Split-hand/foot malformation (SHFM) is a genetically heterogeneous congenital limb reduction defect characterized by the deficiencies of central rays of the autopod. Tandem duplications at 10q24 locus account for approximately 20% of all SHFM cases. Here, we report five affected individuals from four unrelated Indian families with SHFM3 caused by microduplication of 10q24 locus showing varied clinical presentations. This report substantiates and extends the current understanding of this rare, multifaceted, and complex condition.

Introduction  Acrorenal syndrome is autosomal recessive inherited disorder commonly associated with congenital renal disorders and ipsilateral hand/foot anomalies. The hand and foot deformities corrections are challenging to achieve a good functional and cosmetic result. We described a case of acrorenal syndrome with suppressed radial elements and absent thumb in whom we were able to reconstruct the hand. Case Report  A 5-year-old girl presented to us with right renal agenesis and right hand/foot deformity. The hand showed a cleft hand with central deficiency, index finger hypoplastic, and syndactylyzed to middle finger, absent thumb. The hand was nonfunctional because of absent thumb. To improve the functions, it was decided to proceed with thumb reconstruction. The middle finger was pollicized to regain tripod grip and thereby the functions was enhanced. Discussion  The cleft hand belongs to \"failure of finger ray induction group\" in classification by \"International Federation of Societies for Surgery of the Hand (IFSSH).\" Absence of thumb and first web space makes it a strong indication for surgical reconstruction. In our case, thumb was addressed by pollicization of middle finger and we were able to provide a good tripod grip.

This study aimed to compare clinical and radiographical results of total or partial resection of the cross-bone in the management of children with cleft hand. Nine children with 10 cleft hands who underwent resection of the cross-bone were retrospectively identified and divided into two groups based on type of resection: Group T (total resection) including 5 children (5 affected hands; 3 boys; mean age = 3.5 (range, 2-5) years), and Group P (partial resection) including 4 children (5 affected hands; 3 boys; mean age = 3.2 (range, 2-5) years). Mean follow-up was 68 (range, 60-85) months in group T and 47 (range, 40-60) months in group P. To assess clinical status, postoperative cosmetic satisfaction was evaluated by asking the parents, and cosmetic appearance was rated using a visual analogue scale (VAS) at the follow-up. In the radiographical evaluation, change in cleft divergence following reconstruction was assessed as the metacarpal divergence angle between the index and the ring finger metacarpals. Overall, cosmetic satisfaction was rated \"very satisfied\" or \"satisfied\" in 7 of the 10 hands, and functional satisfaction as \"very satisfied\" or \"satisfied\" in all. Mean postoperative cosmetic aspect on VAS was 6.4 (range, 5-8) in group T, and 6.2 (range, 4-8) in group P (p = 0.99). Mean metacarpal divergence angle significantly decreased from 42.2° (range, 35-52°) and 40.2° (range, 36-46°) preoperatively to 21.2° (range, 15-35°) and 19.8° (range = 12-31°) at 3-year follow-up in groups T and P, respectively (p < 0.001 for each group). Both total and partial cross-bone resection provided satisfactory clinical and radiographical medium-term results for of children with cleft hand.

We present two rare microsurgical reconstructions. A case with phocomelia was treated with lengthening of soft tissues following vascularized fibula grafting with epiphysis, and a case with cleft hand was reconstructed with spare-part toe transfer.

Experimental studies showed that central polydactyly, syndactyly, and cleft hand might appear when the same teratogenic factor acts on embryos at the same developmental stage. These observations and some clinical cases support the concept that a common etiologic mechanism is involved in the development of these malformations. We report a clinical case that demonstrates the association previously observed in experimental studies. Here, a patient with unilateral nonsyndromic cleft hand, central polydactyly, first web syndactyly, osseous syndactyly between the ring and long fingers, and minor thumb hypoplasia was presented.

Hand oligodactyly refers to a developmental defect which results in the presence of less than 5 digits on a hand. It presents as a component of 4 main categories of congenital upper limb malformations. Herein, we present a 50-year-old man with an unusual form of hand oligodactyly which is characterized by atypical cleft hand accompanied by complex syndactyly between the thumb and the index finger. Accurate characterization of hand oligodacticity can sometimes be challenging due to unusual phenotypic appearances accompanying the abnormality. Radiological evaluation is of great importance for correct identification and classification of such complex hand anomalies, and the treatment should be highly individualized in these patients.

Cleft hand or split hand foot malformation is a sequence of phenotypes, from a minor shortening of the central digit to a complete absence of the third ray and in the most severe cases, absence of two, three or four rays. It is a rare but spectacular presentation usually involving both hands and feet. Inheritance is mostly autosomal dominant but sporadic cases without family history are also reported, resulting from a de novo mutation/deletion/duplication. Intra-familial clinical variability is the rule, with incomplete penetrance. X-linked or autosomal recessive inheritance has also been described. To date, seven subgroups of split hand foot malformation have been identified and seven loci are currently known. Anatomical records have enhanced our knowledge of this group of disorders of the hands and feet and allowed us to improve surgical procedures and long-term outcome.

Background: Cleft hand is a rare and congenital deformity that affects hands and feet and can be associated with other malformations. The objective was to evaluate the epidemiological and clinical aspects of cleft hand patient in a case series. Methods: Baseline characteristics associated with this deformity, such as sociodemographic characteristics, affected upper limb side, family history, clinical manifestations, and the degree of deficiency according to Barsky, Manske and Halikis, and Valenti classifications, were analyzed in 38 patients treated in the Department of Orthopedic Surgery of the Irmandade da Santa Casa de Misericórdia de São Paulo, Brazil. Results: A predominance of typical hands as classified by Barsky, types II and IV by Manske and Halikis, and type IV by Valenti was found. A high frequency of typical cases (55.3%), as defined by Barsky, had a positive family history (P = .031) and were associated with other clinical manifestations (44.7%), when compared with atypical cleft hand patients (P < .001). Conclusion: In our study, there were more typical cleft hands than atypical, and they were more commonly associated with family history and other clinical manifestations.

UNASSIGNED: Phenotypic expressions of the congenital cleft hand are variable and might baffle even the experienced as to the choice of surgery. The morphological parameters defining the anomaly dictate not only the functional capacity of the anomalous hand but also the degree of possible surgical restoration. Despite a large number of classifying systems available, none encompass all the relevant issues.
UNASSIGNED: The purpose of this work is to present an all-inclusive and universally acceptable classification of the deformity which would graphically represent the entire gamut of possible presentations; principal and associated. Furthermore, based on such staging, the choice of surgical procedure and the stages of surgical intervention can be standardised to ensure the best results for the patient.
UNASSIGNED: This study is based on a series of 27 patients with a total of 38 cleft hands.
UNASSIGNED: The necessity to include and to assess all determinants of function and complexity in these hands to standardise the choice of management, gave rise to the DAST system of classification; an acronym for all the morphological determinants of the anomaly (D = Digits missing, A = Associated anomalies in the hand, S = Site of cleft, T = Functional state of the Thumb). Numerical values were assigned to each component in increasing order of complexity. Score for each determinant as well as the aggregate score indicates the degree of complexity in a graphic manner. The DAST classification has a predictive value in choice of procedure and prognosticating surgical outcomes.

OBJECTIVE: To evaluate the implications of the transverse bone in cleft hand by assessing outcomes after reconstruction in comparison with a control group.
METHODS: This study is a retrospective review of 23 hands in 18 patients following surgical reconstruction of the cleft hand. Eleven hands had a transverse bone component, and 12 hands (control group) did not. Patients and their families were contacted to assess overall satisfaction following reconstruction. Clinical and radiographic records were reviewed to assess aesthetic and functional outcomes, the need for additional surgery, and radiographic divergence angles.
RESULTS: There was no difference in aesthetic or functional subjective outcomes. There was no statistically significant difference in any objective outcome measure between the two groups. The use of the cleft for pinch was more dependent on the status of the index finger and the preoperative thumb-index webspace rather than the presence of a transverse bone. Eleven (4 transverse and 7 control) hands required additional surgery to address abnormal function or posture of the index and ring fingers. Preoperative radiographic divergence angles were larger in the transverse bone group than in the control group, whereas postoperative divergence angles were nearly equivalent.
CONCLUSIONS: Similar outcomes between the two groups demonstrate that the presence of a transverse bone in cleft hand was not associated with worse outcomes following cleft reconstruction. Preoperative narrowing of the thumb webspace and postoperative index finger metacarpophalangeal joint abnormality are associated with worse functional outcomes.
METHODS: Therapeutic Level III.