|Year : 2019 | Volume
| Issue : 4 | Page : 262-264
Situs inversus totalis with symptomatic cholelithiasis: A diagnostic dilemma
Vishal Bodh1, Rajesh Sharma1, RS Jhobta2, Brij Sharma1, Neeti Aggarwal3
1 Department of Gastroenterology, IGMC, Shimla, Himachal Pradesh, India
2 Department of Surgery, IGMC, Shimla, Himachal Pradesh, India
3 Department of Radiology, IGMC, Shimla, Himachal Pradesh, India
|Date of Submission||25-Jun-2018|
|Date of Decision||12-Jul-2018|
|Date of Acceptance||15-Aug-2018|
|Date of Web Publication||21-Nov-2019|
Department of Gastroenterology, IGMC, Shimla, Himachal Pradesh
Source of Support: None, Conflict of Interest: None
Situs inversus is a rare autosomal recessive condition, with incidence that varies from 1 in 5000 to 1 in 20,000 live births. Because of the atypical “mirror-image” anatomy associated with situs inversus, diagnosis of gallstone disease in these patients is difficult, especially in those with an unknown history of this condition. We report a case of a young female suspected to have situs inversus on esophagogastroduodenoscopy. Subsequently, she was confirmed to have situs inversus totalis with symptomatic cholelithiasis. She was managed successfully with single-incision laparoscopic cholecystectomy by a right-handed surgeon.
Keywords: Chest X–ray, electrocardiogram, esophagogastroduodenoscopy, situs inversus totalis, ultrasonogram
|How to cite this article:|
Bodh V, Sharma R, Jhobta R S, Sharma B, Aggarwal N. Situs inversus totalis with symptomatic cholelithiasis: A diagnostic dilemma. CHRISMED J Health Res 2019;6:262-4
|How to cite this URL:|
Bodh V, Sharma R, Jhobta R S, Sharma B, Aggarwal N. Situs inversus totalis with symptomatic cholelithiasis: A diagnostic dilemma. CHRISMED J Health Res [serial online] 2019 [cited 2019 Dec 14];6:262-4. Available from: http://www.cjhr.org/text.asp?2019/6/4/262/271338
| Introduction|| |
Situs inversus is a rare autosomal recessive condition characterized by mirror-image transposition of viscera. The incidence of situs inversus varies from 1 in 5000 to 1 in 20,000 live births. Situs inversus is divided into two types as follows: situs inversus partialis, which involves the thoracic organs (dextrocardia) or abdominal viscera, and the situs inversus totalis, which involves both the thoracic organs and abdominal viscera. Associated abnormalities may be found, including bronchiectasis, sinusitis, and deficient tracheobronchial cilia. This condition is referred to as Kartagener's syndrome.
The rarity of this disease and its varied presentation makes diagnosis and management difficult. We report a case of a young female suspected to have situs inversus on diagnostic esophagogastroduodenoscopy (EGD). Subsequently, she was confirmed to have situs inversus totalis (SIT) with symptomatic cholelithiasis. She was managed successfully with single-incision laparoscopic cholecystectomy by a right-handed surgeon.
| Case Report|| |
A 22-year-old female was referred to our gastroenterology outpatient department from an outside medical facility for diagnostic EGD. She complained of the left hypochondriac and epigastric pain associated with nausea and occasional vomiting. Her general and per abdominal examination were normal except mild tenderness in epigastrium. Her laboratory investigation revealed hemoglobin of 13.6 g/dl with normocytic normochromic erythrocytes and normal leukocyte and platelet counts. Her serum liver and renal biochemical parameters were within normal limits. Keeping a possibility of dyspepsia due to acid peptic disease, she was managed with oral proton pump inhibitor in a once daily dose before breakfast for 1 month. Her symptoms persisted despite oral proton pump inhibitors, and hence, she was referred for diagnostic EGD. Her EGD revealed a mirror image of the antrum and body of the stomach with the left-sided opening of the duodenal bulb [Figure 1]a and [Figure 1]b, and on the scope, retroversion fundus was located at the right side of the scope. However, the gastric and duodenal mucosa was normal, and no ulceration was detected.
|Figure 1: EGD image of stomach and duodenum (a) Arrow showing mirror image of the antrum and body of the stomach. (b) Long arrow showing a left-sided opening of duodenal bulb with small arrow showing folds of the second part of the duodenum|
Click here to view
Keeping the possibility of situs inversus, her ultrasonogram abdomen was done, which revealed the presence of the liver and gallbladder on the left side and spleen on the right side of the midline. The gallbladder showed a calculus of size 13 mm in the neck region and normal bile duct and pancreas. The possibility of situs inversus with cholelithiasis was kept. Her chest X-ray was done which showed dextrocardia without any evidence of bronchiectasis [Figure 2]a. Her electrocardiogram showed right axis deviation, predominantly negativePwaves, QRS complex, and T-waves in lead I and aVL, predominantly positive QRS complex in lead aVR, low voltage with dominantly negative QRS waves and flattened T waves in leads V4–V6 [Figure 2]b. Her electrocardiography findings were suggestive of dextrocardia. The final diagnosis of SIT with symptomatic cholelithiasis was made, and laparoscopic cholecystectomy was planned.
|Figure 2: (a) Chest X-ray posteroanterior view– showing dextrocardia. (b) Electrocardiography – showing right axis deviation, predominantly negative P-wave, QRS complex and T-wave in the lead I and aVL, predominantly positive QRS complex in lead aVR, low voltage with dominantly negative QRS wave and flattened T-wave in leads V4-V6|
Click here to view
Single-incision laparoscopic cholecystectomy was performed by a right-handed surgeon. The operation was successfully completed within 45 min, and the patient showed no obvious bleeding. During 24-h postoperative observation patient's condition was stable. The patient was discharged on the next day and is doing well on follow-up.
| Discussion|| |
Situs inversus viscerum is a term used to describe a condition in which organs or organ systems are transposed from their normal sites to locations on opposite side of the body. More than one genetic mutation that causes ciliopathy and cystic renal diseases have been implicated in its etiopathogenesis. Although situs inversus on its own is not pathological, it may be associated with cardiorespiratory, hepatopancreaticobiliary, gastrointestinal, neurological, orthopedic, and urological abnormalities, some of which may be life-threatening.
Because of the atypical “mirror-image” anatomy associated with situs inversus, the gallbladder is on the left, whereas the stomach and spleen are on the right. Diagnosis of gallstone disease in patients with situs inversus is difficult, especially in those with an unknown history of this condition. Because of the eccentric anatomy of the left-sided gallbladder, the clinical presentation of these patients usually involves left upper-quadrant pain; however, 30% of patients reportedly develop epigastric pain. Our patient had both left hypochondriac and epigastric pain. Approximately 10% of patients complain of right upper quadrant pain, which is a classic presentation in patients without situs inversus.
Laparoscopic cholecystectomy remains the standard operation for treatment of gallstone disease, even in patients with situs inversus. The mirror–image anatomy and the technical difficulties are a surgeon's main challenges while performing operations in patients with SIT, especially in the right-handed surgeon. In 1991, Campos and Sipes reported the first successful laparoscopic cholecystectomy in a patient with situs inversus with a symptomatic gallstone. In 2008, Fernandes et al. described a three-port technique employed by a left-handed surgeon. They placed a 12-mm subumbilical camera port, a 10-mm epigastric port, and a 5-mm left subcostal port to perform a successful laparoscopic cholecystectomy.
There are also reports involving right-handed surgeons. Lochman et al. and Arya et al. operated with an assistant surgeon grasping the infundibulum. The principal surgeon dissected with only his dominant right hand via the epigastric port. Other reports have described the surgeon's position on both the right side of the patient and the middle of the abducted legs of the patient (lithotomy position) to obtain a more ergonomic position.,
Different techniques and different access ports have been used in several different successful operations.,,, Single-incision laparoscopic cholecystectomy confers some advantages for dissection with the right hand.
| Conclusion|| |
Eccentric anatomy of the left-sided gallbladder in situ s inversus totalis, may lead to the presentation of biliary colic as left upper quadrant pain rather than right-sided pain, leading to difficulty in diagnosis of gallstone disease, especially in those with an unknown history of this condition. Single-incision laparoscopic cholecystectomy in lithotomy position by a right-handed surgeon with conventional instruments is a minimally invasive and successful surgery in such patients.
Declaration of patient consent
The authors certify that they have obtained all appropriate patient consent forms. In the form the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.
Financial support and sponsorship
Conflicts of interest
There are no conflicts of interest.
| References|| |
Budhiraja S, Singh G, Miglani HP, Mitra SK. Neonatal intestinal obstruction with isolated levocardia. J Pediatr Surg 2000;35:1115-6.
Arya SV, Das A, Singh S, Kalwaniya DS, Sharma A, Thukral BB, et al.
Technical difficulties and its remedies in laparoscopic cholecystectomy in situ
s inversus totalis: A rare case report. Int J Surg Case Rep 2013;4:727-30.
Hildebrandt F, Zhou W. Nephronophthisis-associated ciliopathies. J Am Soc Nephrol 2007;18:1855-71.
Phothong N, Akaraviputh T, Chinswangwatanakul V, Trakarnsanga A. Simplified technique of laparoscopic cholecystectomy in a patient with situs inversus: A case report and review of techniques. BMC Surg 2015;15:23.
Lochman P, Hoffmann P, Kočí J. Elective laparoscopic cholecystectomy in a 75-year-old woman with situs viscerum inversus totalis. Wideochir Inne Tech Maloinwazyjne 2012;7:216-9.
Campos L, Sipes E. Laparoscopic cholecystectomy in a 39-year-old female with situs inversus. J Laparoendosc Surg 1991;1:123-5.
Fernandes MN, Neiva IN, de Assis Camacho F, Meguins LC, Fernandes MN, Meguins EM, et al.
Three-port laparoscopic cholecystectomy in a Brazilian amazon woman with situs inversus totalis: Surgical approach. Case Rep Gastroenterol 2008;2:170-4.
Ali MS, Attash SM. Laproscopic cholecystectomy in a patient with situs inversus totalis: Case report with review of literature. BMJ Case Rep 2013;doi:10.1136/bcr-2013-201231.
[Figure 1], [Figure 2]