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Year : 2021  |  Volume : 18  |  Issue : 1  |  Page : 18-20

Ulnar club hand: A rare case report

Department of Orthopaedics, Doctors Hospital, Kasargod, Kerala, India

Date of Submission27-Mar-2021
Date of Acceptance03-Apr-2021
Date of Web Publication25-Jul-2021

Correspondence Address:
Prasanth Srinivasan
3/452, Bharathi Nagar, Melapalapatti, Mohanur Taluk, Namakkal - 637 017, Tamil Nadu
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/joasis.joasis_8_21

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Ulnar club hand is a rare postaxial partial or complete longitudinal deficiency of the ulna, with relative incidence of one-tenth to one-third of radial deficiencies. The absence of postaxial metacarpal and digital bones is a frequent finding in this rare disorder. However, we report a rare case of unilateral ulnar club hand with the absence of two preaxial digits and two preaxial metacarpals.

Keywords: Hemimelia, hypoplasia, longitudinal deficiency, ulnar club hand

How to cite this article:
Srinivasan P. Ulnar club hand: A rare case report. J Orthop Assoc South Indian States 2021;18:18-20

How to cite this URL:
Srinivasan P. Ulnar club hand: A rare case report. J Orthop Assoc South Indian States [serial online] 2021 [cited 2023 Apr 1];18:18-20. Available from: https://www.joasis.org/text.asp?2021/18/1/18/322303

  Introduction Top

Ulnar deficiencies are malformations in which there is longitudinal failure of formation along the postaxial border of the upper extremity. The other terms for this deformity include paraxial ulnar hemimelia, ulnar dysplasia, and congenital absence of the ulna. It has an estimated incidence of 1/100,000 live births,[1] which makes it the second rarest upper extremity anomaly. Both sporadic and syndromic cases have been reported, although the former is more common. Fixed flexion deformity of the elbow with associated radiohumeral synostosis is a frequent finding. Hereby, we present a newborn male child of nonconsanguineous marriage, with rare association of ulnar club hand with the absence of two preaxial digits and two preaxial metacarpals, without radiohumeral synostosis.

  Case Report Top

A 27-year-old primi delivered a male baby with left upper limb deformity. It was full term vacuum-assisted delivery with right mediolateral episiotomy, and the birth weight was 2.25 kg. She was not on any drug with a known teratogenic effect. She neither smokes nor drinks alcohol. Her family history was unremarkable. Clinical examination revealed short left upper limb where the length of the forearm segment was reduced, while the arm segment was preserved. The child had only three digits with increased web space between the first and second digits [Figure 1]. The radial most (first) finger had only one flexion crease in the plane of other digits, so we concluded as malrotation of the thumb. The other two digits had two flexion creases, with the second digit being the longest than the ulnar most digit. There was ulnar deviation at the wrist and fixed flexion deformity at the elbow with bowed forearm.
Figure 1: Clinical photographs showing deformity in the left upper limb

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On radiological examination, the ulna was totally absent with hypoplastic, bowed radius [Figure 2]. The entire humerus and shoulder joint were normal. The radius was acutely bend at its proximal end with only three metacarpals and three fingers [Figure 3]. The ulnar most metacarpal is smaller than the second metacarpal. The ossification of the carpal bone occurs at the end of 1 year and so its deficiency was radiographically uncertain. The contralateral limb was normal, and there was no involvement of any other organ system. The mother was advised to start stretching exercises on day 1 itself, during breastfeeding at least 6 times in a day. Stretching exercises were opposite to the direction of deformity to prevent contractures. Regular follow-up of the patient was advised to parents to identify early contractures and the increasing deformity, which may warrant surgical intervention.
Figure 2: X-ray showing absent ulna

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Figure 3: X-ray showing trident hand

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  Discussion Top

The critical period of ulnar deficiency is much earlier than that of other anomalies, which could be the reason for a decreased incidence of this disease entity. Partial deficiency of the ulna with the absence of postaxial metacarpal, digital bones, and radiohumeral synostosis are frequent findings. Patients with postaxial syndactyl, camptodactyly, and clinodactyly[2] are also reported in the literature. However, our patient had rare association of ulnar club hand with the absence of two preaxial digits and two preaxial metacarpals, without radiohumeral synostosis, which makes this a unique presentation.

Ulnar hemimelia may be a part of Poland syndrome, Klippel–Feil syndrome, Goltz–Gorlin syndrome, Cornelia de Lange syndrome, or femur–fibula–ulna syndrome. Its prognosis depends on the severity of syndrome. Since our patient had an isolated limb deficiency with normal systemic examination, it could be sporadic in nature. Several different classification systems have been proposed for ulnar ray deficiency. Ogden et al. provided a classification based on the severity of the ulnar deformity.[3] Swanson described four types of ulnar deficiency. Cole and Manske added thumb/first web deformity to the ulnar ray deficiency.[4] Our patient comes under Swanson classification Type 2, Cole and Manske classification Type c.

The management of ulnar club hand is challenging and the goal is to improve function of the affected limb. In infants, serial casting could be used and should be considered until correction is achieved, and then removable splints may be used to maintain the correction. Indications for surgical intervention are syndactyly, severe radial bowing with the presence of ulnar anlage, dislocation of the radial head with limited elbow extension, forearm pronation and supination, as well as internal rotation deformity of humerus. Most of the affected children, especially if unilateral disease, are managed without surgical intervention. The unilateralism of hemimelia in this patient has been variably reported in many literature and confers better prognosis in terms of functionality.[5]

  Conclusion Top

Ulnar hemimelia is a rare postaxial partial or complete longitudinal deficiency of the ulna. Early intervention is essential to improve the function of the affected limb by preventing contractures, which will reduce the degree of atrophy from disuse, thereby bringing down the need for surgical intervention in future.

Declaration of patient consent

The authors certify that they have obtained all appropriate patient consent forms. In the form the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.


I would like to express my deepest gratitude to Dr. Binoy P. S, Dr. Sreejith. P. K, and Dr. Renjith K. R for their unconditional support behind this work. I would like to appreciate the patient/parents for participation in this study.

Financial support and sponsorship


Conflicts of interest

There are no conflicts of interest.

  References Top

Lamba A, Kumar N, Krishna C, Chhabra S. Ulnar longitudinal deficiency: A rare case report and review. Int J Res Orthop 2021;7:159-61.  Back to cited text no. 1
Malik S, Afzal M. Ulnar aplasia, dysplastic radius and preaxial oligodactyly: Rare longitudinal limb defect in a sporadic male child. J Res Med Sci 2013;18:818-21.  Back to cited text no. 2
Ogden JA, Watsen HK, Bohne W. Ulnar dysmelia. J Bone Joint Surg Am 1976;58:467-75.  Back to cited text no. 3
Cole RJ, Manske PR. Classification of ulnar deficiency according to the thumb and first web. J Hand Surg Am 1997;22:479-88.  Back to cited text no. 4
Shafi M, Hui JH. Common paediatric orthopaedic problems in the upper limb. Singapore Med J 2006;47:654-9.  Back to cited text no. 5


  [Figure 1], [Figure 2], [Figure 3]


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