Author
Information
(*
Speciality Medical Officer, ** Registrar, *** Additional Professor.
Department of Obstetrics & Gynaecology, LTMM College &
General Hospital, Mumbai, India.)
Abstraxt
Situs
inversus
is a rare congenital anomaly where there is a reversal placement of
various thoracic and abdominal organs from their normal anatomical
location.
5-6
% of people with Situs inverses have heart defect & 20 - 25 % are
associated with Kartagener's Syndrome. In most of the cases patients
of these conditions are apparently normal and detected accidentally.
However, due to presence of pre-existing cardiovascular or
respiratory problems these individuals are prone to catch various
infections. These individuals also have risk for sudden death. Here
we present a rare case of situs inversus totalis detected
accidentally in second
full-term pregnancy.
Introduction
Situs
inversus
is a rare congenital anomaly in which there is reversal placement of
various thoracic and abdominal organs. In
Situs inversus
totalis,
mirror image of the anatomic location of all the viscera occurs while
in dextrocardia only the apex of the heart points to right and is
present in the right hemi thorax. [1]
When Situs
inversus
is associated with
sinusitis and bronchiectasis, it constitutes Kartagener’s
syndrome.[2]
Case
Report
A 31
years old patient, married since 10 years, gravida 4, living 1,
spontaneous abortion 2, with 40 weeks of gestation with previous
lower segment cesarean section done, was admitted in our tertiary
care hospital in latent phase of labour. On
admission patient was complaining of pain in abdomen with no
complaints of bleeding or leaking per vaginum. She had a female child of 5 years, delivered at term by emergency lower
segment caesarean section (LSCS) done in view of
cephalopelvic
disproportion. There was no history of intraoperative or post-operative
complication. Second and third pregnancies ended as spontaneous abortions.
Dilatation and curettage was not done both the time. Gravida 4 -
present pregnancy, patient was registered at peripheral hospital and
she had no history of any complications in this pregnancy. On
admission – vital parameters were normal, cardiovascular system-
both heart sounds were heard more prominently on right fifth
intercostal space, no murmur was heard, Respiratory system - clear,
air entry bilaterally equal. Per
abdomen-uterus full term, vertex presentation, fetal heart rate 140
bpm, was getting minimal uterine activity, previous lower segment
pfannenstiel scar was present, and no scar tenderness was noted. Per
vagina - cervical os - one finger loose, 30 % effaced, vertex
presentation, with bilateral convergent wall suggestive of mid pelvic
contraction. Decision of emergency lower segment caesarean section
(LSCS) was taken in view of cephalopelvic disproportion after cardiac
evaluation. Twelve lead Electrocardiogram was done suggestive of
inversion of T waves in aVL, v 1- v 6. Cardiac reference was done and
2D-ECHO was advised. Echocardiography confirmed dextrocardia with
normal cardiac valve areas and normal size ventricle and atria with
ejection fraction of 60%, no PAH, no evidence of vegetation. Fitness
for emergency lower segment caesarean section was given by
cardiologist. Ultrasonography of abdomen was advised and was
suggestive of
situs
inversus-large lobe of liver in left hypochondria, gallbladder in
left hypochondria, and spleen in right hypochondria. Emergency LSCS
was done under spinal anaesthesia, no intraoperative complication
occurred. Intraoperative large lobe of liver in left hypochondria was
noted. Patient was monitored postoperatively, no complication
occurred. In Postoperative period X-ray chest of mother and baby was
done, mother’s X-ray was again suggestive of situs inversus
(pic-1). X-ray chest of baby was normal. Patient was discharged on
day 5 of LSCS. Patient was informed regarding the complication that
can occur with
situs
inversus and screening of other family member was advised.
Figure
1. Chest radiograph showing dextrocardia with fundic bubble of
stomach on right side.
Discussion
The
exact incidence of situs inverses is not known because most of the
patient remains asymptomatic. It
is 0.01% approximately.
Types
of sites inverses:
Situs
inverses
totals:
Generally, there are no complications associated with situs inversus
totalis because the organs are just reversed. Patient can remain
asymptomatic but situs
inversus
totalis may also be associated with some other rare conditions. 5 - 6
% people with situs
inversus
totalis suffers from congenital
heart disease.
20
- 25 %of patients with situs inversus totalis have
Kartagener Syndrome.
Patient
with Kartagener Syndrome also have to face problems of abnormal
bronchioles of the lungs and male infertility.
Dextrocardia
is
a congenital condition in which only heart is
located on the right side of the chest. Left lung is
smaller than the right lung. People may live normally, however few
may suffer from additional congenital heart abnormalities like
transposition
of the great arteries. [3]
Situs
ambiguous
occurs in one in 20,000 births. In situs ambiguous, both liver and
stomach develops in centre, stomach remains behind the liver. [4]
In
case if situs inversus is diagnosed at autopsy, [5]
then
other family members should be counselled and are advised for
screening, as they are prone to have various cardiac abnormalities.
If
it is diagnosed during lifetime, the dangers of smoking and fumes
should be told as they are at high risk for development of various
respiratory infections. These patient should be advised to take
vocational guidance. Cardiac abnormality and respiratory
complications decreases the life span and thus increases the rate of
sudden death in patients of situs inversus.
Conclusion
Situs
inversus is a rare congenital anomaly with reversal placement of
various thoracic and abdominal organs. Usually detected accidentally,
are susceptible to catch various infection and are prone to sudden
death.
- Narayan Reddy KS. Death and its cause. In the Essentials of Forensic Medicine &
- Toxicology. Pub. by K. Sugana Devi, Hyderabad, 22nd Ed., 2003 : p. 417.
- Kobzik L. The Lung. In Robbin's Pathologic Basis of Disease ed. by Cotran, Kumar and
- Collins. Pub. by W.B. Saunders Co., Philadelphia, In India by Harcourt India Pvt.Ltd., New Delhi. 6th Ed. 2000: p.716.
- Chugh SS, Kelly KL, Titus JL: Sudden Cardiac Death with apparently normal heart.
- Circulation, 2000; 102 : 649-654.
- Adults:Ann S. Fulcher, MD, and , Mary Ann Turner, MD From the Department of Radiology, Medical College of Virginia of Virginia Commonwealth University, 401 N 12th St, Rm 3-407B, Richmond, VA 23298-0615.
- Hangartner JRW, Marley NJ, Whitehead A, Thomas AC and Davies MJ. The assessment
- of cardiac hypertrophy at autopsy. Histopathology, 1985; 9: 1295-1306.
Citation
Madhu Kumari, Mulchandani D, Thakur V, Chavan NN. Situs inversus: a rare congenital anomaly. Situs
inversus:
a rare congenital anomaly. JPGO 2015. Volume 2 No 9. Available from: http://www.jpgo.org/2015/09/situs-inversus-rare-congenital-anomaly.html