Publication:

Split hand/foot malformation with longitudinal deficiency of tibia: novel rehabilitation approach of a rare syndrome

Background Split hand/foot malformation with longitudinal deficiency of tibia (SHFLD1) is an extremely rare congenital anomaly, even rarer are the descriptions in the literature of suitable techniques of treatment. Most of the literature available are case reports, based on genetic studies. This study highlights on functional management rather than anatomic correction of deformity. A clinician may not come across a patient with such a rare congenital disorder during his entire career.

Methods On retrospective review of hospital record from July 2008 to July 2018, we observed 5 cases of tibial deficiency associated with split hand and or split foot anomalies and diagnosed as SHHLD syndrome. Clinical and radiological analysis was done to plan out surgical and rehabilitation protocol for individual cases.

Results As per Flatt’s grouping of split hand, 3 limbs (37.5%) were group-1 type, 3 limbs (37.5%) of group-2 type and group-3 type was seen in 2 limbs (25%) of a patient with bilateral involvement. With respect to Jone’s radiological classification system, 4 limbs (57%) were of type Ia, 2 limbs (29%) were having type IV deformity and 1 limb (14%) was with type II deformity. One case agreed for amputation was fitted with above knee prosthesis and another one with custom designed bend knee prosthesis. Eldest case was rehabilitated with tricycle and modified orthosis. Surgical correction was tried in one case.

Conclusions Functional rehabilitation instead of anatomic correction should be tried at late presentation. Cleft closure should be done before development of functional adaptability.