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Journal of Indian Association of Pediatric Surgeons
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Table of Contents   
ORIGINAL ARTICLE
Year : 2021  |  Volume : 26  |  Issue : 4  |  Page : 246-249
 

Diaphragmatic hernia after pediatric living donor liver transplantation: An Indian experience with review of literature


Department of Hepato Pancreato Biliary and Liver Transplant Surgery, Institute of Liver and Biliary Sciences, New Delhi, India

Date of Submission30-Apr-2020
Date of Decision13-Jun-2020
Date of Acceptance15-Jul-2020
Date of Web Publication12-Jul-2021

Correspondence Address:
Dr. Viniyendra Pamecha
Department of Hepato Pancreato Biliary and Liver Transplant Surgery, Institute of Liver and Biliary Sciences, D.1, Vasant Kunj, New Delhi - 110 070
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/jiaps.JIAPS_134_20

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   Abstract 


Aim: Although intestinal obstruction following living donor liver transplantation (LDLT) is relatively common, diaphragmatic hernia (DH) as the attributing cause is rarely reported. The study aimed to find out the incidence, clinical presentation, and management of DH after liver transplant in pediatric patients.
Materials and Methods: A retrospective review of all cases of pediatric liver transplants was performed. Case sheets, operation records, and investigations including radiology were studied.
Results: Out of 79 cases of pediatric liver transplants, two cases of posttransplant DH were identified. The first case is a 2-year-old male child who was transplanted for progressive familial intrahepatic cholestasis, and the second one is a 4-year-old boy who underwent LDLT for hepatitis A-induced acute liver failure. The first child presented abdominal symptoms and the second one with pulmonary symptoms. The DHs were diagnosed at 132 and 70 days, respectively, posttransplant by chest radiographs and computed tomography scan. The defects were located at the posteromedial aspect of the diaphragm in both the cases and were closed by primary closure with mesh reinforcement. Both the children are doing well with no recurrence.
Conclusion: High index of clinical suspicion is mandated in pediatric patients after liver transplant and once the diagnosis is confirmed, urgent surgical exploration is mandatory.


Keywords: Diaphragmatic, hernia, intestinal obstruction, liver transplantation


How to cite this article:
Pattnaik B, Sinha PK, Sadashiv NP, Mohapatra N, Pamecha V. Diaphragmatic hernia after pediatric living donor liver transplantation: An Indian experience with review of literature. J Indian Assoc Pediatr Surg 2021;26:246-9

How to cite this URL:
Pattnaik B, Sinha PK, Sadashiv NP, Mohapatra N, Pamecha V. Diaphragmatic hernia after pediatric living donor liver transplantation: An Indian experience with review of literature. J Indian Assoc Pediatr Surg [serial online] 2021 [cited 2021 Jul 30];26:246-9. Available from: https://www.jiaps.com/text.asp?2021/26/4/246/321191





   Introduction Top


Living donor liver transplantation (LDLT) is increasingly being performed in the pediatric population. Diaphragmatic hernia (DH) is one of the rare yet significant complications described in the literature. A high index of suspicion is of utmost importance, which can further guide early diagnosis and urgent surgical intervention before it progresses to fatal incarceration and gangrene. Although the pathophysiology is not fully established, many predisposing factors have been described such as large size grafts, ascites, pleural effusion, prolonged mechanical ventilation, previous surgery, portal hypertension, malnutrition, and diaphragmatic weakness due to thermal or mechanical injury.[1] The only treatment is the surgical reduction of the herniated bowel and repair of the defect.[2] Herein, we present our experience of two pediatric patients who developed DH after LDLT and have reviewed the literature available to date.


   Materials and Methods Top


Two cases of DH occurred in 79 pediatric patients transplanted. Their pretransplant records, transplant surgery records, case sheet for clinical presentation, and radiology and postsurgery records were studied. Existing literature was reviewed.


   Results Top


Case 1

A 2.5-year-old male child was diagnosed as a case of progressive familial intrahepatic cholestasis type II.[3] He underwent LDLT using left lateral segment (LLS) graft. His postoperative recovery was complicated with sepsis, upper gastrointestinal bleed, and symptomatic right pleural effusion and prolonged positive pressure ventilation for 16 days. Eventually, he recovered well and was discharged on postoperative day (POD) 51 in a satisfactory condition.

Three months later, he presented to the emergency department with complaints of increasing cough for 3 days with increased work of breathing. On evaluation, the mother informed about the low oral intake for the past 2 days though he had no frank vomiting. On Chest X-ray [Figure 1]a, the right hemidiaphragm was found elevated with right-sided DH, which was confirmed on computed tomography (CT) scan [Figure 1]b. After the diagnosis of right DH was made, he underwent urgent exploration. Intraoperatively, a defect of size 4 cm × 5 cm was noted in the posteromedial aspect of the right dome of the diaphragm, just to the right of the inferior vena cava [Figure 1]c and [Figure 1]d. Small bowel loops (distal jejunum and ileum up to ileocecal junction) were noted in the right thoracic cavity. After gentle reduction, the bowel loops were found to be healthy, with no signs of ischemia. The hernial defect was closed primarily with Prolene 3-0 sutures in a continuous fashion and was reinforced with a polytetrafluoroethylene (PTFE) mesh, and a right intercoastal drain was inserted. He made an uneventful recovery and was discharged on day 15 after surgery. He is doing well at the last follow-up (42-month post-LDLT).
Figure 1: (a) Chest X-ray showing bowel and gas bubbles in the right chest. (b) Noncontrast computed tomography showing herniated bowel loops with air–fluid levels in the right hemithorax. (c) Intraoperative picture showing small bowel loops herniating through the diaphragmatic defect (arrow). (d) Right diaphragmatic posteromedial defect

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Case 2

A 4-year-old boy presented to the emergency department with jaundice for 2 months and altered sensorium for 2 days. On evaluation, a diagnosis of acute liver failure due to hepatitis A with suspected drug-induced (had consumed some ayurvedic medication) liver injury was made. He underwent emergency LDLT as he was fulfilling in King's College criteria. LDLT was done using LLS graft (graft recipient weight ratio – 1.68). He was extubated on POD 7; his postoperative course was uneventful and was discharged on day 29.

He presented to the emergency department on day 70 with pain abdomen (periumbilical and right lumbar region) for 1 day with vomiting. The initial chest radiograph showed right-sided DH which was further delineated in the CT scan and was taken up for urgent exploration [Figure 2]a, [Figure 2]b, [Figure 2]c. Intraoperatively, an 8 cm × 5 cm defect was found on the posteromedial aspect of the right hemidiaphragm. After gentle adhesiolysis, most of the small bowel, part of the ascending colon, and proximal transverse colon were reduced from the thoracic cavity. The bowel was found to be slightly edematous, but no frank signs of ischemia were noticed. The defect was primarily repaired with Prolene 1-0 suture and reinforced with PTFE mesh [Figure 2]d. His symptoms resolved and was discharged on day 10. He was doing well at the last follow-up (30-month post-LDLT). The peritransplantation clinical profile of both the patients is compared in [Table 1].
Figure 2: (a) X-ray revealing bowel loops with air–fluid levels and gas bubbles in the right hemithorax. (b) Noncontrast computed tomography showing herniated bowel loops with air–fluid levels in the right hemithorax. (c) Noncontrast computed tomography showing herniated bowel loops and hypertrophied left lateral graft. (d) Intraoperative picture after reinforcement of the hernial defect with prosthetic mesh (arrow)

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Table 1: Peritransplant clinical characteristics

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   Discussion Top


Diaphragmatic complications such as dysfunction or paralysis after LDLT in children are common, with the incidence ranging from 8% to 15%,[4],[5],[6] but DH is rare, with an incidence of 0.6%–3%.[1],[2],[7] Most are case reports, and the largest series had ten patients from King's College Hospital.[2] In our institute, we have so far encountered two DH out of the 79 pediatric transplants (2.53%). Both the cases were right sided and required immediate surgical attention.

The most common presentation is breathing difficulty, followed by features of bowel obstruction. Chest and abdominal X-ray is diagnostic, but contrast-enhanced CT is always done to delineate the defect, the contents, and the relation to any major vascular structures. Once the diagnosis is made, the management is an urgent surgical reduction and primary repair of the defect.[8] Lee et al. have described thoracoscopic repair in a case after LDLT, but laparotomy is presently the most common approach.[9] The repair can be reinforced with mesh as the diaphragm is weak and unprotected.

To form a hernia, there are three basic requirements: first is the presence of a cavity; second, increased intracavitary pressure; and third, weakened wall of the cavity. After the recipient hepatectomy and implantation of left or left lateral graft, the right upper quadrant becomes a cavity which is filled by right and transverse colons along with the small bowel.[10] Various factors that can lead to increased intra-abdominal pressure include the use of large for size grafts, paralytic ileus, and formation of large-volume ascites and prolonged mechanical ventilation.[2] The most important factor is the presence of a weak wall, which is the diaphragm in this case. Chronic liver disease in children is associated with failure to thrive, malnutrition, and sarcopenia, leading to a thinned-out diaphragm with the decreased density of muscle fibers.[11] Factors during surgery such as traumatic dissection and excessive use of energy devices can add to weakness.[12] Recipient hepatectomy in children, particularly after Kasai portoenterostomy, is a difficult undertaking. There are extensive adhesions with collaterals due to portal hypertension in between the liver and the diaphragm, which makes the use of energy devices such as diathermy and argon plasma coagulation inevitable. These all can lead to iatrogenic vascular impairment to the diaphragm.[13],[14] Moreover, right phrenic nerve courses along with the right hemidiaphragm and can get injured during surgery.[15] Due to the constant motion of the diaphragm, injuries take longer to heal. These all lead to herniation of abdominal contents into the thorax assisted by the negative intrathoracic pressure.

Use of immunosuppression, especially steroids and mammalian target of rapamycin (mTOR) inhibitors, has been implicated in pathogenesis. Steroids are known to cause protein depletion and delayed wound healing.[16],[17] mTOR inhibitors which have increased popularity in recent times have been implicated in delayed wound healing, and there are reports of spontaneous rupture of the diaphragm in patients on sirolimus.[18],[19] Both the patients received steroids for 3 months, but none were on mTOR inhibitors.

The prognosis has been good in literature but is a potentially life-threatening complication. Immediate intervention is warranted to prevent massive bowel loss.[2] There are no reports of recurrence in literature, but owing to the inherent weakness of the diaphragm, the patient should be followed at a timely interval.


   Conclusion Top


DH should be kept in mind while dealing with intestinal obstruction or breathing difficulty in a child after LDLT using LLS grafts. We believe a foresightedness of the potential hernia occurrence, careful dissection, and proper hemostasis should be the way forward in reducing this complication.

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

Nil.

Conflicts of interest

There are no conflicts of interest.



 
   References Top

1.
Moon SB, Jung SM, Kwon CH, Kim SJ, Joh JW, Seo JM, et al. Posteromedial diaphragmatic hernia following pediatric liver transplantation. Pediatr Transplant 2012;16:E106-9  Back to cited text no. 1
    
2.
Cortes M, Tapuria N, Khorsandi SE, Ibars EP, Vilca-Melendez H, Rela M, et al. Diaphragmatic hernia after liver transplantation in children: Case series and review of the literature. Liver Transpl 2014;20:1429-35.  Back to cited text no. 2
    
3.
Baker A, Kerkar N, Todorova L, Kamath BM, Houwen RHJ. Systematic review of progressive familial intrahepatic cholestasis. Clin Res Hepatol Gastroenterol 2019;43:20-36.   Back to cited text no. 3
    
4.
Manczur TI, Greenough A, Rafferty GF, Dimitriou G, Baker AJ, Mieli-Vergani G, et al. Diaphragmatic dysfunction after pediatric orthotopic liver transplantation. Transplantation 2002;73:228-32.  Back to cited text no. 4
    
5.
Smyrniotis V, Andreani P, Muiesan P, Mieli-Vergani G, Rela M, Heaton ND. Diaphragmatic nerve palsy in young children following liver transplantation. Successful treatment by plication of the diaphragm. Transpl Int 1998;11:281-3.  Back to cited text no. 5
    
6.
Mack CL, Millis JM, Whitington PF, Alonso EM. Pulmonary complication following liver transplantation in pediatric patients. Pediatr Transplant 2000;4:39-44.  Back to cited text no. 6
    
7.
Shigeta T, Sakamoto S, Kanazawa H, Fukuda A, Kakiuchi T, Karaki C, et al. Diaphragmatic hernia in infants following living donor liver transplantation: Report of three cases and a review of the literature. Pediatr Transplant 2012;16:496-500.  Back to cited text no. 7
    
8.
Wang K, Gao W, Ma N, Meng XC, Zhang W, Sun C, et al. Acquired diaphragmatic hernia in pediatrics after living donor liver transplantation: Three cases report and review of literature. Medicine (Baltimore) 2018;97:e0346.  Back to cited text no. 8
    
9.
Lee S, Seo JM, Younes AE, Oh CY, Lee SK. Thoracoscopic approach for repair of diaphragmatic hernia occurring after pediatric liver transplant. Medicine (Baltimore) 2015;94:e1376.  Back to cited text no. 9
    
10.
Koplewitz BZ, Ramos C, Manson DE, Babyn PS, Ein SH. Traumatic diaphragmatic injuries in infants and children: Imaging findings. Pediatr Radiol 2000;30:471-9.  Back to cited text no. 10
    
11.
Tsunezuka Y, Hatakeyama S, Iwase T, Watanabe G. Video-assisted thoracoscopic treatment for pleuroperitoneal communication in peritoneal dialysis. Eur J Cardiothorac Surg 2001;20:205-7.  Back to cited text no. 11
    
12.
McCabe AJ, Orr JD, Sharif K, De Ville de Goyet J. Right-sided diaphragmatic hernia in infants after liver transplantation. J Pediatr Surg 2005;40:1181-4.  Back to cited text no. 12
    
13.
Rubikas R. Diaphragmatic injuries. Eur J Cardiothorac Surg 2001;20:53-7.  Back to cited text no. 13
    
14.
Reber PU, Schmied B, Seiler CA, Baer HU, Patel AG, Büchler MW. Missed diaphragmatic injuries and their long-term sequelae. J Trauma 1998;44:183-8.  Back to cited text no. 14
    
15.
Dökümcü Z, Divarcı E, Erdener A, Sözbilen M, Ergün O. Acquired right diaphragmatic hernia following pediatric living donor orthotopic liver transplantation. Pediatr Transplant 2015;19:E149-51.  Back to cited text no. 15
    
16.
Steinbrück K, Fernandes R, Enne M. Diaphragmatic hernia after adult orthotopic liver transplantation: A rare surgical complication associated to immunosuppression? J Clin Case Rep 2015;5:11.  Back to cited text no. 16
    
17.
Kahn J, Müller H, Iberer F, Kniepeiss D, Duller D, Rehak P, et al. Incisional hernia following liver transplantation: Incidence and predisposing factors. Clin Transplant 2007;21:423-6.  Back to cited text no. 17
    
18.
Schaubel DE, Sima CS, Goodrich NP, Feng S, Merion RM. The survival benefit of deceased donor liver transplantation as a function of candidate disease severity and donor quality. Am J Transplant 2008;8:419-25.19  Back to cited text no. 18
    
19.
Rossetto A, Baccarani U, Adani GL, Lorenzin D, Bresadola V, Terrosu G. Diaphragm rupture in a liver transplant patient under chronic immunosuppressive therapy with sirolimus: Rare complication after liver transplantation. Updates Surg 2011;63:51-3.  Back to cited text no. 19
    


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