Case of the Fortnight
Functional Reconstruction in a 3 year Old Child with Rhabdomyosarcoma of the Thumb
Tumour seen preoperatively
Presentation
A 3 year child presented with fracture of proximal phalanx and swelling of right thumb at a local hospital. He was treated conservatively with plaster slab immobilization. Since the symptoms and swelling did not subside, another radiograph was taken which showed rarefaction of the bone. He underwent biopsy of the swelling. Biopsy revealed
malignant round cell tumour and he was referred to us for further management.
| Ray amputation of the involved thumb | The hand before reconstruction |
Management
Being an aggressive tumour, the whole of the thumb ray up to the CMC joint was amputated. Detailed study of the tumour revealed Malignant Rhabdomyosarcoma with LCA negative, Cytokeratin negative, Myoglobin strongly positive. He was on cyclical chemotherapy for 1 year with vincristine and actinomycin. Clinically the child was tumour free.
Reconstruction
The thumb was reconstructed by pollicisation of the index finger.
The pollicisation steps
At 1 year followup there is no local and systemic recurrence. Functional assessment showed pinch strength of 40% and grip strength of 50% compared to the other hand.
Post operative pictures
He uses the new thumb for all activities.
| Scissor grip before pollicisation | Using his pollicised index finger like normal thumb |
Discussion
Rhabdomyosarcoma is the most common soft tissue sarcoma in children. More than 7 % of the lesions occur in the upper extremity and hand involvement although rare has been previously reported. These tumours present as rapidly enlarging painful mass acheiving substantial size. The lung and the lymph nodes are the most common metastatic sites. Overall survival from this tumour is improving currently because of use of newer chemotherapic agents such as Vincristine, Actinomycin-D, Cyclophosphamide and Adriamycin.
Limb sparing tumour resection with adjuvant chemotherapy is normally preferred to amputation. Soft tissue sarcomas involving the hand especially the thumb requires careful surgical planning and should be performed by a team of surgeons experienced in hand tumours and the necessary reconstructions that follow wide resection.
In this child as the soft tissue sarcoma involved the thumb which is most crucial for hand function. It requires proper planning to ensure good functional outcome after adjuvant chemo and radiotherapy. As the thumb was amputated at the Carpo Meta Carpel joint the only option of reconstruction was pollicisation.
Pollicisation of index finger consists of transferring the index finger in the position of the thumb to achieve best possible hand function. This technique was first described by Gosset, in 1949 and modified and popularized by Buck Gramcko.
Conclusion
Functional Rehabilitation of major childhood malignancy in the hand is satisfactory. Prognosis is good
with long disease free interval. The important message learnt in this case is that any one who first sees such a patient should be familiar with the presenting signs and symptoms of soft tissue sarcoma. If lesion is suspicious, appropriate tumour work up should be initiated as early as possible. A combined team approach and long term vigilance is required in these patients.
Surgeons
Dr. S Raja Sabapathy
Dr. Ravindra Bharathi
Dr. Hari Venkatramani
Dr. Sanjai Ramkumar
Anaesthesiologists
Dr. V Ravindra Bhat
Dr. G. Venkateswaran