Correction of “Wrist” Deformity in Radial Dysplasia

Abstract

Background:Radial dysplasia affects 1 in 6,000 to 8,000 births, classically presenting with a shortened, bowed ulna and radially deviated hand. The optimal treatment remains unclear, with several opposing approaches advocated. This review aims to clarify the long-term outcomes of nonsurgical and surgical treatment of the “wrist” deformity.Methods:The Embase, MEDLINE, PubMed, Cochrane Central, ClinicalTrials.gov, and World Health Organization International Clinical Trials Registry Platform (ICTRP) databases were searched for published and unpublished studies reporting long-term outcomes of surgical or nonsurgical treatment of children with radial dysplasia. Results were not restricted by date or language. Primary outcomes were hand-forearm angle, ulnar length, and “wrist” active range of motion (ROM). Studies were assessed using the Grades of Recommendation, Assessment, Development and Evaluation (GRADE) criteria. Data for the change in hand-forearm angle were pooled using random-effects meta-analysis, and mean differences and 95% confidence intervals were obtained. Primary outcome data at last follow-up were pooled, and means and standard deviations were obtained. The PROSPERO registration of this study was CRD42016036665.Results:Of 104 studies identified, 12 were included in this review. Five were retrospective cohort studies and 7 were case series. No randomized studies were found. Study quality was low or very low according to the GRADE criteria. The hand-forearm angle of nonsurgically treated patients worsened during childhood, from 66° to 84°, whereas “wrist” active ROM, at 61°, was better than that for most surgically treated patients. Ulnar length with nonsurgical treatment was predicted to be 64% of normal, but was not directly reported. Isolated soft-tissue release provided a modest reduction in hand-forearm angle compared with nonsurgical treatment. Soft-tissue distraction with centralization or radialization achieved the best hand-forearm angle correction (16° radial deviation). Radialization maintained better “wrist” active ROM (46°) and ulnar length than centralization. Microvascular second metatarsophalangeal joint transfer yielded better reported “wrist” active ROM (83°) and good ulnar length compared with other surgical techniques, but a slightly worse hand-forearm angle (28°).Conclusions:There was low-quality evidence that soft-tissue distraction plus centralization or radialization achieved the best correction of the hand-forearm angle for children with radial dysplasia.Level of Evidence:Therapeutic Level IV. See Instructions for Authors for a complete description of levels of evidence.

Radial Club Hand Treated by Paley Ulnarization Generation 3: Is This the New Centralization?

“…Murphy et al [ 11 ] summarized the non-operative treatment vs. soft-tissue distraction with radialization or centralization in correction of wrist deformity in RCH. Patients treated with centralization had a greater improvement in the HFA of 71° compared with patients treated with radialization alone, which showed an improvement of 49°.…”

“…The growth rate in RCH has been shown to be three quarters to half of that of the normal ulna [ 9 ]. Centralization of the wrist is the most common surgical approach for the management of RCH [ 10 ], despite documentation showing high recurrence of radial deviation, growth arrest of the distal ulnar physis and wrist stiffness [ 11 , 12 ].…”

Vascularized second metatarsophalangeal joint transfer for radial deficiency – an update

“…An important question is whether we can achieve better results with a microvascular bone/joint transfer than with conventional centralization or radialization procedures, which are nowadays commonly combined with preoperative distraction. Epiphyseal MTP-II joint transfer is the only operation that simultaneously adds a stabile post on the radial side of the wrist and includes also a mid-size joint that will allow a good motion arc (Murphy et al., 2017). A growing metatarsal bone with the second MTP-II joint will reconstruct the radial half of the wrist.…”

RADIATE – Radial Dysplasia Assessment, Treatment and Aetiology: protocol for the development of a core outcome set using a Delphi survey

“…We recently published a systematic review of the long-term outcomes of both surgical and conservative treatments for RD [9]. This was prospectively registered with the PROSPERO database (CRD42016036665) and conducted using the Cochrane highly sensitive search strategy.…”

“…The surgical treatment of the soft tissues is highly variable. A recent systematic review by our group found that patients suffer poor forearm growth and some degree of recurrent radial wrist deviation, whether treated surgically or conservatively [9]. Currently, there is no core outcome set (COS) specifically for RD, although a generalised standard set for congenital upper limb anomalies is due to be published soon [10].…”