Laparoscopic adrenalectomy in a patient with situs inversus totalis.

Situs inversus totalis(SIT) is a relatively rare condition involving transposition of both the abdominal and thoracic viscera. SIT typically presents as left to right reversal of the viscera combined with dextrocardia, while the individual organs function is normal. Although there are no obvious abnormalities in the function of transposed organs, anatomical irregularity causes important technical difficulties in the surgical treatment of these patients. In this study, we aim to report surgical challenges in laparoscopic adrenalectomy in a patient with SIT.

INTRODUCTION

Situs inversus totalis (SIT) is a relatively rare condition involving transposition of both the abdominal and thoracic viscera. SIT found in only approximately 1/5000–20,000 people.[1] The aetiology of transposition of viscera is obscured and the condition does not influence normal health or life expectancy, but it has important surgical implications.[2] In this study, we aim to report a rare case with right adrenal tumour complicated by total situs inversus that was treated with laparoscopic adrenalectomy.

CASE REPORT

A 26-year-old female patient admitted to our hospital with a complaint of headache. The neurological examination was completely normal. The patient's blood pressure was 230/160 mm Hg. The plain chest X-ray showed no abnormalities except dextrocardia [Figure 1]. We learnt that the patient's mother and sister were also diagnosed with SIT. Biochemical investigations for the aetiology of hypertension revealed a serum cortisol level of 17.06 mcg/dl (normal range: 6.7–22.6 mcg/dl), 5-hydroxyindoleacetic acid level was 4.7 mg/day (normal range: 2–10 mg/day). The urinary epinephrine level was 2.4 mcg/day (normal range: 4–20 mcg/day), whereas urinary norepinephrine level was high as 780 mcg/day (normal range: 92–604 mcg/day). Urinary vanillin-mandelic acid level was 8.63 mg/day (normal range: 0–1 mg/day).

Figure 1

Dextrocardia in the chest X-ray view

Dehydroepiandrosterone sulphate level was 333.2 mcg/dL (normal range: 108–441 mcg/dL) and adrenocorticotropin level was <1.6 pg/mL (normal range: 0–46 pg/mL). The tumour markers (alpha-fetoprotein, Ca 125, carcinoembryonic antigen and Ca 19-9) demonstrated no abnormalities.

Abdominal computed tomography of the upper abdomen also confirmed situs inversus and revealed a regular and homogenous solid tumour in the right adrenal gland, measuring 18 mm × 20 mm [Figure 2a]. A 18F-fluorodeoxyglucose (F18-FDG) positron emission tomography study was performed to further characterisation of the lesion which showed an increased FDG uptake, especially in the late sequences [Figure 2b].

Figure 2

(a) Computed tomography scan of the upper abdomen. The red arrow indicates the adrenal tumour. (b) Increased fluorodeoxyglucose uptake in the right adrenal

The patient was scheduled for a laparoscopic adrenalectomy. Hypertension alleviated with alpha-adrenergic blockade and adequate hydration treatment maintained for 2 weeks preoperatively. After the completion of pre-operative assessments, the patient was operated laparoscopically.

Patient placed in the left lateral recumbent position under the general anaesthesia. The first incision was made at the anterior axillary line, 4 cm below the subcostal margin, which was 12 mm length and used for the camera after the insufflation of the abdomen with 12 mmHg CO2. In the exploration, the spleen was in the right subphrenic space, whereas the liver was in the left subphrenic space due to the situs inversus. Two ports of 10 mm in diameter were inserted below the right midclavicular line 3 fingerbreadths below the right costal margin, respectively. The descending colon dissected from the white line of the peritoneum and retracted medially. The splenophrenic, splenocolic and splenorenal ligaments are divided using electrocautery. The spleen and pancreatic tail are then rotated medially [Figure 3a]. The peritoneal dissection is performed until the left renal vein is reached. The adrenal vein was divided after double clipping, and an adrenal tumour 30 mm × 20 mm × 18 mm in diameter was resected [Figure 3b]. The operation time was 60 min, without any complications. The blood loss was estimated to be about 30 mL. After 4 days hospitalisation, the patient was discharged. After the surgery, the patient's hypertension was completely resolved.

Figure 3

(a) View of the adrenal mass, after the adhesions dissected between spleen and omentum. (b) The adrenal vein after dissection of surrounding soft tissues

The pathologic examination revealed a 3 cm, encapsulated, yellowish nodular lesion. Capsule integrity was preserved. High cellularity, tumour necrosis, invasion to surrounding tissues, tumour cell spindling and vascular or capsular invasion were not observed. Mitotic activity found to be low (<1/10 high power fields). Profound nuclear pleomorphism and hyperchromasia were present in focal area. While cytoplasmic staining with synaptophysin was positive; staining with chromogranin, CK and claret were negative in immunohistochemical evaluation. Phaeochromocytoma of the adrenal gland scaled score was 2/20. The tumour was morphologically found compatible with phaeochromocytoma that behaves in a benign fashion.

DISCUSSION

SIT is a rare anomaly caused by embryonic development disorder. SIT typically presents as left to right reversal of the viscera combined with dextrocardia, while the individual organs function is normal.[3] The difficulty of surgery in patients with SIT is usually due to anatomical irregularities.

The most frequently reported laparoscopic procedures for the patients with SIT are laparoscopic cholecystectomy and appendectomy.[4] When we scan the English medical literature, we found only three similar cases which concern SIT and laparoscopic adrenalectomy. Moreover, all the tumours in these cases were located in the right upper quadrant.[567]

Toutounchi et al.[5] reported a case of an adrenal adenoma with situs inversus levocardia. They claimed that due to the anatomic variations, the operative time was longer when compared to standard laparoscopic adrenalectomy. This prolongation may be due to the increased incidence of organ abnormalities in patients with SIT. Toutounchi et al. faced with horseshoe kidney and abnormally enlarged pancreas in their case. The prolongation of the operative time may be due to this condition. In our case, the operative time was similar with standard laparoscopic procedure, and we did not encounter with any additional anatomical difficulties.

Kashiwagi et al.[6] reported a 45-year-old male with an adrenocortical adenoma. They operated the patient laparoscopically as we did. Kashiwagi call attention to the importance of the presence or absence of complicating anomalies and appropriate port positions for the targeted organ. Although there are many challenges in the laparoscopic surgery of the patients with SIT, Boland et al.[7] take attention to an advantage of SIT. They claim that access to the right adrenal gland in their case, an incidentally detected phaeochromocytoma in a patient with sigmoid tumour and SIT, was actually easier as the need for liver retraction was absent due to its presence on the left side of the abdomen.

Laparoscopic surgery in patients with SIT has been reported to be more difficult when compared to orthotopic patients, especially in laparoscopic cholecystectomies. One of the difficulties of laparoscopic surgery in these patients is due to the anatomical irregularities. The other difficulties are due to the loss of orientation in the mirror image and reduction in the dissection skills for the right-handed surgeons.[8]

To overcome these technical challenges, several modifications have been proposed such as: (1) Retraction of Hartmann's pouch by the first assistant, while the primary surgeon dissects Calot's triangle using his right hand via the epigastric port, (2) use of the epigastric port to retract with the left hand and operate with the right hand through the lateral subcostal port or (3) changing the position of the surgeon from the left side of the patient to near the patient's two abducted lower limbs.[910] In our case, we did not encounter with difficulties such as mentioned in laparoscopic cholecystectomies. And also, we did not need any technical modifications in a surgical procedure for SIT. The mirror image of the left adrenal did not cause a technical difficulty in dissection.

Laparoscopic adrenalectomy, which has already been established as a standard treatment for benign adrenal tumours due to its safety and minimal invasiveness, should not be considered a contraindication in patients with SIT. Since the adrenal glands are bilateral, an experienced surgeon is used to work on both right- and left-sided tumours. Therefore, a mirror image of the adrenal glands in patients with SIT should not be an inextricable situation for an experienced endocrine surgeon. We claim that laparoscopic surgery may be an appropriate treatment option for the patients with SIT after an attentive pre-operative evaluation and a rigorous intra-operative exploration.

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Conflicts of interest

There are no conflicts of interest.