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Pediatric surgery > Esophageal atresia > Flashcards

Esophageal atresia Flashcards

1
Q

congenital malformation of the esophagus

A

Incidence: 1:4000 births

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2
Q

Embryology

4th week:

A 
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
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3
Q

esophageal atrezia pathogenesis?

A
  • unclear explanation

- possible: local vascular deficit

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4
Q

EA Genetic factor

A
  • families known with more members
    having EA
  • 9% of twins with EA
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5
Q

Esophageal malformations classification

- Gross -

A

▪ 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
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6
Q

Associated anomalies: 50-70%!!!

A

▪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%

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7
Q

EA with distal TEF – clinical signs

A 
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
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8
Q

EA with distal TEF - diagnosis

A

▪ Prenatally - ultrasonography!
▪ Failure of nasogastric tube insertion
▪ X-ray - gastric air

  • associated pulmonary malformations
  • contrast agent +/-

▪ esophagoscopy?
▪ bronchoscopy ?

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9
Q

EA with distal TEF – differential diagnosis

A

▪ Meningeal hemorrhage and neonatal anoxia –
swallowing disorders

▪ Functional defects in breathing-swallowing coordination

▪ Esophageal diverticula

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10
Q

EA+distal TEF – preoperatory attitude

A

▪ Continuous aspiration
▪ Postural treatment

▪ Broad spectrum antibiotic
▪ vit. K (hypoprotrombinemia of the newborn)
▪ Hydro-electrolytic balance
▪ Venous access by central catheter

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11
Q

transpleural
- extrapleural

TEF ligation + E-E anastomosis

A

Critical situations: - gastrostomy

  • continuos aspiration
    then: TEF ligations + EE anastomosis
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12
Q

● if the distance between the two

esophageal ends:

The best esophagus is the patient’s own esophagus!

A

▪ 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

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13
Q

Delayed primary anastomosis techniques

A

▪ Howard – bougienage of the superior end

▪ Bianchi – multi-staged
superior end stretching

▪ Puri – spontaneous esophagus growth !!!

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14
Q

Esophageal substitution procedures

A

▪ gastric tube - GAVRILIU
▪ gastric transposition - SPITZ
▪ jejunal interposition
▪colon esophagoplasty

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15
Q

SOS SOS SOS
Esophageal substitution procedures
Postoperative complications

A
  1. Anastomotic fistula
  2. Anastomotic stenosis
  3. TEF recurrence
  4. Swallow disorders
  5. Gastroesophageal reflux
  6. Esophageal motility disorders, dysphagia
  7. Thoracic wall deformities
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16
Q

Prognosis

Waterston - 1962

A

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

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17
Q

Prognostic

Spitz - 1994

A

I ≥ 1500 gr, major CCM (-) , survival 97%
II < 1500 gr or Major CCM (+) , survival 59%
III <1500 gr and Major CCM (+) , survival 22%

18
Q

Congenital diaphragmatic hernia (CDH)

Definition

A

congenital diaphratic defect&raquo_space;
migration of the abdominal viscera in the
thorax

Incidence:
1:4000 births

19
Q

CDH Embriology

A

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

20
Q

CDH Possible herniation spots:

A
  1. retrosternal
  • right: foramen MORGAGNI
  • left: slit LAREY
  1. posterolateral – BOCHDALEK
  2. esophageal hiatus

!: herniation stage overlaps a very important stage in respiratory tree development when – bronchic tree and pulmonary arteries development (Week IV-XVI)

21
Q

CDH
90% of the cases – LEFT defect.
Causes:

A
  • 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

22
Q

CDH Physiopathology

A 
↓ Lung mass
↓ Surfactant
↓ Pulmonary compliance
Pulmonary a. hypoplasia 
↓
Oxigenation ↓
CO2 discharge ↓
↓  
Hypoxia
Acidosis
↓                                              ↓↑
↑ Pulmonary a. pressure    Left-right severe shunt
23
Q

CDH Physiopathology

Vasoactive substances

A

prostaglandins
tromboxanes
leucotrienes

Cardiac function and ↓ cardiac outpout worsens
the clinical presentation

24
Q

Fiziopatologie - 3

A 
Ventilation in
↑pressures
↓
Iatrogenic barotrauma
↓
Fragile alveola breaking
↓
Emphysema
↓
↑ Pulmonary tension
25
Q

CDH

Without surgical intervention:

A
  1. Pulmonary hypoplasia ISN’T 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%
26
Q

CDH

Clinical presentation. Diagnosis

A

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

27
Q

CDH
Radiologic examination
Thoraco-abdominal plain x-ray

A
  1. hydroaeric images
  2. deviation of the heart and trachea
  3. grey abdomen, opaque
28
Q

CDH
Xray examination
Contrast substance use

A

Differential diagnosis for pulmonary congenital malformations

29
Q

CDH

Associated anomalies – 30%

A
  1. Cardiac (hypoplasia) – most frequent
  2. Genitourinary
  3. Gastrointestinal
  4. CNS
  5. Skeletal

CDH can associate pulmonary sequestration in
the hernia sac wall

30
Q

CDH

Tratament

A

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

31
Q

Medical care in pulmonary hypertension:

A
  • 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%

32
Q

Stabilization of the CDH patient

A
  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)
33
Q

CDH

Observations

A

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

34
Q

CDH

Surgical treatment

A

abdominal approach
Large defect:
- prosthetics
- inverted latissimus dorsi flap

35
Q

CDH

Prognostic:

A

reserved

36
Q

CDH

Complications

A
  • gastroesophageal reflux
  • pulmonary complications
  • mechanical occlusion, volvulus, relapse
37
Q

Retrosternal hernia

A 
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.

38
Q

Hiatal hernias

A 
AKERLUND classification:
1. brachioesophagus
2. paraesophageal hernias
3. sliding hernias:
 most frequent in children
 they also gather – cardioesophageal relaxation
 – mobile cardia
39
Q

Hiatal hernias

clinical picture

A

▪ Vomiting – with blood – secondary anemia
▪ Retrosternal pain
▪ Hiccupe
▪ Dyspnea
▪ Cough
▪ Aspiration pneumopathy
▪ Peptic esophagitis → cardioesophageal stenosis

40
Q

Hiatal hernias

Radiologic examination

A

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

41
Q

Hiatal Hernias

Treatment

A
  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

Pediatric surgery (11 decks)

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