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.
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.
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.
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
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
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
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%)
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
32.
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
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
37.
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
39.
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.