Right Sided Bochdalek Hernia with Ureteric Involvement – A Case Report and Review of Management Options

A B S T R A C T

Ureteric herniation through a diaphragmatic defect in adults (Bochdalek hernia – BH) is rare, with less than 15 cases reported in the literature. Most patients present with non-specific abdominal symptoms with or without worsening of renal function, and therefore, the clinical picture can be masked [1]. Management of ureteric involvement ranges from conservative to reconstructive surgery. We report a case of an elderly (>80-year-old) patient who presented acutely with symptoms mimicking an acute abdomen and on imaging was found to have a BH associated with herniation of right ureter causing hydro-ureter. We managed the patient conservatively, and she improved symptomatically, which was evident from her follow-ups. We provide a detailed case report of this situation as well as discuss the literature on the topic to guide treatment decision-making.

Keywords

Bochdalek hernia, diaphragmatic hernia, ureteric hernia, ureteric obstruction, rare ureteric obstruction, conservative management

Background

Ureteric herniation can occur at various anatomical sites, including inguinal canal, femoral canal, sciatic foramen, and diaphragmatic hiatus, with diaphragmatic hiatus being the least common [2, 3]. Florid obstructive uropathy is rarely the predominant clinical picture, with the presentation commonly that of non-specific symptoms including abdominal pain, dyspnea, chest pain, and nausea and vomiting, sometimes leading to ileus [4, 5]. Management of this can therefore be challenging with a missed diagnosis potentially leading to sepsis and multi-organ dysfunction. There is very little indication in the literature to guide the management of ureteric involvement in BH in terms of acute and definitive management. This case report illustrates the complexities associated with the diagnosis and management of BH with ureteric involvement and deranged renal function. The accompanying review of the literature attempts to guide decision-making in this challenging scenario.

Case Report

An elderly patient presented with a 12-hour history of sudden onset right-sided abdominal pain, vomiting, bowels not opening and not passing flatus. It was her second admission in the month for similar complaints. She was known to have hypertension, chronic low backache for which she took codeine, and constipation for which she took lactulose. On admission, she was alert with a national early warning score (NEWS) of 1 for a pulse rate of 104. Abdominal examination revealed a non-distended, soft abdomen, with some tenderness in the right iliac fossa with diminished bowel sounds. Her urine dip test showed 1+ for blood, sugar, WCC, and ketones. Her blood tests showed a lactate of 2.1, elevated WCC 13.7 (normal- 4.00-11.0×109/L), creatinine- 125 (normal- 45-84 µmol/L) and eGFR- 36 (normal- 70-130 ml/min).

CT Abdomen and pelvis with contrast diagnosed herniation of the proximal right ureter through a posteromedial defect in the right hemidiaphragm (Figures 1 & 2). There was associated moderate right hydronephrosis with good contrast uptake in the right kidney. There was also herniated fat just above the right hemidiaphragm. Urgent urology review was sought. She was offered emergency stenting vs. conservative management. A joint decision was taken to consider a trial of conservative management with potential escalation to intervention if the patient deteriorated. She was managed conservatively with IV fluids, anti-emetics, and analgesics. On her repeat blood tests, the WCC was down to 9.7, creatinine 102, and eGFR-45. She was discharged home with an outpatient referral to urology for further work up and management.

Figure 1: Coronal plane showing the right sided herniated ureter.

Figure 2: Sagittal view showing the posterior diaphragmatic defect through which the ureter is coursing.

She further had 2 visits to A&E, once in June and then in august of 2018, with pain in the right abdomen and no other bowel obstruction symptoms. During out-patient urology review, her workup showed a serum creatinine of 117 and eGFR-38. The urology team performed a MAG3 scan (Figure 3), which demonstrated a hydronephrotic, poorly draining but non obstructed right kidney with only mildly impaired function (equal spilt function). Conservative management was continued and future urology out-patient clinic appointment for renal function follow-up were set up, and these continue. She hasn’t had any worsening of renal function or episodes of pyelonephritis on follow up.

Figure 3: MAG3 renogram curves showing reduced uptake and outflow but equal split function.

Discussion

Bochdalek hernia is predominantly seen in children less than 1-year-old with respiratory symptoms and has a poor prognosis [6]. It is caused due to a failure of fusion of the pars lumbalis and pars costalis parts of the diaphragm [7]. Most adults with BH are diagnosed incidentally on imaging. Studies using MDCT and multiplanar reconstruction estimate a prevalence of up to 10.5% in patients having scans for other reasons [8]. Asymptomatic cases most often present equally on the right and left side, whereas symptomatic cases are more common on left with an incidence of 9:1 compared to the right [9]. However, ureteric involvement seems to always present on the right, as noted in the 14 cases reported in the literature. Table 1 summarizes the literature review.

Table 1: This summarizes the management plans for patients with diaphragmatic hernia with ureteric involvement as reported in the literature. Table modified from Abou Heider et al. [14].

Authors (Year of publication)

Age/gender

Laterality

Presentation

Acute Management

Outcome of acute management

Investigations

Indication for definitive management

Definitive management

Outcome of definitive management

Swithinbank (1958)

60/F

Right

Intermittent right flank pain with radiation to pelvis

Morphine, spontaneous resolution

Pain subsidence

Intravenous pyelogram showing loop of ureter with intrathoracic route, hydronephrosis

Refractory symptoms

Surgical repair – herniation reduced, and hiatus closed

Intra- venous pyelography, showed the right ureter pursuing a more normal, sub-diaphragmatic course

Paterson and Lupton (1989)

75/M

Right

Right hypochondrial pain, nausea and vomiting

Morphine, spontaneous resolution

Pain subsidence

IVP, CT showing herniation of PUJ Renogram confirming obstruction

Refractory symptoms

Surgical repair – side to side pelviureteric anastomosis

IVP showed normal ureteric course

Chawla and Mond (1994)

56/M   

Right   

Intermittent flank pain on both sides

Pain relief

Pain subsidence

IVP, CT showing herniation but no obstruction

none

Conservative

Intermittent symptoms with spontaneous resolution

Catalano et al. (1998)           

63/F    

 

Right   

Upon workup for renal stones  

ESWL for stone, pain relief

Pain subsidence

IVP, CT showing abnormal course of ureter with some dilatation

To prevent obstruction with stone fragment after ESWL, and to prevent further stones

Surgical removal of renal stone, reduction of hernia and repair of the diaphragm.

No further stones, normal course of ureter

Sukumar et al. (2010)                       

75/F

Right               

Incidental finding on workup for renal failure and respiratory infection

Supportive management

Pain subsidence

CT, retrograde studies showed Curlicue sign. Prompt drainage suggesting no obstruction

None

Conservative

 

No report of further respiratory or renal compromise

Balakrishnan and Neerhut (2011) 

 

83/F    

Right   

Intermittent Right flank pain     

Retrograde ureteric stent insertion

Resolution of pain

CT showing herniation

none

6 monthly stent changes

No recurrence of pain

Song et al. (2011) 

75/M   

Right   

Right upper quadrant pain           

Retrograde ureteric stent insertion. Removal of stent

Resolution of pain and obstruction

CT showing herniation

Patient opted not to have surgery, no recurrence of hydronephrosis

conservative

No recurrence of pain or hydronephrosis

Hatzidakis et al. (2014)           

86/F                            

 

Right

Septic obstructive pyelonephritis

Nephrostomy followed by antegrade ureteric stenting

Relief of sepsis and obstruction

CT showing obstruction and herniation

none

Stent insertion and change

Straightening of course of ureter.

Almeida et al. (2015)                                   

82/F

Right

Incidental on PET scan for workup of lung nodule

None mentioned

None mentioned

CT showing a knuckle of ureter in chest

None mentioned

Not mentioned

 

None mentioned

Dru and Josephson (2016)                                     

94/F

Right

Sharp right flank pain

Cystoscopy and retrograde stent insertion

Resolution of obstruction

CT showing herniation and obstruction

None mentioned

None mentioned

None mentioned

Lin et al. (2017)                                     

81/F

Right

Right flank pain and renal obstruction

Initial conservative management, then retrograde ureteral stenting on progression of hydronephrosis and MAG3 showing obstruction

Resolution of obstruction

CT showing herniation. Mag3 showed reduced function on the right

Worsening herniation despite stent, presence of stented ureter in thorax

Surgical repair of defect, nephropexy, excision of redundant ureter and pelviureteric end-to-end anastomosis

No herniation or obstruction

Beland et al. (2019)                         

 

84/F

Right   

Obstructing ureteric stone in a herniated ureter

Flexile ureteroscopy and LASER fragmentation of the stone

Removal of stone, persistence of herniation

CT showed stone and ureteric herniation

None mentioned

None mentioned

None mentioned

Abou Heidar et al. (2019)

71/M

Right

Abdominal pain, vomiting and lose stools

Conservative

Improvement in symptoms

CT showing herniation

None

Conservative

None

Current case

>80/F

Right

Right sided abdominal pain

conservative

Improvement in symptoms

CT showing herniation, MAG3 showing no obstruction and normal split renal function

none

Conservative

none


The pathophysiology for this has never been completely elucidated. The origin is undoubtedly embryonic as the presence of BH is an embryonic occurrence [10]. As this is a non-dependent location for herniation, the presentation is probably related more to episodes of sustained raised intra-abdominal pressure as well as weakening of the diaphragm, which probably becomes more apparent with age [10]. Most of the cases report herniation of retroperitoneal fat along with the ureter correlating the pressure hypothesis. Most of the patients reported in the literature had some sort of pulmonary issues such as COPD, bronchitis etc., which could have precipitated the herniation over time.

Of these reported cases, 2/14 presentations were incidental while investigating for other pathologies. The remaining patients presented with mild right sided abdominal discomfort. Only one patient in the literature presented with septic obstructed system [4]. Management varied from conservatively (5 cases), surgical repair (4 cases), and ureteral stenting (4 cases). In two cases reports, the management wasn’t mentioned [3, 4, 10-19]. The range and median age of presentation were 56-94 years and 75 years respectively demonstrating that the ureteric herniation had very little impact on these patients for the most part of their lives. Male to female incidence was 5:9.

Indication for treatment has not been clearly defined in the literature. There are 5 cases in the literature that shows that conservative management is feasible without any adverse outcomes. Our case shows this similarly. Patients undergoing ureteric stent placement seem to have had them placed for resolution of pain and to treat the episode of acute obstructive uropathy [17]. There is conflicting evidence for the use of ureteric stents in the treatment of obstructive uropathy, where pain is the driving factor for management, as stents themselves can cause pain. Reports that have suggested the use of stents have advised this in the presence of objective evidence of renal obstruction on imaging. They have also cautioned readers on the use of stents as the ureter can be very tortuous, necessitating the use of stiff guide wires and longer stents to prevent upward migration. In some patients, stents reduced the herniation, whereas in others, persistence of herniation despite stent was an indication for reconstructive surgery. In our discussion with the patient, her main symptom was pain, with some element of acute obstructive uropathy. Despite these findings, a trial of conservative management with IV fluids and IV antibiotics proved fruitful.

It is essential for those presenting with a septic obstructed system to have drainage either in the form of a nephrostomy or with a ureteric stent. Hatzidakis et al. further illustrate how retrograde stent insertion can be challenging in these situations because of the tortuosity of the ureter and the abnormal anatomical path it traverses [4].

The indication for major reconstructive surgery has been reported to be to reduce the potential for further incarceration and to get patients stent free. Long-term follow-up has not been reported, and therefore the effectiveness of this approach to management needs to be ascertained. The surgical treatment most reported involves plication of the diaphragm with one case mentioning nephropexy, excision of redundant ureter and ureteroplasty. It is interesting to note that most descriptions of major reconstructive surgery were from reports before 2000, and the more recent reports tend to treat this with conservative management.

Decision for major reconstructive surgery needs to be taken with caution as most of these patients seem to be older with comorbidities. We used a MAG3 renogram to guide us in the decision-making process, and as its results were reassuring, we proceeded with conservative management and managed to avoid the risks of major surgery.

Conclusion

Ureteric herniations through BH are uncommon and are picked up on non-invasive imaging for asymptomatic patients or patients with non-specific bowel and chest symptoms. Our case illustrates that uncomplicated patients without objective evidence of obstructive, decline in renal function or sepsis can be managed conservatively with imaging and blood test follow up without intervention. This is particularly attractive as most patients presenting with these findings are of older age with potential underlying comorbidities in whom surgery might not be safe. The literature review attempts to guide clinicians in the management of this challenging and rare condition.

Article Info

Article Type
Case Report and Review of the Literature
Publication history
Received: Tue 01, Dec 2020
Accepted: Wed 16, Dec 2020
Published: Fri 25, Dec 2020
Copyright
© 2021 Shruthi Ashwin Sridhar. This is an open-access article distributed under the terms of the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original author and source are credited. Hosting by Science Repository. All rights reserved.
DOI: 10.31487/j.SCR.2020.12.24

Author Info

Corresponding Author
Shruthi Ashwin Sridhar
Department of General Surgery, Guy’s and St Thomas’ Hospital NHS Foundation Trust, UK

Figures & Tables

Table 1: This summarizes the management plans for patients with diaphragmatic hernia with ureteric involvement as reported in the literature. Table modified from Abou Heider et al. [14].

Authors (Year of publication)

Age/gender

Laterality

Presentation

Acute Management

Outcome of acute management

Investigations

Indication for definitive management

Definitive management

Outcome of definitive management

Swithinbank (1958)

60/F

Right

Intermittent right flank pain with radiation to pelvis

Morphine, spontaneous resolution

Pain subsidence

Intravenous pyelogram showing loop of ureter with intrathoracic route, hydronephrosis

Refractory symptoms

Surgical repair – herniation reduced, and hiatus closed

Intra- venous pyelography, showed the right ureter pursuing a more normal, sub-diaphragmatic course

Paterson and Lupton (1989)

75/M

Right

Right hypochondrial pain, nausea and vomiting

Morphine, spontaneous resolution

Pain subsidence

IVP, CT showing herniation of PUJ Renogram confirming obstruction

Refractory symptoms

Surgical repair – side to side pelviureteric anastomosis

IVP showed normal ureteric course

Chawla and Mond (1994)

56/M   

Right   

Intermittent flank pain on both sides

Pain relief

Pain subsidence

IVP, CT showing herniation but no obstruction

none

Conservative

Intermittent symptoms with spontaneous resolution

Catalano et al. (1998)           

63/F    

 

Right   

Upon workup for renal stones  

ESWL for stone, pain relief

Pain subsidence

IVP, CT showing abnormal course of ureter with some dilatation

To prevent obstruction with stone fragment after ESWL, and to prevent further stones

Surgical removal of renal stone, reduction of hernia and repair of the diaphragm.

No further stones, normal course of ureter

Sukumar et al. (2010)                       

75/F

Right               

Incidental finding on workup for renal failure and respiratory infection

Supportive management

Pain subsidence

CT, retrograde studies showed Curlicue sign. Prompt drainage suggesting no obstruction

None

Conservative

 

No report of further respiratory or renal compromise

Balakrishnan and Neerhut (2011) 

 

83/F    

Right   

Intermittent Right flank pain     

Retrograde ureteric stent insertion

Resolution of pain

CT showing herniation

none

6 monthly stent changes

No recurrence of pain

Song et al. (2011) 

75/M   

Right   

Right upper quadrant pain           

Retrograde ureteric stent insertion. Removal of stent

Resolution of pain and obstruction

CT showing herniation

Patient opted not to have surgery, no recurrence of hydronephrosis

conservative

No recurrence of pain or hydronephrosis

Hatzidakis et al. (2014)           

86/F                            

 

Right

Septic obstructive pyelonephritis

Nephrostomy followed by antegrade ureteric stenting

Relief of sepsis and obstruction

CT showing obstruction and herniation

none

Stent insertion and change

Straightening of course of ureter.

Almeida et al. (2015)                                   

82/F

Right

Incidental on PET scan for workup of lung nodule

None mentioned

None mentioned

CT showing a knuckle of ureter in chest

None mentioned

Not mentioned

 

None mentioned

Dru and Josephson (2016)                                     

94/F

Right

Sharp right flank pain

Cystoscopy and retrograde stent insertion

Resolution of obstruction

CT showing herniation and obstruction

None mentioned

None mentioned

None mentioned

Lin et al. (2017)                                     

81/F

Right

Right flank pain and renal obstruction

Initial conservative management, then retrograde ureteral stenting on progression of hydronephrosis and MAG3 showing obstruction

Resolution of obstruction

CT showing herniation. Mag3 showed reduced function on the right

Worsening herniation despite stent, presence of stented ureter in thorax

Surgical repair of defect, nephropexy, excision of redundant ureter and pelviureteric end-to-end anastomosis

No herniation or obstruction

Beland et al. (2019)                         

 

84/F

Right   

Obstructing ureteric stone in a herniated ureter

Flexile ureteroscopy and LASER fragmentation of the stone

Removal of stone, persistence of herniation

CT showed stone and ureteric herniation

None mentioned

None mentioned

None mentioned

Abou Heidar et al. (2019)

71/M

Right

Abdominal pain, vomiting and lose stools

Conservative

Improvement in symptoms

CT showing herniation

None

Conservative

None

Current case

>80/F

Right

Right sided abdominal pain

conservative

Improvement in symptoms

CT showing herniation, MAG3 showing no obstruction and normal split renal function

none

Conservative

none


Science Repository

Figure 1: Coronal plane showing the right sided herniated ureter.


Science Repository

Figure 2: Sagittal view showing the posterior diaphragmatic defect through which the ureter is coursing.


Science Repository

Figure 3: MAG3 renogram curves showing reduced uptake and outflow but equal split function.



References

  1. Hamid KS, Rai SS, Rodriguez JA (2010) Symptomatic bochdalek hernia in an adult. JSLS 14: 279-281. [Crossref]
  2. Bertolaccini L, Giacomelli G, Bozzo RE, Gastaldi L, Moroni M (2005) Inguino-scrotal hernia of a double district ureter: case report and literature review. Hernia 9: 291-293. [Crossref]
  3. Lin FC, Lin JS, Kim S, Walker JR (2017) A rare diaphragmatic ureteral herniation case report: endoscopic and open reconstructive management. BMC Urol 17: 26. [Crossref]
  4. Hatzidakis A, Kozana A, Glaritis D, Mamoulakis C (2014) Right-sided Bochdalek hernia causing septic ureteric obstruction. Percutaneous treatment with placement of a nephroureteral double pigtail. BMJ Case Rep 2014: bcr2014207247. [Crossref]
  5. Brown SR, Horton JD, Trivette E, Hofmann LJ, Johnson JM (2011) Bochdalek hernia in the adult: Demographics, presentation, and surgical management. Hernia 15: 23-30. [Crossref]
  6. Gedik E, Tuncer MC, Onat S, Avci A, Tacyildiz I (2011) A review of Morgagni and Bochdalek hernias in adults. Folia Morphol 70: 5-12. [Crossref]
  7. Mullins ME, Stein J, Saini SS, Mueller PR (2001) Prevalence of incidental Bochdalek’s hernia in a large adult population. AJR Am J Roentgenol 177: 363-366. [Crossref]
  8. Temizöz O, Gençhellaç H, Yekeler E, Umit H, Unlü E et al. (2010) Prevalence and MDCT characteristics of asymptomatic Bochdalek hernia in adult population. Diagn Interv Radiol 16: 52-55. [Crossref]
  9. Sandstrom CK, Stern EJ (2011) Diaphragmatic Hernias: A Spectrum of Radiographic Appearances. Curr Probl Diagn Radiol 40: 95-115. [Crossref]
  10. Swithinbank AH (1958) Intrathoracic deviation of a ureteric loop. Br J Surg 45: 379-381. [Crossref]
  11. Chawla K, Mond DJ (1994) Progressive bochdalek hernia with unusual ureteral herniation. Comput Med Imaging Graph 18: 53-58. [Crossref]
  12. Sukumar S, Kumar PG, Thomas A (2010) Thoracic curlicue: A case of ureteral herniation. Indian J Urol 26: 131-132. [Crossref]
  13. Song YS, Hassani C, Nardi PM (2011) Bochdalek hernia with obstructive uropathy. Urology 77: 1338. [Crossref]
  14. Heidar NA, Elsemesmani H, Elamine A, Natout M (2019) A Rare Case of Isolated Ureteral Diaphragmatic Herniation: Case Report and Review of Literature. Case Rep Urol 2019: 1092018. [Crossref]
  15. Paterson IS, Lupton EW (1989) Periureteric Junction Obstruction Secondary to Renal Pelvic Incarceration in a Congenital Diaphragmatic Hernia. Br J Urol 64: 548-549. [Crossref]
  16. Catalano O, Nunziata A, Cusati B, Siani A (1998) Retrocrural loop of the ureter: CT findings. Am J Roentgenol 170: 1293-1294. [Crossref]
  17. Balakrishnan V, Neerhut G (2011) Right sided Bochdalek hernia causing ureteric obstruction. BJUI 2011-104.
  18. Dru CJ, Josephson DY (2016) Bochdalek-type Diaphragmatic Hernia Leading to High-grade Kidney Obstruction. Urology 97: e17-e18. [Crossref]
  19. Beland LE, Henry MA, Solomon T, Ogan K (2019) Obstructing Nephrolithiasis in an 84-year-old Patient With a Diaphragmatic Herniated Ureter. Urology 124: e1-e3. [Crossref]