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Year : 2023  |  Volume : 28  |  Issue : 5  |  Page : 433-435

Right phrenic nerve palsy following long-gap esophageal atresia and tracheoesophageal fistula repair

1 Department of Pediatric Urology and Pediatric Surgery, Indraprastha Apollo Hospitals, New Delhi, India
2 Department of Neonatology, Indraprastha Apollo Hospitals, New Delhi, India

Date of Submission11-Jan-2023
Date of Decision03-Jun-2023
Date of Acceptance04-Jun-2023
Date of Web Publication05-Sep-2023

Correspondence Address:
Sujit K Chowdhary
Department of Pediatric Urology and Pediatric Surgery, Indraprastha Apollo Hospitals, Sarita Vihar, New Delhi -110 044
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/jiaps.jiaps_7_23

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Esophageal atresia (EA) and tracheoesophageal fistula (TEF) are surgically correctable congenital anomalies with reported surgical common complications such as anastomotic leaks, recurrent TEF, and esophageal strictures; however, phrenic nerve injury (PNI) is a very rare but possible complication which we have highlighted in our case report. Here, we report a baby girl operated for long-gap EA and TEF having respiratory distress and failed attempts to wean off oxygen support. Serial chest X-rays showed elevated right hemidiaphragm, whereas ultrasound thorax confirmed our diagnosis of right PNI causing diaphragmatic palsy. Conservative management with the hope of spontaneous recovery failed, so diaphragmatic plication was done at 5 weeks from index surgery. Postplication, the baby was weaned off oxygen and pressure support the very 1st day and had improved respiratory physiology.

Keywords: Diaphragmatic palsy, diaphragmatic plication, esophageal atresia, phrenic nerve injury, tracheoesophageal fistula

How to cite this article:
Pokharkar AD, Aditya M, Kandpal D, Balan S, Gupta V, Chowdhary SK. Right phrenic nerve palsy following long-gap esophageal atresia and tracheoesophageal fistula repair. J Indian Assoc Pediatr Surg 2023;28:433-5

How to cite this URL:
Pokharkar AD, Aditya M, Kandpal D, Balan S, Gupta V, Chowdhary SK. Right phrenic nerve palsy following long-gap esophageal atresia and tracheoesophageal fistula repair. J Indian Assoc Pediatr Surg [serial online] 2023 [cited 2023 Oct 2];28:433-5. Available from: https://www.jiaps.com/text.asp?2023/28/5/433/385148

   Introduction Top

Phrenic nerve is a motor nerve supplying the diaphragm, enabling/aiding respiration.[1] Phrenic nerve injury (PNI), especially in neonates and infants who are primarily diaphragmatic breathers, may lead to respiratory failure.

Cardiothoracic surgeries in neonates are known to cause PNI, accounting for 64% of cases.[1] PNI after repair of esophageal atresia (EA) with tracheoesophageal fistula (TEF) is rare with only a few cases reported in the past.[2],[3],[4],[5] Here, we report possibly the first diagnosed case of right PNI following repair of long-gap EA and TEF from India.

   Case Report Top

A term baby was referred to our hospital with a complaint of respiratory distress at birth with significant pooling of oral secretions and failure to pass infant feeding tube beyond 10 cm. Chest X-ray (CXR) done at our hospital showed coiled tube at the level of T1 vertebra with distal bowel gas consistent with the diagnosis of EA and TEF. At exploration through right thoracotomy, TEF was found at the carina with upper pouch found high in the neck and atretic. The distance between the upper pouch and the lower fistula was >3 cm measured using a tape. Extensive mobilization of the upper esophageal pouch was done and end-to-end anastomosis was accomplished under high tension.

The baby was electively ventilated for 3 days postoperatively, and feeds were started on postoperative day (POD) 2 and gradually progressed to full feeds by POD5. Over 5 weeks postoperatively, several attempts to wean the baby of ventilation were unsuccessful. Initial CXR done showed right lung consolidation with no effusion which resolved with antibiotics, but the baby was still dependent on ventilator. Interestingly, all CXRs done postoperatively showed raised right hemidiaphragm by three rib levels compared to the left suggesting right diaphragmatic palsy (DP) [Figure 1]. Ultrasound (USG) thorax also revealed diminished or no movement of the right hemidiaphragm compared to the left, although there was no paradoxical movement. CXR along with USG helped us to make our diagnosis of right DP.
Figure 1: Postoperative day 3 tracheoesophageal fistula repair chest X-ray (note the raised right hemidiaphragm shown in red than its counterpart in blue)

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In view of failed attempt of weaning and deteriorating general condition, we went forward with plication of the right diaphragm. On POD1 postplication, CXR showed the position of both hemidiaphragm at appropriate anatomical level and the baby was successfully weaned off the oxygen support and pressure with resolving respiratory distress [Figure 2].
Figure 2: Postplication chest X-ray (both hemidiaphragm at appropriate anatomical levels right dome showing with orange arrow)

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   Discussion Top

Complications from TEF repair surgery include strictures, reflux, recurring TEF, and respiratory infections. Right PNI is a rare complication after EA and TEF repair that has been reported in few cases.[2],[3],[4],[5] Iatrogenic PNI, which can lead to paralysis, is a common complication of pediatric cardiac surgery and other medical procedures.[6]

Direct injury is unlikely in this case due to the location of incision. Instead, it is possible that several factors contributed to indirect injury. Minimal intervening tissue in neonates can increase the likelihood of lateral thermal damage and retraction injury at relatively distant sites.[2],[5] Extensive mobilization of the upper pouch of the esophagus located high in the mediastinum may cause traction injury to the phrenic nerve or its cervical route as quoted by Man et al.[3]

Diagnosing PNI is challenging, due to nonspecific clinical signs and no universally defined guidelines. Clinicians should keep a high index of suspicion for patients undergoing cardiac and thoracic surgeries. In suspected cases, investigations such as a CXR and ultrasonography should be carried out to confirm the diagnosis. Physical examination in our case revealed similar signs as explained by Mandoorah et al.[3] in their review, comprising decreased breath sounds on the affected side, dullness to percussion, and paradoxical movement of the epigastrium during respiration.

CXR has a positive predictive value of only 33%[7] for diagnosing PNI. Ultrasonography is more sensitive than fluoroscopy[8] which can show decreased or no movement of the hemidiaphragm.

The recovery of a phrenic nerve paralysis is unpredictable, with studies quoting recovery periods ranging from days to months. While supportive ventilation was once a preferred treatment option, it can expose vulnerable patients to additional morbidity and mortality, favoring early surgical intervention.[9] Many authors now recommend early diaphragmatic plication (<14 days from the index surgery) as it has been shown to result in earlier extubation (within 2–3 days) which was also noted in our case, with patient off ventilator from the 1st POD with improving respiratory physiology.[10] Added advantages include shorter intensive care unit (ICU) and hospital stay, lower morbidity, lower risk of pulmonary infections, and thus improved outcomes.

There is, however, conflicting advice in the literature regarding the optimal timing of plication surgery. Some studies recommending it to be undertaken as soon as PNI is diagnosed, while others propose a 1–6-week period of supportive ventilation to allow the possibility of spontaneous recovery before performing surgery.[11] Nonetheless, if the diaphragm atrophies then undertaking plication too late can impair outcomes.[7]

Considering all that, we made a note suggesting early plication to be considered in infants due to their reduced ability to tolerate DP. For our patient, plication was carried out 5 weeks after the index surgery with the hope for the spontaneous recovery of diaphragmatic paralysis due to the indirect nature of the injury, which did not improve throughout the observation period. In afterthought, it might have been better for our patient to have had his paralyzed right diaphragm plicated well before complications of prolonged oxygen and pressure requirement set in. These children should then be followed up every 6 months or yearly as needed to monitor their lung function and watch out for their growth and development.

   Conclusion Top

  1. Difficulty weaning from mechanical ventilation after EA and TEF repair surgery, especially when the upper pouch is high in the neck, should raise the possibility of PNI with DP, after ruling out obvious cardiac or pulmonary pathology
  2. A nonoperative management trial using ventilatory support while monitoring for spontaneous recovery should be considered but should not be overly prolonged
  3. If weaning from ventilator fails, early diaphragmatic plication should be considered due to the increased morbidity and mortality associated with prolonged ICU stays.

Declaration of patient consent

The authors certify that they have obtained all appropriate patient consent forms. In the form, the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.

Financial support and sponsorship


Conflicts of interest

There are no conflicts of interest.

   References Top

Tarmahomed A, Adan A, Thomas A, Sanghavi R, Amin K, Asif W, et al. Management of phrenic nerve injury post-cardiac surgery in the paediatric patient. Cardiol Young 2021;31:1386-92.  Back to cited text no. 1
Henderson PW, Spigland NA. Right phrenic nerve injury as a complication of tracheoesophageal fistula repair. Pediatr Crit Care Med 2010;11:e52-4.  Back to cited text no. 2
Man D, Wheildon MH, Eckstein HB. Paralysis of the right hemidiaphragm following primary anastomosis for oesophageal atresia and tracheo-oesophageal fistula. Z Kinderchir 1982;37:32-3.  Back to cited text no. 3
Haller JA Jr., Pickard LR, Tepas JJ, Rogers MC, Robotham JL, Shorter N, et al. Management of diaphragmatic paralysis in infants with special emphasis on selection of patients for operative plication. J Pediatr Surg 1979;14:779-85.  Back to cited text no. 4
Sagadevan S, Somoo VN, Krishnan A, Sharudin MF, Lim Q, Sani RM. Right phrenic nerve injury: A rare but possible complication of oesophageal atresia and tracheoesophageal fistula repair. Malaysian J Sci Health Technol 2022;8:44-8.  Back to cited text no. 5
de Leeuw M, Williams JM, Freedom RM, Williams WG, Shemie SD, McCrindle BW. Impact of diaphragmatic paralysis after cardiothoracic surgery in children. J Thorac Cardiovasc Surg 1999;118:510-7.  Back to cited text no. 6
Dubé BP, Dres M. Diaphragm dysfunction: Diagnostic approaches and management strategies. J Clin Med 2016;5:113.  Back to cited text no. 7
Houston JG, Fleet M, Cowan MD, McMillan NC. Comparison of ultrasound with fluoroscopy in the assessment of suspected hemidiaphragmatic movement abnormality. Clin Radiol 1995;50:95-8.  Back to cited text no. 8
Floh AA, Zafurallah I, MacDonald C, Honjo O, Fan CS, Laussen PC. The advantage of early plication in children diagnosed with diaphragm paresis. J Thorac Cardiovasc Surg 2017;154:1715-21.e4.  Back to cited text no. 9
Talwar S, Agarwala S, Mittal CM, Choudhary SK, Airan B. Diaphragmatic palsy after cardiac surgical procedures in patients with congenital heart. Ann Pediatr Cardiol 2010;3:50-7.  Back to cited text no. 10
Stauffer UG, Rickham PP. Acquired eventration of the diaphragm in the newborn. J Pediatr Surg 1972;7:635-40.  Back to cited text no. 11


  [Figure 1], [Figure 2]


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