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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.
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 1Axial view of the abdomen CT showing pancreatic head enhancing lesion (blue arrow) with complete left–right inverted anatomy.
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 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 4Ampulla of Vater location in situs inversus totalis when ERCP is performed in the left lateral position.
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.
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.
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].
A challenging case of giant biliary stones in a patient with situs inversus totalis: conventional ERCP combined with intraductal cholangioscopy and laser lithotripsy.
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.
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.
Performing endoscopic retrograde cholagiopancreatpgraphy and endoscopic ultrasound for management of malignant bile duct obstruction in a patient with a situs inversus totalis.
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].
A challenging case of giant biliary stones in a patient with situs inversus totalis: conventional ERCP combined with intraductal cholangioscopy and laser lithotripsy.
Successful endoscopic retrograde cholangiopancreatography using pancreatic guidewire placement for biliary cannulation in a patient with situs inversus and Billroth-I gastrectomy (with video).
Changing 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.
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.
Radial endoscope used. Clockwise rotation of endoscope rather than counterclockwise to explore common bile duct. Linear scanning EUS with fine-needle aspiration.
Performing endoscopic retrograde cholagiopancreatpgraphy and endoscopic ultrasound for management of malignant bile duct obstruction in a patient with a situs inversus totalis.
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.
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].
A challenging case of giant biliary stones in a patient with situs inversus totalis: conventional ERCP combined with intraductal cholangioscopy and laser lithotripsy.
Successful endoscopic retrograde cholangiopancreatography using pancreatic guidewire placement for biliary cannulation in a patient with situs inversus and Billroth-I gastrectomy (with video).
Performing endoscopic retrograde cholagiopancreatpgraphy and endoscopic ultrasound for management of malignant bile duct obstruction in a patient with a situs inversus totalis.
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