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ERCP and EUS technique in situs inversus totalis: preparing for a left-sided plot twist

Open AccessPublished:September 15, 2022DOI:https://doi.org/10.1016/j.vgie.2022.05.008

      Video

      (mp4, (100.19 MB)

      Abbreviations:

      CBD (common bile duct), PD (pancreatic duct), SIT (situs inversus totalis)

      Background

      Situs inversus totalis (SIT) is the rare occurrence of a mirror image reversal of the entire internal visceral anatomy with a reported incidence of 1 out of 8000 to 1 out of 25,000.
      • Mayo C.W.
      • Rice R.G.
      Situs inversus totalis: a statistical review of data on 76 cases with special reference to disease of the biliary tract.
      ,
      • Spoon J.M.
      Situs inversus totalis.
      The altered anatomy in SIT imposes significant challenges to performing diagnostic and therapeutic endoscopy successfully. We describe in this case report a successful EUS and ERCP in a patient with SIT.

      Case report

      A 64-year-old woman with a known history of SIT associated with complex repaired congenital cardiac abnormalities presented with a few days of worsening, dull, right lower-quadrant abdominal pain and a week of acholic stools, jaundiced skin, and diffuse pruritis. Physical examination was notable for significant jaundice and mild bilateral lower quadrant tenderness. Blood tests revealed elevated aspartate aminotransferase (197 U/L), alanine aminotransferase (141 U/L), alkaline phosphatase (814 U/L), and total bilirubin (28.3 mg/dL). A CT scan of the abdomen revealed moderate intrahepatic biliary dilation with a nondistended common bile duct and a heterogeneously enhancing gallbladder wall thickening with a small amount of wall calcification (Figs. 1 and 2).
      Figure thumbnail gr1
      Figure 1Axial view of the abdomen CT showing pancreatic head enhancing lesion (blue arrow) with complete left–right inverted anatomy.
      Figure thumbnail gr2
      Figure 2Coronal view of the CT of the abdomen showing the dilated intrahepatic ducts (orange arrows) with complete left–right inverted anatomy.

      Video description

      The patient was placed in the left lateral position. First, we performed an EGD to help identify the altered anatomy. Afterward, a curvilinear echoendoscope was advanced by performing endoscopy techniques in the usual order but in inverse direction. In particular, the postbulbar examination required a counterclockwise rotation with the left arm hanging straight down to facilitate the visualization of the mesenteric vessels, uncinate part of the pancreas, ampulla, and the ductal anatomy.
      Endoscopic ultrasound showed diffuse circumferential common bile duct wall thickening and a stricture extending from the ampulla to the hepatic bifurcation. The pancreatic duct, head, body, and tail were unremarkable (Fig. 3).
      Figure thumbnail gr3
      Figure 3Endoscopic ultrasound view showing thickened wall common bile duct and normal pancreatic duct. CBD, Common bile duct; PD, pancreatic duct.
      The patient was maintained in the left lateral position and ERCP was subsequently performed, as shown in Video 1 (available online at www.giejournal.org). Given the reversed anatomy, the ampulla of Vater was identified at 2 o’clock compared to patients with regular anatomy, where it is found at 10 to 11 o’clock (Fig. 4). A cholangiogram confirmed a single diffuse stenosis extending from the ampulla to the bifurcation. After biliary sphincterotomy, intraductal brushings were obtained, and a temporary biliary stent was placed (Figs. 5 and 6).
      Figure thumbnail gr4
      Figure 4Ampulla of Vater location in situs inversus totalis when ERCP is performed in the left lateral position.
      Figure thumbnail gr5
      Figure 5Fluoroscopy imaging showing diffuse common bile duct stenosis. CBD, Common bile duct.
      Figure thumbnail gr6
      Figure 6Fluoroscopy imaging confirming common bile duct stent placement. CBD, Common bile duct.

      Outcomes

      There were no immediate adverse events from the procedure, including post-ERCP bleeding or acute pancreatitis. Microscopic examination of bile duct brushings confirmed the diagnosis of adenocarcinoma.

      Discussion

      There are limited reports in the literature of successful ERCP and even fewer of EUS in patients with SIT, as summarized in Tables 1 and 2. The mirror image technique was described, which parallels the inversed anatomy through the performance of all regular endoscopic maneuvers inversely.
      • García-Fernández F.J.
      • Infantes J.M.
      • Torres Y.
      • et al.
      ERCP in complete situs inversus viscerum using a “mirror image” technique.
      Similar to our method, a commonly cited technique relies on inserting the endoscope with a 180-degree clockwise rotation in the stomach or duodenum or both. However, the ease of insertion may come with difficulty cannulating the papilla, which usually sits in the 1 to 3 o’clock position.
      • de la Serna-Higuera C.
      • Perez-Miranda M.
      • Flores-Cruz G.
      • et al.
      Endoscopic retrograde cholangiopancreatography in situs inversus partialis.
      • Kamani L.
      • Kumar R.
      • Mahmood S.
      • et al.
      Therapeutic ERCP in patient with situs inversus totalis and ampullary diverticulum.
      • Lee J.H.
      • Kang D.H.
      • Park J.H.
      • et al.
      Endoscopic removal of a bile-duct stone using sphincterotomy and a large-balloon dilator in a patient with situs inversus totalis.
      • Sheikh I.
      • Heard R.
      • Tombazzi C.
      Technical factors related to endoscopic retrograde cholangiopancreatography in patients with situs inversus.
      • Patel K.S.
      • Patel J.N.
      • Mathur S.
      • et al.
      To twist or not to twist: a case of ERCP in situs inversus totalis.
      • Morales-Rodríguez J.F.
      • Corina Cotillo E.
      • Moreno-Loaiza O.
      Tratamiento quirúrgico de coledocolitiasis en situs inversus totalis: reporte de caso y revisión de la literatura [Surgical treatment of choledocholithiasis in a patient with situs inversus totalis: a case report and literature review] [in Spanish].
      • Nordback I.
      • Airo I.
      ERCP and endoscopic papillotomy in complete abdominal situs inversus.
      • Lee J.M.
      • Lee H.S.
      • Kim C.D.
      Infundibulotomy and endoscopic retrograde cholangiopancreatography in situs inversus totalis combined with choledochocele.
      • Tyberg A.
      • Lee T.
      • Karia K.
      • et al.
      Malignant obstructive jaundice in situs inversus: demonstration of precut and biliary drainage.
      • Hu L.
      • Chai Y.
      • Yang X.
      • et al.
      Duodenoscope combined with laparoscopy in treatment of biliary stones for a patient with situs inversus totalis: a case report.
      • Hu Y.
      • Zeng H.
      • Pan X.
      • et al.
      Therapeutic endoscopic retrograde cholangiopancreatography in a patient with situs inversus viscerum.
      • Ş Çoban
      • Yüksel I.
      • Küçükazman M.
      • et al.
      Successful ERCP in a patient with situs inversus.
      • Téllez-Ávila F.I.
      • Pattel S.
      • Duarte-Medrano G.
      • et al.
      A challenging case of giant biliary stones in a patient with situs inversus totalis: conventional ERCP combined with intraductal cholangioscopy and laser lithotripsy.
      • Yoshida A.
      • Minaga K.
      • Takeda O.
      • et al.
      Successful biliary cannulation using a novel rotatable sphincterotome in a patient with situs inversus totalis.
      • Shimoda F.
      • Satoh A.
      • Asonuma S.
      • et al.
      Successful removal of multiple bile duct stones using a papillary large balloon dilation in a very elderly woman with situs inversus totalis.
      • Tanisaka Y.
      • Ryozawa S.
      • Sudo K.
      • et al.
      Successful endoscopic retrograde cholangiopancreatography using pancreatic guidewire placement for biliary cannulation in a patient with situs inversus and Billroth-I gastrectomy (with video).
      Another technique involved changing the patient position during the procedure, which brought challenges to the endoscopy and was limited by patient intolerance.
      • Venu R.P.
      • Geenen J.E.
      • Hogan W.J.
      • et al.
      ERCP and endoscopic sphincterotomy in patients with situs inversus.
      ,
      • Lipschutz J.H.
      • Canal D.F.
      • Hawes R.H.
      • et al.
      Laparoscopic cholecystectomy and ERCP with sphincterotomy in an elderly patient with situs inversus.
      There are few reports in the literature of successful EUS in patients with SIT. These case reports make note of challenges identifying vascular anatomy and suboptimal views of the pancreas requiring the use of multiple endoscopes or even changing the patient’s position.
      • Kumbhari V.
      • Abi-Hanna D.
      • Bassan M.
      Gastrointestinal: endoscopic ultrasound of a pancreatic lesion in situs inversus.
      • Togliani T.
      • Pilati S.
      • Mantovani N.
      • et al.
      Extrahepatic cholangiocarcinoma in a patient with situs inversus totalis diagnosed by endoscopic ultrasound.
      • Coronel M.
      • Lanke G.
      • Cambell D.
      • et al.
      Performing endoscopic retrograde cholagiopancreatpgraphy and endoscopic ultrasound for management of malignant bile duct obstruction in a patient with a situs inversus totalis.
      Table 1Summary of the reported techniques for ERCP in patients with situs inversus totalis
      TechniquePatient positionEndoscopist position to the table (as seen from the bottom)Key featuresAdvantages and challenges described
      Mirror image
      • García-Fernández F.J.
      • Infantes J.M.
      • Torres Y.
      • et al.
      ERCP in complete situs inversus viscerum using a “mirror image” technique.
      Right lateralLeftPerforming all regular maneuvers inversely.Ease of cannulation of papilla. Requires manipulation with opposite hand. Changed position of room equipment.
      180-degree clockwise turn
      • de la Serna-Higuera C.
      • Perez-Miranda M.
      • Flores-Cruz G.
      • et al.
      Endoscopic retrograde cholangiopancreatography in situs inversus partialis.
      • Kamani L.
      • Kumar R.
      • Mahmood S.
      • et al.
      Therapeutic ERCP in patient with situs inversus totalis and ampullary diverticulum.
      • Lee J.H.
      • Kang D.H.
      • Park J.H.
      • et al.
      Endoscopic removal of a bile-duct stone using sphincterotomy and a large-balloon dilator in a patient with situs inversus totalis.
      • Sheikh I.
      • Heard R.
      • Tombazzi C.
      Technical factors related to endoscopic retrograde cholangiopancreatography in patients with situs inversus.
      • Patel K.S.
      • Patel J.N.
      • Mathur S.
      • et al.
      To twist or not to twist: a case of ERCP in situs inversus totalis.
      • Morales-Rodríguez J.F.
      • Corina Cotillo E.
      • Moreno-Loaiza O.
      Tratamiento quirúrgico de coledocolitiasis en situs inversus totalis: reporte de caso y revisión de la literatura [Surgical treatment of choledocholithiasis in a patient with situs inversus totalis: a case report and literature review] [in Spanish].
      • Nordback I.
      • Airo I.
      ERCP and endoscopic papillotomy in complete abdominal situs inversus.
      • Lee J.M.
      • Lee H.S.
      • Kim C.D.
      Infundibulotomy and endoscopic retrograde cholangiopancreatography in situs inversus totalis combined with choledochocele.
      • Tyberg A.
      • Lee T.
      • Karia K.
      • et al.
      Malignant obstructive jaundice in situs inversus: demonstration of precut and biliary drainage.
      • Hu L.
      • Chai Y.
      • Yang X.
      • et al.
      Duodenoscope combined with laparoscopy in treatment of biliary stones for a patient with situs inversus totalis: a case report.
      • Hu Y.
      • Zeng H.
      • Pan X.
      • et al.
      Therapeutic endoscopic retrograde cholangiopancreatography in a patient with situs inversus viscerum.
      • Ş Çoban
      • Yüksel I.
      • Küçükazman M.
      • et al.
      Successful ERCP in a patient with situs inversus.
      • Téllez-Ávila F.I.
      • Pattel S.
      • Duarte-Medrano G.
      • et al.
      A challenging case of giant biliary stones in a patient with situs inversus totalis: conventional ERCP combined with intraductal cholangioscopy and laser lithotripsy.
      • Yoshida A.
      • Minaga K.
      • Takeda O.
      • et al.
      Successful biliary cannulation using a novel rotatable sphincterotome in a patient with situs inversus totalis.
      • Shimoda F.
      • Satoh A.
      • Asonuma S.
      • et al.
      Successful removal of multiple bile duct stones using a papillary large balloon dilation in a very elderly woman with situs inversus totalis.
      • Tanisaka Y.
      • Ryozawa S.
      • Sudo K.
      • et al.
      Successful endoscopic retrograde cholangiopancreatography using pancreatic guidewire placement for biliary cannulation in a patient with situs inversus and Billroth-I gastrectomy (with video).
      Prone, left lateralRight180-degree clockwise rotation in stomach or duodenum. Alternatively, “pursuing endoscopy in direction inverse to usual.”Difficulty cannulating and performing papillotomy of 1-3 o’clock papilla needing advanced papillotomy techniques.
      Variation of limited clockwise turn
      • Lee J.M.
      • Lee J.M.
      • Hyun J.J.
      • et al.
      Successful access to the ampulla for endoscopic retrograde cholangiopancreatography in patients with situs inversus totalis: a case report.
      ProneRightScope inserted along the lesser curvature of the stomach, with slow clockwise rotation of the endoscope.Ease of cannulation of central-upward ampulla. Difficulty achieving endoscopic access to duodenum.
      360-degree turn
      • Fiocca F.
      • Donatelli G.
      • Ceci V.
      • et al.
      ERCP in total situs viscerum inversus.
      • Feng Q.
      • Yao J.
      Hepatobiliary and pancreatic: common bile duct stones with situs inversus totalis.
      • El II, Hajj
      • Sherman S.
      • Ceppa E.P.
      • et al.
      ERCP and laparoscopic cholecystectomy in a patient with situs inversus totalis.
      ProneRight180-degree rotation in the stomach, then 180-degree rotation in duodenum, both in the same direction.Difficulty controlling endoscope owing to looped shaft. Difficulty cannulating right-upward deviated ampulla.
      Changing patient position
      • Venu R.P.
      • Geenen J.E.
      • Hogan W.J.
      • et al.
      ERCP and endoscopic sphincterotomy in patients with situs inversus.
      ,
      • Lipschutz J.H.
      • Canal D.F.
      • Hawes R.H.
      • et al.
      Laparoscopic cholecystectomy and ERCP with sphincterotomy in an elderly patient with situs inversus.
      VariableRightChanging position from right lateral decubitus to prone upon reaching D2; counterclockwise rotation to identify papilla. Changing position from prone to supine to locate and cannulate papilla.Difficulty cannulating; patient intolerance.
      Table 2Summary of the reported techniques for EUS in patients with situs inversus totalis
      Patient positionKey featuresChallenges described
      Left lateral decubitus then right lateral decubitus
      • Kumbhari V.
      • Abi-Hanna D.
      • Bassan M.
      Gastrointestinal: endoscopic ultrasound of a pancreatic lesion in situs inversus.
      Performing all regular maneuvers inversely. Linear endoscope used to perform fine-needle aspiration in conventional manner. Then switched to radial endoscope to define anatomy.Difficulty identifying vascular anatomy. Suboptimal views of pancreas requiring changing position to right lateral decubitus following the mirror image technique to allow visualization of the entire pancreas.
      Left lateral decubitus
      • Togliani T.
      • Pilati S.
      • Mantovani N.
      • et al.
      Extrahepatic cholangiocarcinoma in a patient with situs inversus totalis diagnosed by endoscopic ultrasound.
      Radial endoscope used. Clockwise rotation of endoscope rather than counterclockwise to explore common bile duct. Linear scanning EUS with fine-needle aspiration.
      Left lateral decubitus
      • Coronel M.
      • Lanke G.
      • Cambell D.
      • et al.
      Performing endoscopic retrograde cholagiopancreatpgraphy and endoscopic ultrasound for management of malignant bile duct obstruction in a patient with a situs inversus totalis.
      Linear endoscope used, with insertion following inversion of usual technique.Requiring comfortable knowledge of anatomy.

      Conclusion

      Situs inversus totalis is a rare congenital disorder that poses significant challenges when performing diagnostic and therapeutic endoscopy. Performing the EGD is not challenging in SIT, but colonoscopy, EUS, and ERCP can be more difficult than usual given the altered anatomy. Starting with an EGD is reasonable to ascertain the “lay of the land” before performing EUS or ERCP. An experienced endosonographer with good knowledge of anatomy can safely and successfully perform linear EUS-guided interventions. In addition, ERCP can be safely and successfully performed with largely “normal” patient and endoscopist positions.

      Disclosure

      All authors disclosed no financial relationships.

      Supplementary data

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