Abstract

Abstract BACKGROUND AND AIMS Dextrocardia is an abnormal congenital positioning of the heart: instead of the heart forming in the foetus on the left side, it flips over and forms on the right side. Situs inversus is a congenital condition in which the main visceral organs are reversed or mirrored from their usual positions. If both conditions are present, it is called situs inversus totalis. The prevalence of situs inversus is about 0.01%. It is generally an autosomal recessive genetic condition, although it can be X-linked. 25% of people with this condition have an underlying primary ciliary dyskinesia. We are reporting a case of a 55-year-old woman who was found to have a situs inversus totalis with an absent left kidney. No case report has been published yet. METHOD 54-year-old G5P4(2213) woman, government worker presented to an outpatient clinic with complaints of dysuria, frequency, hesitancy, suprapubic burning sensation and mild chest pain in January 2016. The patient did not have any cardiopulmonary symptoms like chronic coughs, nasal congestion and palpitations. Physical examination of the cardiovascular system revealed a normal pulse rate of 70 beats/min and her blood pressure was 100/60 mmHg. Her lungs were clear on auscultation. There was no pedal oedema or any signs of fluid overload. Routine urine analysis revealed protein 1+, with no sugar, no white cell count and negative nitrites. Renal function test showed mildly elevated creatinine of 138.0 umol/L with estimated glomerular filtration rate (eGFR) of 49mL/min using Cockcroft-Gault equation, sodium of 141.0 mEq/L, potassium (K) 5.2 mEq/L, blood urea 9.7mmol/L. Routine chest X-ray revealed heart apex in the right side and gastric air bubble seen below right hemidiaphragm. Abdominal and pelvic ultrasound revealed the liver presented in the left hypochondrium with normal texture. The spleen was noted in the right hypochondrium with normal texture. The right kidney was normal ∼ 9.7cm in size, the outline was smooth and regular with maintained corticomedullary differentiation, with no hydronephrosis. The left kidney was not visualized. The urinary bladder was normal in outline, distensibility and thickness. The uterus was anteverted with a regular outline and normal endometrial echo complex. Computed tomography scan with contrast of abdomen was done which revealed the liver was present in the left hypochondrium and the spleen was present in the right hypochondrium, inferior vena cava on left side and aorta on the right side. The left kidney and ureter were not visualized. Normal right pelvicalyceal system and excretion from the right kidney were noted. RESULTS The above-mentioned patient did not have any cyanosis, cough, sinusitis, or recurrent chest or urinary tract infections. To diagnose situs inversus, thorough physical examination, chest X-ray, ultrasonography, computed tomography, or magnetic resonance imaging are necessary. These imaging modalities help to evaluate the structures of the internal organs, such as the location of cardiac apex, the site of aorta relative to midline, the location of the stomach, the liver and gallbladder, presence and appearance of the spleens and presence of the kidneys and vascular structures. CONCLUSION Dextrocardia with situs inversus is a rare congenital malformation that must be fully evaluated when there is a suspicion because in some cases it can have deadly results. Apart from detailed medical history and physical examinations, imaging modalities should be used for reaching the diagnosis. Routine and regular follow-ups must be arranged for the patients who have this condition. Patients with unilateral renal agenesis must have regular kidney function tests as well as other modalities when necessary to reduce the chance of deterioration in kidney function and kidney damage.

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