This document discusses congenital diaphragmatic hernia and tracheoesophageal fistula. It begins by defining congenital diaphragmatic hernia and describing the different types. It then discusses the history, embryology, pathophysiology, classification, clinical manifestations, diagnosis, treatment including surgical repair, complications, and prognosis of congenital diaphragmatic hernia. It also defines tracheoesophageal fistula, describes the different types, discusses embryology, pathophysiology, clinical presentation, diagnosis, associated anomalies, preoperative preparation, surgical repair techniques, and complications.

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