Case report
A 9 years old male child presented with complaints of rapidly
progressive shortness of breath for the last 2 months more on supine
position. The patient had a history of progressively increasing
swellings in upper limbs, lower limbs, and chest wall since the age of 2
years. The patient had a large, irregular, bony-hard, swelling involving
Upper 2/3 of sternum, suprasternal notch and right shoulder.
[fig.1]. The bony mass over suprasternal notch was compressing the
cervical part of the trachea, causing stridor. The swellings of
extremities were of cosmetic concerns only. The father of the patient
also had bony swellings over the right humerus and left femoral bone.
Computed tomography (CT) of the thorax showed a large exophytic bony
mass involving sternum and right side of chest wall [Fig 2A, 2B, 2C,
3A, and 3B]. The mass was encroaching in suprasternal notch causing
compression of the trachea at the level of lower border of C7 vertebra.
The minimum anteroposterior diameter of the trachea was 3.4 mm [Fig.
2D]. Multiple other bony exostoses also have seen arising from ribs
[Fig 2C] and scapula.
The key challenge in this patient was the severe external compression of
the trachea, not permitting the minimum required size of the
endotracheal tube. The case was discussed by the multidisciplinary
team. Considering the above circumstances, it was decided to
perform femoro-femoral Cardiopulmonary Bypass (CPB) under local
anesthesia preceding the induction of general anesthesia (GA). Various
invasive lines were placed under local anesthesia (LA) for
intraoperative monitoring after verbally explaining to the patient. The
right femoral artery and vein were exposed under LA and were canulated
with 16 and 20 Fr sized canula (Edwards FemFlex®) respectively.
Cardiopulmonary bypass (CPB) was established at 80% flow. After that
Anaesthesiologists induced the patient and put Flexo-metallic
endotracheal tube (ET tube) 4 mm size using a fiber-optic laryngoscope
and later, it was exchanged with 5.5 mm using a tube-exchanger. After
confirmation of good ventilation via the ET tube, CPB was weaned. The
total CPB time was 85 minutes.
A ’T’ shaped incision was given over swelling with transverse limb at
the level of the thyroid cartilage and the vertical limb extending up to
xiphisternum. The bony-mass was removed in piecemeal by using chisel,
hammer, and oscillating saw while taking care of major neck vessels and
trachea. While the suprasternal extension of mass was completely
removed, only debulking of sternal body and chest wall mass were done to
achieve fair cosmetics result. No evidence of tracheomalacia was found.
In view of uneventful post-operative recovery, and absence of any
residual respiratory difficulty, the patient was discharged on the
fourth postoperative day. Histopathology report was suggestive of
Osteochondroma. CT thorax done at one-month follow-up showed an adequate
debulking of mass [Fig 3C, 3D] with the normal trachea. The patient
was asymptomatic and aesthetically satisfied after 1 year.