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ORIGINAL ARTICLE
Year : 2022 | Volume
: 30
| Issue : 2 | Page : 29-31
Windblown hand
Kuldeep Singh, Krittika Aggarwal
Department of Burns and Plastic Surgery, PtBD Sharma PGIMS, Rohtak, Haryana, India
Correspondence Address:
Dr. Krittika Aggarwal
Department of Burns and Plastic Surgery, PtBD Sharma PGIMS, Rohtak, Haryana
India
Source of Support: None, Conflict of Interest: None
DOI: 10.4103/tjps.tjps_52_21
Introduction: Windblown hand or congenital ulnar drift of hand is a rare deformity which affects all the digits including the thumb and can lead to functional impairment. Associated syndromes affecting the head, face, and feet should be ruled out. Windblown hand has three main features: (1) Contracture of the fingers with flexion deformity, (2) adduction contracture of the thumb causing narrowing of the first webspace, and (3) ulnar deviation of the fingers at the metacarpophalangeal joint. Early treatment is effective. Methods: We performed surgical correction in three cases of bilateral windblown hands. The extensor digitorum tendons were repositioned over the metacarpophalangeal heads by longitudinal plication of radial sagittal bands. In one case, skin contracture release for the first webspace was also done. Splinting with radial deviation and extension at metacarpophalangeal joint was done for 3 weeks. The average follow-up time was 6 months. Results: No skin necrosis or infection was seen in postoperative period. The patient satisfaction ranged from very good to excellent. For all the patients, first, the nondominant hand was operated, then all opted for surgical correction of the opposite hand as well. Conclusions: Windblown hand can lead to functional impairment and needs early correction. Parents and patients should be counseled about the chances of recurrence and incomplete correction. However, surgical treatment and subsequent physiotherapy can improve the functional range and aesthetics of the hand.
Keywords: Congenital, hand, windblown hand
Congenital ulnar drift of hand, also known as windblown hand, is a known but rare congenital anomaly. It was first described by Boix in 1897.[1] It has not been classified into a particular group according to Oberg, Manske, Tonkin Classification of congenital anomalies of the hand and upper limb.[2] Zancolli and Zancolli considered it to be a type of segmental Arthrogyroposis.[3] They classified it further into three types:
Type 1-Fasciocutaneous deformity, with short skin and abnormal subcutaneous bandsType 2-short tendons of the wrist, thumb, and fingersType 3-short ligaments and capsules with bony deformity.Various syndromes like Freeman Sheldon syndrome have been associated with the condition. It can involve hands, feet and head.[4],[5],[6] The exact management protocol is not known. Early management with splinting is advised to decrease functional deformity but the success is limited.[7] Surgery is needed in most cases. We document the result of three cases who presented during the last 5 years.
MethodsA retrospective review of three cases who presented with ulnar drift of hand during 2015–2020 was done. All patients were men of 10–15 years of age with bilateral hand involvement. No associated feet or craniofacial anomalies were present. Patients were planned for surgical correction of hands-non dominant in the first stage and dominant in the second if opted for, under General Anaesthesia. The relevant data has been shown in [Table 1].
First, skin contractures over the palmar aspect were released using multiple Z-plasty if required. Then incision over metacarpal heads was made and deepened. After dissection, extensor expansion, extensor digitorum tendon, and its sagittal bands were visualised. In all patients, long extensor tendons were present in intermetacarapal space on the ulnar side and were not in the center. Associated abnormality of intrinsic muscles was ruled out. Plication of radial sagittal bands was done longitudinally using Nylon 4-0 to centralize the extensor tendon over the respective metacarpal joint. After ensuring on table correction wound closure was done with Nylon 4-0 and splint age was done.
Splinting with radial deviation and extension at metacarpophalangeal joint was done for 3 weeks. The period between the correction of both hands was 4 and 6 months. Postoperative results were assessed at 6 weeks and 4 months after surgery and, at final follow-up, using the Wood and Biondi classification[8]-Excellent: Normal function and cosmesis; Good: Almost normal function, satisfactory appearance; Fair: Some contractures that interfere with normal function; Poor: Contracted hand that cannot function in activities of daily living. Average follow-up time after correction of both hands was 6 months.
Results[Table 1] shows the details of the patients who underwent the surgery. Operating time ranged from 1 to 2 h. In all cases, extensor tendons were seen in the valley between the metacarpal heads. All the patients underwent re-positioning of extensor digitorum by plication of radial sagittal bands. Skin contracture release for fingers was done by Z plasty on the palmar side. Skin contracture release for the thumb by multiple Z-plasty was done in one patient. No recurrence was seen in the immediate or late postoperative period. The patient satisfaction ranged from very good to excellent. All the patients opted for surgical correction of the opposite hand as well. The recorded satisfaction was the same in the opposite hand as well. [Figure 1] and [Figure 2] show the preoperative and postoperative photographs after surgical correction of left and right hand for case 3.
Figure 1: Showing preoperative (a,b) and postoperative (c,d)photographs for case 3 for left hand correction
Figure 2: (a,b) Showing preoperative photograph for right hand for case 3. (c and d) The photographs(candd) show postoperative results at 1year. Discussion Windblown hand is a known congenital deformity but rare. The windblown hand can be considered as positioned along a continuum of deformity, from mild distal arthrogryposis to clasped thumb to the most severe windblown hand. The windblown hand is a common feature of distal arthrogryposis, which consists of 10 types which include Freeman-Sheldon syndrome and Gordon syndrome.[9] Windblown hand has three main features: (1) Contracture of the fingers with flexion deformity, (2) adduction contracture of the thumb causing narrowing of the first webspace, and (3) ulnar deviation of the fingers at the metacarpophalangeal joint. There are numerous theories to explain the aetiology and pathogenesis of the windblown hand.[3],[10] The “extensor failure” theory described by Fisk et al. in 1974, is significant because the authors noted the anatomical absence of normal extensor tendons.[9],[11] Al-Harthy and Rayan[12] hypothesized that hypoplasia or absence of the sagittal band of the extensor mechanism is the cause of the middle finger in palm flexion deformity and possibly the ulnar drift deformities. It is well known that sagittal bands contribute to the stability of extensor tendons and prevent ulnar and radial deviation at the level of metacarpophalangeal joints. This fact is well illustrated by sagittal band disruption.
Our intra-operative observations included metacarpophalangeal joint instability with sagittal band hypoplasia and volar skin shortening. Identification of the abnormal anatomy in the windblown hand allows the reconstruction of a more functional hand. The treatment protocol is not well defined. Early splintage has been advised[13] and shown to be effective. Surgical treatment is advised before the age of 3 years. However, in our case, most of the patients had presented late. Various authors have documented their techniques for the correction of ulnar drift of the hand.[7],[8],[9],[12],[14] An algorithm has also been suggested.[9] The first step is the release of skin contracture using multiple Z-plasty or skin grafts. If the thumb is involved, flexor pollicis tendon lengthening may be needed. The next step is identifying the tendons and ligaments at metacarpophalangeal joints. The extensor digitorum tendons may be subluxated in the valley between metacarpals, intrinsic muscles may be short or ulnar collateral ligament may be tight. This might need intrinsic muscle Transfer and plication of extensor tendons radially after repositioning them.[8],[14] Osteotomies have also been advised especially in adults,[14] but in our study, the deformity was not so severe. We performed longitudinal plication of radial sagittal bands to correct the subluxation extensor tendons. A similar technique has been reported in one other study for congenital ulnar drift.[12] However, the authors did not report the long-term follow-up results. Recurrence of the deformity is a known complication. Due to the paucity of long-term studies, as the condition is not common, the extent of functional deficit in case of recurrence is not well known. However, it has been documented that both satisfaction was more with treated hands than untreated ones.[8]
ConclusionsWindblown hand or congenital ulnar drift of the hand is a rare deformity which affects all the digits and can lead to functional impairment. Associated syndromes affecting the head, face and feet should be ruled out. Early treatment is effective. At the time of surgery, all the anomalous structures should be identified. Parents and patients should be counselled about the chances of recurrence and incomplete correction. However, surgical treatment and subsequent physiotherapy can improve the functional range of the hand.
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Conflicts of interest
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References
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