|Year : 2020 | Volume
| Issue : 1 | Page : 68-71
Incidental discovery of situs inversus totalis in a 72-year-old man
Bukunmi Michael Idowu1, Tolulope Adebayo Okedere2, Stephen Olaoluwa Onigbinde2
1 Department of Radiology, Union Diagnostics and Clinical Services, Yaba, Lagos, Nigeria
2 Department of Radiology, Obafemi Awolowo University Teaching Hospitals Complex, Ile-Ife, Osun, Nigeria
|Date of Submission||16-Nov-2018|
|Date of Acceptance||18-May-2019|
|Date of Web Publication||13-Mar-2020|
Dr. Bukunmi Michael Idowu
Department of Radiology, Union Diagnostics and Clinical Services, Yaba, Lagos
Situs inversus totalis (SIT) which is also known as dextrocardia with situs inversus is a congenital anomaly of lateralization or sidedness. Although dextrocardia of itself is detectable on obstetrics sonography, it frequently goes undetected and these individuals are often diagnosed later in life. This is a case of SIT in a 72-year-old man, who has had normal life expectancy by Nigerian standards, detected incidentally on imaging evaluation for unrelated complaints. He presented with features of chest infection and intestinal obstruction. While on admission, he was also diagnosed with systemic hypertension and diabetes mellitus. He had no features of Kartagener's syndrome. SIT could be suspected clinically, but a definitive diagnosis often requires imaging. Medical personnel must always maintain a high index of suspicion for this relatively uncommon condition with a profound impact on administration and/or interpretation of clinical, investigative, and resuscitative procedures as well as management of ailments.
Keywords: Dextrocardia, imaging, lateralization anomaly, situs inversus, situs oppositus, situs transversus
|How to cite this article:|
Idowu BM, Okedere TA, Onigbinde SO. Incidental discovery of situs inversus totalis in a 72-year-old man. West Afr J Radiol 2020;27:68-71
| Introduction|| |
Situs inversus totalis (SIT), also called situs transversus or situs oppositus or dextrocardia with situs inversus, is a rare congenital positional disorder characterized by total transposition of abdominal and thoracic viscera about the sagittal plane, leading to a mirror image of the normal positioning of internal organs. SIT is often diagnosed incidentally during thoracic and/or abdominal imaging for other complaints, surgery, or cadaveric dissection. This case is presented to emphasize the need for health workers to maintain a high index of suspicion in order to avoid management mishaps and to re-emphasize the dramatic effect, the condition can have on symptoms, signs, investigations, and treatment procedures. The condition was not detected clinically in this patient until abdominal ultrasound was done.
| Case Report|| |
A 72-year-old male presented with a 5-day history of fever and difficulty with breathing, a week history of abdominal pain, abdominal distension, and difficulty in defecating. The fever was continuous and high grade in nature. Abdominal pain was colicky in nature, with the occasional passage of hard, nodular stool. He had not been diagnosed with systemic hypertension or diabetes mellitus before. He was married with children.
He was in respiratory distress evidenced by flaring ala nasae. Chest examination revealed crepitations in the left middle and lower lung fields. There was no record of the presence of cardiac apex beat on the right in the documented physical examination findings. Blood pressure was 180/120 mmHg. The abdomen was distended and moved minimally with respiration. There was no palpably enlarged organ. Digital rectal examination revealed hard, impacted stool in the rectum. The prostate gland was not enlarged. An initial assessment of partial intestinal obstruction secondary to fecal impaction was made.
Fasting plasma glucose was high (16 mmol/L) on two separate occasions. Full blood count showed neutrophilia.
The situs anomaly was first detected on abdominal ultrasonography, which showed left-sided liver, gall bladder, inferior vena cava, and head of the pancreas, whereas the spleen was on the right side.
Plain chest X-ray showed dextrocardia with right-sided aortic arch [Figure 1] and pneumonic changes in the lung fields. There were no features of bronchiectasis and chronic sinusitis on paranasal sinuses radiography (occipitomental view).
|Figure 1: Plain chest radiograph (posteroanterior view) showing dextrocardia and right-sided aortic arch (arrows). The gastric air bubble is beneath the right hemidiaphragm (star)|
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The supine abdominal radiograph showed dilated large bowel loops suggestive of large bowel obstruction while the erect view showed the liver shadow on the left and gastric air bubble on the right [Figure 2].
|Figure 2: Erect abdominal radiograph showing the liver shadow on the left (arrow) and gastric air bubble on the right (star)|
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Magnetic resonance imaging (MRI) of the abdomen confirmed the reversed, mirror image anatomy [Figure 3] and [Figure 4].
|Figure 3: Nonenhanced, axial magnetic resonance imaging of the abdomen showing transposition of the abdominal viscera with the stomach (star), spleen (arrowhead), and abdominal aorta (upward arrow) on the right; while the liver (horizontal arrow), gall-bladder (downward arrow), inferior vena cava, and portal vein are on the left side of the midline|
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|Figure 4: Nonenhanced, coronal magnetic resonance imaging of the thorax and abdomen showing transposition of the abdominal viscera with the descending thoracic aorta (arrowhead) and spleen (vertical/upward arrow) on the right, whereas the liver (horizontal arrow) is on the left side of the midline. The inferior poles of both kidneys are marked (stars)|
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No associated cardiac abnormality was detected on transthoracic echocardiography.
A final assessment of partial intestinal obstruction secondary to fecal impaction with background type II diabetes mellitus, uncontrolled systemic hypertension, and bronchopneumonia in an elderly man with SIT was made.
Bronchopneumonia was treated with cefuroxime and ramipril for hypertension. Insulin was used to lower the blood glucose level, after which he was maintained on metformin and glibenclamide. Fecal impaction was relieved with enema saponis. Recovery was uneventful.
| Discussion|| |
Situs describes the position of the heart (cardiac atria) and viscera relative to the midline. Situs solitus is the normal position, whereas SIT is the mirror image of solitus. In SIT, the morphologic right atrium is on the left, whereas the morphologic left atrium is on the right. The left lung has three lobes, whereas the right lung has two lobes as opposed to the normal pulmonary anatomy. The liver and gallbladder are situated on the left, whereas the stomach and spleen are on the right. Blood vessels, nerves, lymphatics, and intestines are also transposed. The organs are simply transposed through the sagittal plane without alteration of their relationships; therefore, functional problems rarely occur in SIT.
SIT occurs in one of 10,000 live births, and is present in 0.01% of any population. There are no racial or gender differences in incidence. SIT is generally an autosomal recessive genetic condition, but it can sometimes be X-linked or occur in identical twins. Rarely, it runs in families.
It may be isolated or associated with congenital malformations of the cardiac and hepatobiliary systems. There is 3%–5% incidence of congenital heart disease, most commonly transposition of the great vessels, in SIT. Moreover, about 20% of patients with SIT can have Kartagener's syndrome, which comprises bronchiectasis, chronic sinusitis, and SIT. None of these associations were present in this patient.
SIT could be suspected, evaluated, or confirmed by radiologic and nonradiologic methods. Radiologic methods include plain chest and abdominal radiography, abdominal sonography, fetal sonography, echocardiography, chest and abdominal computerized tomography, abdominal MRI, barium enema, barium meal, and scintigraphy. The most common cause of false-positive and false-negative diagnosis of situs inversus on radiographic studies is the radiographer's or radiologist's inattention to proper labeling. Nonradiologic methods are electrocardiogram and physical examination. The presence of SIT was not suspected on physical examination in this patient.
Failure to recognize situs inversus before performing a radiologic or surgical procedure may result in intervention on the incorrect side in the patient. Endoscopic retrograde cholangiopancreatography could be particularly problematic. Alarmingly, a right-sided spleen in SIT was reportedly misdiagnosed for the shrunken liver of chronic liver disease.
Furthermore, the transposition of viscera poses diagnostic and management dilemmas when the patient develops visceral pathologies because of differences in clinical presentation, diagnostic elusiveness, and the need for modifications of operative techniques., Accurate imaging of the viscera becomes imperative not only for making a correct diagnosis but also for choosing appropriate therapeutic interventions, surgical planning, and the type and location of the surgical incision. Operating time is often doubled because of the altered orientation and ergonomic challenges encountered perioperatively.
The reversal of the organs may also lead to some confusion as many signs and symptoms will be on the reverse side. Diseases such as angina pectoris, myocardial infarction, acute cholecystitis, acute appendicitis, and diverticulitis present atypically, with the pain referred to the “wrong” side. This could lead to potentially fatal delay in intervention.
Most individuals with SIT are asymptomatic and have a normal life expectancy, as seen in this case. Many of them are unaware of their unusual anatomy until they seek medical attention for an unrelated condition as was in the index patient. [Table 1] juxtaposes this case of SIT with some of the previously reported cases in Nigerians. The index patient appears to be the oldest known/reported Nigerian with SIT.
|Table 1: Highlights of some situs inversus totalis cases reported from various parts of Nigeria|
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| Conclusion|| |
Imaging plays a crucial role in confirming the diagnosis and excluding associated congenital anomalies. Such individuals should be educated on the need to promptly provide this information to their doctors in future consultations in order to minimize the risk of misdiagnosis and delayed treatment. Healthcare providers must maintain a high index of suspicion. Consent to publish this case was obtained from the patient.
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.
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[Figure 1], [Figure 2], [Figure 3], [Figure 4]