Limb length discrepancy in cutis marmorata telangiectatica congenita: an audit of assessment and management in a multidisciplinary setting.

BACKGROUND: Cutis marmorata telangiectatica congenita (CMTC) is a vascular malformation, diagnosed based on cutaneous manifestations. It is associated with limb length discrepancy (LLD) and asymmetry, but the exact extent of this and its relationship to the site of the cutaneous manifestations have not been delineated.

OBJECTIVES: To review the orthopaedic problems associated with CMTC, concentrating on the assessment and management of the LLD.

METHODS: This study is a retrospective analysis of orthopaedic, dermatological and vascular data that were collected prospectively at our tertiary referral centre. We identified 80 patients with an initial diagnosis of CMTC; 57/69 patients with a confirmed diagnosis had lower-limb involvement.

RESULTS: An LLD was identified in 51% of cases. The discrepancy was significant (defined as ≥ 2 cm) in nine patients and was confirmed using standing leg-length radiographs. Of these patients, three had epiphysiodesis to correct the discrepancy, and surgery is planned in five others.

CONCLUSIONS: Limb length discrepancy and asymmetry are common in CMTC; however, this is below the significant threshold in most cases. It is therefore recommended that any discrepancy be initially monitored clinically. This should be followed by standing leg-length radiographs at the age of 10 years (girls) or 12 years (boys), or if the LLD is ≥ 2 cm. If this is confirmed radiologically, orthopaedic referral is advised to consider surgical intervention such as epiphysiodesis.