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Symptomatic congenital diaphragmatic hernia following right nephrectomy We report a unique case of a congenital diaphragmatic hernia unveiled following a right nephrectomy in a 74-year-old woman, presenting 9 months later as a large bowel obstruction. A previously well 74-year-old woman underwent a laparoscopic right nephrectomy in March 2013 for a 39-mm right renal cell carcinoma. The operation went well with no diaphragmatic injury sustained and the patient recovered without incident. The patient presented in December, 9 months later, with several weeks of colicky lower abdominal pain and nausea. Her bowels were opening daily. She was haemodynamically stable with normal biochemical markers including a normal white cell count and C-reactive protein. A computed tomography (CT) abdomen showed caecal dilatation with an ‘unusual mass lesion containing gas pockets in the posterior aspect of the right upper quadrant’. A contrast enema study showed contrast up to the hepatic flexure, with no significant flow beyond. A colonoscopy was performed, but was unable to pass the hepatic flexure due to a kink or external compression. No mucosal abnormality was seen. In the following days, the patient experienced progressively worse symptoms, with increasing colicky pain and cessation of flatus. Due to clinical progression to a complete large bowel obstruction and the uncertain aetiology, the decision was made to proceed with an exploratory laparotomy.
Laparotomy revealed herniation of the hepatic flexure through a diaphragmatic defect, in a position typical of a Bochdalek hernia. There was a well-formed sac associated with the hernia and the neck of the defect was thick, requiring incision to release the hepatic flexure. The colon remained viable. An intercostal chest tube was inserted, and the defect was subsequently primarily closed. The patient recovered well and was discharged on day 14 of admission. Retrospective review of imaging performed prior to nephrectomy reveals a probable Bochdalek hernia over which lies perinephric fat and the right kidney (Fig. 1). Surveillance CT scans for monitoring of a pulmonary nodule incidentally shows progressive herniation
Fig. 1. Abdominal computed tomography scan before nephrectomy, showing right kidney with renal cell carcinoma in situ (black arrow) and perinephric fat overlying diaphragmatic defect. Inset shows diaphragm (yellow line) with defect (white arrow).
Fig. 2. Series of surveillance computed tomography scans (for monitoring a lung nodule) at 2, 5, 8 and 9 months post-nephrectomy, showing large bowel within renal fossa and progressive herniation of large bowel through diaphragmatic defect (white arrow).
© 2014 Royal Australasian College of Surgeons
ANZ J Surg •• (2014) ••–••
2
Images for surgeons
More recent CT and autopsy studies have revealed that the incidence of asymptomatic Bochdalek hernia in adulthood is higher than previously thought, with an estimated range between 0.17% and 12.7%.8–10 Asymptomatic hernias are often associated with liver, gallbladder, kidney and/or omentum, and occur more commonly on the right side and in women. To our knowledge, this is the first case describing a previously asymptomatic congenital hernia unveiled after nephrectomy. Misdiagnosis of diaphragmatic hernias is common, regardless of aetiology, due to the wide variation in symptoms and the rarity of the defect. In the literature, only 65% of Bochdalek hernia cases were diagnosed preoperatively.7 Although uncommon in adults, these abnormalities should be known and managed appropriately to avoid potential dire consequences, and associated morbidity and mortality.
References Fig. 3. Contrast enema showing cut-off at hepatic flexure.
through the defect (Fig. 2) with complete bowel obstruction 9 months after nephrectomy (Fig. 3). First described by Victor Alexander Bochdalek in 1867, a Bochdalek hernia is a congenital hernia created by improper fusion of the posterolateral foramina of the diaphragm.1 These are the most common type of congenital diaphragmatic hernias. Over 95% of cases are diagnosed in the neonatal period due to associated pulmonary insufficiency.2,3 There are only 173 cases of symptomatic Bochdalek hernias reported in the adult population in worldwide literature. The mean age of presentation is 40 years old, but there is a wide age range, from 15 to 88 years. Adult presentations of Bochdalek hernias are often insidious and intermittent, with mainly vague gastrointestinal symptoms (abdominal pain, nausea, vomiting, constipation) or respiratory symptoms (chest pain, dyspnoea, wheezing).3–5 Others present with serious complications of incarceration or strangulation, necessitating emergency surgery.2,6 A precipitating factor was noted in only 25% of reported cases. Common precipitating factors were pregnancy and exertion, but also included trauma, large meals, retching and coughing.7 Surgical repair has been performed via five different approaches: laparotomy, thoracotomy, laparoscopy, thoracoscopy or a combination of these. For emergent cases, laparotomy was the most common approach; however, laparoscopic and thoracoscopic repairs have been performed successfully in the last two decades on an elective basis.7
1. Salacin S, Alper B, Cekin N, Gulmen M. Bochdalek hernia in adulthood: a review and an autopsy case report. J. Forensic Sci. 1994; 39: 1112–6. 2. Kacakusak A, Arikan S, Senturk O, Ycel A. Bochdalek’s hernia in an adult with colon necrosis. Hernia 2005; 9: 284–7. 3. Osebold W, Soper R. Congenital posterolateral diaphragmatic hernia past infancy. Am. J. Surg. 1976; 131: 748–54. 4. Ahrend T, Thompson B. Hernia of the foramen of Bochdalek in the adult. Am. J. Surg. 1971; 122: 612–5. 5. Perhomiemi V, Helminen J, Luosto R. Posterolateral diaphragmatic hernia in adults: acute symptoms, diagnosis and treatment. Scand. J. Thorac. Cardiovasc. Surg. 1992; 26: 225–7. 6. Nouheim K. Adult presentation of unusual diaphragmatic hernias. Chest Surg. Clin. N. Am. 1998; 8: 359–69. 7. Brown S, Horton J, Trivette E, Hofmann L, Johnson J. Bochdalek hernia in the adult: demographics, presentation, and surgical management. Hernia 2011; 15: 23–30. 8. Temizoz O, Genchellac H, Yekeler E et al. Prevalence and MDCT characteristics of asymptomatic Bochdalek hernia in adult population. Diagn. Interv. Radiol. 2010; 16: 52–5. 9. Mullins M, Stein J, Saini S, Mueller P. Prevalence of incidental Bochdalek’s hernia in a large adult population. AJR Am. J. Roentgenol. 2001; 177: 363–6. 10. Gale M. Bochdalek hernia: prevalence and CT characteristics. Radiology 1985; 156: 449–52.
Geraldine Ooi, MBBS, BMedSc David Lloyd, MBBS, FRACS Department of Surgery, Launceston General Hospital, Launceston, Tasmania, Australia doi: 10.1111/ans.12856
© 2014 Royal Australasian College of Surgeons
Symptomatic congenital diaphragmatic hernia following right nephrectomy We report a unique case of a congenital diaphragmatic hernia unveiled following a right nephrectomy in a 74-year-old woman, presenting 9 months later as a large bowel obstruction. A previously well 74-year-old woman underwent a laparoscopic right nephrectomy in March 2013 for a 39-mm right renal cell carcinoma. The operation went well with no diaphragmatic injury sustained and the patient recovered without incident. The patient presented in December, 9 months later, with several weeks of colicky lower abdominal pain and nausea. Her bowels were opening daily. She was haemodynamically stable with normal biochemical markers including a normal white cell count and C-reactive protein. A computed tomography (CT) abdomen showed caecal dilatation with an ‘unusual mass lesion containing gas pockets in the posterior aspect of the right upper quadrant’. A contrast enema study showed contrast up to the hepatic flexure, with no significant flow beyond. A colonoscopy was performed, but was unable to pass the hepatic flexure due to a kink or external compression. No mucosal abnormality was seen. In the following days, the patient experienced progressively worse symptoms, with increasing colicky pain and cessation of flatus. Due to clinical progression to a complete large bowel obstruction and the uncertain aetiology, the decision was made to proceed with an exploratory laparotomy.
Laparotomy revealed herniation of the hepatic flexure through a diaphragmatic defect, in a position typical of a Bochdalek hernia. There was a well-formed sac associated with the hernia and the neck of the defect was thick, requiring incision to release the hepatic flexure. The colon remained viable. An intercostal chest tube was inserted, and the defect was subsequently primarily closed. The patient recovered well and was discharged on day 14 of admission. Retrospective review of imaging performed prior to nephrectomy reveals a probable Bochdalek hernia over which lies perinephric fat and the right kidney (Fig. 1). Surveillance CT scans for monitoring of a pulmonary nodule incidentally shows progressive herniation
Fig. 1. Abdominal computed tomography scan before nephrectomy, showing right kidney with renal cell carcinoma in situ (black arrow) and perinephric fat overlying diaphragmatic defect. Inset shows diaphragm (yellow line) with defect (white arrow).
Fig. 2. Series of surveillance computed tomography scans (for monitoring a lung nodule) at 2, 5, 8 and 9 months post-nephrectomy, showing large bowel within renal fossa and progressive herniation of large bowel through diaphragmatic defect (white arrow).
© 2014 Royal Australasian College of Surgeons
ANZ J Surg •• (2014) ••–••
2
Images for surgeons
More recent CT and autopsy studies have revealed that the incidence of asymptomatic Bochdalek hernia in adulthood is higher than previously thought, with an estimated range between 0.17% and 12.7%.8–10 Asymptomatic hernias are often associated with liver, gallbladder, kidney and/or omentum, and occur more commonly on the right side and in women. To our knowledge, this is the first case describing a previously asymptomatic congenital hernia unveiled after nephrectomy. Misdiagnosis of diaphragmatic hernias is common, regardless of aetiology, due to the wide variation in symptoms and the rarity of the defect. In the literature, only 65% of Bochdalek hernia cases were diagnosed preoperatively.7 Although uncommon in adults, these abnormalities should be known and managed appropriately to avoid potential dire consequences, and associated morbidity and mortality.
References Fig. 3. Contrast enema showing cut-off at hepatic flexure.
through the defect (Fig. 2) with complete bowel obstruction 9 months after nephrectomy (Fig. 3). First described by Victor Alexander Bochdalek in 1867, a Bochdalek hernia is a congenital hernia created by improper fusion of the posterolateral foramina of the diaphragm.1 These are the most common type of congenital diaphragmatic hernias. Over 95% of cases are diagnosed in the neonatal period due to associated pulmonary insufficiency.2,3 There are only 173 cases of symptomatic Bochdalek hernias reported in the adult population in worldwide literature. The mean age of presentation is 40 years old, but there is a wide age range, from 15 to 88 years. Adult presentations of Bochdalek hernias are often insidious and intermittent, with mainly vague gastrointestinal symptoms (abdominal pain, nausea, vomiting, constipation) or respiratory symptoms (chest pain, dyspnoea, wheezing).3–5 Others present with serious complications of incarceration or strangulation, necessitating emergency surgery.2,6 A precipitating factor was noted in only 25% of reported cases. Common precipitating factors were pregnancy and exertion, but also included trauma, large meals, retching and coughing.7 Surgical repair has been performed via five different approaches: laparotomy, thoracotomy, laparoscopy, thoracoscopy or a combination of these. For emergent cases, laparotomy was the most common approach; however, laparoscopic and thoracoscopic repairs have been performed successfully in the last two decades on an elective basis.7
1. Salacin S, Alper B, Cekin N, Gulmen M. Bochdalek hernia in adulthood: a review and an autopsy case report. J. Forensic Sci. 1994; 39: 1112–6. 2. Kacakusak A, Arikan S, Senturk O, Ycel A. Bochdalek’s hernia in an adult with colon necrosis. Hernia 2005; 9: 284–7. 3. Osebold W, Soper R. Congenital posterolateral diaphragmatic hernia past infancy. Am. J. Surg. 1976; 131: 748–54. 4. Ahrend T, Thompson B. Hernia of the foramen of Bochdalek in the adult. Am. J. Surg. 1971; 122: 612–5. 5. Perhomiemi V, Helminen J, Luosto R. Posterolateral diaphragmatic hernia in adults: acute symptoms, diagnosis and treatment. Scand. J. Thorac. Cardiovasc. Surg. 1992; 26: 225–7. 6. Nouheim K. Adult presentation of unusual diaphragmatic hernias. Chest Surg. Clin. N. Am. 1998; 8: 359–69. 7. Brown S, Horton J, Trivette E, Hofmann L, Johnson J. Bochdalek hernia in the adult: demographics, presentation, and surgical management. Hernia 2011; 15: 23–30. 8. Temizoz O, Genchellac H, Yekeler E et al. Prevalence and MDCT characteristics of asymptomatic Bochdalek hernia in adult population. Diagn. Interv. Radiol. 2010; 16: 52–5. 9. Mullins M, Stein J, Saini S, Mueller P. Prevalence of incidental Bochdalek’s hernia in a large adult population. AJR Am. J. Roentgenol. 2001; 177: 363–6. 10. Gale M. Bochdalek hernia: prevalence and CT characteristics. Radiology 1985; 156: 449–52.
Geraldine Ooi, MBBS, BMedSc David Lloyd, MBBS, FRACS Department of Surgery, Launceston General Hospital, Launceston, Tasmania, Australia doi: 10.1111/ans.12856
© 2014 Royal Australasian College of Surgeons
