital diaphragmatic hernia (CDH)
Definition
congenital diaphratic defect»_space;
migration of the abdominal viscera in the
thorax
Incidence:
1:4000 births
CDH Embriology
4 components are responsible of diaphragmatic development:
- Septum transversum
- Pleuroperitoneal membranes
- Dorsal mesoesophagus
- Thoracic wall
Week V-X: pleuroperitoneal canal closure defect
~10th week: reintegration of the intestine finds the defect – thoracic migration
CDH Possible herniation spots:
- retrosternal
- right: foramen MORGAGNI
- left: slit LAREY
- posterolateral – BOCHDALEK
- esophageal hiatus
!: herniation stage overlaps a very important stage in respiratory tree development when – bronchic tree and pulmonary arteries development (Week IV-XVI)
CDH
90% of the cases – LEFT defect.
Causes:
- Latent closure of the pleuroperitoneal duct
- Hepatic protection on the right
90% of the cases – LEFT defect.
10-20% of the cases: peritoneal hernia sac existence
CDH Physiopathology
↓ Lung mass ↓ Surfactant ↓ Pulmonary compliance Pulmonary a. hypoplasia ↓ Oxigenation ↓ CO2 discharge ↓ ↓ Hypoxia Acidosis ↓ ↓↑ ↑ Pulmonary a. pressure Left-right severe shunt
CDH 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 ↓ Emphysema ↓ ↑ Pulmonary tension
CDH
Without surgical intervention:
- Pulmonary hypoplasia ISN’T REVERSIBLE on short
term. - Pulmonary compliance gets better in the first week of life.
- The lack of surfactant can be managed by aggressive treatment
- Pulmonary hypertension can be reversible – in the
first week of life the pressure in pulmonary a. can
decrease up to 50%
CDH
Clinical presentation. Diagnosis
3 classic situations:
- Severe respiratory failure after birth, requiring intensive support.
- Minimal signs and symptoms.
- 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
CDH
Radiologic examination
Thoraco-abdominal plain x-ray
- hydroaeric images
- deviation of the heart and trachea
- grey abdomen, opaque
CDH
Xray examination
Contrast substance use
Differential diagnosis for pulmonary congenital malformations
CDH
Associated anomalies – 30%
- Cardiac (hypoplasia) – most frequent
- Genitourinary
- Gastrointestinal
- CNS
- Skeletal
CDH can associate pulmonary sequestration in
the hernia sac wall
CDH
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
- Nasogastric decompression
- Sedation and paralysis
- ↓ air swallowing
- ↑ compliance
- ↓ sympathetic vasoconstriction - Ventilation – as gentle as possible
- Surfactant – unclear restults
- Pulmonary vasodilators
- unselective – unclear
- selective – NO (nitric oxide)
CDH
Observations
Surgery must wait as long as possible in order to
decrease pulmonary hypertensions and raise pulmonary compliance
Therapeutic alternative: lung transplant
chirurgia fetală – percutaneous trachea occlusion
CDH
Surgical treatment
abdominal approach
Large defect:
- prosthetics
- inverted latissimus dorsi flap
CDH
Prognostic:
reserved
CDH
Complications
- 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
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
Hiatal hernias
clinical picture
▪ Vomiting – with blood – secondary anemia
▪ Retrosternal pain
▪ Hiccupe
▪ Dyspnea
▪ Cough
▪ Aspiration pneumopathy
▪ Peptic esophagitis → cardioesophageal stenosis
Hiatal hernias
Radiologic examination
Gastroesophageal reflux
▪ Easy to see dilation on plain x-ray
▪ Contrast substance
Hiatal Hernias
Treatment
- Medical and postural - good results, tried first before surgery
- Surgery
Objectives:
1abdominal reduction hernia 2diaphragm reconstruction
3` anti-reflux procedure
- NISSEN fundoplication– wrapping the gastric
fundus around the terminal esophagus reinforcing
the closing function of the lower esophageal
sphincter
