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Enchondromas are intramedullary neoplasms made of well-differentiated hyaline cartilage. The exact incidence is unknown as the majority are asymptomatic and discovered incidentally. Enchondromas only occur in bones that pre-formed in cartilage. The commonest location is the tubular bones of the hand followed by the femur and humerus. Radiographically, enchondromas can be very large with consequent expansion of the bone, and thinning or complete loss of the cortex.
Multiple enchondromas are rare. In the case of Ollier’s disease, multiple enchondromas may be found within the hand of one limb, or have a much wider, hemisomic distribution, or affect the entire body with a hemisomic prevalence. The disease is non-hereditary and sporadic. It most commonly affects the tubular bones of the hand or foot. In the case of Maffucci syndrome, multiple enchondromas are associated with multiple cutaneous or deep haemangiomas; the presence of haemangiomas may be identified radiographically as phleboliths. Histologically, the enchondromas appear more cellular than solitary enchondromas, with more proliferative histological potential.
Transformation to a secondary chondrosarcoma is seen in both these conditions. In Ollier’s, this may occur in 20-40% of patients whilst in Maffucci, this is much more common and is likely to be greater than 50%. Malignant transformation may be heralded by an increase in size of a lesion or the development of symptoms, typically pain. Both conditions are associated with an increased risk of extra skeletal malignancies such as breast, liver, ovarian and CNS tumours.
The indications for imaging and biopsy of enchondromas in the context of Ollier’s disease is the clinical suggestion of malignant transformation i.e. pain or increase in size. In this case the patient had previously sustained a pathological fracture with resultant deformity and continued to have painful symptoms and dysfunction after fracture union. A biopsy described features consistent with a benign cartilage neoplasm, but recent evidence has questioned the validity of pre-operative biopsy in determining grade in cartilage tumours (https://online.boneandjoint.org.uk/doi/abs/10.1302/0301-620X.100B5.BJJ-2017-1243.R1).

Indications:
The indications for excision of an enchondroma are the clinical, pathological or radiological suspicion or malignant transformation. Relative indications may include recurrent fracture and resultant deformity, as in this case.
Symptoms & Examination:
Malignant transformation of enchondromas may cause pain and swelling, difficulty weight bearing in the lower-limb and dysfunction. Examination may reveal localised pain, deformity, bony hard swelling and limitation of motion. Neurological or vascular compromise would be highly unusual and more suggestive of a rapidly enlarging malignancy rather than a benign or slow growing low-grade chondrosarcoma.
Investigations:
Pre-operative imaging includes radiographs and MRI . If malignant transformation is proven after pre-operative biopsy then staging for a chondrosarcoma mandates chest CT and whole-body skeletal staging with bone scintigraphy or MRI. There are no serological investigations relevant to this diagnosis.
Operative Alternatives: In this case and intercalary resection and reconstruction, though more surgically challenging, was selected to preserve the gleno-humeral and ulno-humeral joints to maximise long-term function in an adolescent patient. Alternatively, joint sacrificing solutions may include proximal humeral allograft-prosthetic composite reconstructions or proximal humeral endoprosthetic reconstructions. It is believed that if good osseointegration is achieved and the joints are preserved, then function will be preserved; the functional outcomes with proximal humeral endoprosthetic replacements are significantly worse.
Non-Operative Alternatives: There are no medical therapies available for the treatment of benign cartilage tumours nor chondrosarcomas.
Contraindications: Medical co-morbidities inconsistent with major surgery.

The patient is positioned supine under general anaesthesia with supplementary supraclavicular blocks and intravenous antibiotic prophylaxis (Fluxloxacillin and Gentamicin) as per local antimicrobial protocols.
An arm table is positioned level with the shoulder to rest the ipsilateral arm doing the procedure.
Alcoholic skin preparation and incision Ioban drapes are used to cover the surgical field. The limb is isolated using sterile drapes.
Fluoroscopy is available throughout.
A second plastic surgical team are prepped to harvest the fibula autograft and perform the microvascular anastomosis.

Distal neurovascular observations
IV Antibiotics 24hours (flucloxacillin)
Poly-sling for 6 weeks: passive pendular glenohumeral and elbow exercises until week 6.
Start active elbow and shoulder motion from week 6
Removal drain 48 hours
Routine X-rays post-op
Repeat X-rays in clinic at 6 week intervals until union
Await histology results.

Options for reconstruction of intercalary segmental defects of the humeral metadiaphysis following oncological resection include fresh frozen or irradiated allografts, vascularised fibula grafts, endoprosthetic replacements and extracorporeal irradiation and reimplantation of the resected humerus.
The Capanna technique was first performed in 1988 and published in scientific literature in 1993 (https://link.springer.com/article/10.1007%2FBF02620523?LI=true). Vascularised fibula reconstructions had previously been used widely for upper and lower limb reconstructions but initially lack the structural bulk of allografts and so are liable to fracture. Allografts have initial strength but suffer from risks of non-union, infection and later graft fractures which have no ability to heal. Therefore combining the allogenicity of a vascularised fibula with the mechanical strength of bulk allograft was thought offer the best of both.
Li et al. reported seven cases with intercalary humeral Capanna reconstruction without infection nor fracture and the mean time to union for the fibula and allograft were 21 and 26 weeks respectively.The authors reported function results within 95% of normal and cited the vascularised fibula leading to early graft incorporation and rapid rehabilitation for their excellent functional outcomes (https://onlinelibrary.wiley.com/doi/pdf/10.1002/jso.21922). By contrast in a meta-analysis of vascularised fibula reconstructions of the humerus (without allograft shell) reported the same mean fibula graft union time and the most common complications were fracture (11.7%), nerve injury (7.5%) & infection (5.7%)(https://onlinelibrary.wiley.com/doi/pdf/10.1002/jso.25032).
Reference
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