Clinical & Medical Case Reports

Extensive vascular lesions can endanger the life of a child by their virtue of consumptive coagulopathy or cardiac failure. A conservative surgical approach is difficult and can be life-threatening due to uncontrolled bleeding. We report successful limb salvage in an infant despite an extensive and infected arterio-venous malformation of upper limb, complicated by severe consumptive coagulopathy

Case :

A term male, 3.2kgs, presented with a swelling over left arm detected antenatally. Antenatal scan- soft tissue swelling 10.8 x 6.8 x 5.4cm from left shoulder to elbow with heterogeneous cystic spaces and minimal vascularity.O/E- non compressible, no bruit heard. 

Investigations:

Doppler – hypoechoic lesion+ heterogeneous echotexture, numerous cystic spaces, weak color Doppler signal- S/O- Hemangioma

MRI- lobulated soft tissue mass 17 x 16 x 9.5cm with an intensive enhancement, necrotic and hemorrhagic areas, involving flexor and extensor muscles of the arm.Supplied by branches of axillary and brachial arteries.Diagnosed as- Hemangioma , hence was started on propranolol 0.5 mg/kg/ day. At 4 months of age surgery became an imperative life saving mode of treatment.  

Operative procedure :

• Planned for amputation/ disarticulation.
• Axillary vessels identified and controlled
• Tumor excised in toto
• Flexor and extensor muscles preserved as much as possible
• Radial nerve through the tumor was divided and re-anastomosed
• Disarticulation was hence avoided.
• Arm and hand movements preserved at 10mth follow-up. 

Conclusion :

A conservative limb salvage surgery is possible even in case of an extensive vascular anomaly. A good vascular control is a must to prevent life threatening hemorrhage in a conservative surgery.