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Ulnar-sided cleft hand
Ulnar-Sided Cleft Hand Michael A. Tonkin, MD, Jagdeep Nanchahal, MD, PhD, Sam Kwa, MD, Sydney, Australia Three cases of ulnar-sided cleft hand are presented in which clefting was accompanied by ring finger absence in 2 and by small finger abduction, supination, shortening, and camptodactyly in all 3. An approach to surgical reconstruction is described with marked improvement in appearance. (J Hand Surg 2002;27A:493– 497. Copyright © 2002 by the American Society for Surgery of the Hand.) Key words: Cleft hand, congenital anomalies.
Central longitudinal deficiency is associated with polydactyly and syndactyly.1 The deficiency may involve one or more rays, with the absence of the middle ray the most common.2 Ulnar-sided defects are rare. Kato et al3 reported 3 cases of clefting between the ring and small fingers without absence. These cases were associated with hypoplasia of the small finger and vascular anomalies. Miura4 reported 11 cases in which all had hypoplasia of the small finger and limitation of proximal joint motion. We describe 3 cases of ulnar-sided cleft, 2 in association with ring finger ray absence, and the surgical approach to the resultant deformities.
Patients and Methods Three cases of ulnar-sided cleft are presented. The fourth ray was absent in cases 1 and 2 (Figs. 1, 2). In case 3 the fourth ray was present with clefting between the ring and small finger as in the cases of Kato et al3 and Miura4 (Fig. 3). Syndactyly was From the Department of Hand Surgery and Peripheral Nerve Surgery, Royal North Shore Hospital, and the University of Sydney, Sydney, Australia. Received for publication on August 3, 2001; accepted in revised form February 8, 2002. No benefits in any form have been received or will be received from a commercial party related directly or indirectly to the subject of this article. Reprint requests: Michael A. Tonkin, MD, Department of Hand Surgery, Royal North Shore Hospital, St Leonards NSW 2065 Australia. Copyright © 2002 by the American Society for Surgery of the Hand 0363-5023/02/27A03-0015$35.00/0 doi:10.1053/jhsu.2002.32960
present in all cases between the index and middle fingers and in case 3 between the middle and ring finger. The first web and thumb were normal in all cases. All cases had characteristic deformities of the small finger: shortening, supination, abduction, and camptodactyly. Patient 2 had symphalangism of the interphalangeal joints of the small finger and distal interphalangeal joint of the middle finger. Surgical correction was done in stages (Table 1). The first stage consisted of closure of the cleft with a Barsky flap2 and corrective osteotomy of the small finger. The osteotomy was done through the shaft of the fifth metacarpal, and the deformity was corrected by adduction, derotation, and lengthening using an iliac corticocancellous graft. The average graft length was 5 mm (range, 4 – 6 mm). Fixation was achieved with K-wires into the fourth metacarpal (Fig. 1D). The K-wires were removed at 6 weeks. Reconstruction of the transverse metacarpal ligament was not done. Subsequently an osteotomy of the small finger proximal phalanx was performed to improve extension of the proximal interphalangeal joint and to address any residual rotational deformity. Syndactyly releases deepened adjacent webs, improving the relative position of the cleft. In case 2 further surgery involved rotational osteotomies of the second and third metacarpals to correct pronation of the index finger and supination of the middle finger. A cartilaginous remnant was excised between the index and middle finger metacarpal heads. Progress of the osteotomy was followed by sequential radiographs to assess union of the osteotomy The Journal of Hand Surgery 493
494 Tonkin, Nanchahal, and Kwa / Ulnar-Sided Cleft Hand
Figure 1. Case 1. (A) Preoperative appearance. (B) Preoperative x-ray. (C) Appearance after cleft closure and osteotomy before syndactyly release. (D) Early postoperative x-ray.
site and relative metacarpal length. The small finger lengthening obtained was measured as a ratio of the length of the fifth metacarpal to the adjacent metacarpal. Parents and patients were questioned as to their satisfaction with appearance and function.
metacarpal was observed in cases 1 and 2, indicating an inability of the small finger to maintain the lengthening initially obtained (Table 2). All patients and parents were satisfied with the improved appearance of the hand and denied any functional loss.
Results
Discussion
After the osteotomy a gradual decline in the ratio of the length of the fifth metacarpal to the adjacent
Ulnar clefting is a rare deformity. The cases previously presented by Kato et al3 and Miura4 de-
The Journal of Hand Surgery / Vol. 27A No. 3 May 2002 495
Figure 2. Case 2. (A) Preoperative appearance. (B) Preoperative x-ray. (C) Postoperative appearance.
scribed clefting between the ring and small fingers without absence of any digits. Miura4 had difficulty in deciding whether such cases should be categorized as a failure of formation or a failure of differentiation. Two of our cases showed absence of the ring finger ray, the third being similar to those reported previously. Logically the presence of a cleft suggests absence of tissue formation, with digit absence indicating an increasing severity of reduction (teratologic sequence) with increasing severity leading to absence of multiple digits.5 The concept of Ogino1 and others, which recognizes the association of syndactyly, polydactyly, and clefting, also incorporates the idea of fusion of parts, in our cases this being the incorporation of the ring finger ray into the middle finger ray. Ogino1 suggested that this association be classified as a “failure of induction of rays” rather than as a “central longitudinal deficiency” as proposed by the International Federation of Societies for Surgery of the Hand—a modification of the original Swanson classification.6 The combination of deficient tissue on the ulnar side of the hand (ring finger) with anomalies of the small finger may suggest these anomalies are consistent with a classification of “ulnar longitudinal deficiency.” If the current definition of central is accepted as index, middle, and ring rays, it would be presumptuous to question what is clearly an unsatisfactory classification on the basis of these 3 cases. The classification remains the best we have. The combination of radial ray deficiencies with forearm
ulnar deficiency means that the ability to define an ulnar deficiency on the basis of ray anomalies is questionable. Currently we believe that the anomaly we describe best lies within the scope of the central longitudinal deficiencies. All 3 patients presented with anomalies restricted to one limb only and were without a family history of an inheritance pattern, a phenomenon that may be explained by variable expressivity and reduced genetic penetrance. Alternatively, a spontaneous gene mutation may have occurred in each and one would expect the most usual form of inheritance, that of autosomal dominance, to be present subsequently. Autosomal recessive and X-linked inheritance patterns of central longitudinal deficiency also have been described.7 Surgical correction of cleft deformities usually is done to improve appearance. Closure of the cleft is not difficult. Hypoplasia, abduction, and supination of the small finger and camptodactyly of the proximal interphalangeal joint pose substantial problems, however. If the web were to be placed appropriately relative to adjacent webs, it would be placed at or beyond the proximal interphalangeal joint of the short small finger. Deepening of adjacent webs where syndactyly exists would improve the relative position of the small finger web. Miura4 documented a patient in whom the small finger was excised but also described a metacarpal osteotomy in another patient to elongate the digit. We attempted to address the problems of shortening, abduction, and malrota-
496 Tonkin, Nanchahal, and Kwa / Ulnar-Sided Cleft Hand
Figure 3. Case 3. (A) Preoperative appearance. (B) Preoperative x-ray. (C) Postoperative appearance. (D) Postoperative x-ray.
tion through a 1-stage metacarpal osteotomy with lengthening by iliac crest bone graft. This procedure has been successful in obtaining length in the short term, but in 2 patients that relative improvement in Table 1. Surgical Management Age at Surgery
Case
MCP Osteotomy and Barsky Flap2
Syndactyly Release
1 2
9 mo 14 mo
3
14 mo
5y 24 mo 3 y (index/middle) 2y
MCP, metacarpophalangeal.
Proximal Phalangeal Osteotomy 5y 3 y 3 mo 3y
length has diminished with time. Further lengthening procedures are possible but perhaps at the risk of metacarpophalangeal joint extension and proximal interphalangeal joint flexion contractures. It is doubtful that alternative methods of lengthening would confer greater potential for prolonged growth unless a growth plate is included with a bone graft. The site of the rotational deformity is difficult to determine but seems to be at the metacarpophalangeal joint with supination of the proximal phalanx. This may be a developmental attempt to adapt to the adjacent cleft. Apparent supination is increased by the proximal interphalangeal joint flexion deformity. We found it necessary to perform a second osteot-
The Journal of Hand Surgery / Vol. 27A No. 3 May 2002 497
Table 2. Radiologic Ratio of Fifth Metacarpophalangeal Joint to Third or Fourth Metacarpophalangeal Joint Case
Before Surgery
After Surgery
Final
1 2 3
62% 77% 79%
80% 95% 95%
73% at 3 y 6 mo 96% at 2 y 4 mo 81% at 4 y 10 mo
omy at the proximal interphalangeal level to improve further the position of rotation of the fifth ray. This osteotomy may be combined with an extension osteotomy of the proximal phalanx to decrease the flexion deformity of the proximal interphalangeal joint but at the expense of full flexion. We have not attempted a soft tissue release of the proximal interphalangeal joint in these cases. The surgical approach, by using established procedures of corrective osteotomy with lengthening of the fifth metacarpal,
closure of cleft, proximal interphalangeal osteotomy, and syndactyly release of adjacent webs has improved markedly the overall appearance of the hand in these cases.
References 1. Ogino T. Teratogenic relationship between polydactyly, syndactyly and cleft hand. J Hand Surg 1990;15B:201–209. 2. Barsky AJ. Cleft hand: classification, incidence and treatment. J Bone Joint Surg 1964;46A:1707–1709. 3. Kato S, Ishii S, Ogino T, Shiono H. Anomalous hands with cleft formation between the fourth and fifth digits. J Hand Surg 1983;8:909 –913. 4. Miura T. Cleft hand involving only the ring and small fingers. J Hand Surg 1988;13A:530 –535. 5. Maisels DO. Lobster-claw deformities of the hands and feet. Br J Plast Surg 1970;23:269 –282. 6. Swanson AB. A classification for congenital limb malformations. J Hand Surg 1976;1:8 –22. 7. Nunes ME, Schutt G, Kapur RP, et al. A second autosomal split hand/split foot locus maps to chromosome 10q24-q25. Hum Mol Genet 1995;4:2165–2170.
Central longitudinal deficiency is associated with polydactyly and syndactyly.1 The deficiency may involve one or more rays, with the absence of the middle ray the most common.2 Ulnar-sided defects are rare. Kato et al3 reported 3 cases of clefting between the ring and small fingers without absence. These cases were associated with hypoplasia of the small finger and vascular anomalies. Miura4 reported 11 cases in which all had hypoplasia of the small finger and limitation of proximal joint motion. We describe 3 cases of ulnar-sided cleft, 2 in association with ring finger ray absence, and the surgical approach to the resultant deformities.
Patients and Methods Three cases of ulnar-sided cleft are presented. The fourth ray was absent in cases 1 and 2 (Figs. 1, 2). In case 3 the fourth ray was present with clefting between the ring and small finger as in the cases of Kato et al3 and Miura4 (Fig. 3). Syndactyly was From the Department of Hand Surgery and Peripheral Nerve Surgery, Royal North Shore Hospital, and the University of Sydney, Sydney, Australia. Received for publication on August 3, 2001; accepted in revised form February 8, 2002. No benefits in any form have been received or will be received from a commercial party related directly or indirectly to the subject of this article. Reprint requests: Michael A. Tonkin, MD, Department of Hand Surgery, Royal North Shore Hospital, St Leonards NSW 2065 Australia. Copyright © 2002 by the American Society for Surgery of the Hand 0363-5023/02/27A03-0015$35.00/0 doi:10.1053/jhsu.2002.32960
present in all cases between the index and middle fingers and in case 3 between the middle and ring finger. The first web and thumb were normal in all cases. All cases had characteristic deformities of the small finger: shortening, supination, abduction, and camptodactyly. Patient 2 had symphalangism of the interphalangeal joints of the small finger and distal interphalangeal joint of the middle finger. Surgical correction was done in stages (Table 1). The first stage consisted of closure of the cleft with a Barsky flap2 and corrective osteotomy of the small finger. The osteotomy was done through the shaft of the fifth metacarpal, and the deformity was corrected by adduction, derotation, and lengthening using an iliac corticocancellous graft. The average graft length was 5 mm (range, 4 – 6 mm). Fixation was achieved with K-wires into the fourth metacarpal (Fig. 1D). The K-wires were removed at 6 weeks. Reconstruction of the transverse metacarpal ligament was not done. Subsequently an osteotomy of the small finger proximal phalanx was performed to improve extension of the proximal interphalangeal joint and to address any residual rotational deformity. Syndactyly releases deepened adjacent webs, improving the relative position of the cleft. In case 2 further surgery involved rotational osteotomies of the second and third metacarpals to correct pronation of the index finger and supination of the middle finger. A cartilaginous remnant was excised between the index and middle finger metacarpal heads. Progress of the osteotomy was followed by sequential radiographs to assess union of the osteotomy The Journal of Hand Surgery 493
494 Tonkin, Nanchahal, and Kwa / Ulnar-Sided Cleft Hand
Figure 1. Case 1. (A) Preoperative appearance. (B) Preoperative x-ray. (C) Appearance after cleft closure and osteotomy before syndactyly release. (D) Early postoperative x-ray.
site and relative metacarpal length. The small finger lengthening obtained was measured as a ratio of the length of the fifth metacarpal to the adjacent metacarpal. Parents and patients were questioned as to their satisfaction with appearance and function.
metacarpal was observed in cases 1 and 2, indicating an inability of the small finger to maintain the lengthening initially obtained (Table 2). All patients and parents were satisfied with the improved appearance of the hand and denied any functional loss.
Results
Discussion
After the osteotomy a gradual decline in the ratio of the length of the fifth metacarpal to the adjacent
Ulnar clefting is a rare deformity. The cases previously presented by Kato et al3 and Miura4 de-
The Journal of Hand Surgery / Vol. 27A No. 3 May 2002 495
Figure 2. Case 2. (A) Preoperative appearance. (B) Preoperative x-ray. (C) Postoperative appearance.
scribed clefting between the ring and small fingers without absence of any digits. Miura4 had difficulty in deciding whether such cases should be categorized as a failure of formation or a failure of differentiation. Two of our cases showed absence of the ring finger ray, the third being similar to those reported previously. Logically the presence of a cleft suggests absence of tissue formation, with digit absence indicating an increasing severity of reduction (teratologic sequence) with increasing severity leading to absence of multiple digits.5 The concept of Ogino1 and others, which recognizes the association of syndactyly, polydactyly, and clefting, also incorporates the idea of fusion of parts, in our cases this being the incorporation of the ring finger ray into the middle finger ray. Ogino1 suggested that this association be classified as a “failure of induction of rays” rather than as a “central longitudinal deficiency” as proposed by the International Federation of Societies for Surgery of the Hand—a modification of the original Swanson classification.6 The combination of deficient tissue on the ulnar side of the hand (ring finger) with anomalies of the small finger may suggest these anomalies are consistent with a classification of “ulnar longitudinal deficiency.” If the current definition of central is accepted as index, middle, and ring rays, it would be presumptuous to question what is clearly an unsatisfactory classification on the basis of these 3 cases. The classification remains the best we have. The combination of radial ray deficiencies with forearm
ulnar deficiency means that the ability to define an ulnar deficiency on the basis of ray anomalies is questionable. Currently we believe that the anomaly we describe best lies within the scope of the central longitudinal deficiencies. All 3 patients presented with anomalies restricted to one limb only and were without a family history of an inheritance pattern, a phenomenon that may be explained by variable expressivity and reduced genetic penetrance. Alternatively, a spontaneous gene mutation may have occurred in each and one would expect the most usual form of inheritance, that of autosomal dominance, to be present subsequently. Autosomal recessive and X-linked inheritance patterns of central longitudinal deficiency also have been described.7 Surgical correction of cleft deformities usually is done to improve appearance. Closure of the cleft is not difficult. Hypoplasia, abduction, and supination of the small finger and camptodactyly of the proximal interphalangeal joint pose substantial problems, however. If the web were to be placed appropriately relative to adjacent webs, it would be placed at or beyond the proximal interphalangeal joint of the short small finger. Deepening of adjacent webs where syndactyly exists would improve the relative position of the small finger web. Miura4 documented a patient in whom the small finger was excised but also described a metacarpal osteotomy in another patient to elongate the digit. We attempted to address the problems of shortening, abduction, and malrota-
496 Tonkin, Nanchahal, and Kwa / Ulnar-Sided Cleft Hand
Figure 3. Case 3. (A) Preoperative appearance. (B) Preoperative x-ray. (C) Postoperative appearance. (D) Postoperative x-ray.
tion through a 1-stage metacarpal osteotomy with lengthening by iliac crest bone graft. This procedure has been successful in obtaining length in the short term, but in 2 patients that relative improvement in Table 1. Surgical Management Age at Surgery
Case
MCP Osteotomy and Barsky Flap2
Syndactyly Release
1 2
9 mo 14 mo
3
14 mo
5y 24 mo 3 y (index/middle) 2y
MCP, metacarpophalangeal.
Proximal Phalangeal Osteotomy 5y 3 y 3 mo 3y
length has diminished with time. Further lengthening procedures are possible but perhaps at the risk of metacarpophalangeal joint extension and proximal interphalangeal joint flexion contractures. It is doubtful that alternative methods of lengthening would confer greater potential for prolonged growth unless a growth plate is included with a bone graft. The site of the rotational deformity is difficult to determine but seems to be at the metacarpophalangeal joint with supination of the proximal phalanx. This may be a developmental attempt to adapt to the adjacent cleft. Apparent supination is increased by the proximal interphalangeal joint flexion deformity. We found it necessary to perform a second osteot-
The Journal of Hand Surgery / Vol. 27A No. 3 May 2002 497
Table 2. Radiologic Ratio of Fifth Metacarpophalangeal Joint to Third or Fourth Metacarpophalangeal Joint Case
Before Surgery
After Surgery
Final
1 2 3
62% 77% 79%
80% 95% 95%
73% at 3 y 6 mo 96% at 2 y 4 mo 81% at 4 y 10 mo
omy at the proximal interphalangeal level to improve further the position of rotation of the fifth ray. This osteotomy may be combined with an extension osteotomy of the proximal phalanx to decrease the flexion deformity of the proximal interphalangeal joint but at the expense of full flexion. We have not attempted a soft tissue release of the proximal interphalangeal joint in these cases. The surgical approach, by using established procedures of corrective osteotomy with lengthening of the fifth metacarpal,
closure of cleft, proximal interphalangeal osteotomy, and syndactyly release of adjacent webs has improved markedly the overall appearance of the hand in these cases.
References 1. Ogino T. Teratogenic relationship between polydactyly, syndactyly and cleft hand. J Hand Surg 1990;15B:201–209. 2. Barsky AJ. Cleft hand: classification, incidence and treatment. J Bone Joint Surg 1964;46A:1707–1709. 3. Kato S, Ishii S, Ogino T, Shiono H. Anomalous hands with cleft formation between the fourth and fifth digits. J Hand Surg 1983;8:909 –913. 4. Miura T. Cleft hand involving only the ring and small fingers. J Hand Surg 1988;13A:530 –535. 5. Maisels DO. Lobster-claw deformities of the hands and feet. Br J Plast Surg 1970;23:269 –282. 6. Swanson AB. A classification for congenital limb malformations. J Hand Surg 1976;1:8 –22. 7. Nunes ME, Schutt G, Kapur RP, et al. A second autosomal split hand/split foot locus maps to chromosome 10q24-q25. Hum Mol Genet 1995;4:2165–2170.