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Multiple Enchondromas of the Hand in Children: Long Term Follow up of Mean 15.4 years
Assaf Kadar, MD; Steven L. Moran, MD
Mayo Clinic, Rochester, MN

Background
Multiple enchodromatosis of bone, termed Ollier's disease, or Maffucci syndrome when associated with hemangiomas, is a rare disease that can affect the pediatric hand. This condition often causes a finger mass, deformity, pain and possible pathological fractures, and has been associated with malignant transformation to chondrosarcoma. The aim of our study is to describe the long term sequela of multiple enchondromatosis of the hand in the pediatric population, specifically the rates of malignant transformation, tumor recurrence, rates of pathological fracture, and phalangeal growth arrest.

Methods
We examined a total of 127 phalanxes and metacarpals in 15 pediatric patients who were treated in our institute. Only patients with follow up of at least 4 years were included. We retrospectively reviewed patients' chart and hand radiograph for symptoms including pathological fractures, indications for surgery, and postoperative complications including tumor recurrence, and malignant transformation. We assessed phalangeal growth arrest with radiographs and normalized phalangeal growth charts.

Results
Mean age of diagnosis was 5.8 years and mean follow up time was 15.4 years. Pathological fractures were common at 46% of pediatric patients, but ceased to occur once reaching adulthood. Outcomes of pathological fractures were excellent, regardless of treatment. Malignant transformation was very rare (0.7% of lesions) and did not occur during childhood. 80% of patients underwent surgical treatment of curettage and bone graft for the lesion, yet recurrence was common and affected 33% of treated patients. Phalangeal Growth arrest was the most common long term sequela and affected 11% of phalanxes and metacarpals. This sequela was significantly more prevalent in patients who had surgical excision of the tumor.

Conclusions
Our findings reassure that malignant transformation of enchodromatosis of the hand is rare and even rarer in the pediatric population. Pathological fracture is a common, but has excellent outcomes. When considering surgery, parents should be counseled about the possibility of phalangeal growth arrest and recurrence of the lesion.


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