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CONGENITAL DIAPHRAGMATIC HERNIA AND TRACHEO ESOPHAGEAL FISTULA
Presentor- Dr. Bikramjit Singh JafraModerator- Dr. Anand Khushwaha
CONGENITAL DIAPHRAGMATIC HERNIA
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
HISTORY1679-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.
EMBRYOLOGY
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.
Classification
• Diaphragmatic hernia
◦ Posterolateral L >R
(Bochdalek) 80%
◦ Anterior (Morgagni) 2%
◦ Paraesophageal 15-20%
• Eventration (15 - 20%)
• Absent diaphragm : rare
Bochdalek hernia
ASSOCIATED ANOMALIESGIT
◦Malrotation of gut (almost always)
◦Esophageal atresia, Omphalocele
CNS lesionsCardiovascular
lesions
CLINICAL MENIFESTATIONSEARLY 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)
DIAGNOSISPrenatal:-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
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
LAB. STUDIESArterial 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 & glucoseContinuous pulse oximetry- for diagnosis and
management of PPHNECHOChromosome studies & microarray analysis
DIFFERENTIAL DIAGNOSISEventration of diaphragmCystic lung lesions
◦Pulmonary sequestration◦Cystic adenomatoid malformation
TREATMENT PRENATAL CARECouncelling of parentsSupport fetus & motherRefer to a tertiary centreNVD preferred (CDH is not an indication for CS)Fetal intervention with in-utero correction
(experimental)
IMMEDIATE Intubation + Stomach Decompression
Pre- op care:- Resuscitation
ET & NG tube insertionBag & Mask C/IUmbilical arterial &
venous accessMaintain proper temp.,
glucose & volume homeostasis
Sedation with Midaz/ Fentanyl/ Morphine
Muscle paralysis C/ICorrect acid-base
disturbances
Ventilation◦Conventional mechanical ventilation◦HFOV◦ECMO
(Using a combination of high respiratory rate, modest peak airway pressure & no PEEP)
NOInotropesSurfactant
NOVEL STRATEGIES
◦Tracheal occlusion in-utero◦Partial liquid ventilation◦Extracorporeal Membrane Oxygenation
(ECMO)
OPERATIVE REPAIRTiming of SurgeryCDH= physiologic emergency NOT surgical emergencyThe 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
APPROACHSubcostal approach = Most CommonIf 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.
COMPLICATIONSShort TermPost-op adhesionsRecurrence
◦ more in PTFE◦ loose fitted patch
Pulm. HTAbdominal Compartment
SyndromeBleedingChylothoraxBowel 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
POOR PROGNOSTIC INDICATORSAsso. Major anomalySymptomatic before 24 hrs of agePulmonary HypoplasiaHerniation to C/L lungNeed for ECMOStomach in the contentsLiver in thoracic cavityFetal- Lung: Head size ratio <1
FOLLOW-UPFailure to thrive is commonNeed 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
Foramen of Morgagni Hernia Failure of the sternal and crural portions of thediaphragm to meet and fuse
◦ Right-sided (90%) but may be B/L◦ Contents-transverse colon/small
intestine/liverDiagnosis Chest radiograph (incidental finding)
◦ Anteroposterior view- a structure behind heart
◦ Lateral view- mass in retrosternal area Chest CT or MRI- confirmatoryRepair is recommended for all patients, in view
of the risk of bowel strangulation◦ Laparoscopically ◦ Open approach◦ Prosthetic material is rarely required
TRACHEO-ESOPHAGEAL FISTULA
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%)
EMBRYOLOGY
PATHOPHYSIOLOGY Two main pathologic entities
◦Dehydration◦Aspiration pneumonitis
EA- Aspiration-RD,Atelectasis and pneumoniaTEF-
◦Proximal-aspiration◦Distal-stomach/bowel distension and elevation of
diaphragmAbnormal Esophageal peristalsisMaldevelopment of cartilage rings
(tracheomalacia)
CLASSIFICATION
Clinical PresentationChoking on 1st feedCoughingCyanosisExcessive salivationFeed regurgitationAspiration pneumoniaDehydrationAbdominal findings
◦C- Distension◦A- Scaphoid abdomen
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
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
Associated congenital anomaliesSYNDROMIC
ASSOCIATION :-DiGeorge sequenceFeingold syndromePierre-robbin sequencePolysplenia sequenceHolt Oram sequenceTrisomy 13,18,21VACTERLCHARGEAnorectal- Triple atresia(EA+DA+AA)
INVESTIGATIONSCBCSerum electrolytesRenal USGECG/ EchoChest X-RayCT / MRIABG
Timing of surgery :-WATERSTON group A- Immediate operative
repairWATERSTON group B- Delay repairWATERSTON group C- Staged repairType of surgery :-Thoracotomy-
◦Extrapleural◦Transpleural
VAT
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
OPERATIVE REPAIRDepends 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.
COMPLICATIONSEarlyAnastomotic leak- 15%Esophageal stricture- 80% require eso.
dilatationRecurrent TEFLateGERD with reactive airway disease- 30-70%Delayed gastric emptyingTracheomalacia
THANK YOU