211:
this surgical intervention the wrist and the second toe are prepared for transfer at the same time. The ipsilateral second toe MTP joint, together with its metatarsal arteries, its extensor and flexor tendons and its dorsal nerves to the skin, is harvested for transfer. The distal and middle phalanx of the toe are removed. The transferred toe, consisting of the metatarsal and proximal phalanx, is fixed between the physis of the ulna and the second metacarpal, or the scaphoid. The tendons of the toe are attached to those of the radial flexor and extensors muscles of the wrist to create more stability to the MTP joint. K-wires are placed to fixate the bones in the desired position. Once the bones are secured anastomosis are made between the vessels of the toe and the vessels of the forearm. After revascularization of the toe, the skin paddle is placed and the skin is closed.
69:, radial side of the carpal bones and thumb. Hypoplasia of the distal humerus may be present as well and can lead to stiffness of the elbow. Radial deviation of the wrist is caused by lack of support to the carpus, radial deviation may be reinforced if forearm muscles are functioning poorly or have abnormal insertions. Although radial longitudinal deficiency is often bilateral, the extent of involvement is most often asymmetric.
170:
arrest of the ulna, and thereby resulting in an even shorter forearm. Sestero et al. reported that ulnar growth after centralization reaches from 48% to 58% of normal ulnar length, while ulnar growth in untreated patients reaches 64% of normal ulnar length. Several reviews note that centralization can only partially correct radial deviation of the wrist and that studies with longterm follow-up show relapse of radial deviation.
174:
109:
A fifth type was added by
Goldfarb et al. describing a radial dysplasia with participation of the humerus. In this classification only anomalies of the radius and the humerus are taken in consideration. James and colleagues expanded this classification by including deficiencies of the carpal bones with a normal distal radius length as type 0 and isolated thumb anomalies as type N.
162:
carpal bones. If the ulna is significantly bent, osteotomy may be needed to straighten the ulna. After placing the wrist in the correct position, radial wrist extensors are transferred to the extensor carpi ulnaris tendon, to help stabilize the wrist in straight position. If the thumb or its carpometacarpal joint is absent, centralization can be followed by
37:
108:
Classification of radial dysplasia is practised through different models. Some only include the different deformities or absences of the radius, where others also include anomalies of the thumb and carpal bones. The Bayne and Klug classification discriminates four different types of radial dysplasia.
193:
described another operation technique, for treatment of radial dysplasia, which is called radialization. During radialization the metacarpal of the index finger is pinned onto the ulna and radial wrist extensors are attached to the ulnar side of the wrist, causing overcorrection or ulnar deviation.
161:
If radial tissues are still too short after soft-tissue stretching, soft tissue release and different approaches for manipulation of the forearm bones may be used to enable the placement of the hand onto the ulna. Possible approaches are shortening of the ulna by resection of a segment, or removing
153:
More severe types (Bayne type III en IV) of radial dysplasia can be treated with surgical intervention. The main goal of centralization is to increase hand function by positioning the hand over the distal ulna, and stabilizing the wrist in straight position. Splinting or soft-tissue distraction may
214:
Vilkki et al. have conducted a study on 19 forearms treated with vascularized MTP-joint transfer with a mean follow-up of 11 years which reports an ulnar length of 67% compared to the contralateral side. De Jong et al. described in a review that compared to study outcomes on centralization, Vilkki
169:
Radial angulation of the hand enables patients with stiff elbows to reach their mouth for feeding; therefore treatment is contraindicated in cases of extension contracture of the elbow. A risk of centralization is that the procedure may cause injury to the ulnar physis, leading to early epiphyseal
144:
In cases of a minor deviation of the wrist, treatment by splinting and stretching alone may be a sufficient approach in treating the radial deviation in RD. Besides that, the parent can support this treatment by performing passive exercises of the hand. This will help to stretch the wrist and also
210:
Prior to the actual transfer of the MTP-joint of the second toe soft-tissue distraction of the wrist is required to create enough space to place the MTP joint. When after several weeks enough space has been created through distraction, the actual transfer of the MTP joint can be initiated. During
206:
of the second toe is transferred to the radial side of ulna, creating a platform that provides radial support for the wrist. The graft is vascularised and therefore maintains its ability to join the growth of the supporting ulna.
65:, is a congenital difference occurring in a longitudinal direction resulting in radial deviation of the wrist and shortening of the forearm. It can occur in different ways, from a minor anomaly to complete absence of the
693:
158:
are removed to create a notch for placement of the ulna. A different approach is to place the metacarpal of the middle finger in line with the ulna with a fixation pin.
145:
possibly correct any extension contracture of the elbow. Furthermore, splinting is used as a postoperative measure trying to avoid a relapse of the radial deviation.
493:"Changing Paradigms in the Treatment of Radial Club Hand: Microvascular Joint Transfer for Correction of Radial Deviation and Preservation of Long-term Growth"
166:. Postoperatively, a long arm plaster splinter has to be worn for at least 6 to 8 weeks. A removable splint is often worn for a long period of time.
181:
device went in. The thumb in the picture is the index finger removed and stitched to where the thumb should be located on a normal hand.
80:. In case of an inherited condition, several syndromes are known for an association with radial dysplasia, such as the cardiovascular
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405:
698:
76:
rather than an inherited condition. It is one of the possible co occurring birth defects of the embryonic mesoderm within
93:
81:
177:
A treated radial club hand with Type N-IV using centralization with many marks where fixation pins via an
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Sestero AM, Van Heest A, Agel J (2006). "Ulnar growth patterns in radial longitudinal deficiency".
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Bates SJ, Hansen SL, Jones NF (2009). "Reconstruction of congenital differences of the hand".
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73:
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be used preceding the centralization. In classic centralization central portions of the
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Netscher DT, Baumholtz MA (2007). "Treatment of congenital upper extremity problems".
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687:
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66:
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Manske PR, Goldfarb CA (2009). "Congenital failure of formation of the upper limb".
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reported a smaller deviation postoperatively and a lower severity of the relapse.
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This overcorrection is believed to make relapse of radial deviation less likely.
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553:
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96:
during upper limb development, intrauterine compression, or maternal drug use (
588:
461:
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508:
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Villki reported a different approach in During this procedure a vascularised
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Bednar MS, James MA, Light TR (2009). "Congenital longitudinal deficiency".
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630:
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124:
Type V: Complete absent radius and manifestations in the proximal humerus
421:
673:
615:"Vascularized metatarsophalangeal joint transfer for radial hypoplasia"
72:
The incidence is between 1:30,000 and 1:100,000 and it is more often a
36:
229:
404:
Goldfarb CA, Manske PR, Busa R, Mills J, Carter P, Ezaki M (2005).
172:
406:"Upper-extremity phocomelia reexamined: a longitudinal dysplasia"
198:
Vascularized metatarsophalangeal (MTP)-joint transfer
663:
667:
26:
21:
445:"The treatment of longitdunal radial deficiency"
694:Congenital disorders of musculoskeletal system
8:
92:.Other possible causes are an injury to the
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491:de Jong JP, Moran SL, Vilkki SK (2012).
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118:Type II: Hypoplastic radius in miniature
301:
299:
297:
295:
241:
114:Type 0: Deficiency of the carpal bones
44:Radial club hand with thumb missing (
7:
378:10.1097/01.prs.0000258535.31613.43
14:
131:can refer to the last 3 types.
122:Type IV: Complete absent radius
120:Type III: Absent distal radius
112:Type N: Isolated thumb anomaly
63:radial longitudinal deficiency
30:Radial longitudinal deficiency
1:
320:10.1097/PRS.0b013e3181a80777
116:Type I: Short distal radius
715:
554:10.1016/j.jhsa.2006.03.016
278:10.1016/j.jhsa.2009.09.002
589:10.1016/j.hcl.2008.10.005
462:10.3109/03093649109164642
43:
34:
509:10.4055/cios.2012.4.1.36
140:Splinting and stretching
94:apical ectodermal ridge
631:10.1055/s-2008-1081403
182:
699:Developmental biology
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422:10.2106/JBJS.D.02011
410:J Bone Joint Surg Am
84:and the hematologic
613:Vilkki SK. (2008).
449:Prosthet Orthot Int
366:Plast Reconstr Surg
308:Plast Reconstr Surg
78:VACTERL association
183:
82:Holt–Oram syndrome
681:
680:
443:Lamb DW. (1991).
179:external fixation
74:sporadic mutation
52:
51:
16:Medical condition
706:
665:
653:
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619:Semin Plast Surg
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601:
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572:
566:
565:
537:
531:
530:
520:
497:Clin Orthop Surg
488:
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434:
433:
401:
390:
389:
372:(5): 101e–129e.
361:
340:
339:
314:(1): 128e–143e.
303:
290:
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261:
59:radial club hand
57:, also known as
55:Radial dysplasia
39:
22:Radial dysplasia
19:
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569:
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416:(12): 2639–48.
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225:Ulnar dysplasia
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17:
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5:
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668:Classification
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660:External links
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625:(3): 195–212.
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567:
542:J Hand Surg Am
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272:(9): 1739–47.
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149:Centralization
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104:Classification
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86:Fanconi anemia
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583:(2): 157–70.
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186:Radialization
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164:pollicization
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129:absent radius
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29:
25:
20:
622:
618:
580:
576:
570:
548:(6): 960–7.
545:
541:
535:
503:(1): 36–44.
500:
496:
455:(2): 100–3.
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413:
409:
369:
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311:
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269:
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191:Buck-Gramcko
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111:
107:
90:TAR syndrome
71:
62:
58:
54:
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46:ectrodactyly
266:J Hand Surg
98:thalidomide
27:Other names
688:Categories
236:References
577:Hand Clin
204:MTP-joint
135:Treatment
127:The term
649:20567714
597:19380058
562:16843156
527:22379554
430:16322613
386:17415231
336:13262697
328:19568146
286:19896016
219:See also
640:2884879
518:3288493
471:1923709
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230:Radius
156:carpus
67:radius
332:S2CID
645:PMID
593:PMID
558:PMID
523:PMID
467:PMID
426:PMID
382:PMID
324:PMID
282:PMID
88:and
635:PMC
627:doi
585:doi
550:doi
513:PMC
505:doi
457:doi
418:doi
374:doi
370:119
316:doi
312:124
274:doi
100:).
61:or
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674:D
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