
==== Front
JPRAS Open
JPRAS Open
JPRAS Open
2352-5878
Elsevier

S2352-5878(24)00117-7
10.1016/j.jpra.2024.07.018
Case Report
Delayed presentation of ulnar drift of the hand; A case report
Aljayban Aeshah Abdulrahman
Al-Dhafer Bassmh A. Baldhafar@kfu.edu.sa
⁎
King Faisal University, Hufof, Saudi Arabia
⁎ Corresponding author. Baldhafar@kfu.edu.sa
05 8 2024
9 2024
05 8 2024
41 394399
1 5 2024
28 7 2024
© 2024 Published by Elsevier Ltd on behalf of British Association of Plastic, Reconstructive and Aesthetic Surgeons.
2024

https://creativecommons.org/licenses/by-nc-nd/4.0/ This is an open access article under the CC BY-NC-ND license (http://creativecommons.org/licenses/by-nc-nd/4.0/).
Purpose of paper

To present a case of delayed presentation of congenital ulnar drift of the hand.

Background

Congenital ulnar drift, frequently known as windblown hand, represents ulnar deviation of fingers with or without other malformations that are usually present since birth. This deformity is rare and can present as an isolated entity or as a part of a syndrome. It is usually detected and managed surgically at birth. However, to our knowledge, delayed presentation beyond the first years of life has not been reported in the literature. Therefore, this paper presents a case of delayed presentation of congenital ulnar drift of the hand and reviews the available literature to highlight the importance of early detection and address associated deformities.

Case Summary

A 12-year-old boy visited a hand surgery clinic complaining of bilateral painless hand deformities since birth that were neither progressive nor associated with any activity restrictions. Despite seeking medical advice from different institutions, no underlying pathology was detected. Physical examination findings supported congenital ulnar drift of the hand, which was further confirmed with radiographs findings of ulnar drift at the metacarpophalangeal joints without presence of any other hand malformations. Management was non-operative because of the lack of functional impairment.

Keywords

Congenital
Ulnar drift
Windblown
Hand
Deformity
==== Body
pmcIntroduction

Congenital ulnar drift (CUD) of the hand, commonly known “windblown hand”, represents a rare deformity the was initially described by Boix in 1897.1,2

The hallmarks of this deformity have been illustrated by Wood et al., who defined the characteristic deformities as bilateral flexion contracture of the fingers, ulnar drift of the fingers at the metacarpophalangeal level, and thumb adduction with narrowing of the webspace, the latter of which is considered the leading cause of functional impairment.1, 2, 3, 4 These features result from varying degrees of soft and bony tissue involvement extending to and not limited to the midpalmar fascia, natatory ligament and sagittal bands of extensors.2,3,5

The development of CUD is different from that associated with rheumatoid arthritis, which is termed Jaccoud's arthropathy.5 Furthermore, CUD could be part of various associated syndromes that were hypothesized of by some authors as synonyms of this complex condition.2,6

Given the functional and aesthetic impact of this deformity; namely webspace narrowing and thumb functional limitation, early surgical intervention was warranted1,3 However, reports on delayed presentation of this condition and its manifestations are lacking

This paper presents a case report of a delayed presentation of bilateral ulnar drift in a 12-year-old boy.

Case presentation

A healthy 12-year-old boy presented to the hand surgery clinic complaining of bilateral hand deformities that were present at birth.

Past medical and surgical histories were insignificant, along with a normal perinatal and developmental history.

The noted deformities were located in the metacarpophalangeal joints (MPJ) of both hands with notable ulnar deviation. The patient reported no pain, progression of deformity, or impairment of daily activities. However, medical attention was sought by parents due to concerns about the cosmetic appearance and the potential for progression.

The deformities were investigated since birth and over the years by multiple orthopedic surgeons and family physicians who ruled out the presence of congenital musculoskeletal anomalies such as spine deformities, developmental dysplasia of the hip and clubfeet, trauma, or juvenile rheumatoid arthritis.

Physical examination revealed a healthy young male with no aberrant dysmorphic features with bilateral ulnar drift of both hands that was more prominent on the left hand and centered over the MPJs. The patient also had webbing of the first webspace. The flexor tendons function was maintained without intrinsic tightness. The extensor tendons were found to sublux minimally over the MPJs upon flexion. However, the patient's range of motion (ROM) was almost full. The deformities at the MPJs were passively correctable but were associated with a sense of tightness centered volarly. Both wrist joints and elbow joints had a full ROM with no laxity. No sensory deficits were observed. Based on these findings, this deformity was classified as a mild deformity based on Zancolli and Zancolli's classification.7 (Figure 1, Figure 2) (Vid. 1)Figure 1 Dorsal view of the hands demonstrating the classical features of congenital ulnar drift, namely ulnar deviation at MPJ along with thumb adduction and narrowing of webspace.

Figure 1

Figure 2 Volar view of the hands demonstrating the deformity.

Figure 2

Plain radiographs showed no skeletal abnormalities apart from the ulnar drift at the MPJs of the fingers. (Figure 3)Figure 3 Xray of both hands showing the ulnar deviation at MPJs with lack of features suggesting Jaccoud's arthropathy.

Figure 3

Following a lengthy discussion with the family and patient, the family opted for non-operative treatment with close monitoring of deformity progression and/or newly developed symptoms.

Discussion

CUD of the hand, otherwise known as windblown hands is a rare deformity that produces a visually striking deformity due to the complex interplay of multiple factors, leading to the characteristic bilateral flexion and ulnar deviation of fingers MPJs with narrowing of the first webspace.2,3,6

Since the original description by Biox in 1897, this isolated deformity has been reported to be associated with multiple syndromes, such as arthrogryposis, and given numerous synonyms such as whistling face syndrome and Freeman-Sheldon syndrome, denoting the complex interplay leading to this condition.1, 2, 3,6

Multiple local pathological changes have been postulated as the underlying causes of this morphology. The absence of extensors as a leading cause for this deformity was hypothesized by Fisk et al. as they suggested that this allowed the normal flexor tendons to function unopposed leading to flexion deformity.4,6,7 Other authors have also suggested other forms of extensor abnormalities. Al-Harthy and Rayan observed an absence of sagittal bands while Wood et al. observed a resultant dislocation of extensor tendons due to an abnormal natatory ligament.4,8 Zancolli and Zancolli attributed the deformity to tightening of the skin and retinaculum cutis which is formed by the midpalmar fascia and natatory ligament, Accordingly, they classified the deformities into three types: mild deformities, severe deformities and deformities with bony involvement.2,4,6,7 These soft tissue changes lead to changes in biomechanics which also lead to skeletal changes at the metacarpal heads as reported by Flat and Borema.6

Both surgical and non-surgical management strategies have been reported. To halt progression, non-surgical options utilizing splints are advised at an early age for mild deformities.9

Surgical management is recommended at an early age to avoid of bony procedures.1 Reported techniques range from addressing the skin using Z-plasties and flaps to addressing soft tissue via contracture releases, tendons lengthening and centralizing, and bony procedures in the form of arthrodesis and de-rotational osteotomies.1,9 However, these procedures carry the risks of recurrence and guarded outcomes.1,4,10

Delayed presentation of CUD has never been reported in the literature, the only other paper discussing a late presentation is that of a surgeon who was eventually diagnosed with Jaccoud's arthropathy.5

In this case report, the patient presented with mild bilateral deformities present since birth with concerns regarding cosmesis, etiology, and long-term sequelae. Given that it was a mild deformity without functional impairment, non-surgical management was selected.

Conclusion

CUD of the hand is a rare and complex deformity that can present as an isolated finding or an associated syndrome. Surgical treatment is based on disease severity and is usually recommended during the early years of life. Delayed presentation is rarely reported and is managed based on functional impairment or unacceptable cosmesis. Excellent postoperative outcomes were difficult to achieve.

Declaration of competing interest

None.

Appendix Supplementary materials

Image, video 1

Consent

Consent was obtained from the legal guardian regarding publishing this case.

Ethical approval

Not required.

Funding

This research did not receive any specific grant from funding agencies in the public, commercial, or not-for-profit sectors.

Acknowledgments

None.

Supplementary material associated with this article can be found, in the online version, at doi:10.1016/j.jpra.2024.07.018.
==== Refs
References

1 Gavaskar K.G. Chowdary N Surgical management of windblown hand: Results and literature review J Child Orthop 3 2 2009 109 114 10.1007/s11832-009-0163-z Epub 2009 Feb 20. PMID:19308621; PMCID: PMC2656951 19308621
2 Rayan G.M. Upton J. III Congenital ulnar drift (windblown hand) Congenital Hand Anomalies and Associated Syndromes 2014 Springer Berlin Heidelberg Berlin, Heidelberg 263 277
3 Wood V.E. Biondi J. Treatment of the windblown hand J Hand Surg Am 15 3 1990 431 438 10.1016/0363-5023(90)90055-v 2348061
4 Wood V.E. Another look at the causes of the windblown hand J Hand Surg Br 19 6 1994 679 682 10.1016/0266-7681(94)90234-8 7706863
5 McKee D. Eliasson S. Griswold J. Congenital ulnar drift in a surgeon Case Rep Orthop 2015 2015 135350 10.1155/2015/135350
6 Grünert J. The windblown hand - diagnosis, clinical picture and pathogenesis Handchirurgie, Mikrochirurgie, plastische Chirurgie: Organ der Deutschsprachigen Arbeitsgemeinschaft für Handchirurgie: Organ der Deutschsprachigen Arbeitsgemeinschaft für Mikrochirurgie der Peripheren Nerven und Gefasse: Organ der V... 36 2–3 2004 117 125 10.1055/s-2004-817873 15162309
7 Zancolli E. Zancolli E. Jr Congenital ulnar drift of the fingers. Pathogenesis, classification, and surgical management Hand Clin 1 3 1985 443 456 10.1016/s0749-0712(21)01374-3 3831046
8 Al-Harthy A. Rayan G.M. Congenital flexion deformity of the middle finger and sagittal band hypoplasia J Hand Surg Am 28 1 2003 123 129 10.1053/jhsu.2003.50001 PMID:12563648 12563648
9 Alzahrani M.A. Farr S. The hand in distal arthrogryposis J Hand Surg Am 47 5 2022 460 469 10.1016/j.jhsa.2021.10.027 Available from 35151520
10 Wimalasiri K.G.K.M. Dematawa P. Familial congenital ulnar drift/windblown hands: Case report in 4 successive generations SL J Perinatal Med 4 2 2023 18 20 10.4038/sljpm.v4i2.71 Available from
