MACRODACTYLY ( LOCAL GIGANTISM)

Local gigantism or localised gigantism is a condition in which a certain part of the body acquires larger than normal size due to excessive growth of the anatomical structures or abnormal accumulation of substances. It is more common in fingers and toes, where it is termed macrodactyly. However, sometimes an entire limb may be enlarged.

CAUSES

Congenital:

• Klippel Trenaunay Weber syndrome: is a  rare congenital medical condition in which blood vessels and/or lymph vessels fail to form properly. The three main features are nevus flammeus (port-wine stain), venous and lymphatic malformations, and soft-tissue hypertrophy of the affected limb.

• Maffucci syndrome :is a sporadic disease characterized by the presence of multiple enchondromas (is a cartilage cyst found in the bone marrow) associated with multiple hemangiomas . 

• Macrodystrophia lipomatosa: is a rare, congenital form of localized gigantism of fingers or toes. Almost always unilateral and can involve both the upper extremities (usually in the distribution of the median nerve with the index and middle fingers most commonly involved) and in the lower extremities (usually in the distribution of the plantar nerves). Predominantly fibroadipose tissue produced in association with arteriovenous fistulas or localized hyperplastic vascular and lymphatic elements.

• Neurofibromatosis 
• Lipoatrophic diabetes 

• Proteus syndrome: congenital disorder that causes skin overgrowth and atypical bone development, often accompanied by tumours over half the body.

ACQUIRED

• Inflammation, due to trauma or infection
• Tumors like osteoid osteoma
• Arteriovenous malformations occurring on a limb, before the closure of epiphyses in long bones
• Elephantiasis, which is quite common in south-east Asia due to prevalence of filariasis.
• Still's disease
• Amyloidosis
• Acromegaly

TREATMENT

As the causes of local gigantism are varied, treatment depends on the particular condition. Treatment may range from antibiotics and other medical therapy, to surgery in order to correct the anatomical anomaly.