Stesura Seveso 327Archivio Italiano di Urologia e Andrologia 2015; 87, 4 CASE REPORT Intraoperative presentation of Bochdalek’s hernia in an adult during robotic-assisted partial nephrectomy: An uncommon situation and literature review Luca Cindolo 1, Francesco Berardinelli 1, Arianna Manzi 2, Francesca Spagnuolo 2, Elisa Fabbri 2, Pietro Castellan 1, Flavia Petrini 2, Luigi Schips 1 1 Urology Dept, Robotic Unit, ASL Abruzzo 2, Italy; 2 UOC Anestesia Rianimazione e Terapia Intensiva, ASL Abruzzo 2, Italy. Bochdalek's diaphragmatic hernia (BDH) is a congenital defect of the diaphragm that usually present during the neonatal period and rarely remain silent until adulthood. We present a 45-year-old-female case with diagnosis of dou- ble left kidney tumor prepared for robot-assisted partial nephrectomy (RPN). During the preoperative procedure she had a reduction of inspiratory volumes and increased pul- monary pressures: the robotic camera revealed the incidental presence of the left diaphragmatic defect. We report a simul- taneous nephron sparing surgery (NSS) and left posterolater- al BDH correction done by the da Vinci Surgical Robot (Intuitive Surgical, Sunnyvale, CA). KEY WORDS: Bochdalek’s hernia; Diaphragmatic defect; Renal tumor; Partial nephrectomy; da Vinci robot. Submitted 30 June; Accepted 30 September Summary No conflict of interest declared. left posterolateral BDH in adult done by the Da Vinci Surgical Robot (Intuitive Surgical, Sunnyvale, CA). CASE REPORT A 45-year-old female was admitted in our clinic after an episode of acute flank pain and the CT scan revealed a double left renal masses. She refused a preliminary renal biopsy and opted for a robot-assisted partial nephrecto- my (RPN). In her medical history there was absence of abdominal or thoracic trauma or previous surgery. During physical examination we inspected no respirato- ry symptoms and normal abdomen. The preoperative chest X-ray, the Computed Tomography (CT) (Figure 1) and the Magnetic Resonance Imaging (MRI) (Figure2) were negative for secondary malignancy or for diaphrag- matic hernia. Her hematological investigations were nor- mal. During the initial preoperative workout of a RPN, she undergone general anesthesia, placed in flank position and pneumoperitoneum was established up to 12mmHg CO2, nevertheless there was an immediate reduction of inspiratory volumes and an increased pulmonary pres- sures. Haemodynamic parameters were steadily stable as well as peripheral arterial saturation. After checking the correct placement of the tube and evaluating the depth of anesthesia, the robotic camera revealed the presence of the left posterolateral BDH: the diaphragmatic defect was measured about 5 cm (Figure 3). The chest was retract- ed and visible; the pleura and the peritoneum were “in continuum”, without herniation of organs. No hernia sac was found. After a consultation with thoracic surgeon, the urologist decided to continue the procedure by a pure robot-assisted approach. The hernia was repaired with interrupted nonabsorbable sutures with Mersilene stitches and a single drainage was left in the thoracic cav- ity to avoid a pneumothorax (Figure 4). Then the planned RPN was done. The operative time was 170 min, the blood loss was 110 ml. The patient had an uncomplicated postoperative recov- ery; in the first post operative day (POD) the drain was clamped and removed in second POD. The POD 3 chest DOI: 10.4081/aiua.2015.4.327 INTRODUCTION Congenital diaphragmatic hernia (CDH) is a develop- mental anomaly characterized by a failure to form a con- tinuous sheet in order to separate the abdominal and thoracic cavities during the embryogenesis. Bochdalek’s hernias (BDH) are the posterolateral hernias and they are the most frequent (80%) with a prevalence of left side (85%) among right side (13%) or bilateral (2%) (1). As a result, BDH represents a perinatal pathology associated with significant morbidity and mortality: infants are affected by a severe pulmonary hypoplasia and pul- monary hypertension. BDH in adult is a rarely clinical entity and it remains silent until the adulthood, usually discovered incidental- ly, without specific symptoms or signs (2). Once diag- nosed, the surgical repair is the recommended therapy and the use of laparoscopy and thoracoscopy are safe surgical approaches with low morbidity and shorter hos- pital stay. Even more, with the evolution of surgical tech- nology, new approaches for repairing these defects can be developed. We present the first report of abdominal robotic repair of Archivio Italiano di Urologia e Andrologia 2015; 87, 4 L. Cindolo, F. Berardinelli, A. Manzi, F. Spagnuolo, E. Fabbri, P. Castellan, F. Petrini, L. Schips 328 clear cytoplasm, absence of necrosis or atypia, low proliferative index MIB-1, and negative surgical margins. The immunophenotype was: positive for AML on the epithelioid and vascular components; positive for HMB-45; focal positive for CD117; pos- itive for MELAN-A; positive for < 1% Ki67; negative for AMACR and Ck7. The clinical and radiological findings at 3-month follow-up were negative. DISCUSSION According to reported literature, Vincent Alexander Bochdalek first described this hernia in 1848. The exactly etiology is still under study, but however the disease is due to the failure of closure of the canal between the septum transversum and the esopha- gus during the 8th week of gestation (1). BDH are the posterolateral hernias and they are the most fre- quent (80%) with a prevalence of left side (85%) among right side (13%) or bilateral (2%) (2). In 25- 57% of cases BHD are associated with other con- genital anomalies and in 10-20% whit chromoso- mal disorders (3). As a result, BDH represents a perinatal pathology associated with significant mor- bidity and mortality: infants are affected by a severe pulmonary hypoplasia and pulmonary hyperten- sion. BDH in adult is a rarely clinical entity and it remains silent until the adulthood, usually discov- ered incidentally, without specific symptoms or signs (4). In a retrospective review of 13.138 CT scans, Mullins et al. found 22 patients with inciden- tal, asymptomatic BDH, which represents an inci- dence of 0.17%, with a female-male ratio of 17:5 (77% woman) (5). In adults, the BDH diagnosis is difficult because of its rarity; moreover, the variety of symptoms and its asymptomatic presentation may be easily confused whit other diseases. The majority of patients present generic chronic gastrointestinal symptoms and/or respiratory symptoms related to dyspnea or breath- lessness: presence of bowel sounds within the chest and the absence of breath sounds are typical findings (6). Contrariwise acute presentation of BDH include severe cardiorespiratory distress with cyanosis, tachypnea, tachycardia, abdominal pain and it’s rep- resent an emergency for the potential life threatening complications. The correct diagnosis is usually dis- covered as an incidental finding and the radiologic evaluation is required just to confirm. Frontal and lateral X-ray chest are the common exams performed to assess the presence of any herniations or abnor- mality. If the radiography is indeterminate, CT-scan and MRI offer a better visualization of the defect and help clinicians in the differential diagnosis. The man- agement of BDH consist in the surgical reduction of the herniations in the abdominal cavity and the repair of the diaphragm defect as soon as possible. Traditionally, it can be performed via laparotomy and/or thoracotomy, it depends from the scenario. Recently, in order to inflicts minimal surgical trauma, thoracoscopic and laparoscopic repair of BDH have also been reported, with excellent results. Figure 1. CT scan showed the left kidney tumor and the absence of any visceral herniations or collapsed lung. Figure 2. RMI confirmed the diagnosis and showed no presence of the BDH. Figure 3. Intraoperative photo of the left diaphragmatic defect. Figure 4. Intraoperative photo showing the Bochdalek diaphragmatic hernia repaired from the abdomen. x-ray showed a fully expanded left lung with no evidence of any herniated bowel loops in the thoracic cavity. The discharge occurred in POD 4 in good general clinical conditions. The histopathology revealed a double angiomyolipoma on the left kidney (4,1 x 3,5 cm and 3,3 x 2,6 cm) with focal areas of epithelioid cells with large Transthoracic approach allows to examine the thoracic cavity and herniated organs in detail; to avoid ischemic change, necrosis and perforation, even in the case of bilat- eral hernia; to separate adhesions between the contents with a good control of bleeding; to confirm the presence of lung hypoplasia and to easily reduce the hernia sac, if present (7). At the opposite, transabdominal approach is preferred for the management of the possible complica- tions (strangulation, malrotation, perforation, peritonitis), which are documented in many BDH, and it allows to examine the entire abdominal cavity, and easily repair the defect (8). In addition, several surgeons prefer to reinforce the suture whit some type of mesh. A recent literature search, showed only two reports of “robotic repair of BDH”. Meehan described a case report of a newborn of 37 weeks and Bethany presented a series of 8 consecutive newborns with diaphragmatic anomalies who underwent robotic repair (9, 10). We present the first report of patient underwent a pure robotic approach using the abdominal route and we noted that the articu- lating instruments offered an easy access to the postero- lateral region. Moreover, referred to newborns size, we didn’t have space problems and we could use the entire range of motion. We were able to close it primarily with- out any mesh, because there was sufficient diaphragmat- ic tissue to approximate. CONCLUSIONS In adulthood, symptomatic Bochdalek hernia is extreme- ly rare and correct diagnosis and early treatment is sig- nificant to avoid the occurrence of morbidity and a high- er complication rate. The application of robotic tech- niques to repair Bochdalek hernia is an excellent option because it’s feasible, reduces the morbidity of surgery, allows a short hospitalization and may result in signifi- cant clinical improvement. CONSENT Written informed consent was obtained from the patient for publication of this case report and any accompanying images. A copy of the written consent is available for review by the Editor-in-Chief of this journal. REFERENCES 1. Brown SR, Horton JD, Trivette E, et al. Bochdalek hernia in the adult: demographics, presentation, and surgical management. Hernia 2011; 15:23-30. 2. Greer, JJ. Current concepts on the pathogenesis and etiology of congenital diaphragmatic hernia. Respir Physiol Neurobiol. 2013; 189:232-40. 3. Zhou Y, Du H, Che G. Giant congenital diaphragmatic hernia in an adult. J Cardiothorac Surg. 2014; 9:31. 4. Kesieme EB, Kesieme CM. Congenital diaphragmatic hernia: review of current concept in surgical management. ISRN Surgery. 2011; 2011:974041. 5. Mullins, ME, Jeffrey S, Sanjay SS, Mueller PR. Prevalence of inci- dental Bochdalek’s hernia in a large adult population. AJR Am J Roentgenol. 2001; 177:363-66. 6. Hamid, KS, Rai SS, Rodriguez JA. Symptomatic Bochdalek hernia in an adult. JSLS. 2010; 14:279-81. 7. Tokumoto N, Tanabe K, Yamamoto H, et al. Thoracoscopic-assist- ed repair of a Bochdalek hernia in an adult: a case report. J Med Case Rep. 2010; 4:366. 8. Patle NM, Tantia O, Prasad P, et al. Laparoscopic repair of right sided Bochdalek hernia - a case report. Indian J Surg. 2013; 75:303-4. 9. Meehan JJ, Sandler A. Robotic repair of a Bochdalek congenital diaphragmatic hernia in a small neonate: robotic advantages and limitations. J Pediatr Surg. 2007; 42:1757-60. 10. Slater BJ, Meehan JJ. Robotic repair of congenital diaphragmat- ic anomalies. J Laparoendosc Adv Surg Tech A. 2009; 19:S123-7. 329Archivio Italiano di Urologia e Andrologia 2015; 87, 4 Intraoperative presentation of Bochdalek’s hernia in an adult during robotic-assisted partial nephrectomy: An uncommon situation and literature review Correspondence Luca Cindolo, MD Francesco Berardinelli, MD Pietro Castellan, MD (Corresponding Author) castellanpietro@gmail.com Luigi Schips, MD Urology Dept, via S. Camillo de Lellis 1 – 66054 Vasto, Italy Arianna Manzi, MD Francesca Spagnuolo, MD Elisa Fabbri, MD Flavia Petrini, MD UOC Anestesia Rianimazione e Terapia Intensiva, ASL Abruzzo 2, Italy