Split hand and foot malformation (SHFM) is a congenital abnormality of the limbs. It is predominantly seen to be affecting the central rays of hand and foot. Bidactyly and monodactyly in hand and foot can also be seen in severe cases. SHFM occurs sporadically but can also be inherited following most commonly an autosomal dominant pattern, but X-linked and autosomal recessive cases have also been reported ^[1]. The incidence of SHFM – is reported to be as low as 1 in 90,000 ^[2].

The patient was 60-year-old male with alleged history of road traffic accident under unknown circumstances, was referred from primary health center for management of long bone injury that the patient had sustained. The patient was an unknown accompanied by staff of organization who look after the destitutes. Furthermore, the patient had a low IQ and it was not possible to elicit proper history. On primary survey, the patient was found to have deformity, crepitus, and tenderness at left thigh. There was no external wound and patient was found to be calm, conscious, and hemodynamically stable. Primary emergency management started and IV fluids administered with injectable analgesics. On further physical examination, there was complete absence of 2^nd and 3^rd digits of both feet along with lobster claw like malformation in the right hand (Fig. 1 and 2). The patient sent for plain radiographs after stabilization of fractured limb by a splint. Radiographs revealed fracture of shaft of femur left side with absence of 2^nd and 3^rd proximal and distal phalanges of both feet (Figs. 3 and 4). All routine investigations were sent and skeletal traction applied to fractured limb through upper tibial pin. Ultrasonography (USG) abdomen, electrocardiogram (ECG), 2D ECHO, and chest radiograph showed no abnormality. The fracture shaft of femur was managed with closed reduction and internal fixation with interlocking nail. Since patient was not demanding improved function of deformed limbs, deformity correction was not done.

SHFM is a group of hereditary skeletal disorders with possible variable phenotypes. It is a clinically heterogeneous condition and has a wide spectrum ranging from shortening of the single digit to monodactyly ^[3]. Furthermore, in cases of SHFLD, a variant there is absence of long bones ^[4]. SHFM can be seen affecting single limb or multiple limbs. It may occur in syndromic forms associated with extra limb formation. Various genetic researches have been conducted and literature reveals mapping of seven different genetic loci associated with the isolated form of SHFM, the chromosomal defects being 7q rearrangements involving genes DLX5, DLX6, DSS1 for SHFM1 ^[5], Xq26 for SHFM2 ^[6], 10q24 duplication involving DACTYLIN for SHFM3 ^[7], mutations in P63 gene at 3q for SHFM4 ^[8], deletion at 2q31 for SHFM5 ^[9], WNT10B mutation involving chromosome 12q13 ^[10] for SHFM6, and duplication 7p13.3 for SHFLD (a variant with absent long bone) ^[11]. Surgical intervention may be required in SHFM to improve functions or appearance of limb. Prosthesis for SHFM is also available. Possibility of recurrence in future siblings should be explained to the parents. Antenatal diagnosis of SHFM can be done with ultrasonography ^[12]. Due to rarity of this malformation, the literature on surgical management of SHFM is limited and one such literature published which reports about an individual who was presented with bilateral SHFM and bidactylous hands. The thumbs were hypoplastic, whereas ulnar digit was broad maybe due to fusion of the two rays also known as super digit. During the surgery, they identified all tendons and muscles of forearm. They also managed to identify flexor and extensor tendons of the missing fingers. These tendons were identified in forearm and traced to the hand but tendons were ended in cleft where they formed few loops in middle of cleft. It was reported that extra tendons maybe the reason for camptodactyly (digital flexion deformity of proximal interphalangeal joint) and rotation of fingers while in flexion. It was also stated that cautious division of cleft and lateral side of finger with excision of extra tendons improved functional and cosmetic outcome of patients ^[13].

Patients with SHFMs should undergo screening for other congenital anomalies. ECG, 2D ECHO, chest radiograph, and USG abdomen should be done. Genetic analysis ideally should be done to identify mutations involved. Surgical intervention is only required when patient demands improved function of limb.

• Diagnosis of associated congenital defects should be done and addressed accordingly.
• Patients to be counseled for malformations if he/she is planning for pregnancy.
• Surgical intervention is required if patient demands improved function of the limb.