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