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Things happen
Uncommon event of a common procedure

        Dr. Abdur Rakib Talukder, MRCS
• 36 weeks, male, weighing 2 kg; NSVD.

• Diagnosed as a case of EA & TEF, accepted from other
  Hospital.

• No other association was diagnosed.
X-Ray at Birth
Isolating TEF

       • Operated at 4th day of life.

       •    Ligation of TEF & primary
           anastomosis.

       • Extra pleural approach.

       • Minimal dissection.
• Extubated on 4th POD .
• Dye study on 7th POD.
Post Repair Upper GI Dye Study




No anastomotic leak.   Right hemi diaphragm was up.
Fluroscopy
• Paralysis of right hemi diaphragm diagnosed.



• Initial trial of conservative management was
  planned.
Summary

•   Extrapleural approach (postero-lateral incision).
•   Chest tube not placed.
•   Easy extubation (4th POD).
•   Established feeds on 7th POD & feeding was well tolerated.
•   Maintaining Oxygen Saturation >95% in room air.



• He was discharged home after 2 weeks.
• At the age of 2 months he was presented to local hospital ER
  and was admitted due to H/O shortness of breath, cough &
  poor feeding for previous 2 weeks.

• Transferred to our hospital for further management.
Chest X-Ray on admission
• Child was admitted in PICU with a diagnosis of Aspiration
  Pneumonia.

• IV antibiotics with supportive measures were started.

• He was on Oxygen 1L/min through Nasal canula.
After resolving Pneumonia
• Conservative management continued.


• End of the 6 week:

      He was oxygen dependent (>2L/min),
      Persistent tachypnoea,
      Not gaining weight,
      Requiring regular nebulization & CPT to maintain a clear air way.
Decision was taken for surgical intervention.
Plication of Diaphragm



           • Right sided posterolateral
             thoracotomy at 8th intercostal
             space.

           • Dome of diaphragm high up.
Plication of Diaphragm




Grasping diaphragm.   Plicating using Pledgeted suture.
Plication of Diaphragm




   Plication in 6 rows each 4 folds.
Lung Expansion
Post Op X-Ray
Eventration of right hemi
  Diaphragm following
     EA & TEF repair
1st case Report


• Paralysis of the right hemidiaphragm following primary
  anastomosis for oesophageal atresia and tracheo-oesophageal
  fistula.



                                   Man D, Wheildon MH, Eckstein HB
                                          Z Kinderchiv 1982;37: 32-3
Anatomy Right Phrenic Nerve
           • Phrenic nerve root C3–C5,

           • At the thoracic inlet: the right phrenic
             nerve is behind the innominate vein and
             crosses in front of pulmonary hilum
             beside internal mammary artery,
             descends to the right of the superior
             vena cava, anterior to the lung.

           • Finally: descends along the inferior
             vena cava toward the diaphragm, branch
             just proximal to the diaphragm into small
             terminal branches.
Incidence

• The 10% of infants and children those undergoing cardiac
  surgery develop Phrenic nerve injury .




                  Phrenic nerve injury in infants and children undergoing cardiac surgery.
                                  Q Mok, R Ross-Russell, D Mulvey, M Green, and E A Shinebourne
   Department of Paediatric Cardiology, Royal Brompton National Heart and Lung Hospital, London.
Pathogenesis
• Two types:

• Congenital eventration: results from inadequate development
  of the muscle or absence of the phrenic nerves.

• Acquired eventration: injury to the phrenic nerve, resulting
  from either a traumatic birth or thoracic surgery for congenital
  heart disease.

• The loss of contractility leads to muscle atrophy with elevation
  of the hemidiaphragm
• Cause of Per Operative Phrenic Nerve Injury: stretching,
  crushing, transection, and hypothermia.

• Cardiac surgery, chiropractic manipulation, trauma or
  anesthetic blocks at neck, right subclavian vein catheterization,
  ICT insertion.

• Metastasis.


     Elevated hemidiaphragm after cardiac operations: incidence, prognosis and
                                       relationship to the use of topical ice slush.
 Curtis JJ, Weerachai N, Walls J, Ann Thorac Surg 48:764, 1989 [PMID 2596912]
Investigation


• Electrophysiologic examination.
• Fluroscopy.
Fluroscopy
• Sniff test:
      • Paradoxical upward motion on affected side.

• Normal excursion of 1-2 ribs:
   • Breathe in, diaphragm down.
   • Breathe out, diaphragm up.


• Paralyzed – paradoxical motion:
   • Breathe in, diaphragm up.
   • Breath out, diaphragm down.
Management

• Management of eventration of diaphragm secondary to phrenic
  nerve injury must be individualized and depends on the
  incapacity of the individual.

    Medical
    Surgical
Medical Management


• When paralysis is thought to be potentially reversible and the
  patient is asymptomatic, it may be managed conservatively.




                                  Malek & Abdulrahman Al-Bassam,
                               http://faculty.ksu.edu.sa/5564/Publications/Forms/AllItems.aspx
• Diaphragmatic plication reserved for those patients who fail to
  regain diaphragmatic function after 4 to 6 wks trial of
  conservative management.




 Right phrenic nerve injury as a complication of tracheoesophageal fistula repair.
                                                    Henderson PW, Spigland NA.
   Division of Pediatric Surgery, Weill Cornell Medical College, New York, USA.
                                    Pediatr Crit Care Med. 2010 Sep;11(5):e52-4.
Indication for surgery


• Failure of conservative treatment measures.

    Recurrent chest infection.
    Oxygen dependency.
    Failure to thrive.
Lap/Open
•   Open thoracotomy is the preferred approach.

• Minimally invasive diaphragm plication techniques have
  emerged as equally effective and less morbid alternatives to
  open plication.




      Diaphragm plication for eventration or paralysis: a review of the literature.
                                                          Groth SS, Andrade RS.
                         Department of Surgery, University of Minnesota, USA.
                                    Ann Thorac Surg. 2010 Jun;89(6):S2146-50.
Phrenic Nerve Reconstruction



• Tried in adult but no published report in neonate/pediatric.
Pacing of Diaphragm
• Pacing of the diaphragm requires an intact phrenic nerve.

• Indications: Bilateral Paralysis, central alveolar
  hypoventilation and high cervical spinal cord injury (Ondine's
  curse); intractable hiccups and end-stage chronic obstructive
  pulmonary disease.

• Phrenic nerve pacing was partially successful in 36%.


                       Phrenic nerve stimulation: the Australian experience.
                                                  Khong P, Lazzaro A, Mobbs R.
                  Department of Neurosurgery, Prince of Wales Hospital, Australia.
Conclusion
• 1st case of Right Diaphragmatic Paralysis after repair of EA &
  TEF was published in 1982.

• Related complication was published in 2007.

• Possible mechanism for our case could be stretching/
  ?Aberrant course of Right Phrenic Nerve.
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Right hemidiaphragm paralysis after EA & TEF repair.

  • 1. Things happen Uncommon event of a common procedure Dr. Abdur Rakib Talukder, MRCS
  • 2. • 36 weeks, male, weighing 2 kg; NSVD. • Diagnosed as a case of EA & TEF, accepted from other Hospital. • No other association was diagnosed.
  • 4. Isolating TEF • Operated at 4th day of life. • Ligation of TEF & primary anastomosis. • Extra pleural approach. • Minimal dissection.
  • 5. • Extubated on 4th POD . • Dye study on 7th POD.
  • 6. Post Repair Upper GI Dye Study No anastomotic leak. Right hemi diaphragm was up.
  • 8. • Paralysis of right hemi diaphragm diagnosed. • Initial trial of conservative management was planned.
  • 9. Summary • Extrapleural approach (postero-lateral incision). • Chest tube not placed. • Easy extubation (4th POD). • Established feeds on 7th POD & feeding was well tolerated. • Maintaining Oxygen Saturation >95% in room air. • He was discharged home after 2 weeks.
  • 10. • At the age of 2 months he was presented to local hospital ER and was admitted due to H/O shortness of breath, cough & poor feeding for previous 2 weeks. • Transferred to our hospital for further management.
  • 11. Chest X-Ray on admission
  • 12. • Child was admitted in PICU with a diagnosis of Aspiration Pneumonia. • IV antibiotics with supportive measures were started. • He was on Oxygen 1L/min through Nasal canula.
  • 14. • Conservative management continued. • End of the 6 week:  He was oxygen dependent (>2L/min),  Persistent tachypnoea,  Not gaining weight,  Requiring regular nebulization & CPT to maintain a clear air way.
  • 15. Decision was taken for surgical intervention.
  • 16. Plication of Diaphragm • Right sided posterolateral thoracotomy at 8th intercostal space. • Dome of diaphragm high up.
  • 17. Plication of Diaphragm Grasping diaphragm. Plicating using Pledgeted suture.
  • 18. Plication of Diaphragm Plication in 6 rows each 4 folds.
  • 21. Eventration of right hemi Diaphragm following EA & TEF repair
  • 22. 1st case Report • Paralysis of the right hemidiaphragm following primary anastomosis for oesophageal atresia and tracheo-oesophageal fistula. Man D, Wheildon MH, Eckstein HB Z Kinderchiv 1982;37: 32-3
  • 23. Anatomy Right Phrenic Nerve • Phrenic nerve root C3–C5, • At the thoracic inlet: the right phrenic nerve is behind the innominate vein and crosses in front of pulmonary hilum beside internal mammary artery, descends to the right of the superior vena cava, anterior to the lung. • Finally: descends along the inferior vena cava toward the diaphragm, branch just proximal to the diaphragm into small terminal branches.
  • 24. Incidence • The 10% of infants and children those undergoing cardiac surgery develop Phrenic nerve injury . Phrenic nerve injury in infants and children undergoing cardiac surgery. Q Mok, R Ross-Russell, D Mulvey, M Green, and E A Shinebourne Department of Paediatric Cardiology, Royal Brompton National Heart and Lung Hospital, London.
  • 25. Pathogenesis • Two types: • Congenital eventration: results from inadequate development of the muscle or absence of the phrenic nerves. • Acquired eventration: injury to the phrenic nerve, resulting from either a traumatic birth or thoracic surgery for congenital heart disease. • The loss of contractility leads to muscle atrophy with elevation of the hemidiaphragm
  • 26. • Cause of Per Operative Phrenic Nerve Injury: stretching, crushing, transection, and hypothermia. • Cardiac surgery, chiropractic manipulation, trauma or anesthetic blocks at neck, right subclavian vein catheterization, ICT insertion. • Metastasis. Elevated hemidiaphragm after cardiac operations: incidence, prognosis and relationship to the use of topical ice slush. Curtis JJ, Weerachai N, Walls J, Ann Thorac Surg 48:764, 1989 [PMID 2596912]
  • 28. Fluroscopy • Sniff test: • Paradoxical upward motion on affected side. • Normal excursion of 1-2 ribs: • Breathe in, diaphragm down. • Breathe out, diaphragm up. • Paralyzed – paradoxical motion: • Breathe in, diaphragm up. • Breath out, diaphragm down.
  • 29. Management • Management of eventration of diaphragm secondary to phrenic nerve injury must be individualized and depends on the incapacity of the individual.  Medical  Surgical
  • 30. Medical Management • When paralysis is thought to be potentially reversible and the patient is asymptomatic, it may be managed conservatively. Malek & Abdulrahman Al-Bassam, http://faculty.ksu.edu.sa/5564/Publications/Forms/AllItems.aspx
  • 31. • Diaphragmatic plication reserved for those patients who fail to regain diaphragmatic function after 4 to 6 wks trial of conservative management. Right phrenic nerve injury as a complication of tracheoesophageal fistula repair. Henderson PW, Spigland NA. Division of Pediatric Surgery, Weill Cornell Medical College, New York, USA. Pediatr Crit Care Med. 2010 Sep;11(5):e52-4.
  • 32. Indication for surgery • Failure of conservative treatment measures.  Recurrent chest infection.  Oxygen dependency.  Failure to thrive.
  • 33. Lap/Open • Open thoracotomy is the preferred approach. • Minimally invasive diaphragm plication techniques have emerged as equally effective and less morbid alternatives to open plication. Diaphragm plication for eventration or paralysis: a review of the literature. Groth SS, Andrade RS. Department of Surgery, University of Minnesota, USA. Ann Thorac Surg. 2010 Jun;89(6):S2146-50.
  • 34. Phrenic Nerve Reconstruction • Tried in adult but no published report in neonate/pediatric.
  • 35. Pacing of Diaphragm • Pacing of the diaphragm requires an intact phrenic nerve. • Indications: Bilateral Paralysis, central alveolar hypoventilation and high cervical spinal cord injury (Ondine's curse); intractable hiccups and end-stage chronic obstructive pulmonary disease. • Phrenic nerve pacing was partially successful in 36%. Phrenic nerve stimulation: the Australian experience. Khong P, Lazzaro A, Mobbs R. Department of Neurosurgery, Prince of Wales Hospital, Australia.
  • 36. Conclusion • 1st case of Right Diaphragmatic Paralysis after repair of EA & TEF was published in 1982. • Related complication was published in 2007. • Possible mechanism for our case could be stretching/ ?Aberrant course of Right Phrenic Nerve.