A patient with situs inversus totalis presenting for emergency laparoscopic appendectomy: Consideration for safe anesthetic management.

Situs inversus totalis is an uncommon congenital positional anomaly in which orientation of all asymmetric organs in the body are mirror image of normal morphology. The condition if undetected may pose a diagnostic problem of abdominal pathology. We present a case of situs inversus totalis with acute appendicitis in adult female who was previously unaware of her situs anomaly. A 35-year-old adult female presented with history of acute pain abdomen in left iliac region; clinically, she was diagnosed to be acute diverticulitis. Further investigation with abdominal computerized tomography (CT) and ultrasound imaging confirmed situs inversus with acute appendicitis. Patient underwent emergency laparoscopic appendectomy under general anesthesia; intraoperative electrocardiogram (ECG) monitoring was done with reverse lead placement.

INTRODUCTION

Situs inversus is a congenital morphological anomaly of positioning of internal viscera characterized by transposition of abdominal viscera, and if is associated with right sided heart (Dextrocardia),[12] it is called as situs inversus totalis. Situs inversus may be abdominal, thoracic, or both. The incidence situs inversus varies from 1:5000 to 1:20000 births.[34] Except for positional anomaly patients, cardiac functions are normal.[56] It was Fabricius who first described situs inversus. Transposed thoracic and abdominal organs are a mirror image of the normal anatomy.[7] The recognition of situs inversus is important for preventing surgical mishaps that result from the failure to recognize the reversed anatomy as may happen in cases of cholelithiasis or appendicitis. Diagnosis can be readily established by using radiography or sonography. However, computerized tomography (CT) is the preferred investigation for its diagnosis. Generally, patients with situs inversus totalis are asymptomatic and have a normal life expectancy.[8] Documenting situs inversus in an individual is important in order to correctly interpret any future symptoms and avoid any inadvertent clinical or surgical mishap. We report a case of situs inversus totalis posted for emergency laparoscopic appendectomy.

CASE REPORT

A 35-year-old woman was presented to surgical OPD with a history of acute lower abdominal pain, vomiting, and low-grade fever. Per abdomen examination revealed tenderness in both iliac fossa with rebound tenderness and rigidity in left iliac fossa. Initially, she was diagnosed to be acute diverticulitis but imaging with CT and abdominal ultrasound confirmed situs inversus with acute appendicitis.

On pre anesthetic evaluation, thyroid swelling was noticed and her pulse rate was 105/min, blood pressure 160/100 mmHg, and temperature 102°F. On examination of cardiovascular system, apex beat was located at right 5th intercostals space 1.5 cm medial to mid clavicular line, on auscultation heart sounds were heard on the right side of the chest. The rest of the examination was unremarkable. Initial laboratory tests revealed TLC 16.4 × 109/L with neutrophilia (80%), thyroid function test was within normal limit. Fine needle aspiration cytology (FNAC) of thyroid swelling revealed nodular colloid goiter. Plain radiograph of chest showed dextrocardia with fundal gas shadow on right side [Figure 1]. Imaging by CT scan showed abdominal situs inversus [Figure 2]. The patient was subjected to cardiac evaluation with 12 lead ECG and echocardiography. ECG showed marked right axis deviation with negative p wave in lead aVL and I [Figure 3], ECG with reverse lead placement showing no abnormality [Figure 4], 2 D echocardiogram with M mode Doppler shown no cardiac shunting, ophthalmic fundoscopy showed grade 1 hypertensive retinopathy.

Figure 1

Chest X-ray showing dextrocardia and right-sided gastric air bubble indicating the presence of both dextrocardia and situs inversus (the most common combination). There was no radiological evidence of bronchiectasis

Figure 2

Imaging by computerized tomography scan showed abdominal situs inversus with dextrocardia and appendicitis

Figure 3

Electrocardiogram with normal lead placement showed marked right axis deviation with negative p wave in lead aVL and I.

Figure 4

Electrocardiogram with reverse lead placement showing no abnormality

Anesthesia was induced with propofol 2.5 mg/kg/min, fentanyl 2 μg/kg, and vecuronium 0.1 mg/kg. Anesthesia was maintained with N2O in O2 (70%:30%), halothane (0.5%-1%), muscle relaxation was maintained with intermittent vecuronium. During anesthesia, patient was stable. Reversal of neuromuscular blockade was performed with glycopyrrolate/neostigmine (0.02/0.05 mg/kg). Extubation was smooth. The patient was transferred to the post-anesthesia care unit (PACU). Postoperative analgesia was maintained with inj diclofenac 75 mg IV infusion. The postoperative course in PACU was uneventful. Patient was referred to physician postoperatively for evaluation of hypertension and was started with tab Atenol 50 mg; patient was discharged on the third day.

DISCUSSION

Incidence of situs inversus varies from 1:5000 to 1:20000 births,[3] situs inversus totalis usually does not cause any significant morbidity to an individual but it can coexist with other congenital anomalies, e.g., cardiovascular (Ventricular septal defect, Atrial septal defect, Tetralogy of Fallot, Transposition of great arteries), respiratory (bronchiectasis, paranasal sinus deformity), digestive system (anal atresia, duodenal stenosis, absent appendix, megacolon), etc., When situs inversus totalis, sinusitis, and bronchiectasis appear together in a patient, it is called Kartagener's syndrome.[9]

Following precautions should be taken during anesthetic management of patient with situs inversus.

Mainstem intubation can occur on left side and should be kept in mind while intubating the trachea.[10]

In case of inversion of great vessels, preference should be given to left internal jugular vein for cannulation (to avoid thoracic duct and to ensure direct access to right atrium).[10]

ECG electrodes and defibrillation pads should be placed in reverse manner.

In thoracic surgery, the anatomy of the bronchi should be considered before selecting a double lumen tube.[1011]

Patient should be evaluated for associated cardiovascular and other comorbidities.

Bronchiectasis and Kartagener's syndrome should be considered in these patients.[10]

Detection and documentation of situs inversus is important to prevent inadvertent future surgical mishap.

In about 31% of patients with left-sided acute appendicitis, first signs are pain and rebound tenderness in right iliac fossa. This is because that although there is visceral transposition, it is without corresponding changes in nervous system. Therefore, in 45% of cases, incisions at wrong sites have been given.[12]

CONCLUSION

Patients with situs inversus totalis are asymptomatic and have a normal life expectancy. The patients with situs inversus with acute appendicitis can be managed successfully with careful evaluation and meticulous planning.