A child with type I ulnar ray deficiency

Ulnar ray deficiency is the least common type of congenital longitudinal deficiency, and it shows several distinct manifestations, including various finger deformities [1].

To date, no reports have been published describing ulnar ray deficiency with multiple finger deformities, including central polysyndactyly, ectrodactyly, and first-web space narrowing. We describe the case of a patient with type I multiple-finger deformities.

A 6-year-old girl had a shorter left arm and 3 digits, with central polysyndactyly on her left hand ([Fig. 1]). The radiographic findings are presented in [Fig. 2]. The extra digit interfered with her ability to grasp and pinch, and she was only able to use the thumb and middle finger for these purposes. Therefore, excision of the non-functional index finger was performed, and the web space was reconstructed to improve the range of motion ([Figs. 3], [4]).

Fig. 1. Clinical photograph of the left hand. The index finger was present in the form of central polysyndactyly, and the ring and little fingers were absent.

Fig. 2. Radiograph of the left extremities. The supernumerary digit on the index finger had a short proximal and distal phalanx and only the head of the metacarpal bone. The hamate, pisiform, triquetrum, and lunate were absent.

Fig. 3. Operative design for reconstruction of web space and correction of syndactyly.

Fig. 4. Findings at 1 day postoperatively. (A) Palmar side and (B) dorsal side.

At 1 year postoperatively, her index finger still had limitations of motion and joint function, and power had not recovered. She usually used her thumb and middle finger to grab, pinch, and grip ([Fig. 5]).

Fig. 5. Findings at 1 year postoperatively. (A) Resting position and (B) grasping position.

Although the appropriate treatment of ulnar deficiency is not clear-cut, previous studies have emphasized the importance of operating upon hand deformities to improve functionality, and it has been stated that syndactyly should be released between 6 and 12 months of life [2] [3]. In this case, the patient’s index finger did not have normal anatomy, and the operation was delayed until 6 years of age. At that time, it could be conducted in a way that would not affect her finger movement. Based on this case, the timing of surgical treatment of complicated hand deformities should be planned carefully in order to maximize function.

Notes

Ethical approval

The study was performed in accordance with the principles of the Declaration of Helsinki. Written informed consent was obtained.

Patient consent

The patients provided written informed consent for the publication and the use of their images.

Conflict of Interest

No potential conflict of interest relevant to this article was reported.

This article was presented as a poster at the Federation of European Societies for Surgery of the Hand meeting on June 22–25, 2016, in Santander, Spain.

• References

• 1 Malik S, Afzal M. Ulnar aplasia, dysplastic radius and paraxial oligodactyly: rare longitudinal limb defect in a sporadic male child. J Res Med Sci 2013; 18: 818-21
• 2 Christopher CS, Neufeld SK. Ulnar ray deficiency. Hand Clinics 1998; 14: 65-75
• 3 Broudy AS, Smith RJ. Deformities of the hand and wrist with ulna deficiency. J Hand Surg Am 1979; 4: 304-15

Publication History

Received: 14 February 2017

Accepted: 22 August 2017

Article published online:
22 May 2022