orphananesthesia

Anaesthesia recommendations for

Situs inversus totalis
Disease name: Situs inversus totalis

ICD 10: Q89.3

Synonyms: Situs inversus, Situs inversus viscerum, Complete situs inversus, Complete
situs inversus viscerum

Disease summary: Situs inversus is a congenital abnormality in which organs in the thorax
and the abdomen are opposite to their normal positions. The normal arrangement of organs
is known as Situs solitus. Situs inversus is the mirror-imaged arrangement of the organs (The
anatomical left lung is in right hemithorax and the anatomical right lung is in left hemithorax.
The stomach and spleen are on the right, liver on the left. The heart is on the right). Situs
inversus is categorized under Heterotaxy syndromes, which results from failure of the
developing embryo to establish normal left-right asymmetry and is associated with a wide
range of cardiac and extracardiac congenital anomalies. During the embryological develop-
ment, a 270 degree clockwise rotation instead of normal 270 degree anti-clockwise of the
developing thoraco-abdominal organs results in mirror image positioning of the abdominal
and thoracic viscera.

Situs inversus is a hereditary syndrome, which is estimated to occur in 1 in 8,000 to 25,000

individuals. It was reported as one in 5000–20000 births. Situs inversus totalis is usually an
autosomal recessive disorder, and sometimes may be X chromosome-related. There is no
established gender or ethnical difference in its incidence, but genetic predisposition and
familial occurrence point towards multiple inheritance patterns. Most cases are caused by
ciliary abnormalities (the primary ciliary dyskinesia disorders). Cilia also are responsible for
determining proper visceral laterality in utero.

Thus, during embryogenesis in patients with primary ciliary dyskinesia, visceral laterality will
be determined at random, and half of these patients will have situs inversus totalis. Situs
inversus totalis is associated with right-sided heart (Dextrocardia). Situs inversus may be
abdominal, thoracic, or both. Most patients are asymptomatic and maintain their normal life.

The association of situs inversus totalis with syndromes such as Kartagener’s syndrome (i.e.
situs inversus, primary cicliary dysfunction (PCD), chronic sinusitis and bronchitectasis),
cardiac anomalies, spleen malformations, mucociliary dysfunction, and airway anomalies
makes the clinical management difficult for the anaesthesiologist.

There is a significantly higher incidence of heart defects in this group compared to normal
hearts. Situs inversus totalis is associated with numerous cardiac anomalies such as atrial
septal defect, ventricular septal defect, transposition of great vessels, abnormal atrioventri-
cular valves, absent coronary sinus, conduction abnormalities, double-outlet right ventricle,
total pulmonary venous defect and pulmonary valve stenosis. Dextrocardia with complete

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situs inversus occurs in approximately 2 of 10 000 births. The most prevalent cardiac defects
associated with situs inversus are ventricular septal defect and transposition of the great
vessels. There are limited reports of sick sinus syndrome diagnosed in patients with
dextrocardia. In general, heart rhythm disturbance is not a significant finding in patients with
situs inversus compared to situs solitus.

The extracardiac anomalies that most frequently challenge the anesthesiologist are gastro-
intestinal abnormalities, hepatic dysfunction, splenic dysfunction, respiratory abnormalities,
and associated midline defects.

Situs inversus is associated with primary ciliary dyskinesia (Kartagener syndrome) in 20-25%
of cases. Kartagener syndrome occurs with the triad of bronchiectasis, chronic sinusitis (as
symproms of PCD) and situs inversus and should be excluded by a carefully obtained
medical history. Situs inversus totalis is present in 50% of individuals with Kartagener syn-
drome. The patients are prone to increased probability of developing respiratory complica-
tions. Aggressive preoperative optimization and treatment of infection is required. A history of
intermittent respiratory tract infections, especially during the winter season, should be
watched.

The coexistence of situs inversus and congenital duodenal obstruction is an extremely rare
situation. A relationship between ectopic thyroid and situs inversus totalis was reported. Most
patients with ectopic thyroid show clinical features of euthyroid, while a few patients show
clinical features of hyperthyroidism. Thyroid function tests are useful for the preoperative dia-
gnosis.

Spinal dysrapism, which includes all forms of neural tube defects from thickened filum
terminalis to meningiomyelocele, is an important abnormality that is associated with situs
inversus regarding neuraxial blocks.

There is some association between situs inversus and renal abnormalities. Kidney function
should be tested preoperatively if situs inversus with Kartagener syndrome is suspected.
Biliary atresia may occur in 28% of infants born with situs inversus as compared to the 0.01%
of the general population. The anaesthetic concerns include managing the abnormalities
related to liver pathology (deranged metabolism, coagulation problems). Situs inversus is
also a very rare feature of Bardet Biedl syndrome.

Medicine is in progress

Perhaps new knowledge

Every patient is unique

Perhaps the diagnosis is wrong

Find more information on the disease, its centres of reference and patient
organisations on Orphanet: www.orpha.net

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 Typical surgery

Thoracic surgery; Cardiovascular surgery; Gastrointestinal surgery; Liver and spleen surgery;
sinus and ear surgery.

Type of anaesthesia

There is no definite recommendation for either general or regional anaesthesia.

There are reports of spinal, epidural, combined spinal-epidural, caudal anaesthesia and
nerve blocks without any complications. The spinal deformities like split cord, spina bifida,
meningomyelocele, scoliosis has been described in the literature; thus, the patient should be
evaluated carefully if any surgery is planned under neuraxial anaesthesia.

A regional block may be the method of choice if feasible. It should be provided that the motor
block does not spread high into the thorax and respiratory muscle tone is maintained.
Successful axillary brachial plexus blockade was reported under midazolam sedation. The
primary advantage of regional technique is non-interference with respiratory muscle function,
especially in patients with Kartagener syndrome. It also allows for improved clearance of
respiratory secretions and early postoperative ambulation. An important consideration is the
association of situs inversus with spinal dysraphism and other spinal malformations.
Regional anesthesia is considered a safe method for any infra-umbilical surgery compared to
general anesthesia, if no spinal anomaly or dysraphism is confirmed.

Thoracic segmental epidural anesthesia was reported in cases of situs inversus associated
with Kartagener syndrome for laparoscopic cholecystectomy. This may be considered due to
chronic airway disease and to prevent prolonged mechanic ventilation. However, the
definitive advantage of this approach may be debated and the safest in the respective
hospital best established anesthesia technique should be preferred to techniques that are
infrequently used, especially in patients with rare diseases.

General anaesthesia can be administered as total intravenous anaesthesia or with volatile

anesthetics. Nitrous oxide can also be used, but should be avoided in case of manifest
cardiac involvement such as atrial septal defect or ventricular septal defect.

There is no contraindication for premedication such as sedation or analgesia for non-

symptomatic patients. In patients with symptoms of the disease, the administration of
sedative and analgesic drugs should only be performed after considering the individual risks
carefully.

Necessary additional pre-operative testing (beside standard care)

The precise diagnosis of situs inversus and a thorough pre-operative evaluation can
minimize the difficulties and the various potential challenges. The most preferred diagnostic
technique involves chest and abdominal radiograms. A chest x-ray helps to assess cardiac
situs and size, pulmonary blood flow, bronchial anatomy, liver symmetry, and stomach. The
patient’s chest X-ray reveals dextrocardia and a right-sided stomach bubble.

Ultrasound can determine the situs of abdominal organs, particularly abnormal liver
symmetry and presence of the spleen and gallbladder. Magnetic resonance imaging is

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helpful for defining extra-cardiac and cardiac anomalies. An upper gastrointestinal contrast
study screens intestinal abnormalities and other digestive tract disorders.

Computed tomography (CT) imaging shows pancreatic and airway anomalies and the
morphology of the right atrium. CT may show lung overinflation, bronchial wall thickening,
and sinus malformations in patients with primary ciliary dyskinesia. Electron microscopy may
be utilized to analyse respiratory mucosa specimens.

Evaluation of cardiovascular anatomy by echocardiography requires careful evaluation of

each cardiovascular segment. An electrocardiogram may help to assess atrial situs and
conduction abnormalities. Cardiac catheterisation and magnetic resonance imaging may be
necessary in some patients to completely delineate cardiac anatomy. Transoesophageal
echocardiography (TEE) has also been described as a useful imaging modality to diagnose
dextrocardia. TEE examination in situs inversus may be challenging and requires vigilance
when differentiating morphologic and anatomic left/right structures.

Lung function test including lung volumes and blood gas analysis may be done to evaluate
grade of pulmonary involvement. Respiratory testing usually reveals little deviation from
normal parameters.

Before anaesthesia, cardiology consultation is suggested. Physical examinations of the

heart, such as percussion and palpation during routine exams, are important.

Fibreoptic bronchoscopy may be necessary for the evaluation of the bronchial tree for one-
lung ventilation. The mirror image airway anatomy demands meticulous planning for the
placement of lung separation devices.

The above mentioned diagnostic options should be performed in patients with suspected
Situs inversus totalis or Kartagener Syndrome. In patients already diagnosed, the anaesthe-
tist should have access at least to a contrast-inhanced CT scan and the echocardiography
results, including pressure gradients. A preoperative heart and lung ultrasound should be
part of any elective procedure in patients with Situs inversus.

Particular preparation for airway management

The association of situs inversus with other syndromes and diseases such as Kartagener
syndrome, mucociliary dysfunction, airway anomalies (Narrowing of trachea) may predispose
the patient to numerous varieties of airway difficulties.

Airway anomalies are rarely seen. Anomalies of relevance include choanal atresia,
micrognathia, cleft lip and palate, tracheo-oesophageal fistula, tracheal bronchus and
aglossia/hypoglossia. When performing nasal intubation, difficult airway management is
required due to nasal cavity narrowing by chronic sinusitis if present. It is best to avoid nasal
intubation due to narrowing of the nasal cavity by chronic sinusitis. Inspired gases should be
humidified to avoid thick secretions. Lung transplant candidates have limited pulmonary
reserve and may desaturate quickly, so airway management should be performed as
expeditiously as possible. During intubation, left endobronchial placement of the endo-
tracheal tube may occur since the left bronchus is more in line with the trachea.

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 Particular preparation for transfusion or administration of blood products

Requirement for blood products in patients with situs inversus totalis may be higher during
high invasive cardiac or thoracic surgery.

Particular preparation for anticoagulation

Patients with situs inversus totalis and Kartagener syndrome may be predisposant to
thromboembolia. Impaired mobility may suggest a higher risk of postoperative thrombosis.

Particular precautions for positioning, transportation and mobilisation

The right gastric place should be kept in mind in regard of positioning of the patients.

Interactions of chronic disease and anaesthesia medications

No special considerations.

Anaesthetic procedure

Successful management requires appropriate understanding of the special anatomy.

Considering the mirrored position of the thoracic viscera is especially important when placing
a central line. As opposed to patients with situs solitus, the left internal jugular vein provides
the most direct access to the morphologic right atrium. In central venous catheter and
pulmonary artery catheter placement, a left internal jugular vein approach may be more
prudent. Ultrasound guidance should be utilized. Total pulmonary venous anomaly and right
superior vena cava are reported to accompany situs inversus. Thus, right internal jugular
vein is a preferable site for central venous cannulation in such a case.

Premedicant drugs that depress ventilation or ciliary activity should be avoided in situs
inversus associated with Kartagener syndrome. Nasal airways and/or nasal intubation should
be avoided in these patients due to sinusitis.

If the patient has a diagnosis of Kartagener syndrome, certain preoperative pulmonary issues
should be identified or addressed before proceeding with surgery. These patients are
predisposed to multiple airway anomalies and infections. Pulmonary percussive therapy,
bronchodilators, incentive spirometry, postural drainage, steroids, and antibiotics are
beneficial to optimise the pulmonary system before surgery.

Induction with inhalational agents is slow in case of pulmonary hypertension but faster with
intravenous agents in right to left shunts.

The abnormalities related to impaired motility of neutrophils require cautious aseptic

techniques during anaesthesia. This includes placement of a needle and catheter insertion
for epidural block.

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Auscultation sites must be changed for confirmation of tracheal intubation. It should be kept
in mind that the left lung will be ventilated better in case of over-advanced endotracheal tube,
not the right one.

A case of prolonged paralysis after administration of succinylcholine has been reported in a

patient with situs inversus totalis. This may show a possible link between situs inversus and
atypical cholinesterase but routine testing for atypical cholinesterase is not recommended at
this point.

The mirror-image anatomy of the bronchi makes lung isolation challenging. Bronchoscopic
inspection of the bronchial anatomy through a large single-lumen endotracheal tube (ETT)
before double-lumen ETT is advantageous. Use of a right-sided double lumen ETT in the left
lung of the patient was reported to maintain the ventilation of the apical segment of the left
lung. The use of a bronchial blocker can also be considered. Confirmation with a fibreoptic
bronchoscope is a reliable option. In children, one-lung ventilation using a single lumen
tracheal tube has also been reported in situs inversus.

In patients with Kartagener syndrome, humidification of gases should be added to the

anaesthesia circuit and frequent suctioning of the endotracheal tube may be required.

During defibrillation, paddles should be placed in reverse position: i.e. on the upper left and
lower right of the chest.

It is recommended to obtain informed consent for possible mini-tracheostomy prior to surgery

from every patient with Kartagener syndrome.

Particular or additional monitoring

Preoperative monitoring will be different because of dextrocardia. For each particular type of
dextrocardia, the electric potentials may reflect differently, depending on the stereotypic
position and situs type. The electrocardiogram lead placement should be reversed as a
mirror image (left-sided limb leads and fifth precordial leads are placed on the right, and
right-sided limb leads on the left). The sinus node is located to the left (mirrored) in case of
situs inversus, so the P wave is inverted. This means that there is a negative P wave in leads
I and aVL, and a positive P wave in aVR. QRS complex is negative in lead I, while the T
wave is inverted if the leads are not reversed. Mirror positioning of the heart results in right-
to-left septal depolarization, so Q waves will be present in the right precordial leads. Invasive
monitoring may be useful for patients with cardiac disease.

Possible complications

Respiratory complications are the primary concern that the anaesthesiologist will face in case
of association with Kartagener syndrome. The patient’s pulmonary status should be
optimised prior to the surgical procedure if general anaesthesia is planned. Antibiotic prophy-
laxis, bronchodilators, chest physiotherapy, steroids, postural drainage, and incentive spiro-
metry may be required. Using disposable air equipment and performing smooth manipula-
tions during intubation and extubation are required to avoid traumatizing the mucosa.
Bronchoconstriction can be avoided by using volatile anaesthetics. Corticosteroids may be
useful prophylactically to prevent bronchospasm. The possibility of secretions blocking the
endotracheal tube necessitates frequent endotracheal tube suctioning. Lung compliance and
peak inspiratory pressures should be observed closely. Mucus plug obstructing the

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endotracheal tube and causing desaturation was reported which required changing the tube.
Attention should be paid for mucus that may obstruct the airways.

Situs inversus totalis patients are at risk for perioperative cardiac insufficiency as well.

Post-operative care

Intensive care is recommended after cardiothoracic surgery with situs inversus totalis. High-
risk patients should be observed in the intensive care unit for arrhythmias and cardiac
ischemia. In patients with situs inversus totalis and Kartagener syndrome, prolonged
ventilation may occur.

Adequate analgesia should be provided for the patient. Inadequate analgesia may lead to
respiratory insufficiency in presence of Kartagener syndrome. Pleuritic chest pain can occur
up to 21% of patients associated with Kartagener syndrome. This kind of pain is usually seen
in patients with bronchiectasis. Thoracic paravertebral block and electrical nerve stimulation
has been successfully used in the management of intractable pleuritic chest pain. Improved
ventilatory function is expected with relief of chest pain. Epidural analgesia should be
considered in patients undergoing thoracotomy.

Disease-related acute problems and effect on anaesthesia and recovery

The diagnosis is usually found incidentally which can be occult in emergency cases. Patients
should be evaluated carefully prior to surgery, especially paediatric patients, as they are
more likely to present with hidden abnormalities. Informing the patient about his/her own
situation and his/her informing the clinician will make the diagnosis easier. Documenting situs
inversus totalis in a person is important after the signs of the disease occur. The absence of
heart sounds on auscultation at the precordial area, confusion during the diagnosis of acute
cholecystitis, acute appendicitis and splenetic lesion on the basis of clinical examination
raises the suspicion of situs inversus totalis. Radiologic evaluation, initially a chest X-ray, is
suggested for diagnosis.

Ambulatory anaesthesia

Ambulatory anaesthesia (according to common guidelines) should only be done in Situs

inversus totalis patients with no cardiopulmonary symptoms and low risk surgery.

Obstetrical anaesthesia

Prevention of aorto-caval compression may be problematic. Due to atypical location of the

vena cava, it is difficult to determine whether left or right uterine displacement is needed.
Uterine positioning is suggested to be to the right.

In obstetric patients with Situs inversus and Kartagener syndrome requiring caesarean
section can be successfully managed with spinal anaesthesia. In these cases the CT scan of
the spinal column, usually performed during the primary diagnostic roll-up, should be
carefully examined.

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Date last modified: February 2020

This recommendation was prepared by:

Author

Alparslan Koç, Anaesthesiologist, Department of Anaesthesiology and Reanimation,

Dumlupınar University Evliya Çelebi Training and Research Hospital, Kütahya, Turkey
[email protected]

Disclosure The author has no financial or other competing interest to disclose. This
recommendation was unfunded.

This recommendation was reviewed by:

Reviewers

Florian Weis, Anaesthesiologist, Department of anaesthesiology and operating intensive

care medicine, Klinik Fürstenfeldbruck, Germany

Carlos Degrandi, Anaesthesiologist, Santos, Brazil

Disclosure The reviewers have no financial or other competing interest to disclose.

Please note that this recommendation has not been reviewed by an anaesthesiologist and a disease expert but by two
anaesthesiologists instead.

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