Congenital diaphragmatic hernia and tracheo esophageal fistula

1. CONGENITAL DIAPHRAGMATIC
HERNIA
AND
TRACHEO ESOPHAGEAL
FISTULA
Presentor- Dr. Bikramjit Singh Jafr
Moderator- Dr. Anand Khushwaha

2. CONGENITAL DIAPHRAGMATIC
HERNIA

3.  A diaphragmatic hernia is defined as a
communication between the abdominal and thoracic
cavities with or without abdominal contents in the thorax.
◦ Congenital
◦ Traumatic
 Location of the defect
◦ Esophageal hiatus (hiatal)
◦ Paraesophageal (adjacent to the hiatus)
◦ Retrosternal (Morgagni)
◦ At the posterolateral (Bochdalek) portion of the
diaphragm

4. HISTORY
 1679-LAZARUS first described in postmorterm
examination of 24 yr old man.
 1888-The first attempt at surgical repair by
Nauman of Sweden in 19 yrs old.
 1940- first successful repair of neonatal hernia
within 24 hrs of birth byWilliam Ladd and
Robert Gross.

5. EMBRYOLOGY

6. PATHOPHYSIOLOGY
It involves three major defects
 A failure of the diaphragm to completely close
during development.
 Herniation of the abdominal contents into
the chest.
 Pulmonary hypoplasia.

7. Classification
• Diaphragmatic hernia
◦ Posterolateral L >R
(Bochdalek) 80%
◦ Anterior (Morgagni) 2%
◦ Paraesophageal 15-20%
• Eventration (15 - 20%)
• Absent diaphragm : rare

8. Bochdalek hernia

9. ASSOCIATED ANOMALIES
 GIT
◦ Malrotation of gut
(almost always)
◦ Esophageal atresia,
Omphalocele
 CNS lesions
 Cardiovascular
lesions

10. CLINICAL MENIFESTATIONS
EARLY PRESENTATION
 Triad-
◦ Dyspnea
◦ Cyanosis
◦ Apparent Dextrocardia
 RD- within 6hrs
-after 48 hrs
(honeymoon period)
 Scaphoid abdomen
 Inc. chest wall diameter
 Dec. B/L breath sounds
 Bowel sounds in chest
 Shifted cardiac impulse
DELAYED PRESENTATION
 Vomitings
 Mild RD
 Intestinal obstruction
 Incarceration of intestine
Iscemia
Sepsis
Shock
 Sudden death
 Gp. B Strepto. Sepsis
(asso. with Rt. sided
CDH)

11. DIAGNOSIS
Prenatal:-
 Ultrasonography-(between 16 and 24 wk of gestation)
◦ Polyhydramnios
◦ chest mass
◦ mediastinal shift
◦ gastric bubble
◦ fetal hydrops
◦ liver in the thoracic cavity
◦ lung : head size ratio- may predict outcome
 High-speed fetal MRI- precisely measure lung
volume indexed to body volume

12. Postnatal:-
 Chest X-ray with NG tube,
including abdomen
◦ Mediastinal Shift
◦ Loops of bowel in chest
◦ Absent lung markings
◦ Paucity of gas in abdomen
◦ If herniated content
involves stomach- NG
tube seen in chest.
 Barrel shaped chest

13. LAB. STUDIES
 Arterial blood gas (ABG) measurements: To assess
for pH, PaCO 2 & PaO2
 Serum lactate: for assessing circulatory
insufficiency or severe hypoxemia associated with
tissue hypoxia
 Serum electrolytes, ionized calcium & glucose
 Continuous pulse oximetry- for diagnosis and
management of PPHN
 ECHO
 Chromosome studies & microarray analysis

14. DIFFERENTIAL DIAGNOSIS
 Eventration of diaphragm
 Cystic lung lesions
◦ Pulmonary sequestration
◦ Cystic adenomatoid malformation

15. TREATMENT
 PRENATAL CARE
 Councelling of parents
 Support fetus & mother
 Refer to a tertiary centre
 NVD preferred (CDH is not an indication for CS)
 Fetal intervention with in-utero correction
(experimental)
IMMEDIATE
 Intubation + Stomach Decompression

16. Pre- op care:-
Resuscitation
 ET & NG tube insertion
 Bag & Mask C/I
 Umbilical arterial &
venous access
 Maintain proper temp.,
glucose & volume
homeostasis
 Sedation with Midaz/
Fentanyl/ Morphine
 Muscle paralysis C/I
 Correct acid-base
disturbances

17.  Ventilation
◦ Conventional mechanical ventilation
◦ HFOV
◦ ECMO
(Using a combination of high respiratory rate,
modest peak airway pressure & no PEEP)
 NO
 Inotropes
 Surfactant

18. NOVEL STRATEGIES
◦ Tracheal occlusion in-utero
◦ Partial liquid ventilation
◦ Extracorporeal Membrane Oxygenation
(ECMO)

19. OPERATIVE REPAIR
Timing of Surgery
 CDH= physiologic emergency NOT surgical emergency
 The ideal time to repair the diaphragmatic defect is under
debate.
 MC = Wait at least 48 hr after stabilization and resolution
of the pulmonary hypertension
 Indicators of stability:-
◦ the requirement for conventional ventilation only
◦ a low peak inspiratory pressure
◦ Fio2 <50
 Newborn on ECMO- consider weaning off before Sx
repair

20. APPROACH
 Subcostal approach = Most Common
 If the abdominal cavity cannot accommodate the
herniated contents, a polymeric silicone (Silastic)
patch can be placed.
 In case of stable infants
◦ Laparoscopic repair
◦ Thoracoscopic repair
 Whenever possible, a primary repair using native
tissue is performed.
 If the defect is too large, a porous
polytetrafluoroethylene (Gore-Tex) patch is used.

21. COMPLICATIONS
Short Term
 Post-op adhesions
 Recurrence
◦ more in PTFE
◦ loose fitted patch
 Pulm. HT
 Abdominal Compartment
Syndrome
 Bleeding
 Chylothorax
 Bowel obstruction
Long Term
 Pulmonary HT
 Pulmonary Hypoplasia
 Volutrauma
 Need for O2 / ECMO
 BPD
 GERD
 Delayed growth
 Neurocognitive defects
◦ transient and permanent
developmental delay
◦ abnormal hearing or vision
◦ seizures
 Pectus excavatum & Scoliosis

22. POOR PROGNOSTIC INDICATORS
 Asso. Major anomaly
 Symptomatic before 24 hrs of age
 Pulmonary Hypoplasia
 Herniation to C/L lung
 Need for ECMO
 Stomach in the contents
 Liver in thoracic cavity
 Fetal- Lung: Head size ratio <1

23. FOLLOW-UP
 Failure to thrive is common
 Need for supplemental oxygen at the time of discharge
is a significant predictor for subsequent growth failure.
 Possible causes include
◦ increased caloric requirements due to chronic lung
disease
◦ oral aversion after prolonged intubation
◦ poor oral feeding due to neurologic delays
◦ gastroesophageal reflux
 Risk for CNS insult and sensorineural hearing loss,
infants should be closely monitored for the first 3 years
of life

24. Foramen of Morgagni Hernia
 Failure of the sternal and crural portions of the
diaphragm to meet and fuse
◦ Right-sided (90%) but may be B/L
◦ Contents-transverse colon/small
intestine/liver
Diagnosis
 Chest radiograph (incidental finding)
◦ Anteroposterior view- a structure behind
heart
◦ Lateral view- mass in retrosternal area
 Chest CT or MRI- confirmatory
Repair is recommended for all patients, in view
of the risk of bowel strangulation
◦ Laparoscopically
◦ Open approach
◦ Prosthetic material is rarely required

25. TRACHEO-ESOPHAGEAL FISTULA

26. TRACHEO-ESOPHAGEAL FISTULA
 mc congenital anomaly of esophagus
 Incidence: 1:3000 live births
 M > F (25:3)
 10-40% are preterm
 Antenatal history: polyhydramnios (60%)

27. EMBRYOLOGY

28. PATHOPHYSIOLOGY
 Two main pathologic entities
◦ Dehydration
◦ Aspiration pneumonitis
 EA- Aspiration-RD,Atelectasis and pneumonia
 TEF-
◦ Proximal-aspiration
◦ Distal-stomach/bowel distension and elevation of
diaphragm
 Abnormal Esophageal peristalsis
 Maldevelopment of cartilage rings
(tracheomalacia)

29. CLASSIFICATION

30. Clinical Presentation
 Choking on 1st feed
 Coughing
 Cyanosis
 Excessive salivation
 Feed regurgitation
 Aspiration pneumonia
 Dehydration
 Abdominal findings
◦ C- Distension
◦ A- Scaphoid abdomen

31. Diagnosis
 BEFORE BIRTH- Prenatal USG-A suspicion is based on the
presence of polyhydramnios and a fetal stomach that either is
absent or shows reduced filling.
 AFTER BIRTH- passage of 8 French oral tube
 Inability to pass a suction catheter into the stomach
 CXR: Coiled orogastric tube in the cervical pouch; air in the
stomach and intestine
 Dilated proximal esophageal pouch
 ATTENTION! barium swallow is inadvisible - risk of
barium aspiration
 For H- type fistula
◦ Bronchoscopy
◦ Dye test
 CT - sagittal computed tomography scan

33. ANTICIPATED PROBLEMS
 Aspiration pneumonia
 Dehydration
 Prematurity
 Immature organ system
 Increased risk of cardiac anomalies
 Increased risk of respiratory distress syndrome
 Hypoglycemia , hypothermia, dehydration
 Post-op apnea & Bradycardia

34. Associated congenital anomalies
SYNDROMIC
ASSOCIATION :-
DiGeorge sequence
Feingold syndrome
Pierre-robbin sequence
Polysplenia sequence
Holt Oram sequence
Trisomy 13,18,21
VACTERL
CHARGE
Anorectal- Triple atresia
(EA+DA+AA)

35. INVESTIGATIONS
 CBC
 Serum electrolytes
 Renal USG
 ECG/ Echo
 Chest X-Ray
 CT / MRI
 ABG

36. Timing of surgery :-
 WATERSTON group A- Immediate operative
repair
 WATERSTON group B- Delay repair
 WATERSTON group C- Staged repair
Type of surgery :-
 Thoracotomy-
◦ Extrapleural
◦ Transpleural
 VAT

38. PREPARATION
 24-48 hr medical stabilization
 NPO
 Prevention of aspiration
 Prone positioning minimizes movement of gastric
secretions into a distal fistula, and esophageal suctioning
minimizes aspiration from a blind pouch
 Pre – operative gastrostomy
 Ensure availability of blood in the OT
 Optimize pulmonary status
 Secure intravenous and arterial access
 Optimize volume status and metabolic state
 ET intubation- avoided- risk of gastric perforation & RD
as the abdomen becomes distended from ventilation

40. OPERATIVE REPAIR
 Depends on specific type
 Surgical ligation & division of TEF and primary end-to-end
anastomosis of the esophagus via right-sided thoracotomy
constitute the current standard surgical approach.
 In the premature or otherwise complicated infant, a primary
closure may be delayed by temporizing with fistula ligation and
gastrostomy tube placement.
 If the gap between the atretic ends of the esophagus is >3-4 cm,
primary repair cannot be done; options include using gastric,
jejunal, or colonic segments interposed as a neoesophagus.
 Thoracoscopic surgical repair is now considered feasible and
associated with favorable long-term outcomes.
 Careful search must be undertaken for the common associated
cardiac and other anomalies.

42. COMPLICATIONS
Early
 Anastomotic leak- 15%
 Esophageal stricture- 80% require eso.
dilatation
 Recurrent TEF
Late
 GERD with reactive airway disease- 30-70%
 Delayed gastric emptying
 Tracheomalacia

43. THANK YOU