SIT, also termed mirror man, is a rare genetic condition in which abdominal, thoracic organs, and blood vessels are reversed 180°. The incidence rate is thought to be in the range of 1 in 8000 to 1 in 25,000 [1, 5]. SIT is frequently associated with kartagener syndrome, which describes a constellation of cardiovascular [6] and hepatobiliary abnormalities [7]. Renal anomalies, including agenesis [8], dysplasia [9], and hypoplasia [10] are the most prominent reported association with SITs. Genes might support this anomaly [11], as similar cases of SIT were reported in our patient’s family (his brother and daughter). The mechanism of this condition is not fully understood. Based on advanced molecular biology techniques, several genes were identified to be involved in this asymmetry such as nodal or lefty in mice [11], other modifier genes or environmental factors are also likely to contribute [12].
In our case, we report a rare association between SIT and an ectopic kidney with a large pelvic stone and an obstruction of the left ureteropelvic junction. This obstruction occurs in 22–37% of ectopic kidneys [13].
Treatment of ectopic kidney stones (EKS) is considered challenging for the urologist [14, 15]. According to the European guidelines of Urology (EGU), non-invasive and minimally invasive treatments such as shock wave lithotripsy (SWL) and percutaneous nephrolithotomy (PCNL) represent the first choice in the management of kidney stones. However, under aberrant circumstances such as an ectopic kidney, laparoscopic-assisted PNL represents a safe and effective treatment approach [15].
Moreover, retrograde intrarenal surgery RIR is another endourological treatment option for the pelvic ectopic kidneys. Binbay et al. [16] and Bozkurt et al. [17] reported a stone-free rate (SFR) of 70.8% and 84.7% respectively after a single session of RIRS and with no major complications.
Robotic surgery for the ectopic kidneys is less commonly performed for kidney stone management. The use of such a technique showed promising results in reducing postoperative pain, perioperative morbidity, and early return to work [18]. Britt et al. reported the first nephrectomy to be performed using robotic techniques in patients with SIT and the third to use a minimally-invasive approach, with equivalent outcomes to conventional surgical methods [19].
The use of open surgery to treat pelvic kidney stones is high in developing countries such as Pakistan and Iran, in which the rate of pyelolithotomy is as high as 30% in pediatric patients [20, 21]. In the UK the incidence of open renal stone surgery is less than 1% [20].
In developing countries, the use of open surgery upon the non-invasive or minimally invasive approaches is mostly due to the unavailability of the equipment [14].
In our case, open surgery was performed for our patient due to limited resources (lack of endoscopic equipment, and expert hands). The results were promising and the patient was discharged without any serious complications. Similar cases underwent open surgery for EKS and no major complications were observed during and after the procedure [14]. Indeed laparoscopic surgery is a safe and useful method for EKS however in the case of SIT the surgeons might find it difficult to maintain the anatomical orientation [22]. In the literature, few articles that documented the laparoscopic approach in patients with SIT were published, describing a complete laparoscopic kidney removal of a renal mass in a patient with SIT [23]. Additionally, Makiyama and colleagues reported a case of retroperitoneal nephroureterectomy of a patient with SIT using a patient-specific simulator before surgery [24]. Indeed, laparoscopic nephrectomy may be a challenging approach in patients with SIT due to the difficulty in maintaining spatial anatomical orientation. This procedure is more convenient in transperitoneal laparoscopic nephrectomy compared with the retroperitoneal approach, because of the lack of landmarks such as organs in the narrow visible field [24].
Careful preoperative management and rigorous planning are required due to the association between SIT and cardiac, pulmonary, and renal anomalies [25].
