Situs Inversus Research Articles

Robotic-assisted pulmonary lobectomy with lung cancer in a patient with situs inversus totalis

BackgroundSitus inversus totalis (SIT) is a relatively rare congenital abnormality in which the major thoracic and abdominal visceral organs are reversed from their usual positions. In patients with SIT and bronchial carcinoma, surgical difficulty increases sharply. It has been reported that the video-assisted thoracic surgery (VATS) still poses the operator to a challenge situation. The similarity of surgical positions and the flexibility of the mechanical arm in robotic surgery, may be beneficial to SIT patients due to reducing technical difficulties. Here, we present a first case of SIT patient with lung cancer, in which Da Vinci robot-assisted thoracic surgery (RATS) was performed successfully.Case presentationA 66-year old patient, previously diagnosed with SIT since childhood, came to our hospital with two pulmonary nodules in his left lung field. The bigger one had increased somewhat for the last 2 years of follow-up. Software Mimics was preoperatively carried out to analyze anatomical variations. RATS was conducted to complete left upper lobectomy and left middle wedge resection. The patient had no intraoperative complications and was discharged day 5 after the operation.ConclusionsThis is the first report of a successful robot-assisted lung cancer resection in a patient with SIT. In such challenging cases as lung cancer and rare anomaly as SIT, RATS is more advantageous and suitable than VATS with the help of software Mimics utilized for 3D reconstruction, which can identify the anatomical abnormalities and facilitate the surgical procedures.

312.7: Liver Transplantation in a Complex Recipient Anatomy.

A 22-year-old man with situs inversus totalis and end-stage liver disease secondary to congenital biliary atresia and previous Kasai portoenterostomy at two months who was referred to our center with portal hypertensive bleeding, recurrent cholangitis, and hepatocellular carcinoma. He completed the liver transplant protocol. Preoperative liver angiotomography revealed, besides the mirror image orientation of the viscera and dextrocardia, inferior vena cava (IVC) and portal vein (PV) with good patency, splenorenal shunt, collateral circulation, and common hepatic artery arising from the superior mesenteric artery. A suitable 59-year-old cadaveric male donor with no anatomic variation was accepted. He underwent to liver transplant, his BMI was 16.7kg/m2, MELD and Child-Pugh scores were 24 and 7 points. The donor’s liver weighed 1725g.A bilateral subcostal incision was made; after extensive lysis of adhesions, the hepatectomy was performed with preservation of the recipient’ s IVC. The donor’s liver was placed on the left side rotated 90° clockwis; this allowed perfect alignment of the recipient and dolor hila. The donor infrahepatic vena cava was anastomosed end-to-side to recipient IVC previous closing of the suprahepatic vena cava. The artery and PV were anastomosed end-to-end without vessel grafts. A choledocojejunostomy was performed using the previous Roux-en-Y. The patient had satisfactory evolution and was discharged on the 8th postoperative day with normal liver function.

Interventional treatment of basilar trunk artery aneurysms associated with situs inversus totalis: A case report

Basilar trunk artery aneurysm (BTAA) has an overall low incidence in intracranial aneurysm, but its rupture is associated with high morbidity and mortality in older people. Situs inversus totalis (SIT) is a rare congenital abnormality characterized by visceral rotation and vascular abnormalities. It has been described in several uncommonly clinical cases, along with middle cerebral artery aneurysms and large carotid cavernous aneurysms. However, the association between interventional embolization for BTAA and SIT has not been reported. We described the angiography findings and interventional treatment of the association of BTAA with SIT.

Redo aortic valve surgery in a case of dextrocardia with situs inversus totalis

IntroductionDextrocardia is a rare congenital cardiac anomaly where the base-apex axis of the heart is directed to the right side. It may be associated with situs solitus, situs inversus, or situs ambiguous. Such cases are technically challenging when heart surgery is needed.Presentation of caseWe presented a case of a patient with dextrocardia and situs inversus totalis, who had severe aortic bioprosthetic valve degeneration needing a redo aortic valve replacement. Our operative strategy was to change the position of the main surgeon to the left side of the patient to perform most surgical steps.DiscussionA meticulous pre-operative surgical plan involving the whole team was very important for a smooth intra-operative course and a favourable outcome. Regarding the position of the main surgeon standing on the left side of the patient, we believed this surgical team arrangement added much to the technical ease for the procedure, since the anatomy was opposite to what our minds are used to. Also, computed tomography scan played a crucial role for a proper preoperative anatomical evaluation and surgical planning.ConclusionOur approach with the surgeon on the left side provided excellent exposure for redo aortic valve replacement in dextrocardia with situs inversus totalis. This surgical management played an important role for the good result in this technically challenging scenario.

WE2.2 Video Presentation of performing a Laparoscopic Cholecystectomy on a patient diagnosed with Situs Inversus Totalis

Abstract Background Situs inversus totalis (SIT) is a condition in which the arrangement of the internal organs is a mirror image of normal anatomy, occurring in 0.0001% of patients. A thorough understanding of the human anatomy and anatomical variances is crucial in everyday clinical practise and emergency surgical care. We present a video on a patient with SIT who underwent a Laparoscopic Cholecystectomy (LC). Case Our patient is a 40-year-old lady with a known diagnosis of SIT. She presented with jaundice and left upper quadrant pain (LUQ). An ultrasound and CT scan confirmed the diagnosis of gall stones, CBD stones and SIT. She underwent an ERCP to clear CBD stones. LC is one of the commonest general surgical procedures, however, this case proved challenging due to reversed anatomy. Intraoperatively, operating positioning was reversed with mirror imaging of ports used normally. Standard 10mm ports were placed in the infraumbilical and epigastric regions and the remaining ports placed in the LUQ. Abdominal organs were inspected and SIT was confirmed. It was ensured that a slow, meticulous delineation of Calot's triangle and window of safety was performed. The cystic duct and artery were clipped. The rest of the procedure was uneventful. Results The patient was discharged on the same day as the procedure. There were no immediate or short-term complications. Gallbladder histology showed chronic cholecystitis, cholelithiasis and cholesterolosis. Conclusion This case demonstrates that with appropriate planning and an advanced skill set, a LC can be performed in a patient with SIT, with little or no complications.

DILATATION DES BRONCHES REVELANT UN SYNDROME DE KARTAGENER : A PROPOS DUN CAS ET REVUE DE LA LITTERATURE

Primary ciliary dyskinesias (PCD) are rare genetic diseases with autosomal recessive transmission, affecting nearly 1/15000 births, due to a structural/functional defect of cilia. Kartagener syndrome (KS) represents 50% of PCDs and is characterised by the triad of bronchiectasis, chronic sinusitis and situs inversus. It is manifested by clinical signs of upper and lower respiratory infections or infertility. Situs inversus is often discovered incidentally. The diagnosis is confirmed by specialized centers and is based on analysis of ciliary ultrastructure by electron microscopy or ciliary beats by light microscopy/high speed video microscopy , measurement of nasal nitric oxide flow and genetic tests. Treatment is supportive and in line with recommendations for the management of cystic fibrosis or idiopathic adult bronchiectasies. It includes respiratory physiotherapy, antibiotic medication to treat infections, vaccinations and local treatment of the otorhinolaryngeal tract if necessary. We present here the case of a 23-year-old woman with a history of recurrent respiratory infections since her early age, who consulted for cough, chronic bronchorrhea and dyspnea. The evolution to respiratory failure is slowed by the early diagnosis and management of exacerbations.

Rare spontaneous monochorionic dizygotic twins: a case report and a systematic review

BackgroundMonochorionic dizygotic twins are a rare condition, mostly related to assisted reproductive technology. This type of twinning is burdened by the same risk of pregnancy complications found in monochorionic monozygotic pregnancies.Case presentationWe report a case of spontaneous monochorionic dizygotic twins sharing situs inversus abdominalis and isolated levocardia, with only one twin affected by biliary atresia with splenic malformation syndrome. We also conducted a literature review of the 14 available documented monochorionic dizygotic twin gestations spontaneously conceived.ConclusionsIt is still unclear how this unusual type of twinning can occur in spontaneous conception. The evidence so far suggest the importance to timely diagnose the chorionicity, in order to adequately manage the typical complications associated with monochorionicity.

Dextrocardia, situs solitus, double–inlet, and double-outlet left ventricle – A case report

Dextrocardia is embryologic malformation characterized by displacement of the largest axis of the heart to the right side of the chest. Dextrocardia is a rare anomaly that is associated with situs inversus and the incidence in the general population is usually 1:10,000, withsitus solitus being 1:30,000 live births. It is associated with complex cardiac lesions, 5% with situs inversus, and 90% with situs solitus color. Doppler echocardiography not only provides additional information but also improves overall specificity of two-dimensional echocardiography. Few case reports of dextrocardia, double-inlet (DILV), and double-outlet left ventricle (DOLV) with situs inversus have been diagnosed and reported by echocardiography, but none are available with situs solitus. To the best of our knowledge, we are presenting the first case report of dextrocardia, situs solitus, double-inlet with DILV, DOLV, and D-malposition of great arteries with pulmonary stenosis, detected by color Doppler echocardiography.

Sleeve gastrectomy in a patient with situs inversus: a case report

Situs inversus (SI) is a very rare congenital disease affecting one in 10 000 people. It is characterized by a mirror image transposition of both abdominal and thoracic organs. Diagnosis of SI is usually made incidentally while investigating for unrelated medical problem. It can be associated with cardiac and respiratory anomalies that may cause perioperative morbidity if not diagnosed before surgery. There are limited case reports in literature of SI patients that underwent bariatric procedures. We report a case of a 32-year-old female with SI partialis and levocardia who successfully underwent laparoscopic sleeve gastrectomy. We concluded that sleeve gastrectomy is safe in patients with SI if diagnosed preoperatively and if all the necessary precautions are taken into consideration before and during the surgery.

Left sided Amyand hernia - A case report.

IntroductionAmyand hernia is a clinical condition wherein content of the inguinal hernial sac is formed by the vermiform appendix.Case presentation1 year 3-month-old male child presented to our OPD with an irreducible left inguinal hernia for which he was taken up for an emergency herniotomy. The terminal ileum, caecum and appendix were found to be the contents of the hernial sac.DiscussionAs the appendix is anatomically located on the right, Amyand hernia more commonly occurs on the right, however its occurrence on the left, is a rare event and is usually associated with congenital anomalies like Intestinal malrotation, Situs inversus and mobile caecum.ConclusionAmyand hernia presenting on the left is extremely rare and high index of clinical suspicion is required to manage such patients. We report one such rare case of a Left sided Amyand hernia in a young child.

Kartagener Syndrome: A Case Report

Kartagener syndrome (KS) is a rare autosomal recessive disorder characterized by a clinical triad of situs inversus, chronic sinusitis, and bronchiectasis. Impaired ciliary motility due to abnormal ciliary structure or function is the main pathophysiological problem in KS. A 38-year-old woman presented to our outpatient clinic with a productive cough, fever, and shortness of breath for 1 month. She has had recurrent episodes of respiratory tract infections since childhood. Clinical investigations revealed situs inversus, sinusitis, and bronchiectasis. She was diagnosed with KS and treated with antibiotics, mucolytics, bronchodilators, and chest physiotherapy. Patients with KS present with chronic recurrent respiratory tract infections due to ineffective mucociliary clearance. Early diagnosis is important to improve prognosis. The main goals in the management of KS are to prevent the progression of the disease, preserve pulmonary function, and improve quality of life.

An Unusual Case of Perforated Appendicitis in Situs Inversus Totalis in Indonesia

Situs inversus is a rare congenital anomaly where the internal organs are transposed in a mirror image. It is commonly unrecognized until a patient comes with a complaint, especially in developing countries where people do not have a routine medical checkup. We report a case of a young female with unusual left lower quadrant pain abdominal pain. Perforated appendicitis leading to peritonitis was confirmed using ultrasonography and successfully treated with laparotomy. Appendicitis should be considered a working diagnosis in lower left quadrant abdominal pain

A Young Bangladeshi Boy with Primary Ciliary Dyskinesia (Kartagener’s Syndrome): A Rare Case

Kartagener’s syndrome, an autosomal recessive inherited disorder, is a subgroup of primary ciliary dyskinesias (PCD). This genetic disorder manifests from early life which distinguishes it from acquired mucociliary disorders. Kartagener’s syndrome presents as a classical triad of situs inversus, sinusitis and bronchiectasis occurring majorly due to impaired ciliary motility. A 16 year-old boy from Fulbaria, Mymensingh with left sided consolidation for 1 month and recurrent episodes of nasal congestion and lower respiratory tract infection (LRTI) since his childhood. Clinical and imaging findings revealed left sided consolidation, chronic sinusitis, bronchiectasis, dextrocardia, and situs inversus. He was treated with orally administered antibiotics, bronchodilators, mucolytics, and chest physiotherapy. He was symptomatically better with the above therapy, and started on a long-term low-dose prophylactic antibiotic. As there is no easy, reliable non-invasive diagnostic test for Kartagener’s syndrome and the correct diagnosis is often delayed by years, it may cause chronic respiratory problems with reduced quality of life. Genetic counseling and fertility issues should be addressed once Kartagener’s syndrome is diagnosed.
 CBMJ 2022 January: vol. 11 no. 01 P: 56-60

A novel nonsense PKD1L1 variant cause heterotaxy syndrome with congenital asplenia in a Han Chinese patient.

Heterotaxy syndrome is a very rare congenital disease, which is caused by the disorder of left-right asymmetry during visceral development. However, pathogenic genetic lesions are found in less than 20% of HS patients. In this cohort study, whole-exome sequencing was performed for 110 patients with situs inversus or situs ambiguous. We identified a novel nonsense variant in PKD1L1(c.1387 C > T; p.463Gln*) in a Chinese patient with heterotaxy syndrome and congenital asplenia. This homozygous variant caused the domain of PKD1L1 complete absence. To our knowledge, this novel variant is the first phenotype of congenital asplenia found in patients with PKD1L1 variants, and the first PKD1L1 variant found in China. Our findings expand the spectrum of PKD1L1 variants and provide support for PKD1L1 variant and congenital asplenia, and the critical role of PKD1L1 during left-right patterning in the Han Chinese population.

Perfusion management in the excision of a right atrial mass in a beating heart with robotic surgery in a pregnant patient with situs inversus totalis

Reoperations in cardiac surgery are very difficult and risky due to possible complications. A 27-year-old female patient at 35 weeks of pregnancy applied to the cardiology department with a complaint of palpitation. In the control transthoracic echocardiography, a 24¥25 mm mass was detected in the right atrium. The patient had situs inversus totalis and had undergone a robotic atrial septal defect closure operation one year ago. After the cesarean operation, robotic cardiac surgery was performed on the beating heart, and the mass in the right atrium was successfully removed. During the procedure, the perfusion strategy was differently conducted than the normal patient management, and the perfusion device was located differently. Cardiac surgery with minimally invasive robotic surgery in a pregnant female with dextrocardia is a special condition that requires many changes in perfusion methods and protocols, and their coexistence create a difficult process for perfusionists.

Torsion of wandering spleen after Fontan operation in a patient with situs inversus: a rare complication.

Torsion of wandering spleen after the Fontan operation with situs inversus is rare.Here, we report the case of a 6-year-old girl with a single ventricle and complete situs inversus who developed torsion of wandering spleen due to splenomegaly caused by post-operative haemodynamics of the Fontan operation. The platelet count was suggested to be useful in predicting splenic torsion.

Duodenal obstruction in a neonate with abdominal situs inversus and isolated levocardia

Duodenal obstruction in a neonate with abdominal situs inversus and isolated levocardia

Thoracoscopic AF ablation in situs inversus dextrocardia with interrupted inferior vena cava continuation in azygos vein

IntroductionSitus inversus totalis, dextrocardia with interrupted inferior vena cava, and azygos vein continuation concomitant with symptomatic atrial fibrillation requiring ablation. This case was deemed not suitable for percutaneous ablation due to anatomic variations and the lack of case reports in the literature.Methods and ResultsWe performed bilateral thoracoscopic epicardial ablation and epicardial left atrial appendage exclusion. The direct vision allowed for a complete box lesion set with bipolar radiofrequency device. Patient remained in sinus rhythm at the 12‐months follow‐up.ConclusionSurgical thoracoscopic epicardial ablation is safe and effective also in congenital defects. Multidisciplinary expertise can offer minimally invasive ablation treatments.

Look beyond the Mirror: Laparoscopic Cholecystectomy in Situs Inversus Totalis-A Systematic Review and Meta-Analysis (and Report of New Technique).

Background: Laparoscopic cholecystectomy in situs inversus totalis (SIT) is a technically and physically demanding procedure for surgeons and there is still a lack of consensus regarding the best technical approach in such cases. We conducted a systematic review and meta-analysis to evaluate port placement, the dominant hand of the surgeon, preoperative imaging, morbidity, and mortality. Methods: We searched MEDLINE, SCOPUS, Web of Science, and the Cochrane Library for studies of patients with SIT that underwent laparoscopic cholecystectomy. Of 387 identified records, 101 met our inclusion criteria, all of them case reports or case series of maximum of 6 patients. Results: Out of the 121 patients included in the analysis, 94 were operated on using a “mirrored American” technique, 12 using the “Mirrored French”, 9 employed single-port techniques, and 6 described novel port placements. Even though most surgeries were conducted by a right-handed surgeon (93 cases), surgeries performed by the seven left-handed surgeons yielded shorter intervention times (p = 0.024). Preoperative imaging (CT, MRI, MRCP, ERCP) also correlated with a lower duration of surgery (p = 0.038. Length of stay was associated with the type of disease, but not with other studied endpoints. Morbidity was less than 1%, and conversion rates and mortality were nil. Conclusions: Cholecystectomy in SIT is a safe but challenging procedure and surgeons should prepare in advance for the unfamiliar aspects of completing such a task. While preoperative imaging and a left-handed surgeon are beneficial in terms of surgery length, when these are not available surgeons should focus on achieving the most comfortable setting based on their experience and tailor their approach to the patient at hand. Further studies are needed in order to properly describe and evaluate intraoperative findings as well as surgeon-dependent factors that could improve future recommendations.

Pancreaticoduodenectomy for pancreatic adenocarcinoma in a patient with situs inversus abdomenalis, a case report.

Introduction and importanceSitus Inversus (SI) is a rare congenital condition in which the abdominal and thoracic organs are located in a mirror image of the normal position in the sagittal plane. Although this condition does not affect normal health or longevity, its recognition is very important for treating many diseases, particularly those requiring surgical intervention. The relationship between situs inversus and cancer remain inconspicuous.Case presentationWe report a 64-year old male with Situs Iinversus Abdominalis with Pancreatic Adenocarcinoma. Radiographic modalities were very important in preoperative assessment of the patient. The patient was managed by pyloric preserving pancreaticoduodenectomy. The patient received adjuvant chemotherapy and free of recurrence for one year after operation.ConclusionSurgeons must recognize the complexity of operative intervention with respect to aberrant anatomy. The occurrence of Situs Inversus in a patient with pancreatic cancer must not deter the surgeon from sound oncologic principles of pancreatic surgery. Referral to these cases to tertiary level center is of utmost importance.